B E R KOW I T Z ’ S

PEDIATRICS
B E R KOW I T Z ’ S
A PRIMARY CARE APPROACH
B E R KOW I T Z ’ S
PEDIATRICS
A PRIMARY CARE APPROACH 6th Edition
PEDIATRICS
Edited by Carol D. Berkowitz, MD, FAAP A PRIMARY CARE APPROACH

PEDIATRICS
B E R KOW I T Z ’ S
The reference of choice for pediatricians, residents, medical physician assistants, pediatric nurse practitioners, and nurses
students, and pediatric nurse practitioners, the newly revised and perfect for use in continuity clinics.
and expanded sixth edition provides clear, practice-oriented This new edition brings you state-of-the-art expertise and
guidance on the core knowledge in pediatrics. Edited by a
leading primary care authority with more than 100 contributors,
insight by Carol D. Berkowitz, MD, FAAP, past president of the Carol D. Berkowitz, MD, FAAP
American Academy of Pediatrics. She is currently executive vice
this edition provides comprehensive coverage of hundreds of chair in the Department of Pediatrics at Harbor-UCLA Medical
topics ranging from temper tantrums and toilet training to
adolescent depression and suicide.
Center and distinguished professor of clinical pediatrics at the
David Geffen School of Medicine at UCLA.
6th Edition
More than 155 (including 5 brand-new) clinical chapters
review pertinent epidemiology and patho­physiology and then
give concise guidelines on what symptoms to look for, what Other AAP resources related to this title
alternative diagnoses to consider, what tests to order, and how to New! Berkowitz’s Pediatrics Instructor’s Guide
treat your patient. 18 SECTION I PRIMARY CARE: SKILLS AND CONCEPTS Chapter 78: head trauma 19

Table 78.2. Modified Glasgow Coma Score for symptoms (eg, headache), physical signs (eg, LOC, amnesia), behav- An epidural hematoma is a collection of blood that accumulates

This is an ideal reference for pediatricians, family physicians,

Younger Children ioral changes, cognitive impairment, or sleep disturbances. Some between the skull bone and the tough outer covering of the brain (ie,
of these minor and subtle neurologic sequelae can last for months dura mater). These are often the result of tears in the middle menin-
Elements Points
after the injury (ie, postconcussion syndrome). Most resolve within geal artery caused by skull fractures. Classically, patients have initial
1. Eye opening a relatively short period, typically 7 to 10 days; however, with more LOC followed by a lucid interval and then rapid deterioration sec-
a. Spontaneous 4 severe trauma the symptoms can last longer. ondary to brain compression. On CT, an epidural hematoma appears

medical students, residents, residency program directors,

b. To command 3 as a large collection of blood with convex borders next to the skull
c. With pain 2 Laboratory Tests (Figure 78.2A). Surgical evacuation is required in most cases.
The subdural hematoma accumulates between the dura and the
d. No response 1 A complete blood cell count and serum electrolyte panel should be
underlying brain tissue. These are associated with skull fractures
2. Verbal responsea performed for all pediatric patients with significant head trauma.
and contusions. On CT, they appear to have a crescent-shaped bor-
Bedside glucose monitoring should be performed in any child with
a. Coos and babbles 5 der (Figure 78.2B). Large subdural hematomas usually require sur-
a head injury with an altered level of consciousness. Toxicology eval-
b. Irritable cry 4 gical evacuation. In infants and young children, these are often the
uation may be indicated in the adolescent who appears to be intox-
c. Cries to pain 3 result of nonaccidental trauma.
icated or has an altered level of consciousness. The infant or child
Diffuse axonal injury (DAI) involves extensive damage to the axo-
d. Moans to pain 2 with an intracranial hemorrhage should undergo screening coag-
nal white matter of the brain that results from shearing forces that
e. None 1 ulation studies (ie, prothrombin time, activated partial thrombo-
typically occur with rapid acceleration or deceleration of the brain
plastin time) as well as a type and screen test or crossmatch, should
3. Motor response (Figure 78.2C). The child with DAI may have normal or nonspecific
Figure 78.1. Functional anatomy of the brain and surrounding structures with surgery be required.
a. Normal spontaneous movement 6 sites of pathology. CT findings.

New in the sixth edition

b. Withdraws to touch 5 1, Caput succedaneum. 2, Subgaleal hematoma. 3, Cephalhematoma. 4, Porencephalic Imaging Studies
or arachnoid cyst. 5, Epidural hematoma. 6, Subdural hematoma. 7, Cerebral contusion. In cases of acute pediatric blunt or penetrating trauma, a noncon-
c. Withdraws to pain 4
8, Cerebral laceration. (From Tecklenburg FW, Wright MS. Minor head trauma in the
trast CT of the head is currently the diagnostic study of choice. It is CASE RESOLUTION
d. Abnormal flexion 3

6th Edition
pediatric patient. Pediatr Emerg Care. 1991;7[1]:40–47, with permission from Wolters The case scenario involves a young child with a significant mechanism of injury,
quite sensitive for the detection of acute hemorrhage and skull frac-
e. Abnormal extension 2 Kluwer Health.) brief LOC, and a depressed, altered mental status. Initial physical findings
ture. It can also provide additional information on the severity of
f. None 1 prompt suspicion of a depressed skull fracture and overlying soft tissue injury.
injury, indicating increased ICP, cerebral edema, or pending herni- Appropriate diagnostic tools after evaluation of airway, breathing, and circula-
In head trauma, primary and secondary brain injury can occur

y All chapters have been reviewed and updated to address

Maximum score 15 ation. Among the findings on CT that indicate severe brain injury tion are cranial CT followed by admission for observation, monitoring, and serial
(Figure 78.1). Primary injury is the structural damage that occurs to
a
Assessment of verbal response is modified in children. are the shift of midline structures, effacement of the sulci, ventric- neurologic examination. Surgical repair of the skull fracture may be necessary.
the cranium and its contents at the time of injury. Secondary injury
ular enlargement or compression, and loss of normal gray/white
is damage to the brain tissue after the initial event. Such damage
Pathophysiology matter differentiation.
may result from hypoxia, hypoperfusion, hypercapnia, hyperther-
An emergent head CT is warranted for any child with altered
Children have significant anatomic differences from adults that pre- mia, and altered glucose or sodium metabolism. The main treat- Selected References

current issues.
dispose them to head trauma and certain types of intracranial injury. mental status, a GCS below 14, penetrating trauma, or focal neuro-
ment strategies for patients who have sustained head trauma focus
logic deficit. The question of which children with minor head trauma Atabaki SM, Stiell IG, Bazarian JJ, et al. A clinical decision rule for cra-
They have a higher center of gravity, an increased head to body on the prevention of secondary brain injury. Primary brain injury
should undergo CT was evaluated in a study of 17,000 children from nial computed tomography in minor pediatric head trauma. Arch Pediatr
ratio, and weaker neck muscles compared with adults. Additionally, can be prevented only through education and safety, such as advo- Adolesc Med. 2008;162(5):439–445 PMID: 18458190 https://doi.org/10.1001/
children have thinner cranial bones and less myelinated brain tis- the Pediatric Emergency Care Applied Research Network (PECARN)
cating for wearing helmets in appropriate situations. archpedi.162.5.439
sue, which predisposes them to intraparenchymal injuries. Whereas database. In this study, a decision rule was retrospectively derived

y Five new chapters, including Health Systems

Brown RL, Brunn MA, Garcia VF. Cervical spine injuries in children: a review
adults are more likely to have focal intracranial hematomas, chil- and then prospectively validated as a method to identify children
Differential Diagnosis at very low risk for intracranial injury. These criteria can be found
of 103 patients treated consecutively at a level 1 pediatric trauma center. J
dren are more likely to develop diffuse cerebral edema. Cerebral Pediatr Surg. 2001;36(8):1107–1114 PMID: 11479837 https://doi.org/10.1053/
CHAPTER 64Even minor head trauma in a child can result in skull fracture or in Box 78.3. If a child is otherwise healthy and meets these criteria,
edema can disrupt cerebral blood flow, resulting in ischemic injury. jpsu.2001.25665
intracranial injuries. Most skull fractures are simple and linear. Other the risk of intracranial injury is extremely low and the child can be
Normally, blood flow to the brain is maintained at a constant Bruce DA. Head trauma. In: Fleisher GR, Ludwig S, Henretig FM, eds. Textbook
fracture types are comminuted, diastatic, basilar, and depressed. safely discharged from the ED or clinic with anticipatory guidance
rate by the process of autoregulation. A complete blood cell count of Pediatric Emergency Medicine. 5th ed. Baltimore, MD: Lippincott Williams

Science, Social Determinants of Health: Principles, and

A comminuted fracture is one involving multiple skull fragments. and return precautions. Neither CT nor a period of observation is

Eating Disorders
and serum electrolyte panel should be performed for all pediatric & Wilkins; 2005>
A diastatic fracture is one with a wide separation at the fracture required. It should be noted that this decision rule was validated as
patients with significant head trauma. With severe brain injury, auto- Crompton EM, Lubomirova I, Cotlarciuc I, Han TS, Sharma SD, Sharma P. Meta-
site. Basilar fractures occur at the base of the skull and often have “rule out” only and meant to identify the child at very low risk. That
regulation is disrupted and blood flow to the brain is determined analysis of therapeutic hypothermia for traumatic brain injury in adult and pedi-
characteristic findings on physical examination (ie, bilateral peri- is, not meeting all the criteria does not mean CT is warranted. atric patients. Crit Care Med. 2017;45(4):575–583 PMID: 27941370 https://doi.
by cerebral perfusion pressure (CPP), which is a measure of the
orbital ecchymosis [ie, raccoon eyes], hemotympanum, postauric- A cerebral contusion is a bruise of the brain tissue and typically org/10.1097/CCM.0000000000002205
mean arterial pressure (MAP) less ICP (CPP = MAP − ICP). Cerebral

Adverse Childhood Experiences: Trauma-Informed Care.

ular ecchymosis [ie, Battle sign]). In a depressed fracture, fragments occurs with a more severe injury, such as a high-speed motor vehi- Dunning J, Daly JP, Lomas JP, Lecky F, Batchelor J, Mackway-Jones K; Children’s
blood flow is therefore compromised when the MAP is too low (ie,
of the skull are displaced inward, potentially damaging intracra- cle crash. A contrecoup contusion may be sustained when the brain Head Injury Algorithm for The Prediction of Important Clinical Events Study
hypotension) or the ICP is too high (ie, cerebral edema). Several of
nial structures. strikes the skull on direct impact, bruising 1 portion of the brain, Group. Derivation of the children’s head injury algorithm for the prediction of
the management strategies in children with severe brain injuries
Head trauma may result in concussion, mild traumatic brain with resulting injury to the side of the brain on rapid deceleration. important clinical events decision rule for head injury in children. Arch Dis Child.
focus on maintaining MAP and reducing ICP; however, control of 2006;91(11):885–891 PMID: 17056862 https://doi.org/10.1136/adc.2005.083980
injury, or intracranial hemorrhage. A concussion is defined as a Clinical manifestations depend on the location of the contusion but
CPP after head injury can be quite difficult. Children have a greater

y A newly created Instructor’s Guide includes advice on how

CASE STUDY trauma-induced impairment of neurologicisfunction. This
andmay occur Giza CC, Kutcher JS, Ashwal S, et al. Summary of evidence-based guideline
capacity for recovery than adults; this is especially true for infants the mother
by her mother because with feels that her daughter too thin often include altered mental status, excessive sleepiness, confusion,
update: evaluation and management of concussion in sports: report of the
is brought the office
toopen or without a loss of consciousness (LOC). Neurologic exami-
at dinner and generally says she is somatic
A 16-year-old
and very young girlchildren, whose sutures and fontanels permit and agitation. Small intraparenchymal hemorrhages and swelling of
mothertoreports thatblood. not eatis much
her daughter doesnation usually normal, but the patient may experience Guideline Development Subcommittee of the American Academy of Neurology.
always appears
expansion of the skullThe
tired. in response edema and online. The teenager claims that she has
not the surrounding tissues are often seen on computed tomography.
Neurology. 2013;80(24):2250–2257 PMID: 23508730 https://doi.org/10.1212/
pills that were advertised
not hungry. Recently, the girl bought diet feels fine and considers herself
why her mother is so upset. She says she
taken the pills, so she does not understand

to best sequence topics in ­continuity clinics and provides

a vegetarian.
healthy because she has recently become although her friends are
public school and attends classes regularly,
The girl is a 10th-grade student at a local She has many friends who
drill team, swim team, and student council.
occasionally truant. She is involved in the drugs, but they occasionally
she nor her friends smoke tobacco or use
have “nicer” figures than she does. Neither menstrual periods are
sexually active and denies a history of abuse. Her

answers to the text case study questions with an emphasis

drink alcohol at parties. The girl is not
3 months prior to this office visit.
irregular, with the last occurring approximately siblings. Although things are “OK” at home, she thinks
and 2 younger
She currently lives with her mother, father, date, but she dislikes that she
her. They have just begun to allow her to
her parents are too strict and do not trust
has a curfew. On the growth chart,
for a thin physique, and vital signs are normal.

on applying chapter concepts and critical thinking

The physical examination is significant body mass index (BMI) is 17 (10th
her height is at the 75th percentile; her
her weight is at the 15th percentile and of the physical examination
was at the 40th percentile. The remainder
percentile). Her weight at a previous visit
is unremarkable.

to the case study. Questions

1. What are the common characteristics
of disordered routine, and body image concerns. Inquiries
focus on symptoms associated with complications
should also
of eating
to esophagitis from

y Case study questions have been enhanced and resources

eating in adolescents? disorders, such as dysphagia secondary
for eating restriction, and
An adolescent may have an atypical presentation recurrent vomiting, constipation from fluid
to eat but rather toxicity from chronic
disorder and may not display a blatant refusal muscle weakness associated with emetine
eating, such as available, so its
may exhibit subtle characteristics of disordered ipecac use (although ipecac is less readily

Berkowitz
physical exercise, or as a detailed menstrual
constant dieting, obsession with a certain effects are seen less frequently), as well

have been revised.

irregular menstruation. history.
present with adolescent’s
The patient with eating disorder also may Particular attention should be paid to the
loss and nutritional or and at school;
general complaints related to weight overall functioning at home, with friends,
Such a patient disorders (eg,
volume deficiencies, such as fatigue or syncope. the presence of other comorbid psychiatric
to standard ideation or
also may exhibit minimal weight gain according depression, anxiety); and a history of suicidal
puberty or progression behavior as a
growth charts or a delay in the onset of

y This edition is completely reorganized into 15 parts using

sexual and/or physical abuse. Out-of-control
of pubertal development. result of substance use also should be assessed.
points to include nervosa and bulimia
2. What are the important historical 3. How is the diagnosis of anorexia
suspected eating
when interviewing the patient with nervosa made?
at risk?
disorder? Which teenagers are considered Diagnostic criteria for anorexia nervosa
(AN) include

a systems-based approach.
eating disorder, intense
When interviewing the patient with suspected restriction of energy intake relative to requirements;
related to changes in or persistent behavior
the history should address specific issues fear of gaining weight or becoming fat,
low-fat diet), eating at a significantly
food preferences (eg, vegetarian, vegan, that interferes with weight gain, even though
calorie counting, weight history, exercise
behaviors, dieting, 15

Student worksheets corresponding to each chapter’s

case study questions are available online in a user-
friendly format so they can be completed to prepare
For other pediatric resources, visit the American for discussions.
Academy of Pediatrics at shop.aap.org.

ISBN 978-1-61002-372-6
90000>

9 781610 023726 AAP

 B E R KOW I T Z ’ S

PEDIATRICS A PRIMARY CARE APPROACH

6th Edition

Carol D. Berkowitz, MD, FAAP

Distinguished Professor of Clinical Pediatrics,
David Geffen School of Medicine at
University of California Los Angeles
Executive Vice Chair,
Department of Pediatrics,
Harbor-UCLA Medical Center
Torrance, CA

BPPCA6e_FM_i-xxiv.indd 1 2/14/20 9:37 AM

 American Academy of Pediatrics Publishing Staff
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The American Academy of Pediatrics is an organization of 67,000 primary care pediatricians, pediatric medical subspecialists, and pediatric surgical
specialists dedicated to the health, safety, and well-being of infants, children, adolescents, and young adults.
The recommendations in this publication do not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into
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Statements and opinions expressed are those of the authors and not necessarily those of the American Academy of Pediatrics.
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This publication has been developed by the American Academy of Pediatrics. The contributors are expert authorities in the field of pediatrics. No commercial
involvement of any kind has been solicited or accepted in development of the content of this publication. Disclosures: Dr Allen disclosed a grant relationship
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consulting relationship with Vifor and with Bristol-Myers Squibb. Dr Kwong disclosed an independent contractor relationship with Thermo-Fisher Scientific.
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 Contributors
Brittany Allen, MD, FAAP Carol D. Berkowitz, MD, FAAP
Assistant Professor, Department of Pediatrics Distinguished Professor of Clinical Pediatrics
University of Wisconsin School of Medicine and Public Health David Geffen School of Medicine at University of California
General Pediatrician and Co-Medical Director of the Pediatric and Los Angeles
Adolescent Transgender Health Clinic Executive Vice Chair, Department of Pediatrics
American Family Children’s Hospital Harbor-UCLA Medical Center
Madison, WI Torrance, CA

David Atkinson, MD Kier Maddox Blevins, MD

Professor of Pediatrics, Department of Pediatrics/Pediatric Cardiology PGY1, Duke Orthopaedic Surgery
University of California Los Angeles Duke University Medical Center
Pediatric Cardiologist, Department of Pediatrics/Pediatric Durham, NC
Cardiology
Harbor-UCLA Medical Center Karen C. Bodnar, MD, IBCLC, FABM, FAAP
Torrance, CA Assistant Professor, Department of Pediatrics
Virginia Commonwealth University School of Medicine
Sarah J. Atunah-Jay, MD, MPH, FAAP Medical Director, Inova Breastfeeding Medicine
Assistant Professor of Pediatrics Department of Pediatrics
Department of Pediatric and Adolescent Medicine Inova Children’s Hospital
Mayo Clinic Falls Church, VA
Rochester, MN
Jori Bogetz, MD, FAAP
Andrew J. Barnes, MD, MPH, FAAP Acting Assistant Professor, Department of Pediatrics
Associate Professor and Fellowship Director Division of Pediatric Bioethics and Palliative Care
Developmental-Behavioral Pediatrics Seattle Children’s Hospital and Research Institute
Department of Pediatrics, University of Minnesota Medical School University of Washington
Minneapolis, MN Faculty, Department of Pediatrics
Seattle Children’s Hospital
Lindsay S. Baron, MD Seattle, WA
Section Head Pediatric Imaging, Department of Radiology
Lowell General Hospital Emily Borman-Shoap, MD, FAAP
Lowell, MA Vice Chair of Education, Pediatric Residence Program Director
Department of Pediatrics, University of Minnesota Medical School
Maneesh Batra, MD, MPH Minneapolis, MN
Professor, Department of Pediatrics
University of Washington Iris Wagman Borowsky, MD, PhD, FAAP
Professor, Department of Pediatrics-Neonatology Professor and Director, Division of General Pediatrics and
Seattle Children’s Hospital Adolescent Health, Department of Pediatrics
Seattle, WA Gisela and E. Paul Konopka Chair in Adolescent Health and
Development
Andrew K. Battenberg, MD University of Minnesota Medical School
Orthopaedic Surgeon, Department of Orthopaedic Surgery Minneapolis, MN
Kaiser Vacaville Medical Center
Vacaville, CA Calla R. Brown, MD, FAAP
Fellow, Academic General, Division of General Pediatric and
Aaron W. Beck, MD, MMS Adolescent Health
Orthopedic Spine Surgeon Department of Pediatrics
Plymouth Bay Orthopedic Associates Inc. University of Minnesota Medical School
Plymouth, MA Minneapolis, MN

iii

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 iv CONTRIBUTORS

Casey Buitenhuys, MD, FACEP Victor Cueto, MD, MS

Staff Physician, Department of Emergency Medicine Clinical Instructor, Department of Pediatrics
Rady Children’s Hospital San Diego Stanford University School of Medicine
Director of Pediatric Emergency Medicine Stanford, CA
Department of Emergency Medicine
Palomar Medical Center Escondido Soina Kaur Dargan, MD, FAAP
Escondido, CA Associate Clinical Professor of Pediatrics
David Geffen School of Medicine at University of California
David B. Burbulys, MD Los Angeles
Health Sciences Clinical Professor of Emergency Medicine, Chief, Division of Pediatric Hospital Medicine
Department of Emergency Medicine Director, Normal Newborn Services
David Geffen School of Medicine at University of California Department of Pediatrics
Los Angeles Divisions of Neonatology & Pediatric Hospital Medicine
Associate Residency Program Director, Department of Harbor-UCLA Medical Center
Emergency Medicine Torrance, CA
Harbor-UCLA Medical Center
Torrance, CA Catherine A. DeRidder, MD, FAAP
Assistant Professor of Clinical Pediatrics
Kelly Callahan, MD, MPT Department of Pediatrics
Clinical Assistant Professor, Department of Pediatrics Keck School of Medicine of University of Southern California
Senior Physician/Pediatrician, Department of Pediatrics Associate Program Director, Violence Intervention Program,
Harbor-UCLA Medical Center Department of Pediatrics
Torrance, CA Los Angeles County + University of Southern California
Medical Center
Jeanne Anne Carriere, PhD Los Angeles, CA
Director, Families and Schools Together
Chapman University in Partnership with the Center for Autism & Robin Winkler Doroshow, MD, MMS, MEd, FAAP
Neurodevelopmental Disorders Associate Professor, Department of Pediatrics
Orange, CA George Washington School of Medicine and Health Sciences
Physician, Department of Cardiology
Gangadarshni Chandramohan, MD, MSc, FASN, FAAP Children’s National Hospital
Professor of Pediatrics, Department of Pediatrics Washington, DC
University of California Los Angeles
Chief, Division of Pediatric Nephrology Melissa K. Egge, MD, FAAP
Department of Pediatrics Assistant Professor, Department of Pediatrics
Harbor-UCLA Medical Center Loma Linda University Children’s Hospital
Torrance, CA Loma Linda, CA
Stanford Children’s Hospital
Grant P. Christman, MD, FAAP Palo Alto, CA
Assistant Professor of Clinical Pediatrics
Department of Pediatrics W. Suzanne Eidson-Ton, MD, MS
Keck School of Medicine of University of Southern California Clinical Professor, Department of Family and Community Medicine
Attending Physician, Department of Pediatrics University of California, Davis Health
Children’s Hospital Los Angeles Attending Physician, Department of Family and
Los Angeles, CA Community Medicine
University of California, Davis Medical Center
Peter Jinwu Chung, MD, FAAP Sacramento, CA
Assistant Clinical Professor of Pediatrics
University of California, Irvine Andrea Fang, MD
Developmental-Behavioral Pediatrician Clinical Assistant Professor, Department of Emergency Medicine
The Center for Autism and Neurodevelopmental Disorders Stanford University School of Medicine
Santa Ana, CA Stanford, CA

Ismael Corral, MD, MBA Baraka D. Floyd, MD, MSc, FAAP

Pediatric Cardiology Fellow, Department of Pediatrics Clinical Assistant Professor, Department of Pediatrics
University Hospitals Rainbow Babies and Children’s Hospital Division of General Pediatrics
Case Western Reserve University School of Medicine Stanford School of Medicine
Cleveland, OH Stanford, CA

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 CONTRIBUTORS v

Ireal Johnson Fusco, MD, FAAP H. Mollie Greves Grow, MD, MPH, FAAP
Fellow, Pediatric Emergency Medicine General Pediatrician, Associate Professor of Pediatrics,
The University of Texas at Austin Dell Medical School Department of Pediatrics
At Dell Children’s Medical Center of Central Texas University of Washington
Austin, TX Associate Program Director, Pediatrics Residency Program
Seattle Children’s Hospital
Amy C. Gaultney, MD, MTS Seattle, WA
Fellow Physician
University of California Los Angeles Mattel Children’s Hospital Sarah M. Gustafson, MD, FAAP
Los Angeles, CA Assistant Professor, Department of Pediatrics
David Geffen School of Medicine at University of California
George Gershman, MD Los Angeles
Professor of Pediatrics, Department of Pediatrics Pediatric Hospitalist, Department of Pediatrics
David Geffen School of Medicine at University of California Harbor-UCLA Medical Center
Los Angeles Torrance, CA
Chief, Division of Pediatric Gastroenterology
Department of Pediatrics Mark Hanudel, MD, MS
Harbor-UCLA Medical Center Assistant Professor of Pediatrics
Torrance, CA Department of Pediatrics, Division of Nephrology
University of California Los Angeles Mattel Children’s Hospital
Richard Goldstein, MD, FAAP Los Angeles, CA
Assistant Professor in Pediatrics
Harvard Medical School Thomas R. Hawn, MD, PhD
Director, Roberts Program on Sudden Unexpected Death in Professor
Pediatrics, Department of Medicine University of Washington
Boston Children’s Hospital Seattle, WA
Boston, MA
Hanalise V. Huff, MD, MPH
Moran Gotesman, MD Resident Physician in Child Neurology
Assistant Clinical Professor of Pediatrics Boston Children’s Hospital
David Geffen School of Medicine at University of California Boston, MA
Los Angeles Resident Physician in Pediatrics
Westwood, CA Harbor-UCLA Medical Center
Pediatric Hematology/Oncology, Department of Pediatrics Torrance, CA
Harbor-UCLA Medical Center
Torrance, CA Kenneth R. Huff, MD
Emeritus Professor, Departments of Pediatrics and Neurology
Jordan Greenbaum, MD David Geffen School of Medicine at University of California
Medical Director, Global Initiative on Child Health and Los Angeles
Well-being Physician Specialist, Department of Pediatrics
International Centre for Missing and Exploited Children Harbor-UCLA Medical Center
Alexandria, VA Torrance, CA
Medical Director, Institute on Healthcare and Human Trafficking
Stephanie V. Blank Center for Safe and Healthy Children Lynn Hunt, MD, FAAP
Children’s Healthcare of Atlanta Clinical Professor, Department of Pediatrics
Atlanta, GA University of California, Irvine
Irvine, CA
Geeta Grover, MD, FAAP
Clinical Professor of Pediatrics, Department of Pediatrics Jung Sook (Stella) Hwang, DO, FAAP
University of California, Irvine, School of Medicine Assistant Clinical Professor, Department of Pediatrics
Developmental and Behavioral Pediatrician University of California, Irvine
The Center for Autism and Neurodevelopmental Disorders Neonatologist, Department of Pediatrics
Santa Ana, CA Children’s Hospital of Orange County
Children’s Hospital of Orange County Orange, CA
Orange, CA

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 vi CONTRIBUTORS

Stanley H. Inkelis, MD, FAAP Khalid M. Khan, MD

Emeritus Professor of Pediatrics, Department of Pediatrics Associate Professor, Department of Pediatrics
David Geffen School of Medicine at University of California Georgetown University
Los Angeles Pediatric Gastroenterologist and Hepatologist
Emeritus Professor of Pediatrics Department of Pediatrics/Transplant Institute
Department of Pediatrics MedStar Georgetown University Hospital
Harbor-UCLA Medical Center Washington, DC
Torrance, CA
Jane S. Kim, MD
Caleb Jeon, MD Assistant Professor
Resident Physician, Department of Dermatology Department of Diagnostic Radiology and Nuclear Medicine
Harbor-UCLA Medical Center University of Maryland School of Medicine
Torrance, CA Baltimore, MD

Jesse Joad, MD, MS, FAAP Kenny Y.C. Kwong, MD

Professor Pediatrics and Associate Dean Diversity and Faculty Associate Program Director of Allergy-Immunology
Life, Emerita Fellowship Program
University of California, Davis Adjunct Associate Clinical Professor of Pediatrics
Davis, CA Los Angeles County, University of Southern California
Medical Center
Doron D. Kahana, MD, CPNS Los Angeles, CA
Director, Medical Nutrition, Ambulatory Care Network,
Los Angeles County Joseph L. Lasky III, MD, FAAP
Gastroenterology, Martin Luther King, Jr. Outpatient Center and Pediatric Hematology/Oncology
H. Claude Hudson Comprehensive Health Center Cure 4 the Kids Foundation
Clinical Assistant Professor, Charles R. Drew University of Las Vegas, NV
Medicine and Science
Los Angeles, CA Marciana Laster, MD, MS
President-Elect, National Board of Physician Nutrition Specialists Assistant Professor, Department of Pediatrics
Division of Nephrology
Tom Kallay, MD University of California Los Angeles Mattel Children’s Hospital
Associate Professor of Clinical Pediatrics Los Angeles, CA
University of California Los Angeles
Division Chief, Pediatric Intensive Care Unit Delphine J. Lee, MD, PhD, FAAD
Department of Pediatrics Associate Professor, Department of Medicine
Harbor-UCLA Medical Center David Geffen School of Medicine at University of California
Torrance, CA Los Angeles
Westwood, CA
Elaine S. Kamil, MD Dermatology Chief and Residency Program Director, Department
Health Sciences Clinical Professor of Pediatrics of Medicine
David Geffen School of Medicine at University of California Harbor-UCLA Medical Center
Los Angeles Director, Cancer Biology and Immunotherapeutics IWI
Professor Emeritus, Department of Pediatric Nephrology The Lundquist Institute for Biomedical Innovation at
Cedars-Sinai Medical Center Harbor-UCLA Medical Center
Los Angeles, CA Torrance, CA
Attending Physician, Department of Pediatrics
Harbor-UCLA Medical Center Steven L. Lee, MD, MBA, FACS, FAAP
Torrance, CA Professor of Clinical Surgery and Pediatrics
Chief of Pediatric Surgery, University of California Los Angeles
Clare Kasper, MD Mattel Children’s Hospital
HS Assistant Clinical Professor, Department of Pediatrics David Geffen School of Medicine at University of California
University of California Los Angeles Los Angeles
Pediatric Hospitalist, Department of Pediatrics Los Angeles, CA
Harbor-UCLA Medical Center
Torrance, CA

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 CONTRIBUTORS vii

Charlotte W. Lewis, MD, MPH, FAAP Fernando S. Mendoza, MD, MPH, FAAP
Associate Professor, Department of Pediatrics Professor of Pediatrics, Division of General Pediatrics,
University of Washington Department of Pediatrics
Attending Physician, Department of General Pediatrics and Associate Dean of Minority Advising and Programs
Hospital Medicine Stanford University School of Medicine
Seattle Children’s Hospital Stanford, CA
Seattle, WA
ChrisAnna M. Mink, MD, FAAP
Houmin Li, MD, PhD Clinical Professor, Department of Pediatrics
Visiting Scholar, Biomedical Research Institute David Geffen School of Medicine at University of California
Harbor-UCLA Medical Center Los Angeles
Torrance, CA Voluntary Faculty, Department of Pediatrics
Associate Professor, Department of Dermatology Harbor-UCLA Medical Center
Peking University People’s Hospital Torrance, CA
Beijing, China
Wendy Miyares, RN, PNP
Henry J. Lin, MD Assistant Clinical Professor, Department of Nursing
Professor, Department of Pediatrics University of California Los Angeles School of Nursing
David Geffen School of Medicine at University of California Westwood, CA
Los Angeles Pediatric Nurse Practitioner, Department of General Pediatrics
Los Angeles, CA Harbor-UCLA Medical Center
Division of Medical Genetics, Department of Pediatrics Torrance, CA
Harbor-UCLA Medical Center
Torrance, CA Deepa Mokshagundam, MD, FAAP
Fellow in Pediatric Cardiology, Department of Pediatrics
Janice Ma, MD George Washington University
Resident Physician, Department of Internal Medicine Fellow in Pediatric Cardiology, Department of
Division of Dermatology Pediatric Cardiology
Harbor-UCLA Medical Center Children’s National Hospital
Torrance, CA Washington, DC

Kevin Madden, MD Michael Nguyen, DO

Assistant Professor, Department of Palliative, Rehabilitation and Resident Physician, Department of Pediatrics
Integrative Medicine Harbor-UCLA Medical Center
University of Texas MD Anderson Cancer Center Torrance, CA
Houston, TX
Patricia Padlipsky, MD, FAAP
Catherine S. Mao, MD Associate Professor of Pediatrics and Pediatric
Clinical Professor of Pediatric Endocrinology Emergency Medicine
Department of Pediatrics Department of Emergency Medicine
David Geffen School of Medicine at University of California Los Angeles David Geffen School of Medicine at University of California
Physician Specialist Pediatric Endocrinology Los Angeles
Department of Pediatrics Medical Director, Pediatric Emergency Department
Harbor-UCLA Medical Center Department of Emergency Medicine
Torrance, CA Harbor-UCLA Medical Center
Torrance, CA
Roxanne L. Massoumi, MD
Resident, Department of General Surgery Suzinne Pak-Gorstein, MD, PhD, MPH, FAAP
University of California Los Angeles Associate Professor, Department of Pediatrics
Los Angeles, CA Adjunct Associate Professor, Department of Global Health
University of Washington
Deborah McCurdy, MD, FAAP Harborview Medical Center/Seattle Children’s Hospital
Clinical Professor, Department of Pediatrics Seattle, WA
David Geffen School of Medicine at University of California
Los Angeles
Director of Pediatric Rheumatology, Department of Pediatrics
Ronald Reagan UCLA Medical Center
Los Angeles, CA

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 viii CONTRIBUTORS

Eduard H. Panosyan, MD Suzanne Roberts, MD, FAAP

Associate Professor of Pediatrics, Department of Pediatrics Clinical Associate Professor of Pediatrics (Clinician-Educator)
David Geffen School of Medicine at University of California Department of General Pediatrics
Los Angeles Keck School of Medicine of University of Southern California
Los Angeles, CA Children’s Hospital Los Angeles
Chief, Pediatric Hematology/Oncology, Department of Pediatrics Los Angeles, CA
Harbor-UCLA Medical Center
Torrance, CA Teresa O. Rosales, MD
Clinical Professor, Department of Ophthalmology
Miriam F. Parsa, MD, MPH, FAAP David Geffen School of Medicine at University of California
Associate Physician, Department of Pediatrics Los Angeles
Division of Rheumatology University of California Los Angeles Stein Eye Institute
David Geffen School of Medicine at University of California Los Angeles Los Angeles, CA
Los Angeles, CA
Physician, Department of Pediatric Rheumatology Melanie Rudnick, MD, FAAP
Cottage Children’s Medical Center Assistant Professor, Department of Pediatrics
Santa Barbara, CA University of Connecticut School of Medicine
Farmington, CT
Bonnie R. Rachman, MD Assistant Professor, Department of Pediatrics
Professor of Pediatrics, Department of Pediatrics Frank H. Netter School of Medicine at Quinnipiac University
David Geffen School of Medicine at University of California North Haven, CT
Los Angeles Attending Physician, Department of Pediatric Hospital Medicine
Medical Director, Pediatric Intensive Care Unit Connecticut Children’s Medical Center
Department of Pediatrics Hartford, CT
Harbor-UCLA Medical Center
Torrance, CA Thusa Sabapathy, MD
Assistant Clinical Professor of Pediatrics
Christian B. Ramers, MD, MPH, AAHIVS Department of Pediatrics
Associate Clinical Professor, Department of Infectious Diseases University of California, Irvine
University of California San Diego School of Medicine Developmental-Behavioral Pediatrician
La Jolla, CA The Center for Autism and Neurodevelopmental Disorders
Assistant Medical Director, Department of Santa Ana, CA
Research/Special Populations
Family Health Centers of San Diego Isidro B. Salusky, MD
San Diego, CA Distinguished Professor of Pediatrics, Department of Pediatrics
Chief, Division of Pediatric Nephrology
Joseph Ravera, MD David Geffen School of Medicine at University of California
Assistant Professor, Department of Surgery, University of Vermont Los Angeles
Director of Pediatric Emergency Medicine, Department of Surgery Los Angeles, CA
University of Vermont Medical Center
Burlington, VT Tracey Samko, MD, FAAP
Attending Physician
Nasser Redjal, MD Keck School of Medicine of University of Southern California
Professor of Clinical Pediatrics and Allergy/Immunology, Attending Physician, Department of Internal Medicine/Pediatrics
Department of Pediatrics Los Angeles County + University of Southern California
David Geffen School of Medicine at University of California Los Angeles Medical Center
Professor of Clinical Pediatrics and Allergy/Immunology, Los Angeles, CA
Department of Pediatrics
Harbor-UCLA Medical Center Nefthi Sandeep, MD
Torrance, CA Pediatric Cardiologist, Department of Pediatrics
Mary Bridge Children’s Hospital
Katherine E. Remick, MD, FACEP, FAEMS, FAAP Pediatric Cardiologist, Department of Pediatrics
Executive Lead, National EMS for Children Innovation and Swedish Medical Center
Improvement Center Seattle, WA
Assistant Professor, Department of Pediatrics and Surgery
Dell Medical School at the University of Texas at Austin
Attending Physician, Emergency Department
Dell Children’s Medical Center of Central Texas
Austin, TX

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 CONTRIBUTORS ix

Anna K. Schlechter, MD Blanca Solis, MD

Fellow, Pediatric Emergency Medicine Associate Clinical Professor, Department of Family and
Department of Pediatrics, Division of Pediatric Community Medicine
Emergency Medicine University of California, Davis Health
Dell Medical School, The University of Texas at Austin Attending Physician, Department of Family and Community Medicine
Austin, TX University of California School of Medicine
Sacramento, CA
Eric R. Schmitt, MD, MPH, FACEP, FAAP
Associate Clinical Professor, Department of Emergency Medicine Charles H. Song, MD
University of California, San Francisco, Fresno Medical Clinical Professor of Pediatrics, Department of Pediatrics
Education Program David Geffen School of Medicine at University of California
Attending Physician, Department of Emergency Medicine Los Angeles
Community Regional Medical Center Chief, Division of Allergy and Immunology
Fresno, CA Department of Pediatrics
Harbor-UCLA Medical Center
Marni E. Shear, DO, FAAP Torrance, CA
Clinical Instructor of Pediatrics
Keck School of Medicine of University of Southern California Stephanie R. Starr, MD, FAAP
Pediatric Hospital Medicine Fellow, Division of Hospital Medicine Associate Professor of Pediatrics
Children’s Hospital Los Angeles Consultant and Vice-Chair for Quality and Safety, Division of
Los Angeles, CA Community Pediatric and Adolescent Medicine
Director for Science and Health Care Delivery Education
Ilana Sherer, MD, FAAP Mayo Clinic Alix School of Medicine
HS Assistant Clinical Professor, Department of Pediatrics Rochester, MN
University of California San Francisco Benioff Children’s Hospital
San Francisco, CA Robin Steinberg-Epstein, MD
Pediatrician, Palo Alto Medical Foundation Clinical Professor of Pediatrics
Dublin, CA Chief, Division of Developmental Behavioral Pediatrics
University of California, Irvine School of Medicine
Erica M.S. Sibinga, MD, MHS, FAAP Developmental Behavioral Pediatrics
Associate Professor, Department of Pediatrics The Center for Autism and Neurodevelopmental Disorders
Johns Hopkins School of Medicine Santa Ana, CA
Baltimore, MD
Miriam T. Stewart, MD, FAAP
Melissa D. Siccama, MD Hospice and Palliative Medicine Fellow
Fellow, Forensics, Department of Forensics Department of Pediatrics
Loma Linda University Children’s Hospital Children’s Hospital of Philadelphia
Loma Linda, CA Philadelphia, PA

Monica Sifuentes, MD Sara T. Stewart, MD, MPH, FAAP

Professor of Pediatrics Department of Pediatrics
David Geffen School of Medicine at University of California Los Angeles Kaiser Permanente South Bay Medical Center
Vice Chair of Education, Department of Pediatrics Harbor City, CA
Harbor-UCLA Medical Center
Torrance, CA Benjamin H. Taragin, MD
Professor, Department of Radiology
Lynne M. Smith, MD, FAAP Ben-Gurion University of the Negev
Professor, Department of Pediatrics Attending Physician, Department of Radiology
David Geffen School of Medicine at University of California Soroka University Medical Center
Los Angeles Beer-Sheva, Israel
Chair, Department of Pediatrics
Harbor-UCLA Medical Center Niloufar Tehrani, MD
Torrance, CA Assistant Professor of Pediatrics
David Geffen School of Medicine at University of California Los Angeles
Samantha Snider, MD Director, Pediatric Primary Care Clinic
Department of Pediatrics Director, Child Advocacy
Harbor-UCLA Medical Center Department of Pediatrics
Torrance, CA Harbor-UCLA Medical Center
Torrance, CA

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 x CONTRIBUTORS

Alan Tomines, MD Michael Weiss, DO, FAAP

Clinical Associate Professor, Department of Pediatrics, University Vice President, Population Health
of California Los Angeles Division of Population Health
Physician, Department of Pediatrics Children’s Hospital of Orange County
Harbor-UCLA Medical Center Orange County, CA
Torrance, CA
Jennifer K. Yee, MD
Hendry Ton, MD, MS Associate Professor, Department of Pediatrics
Clinical Professor, Department of Psychiatry and Behavioral Health David Geffen School of Medicine at University of California
Attending Physician, Department of Psychiatry and Los Angeles
Behavioral Health Division Chief, Pediatric Endocrinology, Department of Pediatrics
University of California, Davis Medical Center Harbor-UCLA Medical Center
Sacramento, CA Torrance, CA

Moin Vera, MD, PhD Ki-Young Yoo, MD

Clinical Assistant Professor, Department of Pediatrics Dermatologist, Department of Dermatology
Keck School of Medicine of University of Southern California Kaiser Permanente South Bay Medical Center
Attending Faculty, Department of Pediatrics Harbor City, CA
Children’s Hospital Los Angeles
Los Angeles, CA Kelly D. Young, MD, MS, FAAP
Health Sciences Clinical Professor of Pediatrics, Department of
Michelle L. Wahlquist, CCC-SLP Pediatrics
Speech-Language Pathologist, Division Lead David Geffen School of Medicine at University of California
The Center for Autism and Neurodevelopmental Disorders Los Angeles
Department of Pediatrics Program Director, Pediatric Emergency Medicine Fellowship
University of California, Irvine Harbor-UCLA Medical Center
Santa Ana, CA Torrance, CA

Nisha Warikoo, MD Meiling L. Fang Yuen, MD

Assistant Clinical Professor Associate Program Director, Department of Medicine/Dermatology
UCI Health Associate Staff, Department of Medicine/Dermatology
Orange, CA Harbor-UCLA Medical Center
Torrance, CA
Joseph H. Waters, MD
Pediatric Resident, Department of Pediatrics
Cohen Children’s Medical Center
New Hyde Park, NY

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 Contents
Preface............................................................................................................................................................................................... xxi
Using This Book.............................................................................................................................................................................. xxiii
Part 1: Primary Care: Skills and Concepts
1. Primary Care: Introduction....................................................................................................................................................... 3
Niloufar Tehrani, MD
2. Talking With Parents................................................................................................................................................................. 7
Geeta Grover, MD, FAAP
3. Talking With Children............................................................................................................................................................. 13
Geeta Grover, MD, FAAP
4. Talking With Adolescents........................................................................................................................................................ 17
Monica Sifuentes, MD
5. Telephone Management and E-medicine............................................................................................................................... 21
Emily Borman-Shoap, MD, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP
6. Informatics............................................................................................................................................................................... 27
Alan Tomines, MD
7. Counseling Families About Internet Use................................................................................................................................ 33
Alan Tomines, MD
8. Cultural Competency Issues in Pediatrics.............................................................................................................................. 39
W. Suzanne Eidson-Ton, MD, MS; Hendry Ton, MD, MS; Blanca Solis, MD; and Jesse Joad, MD, MS, FAAP
9. Global Child Health.................................................................................................................................................................. 45
Suzinne Pak-Gorstein, MD, PhD, MPH, FAAP, and Maneesh Batra, MD, MPH
10. Child Advocacy......................................................................................................................................................................... 51
Marni E. Shear, DO, FAAP, and Grant P. Christman, MD, FAAP

Part 2: Principles of Health Care and Pediatric Management

11. Health Systems Science............................................................................................................................................................ 59
Stephanie R. Starr, MD, FAAP
12. Population Health for Pediatricians........................................................................................................................................ 73
Michael Weiss, DO, FAAP
13. Principles of Pediatric Therapeutics....................................................................................................................................... 79
Bonnie R. Rachman, MD
14. Pediatric Pain and Symptom Management............................................................................................................................ 85
Kevin Madden, MD, and Richard Goldstein, MD, FAAP
15. Complementary and Integrative Medicine in Pediatric Primary Care................................................................................. 95
Miriam T. Stewart, MD, FAAP, and Erica M.S. Sibinga, MD, MHS, FAAP

xi

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 xii CONTENTS

16. Principles of Pediatric Surgery.............................................................................................................................................. 105

Roxanne L. Massoumi, MD, and Steven L. Lee, MD, MBA, FACS, FAAP
17. Image Gently Approach to Pediatric Imaging....................................................................................................................... 109
Jane S. Kim, MD; Lindsay S. Baron, MD; and Benjamin H. Taragin, MD
18. Simulation in Pediatric Health Care..................................................................................................................................... 113
Tom Kallay, MD
19. Pediatric Hospital Medicine.................................................................................................................................................. 121
Melanie Rudnick, MD, FAAP, and Grant P. Christman, MD, FAAP
20. Pediatric Genomic Medicine................................................................................................................................................. 125
Moin Vera, MD, PhD, and Henry J. Lin, MD
21. Principles of Quality Improvement: Improving Health Care for Pediatric Patients.......................................................... 129
Bonnie R. Rachman, MD
22. Pediatric Palliative Care: Principles and Practice................................................................................................................ 137
Jori Bogetz, MD, FAAP, and Richard Goldstein, MD, FAAP

Part 3: Health Maintenance and Anticipatory Guidance

23. Neonatal Examination and Nursery Visit............................................................................................................................. 147
Niloufar Tehrani, MD
24. Maternal Perinatal Mood and Anxiety Disorders: The Role of the Pediatrician............................................................... 155
Carol D. Berkowitz, MD, FAAP
25. Newborn Screening................................................................................................................................................................ 161
Henry J. Lin, MD, and Moin Vera, MD, PhD
26. Caring for Twins and Higher-Order Multiples..................................................................................................................... 167
Soina Kaur Dargan, MD, FAAP, and Lynne M. Smith, MD, FAAP
27. Male Circumcision................................................................................................................................................................. 173
Jung Sook (Stella) Hwang, DO, FAAP, and Lynne M. Smith, MD, FAAP
28. Nutritional Needs................................................................................................................................................................... 179
Sara T. Stewart, MD, MPH, FAAP
29. Breastfeeding.......................................................................................................................................................................... 187
Karen C. Bodnar, MD, IBCLC, FABM, FAAP
30. Sleep: Normal Patterns and Common Disorders................................................................................................................. 193
Geeta Grover, MD, FAAP, and Thusa Sabapathy, MD
31. Oral Health and Dental Disorders......................................................................................................................................... 201
Charlotte W. Lewis, MD, MPH, FAAP
32. Normal Development and Developmental Surveillance, Screening, and Evaluation........................................................ 211
Geeta Grover, MD, FAAP, and Jeanne Anne Carriere, PhD
33. Speech and Language Development: Normal Patterns and Common Disorders.............................................................. 221
Geeta Grover, MD, FAAP, and Michelle L. Wahlquist, CCC-SLP
34. Literacy Promotion in Pediatric Practice............................................................................................................................. 231
Wendy Miyares, RN, PNP
35. Gifted Children....................................................................................................................................................................... 235
Calla R. Brown, MD, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP
36. Children and School: A Primer for the Practitioner............................................................................................................ 241
Geeta Grover, MD, FAAP, and Jeanne Anne Carriere, PhD

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 CONTENTS xiii

37. Immunizations....................................................................................................................................................................... 253

ChrisAnna M. Mink, MD, FAAP
38. Health Maintenance in Older Children and Adolescents.................................................................................................... 259
Monica Sifuentes, MD
39. Health Care for International Adoptees................................................................................................................................ 271
ChrisAnna M. Mink, MD, FAAP
40. Health Care Needs of Children in Foster Care..................................................................................................................... 279
Kelly Callahan, MD, MPT; ChrisAnna M. Mink, MD, FAAP; and Sara T. Stewart, MD, MPH, FAAP
41. Working With Immigrant Children and Their Families...................................................................................................... 285
Ismael Corral, MD, MBA, and Carol D. Berkowitz, MD, FAAP
42. Well-Child Care for Children With Trisomy 21 (Down Syndrome)................................................................................... 291
Moin Vera, MD, PhD, and Henry J. Lin, MD
43. Well-Child Care for Preterm Infants..................................................................................................................................... 299
Soina Kaur Dargan, MD, FAAP, and Lynne M. Smith, MD, FAAP
44. Care of Children With Special Health Care Needs............................................................................................................... 307
Clare Kasper, MD
45. Injury Prevention................................................................................................................................................................... 313
Sarah J. Atunah-Jay, MD, MPH, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP
46. Fostering Self-esteem............................................................................................................................................................. 319
Richard Goldstein, MD, FAAP
47. Sibling Rivalry........................................................................................................................................................................ 325
Carol D. Berkowitz, MD, FAAP
48. Toilet Training........................................................................................................................................................................ 329
Jung Sook (Stella) Hwang, DO, FAAP, and Lynne M. Smith, MD, FAAP
49. Crying and Colic..................................................................................................................................................................... 335
Geeta Grover, MD, FAAP
50. Discipline................................................................................................................................................................................ 339
Carol D. Berkowitz, MD, FAAP
51. Temper Tantrums................................................................................................................................................................... 345
Geeta Grover, MD, FAAP, and Peter Jinwu Chung, MD, FAAP
52. Breath-Holding Spells............................................................................................................................................................ 351
Geeta Grover, MD, FAAP, and Peter Jinwu Chung, MD, FAAP
53. Fears, Phobias, and Anxiety.................................................................................................................................................. 355
Carol D. Berkowitz, MD, FAAP
54. Thumb-sucking and Other Habits........................................................................................................................................ 361
Carol D. Berkowitz, MD, FAAP
55. Enuresis................................................................................................................................................................................... 367
Carol D. Berkowitz, MD, FAAP
56. Encopresis............................................................................................................................................................................... 373
Carol D. Berkowitz, MD, FAAP

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 xiv CONTENTS

Part 4: Adolescent Health

57. Culturally Competent Care for Diverse Populations: Sexual Orientation and Gender Expression.................................. 381
Ilana Sherer, MD, FAAP; Brittany Allen, MD, FAAP; Joseph H. Waters, MD; and Lynn Hunt, MD, FAAP
58. Reproductive Health.............................................................................................................................................................. 389
Monica Sifuentes, MD
59. Vaginitis.................................................................................................................................................................................. 399
Monica Sifuentes, MD
60. Sexually Transmitted Infections............................................................................................................................................ 405
Monica Sifuentes, MD
61. Menstrual Disorders.............................................................................................................................................................. 417
Monica Sifuentes, MD
62. Disorders of the Breast........................................................................................................................................................... 427
Monica Sifuentes, MD
63. Substance Use/Abuse............................................................................................................................................................. 437
Monica Sifuentes, MD
64. Eating Disorders..................................................................................................................................................................... 447
Monica Sifuentes, MD
65. Body Modification: Tattooing and Body Piercing................................................................................................................ 457
Monica Sifuentes, MD
66. Depression and Suicide in Adolescents................................................................................................................................ 465
Monica Sifuentes, MD, and Robin Steinberg-Epstein, MD

Part 5: Acute and Emergent Problems

67. Fever and Bacteremia............................................................................................................................................................. 475
Eric R. Schmitt, MD, MPH, FACEP, FAAP
68. Emerging Infectious Diseases............................................................................................................................................... 483
Christian B. Ramers, MD, MPH, AAHIVS, and Thomas R. Hawn, MD, PhD
69. Febrile Seizures....................................................................................................................................................................... 495
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
70. Respiratory Distress............................................................................................................................................................... 501
David B. Burbulys, MD
71. Stridor and Croup.................................................................................................................................................................. 507
David B. Burbulys, MD
72. Sudden Unexpected Infant Death and Brief Resolved Unexplained Events...................................................................... 513
Sarah M. Gustafson, MD, FAAP, and Lynne M. Smith, MD, FAAP
73. Syncope................................................................................................................................................................................... 521
David Atkinson, MD, and Michael Nguyen, DO
74. Shock....................................................................................................................................................................................... 527
Kelly D. Young, MD, MS, FAAP
75. Approach to the Traumatized Child...................................................................................................................................... 537
David B. Burbulys, MD
76. Abdominal Trauma................................................................................................................................................................ 543
David B. Burbulys, MD

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 CONTENTS xv

77. Acute Abdomen (Appendicitis)............................................................................................................................................ 549

Roxanne L. Massoumi, MD, and Steven L. Lee, MD, MBA, FACS, FAAP
78. Head Trauma.......................................................................................................................................................................... 555
Joseph Ravera, MD
79. Increased Intracranial Pressure............................................................................................................................................. 563
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
80. Management of Dehydration in Children: Fluid and Electrolyte Therapy......................................................................... 571
Gangadarshni Chandramohan, MD, MSc, FASN, FAAP
81. Acute Kidney Injury............................................................................................................................................................... 583
Gangadarshni Chandramohan, MD, MSc, FASN, FAAP
82. Ingestions: Diagnosis and Management............................................................................................................................... 591
Kelly D. Young, MD, MS, FAAP
83. Disaster Preparedness............................................................................................................................................................ 599
Ireal Johnson Fusco, MD, FAAP, and Katherine E. Remick, MD, FACEP, FAEMS, FAAP

Part 6: Head, Neck, and Respiratory System

84. Approach to the Child With Dysmorphism......................................................................................................................... 607
Henry J. Lin, MD, and Moin Vera, MD, PhD
85. Craniofacial Anomalies.......................................................................................................................................................... 613
Carol D. Berkowitz, MD, FAAP
86. Common Oral Lesions........................................................................................................................................................... 621
Charlotte W. Lewis, MD, MPH, FAAP
87. Otitis Media............................................................................................................................................................................ 627
Nasser Redjal, MD
88. Hearing Impairments............................................................................................................................................................. 635
Patricia Padlipsky, MD, FAAP
89. Sore Throat............................................................................................................................................................................. 645
Casey Buitenhuys, MD, FACEP, and Stanley H. Inkelis, MD, FAAP
90. Nosebleeds.............................................................................................................................................................................. 655
Anna K. Schlechter, MD; Katherine E. Remick, MD, FACEP, FAEMS, FAAP; and Stanley H. Inkelis, MD, FAAP
91. Strabismus.............................................................................................................................................................................. 661
Teresa O. Rosales, MD
92. Infections of the Eye............................................................................................................................................................... 667
Teresa O. Rosales, MD
93. Excessive Tearing................................................................................................................................................................... 673
Teresa O. Rosales, MD
94. Neck Masses........................................................................................................................................................................... 677
Casey Buitenhuys, MD, FACEP, and Stanley H. Inkelis, MD, FAAP
95. Allergic Disease...................................................................................................................................................................... 687
Nasser Redjal, MD, and Niloufar Tehrani, MD
96. Wheezing and Asthma........................................................................................................................................................... 699
Kenny Y.C. Kwong, MD, and Nasser Redjal, MD
97. Cough...................................................................................................................................................................................... 713
Nasser Redjal, MD, and Charles H. Song, MD

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 xvi CONTENTS

Part 7: Hematologic Disorders

98. Anemia.................................................................................................................................................................................... 723
Joseph L. Lasky III, MD, FAAP; Moran Gotesman, MD; and Eduard H. Panosyan, MD
99. Bleeding Disorders................................................................................................................................................................. 733
Joseph L. Lasky III, MD, FAAP; Moran Gotesman, MD; and Eduard H. Panosyan, MD
100. Lymphadenopathy.................................................................................................................................................................. 743
Eduard H. Panosyan, MD; Moran Gotesman, MD; and Joseph L. Lasky III, MD, FAAP

Part 8: Cardiovascular System

101. Heart Murmurs...................................................................................................................................................................... 751
Robin Winkler Doroshow, MD, MMS, MEd, FAAP
102. Palpitations............................................................................................................................................................................. 755
Robin Winkler Doroshow, MD, MMS, MEd, FAAP, and Nefthi Sandeep, MD
103. Cyanosis in the Newborn....................................................................................................................................................... 763
Robin Winkler Doroshow, MD, MMS, MEd, FAAP
104. Congestive Heart Failure........................................................................................................................................................ 769
Robin Winkler Doroshow, MD, MMS, MEd, FAAP, and Deepa Mokshagundam, MD, FAAP
105. Chest Pain............................................................................................................................................................................... 775
Robin Winkler Doroshow, MD, MMS, MEd, FAAP
106. Hypertension.......................................................................................................................................................................... 783
Gangadarshni Chandramohan, MD, MSc, FASN, FAAP, and Michael Nguyen, DO

Part 9: Genitourinary Disorders

107. Disorders of Sexual Differentiation....................................................................................................................................... 803
Jennifer K. Yee, MD, and Catherine S. Mao, MD
108. Inguinal Lumps and Bumps.................................................................................................................................................. 809
Sara T. Stewart, MD, MPH, FAAP
109. Hematuria............................................................................................................................................................................... 815
Elaine S. Kamil, MD
110. Proteinuria.............................................................................................................................................................................. 823
Elaine S. Kamil, MD
111. Nephrotic Syndrome.............................................................................................................................................................. 829
Elaine S. Kamil, MD
112. Urinary Tract Infections........................................................................................................................................................ 839
Gangadarshni Chandramohan, MD, MSc, FASN, FAAP

Part 10: Orthopedic Disorders

113. Developmental Dysplasia of the Hip..................................................................................................................................... 849
Kier Maddox Blevins, MD, and Andrew K. Battenberg, MD
114. In-toeing and Out-toeing: Rotational Problems of the Lower Extremity........................................................................... 855
Kier Maddox Blevins, MD, and Andrew K. Battenberg, MD
115. Angular Deformities of the Lower Extremity: Bowlegs and Knock-Knees........................................................................ 863
Kier Maddox Blevins, MD; Andrew K. Battenberg, MD; and Carol D. Berkowitz, MD, FAAP

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 CONTENTS xvii

116. Orthopedic Injuries and Growing Pains............................................................................................................................... 869

Sara T. Stewart, MD, MPH, FAAP
117. Sports-Related Acute Injuries................................................................................................................................................ 875
Monica Sifuentes, MD; Kier Maddox Blevins, MD; and Andrew K. Battenberg, MD
118. Evaluation of Limp................................................................................................................................................................. 881
Andrea Fang, MD
119. Musculoskeletal Disorders of the Neck and Back................................................................................................................ 889
Aaron W. Beck, MD, MMS; Kier Maddox Blevins, MD; Andrew K. Battenberg, MD;
and Carol D. Berkowitz, MD, FAAP

Part 11: Gastrointestinal Disorders

120. Vomiting................................................................................................................................................................................. 899
George Gershman, MD
121. Gastroesophageal Reflux........................................................................................................................................................ 905
George Gershman, MD
122. Gastrointestinal Bleeding....................................................................................................................................................... 911
George Gershman, MD
123. Diarrhea.................................................................................................................................................................................. 919
George Gershman, MD
124. Constipation........................................................................................................................................................................... 925
Doron D. Kahana, MD, CPNS, and Khalid M. Khan, MD
125. Abdominal Pain...................................................................................................................................................................... 933
George Gershman, MD
126. Jaundice................................................................................................................................................................................... 939
Doron D. Kahana, MD, CPNS, and Khalid M. Khan, MD
127. Viral Hepatitis......................................................................................................................................................................... 947
ChrisAnna M. Mink, MD, FAAP

Part 12: Neuropsychiatric Disorders

128. Hypotonia............................................................................................................................................................................... 957
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
129. Headaches............................................................................................................................................................................... 965
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
130. Tics.......................................................................................................................................................................................... 973
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
131. Seizures and Epilepsy............................................................................................................................................................. 979
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
132. Autism Spectrum Disorder.................................................................................................................................................... 989
Robin Steinberg-Epstein, MD
133. Attention-Deficit/Hyperactivity Disorder............................................................................................................................ 997
Andrew J. Barnes, MD, MPH, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP
134. Psychopharmacology in Children....................................................................................................................................... 1005
Robin Steinberg-Epstein, MD, and Nisha Warikoo, MD

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 xviii CONTENTS

Part 13: Dermatologic Disorders

135. Acne....................................................................................................................................................................................... 1015
Samantha Snider, MD
136. Disorders of the Hair and Scalp........................................................................................................................................... 1023
Janice Ma, MD; Delphine J. Lee, MD, PhD, FAAD; and Ki-Young Yoo, MD
137. Diaper Dermatitis................................................................................................................................................................. 1031
Houmin Li, MD, PhD; Delphine J. Lee, MD, PhD, FAAD; and Ki-Young Yoo, MD
138. Papulosquamous Eruptions................................................................................................................................................. 1037
Janice Ma, MD; Delphine J. Lee, MD, PhD, FAAD; and Ki-Young Yoo, MD
139. Morbilliform Rashes............................................................................................................................................................ 1045
Houmin Li, MD, PhD; Delphine J. Lee, MD, PhD, FAAD; and Ki-Young Yoo, MD
140. Vesicular Exanthems............................................................................................................................................................ 1051
Caleb Jeon, MD; Meiling L. Fang Yuen, MD; and Ki-Young Yoo, MD

Part 14: Social Determinants of Health

141. Social Determinants of Health: Principles.......................................................................................................................... 1061
Victor Cueto, MD, MS; Baraka D. Floyd, MD, MSc, FAAP; and Fernando S. Mendoza, MD, MPH, FAAP
142. Adverse Childhood Experiences: Trauma-Informed Care................................................................................................ 1069
Suzanne Roberts, MD, FAAP, and Geeta Grover, MD, FAAP
143. Commercially Exploited Children and Human Trafficking.............................................................................................. 1077
Jordan Greenbaum, MD
144. Physical Abuse...................................................................................................................................................................... 1085
Melissa K. Egge, MD, FAAP, and Melissa D. Siccama, MD
145. Sexual Abuse......................................................................................................................................................................... 1091
Sara T. Stewart, MD, MPH, FAAP
146. Failure to Thrive................................................................................................................................................................... 1097
Carol D. Berkowitz, MD, FAAP
147. Fetal Alcohol Syndrome....................................................................................................................................................... 1105
Melissa K. Egge, MD, FAAP
148. Infants of Substance-Using Mothers................................................................................................................................... 1111
Sara T. Stewart, MD, MPH, FAAP
149. Divorce.................................................................................................................................................................................. 1117
Carol D. Berkowitz, MD, FAAP
150. School-Related Violence and Bullying................................................................................................................................ 1123
Tracey Samko, MD, FAAP, and Catherine A. DeRidder, MD, FAAP
151. Intimate Partner Violence.................................................................................................................................................... 1129
Sara T. Stewart, MD, MPH, FAAP

Part 15: Chronic Diseases of Childhood and Adolescence

152. Cancer in Children............................................................................................................................................................... 1137
Eduard H. Panosyan, MD; Moran Gotesman, MD; and Joseph L. Lasky III, MD, FAAP
153. Chronic Kidney Disease....................................................................................................................................................... 1147
Mark Hanudel, MD, MS; Marciana Laster, MD, MS; and Isidro B. Salusky, MD

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 CONTENTS xix

154. Diabetes Mellitus.................................................................................................................................................................. 1159

Jennifer K. Yee, MD, and Catherine S. Mao, MD
155. Childhood Obesity............................................................................................................................................................... 1165
H. Mollie Greves Grow, MD, MPH, FAAP
156. Juvenile Idiopathic Arthritis and Benign Joint Pains of Childhood................................................................................. 1173
Miriam F. Parsa, MD, MPH, FAAP, and Deborah McCurdy, MD, FAAP
157. Autoimmune Connective Tissue Diseases.......................................................................................................................... 1181
Deborah McCurdy, MD, FAAP; Amy C. Gaultney, MD, MTS; and Miriam F. Parsa, MD, MPH, FAAP

Index..................................................................................................................................................................................... 1195

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BPPCA6e_FM_i-xxiv.indd 20 2/14/20 9:37 AM
 Preface
It has been nearly 25 years since the publication of the first edition of what was then simply Pediatrics: A Primary Care Approach.
That book contained 113 chapters and 500 pages. The section titled Chronic Diseases included HIV, chronic lung disease, and chronic
osteomyelitis, conditions no longer included in this current edition. The impetus for the book came from a series of noontime lectures
I had given to the residents at Harbor-UCLA Medical Center on topics I wish I learned about during my pediatric residency but only
acquired by having my own children and in practice. Most of the authors for the first edition were my fellow faculty.
As we move into the sixth edition, the landscape of pediatrics has continued to change and the book has expanded significantly, with
157 chapters, more than 1,000 pages, and authors from around the United States.
While we have conquered many diseases, particularly infectious ones, other problems have befallen our children. These problems,
such as poverty, violence, and exploitation, were no doubt present when the first edition was written, but they certainly weren’t center
stage on our radars. I am pleased with how we have been able to remain in the forefront of pediatrics, expanding to cover the key issues
confronting children, their parents, and their communities in the 21st century and recognizing the changing face of the practice of
medicine, be it health systems science or population medicine.
I want to thank all our contributors for the quality of their work and for the recommendations for additional chapters. I also want
to acknowledge the suggestions we have received from doctors in other disciplines, including family and emergency medicine and the
nurse practitioner community.
The Publishing staff members at the American Academy of Pediatrics are outstanding. Mary Kelly, senior editor, professional and
clinical publishing, was a particularly patient editor with the reassuring phrase, “It’s all good.” Thanks also to Laura Underhile, editor,
professional/clinical publishing, and Alain Park for their work on the companion instructor’s guide. And to Claudette Hoskins, thanks for
your help, patience, and the ability to figure out what was wrong with my computer!
Carol D. Berkowitz, MD, FAAP

xxi

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BPPCA6e_FM_i-xxiv.indd 22 2/14/20 9:37 AM
 Using This Book
For the first time, Berkowitz’s Pediatrics: A Primary Care Approach has been expanded to a 3-part system that further reinforces the key
messages found in the text. The 3 components of this system are
ww A textbook for use by students and instructors
ww An instructor’s guide
ww Student worksheets
Together, they form a comprehensive program for students, pediatric residents, pediatric nurse practitioners, pediatric physician
assistants, and other health care professionals.

Autism Spectrum Disorder

CHAPTER 132

Textbook
Robin Steinberg-Epstein, MD

CASE STUDY
The mother of 18-month-old twin boys is concerned
because 1 twin is not talking as much as his twin sibling.
about whom the mother is concerned seems to have
extreme stranger anxiety. He appears well otherwise.
The sixth edition of Berkowitz’s Pediatrics: A Primary Care Approach
continues its tradition of providing clear, practice-oriented guidance
Both twins are quite active. The mother feels that even
though the child is quiet, he is very smart. He likes to figure
Questions
1. What is autism spectrum disorder?
out how things work. He seems very sensitive to sounds and
2. How does autism spectrum disorder differ from
covers his ears around loud noises. He loves music and even
language delay?
knows which CD his favorite song is on. He will interact with

on the core knowledge in pediatrics. This patient-focused, practical

3. How does the physician evaluate a child for autism
his sibling but does not seem interested in other children.
spectrum disorder?
During the office visit, both boys are quite active. It
4. Where can a physician refer a patient with autism
is difficult to perform an adequate examination because
spectrum disorder?
the twin with limited language is crying the entire time.
5. What types of treatment are available?
He does not seem to seek out his mother for comfort.
6. Should a child suspected of having autism spectrum

text strives to present users with the situations and challenges they
Although both children have stranger anxiety, the twin
disorder receive further immunizations?

Autism spectrum disorder (ASD) is characterized by impairments in

are most likely to encounter in their careers.

Box 132.1. Diagnostic Criteria
social communication as well as restrictive, repetitive, and stereotypic
for Autism Spectrum Disorder
behaviors or interests. According to the Diagnostic and Statistical
Manual of Mental Disorders, 5th Edition (DSM-5), a person with w Deficits in social communication and interactions
ASD must display persistent communication, interaction, and — Social-emotional reciprocity

Five new chapters have been added to this edition of the text:
behavioral challenges across multiple contexts. These distur- — Nonverbal communication
bances must be present early on but may not be apparent until social — Developing, maintaining, and understanding relationships
demand exceeds the limitation. These characteristics must cause sig- w Preferred patterns of behavior, interests, or activities
nificant impairment and cannot be caused by cognitive impairment — Repetitive, stereotypic motor movements, use of objects, or speech
(Box 132.1). Cognitive impairment is often a comorbidity, however. — Need for sameness, routines, and patterns of verbal or nonverbal

ww Health Systems Science

This new term, ASD, includes the previous terminology of autis- behavior
tic disorder, Asperger syndrome, and pervasive developmental — Fixated interests of abnormal intensity or focus
disorder–not otherwise specified; the term ASD no longer includes — Increased or decreased reactivity to sensory input or sensory aspects
Rett syndrome. Although criteria differ somewhat, all these disor- of the environment
ders had in common an impairment in social communication and

ww Population Health for Pediatricians

repetitive or unusual interests of varying degrees. These disorders Derived from Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.

require similar management and treatment, and assessing the level

equates to 1 in every 38 boys. Affected girls are often more impaired
of impairment is somewhat subjective. Therefore, a single term—
than boys, however. Autism is considered the fastest-growing devel-
ASD—best incorporates all those individuals who are significantly
opmental disability. This increase is, in part, the result of an under-

ww Social Determinants of Health: Principles

affected by its symptomatology.
standing of a broader phenotype.

Epidemiology Clinical Presentation

As recently as 1999, the prevalence of ASD was thought to be 1 in Autism spectrum disorder is truly a spectrum of social communication

ww Adverse Childhood Experiences: Trauma-Informed Care

2,500. More recent numbers from the Centers for Disease Control deficits. Although a certain set of behaviors defines the disorder, any
and Prevention published in 2014 cite a prevalence of 1 in 59 chil- child may have any combination of the symptoms that result in the same
dren in the United States. The prevalence in Europe, Asia, and North outcome—severe and incapacitating social deficits. Furthermore, the
America averages between 1% and 2% of the overall population. challenges experienced by this population are more than just develop-
Boys are affected approximately 4 times as often as girls, which mental delays; the behaviors of these individuals are aberrant and odd.
989

ww Commercially Exploited Children and Human Trafficking

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Instructor’s Guide
CHAPTER 132

Autism Spectrum Disorder

The newly created Berkowitz’s Pediatrics Instructor’s Guide is
designed to help facilitate learner-initiated discussion about core
CASE STUDY
The mother of 18-month-old twin boys is concerned because 1 twin is not talking as much as his twin sibling. Both
twins are quite active. The mother feels that even though the child is quiet, he is very smart. He likes to figure out
how things work. He seems very sensitive to sounds and covers his ears around loud noises. He loves music and

pediatric principles and common pediatric conditions. It also

even knows which CD his favorite song is on. He will interact with his sibling but does not seem interested in other
children.
During the office visit, both boys are quite active. It is difficult to perform an adequate examination because the
twin with limited language is crying the entire time. He does not seem to seek out his mother for comfort. Although
both children have stranger anxiety, the twin about whom the mother is concerned seems to have extreme stranger

provides answers to the case study questions presented in each

anxiety. He appears well otherwise.

Questions
1. What is autism spectrum disorder?
Autism spectrum disorder (ASD) is characterized by
impairments in social communication as well as restrictive,
repetitive, and stereotypic behaviors or interests. According to
though they can neither speak functionally nor comprehend
what they read.
Inconsistent symptoms are the hallmark of this
disorder. Some parents or guardians of children with
chapter of the textbook. The instructor’s guide allows for instructor
and program flexibility as to how the book and the accompanying
the Diagnostic and Statistical Manual of Mental Disorders, ASD describe a phenomenon whereby the children are
5th Edition (DSM-5), a person with ASD must display developing normally until 12 to 15 months of age and then
persistent communication, interaction, and behavioral suddenly lose skills or stop progressing. This finding is
challenges across multiple contexts. particularly concerning.
This new term, ASD, includes the previous terminology 3. How does the physician evaluate a child for autism

questions are used.

of autistic disorder, Asperger syndrome, and pervasive spectrum disorder?
developmental disorder–not otherwise specified; the term
No single diagnostic test, blood or otherwise, can confirm the
ASD no longer includes Rett syndrome. Although criteria
diagnosis of ASD. Diagnosis is based on history, interaction
differ somewhat, all these disorders had in common an
with the child, and meeting DSM-5 criteria.
impairment in social communication and repetitive or
Regular developmental surveillance and screening should
unusual interests of varying degrees. These disorders require
be part of every well-child evaluation, especially between
similar management, and assessing the level of impairment
ages 9 and 30 months. The Modified Checklist for Autism
is somewhat subjective. Therefore, a single term—ASD—best
in Toddlers, Revised, with Follow-Up (M-CHAT-R/F) is
incorporates all those individuals who are significantly affected
an excellent autism-specific screening tool with moderate
by its symptomatology.
sensitivity and high specificity for use at the 18- and 24-month
2. How does autism spectrum disorder differ from visits to identify individuals at high risk for ASD.
language delay? Family history is also important, because ASD is presumed
Language delay is isolated delay in the acquisition and to have a genetic contribution and it may be helpful in
expression of language. Autism spectrum disorder, however, identifying other etiologies.
is truly a spectrum of social communication deficits, often Understanding family structure is helpful in determining
including developmental delays in multiple areas. Some whether abuse, neglect, or maternal depression play a role in
affected individuals, because of an incredible ability to the child’s delay. It is important to remember, however, that
recognize patterns, can read as early as 2 years of age, even ASD is not caused by poor parenting.

267

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Student Worksheets
BERKOWITZ’S PEDIATRICS, 6TH EDITION, STUDENT WORKSHEET

CHAPTER 132

Autism Spectrum Disorder

CASE STUDY
Student worksheets corresponding to each chapter’s case study
questions are available online at https://services.aap.org/en/
The mother of 18-month-old twin boys is concerned because 1 twin is not talking as much as his twin sibling. Both
twins are quite active. The mother feels that even though the child is quiet, he is very smart. He likes to figure out
how things work. He seems very sensitive to sounds and covers his ears around loud noises. He loves music and
even knows which CD his favorite song is on. He will interact with his sibling but does not seem interested in other
children.

publications/berkowitz/ in a user-friendly format that users can

During the office visit, both boys are quite active. It is difficult to perform an adequate examination because the
twin with limited language is crying the entire time. He does not seem to seek out his mother for comfort. Although
both children have stranger anxiety, the twin about whom the mother is concerned seems to have extreme stranger
anxiety. He appears well otherwise.

Questions
1. What is autism spectrum disorder?
download, complete, and print or email to prepare for discussions.
2. How does autism spectrum disorder differ from language delay?

3. How does the physician evaluate a child for autism spectrum disorder?
The case study questions contained in the worksheets are designed
4. Where can a physician refer a patient with autism spectrum disorder?

5. What types of treatment are available?

to help users critically apply the theories presented throughout the
6. Should a child suspected of having autism spectrum disorder receive further immunizations? textbook. Students will be able to answer all case study questions by
CASE RESOLUTION
reading the corresponding chapter in the text. It is also appropriate
to access some of the resources referenced in the chapters.
The child’s parent completed an M-CHAT-R/F, and the child scored a 4 (ie, intermediate risk). A follow-up
interview confirmed that the risk for ASD was significant, and the child was evaluated by a developmental-
behavioral pediatrician and the local governmental agency, where he underwent a comprehensive assessment by
a multidisciplinary team. The diagnosis of autism was confirmed, and his brother was noted to have a language
delay. Both children were placed in an early intervention program. The primary patient was placed in a 1:1
structured teaching environment for 4 months. After exhibiting significant improvement, he was moved to a
therapeutic preschool setting that emphasized generalization of his newly acquired skills, speech therapy,
occupational therapy, and social skills. His brother received speech therapy 2 times per week. Both are due to
start a regular kindergarten class in the fall, with ongoing speech and social support. The primary patient has
been placed on a stimulant medication to control hyperactivity and problems with attention.

The recommendations in this publication do not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account
individual circumstances, may be appropriate. Original resource included as part of Berkowitz’s Pediatrics: A Primary Care Approach, 6th Edition. © 2020 American
Academy of Pediatrics. All rights reserved.

xxiii

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BPPCA6e_FM_i-xxiv.indd 24 2/14/20 9:37 AM
 PART 1

Primary Care:
Skills and Concepts
1. Primary Care: Introduction .................................................3
2. Talking With Parents ...........................................................7
3. Talking With Children .......................................................13
4. Talking With Adolescents...................................................17
5. Telephone Management and E-medicine..........................21
6. Informatics .........................................................................27
7. Counseling Families About Internet Use ..........................33
8. Cultural Competency Issues in Pediatrics ........................39
9. Global Child Health ...........................................................45
10. Child Advocacy ..................................................................51

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BPPCA6e_Ch001_0001-0006.indd 2 2/14/20 10:40 AM
 CHAPTER 1

Primary Care: Introduction

Niloufar Tehrani, MD

CASE STUDY
As a primary care physician, you evaluate a 2-year-old boy Questions
who is presenting to the office for the first time. The mother 1. What are the 4 components of primary care?
states he has always been small; he was born at term but 2. What are the main characteristics of a medical home?
weighed only 2,272 g (5 lb). She is a single mother, and he What are the eligibility criteria for designating a
is her only child. He speaks only 5 words and is quite active. practice as a medical home?
The physical examination is normal, but the boy’s height 3. What is the difference between a consultation and
and weight are less than the fifth percentile. The mother a referral?
reports her son is immunized, but she does not have his 4. Why are laboratory tests done during a routine
immunization records with her at this visit. health maintenance visit?

Primary care is defined as the comprehensive health care a patient viewing patients in the context of their environment and family. In
receives from the same health professional over a longitudinal pediatrics, a child’s problems become the family’s, and the family’s
period. The term was first used in the 1960s to designate the role problems become the child’s. This has become increasingly appar-
of the primary care physician in response to the abundance of sub- ent with the recognition that the social determinants of health (eg,
specialists and lack of generalists among practicing physicians. It problems of poverty, drug use, obesity, teenage pregnancy, and gang
is generally accepted that primary care physicians include pedia- involvement), directly affect a child’s health and quality of life (see
tricians, family physicians, and internists. In 1966, The Graduate Chapter 141). The psychosocial forces in a particular child’s life are
Education of Physicians: The Report of the Citizens Commission intricately interwoven into that child’s health care, and the assess-
on Graduate Medical Education (the Millis Committee Report) to ment of these forces is an essential component of the primary care of
the American Medical Association recognized the importance of that child. Environmental exposures (eg, lead contamination of the
primary care and recommended a national commitment to edu- water supply) have a direct effect on a child’s health, and the primary
cating primary care physicians. Primary care was further defined in care physician must have knowledge of those environmental threats.
1974 by Charney and Alpert, who separated it into component parts: The fourth component of primary care, integration of compre-
first contact, longitudinal care, family orientation, and integration hensive care, involves the use of health and educational resources
of comprehensive care. To comprehend the depth of primary care, in the community to supplement care as a means of addressing the
it is necessary to understand its component parts. increased complexity of pediatric medical problems. Primary care
First contact occurs when a patient arrives for medical care at physicians integrate and coordinate these services in the best inter-
the office of a primary care physician. The visit includes an intake est of patients. Working with social service agencies, home care pro-
history, complete physical examination, screenings appropriate for viders, educational agencies, and government agencies, physicians
age, and an assessment of problems with treatment, if indicated. can use multiple resources for the benefit of patients. Understanding
Of great importance is the establishment of the physician-patient the available community resources is an important part of a primary
relationship. Physicians become the primary medical resource and care physician’s education.
counselors to these patients and their families and the first contacts
when successive medical problems arise.
Longitudinal care, the second component of primary care, Medical Home
implies continuity of care over time. Physicians assume responsi- When patients select a primary care physician, they have identified
bility for issues concerning health and illness. In pediatrics, such a medical home. The medical home incorporates the physical, psy-
care involves monitoring growth and development, following school chological, and social aspects of individual patients into compre-
progress, screening for commonly found disorders, conducting psy- hensive health care services, thus meeting the needs of the whole
chosocial assessments, promoting health, preventing illness with person. This concept of the medical home was first documented
immunizations, and providing safety counseling programs. by the American Academy of Pediatrics (AAP) in 1967 in the book
Family orientation, the third component of primary care, is a Standards of Child Health Care, which noted that a medical home
recognition that the provision of adequate care is dependent on should be a central source of all the child’s medical records. The
3

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 4 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

idea of a medical home developed into a method of providing com- behavior problems. The focus of the pediatrician should be detec-
prehensive primary care and was successfully implemented in the tion, evaluation, and management, with referrals if necessary. Newer
1980s by Calvin Sia, MD, FAAP, in Hawaii. He is considered to be the morbidities secondary to the increasing complexity of our society
“father” of the medical home. In policy statements published in 1992 were outlined in 2001 by the AAP. These include school problems,
and 2002 (the latter reaffirmed in 2008), the AAP defined the char- mood and anxiety disorders, adolescent suicide and homicide, fire-
acteristics of a medical home to be “accessible, continuous, compre- arms, school violence, drug and alcohol abuse, HIV, obesity, and the
hensive, family-centered, coordinated, compassionate, and culturally effects of the media on children. Other psychosocial factors, such as
effective.” Geographic and financial accessibility are key elements in poverty, homelessness, single-parent families, divorce, working par-
making that home work for patients. The most important aspect of a ents, and child care, necessitate that pediatricians work with social
home, however, is that it be a place in which patients feel cared for. service agencies to deliver appropriate care to their patients. The role
Since its implementation in pediatrics in 2004, the medical home of the primary care physician is continually expanding in an effort
model was adopted by the American Academy of Family Physicians to deliver comprehensive care to each patient in a medical home.
and the American College of Physicians. The definition of the med- This care is often rendered by physician-led teams that include other
ical home was expanded to include use of electronic information health professionals.
services, population-based management of chronic illness, and
continuous quality improvement. The concept has been accepted Subspecialist Care
as a form of high-quality health care. Cost and quality of bene-
Considerable advancement has been made in medical knowledge
fits have been well documented. Recognizing these benefits, large
and technology in the past several decades. Total knowledge of all
corporations in collaboration with health professionals formed the
fields is impossible for any individual physician. As a result, the role
Patient-Centered Primary Care Collaborative to promote the idea of
of the subspecialist physician has developed as an adjunct to that
designated medical homes. As part of that collaborative, the National
of the primary care physician. New fields of subspecialties, such as
Committee for Quality Assurance adopted eligibility criteria for a
child abuse pediatrics, have arisen as a response to increased knowl-
practice to define itself as a medical home. Requirements for the
edge. The primary care physician should seek subspecialist consul-
designation include the adoption of health information technol-
tation when the suspected or known disease process is unusual or
ogy and decision-support systems, modification of clinical practice
complicated, in cases that require the use of specialized technol-
patterns, and ensuring continuity of care.
ogy, and in situations in which the primary care physician has lit-
With the advent of health care reform in the United States, as
tle experience with the disease. Generally, subspecialists evaluate
part of the effort to control the rising cost of health care, the federal
patients and concentrate on the organ system or disease process in
government has endorsed the concept of the medical home model.
their area of expertise.
The Academic Pediatric Association has defined the family-centered
Use of a subspecialist is termed secondary care. The primary
medical home to delineate the dependency of the child to the fam-
care physician can elicit the help of a subspecialist in the form of
ily and community in the medical home model. This principle was
a consultation or a referral. When initiating a consultation, the
highlighted in a consensus statement that was developed and jointly
primary care physician seeks advice from the consultant on workup
endorsed by the AAP, American College of Physicians, American or management of the patient. The consulting physician assesses
Academy of Family Physicians, and the American Osteopathic the patient with a history and physical examination, focusing
Association. on the particular specialty. The consultant recommends possible
additional laboratory tests and offers a diagnosis and treatment plan,
Role of the Primary Care Pediatrician after which the patient returns to the primary care physician for
As a primary care physician, the pediatrician has a role that has coordination of further care.
included not only the management of acute illness and injury but Electronic, abbreviated consultations can now be conducted
also the preventive aspects of well-child care with its focus on using an e-consultation system. These consultations give the pri-
immunizations, tracking growth and development, and anticipa- mary care physician a treatment plan, which may also include 1 or
tory guidance. Currently, there exists a renewed emphasis on the more visits to the subspecialist. For example, an 8-year-old girl with
importance of the role of the pediatric primary care physician in weight loss and persistent abdominal pain has an upper gastrointes-
assessing the psychosocial aspects of pediatric patients. Evaluation tinal radiograph series that reveals a duodenal ulcer. Her primary
of social issues such as family dysfunction, developmental problems care physician requests a consultation from a pediatric gastroen-
(including learning disabilities) and behavioral problems (including terologist for an endoscopy to allow definitive diagnosis and up-to-
emotional disorders), termed the new morbidity by Robert Haggerty, date management guidelines. After the procedure, the girl returns
MD, in the 1970s, has become a significant part of the role of the to the primary care physician with recommendations for treatment
physician. In 1993, the AAP stated that pediatricians are obliged to and further care.
have knowledge of physical and environmental factors and behaviors Primary care physicians can also generate a referral to a subspe-
affecting health, normal variations of behavior and emotional devel- cialist, which differs from a consultation. A referral requests that the
opment, risk factors and behaviors affecting physical health, and subspecialist assume complete care of the patient. This transfer of

BPPCA6e_Ch001_0001-0006.indd 4 2/14/20 10:40 AM

 Chapter 1: Primary Care: Introduction 5

care may be to a tertiary care site where a subspecialist provides care the risk of future disease. Organ function, metabolic activity, and
and assumes responsibility for coordinating further patient care. For nutritional status also can be assessed, and evidence of neoplastic or
example, a 4-year-old boy with recurrent fever, hepatosplenomegaly, infectious disease can be provided. Additionally, laboratory studies
and blasts on peripheral blood smear is referred to a pediatric oncol- can be used to identify infectious and therapeutic agents or poisons.
ogist for diagnosis, treatment, and ongoing medical care. Screening laboratory tests are used when the incidence of an
When requesting advice from subspecialists, whether on a con- unsuspected condition is sufficiently high in a general population
sultative or referral basis, the primary care physician should outline to justify the expense of the test (see Chapter 13). Subclinical con-
specific questions with a probable diagnosis to be addressed by the ditions, such as anemia, lead poisoning, and hypercholesterolemia,
subspecialist. For example, a consultation requesting evaluation of are part of some health maintenance assessments.
a child with hematuria is inappropriate. The primary care physician Physicians must remember that variability exists in test results
should perform a basic diagnostic evaluation and suggest the most and that laboratory error can occur. Laboratory results should always
likely diagnosis, after which the child can be referred appropriately. be viewed in the context of the patient. The sensitivity of a test, the
For example, a child with a diagnosis of nephritis should be sent to ability of the test to detect low levels, and the specificity of a test for
a pediatric nephrologist, whereas a child with a diagnosis of Wilms the substance being measured must also be considered by the phy-
tumor should be sent to a pediatric oncologist. sician when evaluating a test result.
When primary care physicians and subspecialists function
cooperatively and offer 3 levels of care (ie, primary, referral, Challenges for the Future
consultative), patients receive the highest quality medical care. The role of the primary care physician in health care delivery
Generally, care provided by subspecialists is characterized as being has increased in importance. In 2010, the Patient Protection and
more expensive and procedure driven. Subspecialists order more Affordable Care Act was signed into law. This law emphasizes the
laboratory studies than primary care physicians, which further importance of the medical home and promotes its implementation.
inflates the cost of medical care. Additionally, if a patient lacks lon- Two of the basic tenets of primary care—accessibility and an ongo-
gitudinal health care and sees multiple practitioners, often repeat ing relationship with the primary care physician, both of which are
laboratory studies are ordered. Compared with subspecialty care, reported by patients to be very important—are recognized as essen-
primary care is believed to deliver more cost-effective medical tial components of the medical home. The challenge continues to
care. The spiraling cost of medical care has resulted in continued ensure continuity in health care funding to preserve the continuity of
nationwide emphasis on producing more primary care physicians. the medical home. Payment reform promises to improve payment to
It should be remembered, however, that the subspecialist plays an primary care practices and rewards high performance. As proposed
essential supplementary role to the primary care physician when in health care reform, through accountable care organizations, pri-
managing complicated diseases. A balance between generalists
mary care physicians would be the foundation of the organization
and subspecialists must be maintained in the education process.
whose mission is management of the continuum of care and cost as
well as ensuring quality of care.
Laboratory Tests Access to same-day care, which is part of the obligation of the
For most conditions, the diagnosis is revealed by the history and medical home and essential to pediatric patients, can be difficult
physical examination in more than 95% of cases. Thus, good commu- in the busy schedule of primary care physicians. Practices must
nication skills are a basic tenet of primary care. Patients frequently accommodate these visits. Community health centers can provide
complain about unnecessary laboratory tests, which increase the excellent medical homes for children in families with low income;
cost of medical care, and the prescription of unnecessary medica- however, these centers can have challenges with accessibility and
tions. To lessen these problems, the primary care physician should adequate referral sources. Walk-in immediate medical care clin-
be discriminating when ordering laboratory tests and prescribing ics and retail clinics have arisen, but episodic visits in a vari-
medications, recognizing their value as well as their potential ety of settings do not deliver comprehensive care for the patient,
iatrogenic effects. and these short visits may not take into account the entirety of
In primary care, laboratory tests are used to help confirm a con- the patient’s medical history. This creates a challenge for the pri-
dition suspected on the basis of the history or physical examination mary care physician and medical home to develop a system to
or diagnose a condition that may not be apparent after a thorough integrate the information from these encounters into the com-
history and physical assessment. In pediatrics especially, the value prehensive medical record.
of each test result should be weighed against the inconvenience, dis- With the advent of hospitalists providing inpatient care, pri-
comfort, and possible side effects in children. Tests in at-risk chil- mary care physicians may not be included in inpatient manage-
dren can also be used as screening tools to prevent disease or identify ment, which can make it challenging for primary care physicians
a disease early so that treatment can begin and symptoms can be to retrieve important information about the care of their patients.
minimized. Laboratory studies can provide a host of other infor- Medical care reform incorporates accountability, demonstration
mation, including data to establish a diagnosis, knowledge neces- of quality of care, and standards of medical practice into the medical
sary to select therapy or monitor a disease, and information about home model, which has resulted in an exponential increase in the

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 6 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

oversight and bureaucracy of medical care. This business of med- Selected References
icine with redundant oversight of medical care has placed a tre-
mendous burden of administrative activities on the primary care American Academy of Pediatrics. AAP agenda for children: medical home.
2014-2015. AAP.org website. www.aap.org/en-us/about-the-aap/aap-facts/AAP-
physician. Physicians face a significant challenge in providing care
Agenda-for-Children-Strategic-Plan/Pages/AAP-Agenda-for-Children-Strategic-
while answering to administrative structures. Additionally, although Plan-Medical-Home.aspx. Accessed July 1, 2019
the use of electronic medical records decreases some of the chal-
American Academy of Pediatrics Committee on Psychosocial Aspects of Child
lenges of information retrieval and communication among medical and Family Health. The new morbidity revisited: a renewed commitment to
providers, it poses other challenges in a potential lack of pediatric the psychosocial aspects of pediatric care. Pediatrics. 2001;108(5):1227–1230
functionality and loss of productivity. PMID: 11694709 https://pediatrics.aappublications.org/content/108/5/1227.long
The biggest challenge for pediatric primary care physicians has American Academy of Pediatrics Medical Home Initiatives for Children With
always been to ensure the future of health care funding to pro- Special Needs Project Advisory Committee. The medical home. Pediatrics.
vide all children access to and availability of a medical home. The 2002;110(1):184–186. Reaffirmed May 2008 PMID: 12093969
Patient Protection and Affordable Care Act aims to provide health American Academy of Pediatrics Committee on Practice and Ambulatory
coverage for nearly all children, but in a multipayer, market-driven Medicine. AAP principles concerning retail-based clinics. Pediatrics.
health care system, significant challenges will remain. A multi- 2014;133(3):e794–e797. Retired May 2017. PMID: 24567015 https://doi.
tude of programs exist to pay for children’s health care, and these org/10.1542/peds.2013-4080
programs vary by state. Families move among payers, which dis- Carrier E, Gourevitch MN, Shah NR. Medical homes: challenges in translating
rupts continuity of care. Universal health care for children is being theory into practice. Med Care. 2009;47(7):714–722 PMID: 19536005 https://
doi.org/10.1097/MLR.0b013e3181a469b0
advocated. Without a secure national plan for financing, children’s
health care will continue to be variable, resulting in disparities in Cheng TL, Wise PH, Halfon N. Quality health care for children and the
Affordable Care Act: a voltage drop checklist. Pediatrics. 2014;134(4):794–802
children’s health.
PMID: 25225140 https://doi.org/10.1542/peds.2014-0881
Hoilette LK, Blumkin AK, Baldwin CD, Fiscella K, Szilagyi PG. Community
health centers: medical homes for children? Acad Pediatr. 2013;13(5):436–442
CASE RESOLUTION PMID: 24011746 https://doi.org/10.1016/j.acap.2013.06.006
You ask the mother about her son’s former physician and obtain signed permis- Lehmann CU, O’Connor KG, Shorte VA, Johnson TD. Use of electronic health
sion to get the prior medical records, including immunizations. You attempt a record systems by office-based pediatricians. Pediatrics. 2015;135(1):e7–e15
hearing assessment as the initial step in evaluating his speech delay, but the PMID: 25548325 https://doi.org/10.1542/peds.2014-1115
patient does not cooperate. You ask the mother about access to food and com-
Rosenbaum S. The Patient Protection and Affordable Care Act and the future of
plete a referral to the Special Supplemental Nutrition Program for Women,
Infants, and Children and provide her with information about the Supplemental child health policy. Acad Pediatr. 2012;12(5):363–364 PMID: 22999352 https://
Nutrition Assurance Program (ie, food stamps). You provide the patient with an doi.org/10.1016/j.acap.2012.07.005
age-appropriate book from Reach Out and Read and make a return appointment Stille C, Turchi RM, Antonelli R, et al; Academic Pediatric Association Task
for 1 month hence to continue care and determine whether the patient needs Force on Family-Centered Medical Home. The family-centered medical home:
any immunizations. specific considerations for child health research and policy. Acad Pediatr.
2010;10(4):211–217 PMID: 20605546 https://doi.org/10.1016/j.acap.2010.05.002

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 CHAPTER 2

Talking With Parents

Geeta Grover, MD, FAAP

CASE STUDY
An 8-month-old boy with a 1-week history of cough and Bring him back here or to his regular doctor if his fever
runny nose; a 2-day history of vomiting, diarrhea, and persists, he doesn’t eat, he has too much vomiting or
fever; and a temperature of 38.3°C (101°F) is evaluated diarrhea, he looks lethargic, or if he isn’t better in 2 days.”
in the emergency department (ED). The mother is very
concerned because her son’s appetite has decreased, and
Questions
1. How much information can most parents absorb
he has been waking up several times at night for the past
at one time? Did this mother receive more
2 days.
information than she can reasonably be expected
A nurse interrupts and says that paramedics are bring-
to remember?
ing a 5-year-old trauma victim to the ED. The appearance
2. How do you assess parental concerns? Did the
of the 8-month-old child is quickly assessed; he seems
physician sufficiently address the mother’s worries?
active and alert. Bilateral otitis media is diagnosed.
3. How do you know whether a parent has understood
Before leaving the examination room the physician says
all the information? Was this mother given a chance
to the mother, “Your son has a viral syndrome and infec-
to clarify any questions she had?
tion in his ears. I am going to prescribe an antibiotic that
4. What are some barriers to effective doctor-parent
you can begin giving him today. Give him ibuprofen as
communication?
needed for the fever. Don’t worry about his vomiting and
5. How does the setting itself influence communication?
diarrhea; just make sure that he drinks plenty of liquids
and don’t give him milk or milk products for a few days.

Communication is the foundation of the therapeutic relationship between physicians, parents, and children. Through practice and
between physicians, patients, and patients’ families. Effective continued awareness of interpersonal abilities, the physician can
communication in the pediatric setting involves the exchange of develop good communication skills. All physicians eventually
information between physicians, parents, and children. In addi- develop their own personal interviewing and examination style.
tion, observing the interaction between parents and children gives What seems awkward and difficult at first soon becomes routine
physicians an opportunity to assess parenting skills and the dynam- and even enjoyable as the physician becomes more comfortable with
ics of the parent-child relationship. The communication needs of patients and their families.
parents and children are quite different, which makes the exchange
of information challenging. Parental concerns should be addressed
in a sensitive, empathetic, and nonjudgmental manner. A non- Parental Concerns
threatening, pleasant demeanor and age-appropriate language help Parents’ preconceived ideas and concerns about their children’s
facilitate communication with children (see Chapter 3). illnesses can greatly influence the exchange of information between
Pediatrics encompasses not only the traditional medical model physicians and parents. At health maintenance or well-child visits,
of diagnosis and treatment of disease but also maintenance of the it is important for the pediatrician to address parents’ nonmedical
health and well-being of children through longitudinal care and and psychosocial concerns, such as their children’s development,
the establishment of ongoing relationships between physicians and nutrition, and growth. Often these questions stem from discus-
families. Personal relationships between physicians and families sions with other parents or, increasingly, from information received
create an atmosphere in which information can be exchanged from various online and media resources (see Chapter 7). Although
openly. The pediatrician’s role in such relationships is to not only such concerns may seem trivial to the pediatrician, they may be
diagnose and treat but also to listen, advise, guide, and teach. extremely important to parents. In addition to addressing the needs
The doctor-patient relationship is truly a privilege. Patients of the child, the health maintenance visit also affords the pediatri-
entrust physicians with their innermost thoughts and feelings, cian an opportunity to assess and address parental needs. Parental
allow them to touch private parts of their bodies, and trust them to depression, substance abuse, family violence, or marital discord all
perform invasive procedures or administer medications. Mutual can have profound effects on children’s health and development.
respect is essential for the development of a healthy relationship Similarly, the conditions in which children and their families live,
7

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 8 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

learn, work, and play can affect both physical and emotional health. into and understanding of their children’s behavior and needs,
Collectively, these conditions are known as the social determinants and actively seek out their observations and incorporate their
of health (see Chapter 141). family preferences into the care plan as much as possible. Benefits
When evaluating children brought in for illness, it is important to of family-centered care include a stronger alliance between the
ask parents what concerns them most. Parental fears may be much physician and family; increased patient, family, and professional
different from medical concerns. Failure to give parents the oppor- satisfaction; and decreased health care costs. Since the passage
tunity to ask questions or to address these concerns in a sensitive of the Health Information Technology for Economic and Clinical
manner may result in dissatisfaction and poor communication. Health (HITECH) Act in 2009, the electronic medical record (EMR)
is increasingly affecting the practice of medicine (see Chapter 6).
The Pediatric Interview Although the EMR has the potential to improve patient understand-
ing of health information as well as to improve sharing of medical
Pediatric interviews are conducted in a variety of settings for many
information, without conscious effort to adjust clinical speaking
different reasons. The first interaction between the physician and
and documentation practices, this new model of practice may also
parent or parents may be during the prenatal interview before the
negatively affect patient-centered communication. Some behav-
birth of the child, in the hospital following the delivery, or in the
iors that facilitate patient-centered communication with the EMR
doctor’s office during the well-baby visit. Later, the physician may
include screen-sharing, cessation of typing during sensitive discus-
see a child in the office for regular health maintenance visits or in the
sions, and maintaining eye contact or continuing to speak while
office, emergency department (ED), or hospital for an acute illness.
typing. In this digital age, physicians are learning to listen, talk,
The specific clinical situation dictates the information that must
think, and type simultaneously.
be gathered and the appropriate interviewing techniques. During the
The medical visit may be divided into 3 parts: the interview,
prenatal visit, the physician should discuss common concerns and
anxieties about the new baby with the prospective parent or parents. physical examination, and concluding remarks. Examples of
In addition, the prenatal visit affords the parent or parents an oppor- doctor-parent and doctor-child communications for each of these
tunity to interview physicians and evaluate their offices and staff. components are provided in Table 2.1.
In the emergency setting, the physician must elicit pertinent
information necessary to make decisions about management within Interview
a short period. Lack of a long-term relationship can make commu- The goal of the interview is to ascertain the chief concern, deter-
nication in the ED particularly challenging. The physician should mine appropriate medical history, and gain an understanding of
mostly use focused, closed-ended questions in this setting. For the family’s perspective of the illness or its specific concerns. It is
the periodic health maintenance visit, however, the use of broad, important to address cognitive (ie, informational) and affective
open-ended questions is more appropriate, and closed-ended (ie, emotional) needs of the family during the interview. The
questions should be used only as necessary for clarification. interview usually begins with open-ended questions to give par-
ent and child an opportunity to discuss their concerns and outline
Communication Guidelines their agenda for the visit. Often, the real reason for the visit is not
Professionalism encompasses technical, intellectual, and human- disclosed until the family believes the physician to be trustworthy
istic competencies. Clinicians are increasingly seeing conditions and honest. Rachel Naomi Remen, MD, coined the term generous
that may not be treatable; however, that does not mean the listening to describe a technique of receiving and respecting infor-
clinician cannot provide healing. Whereas “treatment” focuses on mation without judgment or any agenda to analyze it and deter-
cure, “healing” is about building relationships with patients and mine what to do next. Generous listening creates a space of safety
helping them optimize emotional and physical health so that they that allows parents and children to say what they perceive to be
may continue to pursue what has meaning and value for them. true. After issues have been laid out, closed-ended questions can
Overall principles that are applicable regardless of the setting be used to clarify and further define the information presented. It
include interacting with the child and family in a professional yet often becomes necessary to guide the interview, especially when
sensitive and nonjudgmental manner. Common courtesies, such parents have several broad issues on their agenda for that visit and
as knocking before entering the room, dressing and behaving in a time does not permit discussion of them.
professional manner, introducing oneself, and addressing parents The physician should gently acknowledge parental concerns
and children by their preferred names, are always appreciated and define time limitations. These actions allow the physician to
and welcomed. Taking a few moments to socialize with families focus on the most salient issues of that visit. Additionally, the phy-
develops a more personal relationship that may allow more open sician should limit the use of medical jargon (ie, scientific terms)
conversation about sensitive and emotional issues. and be aware of nonverbal communication. A sincere, empathic, and
Family-centered care is an approach to health care in compassionate communication style helps parents feel truly under-
which the physician realizes the vital role that families play in stood even if the physician can do little to help the situation. Pauses
ensuring the health and well-being of children. Physicians who and periods of silence should be used, especially when discuss-
practice family-centered care convey respect for parents’ insight ing emotionally difficult issues, to convey to parents and children

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 Chapter 2: Talking With Parents 9

Table 2.1. Communication Guidelines and Techniques for the Pediatric Medical Visit
Component of Medical Visit Technique Examples
Interview Open-ended questions. “How is Susie?”
Closed-ended questions. “Does she have a cough?”
Repetition of important phrases. “She has had a high fever for 4 days now?”
Reflective listening. “It sounds like you are concerned that this may be serious.”
Clarification. “What do you mean by, ‘Susie was acting funny’?”
Pauses and periods of silence. “I see that it is difficult for you to talk about this. Take your time.”
Limit medical jargon. “Susie has an ear infection” vs “Susie has otitis media.”
Guide the interview. “Right now, I am most interested in hearing about the symptoms of
this illness.”
Be aware of nonverbal communication. Use eye contact and phrases such as “I see.”
Acknowledge parental concerns. “Worrying about hearing loss is understandable.”
Empathize. “A temperature of 104°F can be very frightening.”
Remember common courtesies. Knock before entering.
Recognize personal limitations. “I am not an expert in this area. I would like to consult with a colleague.”
Summarize. “So, she has had fever for 4 days, but the rash and cough began
1 week ago?”
Physical Examination Show consideration for the child. “It’s OK to be afraid.”
Inform. “That took me some time, but her heart sounds normal.”
Explain procedures. “You may feel a little uncomfortable during the rectal examination.”
Avoid exclamations. “Wow! I have never seen anything like this!”
Concluding Remarks Provide closure. “Our time is over today. May we discuss this at the next visit?”
Minimize discharge instructions. “Call me if her rash recurs.”
Be specific. “I am going to treat her with amoxicillin” vs “I’ll prescribe an antibiotic.”
Praise and positive feedback. “You’re doing a great job.”
Confirm parental understanding. “Please repeat for me Susie’s diagnosis and treatment instructions so
I’m sure I’ve been clear in explaining them to you.”
Give the parent or parents permission to ask questions. “Please feel free to ask me about anything that concerns you.”   
Reassurance. “I know you are worried about her high fever, but I can reassure you
that the fact she is playful and hungry are both good signs.”

that their physician cares enough to listen. Physicians should not Motivational interviewing is one such patient-centered, collab-
underrate their own knowledge; however, they should recognize orative, and directive interaction style that offers an effective means
their limitations and use consultants appropriately. Finally, the phy- of addressing these developmental, behavioral, and social concerns
sician’s understanding of the chief concern and history should be within the context of a primary care setting. Motivational interview-
summarized so that the parent or parents have an opportunity to ing addresses the ambivalence and discrepancies between a per-
clarify points of disagreement. son’s current values and behaviors and the person’s future goals. In
The primary care physician faces increasing demands to address contrast to more traditional medical approaches that rely primar-
not only the physical but also the psychosocial health needs ily on authority and education, motivational interviewing is a
of patients. Patient-centered care is a comprehensive approach to collaborative approach that relies on eliciting the patient’s ideas
medical care that encourages communication between the physician, about change. The physician who practices motivational interview-
patient, and family. The clinician addresses the immediate press- ing understands that trying to move beyond a patient’s readiness
ing medical concerns in the context of each patient’s unique envi- to change is likely to increase that patient’s resistance to treatment;
ronmental circumstances and underlying psychosocial concerns, for example, lecturing to an adolescent who is not yet ready to quit
both of which may directly or indirectly affect health-related out- smoking about the dangers of smoking is unlikely to be effective
comes. Empathy, unconditional positive regard, and genuineness are and may even produce more resistance. Motivational interviewing
essential physician characteristics in this collaborative approach. requires that the physician follow the 4 principles listed in Table 2.2.

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 10 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Table 2.2. Principles of Motivational Interviewing and a consumer model are replacing the traditional paternalistic
medical model in which the physician decided what should be done
Principle Example
and the patient accepted the recommendations without question.
Express empathy. Use reflective listening. In this shared decision making model, it is important to assess
Identify discrepancy between Patient, not physician, presents parental readiness for knowledge (especially in emotionally diffi-
patient’s current behavior and arguments for change. cult situations) and keep family resources and limitations in mind.
treatment goal. Discharge instructions should be minimized, the physician should
Decrease the likelihood of evoking Avoid arguing for change. be specific, and the number of diagnoses, medications, and “as
patient resistance. needed” instructions (ie, indications for seeking medical advice,
Support the patient’s self-efficacy. Patient’s own belief in the possibility such as “return as needed for high fever”) should be limited. When
of change is an important motivator. complicated discharge instructions are given, additional physician
Derived from Miller WR, Rollnick S. Motivational Interviewing: Helping People Change. 3rd ed.
time may be required to ensure parental understanding. Praising
New York, NY: Guilford Press; 2013. parents on care of their children can boost their self-esteem
and confidence and may minimize calls and questions. Parental
understanding should be confirmed; parents should be asked
Operationally, open-ended questions (eg, “How do you feel about to repeat the diagnosis and treatment plan. Simply asking par-
smoking?”), affirmations (eg, “You are tired of having to monitor ents if they have understood is not enough because they often
your blood sugar every day and stick to your diet.”), and reflective say “yes” out of respect for the physician’s time or embarrassment
listening (eg, “You are worried about your daughter’s behavior and that they have not understood what has been said. For exam-
are concerned that if it persists, she may be expelled from school.”) ple, the physician could say, “I want to be sure that I’ve spoken
are important tools of motivational interviewing. In addition, physi- clearly enough. Please repeat for me [child’s name] diagnosis and
cians who practice motivational interviewing ask permission before treatment instructions.”
giving advice (eg, “Would it be OK if I shared some information
with you?”). Alternatively, the physician may state the facts but let Barriers to Effective Communication
the parent interpret the information (eg, “What does this mean to
Barriers to effective communication can be divided into systems-
you?”). Research has also shown motivational interviewing to be an
related barriers and interpersonal barriers (Table 2.3). The pri-
effective tool for use with adolescent patients to increase self-efficacy
mary systems barriers are the setting itself and lack of continuity
to enact change (eg, adolescent smoking cessation).
of care. Because of access problems within the health care sys-
tem (ie, lack of health insurance coverage), many children receive
Physical Examination only episodic care from different physicians in acute care clinics or
Parents keenly observe physicians’ interactions with their chil- EDs. Without the benefit of long-term relationships, doctor-patient
dren during the examination. It is an important time for the communication may suffer.
physician to build a therapeutic relationship with the child (see Interpersonal barriers include physician time constraints,
Chapter 3). The transition between the history and physical frequent interruptions, and cultural insensitivity. Frequent
examination can be made by briefly telling the child and parent interruptions or apparent impatience on the part of the phy-
what to expect during the examination. The physician should sician conveys to parents and children that the physician does
show consideration for the child’s fears. In general, physicians not care or is too busy for them. Language differences may pose
often find it helpful to speak with families at periodic intervals
during the examination about their observations. Prolonged peri-
ods of silence as the physician listens or palpates may be anxiety Table 2.3. Barriers to Effective Communication
provoking for the family. Physicians should explain any proce- Barrier Category Specific Type of Barrier Example
dures that they or their staff are going to perform at a level that Systems Lack of continuity of care Episodic care that is
is appropriate for parents and children. In addition, the physi- primarily illness driven
cian should try to avoid exclamations or comments to self dur-
The setting itself Emergency departments
ing the examination (eg, “Wow, that’s some murmur!”), which
and acute care clinics
may be alarming to the family.
Interpersonal Physician time Appearing impatient or
Concluding Remarks constraints preoccupied
Frequent interruptions Pager goes off or asked to
The conclusion of the visit, which is all too easy to rush through, is
come to the telephone
extremely important. Closure can be provided by summarizing the
diagnosis or outlining plans for a follow-up visit. The parent or par- Cultural insensitivity Suggesting treatments that
ent should be asked to participate by acknowledging closure and are not acceptable within
helping to develop a management plan. Shared decision making the family’s belief systems

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 Chapter 2: Talking With Parents 11

a significant barrier, depending on the region in which the phy- Selected References
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use on the patient-doctor relationship and communication: a systematic review.
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diagnosis, but it is also correlated with improved patient recall of interaction analysis for pediatric practice. Am J Dis Child. 1971;121(2):
110–114 PMID: 5542848 https://doi.org/10.1001/archpedi.1971.02100130064006
instructions and adherence to prescribed courses of treatment. Poor
communication can have negative consequences for the patient (eg, Levetown M; American Academy of Pediatrics Committee on Bioethics.
Communicating with children and families: from everyday interactions to skill in
compromised care) and physician (eg, medicolegal consequences).
conveying distressing information. Pediatrics. 2008;121(5):e1441–e1460. Reaffirmed
Effective communication enhances medical outcomes and patient
December 2016 PMID: 18450887 https://doi.org/10.1542/peds.2008-0565
satisfaction.
Miller WR, Rollnick S. Motivational Interviewing: Helping People Change.
3rd ed. New York, NY: Guilford Press; 2013
Tates K, Meeuwesen L. Doctor-parent-child communication: a (re)view of
the literature. Soc Sci Med. 2001;52(6):839–851 PMID: 11234859 https://doi.
CASE RESOLUTION org/10.1016/S0277-9536(00)00193-3
The doctor-patient interaction presented in the case study illustrates sev- Teutsch C. Patient-doctor communication. Med Clin North Am. 2003;87(5):
eral of the “not to” points discussed herein. The physician did not acknowl- 1115–1145 PMID: 14621334 https://doi.org/10.1016/S0025-7125(03)00066-X
edge parental concerns or make sure that the mother had understood the US Department of Health and Human Services. HITECH Act Enforcement Interim
diagnosis and treatment plan. The mother was presented with more informa- Final Rule. www.hhs.gov/hipaa/for-professionals/special-topics/hitech-act-
tion than she could have reasonably been asked to remember. This interaction enforcement-interim-final-rule/index.html. Accessed March 4, 2019
could have been improved had the physician conveyed to the mother that her
concerns were appreciated and reassured her that her child was going to be all Young KT, Davis K, Schoen C, Parker S. Listening to parents: a national survey
right. Furthermore, the physician should have told the mother the name and of parents with young children. Arch Pediatr Adolesc Med. 1998;152(3):
dosage schedule of the antibiotic to be prescribed and limited the number of 255–262 PMID:9529463
“as needed” instructions. Zuger A. Talking to patients in the 21st century [editorial]. JAMA. 2013;309(22):
2384–2385 PMID: 23757087 https://doi.org/10.1001/jama.2013.7159

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 CHAPTER 3

Talking With Children

Geeta Grover, MD, FAAP

CASE STUDY
The moment you walk into the examination room, the Questions
2-year-old girl begins to cry and scream uncontrollably. 1. How does the age of children influence their under-
She clings to her mother and turns her face away. standing of health and illness?
The mother appears embarrassed and states that her 2. Should physicians speak directly with children about
daughter reacts to all physicians this way. After reas- their illnesses?
suring the mother that you have received such wel- 3. At what age can children begin to communicate
comes before, you sit down at a comfortable distance with physicians about their illnesses?
from the girl and her mother. You smile at the girl and 4. How can older children be involved in the manage-
compliment her on her dress, but she does not seem to ment of their health?
be interested in interacting with you at this point. You 5. How can positioning and placement of children in
place an age-appropriate book on the examination the examination room affect the overall tone and
table, indicating to the child that the book is for her. quality of the visit?
You begin your interview with the mother and try not
to look at the girl. Out of the corner of your eye, you see
that her crying is easing and she has begun to examine
the book you had placed on the table.

Effective communication is essential in developing a meaningful Piaget defined 4 stages of cognitive development, which occur in the
and trusting relationship with children. In pediatrics, interview- same sequence but not at the same rate in all children (Table 3.1).
ing involves balancing the needs of parents and children. Whereas In the sensorimotor stage (birth–2 years of age), children expe-
parents may be more focused on issues pertaining to disease, rience the world and act through sensations and motor acts. They
treatment, or aspects of parenting, children look to physicians with are developing the concepts of object permanence, causality, and
different needs and concerns, depending on their age. Developmental spatial relationships. In the preoperational stage, (2–6 years of age),
maturity, cognitive level, language ability, and sociocultural factors children understand the world only from their own viewpoint.
all play a role in a child’s ability to communicate and affect their As egocentric thinkers, they are unable to separate internal
concepts of health and illness. As children grow and develop, their from external reality, and fantasy play is important. School-age
understanding of health and illness matures and develops as well. children (6–11 years of age) are capable of concrete operational
Developmentally sensitive communication helps build a trusting thinking. These children can reason through problems that relate
relationship that allows pediatricians to guide children as they grow to to real objects. Older children (>11 years of age) have the capac-
make appropriate decisions about their own health as well as assume ity for abstract thought, which defines the formal operations stage.
responsibility for behaviors that may affect their health and well-
being. The American Academy of Pediatrics advises that physicians
Table 3.1. The Four Stages of Cognitive
have both a moral and an ethical obligation to discuss health and
Development According to Piaget
illness with children, and further, in keeping with their developmen-
tal capacities, allow them to be active participants in their own care. Agea Stage Characteristics
Birth–2 years Sensorimotor Experiences the world through sensa-
tions and motor acts
Developmental Approach to
Communicating With Children 2–6 years Preoperational Egocentric thinking
Imitation and fantasy play
Childhood is a time not only of considerable physical growth but
6–11 years Concrete operational Mental processes only as they relate
also of tremendous social, emotional, and cognitive maturation. An
to real objects
appreciation of the cognitive stages of development helps pediatri-
cians develop a healthy relationship with their patients by allowing >11 years Formal operations Capacity for abstract thought
them to communicate with children in an age-appropriate manner. a
Approximate ages.

13

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 14 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

An appreciation of how children’s cognitive development affects cues and the environment itself. A pleasant, child-friendly environ-
their understanding of illness and pain aids physicians in devel- ment with bright colors, age-appropriate wall decorations, and toys
oping therapeutic relationships with their patients. When using a helps make children feel more comfortable. Health professionals
developmental approach to children’s understanding of illness, chil- should be sincere, because children are extremely sensitive to non-
dren’s explanations of illness are classified into 6 categories that are verbal cues. The pediatrician should take a few minutes to enjoy time
consistent with Piaget’s cognitive developmental stages (Table 3.2). with the child; this not only gives the child a chance to evaluate the
Children 2 to 6 years of age view illness as being caused by external physician but also allows the clinician to begin assessing areas of
factors near the body (ie, phenomenalism, contagion). Young children development. A general principle of the pediatric examination is to
engage in so-called magical thinking; proximity alone provides the begin with the least invasive portions of the examination (eg, heart,
link between cause and illness. Children 7 to 10 years of age should be lungs, abdomen) and save the most invasive for last (eg, orophar-
able to differentiate between self and nonself. At this stage, they begin ynx, ears). Pediatricians should maintain their self-control in
to understand that although illness may be caused by some factor out- difficult situations. If they approach their limit, they should step
side the body, illness itself is located inside the body (ie, contamina- outside for a few minutes or ask someone for assistance. Guidelines
tion, internalization). Children 11 years of age and older understand for physician-child communication are provided in Box 3.1. Age-
physiologic and psychophysiologic explanations of illness. specific guidelines exist for children from birth to 6 months of age,
A similar developmental sequence applies to children’s under- 7 months to 3 years of age, 3 to 6 years of age, 7 to 11 years of age,
standing of pain. Younger children may attribute pain to punish- and 12 years and older.
ment for some transgression or wrongdoing on their part. They may
not understand the relationship between pain and illness (eg, “Pain Birth to 6 Months of Age
is something in my tummy.”). Children with concrete operational Newborns and infants through 6 months of age have not yet devel-
thought can appreciate that pain and illness are related, but they may oped a fear of strangers and can therefore usually be easily exam-
not have a clear understanding of the causation of pain (eg, “Pain is a ined in a parent’s arms or on the examination table. Although verbal
feeling you get when you are sick.”). Older children and adolescents interaction is limited, it is important to play with newborns and
begin to understand the complex physical and psychologic compo- infants, hold them, and talk to them. By watching physicians inter-
nents of pain. For example, they realize that although the bone in act with their infants, new parents have an opportunity to learn how
the arm is broken, pain is ultimately felt in the head (eg, “Pain goes to behave with their infants.
up some nerves from the broken bone in my arm to my head.”).
Seven Months to 3 Years of Age
Guidelines for Doctor-Child Infants and children 7 months through 3 years of age are perhaps
Communication the most challenging with whom to develop rapport and on whom to
A developmental framework that accounts for children’s language perform examinations. After entering the examination room, pedi-
skills and causal reasoning abilities is essential in providing appro- atricians should take a few moments to converse or play with these
priate health care to children. Successful communication with infants and children. Such actions help put children at ease and
children depends not only on spoken words but also on nonverbal allow them to get to know their doctor. Children 1 to 2 years of age

Table 3.2. Children’s Concepts of Illness

Stage of Cognitive
Development Age Concept of Illness Question Example of Child’s Answer
Preoperational 2–6 years Phenomenism How do people get colds? “From the sun.”
Contagion How do people get colds? “When someone else gets near them.”
Concrete Operational 7–10 years Contamination How do people get colds? “You’re outside without a hat and you start
sneezing. Your head would get cold—the cold
would touch it—and then it would go all over
your body.”
Internalization How do people get colds? “In winter, people breathe in too much air into
their nose and it blocks up the nose.”
Formal Operations ≥11 years Physiologic/psychophysiologic How do people get colds? “They come from viruses, I guess.”
How do people get a heart “It can come from being all nerve-racked. You
attack? worry too much, and the tension can affect your
heart.”
Adapted with permission from Bibace R, Walsh ME. Development of children’s concepts of illness. Pediatrics. 1980;66(6):912–917.

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 Chapter 3: Talking With Children 15

able to be partners in their care. The physician should make a point

Box 3.1. Physician-Child Communication
of speaking directly with children and not just with parents concern-
Do ing the chief symptom and history. Pediatric encounters are usu-
ww Provide a pleasant environment. ally dominated by parents, but the clinician can encourage children
ww Pay attention to nonverbal cues. to speak by addressing them by name or directing eye gaze toward
ww Be sincere and honest. them. Children can usually provide a good history of their illness,
ww Enjoy interacting with the child. although their concept of time may be misleading. For example, a
ww Speak to the child in an age-appropriate manner. “long, long time” may mean hours, days, or months, and parents
ww Get down to the child’s eye level. need to clarify this. Children should be asked about school, friends,
ww Examine from least to most invasive. and favorite activities. Answers to such questions give the physician
ww Respect the child’s privacy. an idea of children’s social and emotional well-being, which may
ww Maintain self-control. be affecting their physical health. Physicians sometimes overlook
ww Have a sense of humor. children’s need for privacy at this age. Drapes should be used
Don’t appropriately during the examination, and the physician should be
ww Limit the child’s participation. sensitive to the presence of other children or adults in the room.
ww Threaten the child. Physicians should begin to involve older children in the man-
ww Compare the child to others. agement of their illness. Children’s understanding of their illness
ww Engage in power struggles. and its management should be assessed (eg, children with asthma
could be asked, “What is this inhaler for? When are you going to
use it? Show me how.”). Children should be given an opportunity to
express their fears and anxieties, and these concerns should be dis-
will likely busy themselves exploring the room during the history cussed (eg, “Asthma can be scary, especially when you can’t catch
taking. By acknowledging them periodically, physicians build rap- your breath. Have you ever felt like that? Tell me about it.”). Children
port that will help later during the examination. Children 2 to 3 years should be involved in the management of their illness, which allows
of age are usually very apprehensive of the examination. The physi- them to develop a sense of responsibility for their own health and
cian should get down to children’s eye level when speaking to them. medical care. Active participation in their health care visits results in
If applicable and true, reassurances such as, “You’re not going to get greater visit satisfaction, knowledge, and competence of prescribed
any shots today,” can help alleviate their fears. Making false prom- medical therapies.
ises, however, can be detrimental to developing a trusting relation-
ship. Because stranger anxiety has developed, the physician should Age 12 Years and Older
try to do as much of the examination as possible with the child Communication with adolescents is discussed in detail in Chapter 4.
in the parent’s lap. Distractions such as stethoscope toys, flashing
penlights, or keys may be helpful.
Children and Health Literacy
Three to 6 Years of Age Health literacy (see Chapter 34) is a person’s ability to receive, under-
Children’s expressive capabilities are growing at tremendous stand, and use information to make appropriate health decisions. At
rates during this period. Children can usually be engaged in what age does an individual become responsible for his or her own
conversation. Although they should be given repeated opportu- health? Although in the medical setting children often have limited
nities for participation, they should not feel pressured to take involvement in medical decision making about their own health, in
part. Children may doubt the physician’s true intentions and will the community children are routinely making decisions that affect
likely speak only after their comfort level has been achieved. One their well-being. For example, a 4-year-old chooses water instead
way to involve children in the examination is to ask simple ques- of a soda at a friend’s birthday party; a 6-year-old runs home to put
tions about their illness (eg, “Where does it hurt?”). In addition, on her helmet before she rides her neighbor’s new bike; an 8-year-old
children should be given some control during the examination recognizes that her cough is the first sign of an impending asthmatic
(eg, “Should I look in your mouth next or your ears?”), and they attack and goes to the school nurse to use her inhaler; or a 12-year-
may be allowed to handle or inspect physicians’ equipment when old refuses the cigarette she is being offered. Health attitudes and
possible. Knowing what to expect next and having some con- behaviors learned during childhood affect adult lifestyle choices and
trol over the examination can help decrease children’s fear and thereby health. Health literacy skills should be encouraged from a
increase their cooperation. young age. If information is presented in an age-appropriate manner,
children between ages 3 and 18 years are capable of understanding
Seven to 11 Years of Age and using health information. In today’s world of patient-centered
Making a conscious effort to involve children in this age group in all care and ever-increasing accessibility to health information through
phases of their care, from gathering data to developing a care plan, new technologies, it is imperative that physicians encourage patients
can be rewarding because children in this age group are increasingly to be knowledgeable consumers beginning at a young age.

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 16 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Barriers to Effective Communication Selected References

With Children Bibace R, Walsh ME. Development of children’s concepts of illness. Pediatrics.
The manner in which adults speak to children is influenced by what 1980;66(6):912–917 PMID: 7454481
they think children can understand. Physicians tend to overestimate Borzekowski DL. Considering children and health literacy: a theoretical
the understanding capabilities of younger children and underesti- approach. Pediatrics. 2009;124(Suppl 3):S282–S288 PMID: 19861482 https://
mate those of older children. Lack of appreciation for the cognitive doi.org/10.1542/peds.2009-1162D
sophistication of children may result in frustration for all involved. Cahill P, Papageorgiou A. Triadic communication in the primary care paediatric
Younger children are presented with information they may not be consultation: a review of the literature. Br J Gen Pract. 2007;57(544):904–911
able to comprehend, and older children may feel frustrated because PMID: 17976292 https://doi.org/10.3399/096016407782317892
they are being spoken to as though they are much younger. Cahill P, Papageorgiou A. Video analysis of communication in paediatric consul-
Overestimating children’s receptivity to medical information tations in primary care. Br J Gen Pract. 2007;57(544):866–871 PMID: 17976287
https://doi.org/10.3399/096016407782317838
can be particularly problematic in the case of children with chronic
illness. It is easy to assume that children with chronic medical Damm L, Leiss U, Habeler U, Ehrich J. Improving care through better com-
munication: understanding the benefits. J Pediatr. 2015;166(5):1327–1328
conditions or those with more hospitalizations would have more
PMID: 25919744 https://doi.org/10.1016/j.jpeds.2015.01.027
sophisticated understanding of illness causation. Although these
Erickson SJ, Gerstle M, Feldstein SW. Brief interventions and motivational
children may seem more savvy and knowledgeable about medical
interviewing with children, adolescents, and their parents in pediatric health
procedures, little research exists to support the assumption that they care settings: a review. Arch Pediatr Adolesc Med. 2005;159(12):1173–1180
have more sophisticated understanding of illness causation than PMID: 16330743 https://doi.org/10.1001/archpedi.159.12.1173
would be expected based on their age or developmental level, or Ginsburg HP, Opper S. Piaget’s Theory of Intellectual Development. 3rd ed.
that they are better able than their peers without chronic medical Englewood Cliffs, NJ: Prentice-Hall; 1988
concerns to understand and retain medical information. Levetown M; American Academy of Pediatrics Committee on Bioethics.
Another potential barrier in communicating with children is lim- Communicating with children and families: from everyday interactions to skill in
iting their participation. Physicians tend to elicit information from conveying distressing information. Pediatrics. 2008;121(5):e1441–e1460. Reaffirmed
children but exclude them from diagnostic and management informa- December 2016 PMID: 18450887 https://doi.org/10.1542/peds.2008-0565
tion. Especially when dealing with chronic or serious illness, children Myant KA, Williams JM. Children’s concepts of health and illness: understanding
should be asked about their desired level of involvement. Children of contagious illnesses, non-contagious illnesses and injuries. J Health Psychol.
want to know about their illness and will use whatever information is 2005;10(6):805–819 PMID: 16176958
available to them to make sense of their situation. Developmentally Nobile C, Drotar D. Research on the quality of parent-provider communication in
sensitive communication allows children access to usable information, pediatric care: implications and recommendations. J Dev Behav Pediatr. 2003;24(4):
improves their understanding of their situation, decreases fear, and 279–290 PMID: 12915801 https://doi.org/10.1097/00004703-200308000-00010
contributes positively to their ability to participate in management. Palazzi DL, Lorin MI, Turner TL, Ward MA, Cabrera AG. Communicating
Providing pediatric care can be both rewarding and enjoyable, but with Pediatric Patients and their Families: The Texas Children’s Hospital
Guide for Physicians, Nurses and other Healthcare Professionals. Houston, TX:
many physicians have been frustrated or pushed to the limit at some
Texas Children’s Hospital; 2015. https://www.bcm.edu/departments/pediatrics/
point when working with children. On the part of the child, crying or
patient-communication-guide. Accessed September 19, 2019
lack of cooperation generally stems from fear or a sense of lack of con-
Riley AW. Evidence that school-age children can self-report on their health.
trol over the situation. On the part of the physician, engaging in power
Ambul Pediatr. 2004;4(Suppl 4):371–376 PMID: 15264962 https://doi.
struggles or making threatening remarks (eg, “If you’re not good, I’ll org/10.1367/A03-178R.1
have to give you a shot.”) or comparisons between siblings (eg, “Your
Vatne TM, Slaugther L, Ruland CM. How children with cancer communicate and
brother is younger than you and he didn’t cry.”) are not only ineffective think about symptoms. J Pediatr Oncol Nurs. 2010;27(1):24–32 PMID: 19833978
but may make the situation worse. A clear perspective, mindful con- https://doi.org/10.1177/1043454209349358
sideration of developmental level, empathy, and a sense of humor are
much more useful.

CASE RESOLUTION
You learn from the mother that her daughter has been in good health. The
mother has brought in the child for a routine health maintenance visit. You assess
that the child’s development is normal and her immunizations are up-to-date.
As you and the mother talk, the child appears more relaxed and less frightened.
You use the book to engage and distract the child during the examination. She
begins to respond to your questions and cooperate with the examination, but she
chooses to remain on her mother’s lap. Praising a child who is cooperative helps
reinforce preferred behavior.

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 CHAPTER 4

Talking With Adolescents

Monica Sifuentes, MD

CASE STUDY
This is a first-time visit for a 15-year-old girl who is Questions
accompanied by her mother. The mother is concerned 1. When interviewing adolescents, what is the signifi-
because her daughter’s grades have been dropping cance of identifying their stage of development?
since beginning high school, and she appears fatigued 2. What are important areas to cover in the adolescent
and irritable. The mother reports no new activities interview?
or recent changes in the home situation and no new 3. What issues of confidentiality and competence need
stressors in the family. Both parents are employed, the to be discussed with adolescents before conducting
girl has most of the same friends she has always had, the interview?
and her siblings currently are doing well academically. 4. When should information be disclosed to others,
The girl is healthy and has never been hospitalized. despite issues of confidentiality?
After the mother leaves the room, the girl is inter-
viewed alone.

Adolescence is a time of unique change from a cognitive, physical,

Box 4.1. Keys to Successful Interviews
and neurobiological standpoint. Unlike other periods in life in
With Adolescents
which individuals have at least some knowledge or experience
to guide them, adolescence can be characterized by feelings of ww Listen attentively, with minimal interruptions.
physical awkwardness, emotional turmoil, and social isolation. In ww Respect privacy.
addition, the teenage years are dreaded by most parents, who often ww Explain confidentiality.
feel ill-equipped to handle the unpredictability of their children’s ww Use open-ended, nonjudgmental questions; start with general observa-
responses to puberty and daily social interactions. Rather than tions of concern and follow up with specific questions.
directly approach their adolescent, some parents choose to engage ww Define medical terms clearly.
in quiet observation. They fully intend to support their children ww Invite the adolescent to ask questions.
but wait to be approached. Thus, many adolescents do not have the ww Help empower the teenager to address health issues.
active guidance or timely advice of parents during the teenage years ww Reinforce the teenager’s own positive support systems.
and prefer to spend their time alone or in the company of friends
or acquaintances. Fortunately, most adolescents pass through this Adapted with permission from Sacks D, Westwood M. An approach to interviewing adolescents.
Paediatr Child Health. 2003;8(9):554–556.
period uneventfully. In fact, many individuals go through this period
gladly and appreciate finally being permitted to drive a car, gain
employment, or start dating. Stages of Adolescence
The physician should approach interviews with adolescents Adolescents are stereotypically labeled as difficult, complex, and
differently from interviews with younger children, because with time-consuming patients with complicated concerns that result in
adolescents information comes directly from the teenager rather nonmedical diagnoses. In addition, they can be accompanied by
than a parent (Box 4.1). Unlike an interview with a younger pediatric overbearing, demanding parents, or sometimes no parent.
patient, the adolescent interview should focus on several psychoso- The quality and quantity of information obtained from the
cial issues that may be uncomfortable to discuss in the presence of adolescent during the medical and psychosocial interview can be
a parent. Thus, each teenager should be interviewed alone. The goal greatly enhanced by taking developmental milestones into consid-
of the interview is to help adolescents become more comfortable eration. Adolescence can be divided into 3 developmental stages:
discussing issues related to physical and mental health with the early, middle, and late (Table 4.1). For example, interest in discuss-
physician, give adolescents the opportunity to become more respon- ing long-term educational goals varies depending on the age of the
sible for their health care, and discover any psychosocial issues that adolescent. Most 18-year-olds are prepared to discuss college plans,
might interfere with a relatively smooth passage through adolescence. specific vocational interests, and employment opportunities. In

17

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 18 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Table 4.1. Developmental Milestones During This contributes to an atmosphere of trust and honesty. The disad-
Adolescence vantage is possible inhibition by adolescents who are unsure about
disclosing particular incidents (eg, those concerning sexual abuse)
Early Adolescence Middle Adolescence Late Adolescence
for fear of involving other professionals or family members.
(11–13 years) (14–16 years) (17–21 years)
Interviewers should be nonjudgmental, reassuring, and empathetic
Concrete, egocentric ± Abstract thought Abstract thought to reduce the possibility of such an occurrence.
thought processes processes emerge processes well formed The second, less popular approach to the discussion of confi-
Parental supervision ± Parental supervision Limited or no parental dentiality involves informing adolescents at the end of the inter-
prominent supervision view or when and if an exception to maintaining confidentiality
± Risk-taking behav- ± Risk-taking behavior Risk-taking behavior arises. Proponents of this approach argue that adolescents tend
ior with feelings of diminishes; vocational to respond more honestly to questions when they do not believe
invulnerability objectives formalized physicians will inform others, including their parents or legal
± Peer pressure Peer pressure prominent Impact of peer pressure guardians. As mandated reporters, however, physicians have a legal
decreasing responsibility to report sexual and physical abuse; in cases of sui-
Reprinted with permission from March CA, Jay MS. Adolescents in the emergency department: an
cidal or homicidal behavior, it is in the patient’s best interest to
overview. Adolescent Medicine. 1993;4(1):1–10. inform other professionals of this disclosure. The disadvantage to
this method is that these issues often arise at very emotional times
during the interview, and it is difficult to interrupt the patient to
contrast, 12-year-olds are still anchored in the concreteness of early
discuss mandated reporting. If physicians wait until the end of the
adolescence and often are ill-prepared to discuss detailed plans for
interview to inform adolescents about mandated reporting, how-
higher education. Current middle school experiences are much more
ever, patients may leave the office feeling deceived and may not
important to this age group and therefore should be the focus of dis-
return for future visits. For this reason, most health professionals
cussion. Peer pressure is most prominent during middle adolescence;
prefer to inform adolescents at the onset of the interview about con-
thus, 16-year-olds with friends who smoke cigarettes and drink
fidentiality with the hope that it contributes to the development of
alcohol likely have tried or use the same illicit substances.
a trusting relationship.
Knowledge of these developmental differences allows the inter-
An assessment of the adolescent’s ability to make health-related
viewer to more effectively explain instructions and diagnoses to teen-
decisions is another important aspect of the interview. Competence
agers. For example, compared with 14-year-olds, 19-year-olds can
is the ability both to understand the significance of information and
better understand the effects of untreated or recurrent chlamydial
to assess alternatives and consequences to sufficiently identify a
cervicitis on long-term fertility. This is not to say that physicians
preference. Various factors other than age must be considered,
should not discuss these possible consequences with a sexually active
such as maturity level, intelligence, degree of independence, and
14-year-old with chlamydia; rather, they should use more concrete
presence of any chronic illness. This last factor is included because
descriptive wording and repeat the information at future visits. Age
adolescents with chronic conditions may have already participated
guidelines are not rigid, however, and each interview should be
in decisions about their health care. Regardless, it can be difficult
individualized to the particular adolescent and the circumstances
to assess competence from just 1 visit. It may not even be necessary
surrounding the visit.
to make an assessment emergently, except in certain cases, such as
with an unplanned pregnancy.
Issues of Confidentiality and Although it is imperative to interview adolescents alone, every
Competence attempt should be made to involve parents or guardians in physi-
A discussion about confidentiality is essential and can be approached cal and mental health decisions. Although specific state laws allow
in 1 of 2 ways. Each method has distinct advantages and disad- physicians to treat minors in emergent situations and in cases
vantages. To allow conversation to flow more naturally, interview- of suspected sexually transmitted infections without the con-
ers should use the approach with which they themselves are most sent of a parent or guardian, physicians should urge adolescents
comfortable. to inform their parents or guardians of any ongoing problems
The first approach involves informing adolescents at the begin- disclosed during the interview. The ultimate decision, however,
ning of the interview that most issues discussed are held in strict rests with the adolescent. Physicians can assist adolescents in
confidence and will not be repeated to anyone. Exceptions are discussing delicate issues with their parents by role-playing
suicidal or homicidal behavior and a history of or ongoing sexual or with teenagers or by sitting in on the conversation between the
physical abuse. In any of these instances, other professionals are told adolescents and their parents when disclosing sensitive infor-
of the disclosed information, and parents or guardians ultimately mation. Health professionals should become familiar with the
are informed of the disclosure. The advantages of this approach specific consent laws related to minors in the state in which
are that discussion of such logistics at the beginning of the inter- they practice medicine to confirm the legal abilities of minors to
view is less awkward, and the ground rules are clear from the start. consent to sensitive health care services.

BPPCA6e_Ch004_0017-0020.indd 18 2/13/20 5:05 PM

 Chapter 4: Talking With Adolescents 19

Psychosocial Review of Systems connectedness with peers and family, and sleep hygiene practices.
In addition to reviewing the amount of time spent on an electronic
A major part of the adolescent interview involves obtaining a thor-
device each day, physicians also should inquire about texting, sexting,
ough psychosocial history, which typically can be completed in 20 to
and whether the patient is a victim or perpetrator of cyberbullying.
30 minutes. The approach, which is known by the acronym HEADSS
(home, employment and education, activities, drugs, sexuality, sui-
cide/depression), allows interviewers to evaluate the critical areas in Issues That Need Immediate Attention
adolescents’ lives that may contribute to a less than optimal environ- Many issues discussed during the psychosocial interview can be a
ment for normal growth and development (Box 4.2). Questions about source of significant stress and anxiety for adolescents. Evidence of
sexuality, sexual orientation, and gender identity must be asked in psychological or adaptive difficulties must be taken seriously and
a nondirected, open-ended, nonjudgmental fashion, giving adoles- should be reassessed at future visits. Certain disclosures, however,
cents time to respond. This information is imperative to adequately demand immediate attention. Suicidal ideation, with or without a
assess risks for conditions such as social isolation, unintended previous attempt, requires a more in-depth analysis of the gravity
pregnancy, and sexually transmitted infections, including HIV. In of the problem. Mental health professionals should be involved
addition, an inquiry about sexual, physical, and emotional/verbal emergently in the clinical assessment of these precarious situations.
abuse is indicated during this part of the interview. Other issues that require immediate attention include possible
Because most adolescents now have access to the internet 24/7 danger to others and a history of or ongoing sexual or physical
via their cell phone, home computer, or other electronic device, abuse. Depending on the specific circumstance, issues such as a pos-
it is important to discuss screen time with them and their par- sible or confirmed unplanned pregnancy, bullying, substance use,
ents to obtain a more accurate picture of their online activities, and sexual orientation may not necessarily require the emergent

Box 4.2. What to Ask

HEADSS ww Is the adolescent currently using, or has he or she ever used, tobacco,
H: Home electronic cigarettes, or vaporizer?
ww With whom does the adolescent live? ww Is the adolescent currently using, or has he or she ever used, any illicit
ww Have there been any recent changes in the living situation? drugs? What about steroids? Alcohol?
ww How are things between parents/other adults living in the home? ww Does the adolescent ever feel pressured by friends to use drugs or alcohol?
ww Are the parents or guardians employed? S: Sexuality
ww How does the adolescent get along with the parents and siblings? ww What is the adolescent’s sexual orientation and/or gender identity or
ww Does the adolescent feel safe at home? In the neighborhood? expression?
ww Is there a firearm in the adolescent’s home? If so, what does the adolescent ww Is the adolescent currently in a relationship?
know about firearm safety? ww Is the adolescent sexually active?
E: Employment and education ww If so, what was the age of the adolescent’s first sexual experience?
ww Is the adolescent currently in school? ww What types of sexual experiences has he or she had?
ww What does the adolescent enjoy about school? Dislike? ww How many sexual partners has the adolescent had in his or her
ww How is the adolescent performing academically? lifetime?
ww Has the adolescent ever been truant or expelled from school? ww Does the adolescent have a history of sexually transmitted infections?
ww Are the adolescent’s friends attending school? ww Does the adolescent (or the partner) use condoms or another method of
ww Is the adolescent currently employed? How many hours does the protection?
adolescent work each week? ww Does the adolescent (or the partner) use any methods of contraception?
ww What are the adolescent’s future education/employment/vocational goals? ww Does the adolescent have a history of sexual or physical abuse?
A: Activities S: Suicide/depression
ww What does the adolescent do in his or her spare time? ww Is the adolescent bored all the time? Ever sad or tearful? Tired and
ww How much time is spent on technology (the computer, cell phone, unmotivated?
other electronic devices) during the day and at night? ww Has the adolescent ever felt that life is not worth living or ever thought
ww What does the adolescent do for fun? Is the adolescent ever bored? of or tried to hurt their self? More importantly, does the adolescent have
ww With whom does the adolescent spend most of his or her time? a suicide plan or access to a firearm?
D: Drugs
ww Do any of the adolescent’s friends smoke tobacco, use electronic cigarettes,
vape, or use illicit drugs or alcohol?

Adapted with permission from Goldenring JM, Rosen DS. Getting into adolescent heads: an essential update. Contemporary Pediatrics. 2004;21:64. Copyright © Advanstar Communications.

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 20 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

involvement of other providers initially; however, close follow up Selected References

must be assured and arranged by the physician or other health pro-
fessional regardless of consent by a parent or guardian. Breuner CC, Moreno MA. Approaches to the difficult patient/parent encounter.
Pediatrics. 2011;127(1):163–169 PMID:21173004 https://doi.org/10.1542/
peds.2010-0072
Concluding the Interview
Cavanaugh RM. Managing the transitions of early adolescents. Adolesc Health
The adolescent interview should be conducted at a time when the Update. 2008;20:1–10
adolescent is relatively healthy and the interviewer has set aside ample
Clark DL, Raphael JL, McGuire AL. HEADS4: social media screening in
time for a thorough, uninterrupted discussion. However, all topics adolescent primary care. Pediatrics. 2018;141(6):e20173655 PMID:29716979
need not be addressed during the initial interview if time does not https://doi.org/10.1542/peds.2017-3655
permit and the adolescent appears relatively stable based on initial English A, Bass L, Boyle AD, Eshragh F. State Minor Consent Laws: A Summary.
screening. Issues that are not covered in depth at this visit can be 3rd ed. Chapel Hill, NC: Center for Adolescent Health & the Law; 2010
addressed at the next one. The number of appointments needed to Ford C, English A, Sigman G. Confidential health care for adolescents: position
discuss or work through a particular problem or concern is unlimited. paper for the Society for Adolescent Medicine. J Adolesc Health. 2004;35(2):
At the end of each visit, the interview should be summarized, any 160–167 PMID:15298005 https://doi.org/10.1016/S1054-139X(04)00086-2
difficult topics identified, and the issue of confidentiality reviewed Ginsburg KR. Viewing our adolescent patients through a positive lens.
once again. Adolescents should then be asked to identify a person Contemporary Pediatrics. 2007;24:65–76
whom they can trust or confide in should any problems arise before Goldenring JM, Rosen DS. Getting into adolescent heads: an essential update.
the next visit. In some instances, this issue may already have been Contemporary Pediatrics. 2004;21:64–90
discussed during the interview. Adolescents also should be given the Guttmacher Institute. State policies in brief. An overview of consent to reproduc-
opportunity to express any other concerns that were not addressed tive health services by young people. www.guttmacher.org/state-policy/explore/
in the interview and ask additional questions. overview-minors-consent-law. Accessed January 26, 2019
The physician should clearly point out to the adolescent any Hazen E, Schlozman S, Beresin E. Adolescent psychological development:
significant risk factors or risk-taking behaviors that have been a review. Pediatr Rev. 2008;29(5):161–168 PMID:18450837 https://doi.
identified during the interview and assess the teenager’s readiness org/10.1542/pir.29-5-161
to change this behavior. For those adolescents who are not engag- Klein DA, Goldenring JM, Adelman WP. Probing the scars: how to ask the essen-
ing in high-risk behaviors, the physician should acknowledge that tial questions. Contemporary Pediatrics. 2014;31:16–28
things seem to be going well, praise them with sincere positive Knight JR, Sherritt L, Shrier LA, Harris SK, Chang G. Validity of the CRAFFT
feedback, and review their individual strengths and accomplish- substance abuse screening test among adolescent clinic patients. Arch Pediatr
ments. Available resources, such as teen hotlines and popular web- Adolesc Med. 2002;156(6):607–614 PMID:12038895 https://doi.org/10.1001/
archpedi.156.6.607
sites, should be given to the adolescent prior to inviting the parent
or guardian back into the examination room. General concerns Kokotailo PK, Gold MA. Motivational interviewing with adolescents. Adolescent
Medicine. 2008;19:54–68
should then be reviewed with the parent or guardian while main-
taining the adolescent’s confidentiality. The parent or guardian Kreipe RE. Introduction to interviewing: the art of communicating with adoles-
cents. Adolescent Medicine. 2008;19:1–17
also should be given appropriate written or electronic resources
to review, particularly if a sensitive subject has been discussed, Ott MA, Labbett RL, Gold MA. Counseling adolescents about abstinence in
the office setting. J Pediatr Adolesc Gynecol. 2007;20(1):39–44 PMID:17289516
and invited to call the physician if any additional questions or con-
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cerns arise. The follow-up appointment should be arranged with
Sanders RA. Adolescent psychosocial, social, and cognitive development. Pediatr
the adolescent before the conclusion of the visit.
Rev. 2013;34(8):354–359 PMID:23908362 https://doi.org/10.1542/pir.34-8-354

CASE RESOLUTION
The adolescent should be informed about confidentiality and the specific
exceptions to maintaining it. Nonthreatening topics, such as home life, school,
employment, and other outside activities should be explored first, followed
by questions about sexuality, gender, sexual orientation, sexual activity, and
illicit drug use. Suicidal behavior or depression and safety issues should also be
reviewed with the teenager alone and again with the parent if there is a need for
a more formal or immediate mental health evaluation. In addition, computer and
cell phone use as well as sleep hygiene should be evaluated. Identified high-risk
behaviors and their consequences should be discussed with the adolescent at the
end of the interview, and a plan for future visits should be arranged. The mother
should then be invited back into the examination room prior to the conclusion of
the visit to discuss nonsensitive issues unless permission has been obtained from
the teenager to disclose and discuss confidential topics.

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 CHAPTER 5

Telephone Management
and E-medicine
Emily Borman-Shoap, MD, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP

CASE STUDY
The mother of an otherwise healthy 10-month-old Questions
girl calls and tells you that her daughter has a fever. 1. How do telephone and face-to-face encounters
The girl’s rectal temperature has been 39.4°C to 40.0°C between physicians and patients differ?
(103°F to 104°F) for the past 2 days. Although she is 2. What are some general guidelines for effective
fussy with the fever, she plays normally after receiv- doctor-patient communication via telephone?
ing acetaminophen. The girl is eating well and has no 3. What historical information is necessary for appro-
runny nose, cough, vomiting, diarrhea, or rash. priate telephone management?
Mother also mentions 2 other concerns that she 4. What points are important to cover in home treat-
has been meaning to bring up with you. The first ment advice?
involves questions about feeding and how to introduce 5. For nonurgent issues, what are the possible roles of
table foods; the other is sleep problems. Her daughter telephone encounters or e-medicine in patient care?
has been waking up several times a night for the past
month, and mother feels exhausted.

Parents and guardians are increasingly accessing their children’s repeating back to the physician the plan of care, is also an impor-
health professional through avenues other than a typical office visit. tant component of the telephone consultation.
Telephone management and electronic communications make up a
substantial portion of a primary care physician’s time. It is estimated Telephone Communication Skills:
that pediatricians spend more than 25% of their total practice time Establishing Rapport
engaged in telephone medicine. Telephone management includes
Parents and guardians commonly call their pediatric health profes-
triage of acute illness symptoms as well as ongoing preventive care
sional because they are worried about their child. The friendly voice
and management of chronic conditions. Electronic communication
of a staff member in the health professional’s office has a substantial
also plays an increasing role in acute, chronic, and preventive care.
role in reassuring an anxious parent. Each call should begin with a
“verbal handshake.” Staff should identify themselves and the place
Telephone Management in which the call is received and offer to help. They should learn
for Acute Illness the caller’s name, the caller’s relationship to the child, and the
The main components of a telephone management encounter child’s name. Using the child’s name in conversation helps establish
for acute illness mirror those of an office visit: establish rapport, rapport and creates a more personal atmosphere.
gather a complete history, formulate an assessment and plan, ensure Telephone calls for medical advice are often received in
adequate follow-up, and document the encounter. The key difference busy environments, such as emergency departments (EDs) or
is that often a telephone call offers no opportunity for a physical clinics, in which other patients are waiting to be seen. It is easy to be
examination or direct observation of the child; however, video abrupt under these circumstances and not give complete attention
conferencing and the ability to send photographs electronically is to a caller. If a call is not an emergency, staff members can take the
beginning to minimize this difference. Obtaining a thorough history caller’s telephone number and return the call as soon as possible.
remains the critical component of telephone management, however. The health professional who is returning a call to a patient should
Closed-loop communication, with the parent/guardian or patient ensure it is a good time for the patient to receive a return call. Unsafe

21

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 22 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

practices, such as talking while driving, should be avoided by both That is exactly what I would have done.” “I’m glad that you called
the health professional and the patient. about this.” The health professional may even be able to offer reassur-
Studies show that the length of a patient visit does not correlate ance to a parent or guardian who is not managing the child’s illness
with patient satisfaction. Telephone encounters need not be lengthy; correctly by commenting that many parents and guardians try
the average length of a call is reported to be 3 to 5 minutes, depend- the same treatment. After providing that reassurance, a different
ing on the setting. Each call must be pleasant, however, and address treatment approach can be suggested.
the caller’s concerns. Open-ended statements and questions, such Before the end of the conversation, the caller should be asked to
as, “Tell me about your child’s illness,” or “Are there any other symp- “teach back” or summarize what the health professional has recom-
toms?” are useful at the beginning of a call because they give the mended and encouraged to call again if additional problems occur.
caller an opportunity to explain the situation without interruption. For example, the physician might say, “I want to be sure I explained
Establishing rapport is more difficult on the telephone than in myself clearly. Can you tell me what you are planning to do now for
person because on the telephone the health professional is limited your child?” Giving clear guidance about reasons to seek emergency
to verbal communication. In face-to-face encounters, the health care is particularly important.
professional can use words as well as means of nonverbal communi-
cation, such as facial expressions, eye contact, gesturing, and touch, Telephone History Taking
to convey warmth and empathy. The health professional should use Most calls for triage of an acute problem are about upper respiratory
various aspects of verbal communication to convey sincere inter- symptoms, fever, rash, trauma, or gastrointestinal symptoms, that is,
est in a caller’s concerns and should pay attention to these verbal the same problems most commonly encountered in the office. With
cues from the caller. Many components of verbal communica- standardized history taking and home care advice, many patients
tion, including vocal expression, pace, articulation, tone, volume, with these chief concerns can be safely managed at home. Several
and pauses, affect telephone interactions. The health professional excellent published telephone management protocols can aid the
should speak clearly and use vocabulary that the caller understands. health care team in advising patients efficiently and appropriately.
Medical jargon should be avoided. A friendly yet respectful tone and Many practices use nurses as the first point of contact for telephone
a calm, professional manner should be maintained. triage, with the pediatrician serving as second-tier triage for more
Careful listening is crucial to obtaining the information neces- complex or worrisome concerns.
sary to make medical decisions over the telephone. One of the major For the history obtained via telephone, it is necessary to gather
goals of the health professional is to recognize and respond to the sufficient information to make an appropriate decision. Questions
caller’s main concerns and expectations. Researchers have found should be asked with the aim of determining whether an emergency
the following questions useful in the identification of parents’ chief exists and making a diagnosis. The health professional should follow
concerns: “What worries you the most about [use child’s name]
the same organized approach that would be used in the office setting
illness?” and “Why does that worry you?”
(Box 5.1). Key features, such as patient age and past medical history,
In interacting with a caller who rambles, it may be necessary
should guide questioning. For example, if the mother of a 20-day-
for the health professional to focus the conversation. Asking the
old girl reports that the neonate has a temperature of 38.9°C (102°F),
question, “What can I do to help?” should clarify the reason for the
it is necessary to see the newborn in person immediately. An older
call. If it is necessary to verify information, the professional can
child with the same chief report of fever may be safely managed at
summarize what has been heard and ask if they have understood
home, however, depending on the answers to other questions about
correctly. Using a triage protocol book or similar resource can
additional symptoms. Similarly, knowledge that a child who has
help the health professional streamline questions and aid in correct
been exposed to chickenpox has a compromised immune system is
disposition of the patient.
crucial in providing appropriate telephone advice.
The angry caller may elicit defensive or confrontational behavior
Additional specific questions should be asked to clarify the child’s
from the health professional. Responding to anger with arguments
condition and obtain all the information necessary to make a good deci-
is time-consuming, stressful, and pointless. The health professional
sion. Many physicians can access electronic medical records (EMRs)
should be warm and understanding to create an environment
in which a caller who wishes to discuss his or her feelings is
comfortable. Acknowledging anger may encourage open discus- Box 5.1. What to Ask
sion and problem-solving (eg, “You sound upset. I am ready to help ww How old is the child?
you. What can I do?”). Empathizing with the caller (eg, “I don’t ww What is the child’s chief problem? What are the child’s symptoms?
blame you for being upset”; “That must have been very frustrating.”) ww How long has the child had these symptoms?
and apologizing if the caller has experienced delays or barriers in ww How is the child acting?
accessing care is also helpful. ww Does the child have any chronic illnesses?
The health professional can build confidence in the caller by val- ww Is the child taking any medications?
idating the steps that individual has already taken, such as, “You did ww What are you most worried about?
the right thing by giving your child acetaminophen for the fever.

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 Chapter 5: Telephone Management and E-medicine 23

remotely to review the child’s record, which can greatly aid in obtain- treatment should be clear and as easy as possible to implement. If
ing a thorough history. If review of the medical record is possible, the instructions are complicated or lengthy, the health professional
it is reassuring for the health professional to share that information may ask the caller to write them down. When prescribing medi-
with the child’s caregiver by stating, for example, “I am reviewing cation, the physician should ask if the child has any known drug
your child’s medical record in the computer so that I can be sure I allergies; for the prescribed medication the physician should give
have all the information I need to give you good advice.” the dose, frequency of administration, and information about
The physician should focus on the critical features that will affect possible side effects. The health professional should verify that
disposition of the patient (ie, ED vs office visit vs home care). For the caller can follow the telephone advice (eg, a parent has a ther-
example, for the child who is vomiting or has diarrhea, the state of mometer and knows how to use it).
hydration is critical; for the child with a cough, the occurrence of
breathing difficulty is critical; and for the child with head trauma, Closing the Encounter: Ensuring
loss of consciousness is critical. Methods of teaching telephone man- Appropriate Follow-up
agement skills include role-playing, listening to mock parent calls, The health professional should confirm that the caller understands
and reviewing tapes of actual calls. the information and instructions and agrees with the plan. Asking
questions such as, “What questions do you have?” encourages callers
Telephone Advice: Communicating to raise uncertainties and ask for needed clarification. Most impor-
the Assessment and Management Plan tant, if the decision is to manage at home, the caller should always
receive specific instructions about when to call back. The caregiver
In an emergency situation, that fact should be explained to the caller should call if the child’s symptoms change, persist, worsen, or cause
and appropriate follow-up plans made, such as advising the parent or anxiety to the parent or guardian. Additionally, symptoms specific
guardian to call 911 for life-threatening conditions such as respira- to the child’s condition should be followed and the caregiver advised
tory depression or uncontrollable bleeding. If the condition is poten- on when to call back or come in (eg, fever of >2 days’ duration,
tially serious but non–life-threatening (eg, right lower quadrant irritability, decreased urination). If the physician plans to check up
pain or possible fracture), the caller should be advised to bring the on the child by telephone, the physician should confirm and record
child by car to the ED or physician’s office within a specified amount the appropriate callback number or numbers. The caregiver who
of time. For other types of calls, the health professional must seems unduly anxious or uncomfortable with home treatment
decide if and when the child should be seen by a physician and the should be given the opportunity to have the child seen in person by
appropriate course of home treatment. a health professional.
Because most childhood illnesses are mild and self-limited, eval-
uation of the safety of medical advice obtained by telephone requires Documentation
large samples to detect poor outcomes associated with misman-
agement. Research has described a “wellness bias” in which health All calls for medical advice should be documented in the child’s
professionals, who primarily see patients with mild, self-limited medical record for medical reasons (eg, better follow-up, improved
illnesses, may downplay the severity of reported symptoms and continuity of care) and legal purposes. The form used for documen-
choose the most benign diagnostic possibility. This bias may be tation should include the date and time of the call; the name, identity,
more pronounced in a telephone encounter, in which the physician and telephone number of the caller; the name and age of the child;
cannot see the child. One study reported telephone encounters in the chief symptom; other symptoms; possible diagnoses; advice
which physicians seemed to make a decision early in the conversa- given; and the name of the person who took the call.
tion and then “shut out” additional information that should have led
to the consideration of more serious diagnoses. The safest approach
Privacy and Technology
is to always have a high index of suspicion for a serious condition Considerations
and to ask questions to confirm or dispel those suspicions. Research Many calls take place over cell phones. The caller may be able to
shows that parents expect to receive an explanation of their child’s see the telephone number from which the physician is calling. The
illness. The health professional should clearly state what the child’s caller should be given clear instructions as to the appropriate ave-
illness seems to be, the likely cause, and what the parent or guardian nue for calling the physician, for example, “Please call back to the
can anticipate (eg, length of time that the child is likely to be sick, nurse triage line if you need to talk to me again.” Clinic staff may
additional symptoms that may appear). wish to give their patients access to a “direct line” to call back to
Before giving any treatment advice, the health professional the clinic so that patients do not have to go through an automated
should ask the caller the following questions: “What have you done telephone triage with each return call. Some health profession-
so far?”; “Have you given the child any medications?”; and “How is als may choose to block their numbers so they are not visible by
this treatment working?”. If the therapy seems appropriate, the caller caller identification. Others may feel comfortable having a par-
should be encouraged to continue the treatment. Alternatively, the ent call directly to their cell phone but should give clear guide-
regimen should be modified as indicated. Instructions for home lines about doing so.

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 24 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Many cell phones have the capacity for taking and sending The physician should remember, however, that written communica-
photographs. A photograph can be a valuable additional piece of tion is interpreted through the lens of the reader. Delicate or complex
information when evaluating symptoms, such as rash. However, it conversations should first be broached by telephone or in person.
is important for the physician to educate or remind the caller that Similar guiding principles apply with telephone and email commu-
data sent by cell phone is not secure. Many EMRs now include the nications; the physician must establish rapport, give clear advice,
option for patients to upload their own photographs or for health and provide guidance for follow-up. Attention should be paid to
professionals to use a secure application to capture photographs for correct grammar and spelling in emails, because errors may decrease
documentation in the medical record. The latter method is preferred. confidence in the physician. Electronic communication can also
be a useful method to provide patient education materials, such as
Telephone Management: Preventive a link to a helpful website.
Care and Care Coordination Physicians should work with their health care system to clarify
types of communication that are best transmitted electronically. For
Telephone encounters can provide an excellent opportunity for
example, straightforward questions may be answered with a simple
preventive care and anticipatory guidance. Issues such as sleep
email message, but more complex care needs may be appropriate for
problems or behavioral issues may be difficult to discuss during an
a formal e-visit that can be billed to insurance. Research suggests
office visit with the child present. Telephone follow-up provides an
that insurance plans may influence how patients choose to use
opportunity to minimize distraction during conversation between
e-medicine. For example, the patient with a high deductible insur-
parent or guardian and physician. Some pediatric offices develop
ance plan may prefer to first contact the health professional through
protocols and charge a fee for prolonged telephone consultation.
an e-visit to determine if an in-person office visit is necessary.
Telephone follow-up can also help facilitate care coordination for
children with chronic health conditions. For example, a physician
who recommends medication changes for a child who presents with Health Care Disparities
poorly controlled asthma can have a follow-up call with the parents When developing telephone and e-medicine options, it is necessary
1 month later to assess asthma symptoms. Using telephone follow-up to pay particular attention to meeting the needs of patients with lim-
when appropriate allows the health professional to provide patient- ited English proficiency. Studies have shown that patients with limited
centered care (ie, individualize care to the patient’s needs) and English proficiency use e-medicine less frequently than patients with
minimize missed work and school for patients and families. English proficiency, particularly for tasks such as requesting medica-
tion refills. Additionally, 1 study showed that in telephone encounters,
E-medicine patients with limited English proficiency disagreed with the care rec-
ommendation and tended to receive advice to seek higher acuity care
Virtual medicine, or e-medicine, is an increasing avenue for
more often than patients with English proficiency. Recommendations
communicating with patients. Studies suggest that up to 75% of
to promote health equity include continuing to explore ways to ensure
patients would like to communicate with their physician through
email. Patients and parents who choose electronic communication that telephone and e-medicine are being delivered in a manner that
as a means of interacting with their physician report high rates of is equitable and patient-centered for all patients.
satisfaction and tend to have higher levels of education.
Email communication can be convenient for patients and families Conclusions
and may increase patient-centered care. Specific American Academy Telephone management and e-medicine represent the changing face
of Pediatrics, American Medical Association, and American Medical of medical practice. The health professional should be prepared to
Informatics Association guidelines exist for the use of email deliver care that meets the needs of the patient, which may necessi-
with patients. Traditional email poses a variety of data security tate being creative with how and where care is delivered. The approach
concerns, however. Therefore, many EMRs incorporate the ability must be tailored to each patient scenario, and physicians should con-
for patients and their parents or guardians to interact directly with tinually strive to adapt their approach so that they can provide effec-
the EMR. This includes the ability to view laboratory test results, tive and appropriate patient-centered care both in and out of the office.
immunization records, growth charts, and patient problem lists.
Additionally, it may be possible for a parent or guardian to send a
secure message with a health question to the health care team. Clinic CASE RESOLUTION
staff should create guidelines for patients and parents or guard- The physician learns several facts that result in the recommendation that the
ians about appropriate issues to be addressed through electronic child be seen that day. (Had the call been received at night, a visit the next day
messaging, expected response time, and who will provide a response would have been advised.) These facts include the child’s age, the height and
duration of the fever, and lack of any symptoms of localized infection.
(ie, nurse or physician).
The other concerns of feeding questions and sleep issues present excel-
Communication through the EMR should be professional, lent opportunities for management through a follow-up telephone call, elec-
concise, and to the point. Written communication has the benefit of tronic communication, or office visit, depending on parent preference and clinic
eliminating sequential missed calls back and forth (ie, “telephone resources.
tag”) when attempting to reach a parent or guardian by telephone.

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 Chapter 5: Telephone Management and E-medicine 25

Pediatrics. 2006;118(4):1768–1773 PMID: 17015574 https://doi.org/10.1542/

Selected References
peds.2006-2099
American Academy of Pediatrics. Section on Telehealth Care www.aap.org/en-us/ Moreno G, Lin EH, Chang E, et al. Disparities in the use of internet and
about-the-aap/Sections/Section-on-Telehealth-Care/Pages/SOTC.aspx. Accessed telephone medication refills among linguistically diverse patients. J Gen
April 1, 2019 Intern Med. 2016;31(3):282–288 PMID: 26311200 https://doi.org/10.1007/
Brown JL, Swiontkowski MF. Pediatric Telephone Medicine: Principles, Triage, s11606-015-3500-6
and Advice. 3rd ed. Philadelphia, PA: J B Lippincott; 2003 Njeru JW, Damodaran S, North F, et al. Telephone triage utilization among
Bunik M, Glazner JE, Chandramouli V, Emsermann CB, Hegarty T, Kempe A. patients with limited English proficiency. BMC Health Serv Res. 2017;17(1):706
Pediatric telephone call centers: how do they affect health care use and costs? PMID: 29121920 https://doi.org/10.1186/s12913-017-2651-z
Pediatrics. 2007;119(2):e305–e313 PMID: 17272593 https://doi.org/10.1542/ Reed M, Graetz I, Gordon N, Fung V. Patient-initiated e-mails to providers:
peds.2006-1511 associations with out-of-pocket visit costs, and impact on care-seeking and
Gerstle RS; American Academy of Pediatrics Task Force on Medical Informatics. health. Am J Manag Care. 2015;21(12):e632–e639 PMID: 26760425
E-mail communication between pediatricians and their patients. Pediatrics. Schiller JH, Christner JG, Stansfield RB, Watnick CS, Mullan PB. What parents
2004;114(1):317–321 PMID: 15231952 want from emails with their pediatrician: implications for teaching communi-
Kempe A, Bunik M, Ellis J, et al. How safe is triage by an after-hours telephone cation skills. Patient Educ Couns. 2013;92(1):61–66 PMID: 23510794 https://
call center? Pediatrics. 2006;118(2):457–463 PMID: 16882795 https://doi. doi.org/10.1016/j.pec.2013.02.012
org/10.1542/peds.2005-3073 Schmitt BD. Pediatric Telephone Protocols: Office Version. 16th ed. Itasca, IL:
Lee TJ, Guzy J, Johnson D, Woo H, Baraff LJ. Caller satisfaction with after-hours American Academy of Pediatrics; 2018
telephone advice: nurse advice service versus on-call pediatricians. Pediatrics. Ye J, Rust G, Fry-Johnson Y, Strothers H. E-mail in patient-provider commu-
2002;110(5):865–872 PMID: 12415022 https://doi.org/10.1542/peds.110.5.865 nication: a systematic review. Patient Educ Couns. 2010;80(2):266–273 PMID:
Melzer SM, Reuben MS; American Academy of Pediatrics Section on Telephone 19914022 https://doi.org/10.1016/j.pec.2009.09.038
Care, Committee on Child Health Financing. Payment for telephone care.

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 CHAPTER 6

Informatics
Alan Tomines, MD

CASE STUDY
You are a physician in a small pediatric practice. Your Questions
hospital implemented an electronic health record system, 1. What are the important informatics concepts to
which has been made available within the hospital and in understand?
the offices of its affiliated practices. The hospital chief of 2. What are the important drivers of health information
staff asks you to participate on the hospital’s informatics technology?
committee. You have served in the past on other clinically 3. What are the challenges to physician acceptance of
oriented steering committees, but you do not consider electronic health records?
yourself a technology expert and you express your trep- 4. What are the special pediatric considerations in
idation to the chief of staff, who asks you to speak with electronic health records?
the head of the informatics committee.

To make optimum clinical decisions, physicians must have infor- system is the sum of the people, work processes, and information
mation about their patients’ health that is current, accurate, reliable, technology that supports an activity. Depending on the degree to
and complete. The physician should be able to access this infor- which technology is applied, the processes of an information system
mation wherever and whenever necessary. To the extent possible, may be automated, manual, or a combination of both.
the physician should be presented with information that fosters an
evidence-based approach to decision making, and the decisions Informatics Defined
made should be communicated to other health professionals in a
manner that is clear and error-free. The physician should be able to Medical informatics is the science of the appropriate application of
review measures of the quality of care provided. Health information information technology to health care work processes. Specialists
technology (HIT) holds the promise of increased access to patient in informatics (referred to as informaticians or informaticists) serve
health information, improved patient safety, reporting of desired as liaisons between clinical and technology staff to ensure that HIT
health outcomes, and improved health care efficiency with the poten- is optimally applied to address clinical information and workflow
tial for decreased health care expenditures. The implementation and needs. Medical informatics places great emphasis on nontechno-
acceptance of HIT is not without challenges, however. logic considerations that can affect the successful implementation
and acceptance of health information systems, including informa-
tion science, cognitive psychology, project management, organi-
Basic Concepts
zational and change management, health care policy, and ethics.
Although the terms “data,” “information,” and “knowledge” are The field of medical informatics can itself be subdivided into
sometimes used interchangeably, these are distinct concepts. Data specific clinical domains, such as nursing, pharmacy, veterinary
are mere observations or facts (eg, hemoglobin equal to 9 g/dL). medicine, dentistry, and imaging. Biomedical informatics is a term
Information is data placed in meaningful context (eg, hemoglobin of broader scope that encompasses medical informatics; bioinfor-
equal to 9 g/dL in a 3-month-old who is breastfed). Knowledge is matics, in which the primary domain is genomics and bioengineer-
the understanding of information, including an assessment of its ing; and public health informatics.
completeness (eg, hemoglobin equal to 9 g/dL in a 3-month-old
infant who is breastfed may represent a physiologic nadir but may
also represent blood loss, increased destruction of red blood cells, Electronic Health Information Systems
or decreased production of red blood cells). All electronic health information systems ultimately are used in man-
Information technology refers to any hardware or software that aging some aspect of patient care, and for ease of discussion in this
supports the management of data, including how the data are chapter these systems are presented in 4 broad categories: electronic
acquired, stored, retrieved, transformed, interpreted, and dissem- records of patient care, ancillary clinical systems, administrative sys-
inated. For example, a database is an organized collection of data tems, and telemedicine. Table 6.1 lists several common abbrevia-
that facilitates the storage and retrieval of those data. An information tions used in electronic health information systems and informatics.

27

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 28 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Table 6.1. Abbreviations Used in Informatics illegible handwriting, decrease delays in the receipt and execution
of orders, and allow entry of orders away from the care setting.
Abbreviation Expansion
Additionally, a CPOE paired with a CDSS can leverage patient-
ARRA American Recovery and Reinvestment Act of 2009 specific information in a CDR to prevent harm resulting from drug-
CDR Clinical data repository drug interactions, drug-allergy interactions, or errors in age- or
CDSS Clinical decision support system weight-based dosing.
CPOE Computerized physician order entry Although an EMR generally operates within the functional
boundary of a hospital or practice, the history of a patient’s health
EHR Electronic health record
care is not limited to these settings. The personal health record
EMR Electronic medical record (PHR) is a summary of an individual’s health history—usually self-
eRx Electronic prescribing maintained—that contains information collected from encounters
HIE Health information exchange with different health professionals, medicolegal documents (eg,
HIPAA Health Insurance Portability and Accountability Act of 1996 living wills, advance directives), and other health information that
may be relevant to patients (eg, regimens for nontraditional reme-
HIS Hospital information system
dies, logs of home testing for blood pressure or glucose). The concept
HIT Health information technology of a PHR is not new; patients with multiple medical problems have
HITECH Health Information Technology for Economic and Clinical long maintained paper-based PHRs out of necessity to ensure they
Health Act have at least 1 reliable and portable source containing a complete
IIS Immunization information system (also known as an medical history. The electronic PHR is an evolving entity, ranging
immunization information registry) from scanned paper documents stored on portable devices to web-
LIS Laboratory information system based applications that connect with EMRs and capture data from
medical devices. The PHR is not considered a legal record of care.
P4P Pay for performance
The next stage in the evolution of the EMR is the electronic health
PACS Picture archiving and communication system
record (EHR), which has the functionality of a full-fledged EMR
PHI Protected health information with the additional capability for exchanging data among multi-
PHR Personal health record ple different EMRs to provide the entire longitudinal history of the
RIS Radiology information system patient. With this data exchange capability, an EHR may also support
the needs of population health, such as identification of patient-
applicable clinical trials, mandatory reporting of notifiable disease,
and provision of anonymized clinical data to support clinical and
Electronic Records of Patient Care public health research. (The term EHR has been used interchange-
A medical record serves as the legal record of care provided to a ably with and has largely supplanted the term EMR.)
patient by a health professional or health care organization. An A health information exchange (HIE) is an enabling technology
electronic medical record (EMR) is an information technology that that acts as a hub for the secure exchange of data between EHRs.
supports the traditional role of the medical record, including serving Via an interface (a program that allows 1 information system to
as an archive for clinical documentation, such as physician orders, communicate with another), an EHR can connect to an HIE and
progress notes, and laboratory and imaging results. A full-fledged exchange health information with other EHRs that are connected
EMR is more than an electronic version of the traditional paper- to that HIE. Although an HIE generally serves a specific geographic
based record, however. It provides capabilities that support the region, in future the interconnection of HIEs may allow an EHR in
enhanced delivery of care. 1 region to share data with an EHR in another region, thereby pro-
Important components of an EMR include a clinical data viding nationwide or worldwide access to a patient’s entire history
repository, a clinical decision support system, and computerized of episodic care.
physician order entry. A clinical data repository (CDR) is a real-time
database containing the clinically relevant patient data of an insti- Ancillary Clinical Systems
tution. It supports timely access of patient information for phy- Ancillary clinical systems provide information management and
sician decision making and provides information used by other automation for specific health care services or domains, including
EMR components. A clinical decision support system (CDSS) is a pharmacy, laboratory, imaging, and immunizations.
special computer program that applies medical knowledge to data A pharmacy information system tracks patients’ prescription and
from a CDR to produce patient-specific care recommendations. payment information and can improve patient safety by checking for
A computerized physician order entry (CPOE) is a specialized medication interactions and appropriate dosing. A related concept
computer program that allows health professionals to write is electronic prescribing (e-prescribing or eRx), which is a specialized
electronic orders that are directed to the appropriate clinical staff or type of CPOE that allows physicians to prepare and transmit
ancillary department. A CPOE can decrease errors resulting from prescriptions electronically to a pharmacy information system.

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 Chapter 6: Informatics 29

When connected to an EHR, a pharmacy information system Key Drivers for Adoption
can receive and process prescriptions, and send back dispensing of Informatics
information to support medication reconciliation.
Laboratory information systems (LISs) are used to manage The key drivers for the adoption of HIT are improved patient safety,
the receipt of laboratory orders, track specimens, capture data the ability to measure health care outcomes, increased efficiency
from automated analyzers, and present laboratory results to the of work flow in the patient care setting, and reduced health care
ordering health professional via direct access to the LIS, or via an expenditures.
interface that transfers the results to an EHR. An LIS can support Improved patient safety through the reduction of preventable
patient safety through timelier access to laboratory results and data errors is the primary driver of information technology adoption
for use in making individualized patient care decisions. in many health care organizations. In the report To Err Is Human:
A picture archiving and communication system manages the Building a Safer Health System, the Institute of Medicine (now known
storage and distribution of patients’ electronic medical images, as the Health and Medicine division of the National Academies)
which often are captured directly from computerized or digital estimated that up to 98,000 deaths annually in the United States
radiography devices, as well as other imaging modalities (eg, are attributable to medical errors. Most errors were noted to be
computed tomography, magnetic resonance imaging, ultrasonogra- preventable and caused by systems and processes that increase or fail to
phy). A radiology information system incorporates the functionality prevent human errors. Computerized physician order entry is an
of a picture archiving and communication system while managing EHR technology with the potential to improve patient safety by alert-
other service activities, such as reporting, scheduling, and billing. ing a physician to errors before an order is submitted in an EHR.
An immunization information system (IIS, also referred to as Changes in the health care marketplace are driving the measure-
an immunization registry) is used to document and track patient ment of health care outcomes. Pay for performance, which relates
vaccinations. An IIS can send reminder or recall notices, as appro- payment to measures of quality of care provided; medical “report
priate, to physicians and parents or guardians when vaccina- cards” that permit comparison of health care plans and profession-
tions are due or when administered vaccines are determined to be als; and accountable care organizations are examples of market driv-
ineffective or unsafe. An IIS usually is maintained regionally by ers. Electronic health records are critical to collecting and analyzing
public health entities; thus, it also collects reports on adverse data to calculate these measures. Additionally, the decision support
vaccine events and provides summaries of regional vaccination system of an EHR may improve health care outcomes through guide-
prevalence. These systems usually have the capability to exchange line adherence, such as by reminding both physicians and patients
data with capable EHRs. about care options that may have been overlooked.
Improvements in efficiency are another driver in the adoption
of information technology. For example, efficiency can be gained
Administrative Systems by automating highly repetitive, data-intensive activities such as
A hospital information system comprises all the clinical and non- billing and scheduling. The introduction of information technol-
clinical information systems of an institution. A hospital informa- ogy into a clinical setting without thorough consideration of effects
tion system may be a single integrated information system or may on work flow, however, may introduce unanticipated workflow
represent multiple interconnected information systems. Hospitals consequences that decrease efficiency or result in the creation of
and large practices often have separate information systems to workarounds that reduce the effectiveness of the newly imple-
manage individual administrative functions, such as appoint- mented technology.
ment scheduling, insurance eligibility, and billing and payment. In 2016, health care spending represented nearly 18% of the gross
A practice management system is an information system designed domestic product of the United States and increased at a rate nearly
to manage administrative tasks for small- and medium-sized twice that of inflation. Information technology is considered to be
clinical practices. a possible source of cost savings because its use has the potential to
reduce duplication of diagnostic studies as well as the time spent on
administrative tasks. As predicted in the 2001 report Crossing the
Telemedicine
Quality Chasm: A New Health System for the 21st Century, the use
Telemedicine is not an information system per se but a related of email has served to meet the needs of patients more quickly and
concept of interest in informatics. Telemedicine is the use of infor- at lower cost than a traditional visit.
mation and communication technologies to deliver health care over
a distance, often to support patient care in rural or underserved
areas. Many specialties exist within telemedicine, and they roughly Challenges
correspond to distinct medical specialties, such as teleradiology Despite the existence of significant health care drivers for the adop-
(ie, the transmission of radiologic images electronically for interpre- tion of informatics, several challenges prevent the easy implemen-
tation) and telesurgery (ie, the use of video and robotic technology to tation and acceptance of HIT, including information security,
perform surgery). The use of email and websites to consult with technology costs, organizational change, system usability, and the
patients is also a form of telemedicine. effect on physician-patient interaction.

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 30 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

The ease with which information systems can exchange data It is also important to consider the effect of technology on the
should not belie the care required to protect electronic patient patient-physician interaction. Physicians increasingly engage in elec-
data. Protected health information (PHI) is any information about tronic documentation and ordering during patient visits. Although
a patient (eg, name, medical record number) that may be used to some patients have a favorable opinion on the use of HIT, physicians
identify that patient. Patients have the expectation that their PHI should be sensitive to patients who feel that technology is intru-
will be kept private; health professionals’ assurance that private sive; as appropriate, physicians should acknowledge the intrusion
information will not be revealed is referred to as confidentiality; the and identify potential benefits to the patient, including efficiency
policies and technologies that support confidentiality are called of access to information and more efficient communication of pre-
security. The Health Insurance Portability and Accountability Act scriptions to pharmacies or orders to ancillary services. Additionally,
of 1996 defines the measures that must be taken to ensure that PHI physicians should remain vigilant and not allow the EHR to detract
is kept secure and is made available only to authorized individuals attention from their patients during the office visit.
or organizations participating in a patient’s health care. Although Human-computer interaction is the study of the interactions
health information systems often use sophisticated technical barri- between people and information technology. It is important for users
ers to protect patient data, recent instances have occurred in which to perceive that the technology is both useful (ie, supports the work
hospitals’ EHRs have been accessed and control of them gained being done) and usable (ie, readily learned, efficient, helps in avoid-
by unauthorized parties who sought ransoms to restore control of ing and correcting errors). Perception by the physician that an EHR
the EHR. Generally, such data breaches are the result of failure to is difficult to use or interferes with patient care or patient interac-
adhere to security safeguards, such as sharing passwords or fail- tion may impede the acceptance of EHRs as supportive tools. The
ing to update security software. Educating health professionals is topic of usability of HIT is an emerging area of research in the field
critical to ensuring the confidentiality of patient data. of informatics.
The cost of implementing health information systems can be
prohibitive for health care delivery organizations, particularly small
practices. To address this barrier, the Health Information Technology Pediatric Considerations
for Economic and Clinical Health (HITECH) Act was included in the Although certified EHRs are present in more than 95% of hospitals,
American Recovery and Reinvestment Act of 2009. The HITECH Act only 3 in 4 children’s hospitals have successfully demonstrated their
authorized the provision of Medicare and Medicaid incentives to meaningful use; similarly, pediatricians trail other primary care
physicians and hospitals that adopt EHRs and demonstrate “mean- specialists in demonstrating the meaningful use of EHRs. Although
ingful use” of EHRs by meeting specific objectives toward improved pediatric patients represent approximately 25% of the US pop-
health care delivery and outcomes. These objectives include using ulation, pediatric EHR functionality often is underdeveloped.
EHRs for basic activities, such as recording patient demograph- When considering these systems for pediatric settings, particular
ics and vital signs; maintaining active problem, medication, and attention should be focused on the highly specific data, task, and
allergy lists; providing patients with summaries of outpatient policy needs of pediatric practice.
visits and inpatient discharge instructions; electronic prescriptions The presence of functionality to support clinical tasks that
and CPOE; and providing drug-drug and drug-allergy checks. The are generally, if not uniquely, related to pediatrics should always
objectives also include more complex functions, such as provid- be examined. Immunization management, including the ability
ing data to public health agencies for disease surveillance as well to assess a patient’s status or exchange data with an IIS, is highly
as immunization and cancer registries; generating lists of patients desirable. Weight-based dosing and tracking of specialized growth
with specific conditions for quality improvement and research; parameters (eg, for Down syndrome, for preterm infants) are other
identifying and providing patient-specific education resources; and functions that are often overlooked. Age-specific documentation and
supporting medication reconciliation and care summaries across educational materials may be lacking or may require customization.
health care settings. To assure physicians and hospitals that they The data and terminology of an information system should suit
are adopting EHRs that allow them to meet meaningful use regula- the highly specific needs of pediatric practice. For example, units of
tions, the US Department of Health and Human Services developed measure should reflect the requisite data precision needs of pediat-
criteria for certifying EHRs. By 2016, more than 95% of eligible ric patients. Options to display patient age in hours rather than years,
hospitals and 60% of office-based physicians had demonstrated months, or even days and weight in grams is critical to appropriate
meaningful use of certified EHRs. care in the neonatal period. Laboratory values should be accompa-
Management of organizational change is an important part of nied by normative ranges for age. Patient identification must account
successful information system implementations. Introduction of for the frequent changes in names and numeric identifiers that may
technology inevitably results in workflow changes for physicians occur in infancy or during change of custody. Pediatric terminology,
and nurses and may result in repurposing of administrative staff. such as developmental milestones, type of cry, or characterization of
Resistance to these changes should be anticipated, and strategies to stool, are often overlooked in information systems designed for adults.
mitigate resistance should be instituted, including involvement of Pediatric policy issues should also be reflected in the design of
health professionals in the selection and ongoing enhancement or an information system. Authorization for the child or the child’s
optimization of EHRs. parent(s), guardian(s), or other legal authority to view all, none, or

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 Chapter 6: Informatics 31

portions of a child’s medical record should be enforced electroni- Centers for Medicare & Medicaid Services. National Health Expenditure fact
cally as it would be for the paper medical record. This is particularly sheet. www.cms.gov/research-statistics-data-and-systems/statistics-trends-and-
true for adolescent records, in which the need for granular aware- reports/nationalhealthexpenddata/nhe-fact-sheet.html. Last modified February
20, 2019. Accessed April 1, 2019
ness of EHR data elements by the pediatrician as well as privacy con-
trols to restrict what a parent, guardian, or other legal authority may Institute of Medicine Committee on Quality Health Care in America. Crossing
the Quality Chasm: A New Health System for the 21st Century. Washington, DC:
see, should be consistent with the relevant statute. Likewise, second-
National Academies Press; 2001
ary use of patient data for public health, research, or commercial
Institute of Medicine Committee on Quality of Health Care in America. In: Kohn
purposes should be allowed or restricted as appropriate.
LT, Corrigan JM, Donaldson MS, eds. To Err Is Human: Building a Safer Health
Currently, no electronic medical home for the care of children System. Washington, DC: National Academies Press; 2000
exists. The electronic history of a child’s care may be distributed across
Lehmann CU; American Academy of Pediatrics Council on Clinical Information
EHR systems, immunization registries, school health information Technology. Pediatric aspects of inpatient health information technology
systems, and specialty registries for rare conditions, trauma, or foster systems. Pediatrics. 2015;135(3):e756–e768 PMID: 25713282 https://doi.
care. With the promise of HIEs as a conduit for data exchange, it org/10.1542/peds.2014-4148
may be possible to create a virtual medical home in the future. It is Lehmann CU, O’Connor KG, Shorte VA, Johnson TD. Use of electronic health
important that pediatricians work toward reducing the legislative, record systems by office-based pediatricians. Pediatrics. 2015;135(1):e7–e15
technologic, and cultural barriers to linking child health information PMID: 25548325 https://doi.org/10.1542/peds.2014-1115
systems without compromising the security or confidentiality of PHI. Middleton B, Bloomrosen M, Dente MA, et al; American Medical Informatics
Pediatricians should be actively involved in the acquisition Association. Enhancing patient safety and quality of care by improving the
and development of information technology to ensure that child- usability of electronic health record systems: recommendations from AMIA.
specific data and policy needs are addressed. Pediatricians should J Am Med Inform Assoc. 2013;20(e1):e2–e8 PMID: 23355463 https://doi.
org/10.1136/amiajnl-2012-001458
also be involved in national policy initiatives to ensure that health
information systems are certified for pediatric use and integrated Nakamura MM, Harper MB, Castro AV, Yu FB Jr, Jha AK. Impact of the
meaningful use incentive program on electronic health record adoption by
to support care that is child-centered.
US children’s hospitals. J Am Med Inform Assoc. 2015;22(2):390–398 PMID:
25755126 https://doi.org/10.1093/jamia/ocu045

CASE RESOLUTION The Office of the National Coordinator for Health Information Technology Health
IT Dashboard. Quick stats. https://dashboard.healthit.gov/quickstats/quickstats.
You speak with the head of the informatics committee. You learn that you have
php. Last updated February 6, 2019. Accessed April 1, 2019
been asked to participate because of your understanding of physician work-
flow in the office setting and that you are intended to advocate for the highly Sittig DF, Singh H. Legal, ethical, and financial dilemmas in electronic health
specific data needs and policies associated with the pediatric population. You record adoption and use. Pediatrics. 2011;127(4):e1042–e1047 PMID: 21422090
are expected to use your pediatric expertise and draw on your leadership Spooner SA; American Academy of Pediatrics Council on Clinical Information
experience to obtain stakeholder buy-in of information systems. Additionally, Technology. Special requirements of electronic health record systems in pediat-
in collaboration with other pediatricians and physicians, you will work to rics. Pediatrics. 2007;119(3):631–637 PMID: 17332220 https://doi.org/10.1542/
improve the efficiency, effectiveness, and relevance of the EHR in supporting the peds.2006-3527
physicians’ work and working to achieve improved patient outcomes.

Selected References
Anoshiravani A, Gaskin GL, Groshek MR, Kuelbs C, Longhurst CA. Special
requirements for electronic medical records in adolescent medicine. J Adolesc
Health. 2012;51(5):409–414 PMID: 23084160 https://doi.org/10.1016/j.
jadohealth.2012.08.003

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 CHAPTER 7

Counseling Families
About Internet Use
Alan Tomines, MD

CASE STUDY
A 16-year-old girl is accompanied by her mother for a Questions
routine visit. The girl is doing well in school, is active in 1. What are the commonly used internet services?
team sports, and has a small circle of friends who are 2. What are the benefits and risks of the internet?
well-known to her mother. The mother describes no new 3. What strategies may be used to make the internet
problems at home and no changes in behavior. However, safer to use?
the mother is concerned that her daughter “spends too 4. What signs may indicate that an adolescent is
much time on the computer.” engaging in risky online behaviors?

The internet is a worldwide system of interconnected computer further expanded the exchange of scientific information. In 1990,
networks. At its inception, it was the exclusive domain of the military ARPANET was retired, and many of its networks were absorbed into
and academia; currently, the internet is a public gateway to elec- NSFNET. The resulting network, which was renamed the “Internet,”
tronic information, communication, and commercial services. The extended its reach into the business sector and to international
internet has profoundly changed the way we learn, work, play, and researchers but remained relatively inaccessible to the lay public.
interact with others. It has become an integral part of the education During the early 1990s, the European Organization for Nuclear
and socialization of children, making it an influence of interest for Research (known as CERN) developed protocols to facilitate the
parents, physicians, and researchers. The physician can provide prag- sharing of physics research data over the internet. These proto-
matic counsel to families that focuses on the benefits and risks of the cols allowed the sharing of computer files composed of text and
internet, strategies for safer use, and assessment of online behav- images as documents that could be viewed using a special computer
ior in adolescents. The physician should also be prepared to discuss program called a “browser.” This system of linked documents was
health information that families find on the internet. collectively dubbed the World Wide Web or simply “the Web,” with
related collections of documents referred to as web pages or web-
A Brief History of the Internet sites. In 1993, CERN made its work freely available, resulting in the
The internet began in the 1960s as the Advanced Research Projects rapid proliferation of publicly accessible commercial and personal
Agency Network (ARPANET), a US Department of Defense project websites—a model that has continued to the present day.
to share government-funded, university-based computer resources
across a reliable communications network. Through the 1970s, Internet Services and Concepts
ARPANET found acceptance with academics and researchers as Internet access usually is obtained through an internet service
a conduit for the exchange of scientific information, in large part provider (ISP), a company that has an internet connection that
because of the newly developed email technology for sending and it shares with consumers on a subscription basis. The most com-
receiving messages. monly used internet services are email and the Web. A person
Recognizing the potential of the ARPANET, the National Science actively using an internet service is said to be “online.” Use of the
Foundation (NSF) began a parallel effort to connect the computer Web is sometimes referred to as surfing and is facilitated by search
science departments at universities that were not affiliated with engines, such as Google and Bing, that use keywords or topics to
ARPANET. Initially called the Computer Science Network (CSNET) find relevant documents, images, websites, or other services on
and later renamed NSF Network (NSFNET), this new network the internet.

33

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 34 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Internet services may be accessed by a wide range of computing socioeconomic status. Although currently most households have
devices, from traditional personal and laptop computers to mobile access to a computer with access to the internet, millions of indi-
platforms, such as tablets and smartphones (cell phones that pro- viduals do not have access. The pediatrician may help families gain
vide access to internet services). Smartphones and tablets access access to the internet by identifying institutions that provide safe
scaled-down versions of web-based content via specialized applica- online environments, such as libraries, community centers, and
tions commonly referred to as “apps.” schools. As patient-centered internet-based health interventions are
Because of their popularity in the pediatric age group, a few inter- increasingly being studied as a supplement to traditional health care
net services are worthy of physicians’ notice. Web logs (or blogs) delivery, addressing disparities in internet access may help bridge
are web-based journals. Like a traditional paper-based journal, a gaps in care in underserved communities.
blog supports the written presentation of activities, thoughts, or The physician should also be aware that children with disabilities
feelings. A blog that is delivered using video usually is referred to as may have difficulty using the internet without assistive technology,
a video blog (or vlog). Blogs and vlogs may be public (available for that is, hardware or software designed to improve the accessibility
anyone to read) or private (having restricted access). of computers. Children with visual impairment or blindness may
Social networking websites, such as Facebook, Twitter, and be aided by the use of special software, including screen magni-
Instagram, are similar in content to blogs and vlogs, with the added fiers, braille embossers, or screen readers (ie, software that uses a
dimension that users are encouraged to create a network of online computer-generated voice to read email and web pages), although
friends by establishing links to other users’ social networking many websites are not compatible with screen readers. Children with
accounts. Blogs, vlogs, and social networking websites are exam- hearing impairment or deafness may be aided by ensuring that their
ples of asynchronous services in which communication between users computers are set to provide visual cues rather than audio prompts;
does not have to take place in real time and the content of which typically, such functionality is built into operating systems. Children
may be moderated and censored by the owner of the service for with mobility or dexterity challenges may have difficulty using a
appropriateness. traditional keyboard and mouse; alternative keyboards and point-
By contrast, chat rooms are internet venues that permit the syn- ing devices, computer touch screens, and tablet devices can be sug-
chronous (ie, real-time) exchange of text messages between multiple gested as supporting technologies. Pointing devices that use sound
simultaneous participants. Chat rooms as independent entities have or infrared beams, as well as software that responds to the spoken
largely been supplanted by other similar synchronous technologies, word, may be viable alternatives in cases in which manual control
including direct messaging communication offered through social is not possible.
networking websites as well as instant messaging, a popular premium
service often offered with smartphones. Although sometimes referred Internet Threats
to as “text messaging,” instant messaging (IM) is a more inclusive term
Although the internet has many benefits, being online entails some
that reflects the immediacy of interaction as well as the expansion of
risks. The physician should be able to inform parents and guardians
content exchanged to include multimedia, such as images or audio
about the threats posed by the internet.
and video files.
To understand internet threats, it is helpful to recognize that the
The aggregate of synchronous and asynchronous social network-
internet has neutral properties that do not cause threats but that
ing internet services is collectively referred to as social media. The
allow them to exist. First, the internet is anonymous: People may
ready accessibility of internet access, the portability of internet-ready
not be who they represent themselves to be. Second, the internet is
devices, and the convenience of social media apps makes it possible
interactive: Unlike traditional media (eg, newspapers, radio, televi-
to have a continuous online presence.
sion), the internet user may have real-time interactions with another
person, or with sophisticated computer programs that respond as
Internet Benefits and Access if human. Third, the internet has few restrictions: Anyone can put
The physician should be able to identify the benefits of internet use. almost anything on the internet, without regard to credibility or
The internet provides access to a wealth of educational resources appropriateness. Finally, the internet is public and permanent:
and cultural experiences. Access to these resources allows chil- Although information on a website may be removed, it is possible
dren to exercise their reading, writing, information-seeking, and for anyone who visits a website to make an electronic copy of what is
technology skills. The internet also provides the opportunity to eas- there, and many websites are archived and may be retrievable from
ily communicate with family and friends. Additionally, children with websites, such as the Internet Archive (http://archive.org), long after
common interests can use internet services to commiserate with their removal from internet search engines.
and encourage other children. For example, the social network- Although these properties of the internet are neutral, they enable
ing websites Ben’s Friends (www.bensfriends.org) and Rareshare some of the most common internet threats: exposure to strangers
(https://rareshare.org) connect patients with uncommon medical and/or predators; interpersonal victimization; exposure to pornog-
conditions to help them share their collective experiences. raphy; and participation in online gaming, gambling, and shopping.
The physician may also actively participate in reducing dispar- The anonymity and interactivity of the internet enables online
ities in internet access. Internet use and access is correlated with predators. More than one-third of adolescents online have “friends”

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 Chapter 7: Counseling Families About Internet Use 35

whom they have never met in person. Approximately three-fifths internet. These recommendations include the avoidance of most
of adolescents active online have received an instant message or screen media for children younger than 18 months; limiting screen
email from a stranger, and approximately 1 in 6 has been contacted use to 1 hour per day for children ages 2 through 5 years; and
by someone who made them feel scared or uncomfortable. More consistent time limits for children age 6 years and older, specifically
than 90% of teenagers have shared personal information about identifying activities that are not allowed. The AAP offers a Family
themselves online, including name, birth date, interests, and Media Plan tool (www.healthychildren.org/English/media/Pages/
contact information. default.aspx) to assist parents and guardians in creating develop-
Online interpersonal victimization is the receipt of harassment or mentally appropriate plans for managing digital media use.
unwanted sexual attention over the internet. One-fifth of children By sharing time online, parents and guardians can promote
have reported being victimized; 1 factor that places individuals at and model responsible internet behavior. These adults should talk
high risk for victimization is talking about sex with someone online. with their children about what they see together on the internet
Cyberbullying is a specific type of online interpersonal victimization and encourage children to share what they have experienced when
consisting of receipt of electronic communications that are harmful online alone, whether good or bad. Placing the computer or other
or threatening. Cyberbullying may be as prevalent online as internet-enabled device shared by the family in a public location in
“traditional” bullying, with nearly one-fifth of middle school–age the home will encourage the idea that the internet is a shared experi-
children reporting that they had been cyberbullied at least once ence; however, caregivers should be aware that mobile devices allow
in the previous 12 months. Approximately 88% of teenagers have children to access the internet independently and covertly.
witnessed other people being mean or cruel online, with 21% Parents and guardians should set and enforce house rules for
saying that they have joined in. internet behavior. Children should be encouraged to be good cit-
Although children may be individually targeted by strangers or izens, including not doing anything that may be hurtful to oth-
cyberbullies, they may also passively encounter undesired internet ers and not plagiarizing information that they find freely on the
content. More than 40% of children reported having been exposed internet. Children should not communicate with or plan to meet
to pornography online, and nearly two-thirds of those children strangers known only to them through the internet, nor should they
described this exposure as unwanted. In adolescents between the respond to messages that are unsolicited or that make them feel
ages of 15 and 17 years, more than 70% reported accidentally being uncomfortable. Children should not share personal information or
exposed to online pornography, with a risk factor for exposure being pictures with others on the internet, nor should they download files
the downloading of images. from the internet. For older children, safety pledges can be used as
Sexting is the exchange of sexually explicit text, images, or formal agreements of acceptable use.
multimedia, generally via IM. Sexting sits at the convergence of To facilitate parental or other caregiver oversight of internet use,
pornography and cyberbullying, with the added danger that some the physician should advise that computers and mobile devices may
adolescents may not perceive sexting as harmful or threatening. In be configured to allow internet access only during certain hours of
some jurisdictions, minors who have participated in sexting have the day and to disable access to the internet after a specified amount
been charged with possessing child pornography. of time has elapsed. As possible, children should be provided with a
In addition to the aforementioned threats, recent studies point separate account to access devices and the internet. Having a sepa-
to the internet and digital media use as having a role in addictive rate account promotes autonomy for older children while allowing
behavior and attention-deficit/hyperactivity disorder as well as parents or guardians to restrict internet content. Monitoring soft-
in detracting from healthy behaviors, such as physical activity and ware automates tracking of internet usage by creating a reviewable
adequate sleep. With the wide variety of benefits of and threats posed record of websites and images viewed, messages sent and received,
by the internet, the physician should be prepared to provide parents and even individual keystrokes entered. The potential benefits of
and guardians with strategies for appropriate and safe internet use. monitoring software should be weighed against the invasion of the
child’s privacy, and parents and guardians should understand that
Strategies for Appropriate some children are savvy enough to erase their internet browsing
Internet Use history; consequently, the absence of a web browsing history
may be a cause for suspicion. Parents and guardians should also
The physician may assist parents and guardians with promoting
regularly monitor IM activity on smartphones.
appropriate internet use by informing them of the benefits and
threats, helping them set guidelines for screen time and content,
and encouraging them to be active participants with their children Strategies for Safer Internet Use
online. Parents or guardians who may not be technically savvy or Although encouraging appropriate use and behavior is important
may require basic instruction on internet use may be advised of to enjoying the benefits of the internet, the physician should
the availability of classes offered by libraries, schools, and commu- also recommend the use of internet safety tools to protect against
nity groups. inappropriate use and external threats.
The American Academy of Pediatrics (AAP) has provided Software to protect malware is not directly targeted to children,
age-specific guidance for the use of digital media, including the but it is something about which parents and guardians should be

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 36 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

aware. Malware generally includes viruses and worms (ie, software Adolescents on the Internet
programs that can corrupt the information saved on a computer),
Most adolescents have access to the internet from home, and many
spyware (ie, software that tracks internet activity and sends this
more may gain access to the internet at school or at a friend’s house.
information to another person), and pop-ups (ie, a new browser
Additionally, 95% of adolescents report that they have access to a
window that may contain marketing or other undesired content).
smartphone, and 45% report that they have a nearly continuous
Viruses and worms are generally downloaded as attachments in
online presence. Parents and guardians should be aware that
email, whereas spyware and pop-ups are introduced by surfing
adolescents who have excessively restrictive internet rules at home
the Web. Attempts should be made to protect against spam (ie,
are more likely to attempt internet access outside the home, where
unsolicited email) as well, which may contain undesired content
it may be more difficult to monitor their activity.
or solicitations.
Adolescents have mixed views on the effect of the internet, specif-
Filters are special computer programs that allow the presentation
ically social media, on their lives. Approximately 45% of adolescents
of acceptable internet content and that block content deemed to be
believe that social media has neither a positive nor a negative effect,
inappropriate. Filtering may use 1 or more of the following meth-
approximately 30% describe its effect as mostly positive, and nearly
ods: “blacklists” of websites that are specifically blocked; “white lists”
25% describe its effect as mostly negative. Open parent-/guardian-
of websites that are specifically permitted; the blocking of specific
child communication about internet use should be encouraged
words or terms; and the maturing technology of blocking suspicious
so that adolescents feel that they can discuss what they see on the
image content. Filters are neither perfectly specific nor perfectly
internet with their parent or guardian without jeopardizing their
sensitive and may require adjustment by the parent or guardian
internet access privileges.
to achieve an acceptable level of filtering. Parents and guardians
Adolescents may engage in risky online behaviors, including
may contact their ISP to enable server-side filtering (ie, filtering
communicating with and planning to meet strangers in person.
of content before it enters the home). If server-side filtering is too
Based on review of past cases, the Federal Bureau of Investigation
restrictive for some members of the household or not sufficiently
has identified specific behaviors that may indicate a child is engaging
restrictive for others, a client-side filtering approach may be consid-
in risky online behaviors, including spending several hours online,
ered, in which filtering software is installed directly on computers
especially at night; having pornographic images on the computer;
or mobile devices. Client-side filters work with standard web brows-
turning the monitor off or quickly changing the screen when a par-
ers, although special child-oriented web browsers may be acquired
ent or other caregiver enters the room; and using unrecognized
that have client-side filtering built in.
user names or accounts. Other offline behaviors that should arouse
When a child encounters inappropriate internet content or
suspicion include telephone calls from unknown adults, outgoing
messages that are hurtful or distressing, parents and guard-
calls to unrecognized telephone numbers, gifts or packages received,
ians should alert their ISP as well as the owner of the website on
and unexplained credit card activity.
which the content was or messages were discovered. As appro-
The physician should consider addressing online activities
priate, the parent or guardian should also contact the appropriate
in the adolescent psychosocial review of systems. Asking adoles-
legal authorities and the National Center for Missing & Exploited
cents about online activities may unveil risky online behaviors or
Children (www.missingkids.com). Commercial websites that col-
provide an opportunity to discuss concerns about their health and
lect personal information from children younger than 13 years
well-being. Nearly one-third of adolescents have searched the inter-
are required to follow the Children’s Online Privacy Protection
net for health information, often related to sex and sexually transmit-
Act of 1998, which is enforced by the Federal Trade Commission.
ted infections, nutrition, and exercise and fitness. Adolescent girls
According to this law, internet website operators must post their
in particular have a tendency to search for information on physical
policy indicating what personal information is collected, how that
abuse, sexual abuse, and dating violence as well. Online activities
information will be used, and if that information will be shared
worthy of inquiry include health topics searched for on the inter-
with a third party. Parents or guardians must consent to the col-
net, which social networking accounts the adolescent has, sharing
lection and use of personal information and may revoke this con-
personal information on the internet, communicating with strang-
sent at any time.
ers, meeting people known only via the internet, and engaging in
Additional internet safety information is available to families
(or being a victim of) cyberbullying or sexting. Adolescents should
through reputable web-based resources, such as the Federal Trade
also be reminded that the internet is not private and that colleges
Commission resource OnGuardOnline (www.onguardonline.gov),
and employers may discover information about the adolescent on
the National Center for Missing & Exploited Children’s NetSmartz
what appear to be “private” social networking pages.
Workshop (www.netsmartz.org), iKeepSafe (www.ikeepsafe.org),
and INOBTR (“I Know Better”) (www.inobtr.org). Parents and
guardians should also be aware of other venues in which their chil- Health Information on the Internet
dren may access the internet, such as schools, libraries, and friends’ Patients regard physicians as the preferred and most trusted source
homes, and they should find out what internet safety policies and for health information; however, patients increasingly seek out
technologies have been instituted in these environments. health information on the internet before, or sometimes in lieu of,

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 Chapter 7: Counseling Families About Internet Use 37

consulting with their physician. It is important to remind families Selected References

that health information on the internet may be outdated, incom-
plete, incorrect, intentionally misleading, or easily misinterpreted. American Academy of Pediatrics Council on Communications and Media. Media
use in school-aged children and adolescents. Pediatrics. 2016;138(5):e20162592
When a patient has questions about information found on the
PMID: 27940794 https://doi.org/10.1542/peds.2016-2592
internet, the physician should take care not to disregard the infor-
Chung JE. Patient-provider discussion of online health information: results
mation outright. These inquiries provide opportunities to educate
from the 2007 Health Information National Trends Survey (HINTS). J Health
the patient and address issues of concern, and the patient may value Commun. 2013;18(6):627–648 PMID: 23590202 https://doi.org/10.1080/
the physician’s opinion of the information. 10810730.2012.743628
The physician may support patients by suggesting evidence-based DeMartini TL, Beck AF, Klein MD, Kahn RS. Access to digital technol-
health information resources on the internet, such as the AAP’s offi- ogy among families coming to urban pediatric primary care clinics.
cial website for parents (www.HealthyChildren.org); the US National Pediatrics. 2013;132(1):e142–e148 PMID: 23753100 https://doi.org/10.1542/
Library of Medicine website MedlinePlus (www.medlineplus.gov); the peds.2013-0594
Centers for Disease Control and Prevention (www.cdc.gov); and the Hamm MP, Newton AS, Chisholm A, et al. Prevalence and effect of cyberbul-
US Department of Health and Human Services (https://healthfinder. lying on children and young people: a scoping review of social media studies.
gov). The specialist should be prepared to identify online information JAMA Pediatr. 2015;169(8):770–777 PMID: 26098362 https://doi.org/10.1001/
sources and support groups, particularly for rare, chronic, or debil- jamapediatrics.2015.0944
itating conditions. The physician should caution families caring for Livingstone S, Smith PK. Annual research review: Harms experienced by child
children with special medical conditions about the characteristics of users of online and mobile technologies: the nature, prevalence and manage-
ment of sexual and aggressive risks in the digital age. J Child Psychol Psychiatry.
less reliable online resources or support groups, which may include
2014;55(6):635–654 PMID: 24438579 https://doi.org/10.1111/jcpp.12197
novel or alternative treatment regimens, advice to stop treatment, or
Moreno MA, Egan KG, Bare K, Young HN, Cox ED. Internet safety education
charging of fees for participation or treatment.
for youth: stakeholder perspectives. BMC Public Health. 2013;13(1):543 PMID:
23738647 https://doi.org/10.1186/1471-2458-13-543
Park E, Kwon M. Health-related internet use by children and adolescents:
CASE RESOLUTION systematic review. J Med Internet Res. 2018;20(4):e120 PMID: 29615385
The pediatrician learns that the adolescent uses her laptop computer for 1 https://doi.org/10.2196/jmir.7731
to 2 hours every afternoon to complete her homework assignments and is on Pew Research Center. Teens, social media & technology 2018. Pew Research
the internet for an additional 2 to 3 hours every evening streaming videos on Center website. www.pewinternet.org/2018/05/31/teens-social-media-technology-
YouTube, posting images and thoughts to her Instagram and Snapchat accounts, 2018/. Accessed August 6, 2019
and occasionally checking her Facebook page. The pediatrician advises the girl Reid Chassiakos YL, Radesky J, Christakis D, Moreno MA, Cross C; American
that, for safety reasons, personal information should never be shared over the
Academy of Pediatrics Council on Communications and Media. Children
internet, adding that colleges and employers often research prospective candi-
and adolescents and digital media. Pediatrics. 2016;138(5):e20162593 PMID:
dates online. The pediatrician also asks whether the patient has received, wit-
27940795 https://doi.org/10.1542/peds.2016-2593
nessed, or participated in hurtful or distressing communications or imagery
online. With both the mother and daughter present, the pediatrician discusses Straker LM, Howie EK. Young children and screen time: it is time to consider
the option of drafting a parent-child contract that clearly establishes the rules for healthy bodies as well as healthy minds. J Dev Behav Pediatr. 2016;37(3):265
acceptable internet use in and out of the home, including appropriate time lim- PMID: 26825042 https://doi.org/10.1097/DBP.0000000000000265
its. The pediatrician also directs the mother to internet-based resources for safe- Wolak J, Mitchell K, Finkelhor D. Unwanted and wanted exposure to online
guarding the home environment and mobile devices, as well as a workshop at pornography in a national sample of youth internet users. Pediatrics.
the local library on internet security. 2007;119(2):247–257 PMID: 17272613 https://doi.org/10.1542/peds.2006-1891

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 CHAPTER 8

Cultural Competency
Issues in Pediatrics
W. Suzanne Eidson-Ton, MD, MS; Hendry Ton, MD, MS;
Blanca Solis, MD; and Jesse Joad, MD, MS, FAAP

CASE STUDY
You are seeing AJ, a 12-year-old Mexican American boy, As is your practice with all adolescents, you ask
for a well-child visit. His mother speaks Spanish and “a to speak with AJ alone. During your assessment, you
little” English, is single, and works full time in motel learn that he is attracted to boys but has not shared this
custodial services. After school and during summers, AJ information with anyone. He is certain that his brother
is cared for by his 17-year-old brother and his mater- will not approve and that his mother will be heartbro-
nal grandmother, who lives a block away. AJ’s weight ken. He is sometimes teased at school because he is “not
and body mass index are well above the 95th percentile tough enough,” and he fears some of the bigger bullies
for his age. When discussing his diet, you learn that might try to jump him if he hangs around after school to
his mother buys packaged foods that he can make for participate in any after-school sports activities.
himself when she is away. She is concerned that he
will not eat if she does not buy the processed, fatty
Questions
1. What is the definition of culture?
foods he likes. Additionally, these types of foods are
2. What is cultural competence? What is cultural
more plentiful than healthier options at the local mar-
destructiveness?
ket at which she shops. AJ sometimes eats at his grand-
3. What is meant by unconscious bias?
mother’s home, but she is elderly and does not cook
4. Why is it important to use a certified interpreter
much anymore. When discussing physical activity, AJ
when talking to the parent with limited English
states that he wants to play soccer. His mother is con-
proficiency? When is it appropriate for the pediatric
cerned about this, however, because he often complains
patient to interpret for their parent?
of headaches and stomachaches when it is time for prac-
5. How does understanding the perspective of the patient
tice, and she does not want to buy the equipment if he
and the parent affect medical decision making?
will quit after a few weeks, as has happened in the past.

The provision of culturally competent care is no less important for of learned behaviors and perspectives that serve as a template
pediatric patients than adult patients. Those who provide health care to shape and orient future behaviors and perspectives from
to children face many important issues concerning culturally com- generation to generation and as novel situations emerge. It
petent care, including health disparities and health care disparities shapes how and what symptoms are expressed and influences
based on socioeconomic status, ethnicity and/or racial identity, sex- the meaning that individuals attribute to symptoms, including
ual orientation, and gender identity. Many issues of cultural compe- one’s beliefs about the causes, effects, and potential remedies
tency, health disparities, and approaches to cross-cultural care are for these symptoms. Culture is a broad category that includes
similar across minority populations in the United States, including not only race and ethnicity but also sexual orientation, gender
some rural white populations. roles, gender identity, socioeconomic status, nationality, and
other group affiliations. The interaction between the culture of
Definition of Culture the patient and family and that of the physician is often signif-
Culture is a set of meanings, norms, beliefs, and values shared icant and can result in bias in both assessment and treatment.
by a group of people. It is dynamic and evolves over time and These biases, in turn, can contribute to health and health care
with each successive generation. Culture encompasses a body disparities.

39

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 40 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Health Disparities obesity or overweight. Similar findings have been observed in

other populations (eg, Americans of Asian descent). Acculturation
Health disparities are differences in health outcomes across different
across generations in Hispanic communities has been associ-
groups. Health care disparities, as defined by the Institute of Medicine
ated with decreased fruit and vegetable consumption, increased
(now the Health and Medicine division of the National Academies)
soda consumption, and decreased physical activity. These effects
in the document Unequal Treatment: Confronting Racial and Ethnic
of acculturation have been shown in other immigrant groups as
Disparities in Health Care, are “racial or ethnic differences in the quality
well. Acculturation is a complex variable in relation to pediatric
of health care that are not due to access-related factors or clinical needs,
obesity, however. For example, in 1 study, Mexican American
preferences, and appropriateness of intervention.” Health disparities
mothers who were less acculturated had children with higher body
and health care disparities exist within the United States and particu-
mass indices.
larly affect minority populations. Although health disparities may be
caused by many social determinants of health, such as socioeconomic
status and access to safe neighborhoods, healthy food, and health care, Cultural Competence
health care disparities are directly related to what happens to cer- Physicians who strive for cultural competence attempt to minimize
tain patients in the health care system after they have accessed care. their bias and seek to incorporate cultural assets into their work with
Addressing health disparities moves medicine and society closer to patients. Cultural competence is a continuum (Figure 8.1). Cultural
health equity, which is achieved, as defined by Braveman et al, “when destructiveness, the least developed state along that continuum,
everyone has a fair and just opportunity to be as healthy as possible.” comprises attitudes and practices that are meant to be harmful to
Latinx is a gender-neutral term that refers to individuals of Latin cultures and individuals within the particular culture. Physicians
American heritage. Latinx children in the United States face signifi- at this level may overtly discriminate against individuals based
cant health and health care disparities. (Throughout this chapter the on their culture (eg, intentionally making homophobic, racist, or
term Hispanic is used for information reported from any study in sexist remarks to colleagues). Physicians who have cultural inca-
which that term was used.) The Hispanic population has among the pacity do not overtly discriminate but lack the ability and willing-
highest prevalence of overweight in children, but American Indian/ ness to recognize and intervene when discrimination happens. As
Alaska Native children having the highest rates of overweight, at a result, they ultimately reinforce culturally oppressive behaviors
nearly one-third of that population. Additionally, in the United States and policies. In the cultural blindness phase, an individual believes
Hispanic children also have a lower fitness level and watch more that culture makes no difference and that all people are the same.
television than white children. The reasons behind these dispar- This viewpoint ignores cultural strengths, encourages assimilation,
ities are complex and multifactorial. Many ethnic minorities and and often results in blaming the victims of racial injustices for their
limited English proficiency (LEP) groups also face disparities in problems. Physicians at this stage may have difficulty believing
the health care they receive. For example, for minority groups in the validity of health disparities, despite the enormous body of
the duration of their routine well-child visits is shorter and Latinx evidence supporting the existence of such disparities. Although
children are less likely than white children to receive counseling at these physicians strive to give quality care to everyone, they do not
these visits. recognize culture-specific experiences that affect patients’ health,
In addition to these examples of lack of access to quality health such as poverty or violence based on race, sexual orientation, or
care, many social, political, and cultural elements contribute to
health disparities observed in certain ethnic groups. Evidence exists
to indicate that these social determinants have a more significant
effect on health than medical care does (see Chapter 141). Although Cultural
physicians can effectively address the social determinants of health proficiency

through activism and advocacy, it may be difficult to do so on Cultural

an individual patient care level. Nevertheless, it is important to competence

understand these issues and how they may contribute to patients’ Cultural
health outcomes. For example, lack of access to quality, low-cost precompetence

foods in one’s neighborhood, lack of transportation, advertis- Cultural

ing practices that target low-income communities, and lack of sensitivity

access to safe playgrounds are examples of social determinants Cultural

that contribute to obesity. It is also essential to understand the blindness

influence of culture on patients’ health beliefs, health behavior, Cultural

and health status. Cultural issues are further discussed later in incapacity

this chapter. Cultural

Finally, acculturation is also likely to affect health, health beliefs, destructiveness
and health behaviors. Second-generation Hispanic adolescents
are twice as likely as first-generation Hispanic adolescents to have Figure 8.1. Steps in achieving cultural proficiency.

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 Chapter 8: Cultural Competency Issues in Pediatrics 41

gender, and they miss opportunities to incorporate culturally

Box 8.1. Strategies for Using an Interpreter
relevant strengths into treatment, such as collaboration with tradi-
in the Clinical Visit
tional healers, extended family, or faith-based organizations. Cultural
precompetence is characterized by a willingness to deliver quality ww Talk with the interpreter briefly before the interview to discuss overall
services and a commitment to civil rights but a lack of knowledge goals for the interview and ground rules (eg, use word-for-word
and experience to implement culturally relevant services. Culturally interpretation). If using a certified medical interpreter via telephone,
pre-competent physicians often struggle with knowledge that dispar- have a brief discussion about the goals for the interview.
ities exist and that perhaps they continue to contribute to them but ww Give the interpreter and the patient permission to ask questions about
are at a loss as to what to do about it. In contrast, physicians at the terminology with which they may be unfamiliar.
stage of cultural competence seek to expand their cultural knowledge ww Use nonverbal behaviors, such as eye contact, nodding, and facial
and resources, often in consultation with culturally diverse com- expressions, to signal to the patient that the physician understands what
munities. They also continuously self-assess and adapt traditional is being communicated.
service models to enhance care for culturally diverse patients. Using ww Speak directly to the patient and address the patient in the first person.
native culture-based talking circles with American Indian youth to ww Use short, simple statements and speak on 1 topic at a time.
improve their understanding of obesity is an example of working at ww Avoid medical jargon and ambiguous statements.
this stage of cultural competence. Finally, physicians who exhibit ww Meet briefly with the interpreter after the interview to confirm pertinent
cultural proficiency hold cultural diversity in highest esteem and information, ask about cultural information, and discuss feedback.
continuously advocate for increased cultural competence through- Do this with telephone interpreters as well.
out the system of care. It is important to recognize that physicians ww Remember that interpreters are not medical professionals. Therefore,
move back and forth along this continuum dynamically, influenced consider information volunteered by interpreters as being reliable as
by their own experiences. For example, physicians who identify with collateral information provided by other individuals who are not health
being in the culturally competent phase when working with Latinx professionals.
patients may find themselves having cultural incapacity when work-
ing with gay or lesbian patients.
Another helpful model is the concept of cultural humility, Unconscious Bias
which is a process of self-reflection and commitment to a lifelong
In addition to ensuring effective communication, it is important
learning process, thereby engaging the physician in an ongoing cou-
for the physician to address the unconscious biases that the physi-
rageous and honest process of self-critique and self-awareness. Both
cian may have about a patient. Although few health professionals
the cultural competence model and the cultural humility model
intentionally discriminate based on culture, studies suggest that
reflect cultural competency as a process rather than an end result
physicians are likely to have unconscious biases that can signifi-
or a fixed state.
cantly affect patient care.
Physicians can take steps to manage unconscious bias. It is
Communication essential to first acknowledge that bias does exist in the health care
Initially, during every clinical visit, it is important to maximize com- system and within the lived experience of patients as well as phy-
munication with the patient and the parent (see Chapters 2, 3, and 4). sicians. It is good practice for health professionals to explore the
Effective communication results in an improved physician- Project Implicit website (https://implicit.harvard.edu/implicit) and
patient relationship, treatment adherence, and health outcomes. take the online tests designed to detect bias across various cultural
Poor communication may have negative consequences. Patients dimensions, including race, sex, gender, and sexual orientation. Most
with LEP, in particular, have increased barriers to health care, people who take these tests quickly realize that they carry biases they
decreased health quality, and decreased health status. In a Joint had no idea existed within themselves. For many, acknowledging
Commission study involving 6 hospitals, researchers found that their own biases may be the most difficult step. The next step is to
patients with LEP were at increased risk for iatrogenic harm. In work toward replacing one’s own biases with positive and realistic
contrast, use of interpreters is associated with increased follow- associations about other groups. For example, seeking out dialogue
up with preventive and primary care services. Ideally, a trained and companionship with people culturally different from oneself can
medical interpreter should be available to communicate with help replace one’s negative assumptions with more positive associa-
patients or parents who do not speak English. Although it hap- tions. Finally, it is important to be mindful of the potential influence
pens often in clinical practice, it is inappropriate to rely on of unconscious bias on medical decision making, whether treat-
the patient to interpret for the parent. It is clearly a difficult position ing a patient of similar background to the physician or a patient of
for a child or adolescent to interpret health information for a par- a quite different background. It is essential to consider cultural
ent. It is not guaranteed that the patient understands what the phy- context; however, it is also important to be aware that each patient
sician is communicating or that the patient is interpreting correctly is a unique individual. In any situation in which bias may be pres-
for the parent. It also disturbs the power balance between parent ent, the physician should speak less and listen more, striving to avoid
and child. Strategies for using interpreters are provided in Box 8.1. assumptions.

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 42 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

Eliciting Patients’ Perspectives perspectives are addressed. The questions in Box 8.2 can help
a physician further understand the health beliefs of the patient
Optimization of clinical collaboration is dependent on each
and family.
stakeholder—patient, family, and physician—having an oppor-
tunity to express their understanding of the health issue
Decision Making
in question. Typically, however, the physician obtains the
patient history with limited consideration of the patient’s and It is also important for the physician to understand how the patient
family’s beliefs about the health problem. Difficulties may arise or family makes medical decisions. Many physicians look to a child’s
if the patient or a family member disagrees with the physician’s parent or guardian as the primary decision maker. This process
explanations of the illness. In many cultures, openly disagree- may be culturally influenced, however. Parents or guardians from
ing with a physician’s assessment and treatment recommen- strongly collectivistic cultures, in which the needs and priorities of
dations is considered disrespectful. Instead, the patient and the group supersede those of the individual, may wish to involve
family may acknowledge the physician’s status by expressing agree- others (eg, senior family members, spiritual leaders, clan leaders)
ment but maintain their own ideas about how to address the issue. in health care decision making. Failure on the part of the physician
For example, although a parent may report the intention to fol- to allow the inclusion of these persons in the decision making
low through with the physician’s recommendation to change to a process may result in the parent or guardian feeling forced into a
healthier diet to improve the health of the child, the parent may con- decision, may precipitate conflict within the family, and may result
tinue to purchase less healthy ready-made foods because of real or in the family or community blaming the parent or guardian should
perceived difficulties getting healthier foods given the parent’s a bad outcome occur. Suggested questions for facilitating a discus-
long work hours and the lack of healthy food in the family’s neigh- sion about decision making are presented in Box 8.2.
borhood. The physician may characterize obesity as a primarily
biologic problem, whereas the parent may view the condition pri- Sexual Orientation and Gender
marily as a problem of access and time. Identity
Furthermore, the parent may not perceive the child’s overweight At any clinic visit, it is extremely important for the physician
status as a problem at all. The relationship of pediatric obesity to to remain nonjudgmental and be supportive of the patient’s
cultural ideals and norms around food, eating, and parenting is sexual orientation and gender identity development. (See
complex. Some evidence in ethnographic studies shows that,
in some Latinx cultures, feeding children and especially giv-
ing “treats” is a means of showing love. A parent or guardian Box 8.2. What to Ask
with a child who is a so-called healthy eater may not want to
limit the child’s enjoyment of food. Additionally, body size ide- General Questions
als vary across communities, and some evidence indicates that ww About which problem or problems are you most concerned?
in some Latinx communities children who meet criteria for over- ww What do you think has caused the problem?
weight are considered more attractive and healthier than thinner ww What are your concerns about this problem? How does it affect you and
children, who may be considered frail. Poverty and the experi- your child?
ence of food scarcity can further exacerbate overeating dur- ww What treatments do you think might work for it?
ing times when food is more accessible. Finally, particularly ww What have you done in the past? How satisfied were you with the results?
when other friends or family members offer food to a child, it ww What are your goals for treatment?
may be considered socially unacceptable for the parent to refuse ww What is your understanding of the treatment offered? What are your
or criticize the type of food being offered. Understanding such concerns about it? What are the barriers for completing or carrying out
cultural norms can be helpful in treating individual patients. It is the treatment?
important to remember, however, that particular individuals and Health Issues Identified by the Physician
families have their own cultural norms that may or may not be ww What do you think about the concerns that I have raised with you (ie, the
consistent with others in their ethnic group. problems the physician has identified [eg, obesity])?
Conflicts in health beliefs between the physician and the ww Have you been told about this concern before? If so, what is your
patient and/or family may result in treatment nonadherence and understanding of it?
dropout. It is important that the physician and the patient and/or Decision Making
family discuss their respective health perspectives. The better the ww How do you usually make decisions about health care?
physician’s understanding of the health beliefs of the patient and ww Do you involve anyone else in that process? Who? What are their roles?
family, the more effectively the physician can address differences ww Who usually makes the final decision?
and build on common ground. Likewise, patients and families ww How comfortable are you with using that process to make decisions
may feel a greater level of comfort with a physician’s recommen- about your child’s current situation?
dations if their questions and concerns about the physician’s

BPPCA6e_Ch008_0039-0044.indd 42 2/13/20 5:06 PM

 Chapter 8: Cultural Competency Issues in Pediatrics 43

Chapters 57 and 150, respectively, for suggestions on providing

culturally competent care around sexual orientation and gen- CASE RESOLUTION
der identity and on addressing bullying.) Two helpful resources While speaking with AJ alone, you express support for his early sexual identity
are the Fenway Institute National LGBT Health Education development, identify safe adults in his life with whom he can discuss his
Center (www.lgbthealtheducation.org/lgbt-education/learning- feelings and concerns, and identify community resources for LGBTQ+ individuals.
modules/) and the Family Acceptance Project (https://family- When AJ’s mother returns to the room, you also discuss AJ’s and his mother’s
perspectives about food, his weight, and his activity level, using a medically
project.sfsu.edu).
trained interpreter to ensure clear communication with his mother. You iden-
As the result of inequitable treatment, sexual minority indi- tify some of this family’s strengths: AJ’s mother’s desire to help him be healthier;
viduals experience significant health disparities. Discrimination, AJ’s relationship with his grandmother, who cooks healthy traditional foods; and
bullying, and abuse can result in mental health issues, such as AJ’s supportive relationship with his older brother. After discussion, the patient
depressive disorders, posttraumatic stress disorder, suicidal- decides that he would like to learn to cook from his grandmother and will commit
to cooking a family dinner once a week, which delights his mother. His brother
ity, and substance use. Individuals who are lesbian, gay, bisex-
has also agreed to take him to soccer practice and games for a neighborhood
ual, transgender, or questioning (LGBTQ+) also have more soccer team. AJ likes this idea, because he feels safe from bullies with his brother
barriers to access health services than heterosexual cisgender present and feels that soccer will help him be stronger and more confident. You
individuals, resulting in lower rates of screening and preven- arrange for a follow-up visit in several months to check in with AJ and his mother
tive services. (A cisgender person is one whose gender iden- about their progress and to brainstorm about further interventions. This will
tity or expression matches the cultural norm for the person’s include 1-on-1 time with AJ and further discussion of his sexuality.
biologic sex.) Youth who are LGBTQ+ are also more likely
to face family alienation and homelessness. Disclosure of
sexual orientation and physician attitude are important medi- Selected References
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however, 90% of youth in 1 study reported having reserva- And What Difference Does a Definition Make? Princeton, NJ: Robert Wood
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importance of physician training on facilitating these impor- Braveman P, Gottlieb L. The social determinants of health: it’s time to consider
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Longitudinal Study of Adolescent Health, in which 20,000 ado- ture on childhood obesity: implications for prevention and treatment: a consen-
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was significant compared with white and Asian peers. Additionally, Point. 1988;3(1):1–9 https://www.pathwaysrtc.pdx.edu/pdf/fpF88.pdf Accessed
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to be victims of violence. These findings show that issues of sexual Elm JHL, Lewis JP, Walters KL, Self JM. “I’m in this world for a reason”: resilience
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J Lesbian Stud. 2016;20(3-4):352–371 PMID: 27254761 https://doi.org/10.1080/
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Flores G, Abreu M, Tomany-Korman SC. Limited English proficiency,
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primary language at home, and disparities in children’s health care: how language
tation in the context of the patient’s family and ethnic commu- barriers are measured matters. Public Health Rep. 2005;120(4):418–430 PMID:
nity. For example, in some traditional communities, such as some 16025722 https://doi.org/10.1177/003335490512000409
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ered to possess certain spiritual gifts. Although LGBTQ+ youth in 2010;125(4):e979–e1020. Reaffirmed May 2013 PMID: 20351000 https://doi.
Latinx families do face challenges, particularly in situations in which org/10.1542/peds.2010-0188
antigay religious views are of strong influence, strong cultural iden- Gaw AC. Concise Guide to Cross-Cultural Psychiatry. Washington, DC: American
tity can serve as a grounding influence for LGBTQ+ individuals. For Psychiatric Association Publishing; 2001
example, in 1 study, young urban Hispanic men who have sex with Green AR, Carney DR, Pallin DJ, et al. Implicit bias among physicians and its pre-
men were less likely to engage in risky sexual behavior if they were diction of thrombolysis decisions for black and white patients. J Gen Intern Med.
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Hafeez H, Zeshan M, Tahir MA, Jahan N, Naveed S. Health care disparities
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Institute of Medicine. The Health of Lesbian, Gay, Bisexual, and Transgender Health. J Nutr. 1998;128(4):701–706 PMID: 9521631 https://doi.org/10.1093/
People: Building a Foundation for Better Understanding. Washington, DC: jn/128.4.701
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third generation U.S. immigrants: the National Longitudinal Study of Adolescent S1526-9523(02)00218-0

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 CHAPTER 9

Global Child Health

Suzinne Pak-Gorstein, MD, PhD, MPH, FAAP, and Maneesh Batra, MD, MPH

CASE STUDY
You are watching television when the programming is Questions
interrupted by breaking news that a severe earthquake 1. What are the global trends in childhood disease and
has struck a developing country you have recently mortality? How does this compare with the United
visited. You wonder if and how you could become States?
involved in efforts to help the country respond to 2. What is global health?
the disaster, prevent diseases, and rebuild its health 3. What is the role of the pediatrician in global health?
care infrastructure. 4. What are the key organizations in global health with
which pediatricians work?
5. How can the pediatrician carry out international
work in an ethical and effective manner?
6. What are useful global health resources?

Background of Africa and Asia combined account for 86% of all child deaths, with
one-third of these deaths occurring in South Asia and half in sub-
Worldwide, an estimated 5.3 million children younger than 5 years
Saharan Africa. Less than 1% of deaths occur in high-income
of age died in 2018, for an average of nearly 15,000 children dying
countries.
each day (Figure 9.1). This represents a 58% reduction from the
Nearly one-third of all child deaths worldwide were caused by
12.6 million deaths of children younger than 5 years of age estimated
3 communicable diseases: pneumonia (16%), diarrhea (8%), and
in 1990. Even so, significant disparities in child mortality persist and
malaria (5%). Most of these lives could be saved through increased
have become increasingly concentrated geographically, with specific
access to low-cost prevention and treatment measures, including
regions of the world bearing a disproportionate burden. The continents

Under-five mortality rate

(deaths per 1,000 live births)

>100
75 to 100
40 to 75
25 to 40
10 to 25
Ä10
No data

Figure 9.1. Mortality rates for children younger than 5 years in 2018.
Reprinted with permission from United Nations Inter-agency Group for Child Mortality Estimation (UN IGME). Levels & Trends in Child Mortality: Report 2019. Estimates
Developed by the UN Inter-agency Group for Child Mortality Estimation. New York, NY: United Nations Children’s Fund; 2019.
45

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 46 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

antibiotics for acute respiratory infections, oral rehydration ther-

Box 9.1. United Nations Sustainable
apy for diarrhea, immunizations to protect against pneumococcal
Development Goalsa
pneumonia and diarrhea caused by rotavirus gastroenteritis, and
the use of insecticide-treated mosquito nets and appropriate drugs 1. End poverty in all its forms everywhere
for malaria. 2. End hunger, achieve food security and improved nutrition and
Undernutrition is an underlying cause of at least one-third of promote sustainable agriculture
all deaths in children younger than 5 years. Reducing chronic and 3. Ensure healthy lives and promote well-being for all at all ages
acute undernutrition would have a substantial effect on reducing 4. Ensure inclusive and equitable quality education and promote lifelong
child mortality. Furthermore, improved coverage of specific nutri- learning opportunities for all
tional interventions, such as early and exclusive breastfeeding, are 5. Achieve gender equality and empower all women and girls
cost-effective and reduce the prevalence of pneumonia and diarrhea. 6. Ensure availability and sustainable management of water and
The first month after birth is a perilous phase for a child’s sanitation for all
survival, with an estimated 47% of deaths in children younger than 7. Ensure access to affordable, reliable, sustainable and modern energy
5 years occurring during this period. Most of these deaths occur at for all
home in the first postnatal week as the result of preterm birth (18%); 8. Promote sustained, inclusive and sustainable economic growth, full and
intrapartum-related complications, such as birth asphyxia (12%); productive employment and decent work for all
and neonatal sepsis (7%). Thus, improving neonatal care is essen- 9. Build resilient infrastructure, promote inclusive and sustainable
tial to the goal of improving child health. Increased access to basic, industrialization and foster innovation
inexpensive interventions is necessary to reduce neonatal mortality 10. Reduce inequality within and among countries
rates globally, including delivery by skilled birth attendants, hygienic 11. Make cities and human settlements inclusive, safe, resilient and
umbilical cord care, and the training of community health workers sustainable
to assess and begin early treatment for neonatal infections. 12. Ensure sustainable consumption and production patterns
Mortality among young children aged 5 through 14 years is 13. Take urgent action to combat climate change and its impacts
lower than that of children younger than 5 years (7.2 deaths per 14. Conserve and sustainably use the oceans, seas and marine resources for
1,000 live births and 39 deaths per 1,000 live births, respectively). sustainable development
Decreases in mortality for these older children and adolescents 15. Protect, restore and promote sustainable use of terrestrial ecosys-
have slowed since the year 2000, however. In 2017 an estimated tems, sustainably manage forests, combat desertification, and halt and
0.9 million children 5 through 14 years died, which represents reverse land degradation and halt biodiversity loss
approximately 2,465 deaths each day. Most deaths among these 16. Promote peaceful and inclusive societies for sustainable development,
children occurred in sub-Saharan Africa (54%) and Southern Asia provide access to justice for all and build effective, accountable and
(25%). Injuries accounted for a larger proportion of deaths (30%) inclusive institutions at all levels
among these older children than among children younger than 17. Strengthen the means of implementation and revitalize the global
5 years, with drowning and road injuries causing 14% of deaths in partnership for sustainable development
the older age group. Leading causes of death among adolescents ages
10 through 19 years include road injury, HIV, suicide, lower respira-
a
These goals were developed and signed by all United Nations member countries in 2015. There are
17 goals with 169 targets (with 1 specific goal for health that includes 13 health goal targets) and a
tory infections, and interpersonal violence.
series of measurable indicators for each target.

Sustainable Development Goals

The third SDG (SDG-3) specifically focuses on population
In 2015, world leaders agreed to the 17 Sustainable Development health, to “ensure healthy lives and promote well-being for all at all
Goals (SDGs) for improving global well-being by 2030. The SDGs ages.” Although the MDGs included targeted relative reductions in
were built on lessons learned from the 8 Millennium Development mortality (eg, reduce under-5 mortality by two-thirds), the SDG-3
Goals (MDGs), which were specific, measurable targets set by the sets absolute targets to end preventable deaths of neonates in
United Nations in 2000 to eradicate poverty, hunger, illiteracy, and particular and other children younger than 5 years of age. Specifically,
disease by 2015. Progress toward meeting the MDGs has been sig- these more equitable goals call on all countries to reduce neonatal
nificant, although uneven, with the poorest and most disadvantaged mortality to no more than 12 deaths per 1,000 live births and under-5
countries as well as marginalized communities and social groups mortality to no more than 25 deaths per 1,000 live births by 2030.
within developed countries not attaining these goals.
The SDGs aim to address the underlying determinants of Inequities in Health
poverty and poor health in a sustainable manner by expanding Significant disparities exist in child health. Sub-Saharan Africa grap-
its goals to address the systems-based, underlying socioeco- ples with a high child mortality rate (ie, 76 per 1,000 live births in
nomic, political, and environmental factors. The 17 interconnected 2017); that is, 1 in 13 children in that region dies before 5 years
SDGs are comprehensive and apply universally to all nations, both of age. In comparison, Western European countries have an under-
developed and developing (Box 9.1). 5-year mortality rate of 4 deaths per 1,000 live births, and in

BPPCA6e_Ch009_0045-0050.indd 46 2/13/20 5:06 PM

 Chapter 9: Global Child Health 47

high-income countries fewer than 5 per 1,000 children die before global and local health disparities, and their collective voice has been
their fifth birthday. powerful. Global health work varies widely in scope and extent. The
In 2017, an estimated 82% of all the growth in global wealth went to duration of GH activities ranges from single short-term medical
the top 1%, whereas the bottom 50% saw no increase at all. The SDG-10 missions to long-term postings in resource-limited settings.
calls for reducing inequalities in income as well as those based on age, Involvement in GH encompasses the direct provision of clinical
sex, disability, race, ethnicity, religion, or other status within a coun- services, technical assistance for program development, research,
try. Large inequalities in child health exist between, as well as within, education and training of health workers, and governmental advo-
countries. For example, in Bolivia and Peru, the richest one-fifth of the cacy for policy changes. The goals of GH activities range from forging
population has almost universal access to a skilled attendant at birth, novel directions in areas of basic science and epidemiologic, clinical,
compared with only 10% to 15% among the poorest one-fifth. Women and operations research to addressing the needs of the world’s poor-
in poor rural households accounted for two-thirds of unattended births. est communities. Ideally, GH experiences should be transformative
Although the United States spends more on health than any other for the health professionals who engage in these experiences and for
country ($10,348 per capita), it ranks lower than other highly devel- the poor communities of the world that they serve.
oped nations with respect to its under-5 mortality rate (7 deaths By committing to a single international site (eg, hospital, rural
per 1,000 live births), which is greater than rates in most of Europe. clinic, community) and working with a partner based at that site,
Additionally, the United States lags behind other comparable coun- the pediatrician can engage in a longitudinal supportive relation-
tries with worse life expectancy and higher rates of disease bur- ship that is sustainable and effective. Pediatricians can also make a
den as calculated in disability-adjusted life-years. Significant health sustainable impression by empowering in-country partners through
inequities are also apparent within the United States. Black children training of trainers, such as community health workers, supervisors,
age 1 to 4 years have the highest death rates (38.8 per 100,000), fol- and clinicians responsible for health professional trainees.
lowed by Native American children (30.5 per 100,000); Asian/Pacific Pediatricians may engage in GH activities in multiple domains,
Islander children have the lowest death rates (16.5 per 100,000). including patient care, teaching and training, research, and
Much of the differences in health outcome are the result of dispari- advocacy. Pediatricians have also played an important role in
ties in nonmedical social determinants, such as income and educa- responding to humanitarian emergencies in the United States as
tion. Compared with other Western countries, however, the United well as in other countries, such as in the aftermath of devastating
States spends disproportionately more on health care than on social earthquakes and other natural disasters. Additionally, pediatricians
services that could indirectly improve health outcomes. with skills in research and evaluation may contribute to GH through
To attain the SDGs of reducing child mortality, targeted inter- clinical research and program evaluations.
ventions that focus on the poorest populations are needed that could Many GH opportunities do not require an overseas trip. Although
close gaps in intranational health disparities. It has been projected vulnerable populations and health inequities certainly exist in
that policy interventions aimed at reducing country-level inequities low-income countries, significant inequities abound in the United
would have a major effect on the under-5 mortality rate. Worldwide, States. Among developed countries, those with the highest health
had the child mortality rates of all countries been reduced to that with status have the lowest levels of health inequality. The United
the lowest rate (2.1 deaths per 1,000 live births), a total of 5.1 mil- States ranks at the bottom of this list, with 1 of the poorest health
lion deaths of under-5 children could have been prevented in the year rankings and the highest inequalities in health. Opportunities to
2017 alone, which would represent a 95% reduction of child deaths. engage in local GH work are plentiful and include supporting the
The Health and Medicine division of the National Academies care and resettlement of local refugee and immigrant families, sup-
(formerly the Institute of Medicine) definition of global health (GH) porting international adoptees, serving migrant farmworkers, and
encompasses “health problems, issues, and concerns that tran- supporting Native American health issues.
scend national boundaries, and may best be addressed by coop- The pediatrician may also work locally for GH by advocating for
erative actions....” For the World Health Organization, GH involves equity of health care at all levels domestically and globally, work-
health problems that affect global politics and economies and arise ing in the home office of a US-based GH organization, and provid-
from disparities in sociopolitical and economic status. Inequalities in ing expertise to support international organizations dedicated to
health within and between countries arise from inequalities within helping vulnerable children. Finally, pediatricians can make a
and between societies. Consequently, the emerging field of GH significant impact in GH by lobbying the US government for
intersects medical and social science disciplines such as demograph- more international relief funding or supporting corporations with
ics, economics, epidemiology, political economy, and sociology. ethical international trade practices.

Integrating Global Health Into Beyond the Hospitals: Global Health,

Pediatric Careers Community Health, and Advocacy
Children constitute the most vulnerable group in any society, and the Increasing numbers of medical students and residents in the United
strongest medical advocate for the health of children is the pediatri- States seek training opportunities in developing countries, and
cian. Consequently, pediatricians have been leaders in addressing most of these trips are spent in a foreign hospital on a pediatric

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 48 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

ward providing direct clinical care. In addition to observing and

Box 9.2. Examples of Global Health Organizations
managing tropical diseases and more severe forms of commonly
encountered pediatric conditions, visiting physicians and stu- International Health or Multilateral Organizations
dents learn and experience common themes in these low-resource ww World Health Organization (WHO)
settings: underpaid and under-trained health workers, hospital ww United Nations Children’s Fund (UNICEF)
administration untrained in health management, dilapidated facil- Bilateral Government Organizations
ities, basic medicine shortage, lack of tools in testing and imag- ww US Agency for International Development (USAID)
ing, and higher mortality rates. Because the needs at such hospitals ww Centers for Disease Control and Prevention (CDC)
and clinics are glaring, the physician instinctively seeks to fill the ww Peace Corps
gaps with what defines quality health care in the United States—
International Donor Foundations
more medicines, equipment, clinical staffing (in the form of visiting
ww Bill & Melinda Gates Foundation
physicians), and perhaps training. However, the long-term effect
ww Wellcome Trust
of a brief visit, even repeated visits, may be further improved through
ww Children’s Investment Fund Foundation (CIFF)
efforts to prevent disease using public health approaches and
focusing on communities. US-Based Nongovernmental Organizations
With additional training in areas such as public and community ww Partners In Health
health or health service management, the pediatrician may act as an ww Global Health Council
“agent of change” by undertaking systems-based quality improve- ww Save the Children
ment approaches, public health measures, and community-based International Nongovernmental Organizations
strategies to bring about lasting positive effects on child health. Specialized services or training
Box 9.2 lists some key categories of GH organizations. In col- ww Short-term service/training trips (eg, cleft palate repairs)
laboration with community groups, the clinician or student can Emergency relief and rehabilitation
effectively empower community health workers and work within ww Doctors Without Borders (Médecins Sans Frontières)
local community-based nongovernmental organizations (NGOs) to ww International Committee of the Red Cross
contribute to lasting and contextually appropriate change. ww CARE International
Getting a sense of how most of the world lives is a tremen- ww International Rescue Committee (IRC)
dous gift, honor, and burden that accompanies these experiences. Consultant Organizations: For-profit and Not-for-profit
Continuing to advocate for children living in poverty throughout Global health consultant organizations may take government contracts to
the world after returning from a short-term medical experience is a provide international support
great way to enact one’s ongoing responsibility. Additionally, although ww Seed Global Health (formerly Global Health Service Partnership)
perhaps less glamorous than traveling overseas, working within ww FHI 360 (formerly Family Health International)
the US health care and political system to promote awareness for ww John Snow, Inc. (JSI)
change has the potential to catalyze lasting and significant improve- Faith-Based Organizations
ments in child health. Similarly, through work to empower local ww World Vision
refugee communities in the United States or to advocate for more ww Aga Khan Foundation
equitable access to health care for vulnerable immigrant children, ww American Friends Service Committee
pediatricians can make a significant and lasting difference in the
Academic Institutions
efforts to close the gap in health disparities.
An increasing number of academic institutions are developing global health
programs in partnership with international groups, overseas academic
Ethical Issues in Global Health institutions, or ministries of health
The traditional model for medical experiences for US-based ww Consortium of Universities for Global Health (CUGH)
physicians presumes that individuals in the United States possess Local and Domestic
the knowledge, skills, and resources to improve the conditions of ww Global health advocacy organizations
people living in developing countries. Most students and physicians ww Refugee resettlement agencies
who have participated in such an experience, however, report that ww Native American health services
they themselves derive the greatest benefit from such experiences. Resources
Although the desire to improve the conditions of children by ww American Academy of Pediatrics Section on International Child Health
providing clinical care is well-intentioned and altruistic, the poten- (AAP SOICH)
tial exists for short- and long-term harm. In the short term, caring ww Academic Pediatrics Association Global Health Special Interest Group
for children in such settings with conditions that are unfamiliar to ww Physicians for Social Responsibility (PSR)
visiting students or physicians and out of the scope of their training
can result in errors in diagnosis and management. Often, students
have less supervision of their clinical work in these settings, which

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 Chapter 9: Global Child Health 49

Table 9.1. Preparation for Working in Resource-Limited Settings: Themes,

Subcategories, and Examples
Category Themes Subcategories Example of Recommendations for Best Practices
Logistics and safety Personal health Create strategies for risk reduction, including for transportation,
Safety environment, food, risk-taking behaviors, and recreational activi-
Travel logistics ties, and emphasize that local laws (eg, seat belts, restricted areas)
Medical licensure apply to all visitors
Malpractice insurance
Knowledge and skills Medical knowledge Familiarize oneself with local disease patterns, reference materi-
Health systems knowledge als, on-site formularies, and clinical practice guidelines, including
Procedural and practical skills (if applicable) resources available through the World Health Organization
Attitudes and behaviors Personal motivations Recognize that behaviors, skills, and competencies of local providers
Learner humility are culturally based and resource-dependent, and avoid compari-
Cultural humility son with home institutions, superior or judgmental attitudes, and
Understanding of culture shock and reentry shock denunciations of differences in clinical care
Professionalism and behavior
Local resources Clinical resources Familiarize oneself with the host site formulary, laboratory sup-
Human resources plies, radiology capabilities, medical technology, medical transpor-
Needs and assets tation services, patient costs, and patient payment models before
departure to best inform adaptations in diagnostic evaluations and
management
People History, politics, and economics Arrange plans for interpreter services during the visit, if necessary,
Culture and recognize the burdens imposed on local health professionals
Religion when assisting with interpretation
Local health beliefs
Language
Ethics Donations Follow host institution guidelines and accepted international guide-
Research and projects lines about the donations of medications, technology, and supplies
Patient privacy
Patient care with resource limitations
Scope of practice
Supervision
Sustainability
Effect on hosts
Communication Predeparture, on-site, and post-return communications Schedule meeting with returning traveler to assess wellness, pro-
vide an opportunity for debriefing of the experience, obtain input
on the program or partnership strengths and weaknesses, and
determine the effect of the experience on clinical practice and
career plans
Partnerships Choosing opportunities Provide transparent goals and objectives for both visitors and the
Predeparture selection processes sending institutions and ensure that the host institution has an
Clear expectations opportunity to review them and modify them if necessary
Evaluation
Adapted with permission from St Clair NE, Pitt MB, Bakeera-Kitaka S, et al; Global Health Task Force of the American Board of Pediatrics. Global health: preparation for working in resource-limited settings.
Pediatrics. 2017;140(5):e20163783.

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 50 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

can result in harm to patients and students’ growth as physicians. requires building an evaluation process early on that incorporates
In the long term, provision of clinical care by visiting students or the perspectives of all involved, including local officials, health pro-
physicians can undermine the existing health system infrastructure. fessionals, and community members. This includes assessment of
As the number of medical students, trainees, and physi- whether educational objectives are being met for all stakeholders,
cians from the United States who visit developing countries for including the host site.
short-term training experiences continues to grow, concern for
medical tourism and the long-term effect of these training expe-
riences on the under-resourced hosts is rising. As indicated in
CASE RESOLUTION
You learn about a US-based NGO with a long history of partnership and work in
Table 9.1, key factors for ensuring effective, sustainable, and
the earthquake-stricken country. You research that NGO further and learn that it
ethical international collaborations include forethought, planning, is a reputable group with long-term interests in the country. You speak to friends
and long-term partnership. Key guiding principles of effective and who have recently visited the country and learn more about what skills and
sustainable partnerships include equity, inclusivity, sustainabil- resources are needed. You arrange to join a team of experienced health workers
ity, mutual benefit, prevention of adverse effect, social justice, and from the NGO by taking time from work and garnering support from your family
humility. Although most partnerships in GH involve trainees and to manage in your absence. You undergo an in-depth orientation of the site, peo-
ple, sociopolitical situation, team roles, and expected activities through a series
practitioners from a higher-income country visiting a facility in
of discussions with all participants.
a low-income country, everyone may derive greater benefit from
reciprocal exchanges; such bidirectional exchanges impart true
parity within partnerships. Selected References
Proper predeparture preparation and education for any over-
seas medical experience is a critical first step and entails orienta- Arora G, Russ C, Batra M, Butteris SM, Watts J, Pitt MB. Bidirectional exchange
in global health: moving toward true global health partnership. Am J Trop Med
tion about the country and its sociopolitical context and public
Hyg. 2017;97(1):6–9 PMID: 28719333 https://doi.org/10.4269/ajtmh.16-0982
health priorities. Knowledge of the key medical problems facing the
Batra M, Pitt MB, St Clair NE, Butteris SM. Global health and pediatric education:
communities as well as international and local treatment guide-
opportunities and challenges. Adv Pediatr. 2018;65(1):71–87 PMID: 30053931
lines and strategies to deliver care within the confines of existing
https://doi.org/10.1016/j.yapd.2018.04.009
local resources are also important. Critical key minimal standards
Dieleman JL, Baral R, Birger M, et al. US spending on personal health care and
for preparation are described in Table 9.1.
public health, 1996-2013. JAMA. 2016;316(24):2627–2646 PMID: 28027366
The fundamentals for the creation of a meaningful interna- https://doi.org/10.1001/jama.2016.16885
tional partnership with a community involve multiple steps.
Oxfam International. Reward Work, Not Wealth: To End the Inequality Crisis, We
The first step is the development of a mission or an identified Must Build an Economy for Ordinary Working People, Not the Rich and Powerful.
shared purpose with partners before visiting a new international Oxford, UK: Oxfam International; 2018 https://doi.org/10.21201/2017.1350.
site. Second, it is critical to establish a collaboration with a local Accessed July 2, 2019
agency, such as an NGO or a governmental agency, to promote Steenhoff AP, Crouse HL, Lukolyo H, et al; GH Task Force of the American Board of
sustainability and enhance effectiveness of the care delivered. A Pediatrics. Partnerships for global child health. Pediatrics. 2017;140(4):e20163823
third important step for long-term effect and partnership is to PMID: 28931576 https://doi.org/10.1542/peds.2016-3823
ensure that the education of community members, other physi- St Clair NE, Pitt MB, Bakeera-Kitaka S, et al; Global Health Task Force of
cians, and trainees is a part of the mission and that appropriate the American Board of Pediatrics. Global health: preparation for working in
educational experiences are structured into the trip and plan- resource-limited settings. Pediatrics. 2017;140(5):e20163783 PMID: 29074610
ning. Closely related to this is the appreciation of the reciprocal https://doi.org/10.1542/peds.2016-3783
nature of any education: The visiting physician is in a position to Suchdev P, Ahrens K, Click E, Macklin L, Evangelista D, Graham E. A model
learn just as much if not more than the local health workers about for sustainable short-term international medical trips. Ambul Pediatr.
2007;7(4):317–320 PMID: 17660105 https://doi.org/10.1016/j.ambp.2007.04.003
working in the local setting.
A fourth fundamental step for meaningful partnerships United Nations Development Programme. Human Development Indices and
Indicators: 2018 Statistical Update. New York, NY: United Nations Development
involves ensuring that service is truly being provided to the com-
Programme; 2018 http://hdr.undp.org/sites/default/files/2018_human_development_
munity by learning about the health priorities of the community statistical_update.pdf. Accessed July 2, 2019
from the NGO or other local agency. It is also important to encour-
United Nations Inter-agency Group for Child Mortality Estimation (UN IGME).
age teamwork by working with appropriate supervision, including Levels & Trends in Child Mortality: Report 2018. Estimates Developed by the
physicians from the host country. Effective international collabo- UN Inter-agency Group for Child Mortality Estimation. New York, NY: United
ration also requires sensitivity to the costs and burden that a visi- Nations Children’s Fund; 2018 www.unicef.org/publications/index_103264.html.
tor has on the host. Finally, assurance of partnership effectiveness Accessed July 2, 2019

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 CHAPTER 10

Child Advocacy
Marni E. Shear, DO, FAAP, and Grant P. Christman, MD, FAAP

CASE STUDY
A 7-year-old boy is brought to the emergency depart- the child’s breathing improves somewhat. He is admitted
ment by his mother with acute onset of respiratory to the inpatient pediatric service for ongoing asthma
distress. He awoke from sleep with a coughing fit and management and care.
has not been able to catch his breath since. His mother
explains that her son was admitted to the hospital with
Questions
1. What does it mean to be a child advocate?
similar symptoms 1 month previously and was diag-
2. Aside from caring for individual patients, how
nosed with asthma at that time. Although the boy was
can pediatricians promote the well-being of their
prescribed 2 inhalers during his hospital admission, his
communities?
mother reports she no longer has these because her son
3. What is the role of the pediatrician in child
has not needed them. She also explains that her child
advocacy?
has a daily nighttime cough and frequent coughing with
4. What are the levels of advocacy?
exercise. After administration of an oral steroid load and
5. How does the pediatrician implement advocacy?
3 doses of ipratropium bromide and albuterol sulfate,

An advocate is someone who speaks on behalf of a person or cause. women and new mothers. The AMA, which was concerned about
No group in our society has a greater need for advocates than government interference in the practice of medicine, condemned the
children. Children are ill-equipped to face the many threats to act, whereas the AMA’s own Section on Diseases of Children sup-
their health; they cannot obtain their own health insurance, access ported it. Conflict related to the Sheppard-Towner Act ultimately
available social services, or get themselves to the doctor when resulted in the pediatric group leaving the AMA and founding the
sick. Additionally, children have limited influence compared with American Academy of Pediatrics in 1930. The American Academy
adults in our society. They cannot vote, donate money to political of Pediatrics has been advocating for children ever since.
campaigns, or speak publicly to advance their interests. The word
“advocate” is derived from a Latin root meaning “one who has been
called to another’s aid.” From the beginnings of pediatrics as an inde- The New Morbidity
pendent branch of medicine, pediatricians have answered this call Advocacy remains important because the “new” morbidities in
to advocate for the health and well-being of children. pediatric medicine, some of which are new and others of which are
Abraham Jacobi, MD, who is often referred to as the father of only newly recognized, are related to social and economic forces.
pediatrics in the United States, spent his career in the late 1800s Child health outcomes improved dramatically in the 1900s with
and early 1900s advocating for children through legislation in the development of vaccines, antibiotics, and new and improved
New York and the District of Columbia. He addressed issues such surgical care to manage the classic morbidities of infectious dis-
as breastfeeding, food and water contamination, and conditions in ease, infant mortality, poor nutrition, epidemics, overcrowding, and
foundling homes. He urged physicians to be involved in public life chronic disease. New morbidities that were recognized in the 1960s
and policy making, and he was the founder and first president of to 1980s, as described by Robert Haggerty, MD, included family
the American Medical Association (AMA) Section on Diseases of dysfunction, learning disabilities, emotional disorders, and edu-
Children. Another founder of pediatrics in the United States, Job cational problems. In the 1980s to early 2000s, Judith Palfrey, MD,
Lewis Smith, MD, recognized the need for a clean water supply and documented new challenges for pediatricians: social disarray, polit-
decent housing to decrease the high infant mortality rate of his time. ical ennui, the sequelae of high-tech care, and new epidemics of
He worked through public advocacy to improve living conditions violence, AIDS, cocaine, and homelessness. The newest morbidities
for all children, and he was the founder of the American Pediatric of the 21st century include the increased prevalence of childhood
Society, which was the first pediatric medical society. obesity, bullying, significant health disparities among cultural and
In 1921, the US Congress passed the Sheppard-Towner Act, the socioeconomic groups, and the growing population of children with
first major federal program to specifically address maternal and child special health care needs. Mounting evidence, such as provided by
health. It provided matching funds to states for services for pregnant the Adverse Childhood Experiences Study conducted from 1995

51

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 52 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

through 1997 by the Centers for Disease Control and Prevention Levels of Advocacy
and Kaiser Permanente, has demonstrated the critical need to iden-
Every pediatrician serves as a child advocate on a daily basis. With
tify and mitigate sources of toxic stress, which can pose a long-term
every patient encounter, the pediatrician advocates for care in the
threat to the developing brain.
best interest of the patient. This first level of advocacy includes treat-
Although improvement has occurred in recent years, many
ing the individual’s immediate medical needs, that is, performing
children in the United States continue to face challenges in obtain-
screening tests, providing anticipatory guidance, and coordinat-
ing access to quality health care. The 2015 National Health Interview
ing referrals as necessary. In addition to providing direct medical
Survey found that children without health insurance were less likely
care, the pediatrician may advance the welfare of the child by, for
to have a usual source of care and were more likely to postpone seek-
example, writing letters to help a patient obtain social services, or
ing care than children with health insurance (Figure 10.1). In the
visiting a patient’s school for a meeting on creating or reviewing an
past several decades, government programs such as Medicaid and
Individualized Education Program.
the Children’s Health Insurance Program have expanded the avail-
The second level of advocacy is community advocacy. The AAP
ability of health care coverage to children of limited financial means.
policy statement “The Pediatrician’s Role in Community Pediatrics,”
The passage of the Patient Protection and Affordable Care Act in 2010
describes “community pediatrics” as a perspective that broadens the
(PPACA) resulted in significant increases in access to health care
perspective from a focus on the individual patient to all the chil-
for many pediatric populations. The number of children younger
dren in the community; a recognition that family, education, society,
than 18 years with no health insurance coverage decreased from
culture, spirituality, economy, environment, and politics all affect the
6.6 million in 2012 to 3.9 million in 2016; however, 1 in every
health of children; a synthesis of clinical practice and public health
20 children remained uninsured as of 2016. Among children in
principles directed to providing health care to a child and promoting
poverty, the uninsured rate was 7%, compared with a rate of 5% for
the health of all children; a commitment to collaborate with the com-
children not in poverty. Racial and ethnic minorities were also more
munity to optimize health care for all children, especially disadvan-
likely to be uninsured, with Hispanic children having the highest rate
taged children; and recognition that community pediatrics is integral
at 7.9%. As of 2016, approximately 40% of all children were receiv-
to the role of the pediatrician. The pediatrician has a responsibility
ing coverage from public insurance programs, an increase from 2010.
to improve conditions in the community to benefit patient health.
With ongoing legislative efforts to either repeal PPACA or reduce bene-
To do this, they must be familiar with the services that are available
fits, the future of access to health care for children remains in jeopardy.
for children. They can develop relationships with child care centers,
These significant issues affecting child health cannot be ade-
schools, community coalitions, city governments, and local organi-
quately addressed on an individual basis. Advocacy on a commu-
zations to advocate for the best interests of children. Examples of
nity or national scale is required to improve child health outcomes
potential involvement in the community include serving as a board
and quality of life for children.

30%

25.5% 26.1%
25%

20%
17.5% 18.1%
16.6%
15% 14.2%
12.6% 12.2%

10%

5% 4.7%
3.2% 3.0%2.4% 2.9%
1.9% 1.9% 1.6% 1.0% 0.7%
0%
No usual source Postponed Went without Last MD contact Unmet dental Last dental visit
of care* seeking care needed care due >2 years ago need due to >2 years ago
due to cost* to cost* cost*
Uninsured Medicaid/other public Employer/other private

Figure 10.1. Children’s access to care by health insurance status in the United States in 2014.
* In past 12 months. Questions about dental care were analyzed for children age 2–17 years. All other questions were analyzed for all children younger than
age 18 years. MD contact includes other health professionals. Respondents who said usual source of care was the emergency department were included
among those not having a usual source of care. All differences between the uninsured and the 2 insurance groups are statistically significant (P < 0.05).
Adapted with permission from Kaiser Family Foundation analysis of 2015 National Health Insurance Survey (NHIS) data. https://www.kff.org/
wp-content/uploads/2015/11/children_s-access-to-care-by-health-insurance-status.png

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 Chapter 10: Child Advocacy 53

member of a community organization, developing health agendas, practical solution. Having credibility in the community makes the
working with an existing organization to design and fund a commu- task of collaboration much easier. Collaboration requires the abil-
nity service project, and being a source of information for the com- ity to compromise and be flexible in developing and implement-
munity on child health issues. ing plans. Larger projects may require funding, and grants may be
On the state level (the third level), pediatricians can work to sought from advocacy organizations, foundations, the government,
improve health care resources or develop policies to help and protect or even local businesses. Data should be collected during the inter-
children. Opportunities for involvement include working on legisla- vention to monitor the success of the project and then shared pub-
tion, budgets, regulations, and initiatives or working with the execu- licly with members of the community as well as with leaders who can
tive branch of local and state government. The fourth level of advocacy shape policy (eg, legislators). If the project is successful, its methods
is the federal level; at this level, pediatricians can educate their sen- may be adopted by child advocates in other communities.
ators and congresspersons on child health issues. Pediatricians may
also testify before a congressional subcommittee. The fifth and final Legislative Advocacy
level of advocacy is the international level. For example, a pediatrician
Although involvement in the legislative process is initially daunt-
may choose to work with the World Health Organization to improve
ing for the physician without political expertise, often it is the only
immunizations for all children worldwide. Global child health is
means by which to effect a desired change for children’s health.
currently a focus of many advocacy training programs.
Information about the content and progress of existing bills is read-
At the local, state, and national levels, voting remains a power-
ily available online, and legislators can be contacted by letter, email,
ful tool for the pediatrician to advocate for child health and welfare.
or telephone to offer a position. It is helpful to become familiar with
In addition to voting themselves, pediatricians can encourage their
the process by which a bill becomes a law, both at the state and the
patients to register to vote when they reach the age of eligibility.
federal level; the identities of the important players change as a bill
progresses through the various subcommittees and committees and
Becoming a Child Advocate
ultimately proceeds to a floor vote.
To become an effective child advocate, the pediatrician must first Pediatricians may also arrange to meet with a legislator or staff
identify an issue that he or she wants to change or set a goal to member at a district or capital office to discuss their position per-
improve the lives of children. The more specific the issue or goal, the sonally. In the dual role of scientist and healer, the pediatrician is
easier it is to develop a solution. Ideas often arise from clinical prac- in a unique position to inspire both the heart and the mind. It is
tice, in which repeatedly engaging in individual advocacy efforts on important to state the problem clearly and explain why a new law is
behalf of patients with the same problems suggests the need for a the solution, present well-researched facts that support the position,
larger solution. The first step in taking action is to obtain background and use clear language, avoiding medical jargon whenever possible.
information about the problem and collect objective data that sup- The pediatrician should minimize the appearance of self-interest by
port the need for change, then define the nature of the problem focusing on how the proposal will help children, rather than how
and the affected population in clear and precise terms. Child health it will benefit the profession. It may also help to connect with the
data from public agencies and private organizations are increasingly legislator by sharing a story about a patient encountered in prac-
accessible via the internet. tice who has been affected by the problem, especially if the patient
is a constituent of the legislator (although the patient’s identity
Community Projects must never be discussed without the patient’s consent). Providing
Alternatively, a pediatrician may find that an issue is best addressed the legislator a concise fact sheet summarizing the position and
through a community advocacy project. In developing such a the pertinent background information helps ensure that the posi-
project, the pediatrician’s relationship with the community is of tion is not forgotten when the legislator is considering the issue at
utmost importance, and the pediatrician should endeavor to become a later date.
familiar with the community as a whole. Community exploration, The pediatrician should be prepared to encounter opposition
which may be as simple as walking or driving through a community from some legislators and avoid responding with angry statements
and observing, can reveal areas of need, such as dilapidated housing that would be alienating. Effective advocacy requires building rela-
or unsafe streets. Equally important is the discovery of the communi- tionships with legislators over the long-term, and a legislator who
ty’s assets, including institutions such as places of worship, schools, opposes a position 1 year may be a potential supporter the next
and banks, which strengthen a community and are potential sources year, when the political climate changes, or may be a potential ally
of support for and counsel about the project. Pediatricians should on another important issue. Among the several other pitfalls to be
view themselves as members of the community, acting from within avoided include making or agreeing with partisan statements or
and in collaboration with the community, rather than as outsiders claiming to represent an organization (eg, AAP) or an institution
bringing about change externally. (eg, a university) without authorization. When asked a question to
The next step is to develop an intervention. After the possi- which they pediatrician does not know the answer, it is best to avoid
ble solutions are considered, the pediatrician should collaborate guessing and to instead offer to do further research and provide the
with community stakeholders to develop and implement the most requested information to the legislator at a later date.

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 54 PART 1: PRIMARY CARE: SKILLS AND CONCEPTS

When developing a new legislative proposal from scratch, it is story about a child who exhibited signs of autism shortly after his
necessary to remember that although the factors contributing to 1-year-old physical examination might write a letter to the editor
child health are numerous and complex, each legislative proposal discussing the lack of scientific evidence for a connection between
must by nature be concrete and limited. It may be best to start vaccinations and autism. Over time, a pediatrician can develop
small and work for incremental change. The first step is to iden- relationships with local journalists, who can then turn to the pedi-
tify a clear and, if possible, measurable, objective and define the atrician for information when covering child health stories.
target population. Other important information to know when A directed media campaign may also be a key element in
drafting a proposal includes any potential funding sources an advocacy project. At the community level, the media can
(if applicable) and which government agencies might be involved help educate the public about child health practices, notify the
in implementation or enforcement. The pediatrician should part- public of events, and bring out potential allies and coalition mem-
ner with 1 or more legislators early on, not only because a bill bers. When advocating for legislation, the pediatrician can use
must be sponsored by a legislator to be considered for passage the media to reach legislators directly and, equally important,
but because many of the finer points of the legislative process reach thousands of the legislators’ constituents simultaneously,
are outside the experience of the average pediatrician. Building a who may in turn help pressure their legislators for change. In
coalition of support within the community and involving impor- such situations, it is essential to plan a media strategy in advance
tant stakeholders such as politicians, business professionals, other by determining the most important target audience, selecting the
health professionals, educators, and parents, will help the bill gain appropriate types of media to approach, crafting a message appro-
political support. priate to those media, and preparing thoughtfully for encounters
Opposition should be expected, and potential sources of opposi- with journalists.
tion should be identified in advance. If opposition from a powerful
interest group is anticipated, it may help to meet with a represen- Getting Connected
tative of that group to explain the proposal. Potential arguments
The AAP is a vital resource for pediatricians interested in child
might include ways in which the proposal is really in the group’s
advocacy, with opportunities for involvement offered through the
best interest, the moral imperative to help children, or the potential
AAP Department of Federal Affairs, as well as the various state
for negative publicity by opposing an initiative to benefit children.
chapters advocating at the state and local levels. The AAP has
Compromising on aspects of the proposal should be considered when
also established a network of pediatricians who advocate through
doing so might turn a detractor into an ally. When facing intracta-
ble opposition from powerful interests, pediatricians and support- social media, using Twitter as a communication platform on
ers should strive to recruit even stronger allies into their coalition. which Tweetiatricians can initiate and facilitate conversations
The process of turning a policy idea into legislation may be on various topics related to child health. A social media toolkit
lengthy. It may be necessary to reintroduce a bill repeatedly over is available through the AAP to help pediatricians choose the
several years before achieving passage. After a bill becomes a law, best platform for their specific outreach goals. In an effort to
advocates must continue working to ensure that necessary funds promote understanding of the effect of poverty and social deter-
are allocated during the budgeting process, that public agencies minants on child health, the Academic Pediatric Association
implement the law as intended, and that the law is reauthorized has developed a robust curriculum to help deepen understand-
when necessary. Physicians who are recognized as experts in child ing of income disparities, social determinants of health, health
health policy will be called on to testify before committees in care delivery systems in the United States, and opportunities for
Congress or the state legislature on policy issues affecting children. legislative advocacy. Other national organizations with which
pediatricians may become involved and that provide advocacy
toolkits include Docs For Tots (http://docsfortots.org), Children’s
Media Advocacy Defense Fund (www.childrensdefense.org), Children Now
The media, including newspapers, magazines, radio, television, (www.childrennow.org), the National Center for Children
and the internet, are extremely influential. News stories about in Poverty (www.nccp.org), and the Child Welfare League of
child health and welfare may not always be written from a child- America (www.cwla.org).
friendly point of view. The pediatrician plays an important role Improving the health of all children through advocacy is consid-
in providing the media with better information and a different ered to be the responsibility of pediatricians and can be a tremen-
angle on a story. For instance, a pediatrician reading a newspaper dously rewarding part of pediatric practice.

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 Chapter 10: Child Advocacy 55

Chamberlain LJ, Hanson ER, Klass P, et al. Childhood poverty and its effect
CASE RESOLUTION on health and well-being: enhancing training for learners across the medical edu-
cation continuum. Acad Pediatr. 2016;16(3 suppl):S155–S162 PMID: 27044694
Managing this child’s asthma is only the first aspect of thorough pediatric care.
https://doi.org/10.1016/j.acap.2015.12.012
For the pediatrician to advocate for this patient and prevent a third admission
for a subsequent and potentially worse asthma exacerbation, it is necessary to Garner AS, Shonkoff JP; American Academy of Pediatrics Committee on Psychosocial
obtain essential information, such as the patient’s social history and the envi- Aspects of Child and Family Health, Committee on Early Childhood, Adoption, and
ronment in which he lives. A thorough social history is obtained and reveals that Dependent Care, Section on Developmental and Behavioral Pediatrics. Early child-
the child lives with his mother and grandmother. The mother recently lost her hood adversity, toxic stress, and the role of the pediatrician: translating develop-
job, and she and her son moved into a 1-bedroom apartment with the child’s mental science into lifelong health. Pediatrics. 2012;129(1):e224–e231. Reaffirmed
grandmother. The apartment has old carpeting, and the mother expresses con- July 2016 PMID: 22201148 https://doi.org/10.1542/peds.2011-2662
cern about mold on the walls. The child’s grandmother smokes cigarettes, but Gilbert LK, Breiding MJ, Merrick MT, et al. Childhood adversity and adult
she avoids smoking in the apartment when the child is home. The patient and chronic disease: an update from ten states and the District of Columbia, 2010.
his family are counseled about his diagnosis of asthma and potential asthma Am J Prev Med. 2015;48(3):345–349 PMID: 25300735 https://doi.org/10.1016/
triggers. The grandmother expresses interest in smoking cessation, and resources
j.amepre.2014.09.006
are provided. The family receives an asthma action plan and education on the
use of a metered dose inhaler with a spacer, and the pediatrician uses the Hoffman BD, Rose J, Best D, et al. The Community Pediatrics Training Initiative
teach-back method to ensure understanding of the plan of care at discharge. project planning tool: a practical approach to community-based advocacy.
The pediatrician becomes concerned about the high prevalence of asthma in MedEdPORTAL. 2017;13:10630 PMID: 30800831 https://doi.org/10.15766/
the community and explores coalitions in the area that recognize similar con- mep_2374-8265.10630
cerns. He becomes a member of the coalition steering committee and works with Kaczorowski J, ed. Community pediatrics: making child health at the com-
the local health department and other community stakeholders to develop a munity level an integral part of pediatric training and practice. Pediatrics.
home-based intervention program in which community health workers provide 2005;115(suppl 3):1119–1212
families with in-home environmental assessments, education, and support. As
a result of the coalition’s efforts, the child’s home is 1 of many apartment com- Palfrey JS. Child Health in America: Making a Difference through Advocacy.
plexes in the area assessed by the local housing authority, and resultant action is Baltimore, MD: Johns Hopkins University Press; 2006
taken to bring the property up to health and safety standards. Palfrey JS, Hametz P, Grason H, McCaskill QE, Scott G, Chi GW. Educating
the next generation of pediatricians in urban health care: the Anne E. Dyson
Community Pediatrics Training Initiative. Acad Med. 2004;79(12):1184–1191
PMID: 15563653
Paulson JA. Pediatric advocacy. Pediatr Clin North Am. 2001;48(5):1307–1318
Selected References PMID: 11584815
Bar-on ME. The use of public education in practice. Pediatr Rev. 2001;22(3): Rushton FE Jr; American Academy of Pediatrics Committee on Community
75–81 PMID: 11230625 https://doi.org/10.1542/pir.22-3-75 Health Services. The pediatrician’s role in community pediatrics. Pediatrics.
Barnett JC, Berchick ER. Health Insurance Coverage in the United States: 2016. 2005;115(4):1092–1094. Reaffirmed January 2010 PMID: 15805396 https://doi.
Washington, DC: U.S. Government Printing Office; 2017. Current Population org/10.1542/peds.2004-2680
Reports, P60–260 Sheehan K, ed. Pediatric advocacy. Pediatr Ann. 2007;36(10):624–625

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 PART 2

Principles of Health
Care and Pediatric
Management
11. Health Systems Science......................................................59
12. Population Health for Pediatricians..................................73
13. Principles of Pediatric Therapeutics..................................79
14. Pediatric Pain and Symptom Management.......................85
15. Complementary and Integrative Medicine in
Pediatric Primary Care.......................................................95
16. Principles of Pediatric Surgery.........................................105
17. Image Gently Approach to Pediatric Imaging.................109
18. Simulation in Pediatric Health Care................................113
19. Pediatric Hospital Medicine.............................................121
20. Pediatric Genomic Medicine............................................125
21. Principles of Quality Improvement:
Improving Health Care for Pediatric Patients.................129
22. Pediatric Palliative Care: Principles and Practice...........137

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BPPCA6e_Ch011_0057-0072.indd 58 2/14/20 10:40 AM
 CHAPTER 11

Health Systems Science

Stephanie R. Starr, MD, FAAP

CASE STUDY
You are seeing Sara, a 14-year-old girl with multiple be patient- and family-centered, but you are concerned
health issues (ie, asthma, obesity, acanthosis nigricans, about the risks and costs of unnecessary testing.
mood disorder, attention-deficit/hyperactivity disorder,
posttraumatic stress disorder) for a follow-up visit for a
Questions
1. How might you approach the conversation with Sara and
recent concussion. You know her family (ie, mother and
her mother in response to their request for imaging?
sister) well. She has frequent emergency department vis-
2. How are evidence-based medicine, “less is more”
its for abdominal pain, asthma exacerbations, and head-
conversations, and shared decision making related
aches. You frequently lack sufficient time during visits to
to high-value care?
address all her concerns and the health issues you want
3. What other health systems science-related issues
to discuss. The mother has been unemployed for several
do you recognize in Sara’s story, and what systems
years. Your team has had some challenges reaching her
strategies might be considered to improve her
family when visits are missed. You sincerely want to help
health and experience of care?
Sara and her mother meet their health goals for Sara but
4. What microsystem-level actions could you and your
feel that providing the best care during office visits is not
care team use to improve the health care and out-
making a significant difference in her health.
comes of similar patients in your pediatric practice?
At today’s visit, the mother asks for head imaging
5. What macrosystem-level actions could you and your
because she is concerned about Sara’s ongoing dizziness
colleagues take to improve the health care and
and headaches; however, her neurologic examination
outcomes of similar children in your health system
is normal and she does not have any “red flag” symp-
or community?
toms or signs that warrant head imaging. You want to

of health are estimated to contribute more toward health outcomes

Introduction
than health behaviors, care via the health care system, or genet-
Rapid Evolution of US Health ics. Advances in shared decision making and conversations with
Care and Persistence of Significant patients and families on choosing tests and treatments that are
Gaps in Health high value to patients, families, clinicians, and the system require
For decades, primary care pediatricians have focused on the indi- new clinical skills not traditionally taught in caring for individ-
vidual needs of infants, children, adolescents, young adults, and ual patients.
their families, and have made every effort to provide the highest As children, their families, and their health issues have changed,
quality care to their patients. The components of primary care and so have pediatric practices and the broader US health care system.
the patient-centered medical home are ideally combined to opti- In 2007, the Institute for Healthcare Improvement proposed Triple
mize the care of children using a holistic approach (see Chapter 1). Aim as the ultimate goal of health care, that is, optimal health of
The role of the primary care pediatrician is changing from the population and best experience of care at the lowest cost. Health
managing acute illness to preventing illness to diagnosing and treat- care stakeholders (ie, patients, families, payors, insurers, health pro-
ing new morbidities, such as mental health disorders, neurodevel- fessionals, health care systems, society) more commonly frame this
opmental disabilities, chronic disease, and other disorders that often as value, that is, the quality of care (ie, care that is safe, timely,
stem from socioeconomic determinants of health, including poverty, effective, efficient, equitable, and patient-centered) divided by the
family dysfunction, abuse, and other adverse childhood experiences. cost of care over time. Health care spending does not correlate to
Although advocacy (see Chapter 10), culturally competent care health outcomes, and health care costs are the largest contribu-
(see Chapters 8 and 57), and global child health (see Chapter 9) have tor to personal US bankruptcies. Substantial gaps in health care
long been emphasized in caring for pediatric patients, these new access, equity, affordability, safety, effectiveness, and efficiency per-
morbidities and other changes in the lives of patients and their sist despite significant advances in biomedical science. Primary care
families require an explicit focus on new topics. Social determinants pediatricians and other health professionals are advancing child

59

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 60 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

health via health care improvement (ie, quality improvement [QI], Medical Education and the Needs
patient safety [see Chapter 21]) initiatives locally, regionally, and of Society
nationally. Doing so requires the ability to see levels of the health
Medical education has an obligation to improve quality of life,
care system beyond the individual patient alone and to espouse
reduce the burden of disease, and help advance the Triple Aim to
the professional responsibility to care for the system in addition
fulfill its contract with society. Despite gaps in health care quality
to caring for individual patients. In current practice, some health
and rising costs of care, little has changed in physician education
professionals have added a fourth aim: wellness of the health pro-
since Abraham Flexner proposed reform in 1910. Accreditation
fessionals and others on the care team.
and certification bodies have added requirements in some
Many interactions and systems issues that affect health care and
areas, such as the Accreditation Council for Graduate Medical
health underlie every physician-patient interaction (Figure 11.1).
Education Systems-Based Practice and Practice-Based Learning
This complex health care system is rapidly changing, including but
and Improvement competencies in residency and fellowship, but
not limited to payment models and insurance reform, new care
many changes are incremental and gaps persist. Because medi-
delivery models, population health strategies, and emerging tech-
cal costs are the most common reason for personal bankruptcy
nology. Medicine and health care have become a team sport, with
filings in the United States and because of the desire to first
less focus on the physician-patient interaction alone and increased
do no financial harm, some health professionals have advocated
incorporation of interprofessional health teams into the patient-
for high-value care as the seventh competency required for grad-
centered medical home model. Although pediatric health issues
uate medical education.
have changed significantly and the health care system is constantly
Traditional physician training in basic and clinical science alone
evolving, little has changed in the training of the primary care
does not meet the current health needs. Many practicing health
pediatric workforce. The American Academy of Pediatrics (AAP)
professionals identify additional learning that would help them
“Agenda for Children 2017-2018” includes topics not traditionally
practice in the rapidly evolving system and achieve the Triple Aim.
learned in medical school or residency, including access, finance,
To help medical education evolve to match the pace of change in
and social determinants of health. Practicing pediatric health pro-
practice, and in recognition that rigorous basic and clinical sci-
fessionals enter the profession with a steadfast commitment to
ence training is insufficient to meet the Triple Aim, many medical
improving the health of children, and many identify additional
educators are advocating for training physicians in a “third” science:
learning that would help them practice in this rapidly evolving
health systems science (HSS; Figure 11.2).
health care system.

Care provided
to an individual patient

Teamwork and interprofessional Proactive,

collaboration person-centered
care

Health-information technology
and data
Health
system Healthcare
integration value
Behavioral and social Systems
determinants thinking
of health
Population
health
management

Policy and healthcare

reform Health system improvement

Figure 11.1. The “iceberg” of health care concepts affecting health.

Reprinted with permission of the American Medical Association from Health Systems Science, first edition. ©Copyright American Medical
Association 2017. All rights reserved.

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 Chapter 11: Health Systems Science 61

planned systems-related curricula at their schools (Figure 11.3).

Health systems science in medical education is defined as the
principles, methods, and practice of improving quality, outcomes,
and costs of health care delivery for patients and populations with
Basic Clinical
science science systems of medical care. Stated another way, HSS can also be defined
as the concepts and skills needed by health professionals to ensure
that the basic and clinical sciences and the good intentions of health
professionals have maximal effect on the Triple Aim.
Health The core content domains within HSS were described as health
systems care structures and processes; health care policy, economics, and
science
management; clinical informatics and health information technol-
ogy; population and public health; value-based care, and health
system improvement. Content areas (ie, cross-cutting domains)
traditionally included in many schools but re-envisioned within
Figure 11.2. The 3-pillar model of medical education. HSS included scholarship; leadership and change agency; profes-
sionalism and ethics; teamwork and interprofessional education;
Health Systems Science in Medical and evidence-based medicine and practice. Systems thinking was
described as the linking domain for the HSS framework.
Education
Some schools use terms similar to HSS, such as “science of health
Health Systems Science Education: care delivery,” “health care delivery science,” and “foundations of
Definition, Framework, and Relevance health care delivery” (Figure 11.4). Many medical schools in the
to Primary Care Pediatricians United States and elsewhere are incorporating HSS-related curricula,
In 2017, experts from 11 medical schools published a curricular with variation in scope and depth. The nomenclature related to this
framework for HSS education to help address health and health care third science and the evidence for best methods to teach and assess
gaps. The curricular framework was proposed based on existing or it will continue to evolve. The old mantra “we are training students

Leadership and Linking

change agency domain
ing Sy
nk ste
thi m
s
s
em
th

Core domains
in
st
Sy

kin
g

Health care structures and Teamwork and

Scholarship processes interprofessional
Health care policy, economics, and education
management
Clinical informatics and health
Sy
ing

information technology
t
think

em

Population and public health Cross-cutting

s thin
ms

Value-based care domains

ste

kin

Health system improvement

y

g
S

Evidence-based
Professionalism
medicine and
and ethics
Systems thinking
practice

Figure 11.3. Core, cross-cutting, and linking domains for a health systems science (HSS) curricular framework. Core
curricular domains are content areas that align directly with HSS. The cross-cutting domains are content areas that
traditionally may have been included in undergraduate medical education curricula but that have a new context in
the HSS. The 1 linking domain—systems thinking—unifies or links the core curricular or cross-cutting domains to
other core curricular or cross-cutting domains (ie, internal linking, depicted in this figure) and to other areas of the
curriculum, such as the basic and clinical sciences (ie, external linking, not depicted in this figure).
Reprinted with permission from Gonzalo JD, Dekhtyar M, Starr SR, et al. Health systems science curricula in undergraduate medical
education: identifying and defining a potential curricular framework. Acad Med. 2017;92(1):123–131.

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 62 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

health insurance) more easily than they can anticipate or see other
gaps through the lens of the patients’ and families’ view as they expe-
rience care across a system. It can be difficult to really know what
High
value patients and families experience from the moment they call to try
care and schedule an office visit, to parking, to checking in to the front
Health desk, to being led to a room by a nurse or other care team member,
policy, Population- to being seen by the pediatrician, to going to the laboratory (on site
economics, centered
technology care or at a distance) for a blood test, to accessing test results and inter-
pretation from the pediatrician.
Health professionals should ideally understand 3 essential
concepts related to health care systems: systems thinking; the struc-
Patients tures, processes, and outcomes comprising the building blocks of
Person- Team-
centered based
health care delivery; and effect on the patient of every level of the
care care health care system.

Systems Thinking
Leadership
The Waters Foundation has summarized 14 habits of systems
thinkers that encourage health professionals to be flexible in their
thinking, identify new insights, and appreciate other perspectives,
including recognition that a system’s structure generates its behav-
Figure 11.4. Health care curricular framework.
ior. Stated another way, every process is perfectly designed to get
Reprinted with permission from Starr SR, Reed DA, Essary A, et al. Science of health
the results it gets, so to get a different outcome, the structure and/
care delivery as a first step to advance undergraduate medical education: a multi-
institutional collaboration. Healthc (Amst). 2017;5(3):98–104.
or process must be changed as well. Systems thinkers can apply tools
(many of which are used in QI training) to augment these 14 habits.

The Building Blocks of Health Care Delivery:

to care for tomorrow’s patients” is no longer the ideal; schools are Structures, Processes, and Outcomes
demonstrating that medical students can improve the care of today’s Avedis Donabedian, MD, MPH, considered by many to be the grand-
patients as they learn about HSS. father of US health care quality, explained the importance of improv-
Pediatricians in training and those in practice likely have signif- ing health care quality by using structural, process, and outcomes
icant variation in their understanding and practice of HSS-related measures. These measures provide a granular way of showing that
concepts and skills based on their undergraduate medical educa- health improvement efforts are “moving the needle” and having a
tion, graduate medical education, and/or their practice experiences. positive effect (see Chapter 21). Examples of structure, process, and
Although health professionals may have experience and expertise outcomes measures are given in Table 11.1.
in some HSS-related areas, which for pediatricians includes a focus
on advocacy and culturally competent care, they may not be able to Effect on Patients: Recognizing the Levels
easily explain relationships across HSS topics to team members of the Health Care System
and/or the students and residents they teach. Without a broad The effect—both positive and negative—on patients occurs at
definition of the third science concepts that affect health and health every level of the health care system, and clinicians must be
care, pediatricians may miss opportunities to recognize and close able to recognize the levels of the health care system on behalf
gaps in care for children and their families. Pediatricians com- of patients and clinical team members. Patient- and family-
mitted to caring for children can have great impact when they see centered pediatricians and their care teams consider the experi-
their professional duty is both to doing their work (caring for indi- ence of care, disease, and treatment burden from the perspective
vidual patients) as they improve their work (caring for the health of the patient and family. Pediatricians who think with a systems
care system and for populations of patients). Envisioning HSS as perspective are more likely to identify additional opportunities
a third science with a conceptual framework can foster system- to improve care not only for their individual patients, but also
atic thinking and help pediatricians recognize the actions they can for groups of similar patients. They can make or influence
take (or “levers” they can pull) to meaningfully improve child and changes (ie, pull levers) not only at the patient-clinician level,
family health. but also at the microsystem, mesosystem, and macrosystem
levels (Figure 11.5).
Systems Thinking: Components The microsystem is most familiar to pediatricians; it is the front-
and Levels line clinical team that, in addition to physicians, may include nurses,
Systems thinking is at the core of HSS. Pediatricians may recog- nurse practitioners, clinical assistants, secretaries, and reception-
nize gaps in care that occur during office interactions with patients ists. Nurses on the team may have several specialized roles based
(eg, inability to identify a dentist who accepts the family’s government on their scope of licensure, including clinic nurse, triage nurse,

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 Chapter 11: Health Systems Science 63

Table 11.1. Examples of Measures to Assess the Quality of Pediatric Asthma Care
Intermediate Clinical Outcome
Structure Measures Process Measures Measures Clinical Outcomesa
Spirometry equipment and Percentage of patients within the past Asthma control short-term Quality of life
interpretation 12 months with Use of steroids in past year
Asthma population database with Updated asthma action plan ED visits, hospitalizations in past year
support staff ED visit or hospitalization
Completed asthma control
questionnaires
Received influenza vaccine
Abbreviation: ED, emergency department.
a
Most important to patients and families.

Community, market,
and social policy

Self care

Macrosystem

Patient-clinician
dyad Mesosystem

Microsystem

Figure 11.5. Levels of the health care system. Self care is care provided by patients and their families. Note that patients
and/or families can obtain resources for their health directly from the community (outer circle). The microsystem is the
front-line interprofessional clinical team with whom patients and/or families interface. In pediatric primary care, physicians,
nurse practitioners, nurses, and secretaries are part of the primary care team/practice. The mesosystem comprises connected
microsystems that patients and/or families traverse in their experience of care (eg, primary care team, inpatient care team,
radiology team, pharmacy team, outpatient specialty care team, emergency department team). The macrosystem comprises
mesosystems that patients and/or families traverse in their experience of care (eg, community health center, referral
specialty health center, public health).
Reprinted with permission from Nelson EC, Batalden PB, Godfrey MM. Quality by Design: A Clinical Microsystems Approach. San Francisco, CA:
Jossey-Bass; 2007.

and care manager (eg, for complex patients). Ideally, every team possible pneumonia, a patient may move across 3 microsys-
member has a role that enables that person to work at the peak of tems (ie, pediatric, radiology, and pharmacy teams) before leav-
that individual’s experience and licensure, and all perspectives are ing the clinic. Opportunities to improve the care of other patients
leveraged to recognize gaps in care and contribute to closing gaps like that one may occur at the microsystem level (eg, improv-
(eg, via QI initiatives). ing wait times) or may require a coordinated effort across
Mesosystems are collections of microsystems that a patient 2 microsystems. Quality improvement and advocacy are levers that
may move across during an episode of care. For example, a can be pulled to make or influence change at this level.
large multispecialty group practice at 1 clinic may have multi- Similarly, macrosystems are collections of mesosystems across
ple pediatric, family medicine, and internal medicine teams which a patient may move during an episode of care. An ill child
(ie, microsystems) as well as other microsystems (eg, labo- seen in a primary care mesosystem who is transported to the emer-
ratory, radiology, and pharmacy teams). During a visit for gency department mesosystem (with, for example, emergency

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 64 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

medicine microsystem, laboratory microsystem, radiology micro- the pediatrician should consider how excellence in this first contact
system) and is later admitted to the hospital (with similar meso- would be defined by patients and their families. In addition to
system members) encounters an entire macrosystem. The quality the patient-physician relationship and cultural effectiveness (see
of care this child receives is dependent in part on the strengths or Chapters 8 and 57), patients and families might prioritize a focus
weaknesses not only of each team, but on how the teams commu- on needs, desires, and goals unique to the child and/or family.
nicate during handoffs and how everyone is able to envision the Pediatricians might also include screening for poverty and for finan-
patient’s journey from the start of his or her experience (ie, in the cial harm from medical treatment. Currently, this first contact may
primary care office) to the end (ie, hospitalization and eventually, be with another care team member via a nurse triage telephone call
discharge home). To reiterate, both QI and advocacy can occur at or via an online patient portal message.
the macrosystem level.
Longitudinal Care
Longitudinal care requires providing optimal care over time to indi-
Applying Health Systems Science vidual patients and to populations of patients, ideally within the med-
to the Components of Pediatrics ical home model. The Joint Principles of the Patient-Centered Medical
Primary Care Home (PCMH) were co-published in 2007 by the AAP, American
The components of primary care as described by Charney and Academy of Family Physicians, American College of Physicians, and
Alpert in 1974 include first contact, longitudinal care, family orien- the American Osteopathic Association. An emphasis on the Triple Aim
tation, and integration of comprehensive care (see Chapter 1). For all and the change in payment models (from fee-for-service to payment
pediatricians working in the current US health care system, these for value) have helped fuel this population health approach. Care is no
components remain critical and relevant, but the pediatrician with longer limited to face-to-face physician visits and phone triage with
a systems view will recognize the need for additional components nurses; it includes nonvisit care via electronic patient portals between
and will understand that it is necessary to evaluate each compo- in-person visits to address simple new concerns, determine if a visit
nent at multiple levels of the system to maximize the health of all is necessary, provide test results, and follow up with patients and
children. Examples of each component at multiple levels of the families on chronic health issues.
health care system are described in Table 11.2. These examples are The AAP aptly describes the population health mindset as a
opportunities for pediatricians and colleagues in their clinical (ie, change by health professionals from passivity to proactivity. Rather
microsystem) teams to see opportunities to promote high-value care than waiting for patients to come to providers and care teams, the
and improve child health and, in doing so, advance the Triple Aim. care teams reach out to patients and families. Not all patients in
a given practice have the same level of need; thus, different sys-
Pre–First Contact tems approaches are necessary. Relatively healthy patients benefit
The process or steps that patients and families encounter before having from preventive interventions (eg, immunizations) and screenings
their first contact (ie, office visit) was not conceptualized when medi- (eg, review of growth at well visits, lead and developmental screen-
cine and care delivery outside of public health was purely transactional, ings). Children with a single chronic disease (eg, persistent asthma)
an interaction between the patient and the physician (or in the inpa- may benefit from practice-level processes to improve their health
tient setting, a patient and a hospital team). Variation in access to care outcomes by targeting identification of emergency department visits,
based on health insurance, geography, transportation, and other bar- monitoring oral steroid use, and regularly updating asthma action
riers now affect how a patient interacts with primary pediatric teams plans. For children with medical complexity, care management may
in primary care medical homes. Pediatricians and pediatric medical be necessary to help families and the PCMH team optimize the value
homes seeking to improve child health care and health must concep- of care rendered (eg, assigning a primary nurse who knows the child
tualize their role in ensuring the patients in their care have access to well and who can triage concerns as they arise).
care when they need it and to preventive care to maintain their health. The National Resource Center for Patient/Family-Centered
For example, in 2015 the National Academies of Sciences, Medical Home (formerly the National Center for Medical Home
Engineering, and Medicine (NASEM) published a white paper titled Implementation), a collaboration between the AAP and the Maternal
Improving Diagnosis in Health Care. The authors proposed a systems and Child Health Bureau of the Health Resources and Services
view of diagnostic errors, with the recognition that accurate and Administration of the US Department of Health and Human
timely diagnosis cannot be made without access to the care team Services, summarized a number of promising practices and their
(Figure 11.6). Pediatricians must envision the health care system PCMH innovations, including family orientation and integration
from the patients’ and families’ point of view and recognize oppor- of comprehensive care.
tunities to improve the likelihood that children who need the first
contact are able to benefit from the team’s care. Family Orientation
Children and their caregivers will not achieve optimal health or have
First Contact an optimal experience of care unless pediatricians and their care
A first contact in a pediatric medical home may be a visit for an teams are deliberate in their commitment to systematic culturally
acute or chronic health issue or for a well visit. Using a systems view, competent and effective care, both for individual patients and groups

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 Chapter 11: Health Systems Science 65

Table 11.2. Examples of Opportunities to Increase Health Care Value for Patients and Families
Care Opportunity Individual Patient (and Family) Microsystem Mesosystem/Macrosystem
Pre–first contact Pre-visit contact (electronic or paper) Open access scheduling Decrease barriers to transitioning new
soliciting the family’s initial health Expanded clinic hours patients from inpatient settings (eg,
concerns and the family’s story that they neonatal intensive care unit, hospital)
Website or other electronic presence to
want to share with the team in advance into the medical home model
welcome families and explain resources
First contact (ie, first Culturally competent care (see Chapters 8 Screening for mental health issues in Strategies to decrease wait times and
clinic visit) and 57) pediatric patients (eg, PHQ-9M for patients increase efficiency of the visit from the
Routine inclusion of the question “What age 12–17 years) and in mothers of infants family’s perspective
matters to you?” at the first visita (eg, postpartum depression screening)
Adverse childhood experiences screening
Food scarcity screening
Longitudinal care Advocacy (eg, with school system for IEP) Step 5 in high-value care framework (QI): Strategies to improve transitions of care to
for health needs Identify systems-level opportunities to adult providers for young adults with
Health coaching improve value (see Figure 11.6)b medical complexity across pediatric
Non-visit care (eg, online portal) primary care, adult primary care, and
Steps 1–4 in high value care framework
specialty groups
(see Figure 11.6)b Care management for patients such as
those with complex presentations, asthma, Strategies to collaborate on matters of
or depression school-related health, such as with coaches
for concussion services, school staff
Identification of and contact with high-risk
about meals and nutrition, and in-school
children needing the influenza vaccine
influenza vaccine clinics
Family orientation Shared decision making that incorporates Needs assessments of families in the Advocate for needs assessments of families
patient and family preferences, values, and practice in terms of issues such as in other areas of multispecialty practices
context (ie, circumstances)c preferred communication and extended
hours
Integration of Use of handoff tool with accepting Registered nurse care management Work with inpatient and outpatient
comprehensive care physician when a patient is admitted to the program with registry for medically specialty, emergency department, and
hospital or to another new care team complex children hospital colleagues to improve transitions
Electronic tool for communicating patient of care and patient safety when patients
care goals between patient, family, and move from 1 setting to another
health care teams
Abbreviations: IEP, Individualized Education Program; PHQ-9M, 9-item Patient Health Questionnaire Modified for Teens; QI, quality improvement.
a
Institute for Healthcare Improvement. What matters? IHI.org website. www.ihi.org/Topics/WhatMatters/Pages/default.aspx. Accessed August 19, 2019.
b
Modified from Smith CD; Alliance for Academic Internal Medicine–American College of Physicians High Value, Cost-Conscious Care Curriculum Development Committee. Teaching high-value, cost-
conscious care to residents: the Alliance for Academic Internal Medicine–American College of Physicians Curriculum. Ann Intern Med. 2012;157(4):284–286.
c
Hoffmann TC, Montori VM, Del Mar C. The connection between evidence-based medicine and shared decision making. JAMA. 2014;312(13):1295–1296.

of patients. Care team members must have knowledge of the social delivery innovations include electronic specialty consults as well
determinants of health and their effect on health (see Chapter 141). as integrated behavioral health and other specialty care models
This is particularly important when establishing a strong relation- integrated within the PCMH. It also includes integration as part of
ship with children and their caregivers at the first visit. ongoing care (eg, in conjunction with community partners, such as
public health, schools, child care centers, home health and nursing
Integration of Comprehensive Care agencies, and pharmacies).
Integration of comprehensive care requires pediatricians and their To Err is Human, published in 1999 by the Institute of Medicine
PCMH team members to integrate the care of patients within the (now the Health and Medicine division of the National Academies),
PCMH and across other parts of each patient’s health ecosystem catalyzed the US patient safety movement. A critical component of safe
to ensure that data are shared appropriately and handoffs occur care is the expert and deliberate process for communicating with teams
effectively. This includes the interaction between primary and and across teams at times of transition. This includes transitions across
secondary (or specialty) care (see Chapter 1). Several new care care settings during an episode of care (eg, emergency department to

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 66 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

ation integrati
orm o
Inf & interpretation n

formation bee
t in n
en co
ci

ffi

lle
su

cte
Clinical

Has

d?
history and Physical
Patient Patient
interview examination
experiences engages with Communication
Treatment Outcomes
a health health care of the diagnosis
problem system Referral and Diagnostic
I n f o h e ri consultation testing
The explanation of The planned path Patient and

no g
gat

sis
a g rkin
r m ng

the health problem of care based on system outcomes

that is communicated the diagnosis Learning from
at

o
io

W
to the patient diagnostic errors,
n

di
near misses, and
accurate, timely
diagnoses

Time

Figure 11.6. The diagnostic process.

Reprinted with permission from National Academies of Sciences, Engineering, and Medicine. Improving Diagnosis in Health Care. Washington, DC: The National Academies Press;
2015. https://doi.org/10.17226/21794.

hospital, hospital to PCMH, PCMH to specialty care). The Agency for handoffs to those conducting forensic interviews and to educate child
Healthcare Research and Quality Team STEPPS program provides a caregivers on reporting requirements and parents on abuse preven-
model for training in interprofessional teams to improve patient safety. tion strategies (ie, macrosystem). Conceptualizing the system in this
The I-PASS program is a framework studied in pediatric residencies to manner may also help pediatricians consider the stakeholders who
increase patient safety during provider handoffs. Common handoffs in can align with their efforts and/or inform their efforts to help ensure
integrated, comprehensive care include dismissal of patients with com- success.
plex medical issues from an acute care hospital setting to the PCMH.
Population Health
Pediatric Primary Care Examples As mentioned previously, 1 intervention to improve pediatric health
is care coordination of children with complex health needs. In 2014,
of Health Systems Science Principles
the AAP published recommendations for care coordination of chil-
Advocacy dren with medical complexity, including features of care coordina-
Advocacy has long been a professional expectation of pediatri- tion excellence, such as use of health information technology and
cians on behalf of children, a segment of society without a legal health outcomes tracking over time. This recommendation was
voice. Advocacy occurs at the individual patient-pediatrician level reaffirmed in 2018. As with many population health strategies, the
(eg, working with schools to evaluate students who may qualify perspective focuses on the health of all patients empaneled to the
for special education services) or at a higher level (eg, the state PCMH, not just those who come in for face-to-face visits.
legislature for effective booster seat laws). Carol Berkowitz, MD, Recent years have seen several successful population health models
has described advocacy for individual patients as advocacy with within primary care settings, including collaborative care models for
a “little a,” and for groups of patients as advocacy with a “big A.” adolescent depression and other integrated behavioral health strategies.
Systems thinking can help reveal opportunities to advance child Primary care pediatricians have collaborated with pediatric subspe-
health in the realm of advocacy. For example, pediatricians can use cialists and nurse care managers using patient registries to proactively
the AAP Oral Health Toolkit to advance oral health initiatives for care for their population of children and adolescents with persistent
children at multiple levels of the system, including the community. asthma. Registries can also be helpful to proactively manage children
Alternatively, if during a face-to-face clinic visit a pediatrician notes with medical complexity and children with other chronic conditions,
findings that are suspicious for child abuse and maltreatment, the such as attention-deficit/hyperactivity disorder.
pediatrician is legally bound to advocate for the child by reporting to
Child Protective Services. Pediatricians can work with their clinical Social Determinants of Health
(ie, microsystem) team to develop new strategies for making children As previously noted, social determinants of health (see Chapter 141)
more comfortable during visits when suspected abuse is the chief con- are estimated to have a more significant effect on health than
cern. They can work with community partners to ensure child-friendly health behaviors, health care, or genetics. Pediatricians must

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 Chapter 11: Health Systems Science 67

elicit information about social determinants of health to be history and physical examination alone. A patient presenting with a
successful in caring for individual patients. At the microsystem limited diet and fatigue may require only 1 laboratory test (ie, com-
level and above within the health system, pediatricians and their plete blood count) to confirm iron deficiency anemia. Many diag-
care teams can use population health approaches to identify sub- noses are made over time, however.
populations of patients in their practice (eg, refugee families) In 2012, the Alliance for Academic Internal Medicine and
and conduct a needs assessment to tailor the practice to meet the the American College of Physicians published a 5-step model for
needs of these patients. teaching high-value care to residents (Figure 11.8). These evidence-
Beyond the health system, many opportunities exist for pediatri- based strategies report positive health impacts within 5 years of
cians and other child health advocates to influence change to mini- implementation and are cost effective and/or cost saving over the
mize the negative effect of social determinants of health. The Centers lifetime of the population. They can help pediatricians see the rela-
for Disease Control and Prevention developed the “Health Impact tionships across the key HSS-related knowledge and concepts of
in 5 Years” initiative, which highlights nonclinical, community- evidence-based medicine, shared decision making, and health care
wide strategies (Figure 11.7). These evidence-based strategies report improvement.
positive health impacts within 5 years, and cost effectiveness and/or Steps 1 and 3 of the model are part of critical appraisal of the
cost savings over the lifetime of the population. literature and evidence-based medicine. Step 2 provides an
opportunity for pediatricians to stop medications or reconsider
High-Value Care unnecessary testing if it does not provide value to the patient.
The role and limitations of laboratory tests and other diagnostic Step 4, which is part of shared decision making, requires pedi-
studies in making an accurate diagnosis for patients and families has atricians to apply what they have learned from the evidence to
long been taught as part of the basic and clinical sciences. With a sys- the patient in front of them. More broadly, this is the stage at
tems approach, the NASEM model for improving diagnoses empha- which shared decision making, whether formal or informal, is
sizes diagnoses as a series of hypothesis testing (see Figure 11.6). necessary to ensure that the patient (as age allows) and family
For example, the diagnosis of acute otitis media is made based on are given adequate information to make a decision that reflects

School-based programs to
increase physical activity
School-based violence
prevention Counseling
and education
Safe routes to school
Motorcycle injury
Clinical interventions
prevention
Tobacco control Early childhood
interventions Long lasting education
Access to clean syringes protective interventions Clean diesel bus fleets
Pricing strategies for Public transportation
alcohol products system
Multi-component worksite Home improvement
obesity prevention loans and grants
Changing the context
Making the healthy choice the easy choice Earned income tax
credits
Water fluoridation

Social determinants of health

Figure 11.7. Public health impact pyramid. The pyramid depicts the potential impact of different types of public health interventions from
greatest potential impact at the base (because they reach entire populations of people and require less individual effort) to least potential
impact (because they target specific populations and require more individual effort).
Reprinted from Centers for Disease Control and Prevention, Office of the Associate Director for Policy and Strategy. Health Impact in 5 Years. CDC.gov website.
www.cdc.gov/policy/hst/hi5/index.html.

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 68 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Healthcare
EBM EBM SDM improvement

Step 4:
Step 5:
Step 1: Step 2: Step 3: Create plan
Identify
Understand Decrease Choose that
systems-level
risks, benefits, interventions interventions incorporates
opportunities to
and costs of of minimal/no that maximize patient
improve
interventions value value values,
value
concerns

Figure 11.8. Framework for teaching high-value care, showing some of the relationships to other health systems science–related concepts.
Abbreviations: EBM, evidence-based medicine; SDM, shared decision-making.
Adapted with permission from Smith CD; Alliance for Academic Internal Medicine–American College of Physicians High Value, Cost-Conscious Care
Curriculum Development Committee. Teaching high-value, cost-conscious care to residents: the Alliance for Academic Internal Medicine–American
College of Physicians Curriculum. Ann Intern Med. 2012;157(4):284–286.

their context (eg, other aspects of their lives they are balancing Physicians may need help determining which tests to order using
as a family, the work of being a patient), their preferences, and a high-value approach that reflects the individual considerations for
their values. Step 5 is health care improvement at the microsys- each patient while also reflecting what is known to be effective care.
tem level or above, that is, QI or safety efforts to increase value Choosing Wisely collaborated with the AAP to identify 10 tests or
for multiple patients. treatments that pediatricians should question (Box 11.1).

Box 11.1. Ten Things Physicians and Patients Should Question

1. Antibiotics should not be used for viral respiratory illnesses (sinusitis, Minor head injuries occur commonly in children and adolescents.
pharyngitis, bronchitis and bronchiolitis). Antibiotics should not be used Approximately 50% of children who visit hospital emergency departments
for upper respiratory illnesses characterized by congestion, cough, or pharyn- with a head injury are given a CT scan, many of which may be unneces-
geal pain unless criteria for bacterial sinusitis or Group A streptococcal pharyn- sary. Unnecessary exposure to x-rays poses considerable danger to children
gitis are met. The vast majority of these infections are caused by viruses. including increasing the lifetime risk of cancer because a child’s brain tissue
Respiratory infections account for the majority of antibiotic prescrip- is more sensitive to ionizing radiation. Unnecessary CT scans impose undue
tions for children, and it is estimated that 50% of antibiotic prescriptions costs to the health care system. Clinical observation prior to CT decision-
for respiratory infections in children are unnecessary. Antibiotic use for making for children with minor head injuries is an effective approach.
viral respiratory illnesses not only leads to higher healthcare costs and 4. Neuroimaging (CT, MRI) is not necessary in a child with simple
more adverse events, but also can lead to antibiotic resistance. febrile seizure. Imaging, including head CT, brain MRI, and skull films are
2. Cough and cold medicines should not be prescribed, recom- associated with some risk and do not help with diagnosis or treatment of
mended, or used for respiratory illness in young children. Research simple febrile seizures. MRI is associated with risks from required sedation
has shown these products offer little benefit to young children and can and high cost. Head CTs can slightly increase the long-term risk for cancer.
have potentially serious side effects. Many cough and cold products 5. Computed tomography (CT) scans are not always necessary in the
for children have more than one ingredient, increasing the chance of routine evaluation of abdominal pain. CT imaging in the emergency
accidental overdose if combined with another product. department evaluation of children with abdominal pain is frequent and can
3. Computed tomography (CT) scans are not necessary in the imme- be inconsistently used, including overused. While radiation is necessary to
diate evaluation of minor head injuries; clinical observation/ perform a CT scan, there is both misunderstanding and often concern about
Pediatric Emergency Care Applied Research Network (PECARN) the radiation necessary and the debate over the potential long-term
criteria should be used to determine whether imaging indicated. development of cancer from this radiation. There also is the potential for

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 Chapter 11: Health Systems Science 69

Box 11.1. Ten Things Physicians and Patients Should Question (continued)
an unnecessary amount of radiation from inappropriately performed CT medication in the so-called “happy-spitter.” There is scant evidence
examinations, as there are unique approaches and considerations with CT that gastroesophageal reflux (GER) is a causative agent in many conditions
examinations in children that allow for lower radiation doses. CT can be though reflux may be a common association. There is accumulating evidence
very valuable in the setting of pediatric abdominal pain, but only when it is that acid-blocking and motility agents such as metoclopramide (generic)
the correct test to do at the time (as opposed to waiting, or using another are not effective in physiologic GER. Long-term sequelae of infant GER is
test that does not depend on ionizing radiation especially ultrasound), and rare, and there is little evidence that acid blockade reduces these sequelae.
performed in the right way (child-sized CT techniques). The routine performance of upper gastrointestinal (GI) tract radiographic
6. Don’t prescribe high-dose dexamethasone (0.5 mg/kg per day) for imaging to diagnose GER or gastroesophageal disease (GERD) is not justified.
the prevention or treatment of bronchopulmonary dysplasia in pre- Parents should be counseled that GER is normal in infants and not associated
term infants. High-dose dexamethasone (0.5 mg/kg day) does not appear with anything but stained clothes. GER that is associated with poor growth
to confer additional therapeutic benefit over lower doses and is not recom- or significant respiratory symptoms should be further evaluated.
mended. High doses also have been associated with numerous short- and 9. Avoid the use of surveillance cultures for the screening and treat-
long-term adverse outcomes, including neurodevelopmental impairment. ment of asymptomatic bacteriuria. There is no evidence that surveil-
7. Don’t perform screening panels for food allergies without previ- lance urine cultures or treatment of asymptomatic bacteriuria is beneficial.
ous consideration of medical history. Ordering screening panels (IgE Surveillance cultures are costly and produce both false positive and false
tests) that test for a variety of food allergens without previous consider- negative results. Treatment of asymptomatic bacteriuria is harmful and
ation of the medical history is not recommended. Sensitization (a posi- increases exposure to antibiotics, which is a risk factor for subsequent
tive test) without clinical allergy is common. For example, about 8% of the infections with a resistant organism. This also results in the overall use of
population tests positive to peanuts but only approximately 1% are truly antibiotics in the community and may lead to unnecessary imaging.
allergic and exhibit symptoms upon ingestion. When symptoms suggest a 10. Infant home apnea monitors should not be routinely used to prevent
food allergy, tests should be selected based upon a careful medical history. sudden infant death syndrome (SIDS). There is no evidence that the use
8. Avoid using acid blockers and motility agents such as metoclo- of infant home apnea monitors decreases the incidence of SIDS and should not
pramide (generic) for physiologic gastroesophageal reflux (GER) be used routinely for this purpose; however, they might be of value for selected
that is effortless, painless, and not affecting growth. Do not use infants at risk for apnea or cardiovascular events after discharge.

Reprinted with permission from American Academy of Pediatrics. Ten things physicians and patients should question. ChoosingWisely.org website. www.choosingwisely.org/societies/american-academy-
of-pediatrics.

Summary and Future Challenges

in Health Systems Science CASE RESOLUTION
You respond to the family’s request for imaging using the guidance of the American
The knowledge, skills, and perspectives needed by pediatricians College of Physicians for these conversations. You determine the perspectives of Sara
to effectively improve and maintain the health of children will and her mother (“What are you afraid we will find?” and “What do you think is going
continue to rapidly change in the coming decades, and health on, and what are you worried about?”); explain your reasoning for not recommend-
professionals must be able to envision the need for basic science, ing imaging (“The good news is that you do not have any worrisome symptoms.”);
clinical science, and HSS to help all children realize their potential. make it clear that you are on Sara’s side (“I wish more testing could help you, but it
can actually make things worse.”); and make a clear follow-up plan and review red
The pace of change in pediatric trainee education and continuing
flag signs and symptoms (“I want to see you in 2 weeks, but contact us sooner if there
professional development must align with that of the practice and are changes that concern you.”).
society. The professional identity of pediatricians and other health With your understanding of HSS-related concepts, such as social determinants of
professionals must focus on caring for patients and their families, health and how system issues can negatively affect health, you work with your clin-
for populations of patients, and for the system. Advancements in ical (ie, microsystem) team to connect Sara’s mother to a community health worker.
medical education concerning best practices in HSS education (for The health worker meets with Sara and her family in their home to learn more about
some of the challenges they are experiencing in accessing basic necessities as well
undergraduate medical education, graduate medical education, and as the health care system. The health worker connects Sara’s mother to a primary
continuing professional development) must evolve in a way that is care provider to help her address her own chronic illnesses and helps Sara’s family
authentic and meaningfully integrated with the work of interpro- access county services for housing and food. Your clinical team decides to initiate
fessional care teams. Finally, health services research, a topic that a QI project with the goal of reducing the number of missed follow-up visits for
is beyond the scope of this chapter, must continue to elucidate best pediatric patients. In the course of the project, your team develops a care manage-
ment approach for children with multiple chronic diseases. As part of your success-
means of ensuring that high-value care occurs the first time, every
ful project, you begin screening for adverse childhood experiences to better recognize
time in a manner that complements the context, preferences, and their effect on health, and several colleagues in your practice share what you have
values of the patient and family. learned with the local health department and school system.

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 70 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Hoffmann TC, Montori VM, Del Mar C. The connection between evidence-based
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 CHAPTER 12

Population Health
for Pediatricians
Michael Weiss, DO, FAAP

CASE STUDY
You are preparing to see a patient familiar to your prac- you receive a monthly capitation payment for her care in
tice for an acute visit. You have not seen her for 11 months addition to potential value-based incentives around cer-
and, as you review the chart, you recall that the child is tain quality indicators.
5 years old and was born with a myelomeningocele at
L4-5. She underwent surgery as an infant with place-
Questions
1. What are the specific challenges associated with
ment of a ventriculoperitoneal shunt and gastrostomy
caring for this child?
tube. She has used a motorized wheelchair for 2 years
2. How do you begin to organize her multiple special
and requires intermittent urinary bladder catheteriza-
needs?
tion. Generally, she has done well and continues to see
3. What are the clinical implications of the methodology
specialists in gastroenterology, neurosurgery, neurology,
by which you are paid for her care?
and urology. She attends public school, where she has
4. What strategies can you use to ensure this child
an Individualized Education Program; receives occupa-
receives the entirety of care required for her to
tional, speech, and physical therapy; and qualifies for
thrive?
in-class assistance. On further review, you note she is
5. Who is your team?
insured by your local Medicaid managed care plan and

Population Health: What and Why? of the main population health concepts and facilitates the successful
Population health has been defined in many ways. In simple terms, navigation of the complex health care system, helping pediatricians
population health is about keeping a defined population healthy provide improved care for their patients and families.
through proactive, preventive measures that minimize fragmented, In the frequently quoted works, To Err Is Human and Crossing the
inefficient care and improve clinical outcomes while reducing the Quality Chasm, the Institute of Medicine (now known as the Health
overall cost of care. and Medicine division of the National Academies) brought to light the
During pediatric training and throughout their career, pediatri- prevalence of preventable medical errors and the disparity in quality
cians appropriately spend most of their time developing and refining of care across the United States. These reports estimated that 44,000
the knowledge and technical skills required to provide high-quality to 98,000 preventable deaths annually at a cost between $17 billion
care to patients. Pediatricians learn about disease, enhance com- and $29 billion attributable to lost income and additional care neces-
munication skills, and learn procedures, such as endotracheal sitated by errors. Further, Institute of Medicine concluded that “The
intubation, lumbar puncture, and intravenous catheter placement U.S. health care delivery system does not provide consistent, high-
techniques. quality medical care to all people.” In 2009, Atul Gawande, MD,
Over the past 3 to 4 decades, increasing emphasis has been placed MPH, a Boston-based surgeon and health policy researcher, pub-
on understanding how effective and efficient our delivery of health lished “The Cost Conundrum,” in which he brought to light the wide
care is and how we measure these outcomes. This new focus filters variation in health care spending and outcomes across the United
down to frontline pediatricians, who must develop a clear under- States. For 1 region in south Texas, he pointed to nearly double
standing of much more than clinical care. Pediatricians need to the national average per-capita spending on health care with no
understand health care payment methodologies, clinical quality demonstrable improvements in quality versus the rest of the coun-
metrics, care model design improvements, and care coordination try. This, along with data from the Commonwealth Fund (Figures
programs. A fundamental understanding of these concepts is now 12.1 and 12.2), brought to the public eye that the trajectory of health
a vital component of successfully caring and advocating for infants, care spending in the United States was unsustainable and, more
children, adolescents, and young adults. This chapter reviews some importantly, that patients were not receiving better-quality care
73

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 74 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

25 Public
Private
20

15
Percent

10

0
1960 1970 1980 1990 2000 2005 2010 2015 2020
Data: Centers for Medicare and Medicaid Services, Office of the Actuary.
Note: GDP = gross domestic product.

Figure 12.1. Growth in health spending as a share of the economy.

Reprinted with permission from Lambrew JM. Getting ready for health reform 2020: what past presidential campaigns can teach us. The
Commonwealth Fund website. www.commonwealthfund.org/publications/fund-reports/2018/jun/getting-ready-health-reform-2020-
presidential. Published June 26, 2018. Accessed August 22, 2019.

Higher
health system AUS UK
performance NETH
NOR
NZ
GER SWIZ

Eleven-country average SWE

CAN

FRA

US

Lower
health system
performance
Lower health care spending Higher health care spending
Note: Health care spending as a percent of GDP.
Source: Spending data are from OECD for the year 2014, and exclude spending on capital formation of health care providers.

Figure 12.2. Health care system performance compared with spending.

Reprinted with permission from Schneider EC, Sarnak DO, Squires D, Shah A, Doty MM. Mirror, mirror 2017: international comparison reflects
flaws and opportunities for better U.S. health care. The Commonwealth Fund website. www.commonwealthfund.org/publications/fund-
reports/2017/jul/mirror-mirror-2017-international-comparison-reflects-flaws-and. Published July 14, 2017. Accessed August 22, 2019.

as a result of the spending. Subsequently, the notion of the Triple Health Care Payment Methodology
Aim of better health for populations, better care experiences, and and Practice
lower per-capita costs was introduced by the Institute for Healthcare
Improvement (and more recently enhanced by others to become the A basic understanding of the funding stream for the care patients
Quadruple Aim by adding joy of practice). The concept of account- receive is an important step toward improving quality, care coordi-
able care organizations then evolved from the Patient Protection nation, and clinical outcomes. By far, the largest payers for children’s
and Affordable Care Act. This framework has served as the template health care in the United States are Medicaid and the Children’s
for population health work across the country, in which the focus is Health Insurance Program (CHIP), which cover nearly 30 million
on caring for populations of patients with proactive, team-based and 9 million children, respectively. Children qualify for the pro-
care, using accurate and timely data to effect positive clinical and grams based on the income level and the number of members in
service-based outcomes (see Chapter 21). their family. Qualifications vary by state and are usually based on a

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 Chapter 12: Population Health for Pediatricians 75

percentage of the federal poverty level. CHIP serves as a supplement The bottom line for pediatricians is to take the initiative to recog-
to assist those families who may not qualify for Medicaid. Coverage nize the specifics of how their population of patients is being funded
for basic care, called early and periodic screening, diagnosis, and and implement strategies to ensure clinical and operational success.
treatment, or EPSDT, such as immunizations, developmental screen- This understanding allows pediatricians to provide the best possi-
ing, and health maintenance visits, is mandated by these programs. ble care for patients, advocate for appropriate and comprehensive
Private insurers make up most of the remaining payers. Private pediatric-specific insurance coverage, and meet the business
payment may occur through a preferred provider organization (PPO) requirements of a practice.
or a health maintenance organization (HMO). With a PPO plan,
patients are usually able to access any health professional covered by Understanding Quality Metrics
their insurance without the need for a specific referral that would
Defining clinical quality in medicine has been a long-standing
be reviewed for clinical necessity in an HMO. High-level procedures
challenge. It has been defined as identifying the correct diagnosis
and interventions may still require approval before they can be com-
and initiating appropriate treatment that results in resolution of
pleted (eg, magnetic resonance imaging scans, surgeries). In an HMO
the condition. It has also been defined as the receipt of a prompt
plan, patients are encouraged to access their chosen or assigned pri-
appointment with the physician of choice in a friendly, welcoming
mary care professional (PCP) for most conditions. If the PCP believes
environment, followed by timely communication and follow-up.
specialty care is required, an authorization request is generated and
Both outcomes are part of the quality spectrum because clinical
reviewed by the health plan for medical necessity before the patient
success and service excellence are dually important.
can access the specialty care. Much debate exists over the preferred
The other challenge has been how to identify pediatric-
approach, as each has positive and negative components. The PPO pro-
specific metrics that are clinically meaningful and objectively
vides more freedom of choice and ease of access, while the HMO cre-
measured, and which interventions by the physician responsible
ates an accountability and actionable data stream to the PCP of who
for the patient can effect positive change. Measures have evolved
serves as the medical home for patients. Data from regions where
over time from process focused to outcome focused, with new
HMOs have been prevalent for some time show that quality and cost
emphasis on patient-reported outcome measures (Table 12.1).
containment are enhanced in the HMO environment.
Clearly, the emphasis of various interventions is on the ultimate
Within these insurance coverages there are also a variety of
benefit to the patient, rather than completion of the interven-
methods whereby payment to health professionals and hospitals
tion itself.
can be made. In the traditional approach, known as fee for service,
a health professional is paid based on a prearranged fee schedule
for each encounter that occurs. Bundled payments, which are less
Table 12.1. Categories of Quality Metrics
common in pediatrics (particularly in the ambulatory setting), are
set payments that are made for episodes of care. For example, for Measure
a joint replacement procedure, a bundled payment would include Type Description Example
preoperative evaluation, surgery (including any required hardware), Structure Sufficiency of resources Proper use of a certified EHR
and postoperative care, including physical therapy and any durable and proper system design
medical equipment. Process Assesses the interaction Completing a HbA1c test for a
In the new accountable care paradigm, models involving between the patient/fam- patient with diabetes
population-based payment, also known as capitation, are much ily and practitioner. Completing a scheduled health
more prevalent. In these models, health professionals are paid a fixed Describes the means supervision visit
per-member, per-month fee for caring for their patients. One advan- by which services are Use of evidence-based guidelines
tage of this methodology is that patient attribution is very clear delivered
(addressed later in this chapter). Regardless of the number of times
Outcome Assesses the effect the care Number of hospitalizations
a patient is seen, the monthly payment for services is fixed. This
delivered has on clinical for a specific condition
model is aligned with population health principles in that preven-
outcomes (eg, asthma)
tion and proactive care are emphasized to create better quality and
Patient- Status of a patient’s health School absenteeism
keep health care costs in check. In these models, services such as
reported condition that comes Ability to participate in
immunizations, mental health, and injectable medications are often
outcome directly from the patient typical social activities or
carved out of the monthly payment and paid for on a fee-for-service
or family sporting events
basis due to the high cost involved.
As a complement to these payment methodologies, especially with Patient Patient or parent ques- CAHPS survey
a population-based payment approach, value-based care is being uni- experience tionnaires addressing their Can be hospital focused or
versally incorporated. This usually involves additional financial incen- experience with the care ambulatory physician focused
tives for demonstrating clinical quality outcomes and appropriate they received
resource use for a defined population for which the health professional Abbreviations: CAHPS, Consumer Assessment of Healthcare Providers and Systems; EHR,
is responsible. (Quality metrics are explored later in this chapter.) electronic health record; Hb, hemoglobin.

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 76 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

There are several evidence-based, nationally accepted pediat- satisfaction). Typically, patients receive a written or telephone-
ric Healthcare Effectiveness Data and Information Set (HEDIS) based survey to assess the perceived level of care they received.
measures that are endorsed by the National Committee for Quality The standardized Consumer Assessment of Healthcare
Assurance, National Quality Forum, Pediatric Quality Measure Providers and Systems Clinician and Group (CG-CAHPS) sur-
Program, and others. Each measure shares a similar format that vey is most commonly employed. Questions cover multiple
includes a description of the clinical issue being addressed, patient domains that address service, timeliness of care, communica-
inclusion and exclusion criteria, and documentation and coding tion, and shared decision making. A representative CG-CAHPS
requirements (Table 12.2). These measures are often part of value- question is: “Did your provider explain things in a way that was
based payment programs sponsored by health plans and organized easy to understand?”
medical groups and, in the information superhighway era, may be Greater emphasis in this area is demonstrated by the fact that
cited in the public domain to compare physicians, hospitals, and most value-based programs include patient and family experience
medical groups to national or local benchmarks. as up to 30% of the overall rating of a health professional.
Quality metrics are not without limitations. For almost every Understanding these metrics, how they can be used to improve
measure there are certain nuances that may pose potential chal- care, and how to address the technical and operational challenges
lenges. For example, in the Appropriate Treatment of Children With associated with them is now an imperative for pediatricians in all
Upper Respiratory Infection (URI) measure (see Table 12.2), a child disciplines. These metrics have additional utility, as they relate to
may be seen by the primary care physician, diagnosed with a viral office-based quality improvement activities and Maintenance of
URI, and appropriately not given an antibiotic. Later the same day, Certification.
the family may seek care by another primary care physician, who
prescribes an antibiotic, and the child is now viewed as out of com- Care Coordination Fundamentals
pliance with the measure. The physician with accountability acted In 1967 the American Academy of Pediatrics first described the
appropriately but does not receive the correct credit for doing the notion of the patient-centered medical home. Subsequently, in
right thing. 2007, a joint statement endorsing the medical home concept was
A second general category of quality metrics revolves around published by the American Academy of Pediatrics, American
appropriate use of clinical resources. Measures in this domain typ- Osteopathic Association, American College of Physicians, and
ically include the frequency of emergency department (ED) visits, American Academy of Family Physicians. The patient-centered
use of high-cost imaging studies (ie, magnetic resonance, computed medical home concepts were updated in 2017 (Box 12.1). Cooley
tomography), or inpatient admissions for so-called ambulatory- et al cited enhanced pediatric medical home capabilities as
sensitive conditions. For instance, ED visits for asthma may be pre- a harbinger of improved quality and lower cost of care. With
ventable if a child is appropriately prescribed an inhaled cortico- the increasing demands of electronic health records (EHRs),
steroid, educated on proper spacer use, and given an asthma action quality metric performance, and other administrative duties,
plan. Appropriate resource metric results are typically compared understanding and implementing appropriate care model design
with regional or national benchmarks. principles into one’s practice is a necessity. Four such concepts
Another category of quality metrics revolves entirely around are patient attribution, risk stratification, use of data and ana-
patient and family experience (previously referred to as patient lytics, and the care team.

Table 12.2. Example Healthcare Effectiveness Data and Information Set Measure Specifications
HEDIS Measure Description Specification Definition ICD-10-CM Codesa
Appropriate Treatment Measure evaluates the percentage of wwPatients with an outpatient or ED wwAcute nasopharyngitis wwJ00
of Children With Upper children aged 3 months to 18 years visit with a single diagnosis of URI wwAcute laryngopharyngitis wwJ06.0
Respiratory Infection (URI) who were given a diagnosis of URI and not prescribed an antibiotic on, wwAcute URI wwJ06.9
and were not dispensed an antibiotic or 3 days after, the date of the URI
prescription. diagnosis.
wwEnsure any secondary diagnoses
indicating the need for an antibiotic
are submitted on the claim.
Well-Child Visit 3–6 Years Measure documents at least 1 well- Visit includes a health and develop- Services specific to an wwZ00.110
(WC34) child (health supervision) visit with mental history, physical examination, acute or chronic condition wwZ00.01
the primary care physician during the health education, and anticipatory do not count toward this wwZ00.121
measurement year. guidance. measure. wwAdditional codes
a
Codes subject to change; current as of 2020.
Abbreviations: ED, emergency department; HEDIS, Healthcare Effectiveness Data and Information Set; ICD-10-CM, International Classification of Diseases, 10th Revision, Clinical Modification; URI, upper
respiratory infection.

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 Chapter 12: Population Health for Pediatricians 77

pharmacy benefit managers, or other sources of aggregated informa-

Box 12.1. 2017 National Committee for
tion. The health professional should be aware that these data are often
Quality Assurance Patient-Centered Medical
claims based, and there may be a significant lag from the date of care
Home Concepts
to the actual report. Using this information facilitates proactive, pre-
ww Team-based care and practice organization ventive care and offers the ability to assess quality improvement activ-
ww Knowing and managing your patients ities. Examples of this type of data include HEDIS performance, ED
ww Patient-centered access and continuity and inpatient utilization by patients, and appropriate use of antibiot-
ww Care management and support ics. Accessing timely, accurate data can be very challenging. It is highly
ww Care coordination and care transitions recommended that pediatricians develop partnerships with payers,
ww Performance measurement and quality improvement hospitals, regional health information exchanges, and large physician
groups to help aggregate data whenever possible. This approach also
Adapted from National Committee for Quality Assurance. NCQA PCMH Recognition: Concepts. allows pediatricians to compare their performance with national and
National Committee for Quality Assurance. www.ncqa.org/programs/health-care-providers-
regional benchmarks.
practices/patient-centered-medical-home-pcmh/pcmh-concepts/.

The Care Team

Patient Attribution The pediatrician should be focused on diagnostic skills, understand-
A foundational component of care coordination is knowing the ing and implementing appropriate therapies, and nurturing a strong
patients a pediatrician is responsible for. This knowledge allows for and trusted relationship with patients and families. Unfortunately,
proactive, rather than reactive, approaches to care. For example, know- the literature clearly demonstrates that physicians are spending
ing which children in the practice have not yet received an appropriate more time in activities unrelated to these important competencies.
immunization or timely health supervision visit allows the pediatri- Charting in complex EHRs, filling out forms, calling different clini-
cian to reach out to schedule such care. Attribution is facilitated by cians to coordinate care, seeking medical records, and returning low-
the population-based payment paradigm, as compensation is tied to level calls are a few of the activities that dominate a pediatrician’s day.
a defined population for which the pediatrician is clinically and finan- How can this be adjusted to work for the patient and the physician?
cially responsible. In situations in which children are migrating among In recent years, production improvement programs, such as Taiichi
various physicians, attribution may be further complicated, and vari- Ohno’s Toyota production system, which inspired Lean Manufacturing
ous methods have been devised to assist with this process. In such sit- in the United States, have been applied, very successfully, to medical
uations, most attribution models emphasize the frequency of primary care. The concept is to avoid waste in all its forms (eg, time), create
care health supervision visits to a single pediatrician as the best predic- standard work for all members of the team, and ensure each person
tor of responsibility for clinical outcomes for a given child. Attribution is working to the height of their respective training and licensure. (An
need not be dictated by the health plan; internal practice-based attri- in-depth review of Lean methodology is beyond the scope of this chap-
bution can also be a successful approach. The important point is that ter but highly recommended for additional reading.) For example, a
a pediatrician must know their population to be effective. medical assistant should be filling out certain forms under the direc-
tion of the medical team, rather than having licensed personnel per-
Risk Stratification forming that task. Likewise, a physician should not be searching for
Once a population is defined, the next step is to stratify the patients medical records or making copies.
to focus efforts on the areas of greatest need. Patients can be strat- The correct application of care team models creates a well-
ified by age, medical condition, certain clinical gaps in care, or any delineated set of roles with clear understanding of who the respon-
other medical or psychosocial determinant. Multiple risk stratifica- sible team member is for each task. The team acts proactively and
tion tools have been identified, but their use is limited in the primary anticipates the needs of the patient. Approaches such as huddles
care office setting. More commonly, health risk assessment ques- and visual performance boards facilitate this proactive approach and
tionnaires are employed for this purpose. High-risk patients can be highlight clinical and service goals and outcomes.
aggregated and assigned to care coordinators, where available, for
close follow-up and care plan oversight. For example, patients in
a practice with persistent asthma can be identified, and their care
Putting It All Together
can be reviewed to ensure they are prescribed appropriate inhaled The role of the pediatrician has expanded in recent years. Pediatricians
corticosteroids. This risk stratification process does not omit atten- must provide the best clinical care possible and are now required to
tion to the multiple well children in the practice who require ongo- understand a multitude of payment models, quality metrics, and data
ing proactive anticipatory guidance, immunizations, and education. sources that facilitate such care. A failure to incorporate this broader
knowledge base into daily practice can adversely affect outcomes for
Use of Data and Analytics patients. As advocates for children, the more pediatricians can under-
Incorporating the use of data and analytics into practice is essential. stand about this new paradigm, the more successful they can be in
These data sources may include health plans, government payers, such ensuring that infants, children, adolescents, and young adults are
as Medicaid, hospitals, EHRs, regional health information exchanges, provided the most efficient quality care possible.

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 78 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Gawande A. The cost conundrum. The New Yorker. June 1, 2009

CASE RESOLUTION Gilchrist-Scott DH, Feinstein JA, Agrawal R. Medicaid managed care structures
Your medical assistant performs a pre-visit chart review that includes identifica- and care coordination. Pediatrics. 2017;140(3):e20163820 PMID: 28838950
tion of any potential gaps in care, recent hospitalizations or ED visits, review of https://doi.org/10.1542/peds.2016-3820
specialty notes for updated medication doses, pending laboratory tests or diag- Hudak ML, Helm ME, White PH; American Academy of Pediatrics Committee
nostic testing, and immunization records. The school Individualized Education on Child Health Financing. Principles of child health care financing.
Program is reviewed to ensure therapies are occurring at the appropriate Pediatrics. 2017;140(3):e20172098 PMID: 28864710 https://doi.org/10.1542/
frequency with associated progress reports demonstrating improvement. During peds.2017-2098
your morning huddle with your care team, you determine that the child has seen
Institute of Medicine Committee on Quality of Health Care in America. Crossing
2 of her subspecialists since she last visited your office, so your team updates
the Quality Chasm: A New Health System for the 21st Century. Washington, DC:
her medication list and overall care plan to reflect the latest information. You
National Academies Press; 2001
also determine that she is overdue for a health supervision visit, and your staff
converts her acute visit today to a such a visit to include her annual influenza Integrated Healthcare Association. California Regional Health Care Cost and
vaccine. You note that she has a registered nurse who acts as her case manager, Quality Atlas. https://atlas.iha.org. Accessed August 22, 2019
who is provided by her Medicaid managed care program, and ensure that your Kohn LT, Corrigan JM, Donaldson MS, eds. To Err Is Human: Building a Safer
team connects with the case manager and social worker to help coordinate care Health System. (Committee on Quality of Health Care in America, Institute of
and address any social determinants of health that may pose a challenge. During Medicine). Washington, DC: National Academies Press; 2000
her visit you include the family in all decision making about her care and pro-
vide a written summary of the visit and a specific action plan. Your office securely Lambrew JM. Getting ready for health reform 2020: what past presidential cam-
messages the family in 48 hours to reinforce the care plan and ask if there are paigns can teach us. The Commonwealth Fund website. www.commonwealthfund.
any other questions. org/publications/fund-reports/2018/jun/getting-ready-health-reform-2020-
presidential. Published June 26, 2018. Accessed August 22, 2019
Medicaid.gov. www.medicaid.gov. Accessed August 22, 2019
Schneider EC, Sarnak DO, Squires D, Shah A, Doty MM. Mirror, mirror: inter-
Selected References
national comparison reflects flaws and opportunities for better U.S. health care.
Adirim T, Meade K, Mistry K; American Academy of Pediatrics Council on The Commonwealth Fund website. www.commonwealthfund.org/publications/
Quality Improvement and Patient Safety, Committee on Practice and Ambulatory fund-reports/2017/jul/mirror-mirror-2017-international-comparison-reflects-
Management. A new era in quality measurement: the development and appli- flaws-and. Published July 14, 2017. Accessed August 22, 2019
cation of quality measures. Pediatrics. 2017;139(1):e20163442 PMID: 28025242 Simon TD, Cawthon ML, Stanford S, et al; Center of Excellence on Quality of Care
https://doi.org/10.1542/peds.2016-3442 Measures for Children with Complex Needs (COE4CCN) Medical Complexity
Baron RJ. What’s keeping us so busy in primary care? A snapshot from one Working Group. Pediatric medical complexity algorithm: a new method to strat-
practice. N Engl J Med. 2010;362(17):1632–1636 PMID: 20427812 https://doi. ify children by medical complexity. Pediatrics. 2014;133(6):e1647–e1654 PMID:
org/10.1056/NEJMon0910793 24819580 https://doi.org/10.1542/peds.2013-3875
Cooley WC, McAllister JW, Sherrieb K, Kuhlthau K. Improved outcomes associ-
ated with medical home implementation in pediatric primary care. Pediatrics.
2009;124(1):358–364 PMID: 19564320 https://doi.org/10.1542/peds.2008-2600

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 CHAPTER 13

Principles of Pediatric
Therapeutics
Bonnie R. Rachman, MD

CASE STUDY
An 18-month-old girl who has had a cough, runny nose, Questions
and fever for 2 days is brought to your office for evalua- 1. What are the current clinical practice guidelines
tion. The previous night she awoke from sleep crying and for antibiotic treatment of otitis media? How does
pulling at her ear. The patient has no other symptoms. treatment change with the age and symptoms of
Her mother states she has had previous ear infections; the patient?
the most recent occurred 2 months ago. The last time she 2. How does the previous reaction to a medication
took amoxicillin she broke out in hives. Otherwise, the influence the antibiotic choice?
patient has no significant medical history. 3. How do factors (eg, parental work, child care) affect
On physical examination, the patient is febrile with administration of the medication?
a temperature of 38.9°C (102°F) and has yellow rhinor- 4. What role do over-the-counter medications have in
rhea. The ear examination reveals a red, bulging, non- the management of the patient’s symptoms?
mobile tympanic membrane in 1 ear, while the other
tympanic membrane appears normal. The remainder of
the examination is benign.

A drug or therapeutic can be defined as any substance that is for allergies or pain relief. Given that the use of OTC medications is
ingested, absorbed, or injected that alters the body’s function. so prevalent and that many OTC medications contain multiple active
Examples include prescription medications, over-the-counter (OTC) ingredients, concerns about their safety profile for children is an issue
medications, homeopathic preparations, herbal remedies and teas, that needs to be addressed by pediatricians with families at routinely
vitamin supplements, and illicit substances. Different drug catego- scheduled office visits as well as by regulatory agencies.
ries have differing safety profiles and regulation levels. Prescription Another concern is the use of prescription or OTC medications as
drugs are the most regulated. Homeopathic preparations, some drugs of abuse. Of particular concerns are adolescents who use alco-
vitamin formulations, and most herbal remedies may have little, if hol, illicit drugs, and medications, including OTC cough medications
any, standardization, safety testing, or regulation. Illicit drugs have containing dextromethorphan. In the 2017 Monitoring the Future
no quality testing. survey of 8th, 10th, and 12th graders nationwide, approximately
The use of prescription and OTC medications is very common in 3% of the survey participants reported using OTC cough medicine
pediatrics. The Slone Survey, a random digit-dial survey of medica- to “get high” in the past year. Also, in the past year, 49% reported
tion use, found that families with children reported that more than use of alcohol, 28% reported use of marijuana, 61.3% reported use
55% of children younger than 12 years had taken some medication of Vicodin, and 1.9% reported use of oxycodone.
preparation within the last 7 days. Of those taking a medication, 22% Furthermore, according to the Centers for Disease Control and
were taking at least 1 prescription medication. In a given month, Prevention, each year more than 60,000 children are brought to the
more than 50% of preschool-age children had received some OTC emergency department (ED) for medication overdoses; OTC med-
medication. In 2011 to 2012, 7.5% of US children and teenagers aged ications were implicated in more than 26,000 visits. Ninety percent
6 to 17 years took medication for emotional or behavioral difficulties. of ED visits for medication overdoses resulted from unsupervised
The number of children aged 12 years and younger being adminis- ingestions of prescription and OTC drugs, with peak incidence in
tered an OTC medication in a given period is more than twice that of the younger-than-5-year age group.
a prescription medication. The most commonly used OTC medica- Most drugs prescribed for children have not been tested in
tions are acetaminophen and ibuprofen. In the adolescent age group, children. Before the US Food and Drug Administration (FDA)
aged 12 to 17 years, more OTC products are used for acne and less initiated a pediatric program (Best Pharmaceuticals for Children

79

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 80 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Act of 2002 [amended in 2007]), only about 20% of drugs approved

Box 13.1. Factors in Choosing
by the FDA were labeled for pediatric use. Currently, about one-
Appropriate Therapeutics
third of drugs prescribed to children have been studied for safety
and efficacy in pediatric populations. The practice of prescribing Patient Characteristics
drugs for off-label conditions is found in approximately 50% of all ww Age
physician prescriptions. By necessity, doctors have routinely given ww Medical history
drugs to children off-label, which means the drug has not been ww Allergies
approved for use in children based on the demonstration of safety ww Use of other medications
and efficacy in adequate, well-controlled clinical trials. It does not Disease Epidemiology
imply that the drugs are unsafe, improper, or illegal. The Pediatric ww Epidemiology
Research Equity Act of 2003 (amended in 2007) requires pediat- ww Age-specific factors
ric studies and covers drugs and biologics. Under the Pediatric
Safety Profile
Research Equity Act, the FDA can require pediatric studies of a
ww Therapeutic index
drug submitted in a new drug application if it determines the prod-
ww Black box warning
uct is likely to be used in a substantial number of pediatric patients
or if the product would provide a meaningful benefit in the pedi- Patient Compliance
atric population over existing treatments. In a systematic review ww Taste
of the frequency of off-label use of antibiotics in clinical pediatric ww Purpose of treatment
practice, the percentage of off-label antibiotic prescriptions varied ww Storage
from 1% to 94%. Some of the wide variation observed in pediatric ww Side effects
patients might be attributed to the heterogeneity among the study Cost-effectiveness
populations’ ages of children. ww Drug availability
Off-label use creates a liability for the physician. The burden of ww Formulary restrictions
an adverse outcome is on the manufacturer or license holder when
there is an on-label use of a medication and an iatrogenic injury
is sustained. When the medication is prescribed off-label, legal removed. Children with intellectual disability who are given central
liability is on the prescriber unless parents and/or guardians are nervous system–altering medications can be difficult to monitor
appropriately informed and give their consent. for changes in mental status seen with systemic infection. In addi-
Differences in pharmacokinetics among infants, children, and tion, children with glucose-6-phosphate dehydrogenase deficiency
adolescents can result in differences in drug efficacy and toxicity. can suffer from drug-induced hemolytic anemia when given cer-
To choose the most important therapeutic, health care profession- tain drugs, such as sulfonamides. Children who are malnourished
als must consider a number of factors, including patient character- are at greater risk for drug toxicity because low serum albumin
istics, disease epidemiology, safety profile, patient compliance, and levels decrease the amount of bound drug and result in increased
cost-effectiveness (Box 13.1). levels of unbound drug in circulation.
Allergies to medications and reactions to prior drugs are impor-
Patient Characteristics tant pieces of information to glean from the patient and family.
Infants and children have different physiological characteristics Specific information about symptoms or problems while taking a
than adults, including immaturity of metabolic and organ func- drug are of great significance because side effects can be mislabeled
tion. Body weight and surface area are considerations in drug dosing as drug allergies. Drug allergies are reactions that have an immuno-
for pediatrics. In addition, the therapeutic window for many drugs is logic basis. Of the 4 types of immune mechanisms associated with
smaller for children than adults. Pharmacological factors, including drug allergies, immunoglobulin (IgE)-mediated reactions are of the
age-based variability in absorption, metabolism, and excretion of greatest concern because they can result in anaphylaxis. Adverse reac-
drugs in children compared with adults, as well as age-specific con- tions, such as vomiting, can be idiosyncratic or nonimmune medi-
traindications of certain medications, pose special vulnerabilities for ated. A side effect is an expected but undeniable consequence of
children to the adverse effects of overdosing. Conversions of doses taking a medication. Families often attribute any symptoms a patient
from ingredient amounts to volumes for liquids labeled for home experienced while taking a medication to a drug allergy. It is, there-
use are also problematic. fore, important to know which side effects are associated with a med-
The patient’s medical history plays an important part in the ication to determine whether an allergy is present. Any medication
choice of an appropriate therapeutic. Depending on the patient’s that has been associated with an allergic reaction (eg, skin eruptions,
condition and medical and surgical history, the absorption, metabo- swelling, urticaria, respiratory difficulty) should not be used with-
lism, or usual effectiveness of a therapeutic may be altered. Children out consulting a specialist.
with short bowel syndrome can potentially have difficulty absorb- Also important is to consider what other medications the patient
ing oral medications depending on which portion of the bowel was is taking. This may influence the drug of choice. Metabolism of 1

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 Chapter 13: Principles of Pediatric Therapeutics 81

drug may influence the metabolism of another. Drug metabolism of the medication but requires the physician and family to evaluate
is divided into 2 types. Phase 1 reactions include oxidation, reduc- the risks and benefits of its use.
tion, and hydrolysis. Phase 2 reactions involve adding subgroups
to a drug. Patient Compliance
Other drugs can affect reactions during either phase. For example, Medications and therapeutics are only useful if patients take them
the enzyme activity of the cytochrome P 450 system involved in as prescribed. Factors that contribute to patient adherence include,
oxidation can be induced or inhibited depending on other medications but are not limited to, administration convenience and ease of
being taken, resulting in an altered rate of metabolism. A common taking the medication, the patient’s and family’s understanding of
drug used in pediatrics that inhibits the cytochrome P 450 system is the benefits and risks of the medication, and adverse effect profile.
erythromycin, while phenobarbital and phenytoin induce the system. Because children are dependent on their caregivers to admin-
Drug interactions are not a contraindication to the use of a therapeu- ister medications properly, the caregiver must understand why the
tic, but it may be necessary to closely monitor the dose or serum level. drug was prescribed, how it should be dispensed, and why it must
be taken for a specific length of time. For a medication to achieve
Disease Epidemiology its maximal therapeutic usefulness, accurate dosing is important.
Etiologies for many common diseases may differ depending on the A staff member should demonstrate how to measure and dispense
patient’s age group. Some drugs to which the patient’s condition may the medication. The caregiver should then be asked to demonstrate
be susceptible are contraindicated in certain patient populations. the process. This will make an important difference in adherence
Treatment duration may also vary depending on the patient’s age group to the medication regimen. Having a standardized drug dispensing
(eg, an adult with an uncomplicated urinary tract infection may receive tool, such as a syringe or marked dosing cup, will potentially improve
only a 1- to 3-day course of treatment, while a child may receive 7–10 the ability to give the appropriate medication dose successfully.
days of treatment because of the high recurrence of infection that has In pediatrics, palatability is a major factor in patient adherence.
been observed in children given a shorter treatment duration). If children do not like the taste or texture of a medication, they will
refuse to take it. Physicians who prescribe medications for children
should be familiar with the taste and texture and be prepared to find
Safety Profile alternatives. Prednisone is a common example of this; it is avail-
Medications have benefits and risks; these constitute a safety profile. able as a pill and a suspension. The suspension may taste differently
When choosing a medication, the safety profile (ie, risks vs bene- depending on the preparation prescribed. Children often vomit the
fits) must be evaluated. Some risks may be caused by the duration of less-palatable version. Knowing in advance what children will take
treatment, dose, or interactions with other medications. Therapeutic may prevent future problems associated with noncompliance.
index, which is 1 means of quantifying the risk associated with
a specific dose, may be a consideration in medication choice. Cost-effectiveness
The therapeutic index is the difference between the dose that pro-
Cost-effectiveness is defined as outcome per unit cost. Outcome is the
vides a desired effect and the dose that provides an undesired effect.
treatment of the condition or alleviation of the symptoms for which the
For example, a medication with a wide therapeutic index is one in
medication was prescribed. Cost includes the price of the medication
which a desired effect is achieved with a dose much lower than that
or treatment as well as the physician’s and family’s time. Therefore, if
required to produce a toxic effect. In general, a medication with
a patient is prescribed a medication that is less effective for the child’s
a wide therapeutic index, such as ibuprofen, is considered safer
condition because of cost, it many end up costing more because of
than a medication with a narrow therapeutic index. The higher the
the need for additional doctor visits and more medications. In many
morbidity and mortality associated with a condition, the narrower
practice settings, the pharmacy has a set formulary of medication lim-
the accepted therapeutic index can be. Food-drug interactions and
iting the selection of medications for specific conditions. From the
drug-drug interactions are other risks that can be anticipated. There
patient side, many families have limited or no health insurance and
are also risks that are independent of the dose of the therapeutic,
may have to pay the full cost of a medication. The cost of treating a
which are called idiosyncratic effects. These are unexpected and usu-
common condition such as otitis media can differ as much as 10-fold
ally unavoidable risks associated with the medication.
depending on the medication prescribed. Thus, an appropriate drug
In the United States, a black box warning, also known as a black
of choice is influenced by the out-of-pocket cost to the family. The per-
label warning, is a type of warning that appears on prescription drugs
ceived cost-effectiveness of a therapeutic is different for each situation
that may cause serious side effects. A black box warning is the strict-
and depends on external factors affecting the physician and family.
est warning that can be put on a label. The name came about from
the black border that usually surrounds the text of the warning. Black
box warnings mean that medical studies indicate that a drug carries Drug Dosing
a significant risk of serious or even life-threatening adverse effects. Compared with the adult population, in which drug doses are based
An example of a medication used in pediatrics that has black box on a standard dose for an individual regardless of age, size, or weight,
warnings is antidepressants. This warning does not prevent the use pediatric medications are usually dosed based on weight or body

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 82 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

surface area. For medication dosing determined by weight, when technology, unit dose dispensing systems, and educational programs
a child reaches 40 to 50 kg, the dosing is often changed to a stan- for all health care professionals. There is no literature to support
dard adult dose. With the increasing prevalence of childhood obesity, these recommendations. There have been multiple studies trying to
many school-age children weigh more than 50 kg, thereby making it identify definitive interventions, but, to date, no studies have been
important to know the maximum daily dose because calculating mil- able to elucidate effective solutions.
ligrams per kilogram can easily exceed this amount. For drugs with The American Recovery and Reinvestment Act of 2009 provided
narrow therapeutic indexes, such as chemotherapy or immunosup- much-needed momentum toward widespread electronic health
pressive medications, body surface area is used to determine appro- records, which include e-prescribing. Systems that have computer-
priate dosing. Once a therapeutic agent is chosen, an appropriate ized physician order entry or e-prescribing can reduce medication
dosing schedule must be determined. Dosing guidelines can be pre- errors by having alerts for inappropriate dosing, improved legibil-
sented as total dose per 24 hours or an amount per dose. An exam- ity, warnings for drug interactions, and just-in-time information on
ple for dosing acetaminophen for a 1-year-old is given in Box 13.2. the most appropriate drug choice. Not all errors will be rectified by
e-prescribing; examples of errors that elude decision-support pro-
Medication Errors and Adverse grams include inappropriate selection of medication for the condi-
Drug Events tion being treated and failure to recognize a change in patient status.
Pediatric patients are at greatest risk for medication errors because
of the need to calculate dose based on weight. Between 5% and
27% of all pediatric medication orders result in medical error. CASE RESOLUTION
Pediatric inpatients incur 3 times more medication errors than After obtaining the history and performing a physical examination, the pedi-
adult inpatients. The incidence of adverse drug events in pediat- atrician determines that the patient has acute otitis media. Depending on the
age of the child and the severity of symptoms, American Academy of Pediatrics
rics is about 2.3%. The most vulnerable populations are patients
clinical practice guidelines suggest a stratified approach to therapeutics. For
younger than 2 years; those in intensive care units; those in the ED, infants and children between 6 and 24 months of age, pediatricians can treat
especially if they are seriously ill; or those receiving chemotherapy. with antibiotics if the diagnosis is certain or observe the patient without antibi-
More than half of these errors occur during the prescribing phase otics if the patient is otherwise healthy. In this case, the pediatrician discusses
when the medication dose is calculated. Dosing error checking is the options with the family and, because of the severity of pain and previous
complicated by the fact that children’s weights vary from as little ear infections, chooses to treat the infection. The antibiotic of choice for treat-
ment of otitis media is amoxicillin at a dose of 80 to 90 mg/kg/day. The mother
as 500 g to much more than 100 kg. Therefore, a dose range for a states that her daughter had hives with amoxicillin, a type 1 hypersensitivity.
medication may be very large. The second most common causes of Other antibiotic choices would include cefdinir 14 mg/kg/day in 1 to 2 doses per
error in pediatric prescriptions is missing information and illegi- day. Treatment of pain is essential with otitis media. The patient can take oral
bility. In 2001, the Institute for Safe Medication Practices published acetaminophen or ibuprofen.
guidelines to decrease pediatric medication errors. Their recommen- The mother raises a concern about her daughter’s cough and runny nose and
would like to use an OTC cough medication. The US FDA does not recommend
dations included computerized physician order entry, bar coding
use of cough preparations in this age group. Educating the mother about conser-
vative therapies, including nasal suctioning, humidification, and nasal saline, to
treat her daughter’s respiratory symptoms is more appropriate.
Box 13.2. Dosing Example: Acetaminophen If the patient were not allergic to amoxicillin, it would have been the drug of
choice. It is inexpensive, has a narrow microbiological spectrum, and is palatable.
A 1-year-old has a fever, and the family wants to know how much acetamin- Amoxicillin does require refrigeration, which would be of concern if the family
ophen to give her. She weighs 11.2 kg (24.7 lb). The recommended dose were traveling. While this case illustrates several obvious constraints, it is impor-
of acetaminophen is 10 to 15 mg/kg every 4 to 6 hours. Acetaminophen is tant to emphasize that choosing the appropriate medication is dependent on the
sold as a suspension with the concentration of 160 mg/5 mL. intrinsic needs of the patient and extrinsic factors that can affect adherence and,
1. Determine the amount of medication needed by multiplying the weight ultimately, the effectiveness of treatment.
of the child by the recommended dose.
11.2 kg × 10 mg/kg = 112 mg acetaminophen
2. Determine the volume of medication based on the concentration of Selected References
acetaminophen to be used. American Academy of Pediatrics Steering Committee on Quality Improvement
Dosing volume = (amount of medication) ÷ (concentration of medication) and Management, Committee on Hospital Care. Principles of pediatric
Suspension patient safety: reducing harm due to medical care. Pediatrics. 2011;127(6):
Dosing volume = 112 mg ÷ 160 mg/5 mL 1199–1210. Revised February 2019 PMID: 21624879 https://doi.org/10.1542/
= (112 × 5)/160 peds.2011-0967
= 3.5 mL Bell EA, Tunkel DE. Over-the-counter cough and cold medications in
This family can be instructed to give the 1-year-old 3.5 mL of children: are they helpful? Otolaryngol Head Neck Surg. 2010;142(5):647–650
the suspension of acetaminophen orally every 4 to 6 hours as PMID: 20416449 https://doi.org/10.1016/j.otohns.2010.01.019
needed for fever. Benjamin L, Frush K, Shaw K, Shook JE, Snow SK; American Academy of
Pediatrics Committee on Pediatric Emergency Medicine; American College of

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 Chapter 13: Principles of Pediatric Therapeutics 83

Emergency Physicians Pediatric Emergency Medicine Committee; Emergency Pollock M, Bazaldua OV, Dobbie AE. Appropriate prescribing of medications: an
Nurses Association Pediatric Emergency Medicine Committee. Pediatric eight-step approach. Am Fam Physician. 2007;75(2):231–236 PMID: 17263218
medication safety in the emergency department. Pediatrics. 2018;141(3): Shaddy RE, Denne SC; American Academy of Pediatrics Committee on Drugs
e20174066 PMID: 30352389 https://doi.org/10.1542/peds.2017-4066 and Committee on Pediatric Research. Guidelines for the ethical conduct of
Gerstle RS, Lehmann CU; American Academy of Pediatrics Council on Clinical studies to evaluate drugs in pediatric populations. Pediatrics. 2010;125(4):
Information Technology. Electronic prescribing systems in pediatrics: the 850–860. Reaffirmed February 2018 PMID: 20351010 https://doi.org/10.1542/
rationale and functionality requirements. Pediatrics. 2007;119(6):e1413–e1422 peds.2010-0082
PMID: 17545368 https://doi.org/10.1542/peds.2007-0889 US Food and Drug Administration. Science & research. Pediatrics. http://www.
Hersh AL, Shapiro DJ, Pavia AT, Shah SS. Antibiotic prescribing in ambula- fda.gov/ScienceResearch/SpecialTopics/PediatricTherapeuticsResearch/default.
tory pediatrics in the United States. Pediatrics. 2011;128(6):1053–1061 PMID: htm. Updated September 12, 2018. Accessed August 22, 2019
22065263 https://doi.org/10.1542/peds.2011-1337 Vernacchio L, Kelly JP, Kaufman DW, Mitchell AA. Medication use among children
Hughes RG, Edgerton EA. Reducing pediatric medication errors: children are <12 years of age in the United States: results from the Slone Survey. Pediatrics.
especially at risk for medication errors. Am J Nurs. 2005;105(5):79–80, 82, 84 2009;124(2):446–454 PMID: 19651573 https://doi.org/10.1542/peds.2008-2869
passim PMID: 15867545 https://doi.org/10.1097/00000446-200505000-00035 Young SS, Blandino DA, Engle JP, et al. Appropriate Use of Common OTC
Lieberthal AS, Carroll AE, Chonmaitree T, et al. The diagnosis and management Analgesics and Cough and Cold Medications. Leawood, KS: American Academy
of acute otitis media. Pediatrics. 2013;131(3):e964–e999 PMID: 23439909 https:// of Family Physicians; 2008
doi.org/10.1542/peds.2012-3488

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BPPCA6e_Ch013_0079-0084.indd 84 2/13/20 5:17 PM
 CHAPTER 14

Pediatric Pain and Symptom

Management
Kevin Madden, MD, and Richard Goldstein, MD, FAAP

CASE STUDY
You are caring for a Kayla, a 10-year-old girl with stage have remained closely involved throughout her illness
4 neuroblastoma who is at home receiving palliative and would like to help with the management of her
care. Her tumor is refractory. She receives oral chemo- symptoms.
therapy and transfusions as an outpatient to offset the
bone marrow depletion caused by her tumor. Pain from
Questions
1. What is the approach to pain management in
her metastases is becoming increasingly problematic,
children?
especially in her chest wall and right femur. Her spine
2. How does the physician assess the level of pain in
is also involved, but she does not experience weak-
children?
ness. Although fatigued, she derives great pleasure from
3. What is meant by adjuvant therapy?
attending school and being surrounded by friends and
4. What are nonpain symptoms that can cause
family members, playing as she is able. She hates the
distress?
hospital and her parents have chosen to avoid it, intend-
5. What is the management of nonpain symptoms?
ing to keep her comfortable at home until she dies. You

Ill or injured children experience distressing physical symp- illnesses are cared for and sometimes die at home, the manage-
toms, particularly in the case of illness or injury that is chronic or ment of their illnesses will increasingly involve their primary care
life-limiting. The appropriate management of such symptoms is pediatrician.
fundamental to minimizing discomfort and optimizing quality of This chapter reviews the basic medical approaches to pain
life. Pain can not only diminish a child’s physical well-being but and symptom management in children, particularly those
also can affect psychological, social, and spiritual health. Children with serious illnesses. In addition to pain, approaches to
experiencing poorly controlled pain will withdraw interperson- nausea, anorexia, fatigue, secretions, and delirium are presented.
ally and be unable to engage in the activities that make their life, The focus is on the medical management issues a primary care pedi-
however limited, meaningful. Similarly, a child troubled by atrician may attempt to manage in the community setting. When
nausea will experience distress from the symptom while also the primary care pediatrician is insufficiently familiar with such
losing the simple pleasure of eating and its accompanying com- treatment, consultation with pain or palliative care specialists is
forts. Symptom management improves the lives of children who appropriate.
are medically fragile.
Parents or caregivers of children who are seriously ill worry most Pain
about their child’s symptoms not being satisfactorily controlled. Pain is an integrated biophysical and “existential” construct. It
Studies of dying children have noted that distressing symptoms often involves complex mechanisms of nociception modulated by bio-
go untreated. Research investigating the symptoms and experience chemical factors, neuroplasticity, genetic and familial factors, and
of children dying from cancer found that most of the children expe- an individual’s past experience with painful events. Each child
rienced fatigue, pain, dyspnea, anorexia, nausea and vomiting, and experiences pain in a unique way and quickly develops learned
constipation in the last month of their lives (Figure 14.1). Similar behaviors related to it. As such, no simple correlation exists
findings have since been noted in other populations. Children with between the objective degree of injury and the experience of
neurologic impairment are particularly at risk because of their lim- pain. More accurately, physical, psychological, interpersonal, and
ited verbal abilities and atypical responses to pain. Symptom man- existential factors all contribute in important ways to the expe-
agement of seriously ill children has been improved significantly rience of pain. A comprehensive approach to pain addresses all
since early studies. As more children with chronic and serious these elements.
85

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 86 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

100

80
Percentage of Children

60

40

20

Fatigue Pain Dyspnea Poor Nausea and Constipation Diarrhea

appetite vomiting
Distress Distress Distress Distress Distress Distress Distress
from fatigue from pain from dyspnea from poor from nausea from from
appetite and vomiting constipation diarrhea

Figure 14.1. The presence and degree of distress from specific symptoms in the last month of life.
Adapted with permission from Wolfe J, Grier HE, Klar N, et al. Symptoms and distress at the end of life in children with cancer. N Engl J Med. 2000;342[5]:326–333.

Assessment Standardized pain scales are used to assess the intensity of pain.
The 2 basic types of pain are nociceptive and neuropathic. An Analog pain scales, which generally score intensity on a scale of 1
understanding of the presentation of each type can help differen- to 10, have some reliability when used in the same patient over time
tiate the source of pain in children. Nociceptive pain is the activa- (Figure 14.2). Younger children may have difficulty with the con-
tion of peripheral nerve receptors when noxious stimuli cause tissue cept of quantity or the meaning of greater intensity. An important
damage, and its intensity is related in part to the location and the modification of the analog scale for children with impaired com-
amount of damage. Somatic pain refers to nociceptive pain from munication skills or cognition is the Individualized Numeric Rating
musculoskeletal, bony, or superficial sources (eg, skin, mucosa). Scale, on which parental or caregiver observations of their child’s
Deep somatic pain tends to be localized and concentrated and is facial expression, body movements, activity and interaction, crying,
described as stabbing, aching, or throbbing (eg, bone pain is deep and ability to be consoled as they experience worsening pain are
and aching). Superficial somatic pain is sharper and can be burn- used to label the points of the scale.
ing or pricking. The source of nociceptive pain can also be visceral. For children older than 3 years, the Wong-Baker FACES Pain
Visceral pain is usually poorly localized; can be described as cramp- Rating Scale (Figure 14.3) is often used. After showing children the
ing, gnawing, or pressure; and may follow daily patterns of vary- faces, they are instructed that each face is for a person who has no
ing intensity. pain (no hurt), some pain, or a lot of pain. The child is then asked
Neuropathic pain is caused by injury or dysfunction of the cen- to choose the face that best describes how the child is feeling. More
tral nervous system (CNS) or peripheral nerves. It can be described comprehensive pain assessment tools are available that also assess
as burning, tingling, shooting, or scalding. Its presence points to function and mood, but they are less widely used. The perspective
neuropathies, CNS insult, or evolving damage to the nervous sys- of parents or caregivers and others familiar with the child is crucial
tem. Understanding whether the source of pain is somatic, visceral, to any assessment of a child’s pain.
or neuropathic helps guide treatment decisions.
Pain should be a part of the medical evaluation of every child.
0-10 Numeric Pain Rating Scale
Pain is phenomenological and thus, although its existence is “real”
and “objective,” it can be experienced and described only by the
affected person. Fundamentally, the patient’s report of the presence 0 1 2 3 4 5 6 7 8 9 10
No Moderate Worst
or severity of pain is the key to the assessment. In children, especially pain pain possible
pain
children with developmental issues, objective assessment tools may
be useful to identify the presence of pain and quantify its severity. Figure 14.2. Visual analog pain scale.
In most patients these scales are also helpful in understanding the Reprinted with permission from McCaffery M, Pasero C. Pain: Clinical Manual. 2nd ed.
symptom of pain over time. St Louis, MO: Mosby; 1999.

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 Chapter 14: Pediatric Pain and Symptom Management 87

considerable investment by the physician to provide a comprehen-

sive pain plan that highlights consistent reassessment of the need
for an opioid, directly addresses the specter of dependency before
initiation of an opioid, and reassurance that the child’s best interest
will always be the focus of treatment.
Pain must be understood as a multidimensional symptom with
a meaning for the individual that influences the experience of it,
worthy of its own attention. In the context of “total pain,” other eti-
Figure 14.3. Wong-Baker FACES Pain Rating Scale. ologies of pain should be considered, assessed, and managed, espe-
Reprinted with permission from Wong-Baker FACES Foundation [2014]. Accessed August cially when a child’s or parent’s/caregiver’s report of pain intensity
17, 2019, www.WongBakerFACES.org. seems incongruous with the amount of opioid provided. Total pain
conceives of pain as having 4 interrelated domains: physical, psycho-
The child in pain must be relieved of it; however, it is advanta- logical, social, and spiritual. The use of opioids may unintentionally
geous to make a strong effort to understand the source of the pain alleviate psychological, social, and spiritual pain as the limbic system
and manage conditions amenable to nonpain medication. For exam- also possesses mu receptors, but it cannot substitute for attention to
ple, chest pain may be caused by candidal esophagitis or abdominal all the elements of pain. Activation of these specific mu receptors
pain resulting from constipation. Thinking clearly about the etiology upregulate mood, which can easily become intertwined and con-
of pain rather than simply providing analgesic agents in a reflexive fused with relief of physical pain.
manner has the benefits of preserving alertness, sparing side effects, Dose escalation should proceed until the pain is controlled, pro-
and sustaining the least impaired quality of life. Pain can be elim- vided that side effects are tolerable. Opioids have no maximum or
inated by eliminating the underlying condition responsible for it. “ceiling” dose. The child on long-term opioid treatment often receives
The optimal management of pain also includes addressing psycho- surprisingly high doses yet, provided the treatment is successful in
social and spiritual distress and making efforts to enhance a child’s controlling pain, is comfortable and functional. Generally, infants
function as part of an integrated approach to pain-related distress. younger than 6 months should be started at one-third the general
pediatric dose. Anticipated side effects of opioids include constipa-
Medical Management tion, pruritus, and nausea and vomiting. The pruritus and nausea
Opioids are the pharmacologic mainstay of pain management. and vomiting are usually short-lived, disappearing in a week, but
Familiarity with the basic principles of treatment with these agents constipation persists. All patients on scheduled opioids should also
is fundamental to care for the child with serious acute and life- receive scheduled doses of stool softeners and stimulant laxatives.
limiting illness. In cases of complex pain or those involving daunting Parents or caregivers and some physicians may overestimate
polypharmacy, it may be best to seek guidance from pain or palliative the benefit of distraction in pain management. Distraction is best
care specialists. Additionally, certain medications (ie, methadone, thought of as an assessment tool and may be an adjunct to but not
selective norepinephrine reuptake inhibitors) or invasive approaches a treatment for pain. If a child can be substantially distracted from
(ie, intrathecal pumps, regional blocks, surgical or radiotherapeutic the pain for a significant period, the pain need not be managed with
approaches to pain control), or the management of chronic pain, par- medication. If the child’s distress is apparent despite the distraction,
ticularly in adolescents, should involve consultation with or referral however, pharmacologic intervention for the pain is necessary. What
to pain and palliative care specialists. must be avoided is a distressed child engaging in the distraction to
The use of opioids in children since approximately 1990 roughly please adults while the pain goes unabated. Similarly, the use of
parallels wider trends observed in their use in adults. The 1990s and placebos has no place in the treatment of pain.
early 2000s saw a dramatic increase in the number of opioid pre- The World Health Organization 2-step approach to pharmaco-
scriptions in the United States, motivated by the assessment that logic management of pain is the most important guide to pain man-
pain in seriously ill children was being undertreated. Changing agement in children. It presents a stepwise and additive approach
prescribing practices also heralded a new public health crisis, the to “capture” a child’s pain as well as to augment the pharmacologic
opioid-overdose epidemic. Currently, opioid overdoses are the lead- treatment of pain. The model is premised on using a 2-step strategy,
ing cause of death of Americans younger than 50 years. The philo- dosing at regular intervals, using the appropriate route of adminis-
sophical pendulum is now swinging back towards a more scrutinized tration, and individualizing treatment to the particular child.
prescribing environment. Some commercial pharmacies limit new The child with mild pain who has not received any analgesic
prescriptions of opioids for noncancer pain to a 7- to 10-day sup- medication is managed with oral analgesics, such as acetamino-
ply, and many state medical boards now require physicians to review phen and ibuprofen (step 1). In certain cases, intravenous acetamin-
prescription drug monitoring program databases on a routine ophen or ketorolac tromethamine may be used. Adjuvants may be
basis. Parents have responded to this widely publicized phenome- added if appropriate. Acetaminophen can be given orally or rectally.
non with renewed caution about opioids, while seeking the bene- Compounded ibuprofen can be given rectally. The topical applica-
fits of appropriate use. Proposing an opioid for a child now demands tion of these agents in ketoprofen cream or aspirin cream may also

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 88 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

be of benefit. Rarely, a role may exist for a cyclooxygenase-2 inhibi- pain) may be adequately managed with gabapentin or pregabalin,
tor. All nonsteroidal anti-inflammatory drugs have ceiling doses and minimizing the use of opioids.
gastrointestinal toxicities. With the exception of acetaminophen and Step 2 addresses children with moderate to severe pain and
celecoxib, all affect platelet function and hemostasis. involves the addition of opioids to the treatment plan (Table 14.1).
Adjuvants are non-analgesic drugs that are helpful in the man- “Weak opioids” (eg, codeine, tramadol hydrochloride) are no longer
agement of a child’s pain. For example, anxiety experienced by a recommended. Research has shown that codeine may be a weaker anal-
child who is medically fragile may potentiate the child’s pain and dis- gesic than a standard dose of many nonsteroidal anti-inflammatory
tress, even at step 1. A benzodiazepine may be helpful in such cases, drugs, and it has a ceiling effect. The oral bioavailability of codeine
but it should be prescribed with an awareness of other medications. is widely unpredictable, at 15% to 80%. Most importantly, codeine
An alternative to benzodiazepines for anxiety is the antipsychotic is a prodrug that must be metabolized by the liver into morphine.
agent haloperidol. At very low doses it provides an anxiolytic effect This is problematic because it is estimated that 35% of children
without sedation or the synergism with opioids that can result do not metabolize codeine in the anticipated manner, resulting
in respiratory depression. A lidocaine patch can help with some in great uncertainties in a calculated effect. The use of tramadol
somatic pain. Certain forms of mild to moderate pain (ie, neuropathic hydrochloride is only weakly recommended because of concerns

Table 14.1. Essential Pharmacopeia for Symptom Management in Pain and Palliative Care
Symptom Agent Initial Oral Dosing Initial IV Dosing Available Formulations
Pain Hydromorphone 0.04–0.06 mg/kg every 3–4 hours 0.015 mg/kg every 2–3 hours Immediate release: tablet, liquid
(max, 2 mg/dose) (max, 0.6 mg/dose) Extended release: tablet
IV
Morphine 0.2–0.3 mg/kg every 3–4 hours (max, 0.05–0.1 mg/kg every Immediate release: tablet, liquid
15 mg/dose) 2–3 hours (max, 5 mg/dose) Extended release: capsule, tablet
IV
Oxycodone 0.1–0.2 mg/kg every 3–4 hours (max, N/A Immediate release: tablet, liquid
10 mg/dose)
Anorexia Cyproheptadine hydrochloride 0.25 mg/kg/day in 2–3 divided doses N/A Tablet
(max, 12 mg/day)
Megestrol acetate 10 mg/kg/day in 1–4 divided dosesa N/A Tablet, liquid
Delirium Haloperidol 0.01–0.02 mg/kg every 4–6 hours 0.01–0.02 mg/kg every 4–6 Tablet, liquid, IV
(max, 0.5–1 mg/dose) hours (max, 0.5–1 mg/dose)
Olanzapine 1.25–5 mg every 4–6 hours N/A Tablet, oral disintegrating tablet
Quetiapine fumarate 12.5–50 mg every 6–8 hours N/A Tablet
Dyspnea Any opioid 33%–50% of opioid dose for pain 33%–50% of opioid dose for pain Any formulation
Dystonia Baclofen 2.5–5 mg every 8 hours N/A Tablet, liquid
Diazepam 0.5 mg/kg/dose every 6 hours as 0.5 mg/kg/dose every 6 hours Tablet, IV, rectal gelb
needed as needed
Fatigue Methylphenidate hydrochloride 2.5–5 mg daily or twice a day N/A Tablet, transdermal patchc
Nausea Ondansetron hydrochloride 0.15 mg/kg/dose every 8 hours 0.15 mg/kg/dose every 8 hours Tablet, oral disintegrating
(max, 8 mg/dose) (max, 8 mg/dose) tablet, liquid, IV
Metoclopramide 0.1–0.2 mg/kg every 6 hours (max, 0.1–0.2 mg/kg every 6 hours Tablet, liquid, IV
hydrochloride 5 mg/dose) (max, 5 mg/dose)
Respiratory Atropine 1% ophthalmic 1 drop every 2 hours as needed N/A Liquid
secretions drops
Glycopyrrolate 0.04–0.05 mg/kg every 4 hours 0.004–0.005 mg/kg every 4 hours Tablet, liquid, IV
Abbreviations: IV, intravenous; max, maximum; N/A, not applicable.
a
Only for children >10 years of age.
b
Dose equivalent to oral or IV dose.
c
Dose equivalent to oral dose.

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 Chapter 14: Pediatric Pain and Symptom Management 89

about risks for seizures and hyperserotonergic symptoms with

Box 14.1. Opioid Conversion Example
its use.
When pain persists or worsens, morphine, hydromorphone, Kayla is receiving 30 mg of long-acting oxycodone every 8 hours and
and oxycodone are recommended initial choices. Doses are esca- 15 mg of immediate release oral morphine every 4 hours as needed for
lated until pain is controlled, using the least invasive form of breakthrough pain. She receives approximately 4 doses of as-needed
administration necessary. This step may involve the addition of morphine per day. She can no longer walk and finds it progressively more
controlled-release preparations of opioid or methadone, while difficult to swallow pills. The decision is made to rotate (ie, change) her
using shorter-acting agents for breakthrough pain. To calculate the opioid to intravenous (IV) hydromorphone.
as-needed dose for breakthrough pain, a general rule is that the Steps in opioid conversion
as-needed dose is 10% to 15% of the 24-hour total opioid dose or its 1. Calculate the total 24-hour dose of each opioid
equivalent, given orally every 3 to 4 hours. a. 30 mg oxycodone × 3 = 90 mg oxycodone
An in-depth discussion of methadone and fentanyl citrate b. 15 mg morphine × 4 = 60 mg morphine
exceeds the scope of this chapter, but it is worth making several 2. Convert 24-hour total of each opioid to the morphine equivalent daily
points about them. Neither has active metabolites, making them dose (MEDD), always expressed as oral mg of morphine
important options for patients with renal failure in whom accumu- a. 90 mg oxycodone = 135 mg oral morphine (oxycodone is ≈1.5 times
lated metabolites from other opioid agents can cause myoclonus, more potent than oral morphine)
confusion, or hyperalgesia. Fentanyl citrate, a potent analgesic, is b. 60 mg oral morphine = 60 mg oral morphine
available in a transdermal patch, which is an important nonintra- c. MEDD = 135 + 60 = 195 mg oral morphine
venous, non-oral option for children having difficulty swallowing. 3. Convert MEDD to new opioid (use opioid equivalency table [Table 14.2])
The child must have adequate body fat, because the drug is deposited a. 195 mg / 20 (IV hydromorphone is ≈20 times as potent as oral
across the rate-limiting membrane into fat reservoirs and is absorbed morphine) = 9.75 mg IV hydromorphone/day
from that reservoir into the child’s bloodstream. Methadone has 4. Dose reduce the MEDD by 33%-50% to account for incomplete
some distinct analgesic advantages. It is inexpensive, its half-life cross-tolerance
allows for more convenient twice-a-day or 3-times-a-day dosing, a. 9.75 mg × 0.66 = 6.5 mg/day
and it is especially helpful in managing neuropathic pain. It is com- i. 6.5 mg / 24 hours = 0.27 mg/hour
plicated to dose, however, and its long and variable half-life make it 5. Determine an as-needed dose, that is, 10%-20% of the 24-hour
a more complicated choice for those not familiar with its use. opioid dose
The management of moderate and severe pain over time can a. 6.5 × 0.1 = 0.65 mg IV every 2 hours as needed for breakthrough
result in the development of difficult unintended effects, including pain OR
sedation, nausea and vomiting, pruritus, and urinary retention. At b. 6.5 × 0.2 = 1.3 mg IV every 2 hours as needed for breakthrough pain
high doses or when metabolites accumulate, patients may develop
For Kayla
delirium, myoclonus, or hyperesthesia. Opioid rotation can be quite
IV hydromorphone infusion of 0.27 mg/hour with IV hydromorphone
helpful in these situations. This method involves converting 1 form
0.65-1.3 mg IV every 2 hours as needed for breakthrough pain
of opioid to an equivalently analgesic dose of another.
Rotation of the opioid agent (ie, using a different opioid) holds the
promise of maintaining analgesia with lesser amounts of the newly
introduced drug and eliminating side effects. Tolerance is the term care physician may choose to consult with palliative care or pain
used to describe the need over time for increased amounts of a spe- treatment specialists to ensure that the child’s needs are adequately
cific opioid to achieve the desired analgesic effect. Although opioids addressed. Alternatively, hospice medical directors can provide pal-
lack a ceiling dose, higher doses may have an accompanying increase liative care consultations if hospital-based teams are not available.
in adverse effects (ie, the emergence of the intolerable side effects The optimum of opioids is impacted by factors related to both
noted previously, the accumulation of toxic metabolites, or high cost patients and physicians. Foremost is the concern about respira-
resulting from the required amounts of the drug). Because of mul- tory depression. The risk of respiratory depression while using an
tiple mu opioid peptide receptors as well as different selectivity of accepted escalation rationale is quite small, however. Patients may
opioids for specific mu opioid peptides, the cross-tolerance from fall asleep when their pain is “captured,” but this generally is the
1 opioid to another is not complete. An equianalgesic dose of another result of exhaustion related to the previously unrelieved pain. Even
opioid will require less of the medication and achieve a more favor- in mild to moderate pain, patients do not notice a change in dose
able balance between analgesia and side effects. See Box 14.1 for an increase when they are less than 25% above baseline. In cases of
example of opioid conversion. moderate to severe pain, a dose escalation of 50% to 100% is appro-
Physicians lacking experience in pain management may be con- priate. For acute pain crises, protocols exist that allow physicians to
cerned about the difficulty of addressing uncontrolled pain. Because safely mitigate pain, but consultation with pediatric palliative care
expert and effective management is a critical skill in the care of seri- or pediatric pain specialists may help the less comfortable physician
ously ill children, especially those facing the end of life, the primary act more decisively. It is nonetheless important to recognize that the

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 90 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Table 14.2. Opioid Equivalency

Equianalgesic Dose
Drug Oral Route Parenteral Route Conversion Ratio to Oral Morphine of Oral Morphine
Morphine sulfate 30 mg 10 mg of parenteral morphine Parenteral morphine is 3 times as potent as oral 30 mg
morphine.
Oxycodone 20 mg N/A Oral oxycodone is approximately 1.5 times more 30 mg
potent than oral morphine.
Hydrocodone 20 mg N/A Oral hydrocodone is approximately 1.5 times more 30 mg
potent than oral morphine.
Hydromorphone 7 mg 1.5 mg of parenteral Oral hydromorphone is approximately 4–7 times 30 mg
hydromorphone as potent as oral morphine.
Parenteral hydromorphone is 20 times as potent
as oral morphine.
Fentanyl citrate N/A 15 mcg/hour Transdermal fentanyl citrate is approximately 30 mg
80 times as potent as morphine.a
Abbreviation: N/A, not applicable.
a
This is based on studies converting from morphine to fentanyl citrate. Currently, there are no empirical studies converting fentanyl citrate to morphine.
Adapted with permission from Periyakoil VJ. Opioid conversion equivalency table. Palliative.stanford.edu website. https://palliative.stanford.edu/opioid-conversion/equivalency-table/. Accessed July 3, 2019.

co-administration of an opioid and benzodiazepine increases the risk lasting only minutes and thereby making it difficult to manage exac-
for apnea and respiratory insufficiency. When alveolar pCO2 (par- erbations of pain. It also causes allodynia, dysesthesia, and hyper-
tial pressure of carbon dioxide) reaches 60 mm Hg, normal alveo- algesia. The results of various approaches to the management of
lar minute ventilation increases almost 10-fold from baseline, yet neuropathic pain are mixed, and a multimodal approach, includ-
the compensatory increase in alveolar minute ventilation is largely ing an emphasis on physical therapy and more than 1 therapeutic
absent when an opioid (eg, fentanyl citrate) is co-administered with agent, is recommended. Adjuvant medications are offered as first-
a benzodiazepine (eg, lorazepam). In the rare situation in which nal- line therapy in children, with gabapentin beginning at 10 mg/kg/
oxone hydrochloride is required to reverse a worrisome respiratory day divided 3 times a day and titrating upward to doses as high as
effect, the experienced physician can slowly titrate it to reverse respi- 60 mg/kg/day. Emerging evidence in adults indicates that the
ratory insufficiency and preserve the analgesic effect of the opioid serotonin–norepinephrine reuptake inhibitor duloxetine hydrochlo-
rather than administer the entire dose at once. Physicians may also ride may provide better neuropathic pain control than gabapentin,
be unfamiliar with appropriate dosing, may not view pain control with fewer side effects (eg, fatigue, excessive somnolence). Strong
as a priority of care, or may be worried about difficulties ensuring opioids, including methadone hydrochloride, may be included in
follow-up and ongoing assessment. Families, for their part, may an appropriate regimen. Tricyclic antidepressants tend to be used
believe that pain is an inevitable part of the child’s disease, may less for children in the management of neuropathic pain because of
worry about the “symbolism” of starting a morphine drip as a concerns of arrhythmia risk.
hastening of death, or may worry about addiction or the stigma Dystonia and neuroirritability, although not properly neuropathic
of having their child on such medications. Cultural and religious pain, present similar challenges in children with neurodegenerative
factors may also elicit reluctance. Such concerns should be addressed conditions. Generalized dystonia is a condition in which sustained,
directly. The greatest concern should be to minimize any distress erratic, painful muscle contractions occur, causing twisting and
caused by inadequately managed pain. repetitive movements or abnormal postures. Neuroirritability is a
The management of neuropathic pain presents a considerable term used to describe the difficulty in settling and persistent crying
challenge. The source of neuropathic pain is the insult or dysfunc- seen in some children who are cognitively impaired with metabolic
tion of the CNS, peripheral nervous system, or autonomic nervous and neurodegenerative conditions. These symptoms are distressing
system. Pediatric patients may experience this pain as the result of and often occur in children whose impairments make assessment
degenerative CNS processes or injury as well as the result of treatment- of their experience difficult. Benzodiazepines, typically diazepam,
related injuries to the nervous system from drug toxicities, radia- are the first-line therapy for dystonia (see Table 14.1). Trihexyphenidyl
tion therapy, surgery, or physical compression of a nerve by a tumor. hydrochloride is also commonly considered, as are valproate sodium,
Infection may also cause neuralgia. Children describe neuropathic baclofen, carbamazepine, and tetrabenazine. In severe, intractable
pain as jolts of burning, stabbing, or shooting; the pain seems to cases the implantation of deep brain stimulators is increasingly con-
worsen at night and, compared with nociceptive pain, usually has sidered, although the empiric basis for that decision remains under
an abrupt, unpredictable onset and a shorter duration, sometimes study. Anticonvulsant agents are the mainstay in the management

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 Chapter 14: Pediatric Pain and Symptom Management 91

of neuroirritability. Phenobarbital is often the first medication tried. gastrointestinal tract, and the vestibular system. The CTZ responds
The management of these conditions is generally determined in con- to toxins and medications in blood and spinal fluid; the cerebral cor-
sultation with a child neurologist. tex responds to sensory input, anxiety, meningeal irritation, and ele-
vated intracranial pressure; peripheral pathways are stimulated by
Management of Nonpain Symptoms mechanical stretch in intestinal obstruction and by mucosal injury;
and the vestibular system is affected by motion and labyrinth disor-
Agents used in the management of nonpain symptoms are listed
ders. Each pathway involves different neuroreceptors for targeting
in Table 14.1.
in mechanism-based therapy.
Dyspnea The CTZ can be suppressed by the blockage of dopamine D2
Many similarities exist between the management of severe dyspnea receptors with haloperidol, olanzapine, prochlorperazine, chlor-
and severe pain. Dyspnea is caused by increased work of breath- promazine, or metoclopramide hydrochloride. Peripheral pathways
ing, hypoxia, and hypercapnia and the driving desire of the brain to can be addressed by identifying the underlying cause and with the
relieve these conditions. Experientially, it may cause a feeling simi- blockage of 5-hydroxytryptamine, serotonin 3 receptors by ondan-
lar to that of being underwater too long and needing to surface. Like setron hydrochloride. The cortex can be addressed with anxiolytics,
pain, dyspnea has a physical basis; however, the distress is potenti- attention to sensory stimuli (eg, smells, tastes), and, in cases of ele-
ated by psychological, interpersonal, and existential aspects. After vated intracranial pressure, high-dose steroids. Dronabinol may also
oxygen, opioids are the treatment of choice, and they are dosed at be helpful. The vestibular system is managed by blocking muscarinic
one-third the dose for pain management. The inexperienced phy- acetylcholine and histamine receptors with scopolamine, hyoscya-
sician may be concerned about exacerbating the patient’s dyspnea mine, and diphenhydramine. Although promethazine hydrochlo-
by causing respiratory depression, but strong evidence exists for the ride is useful in adults, it should be avoided in children because of its
effectiveness and safety of low-dose opioids: At appropriate doses, implication in sudden death in some individuals in that population.
opioids reduce breathlessness and provide benefit to patients with The symptoms of nausea and vomiting often involve multiple
limited risk of respiratory depression. Because of the anxious state pathways. For instance, opioid-induced nausea and vomiting may
dyspnea often causes and because anxiety worsens dyspnea, an be the result of constipation or gastroparesis, stimulation of the CTZ,
appropriate dose of a benzodiazepine may also be helpful, although or sensitization of the labyrinth. Choices must be made about how
never as the principal medication. Environmental measures, such to approach the possible causes in a stepwise manner. The litera-
as the breeze from an electric fan on the patient’s face or declutter- ture suggests that mechanism-based therapy is more effective, uses
ing the room, also can bring relief. It is important to recognize that a systematic approach that identifies all possible contributors, and
dyspnea is a subjective sensation and that although the patient may encourages the management of underlying causes. It uses medi-
report improved dyspnea, tachypnea, increased work of breathing, cation in a targeted manner, thereby reducing the risk of untow-
and retractions may persist. The goal of managing dyspnea is mak- ard effects and oversedation. Finally, the risk of extrapyramidal side
ing the patient feel better, not necessarily look better. effects is greater in children than adults, and when using high doses
of metoclopramide hydrochloride or the phenothiazines, premedi-
Nausea and Vomiting cation with diphenhydramine is recommended.
Nausea and vomiting are common symptoms in children with com-
plex illnesses and those facing the end of life and are significant Anorexia
sources of distress and discomfort. These conditions can have differ- Anorexia is an anticipated symptom at the end of life. Some parents
ent etiologies, principally gastrointestinal, CNS, or treatment related. and caregivers have great difficulty with this symptom and can feel
The child with neurologic impairment can experience retching, and overwhelmed by the loss of a concrete expression of their nurtur-
many of the medications used in the management of the impairment ing. They want their child to eat to be better able to fight the illness.
have nausea among their side effects. Children are especially vul- Food preparation and feeding the child is something concrete a par-
nerable to anticipatory nausea, a conditioned behavioral response in ent or caregiver can do for their child. Children who are dying want
anticipation of a medication or procedure that has caused nausea and to please their parents or caregivers as much as healthy children if
vomiting in the past. Anorexia can be a symptom of low-grade nau- not more so. It is important to acknowledge the feelings behind the
sea. An approach to nausea and vomiting based on an understanding desire of parents and caregivers for their child to eat. Small portions
of the cause of the symptom exacerbation and of the symptom mech- of the child’s favorite foods or new favorites the child requests may
anism can prove valuable. A careful history and physical examina- be comforting to all.
tion, with special attention to medications and procedures, is crucial. Times exist earlier in an illness when it may be appropriate to
Although a patient’s presentation may be complex, determina- improve appetite. Cyproheptadine hydrochloride has a long history
tion of appropriate management may be simplified by understand- of safe use in children and has been shown to be helpful in some
ing the 4 pathways to nausea and vomiting. The vomiting center, cases. Megestrol acetate has been shown to be effective in increasing
which lies in the brain stem, receives input from the chemoreceptor weight but not muscle mass and may be considered in children over
trigger zone (CTZ), the cerebral cortex, peripheral pathways in the the age of 10 years, although use in children has not received US Food

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 92 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

and Drug Administration approval. Although cannabinoids stimu- in decreasing fatigue. Medical therapy with methylphenidate hydro-
late appetite, they have not been demonstrated to increase weight. chloride or modafinil may be helpful, dosed at lower amounts than
The use of steroids, which are likely to improve appetite, often comes typically prescribed for attention-deficit/hyperactivity disorder.
at an unacceptable cost of irritability and immunosuppression.
One other consideration related to feeding is whether children Delirium
are “starving” when they no longer have the desire to eat, a com- Delirium is most simply conceived as organic brain dysfunction and
monly expressed concern of parents and caregivers. Research com- is common at the end of life. The diagnosis of delirium can be chal-
paring the anorexia-cachexia syndrome with starvation shows 2 lenging, especially in children, because of the vast developmental
quite different metabolic states. Differences in energy expenditure, range. The Diagnostic and Statistical Manual of Mental Disorders,
protein synthesis and proteolysis, glucose metabolism, and hor- Fifth Edition, diagnostic criteria are a disturbance in attention, a
monal levels reinforce the belief that anorexia-cachexia is a hyper- change in cognition, and whose onset is acute and whose inten-
metabolic state, whereas starvation in the healthy individual is an sity fluctuates during the day. Delirium can manifest as hyperac-
effort by the body to conserve itself. An understanding of this dif- tivity (ie, agitated, combative), hypoactivity (ie, withdrawn, appear
ference enables the physician to explain to parents or caregivers to be sleeping), and mixed (ie, hyperactive and hypoactive). Given
how the disease is robbing the child’s energy and that it is not the these challenges, use of a validated, behavioral-observational tool is
fault of their efforts or the lack of food. In fact, research on adults encouraged, such as the Cornell Assessment of Pediatric Delirium. It
dying of advanced cancer found that even the patients who ate the is important to counsel families that delirium can cause disinhibited
most lost weight. behavior, commonly seen as increased pain expression. Delirium
must be aggressively managed with haloperidol, olanzapine, or que-
Respiratory Secretions tiapine fumarate to also achieve good pain control.
Respiratory secretions are a common symptom in children who are
seriously ill. Children with neuromuscular diseases and neurologic
impairment can have difficulty managing secretions. The presence of CASE RESOLUTION
a tracheostomy can further complicate matters. The use of suction-
Kayla lived for 4 more months, was never again admitted to the hospital, and
and cough-assist machines is important, particularly in the setting until her last days, remained engaged and as playful as her fatigue allowed. Her
of acute respiratory illnesses that cause increased secretions. In chil- primary care pediatrician teamed with the pediatric palliative care team at the
dren at this stage of illness, drying agents (eg, glycopyrrolate, atro- closest children’s hospital and was assisted with many of the decisions about symp-
pine, scopolamine, hyoscyamine) are sometimes helpful, but they tom management. In her last days, the patient was on methadone, morphine
boluses, lorazepam, citalopram hydrobromide, gabapentin, ondansetron hydro-
may increase the risk of acute obstruction by thickening secretions
chloride, polyethylene glycol 3350, senna, and as-needed methylphen­idate hydro-
and causing mucus plugging. chloride. Her pediatrician made home visits and remained available for evolving
Terminal secretions (the “death rattle”) are understandably quite symptoms. The pediatrician was in the child’s home when the child died.
distressing to parents and caregivers, although the child is not typ-
ically bothered by them. These secretions are not relieved by suc-
tioning, and they often worsen because the stimulus of the suction
Selected References
catheter can reflexively increase saliva and mucosal secretions; how-
ever, the obstruction and noise of secretions often respond to repo- Breau LM, Camfield CS, McGrath PJ, Finley GA. The incidence of pain in children
sitioning of the head and neck. Drying agents are usually prescribed with severe cognitive impairments. Arch Pediatr Adolesc Med. 2003;157(12):
1219–1226 PMID: 14662579 https://doi.org/10.1001/archpedi.157.12.1219
in this setting. Switching between agents lacks much advantage if the
effect is not substantial, although more than 1 agent should be tried. Madden K, Bruera E. Very-low-dose methadone to treat refractory neuropathic
pain in children with cancer. J Palliat Med. 2017;20(11):1280–1283 PMID:
Glycopyrrolate is the only such agent that does not cross the blood-
28609177 https://doi.org/10.1089/jpm.2017.0098
brain barrier and thus, it is the least likely to cause CNS effects such
Pritchard M, Burghen EA, Gattuso JS, et al. Factors that distinguish symp-
as confusion, agitation, and delirium.
toms of most concern to parents from other symptoms of dying children.
Fatigue J Pain Symptom Manage. 2010;39(4):627–636 PMID: 20413052 https://doi.
org/10.1016/j.jpainsymman.2009.08.012
Fatigue is a highly prevalent symptom at the end of life. In a study
Rork JF, Berde CB, Goldstein RD. Regional anesthesia approaches to pain
of children with terminal cancer, 96% were found to have experi-
management in pediatric palliative care: a review of current knowledge.
enced fatigue in their last month. This symptom is especially dis- J Pain Symptom Manage. 2013;46(6):859–873 PMID: 23541741 https://doi.
heartening, because fatigued children may have the desire to do org/10.1016/j.jpainsymman.2013.01.004
things that provide a quality of life but not feel up to the task. Fatigue Solodiuk J, Curley MA. Pain assessment in nonverbal children with severe
may be caused by illness, treatments, stress, isolation, poor sleep cognitive impairments: the Individualized Numeric Rating Scale (INRS).
hygiene and circadian disorientation, mood, or the lack of pleasure J Pediatr Nurs. 2003;18(4):295–299 PMID: 12923744 https://doi.org/10.1016/
or activity. The sedative effects of some medications certainly con- S0882-5963(03)00090-3
tribute. Sleep, exercise, nutrition, anemia treatment, increased inter- Traube C, Silver G, Kearney J, et al. Cornell Assessment of Pediatric Delirium:
actions, and activity all have been shown to have a beneficial effect a valid, rapid, observational tool for screening delirium in the PICU.

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 Chapter 14: Pediatric Pain and Symptom Management 93

Crit Care Med. 2014;42(3):656–663 PMID: 24145848 https://doi.org/10.1097/ Wolfe J, Grier HE, Klar N, et al. Symptoms and suffering at the end of life in
CCM.0b013e3182a66b76 children with cancer. N Engl J Med. 2000;342(5):326–333 PMID: 10655532
Ullrich CK, Dussel V, Hilden JM, et al. Fatigue in children with cancer at the end https://doi.org/10.1056/NEJM200002033420506
of life. J Pain Symptom Manage. 2010;40(4):483–494 PMID: 20678889 https:// Wood GJ, Shega JW, Lynch B, Von Roenn JH. Management of intractable nau-
doi.org/10.1016/j.jpainsymman.2010.02.020 sea and vomiting in patients at the end of life: “I was feeling nauseous all of the
Wee B, Hillier R. Interventions for noisy breathing in patients near to death. time . . . nothing was working”. JAMA. 2007;298(10):1196–1207 PMID: 17848654
Cochrane Database Syst Rev. 2008;(1):CD005177 PMID: 18254072 https://doi. https://doi.org/10.1001/jama.298.10.1196
org/10.1002/14651858.CD005177.pub2 World Health Organization. WHO Guidelines on the Pharmacological Treatment
Williams DG, Hatch DJ, Howard RF. Codeine phosphate in paediatric medicine. of Persistent Pain in Children with Medical Illnesses. Geneva, Switzerland: World
Br J Anaesth. 2001;86(3):413–421 PMID: 11573533 https://doi.org/10.1093/ Health Organization; 2019. www.who.int/ncds/management/palliative-care/
bja/86.3.413 cancer-pain-guidelines/en/. Accessed August 17, 2019

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 CHAPTER 15

Complementary and
Integrative Medicine in
Pediatric Primary Care
Miriam T. Stewart, MD, FAAP, and Erica M.S. Sibinga, MD, MHS, FAAP

CASE STUDY
A 14-year-old girl is brought to your office for follow-up Questions
on her migraine headaches. She has no other significant 1. What are CIM therapies?
medical history but has experienced intermittent migraine 2. How does a provider explore if any CIM approaches
headaches over the past few years. The headaches occur are appropriate for the treatment of chronic or recur-
approximately weekly in the evenings, do not wake her rent conditions, such as headaches, in a child or an
from sleep, and improve with ibuprofen (400 mg), which adolescent?
was previously prescribed at your office. At this visit, the 3. What is the best way to determine whether a family
girl states that she wishes she did not have to take medi- is using CIM?
cation for her headaches. Her mother reports that a fam- 4. What is the best way to communicate with a family
ily friend has suggested acupuncture or herbs for the about CIM therapies?
headaches and asks whether there are other complemen- 5. What is the best way to monitor the safety of CIM
tary and integrative medicine (CIM) approaches that they approaches?
could try.

Complementary and Integrative as the umbrella term to describe CIM, but in view of the rare use of
Medicine these modalities in place of conventional medicine and the increas-
ing use of an integrative approach, integrative medicine is replac-
Complementary and integrative medicine (CIM) refers to a wide ing alternative medicine in many settings, including the National
variety of therapies that are not typically part of “conventional” Institutes of Health (NIH) National Center for Complementary and
medical approaches. Conventional medicine (sometimes called Integrative Health (NCCIH), formerly known as the National Center
Western medicine) is the general approach of medical doctors, for Complementary and Alternative Medicine (NCCAM).
doctors of osteopathy, and allied health professionals. The specific Pediatricians are learning about and using integrative therapies
therapies thought of as CIM when compared with conventional in a variety of ways. Many pediatric clinicians review evidence in the
medicine may change over time as research on CIM practices grows medical literature for specific therapies in certain clinical scenarios
and those that are found to be of benefit are incorporated into and integrate approaches into their clinical practice that show poten-
evidence-based conventional medicine. tial for benefit and safety. Interested clinicians may also attend con-
CIM therapies may be used in a number of different ways. When tinuing education courses and/or trainings in integrative approaches
CIM therapies are used by patients in addition to the therapies rec- to gain knowledge and skills that can be incorporated into direct
ommended by conventional medical providers, they are termed com- patient care. At many institutions, exposure to integrative medi-
plementary; this is by far the most common way CIM therapies are cine has expanded during residency training, including didactic
used. Alternative medicine refers to therapies used instead of con- and experiential instruction. In addition, a small but growing num-
ventional care. Increasingly, health professionals are incorporating ber of pediatric clinicians have received extensive training in inte-
both conventional approaches and evidence-based nonconventional grative medicine at the fellowship level. Thus, a broad spectrum of
approaches into their practice, a practice referred to as integrative approaches to pediatric integrative medicine currently exists.
medicine. Complementary and alternative medicine has been used

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 96 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Epidemiology additional hope when conventional medical care fails. Underlying all
these motivations is the desire for the child’s health and well-being
The use of CIM has risen steadily over the past several decades and
and the quest for safe and effective treatments of disease. Physicians
now comprises a significant subset of health-related visits and expen-
and families can find common ground in this most basic of moti-
ditures. In 2012, 33.2% of American adults surveyed reported having
vations, which can inform conversations about CIM and conven-
used some form of CIM in the previous 12 months. Out-of-pocket
tional medical care.
expenditures for CIM totaled $30.2 billion in 2012, up from $27 billion
in 1997. These statistics demonstrate the importance of physician
awareness of CIM and support the routine inclusion of questions
CIM Categories
about CIM in the medical history. Complementary and integrative medicine therapies can be thought
A growing body of evidence reveals that the pediatric popula- of as falling into 5 categories: whole medical systems, mind-body
tion is also using CIM. A large-scale survey of caregivers revealed therapies, biomechanical therapies, bioenergetic therapies, and
that approximately 1 in 9 children uses CIM (11.6%). Higher preva- biochemical therapies (Table 15.1). Besides whole medical sys-
lence, up to 60%, has been found among certain populations, such as tems, these categories are chosen to reflect purported similarities
children with cancer, epilepsy, sickle cell disease, or another chronic in the underlying mechanism of effect. A particular therapy may be
disease. The most commonly used CIM modalities are dietary sup- used as part of a whole medical system approach or on its own. For
plements, chiropractic or osteopathic manipulation, yoga, and deep example, acupuncture may be part of an individualized, compre-
breathing exercises. A survey in 2017 revealed that use of yoga by hensive traditional Chinese medicine (TCM) treatment approach,
children and use of meditation by children had increased over the also consisting of herbs and lifestyle recommendations, or a
previous 5 years from 3.1% to 8.4% and from 0.6% to 5.4%, respec- standardized acupuncture treatment may be used without
tively. Complementary and integrative medicine therapies are most evaluation and treatment by a TCM provider, in which case it
likely to be used for back or neck pain, head or chest cold, anxiety or can be thought of as a bioenergetic therapy. In addition, a
stress, and other musculoskeletal conditions. Adolescents are more particular therapy may belong to more than 1 category; for
likely to use CIM than younger children. Other factors associated example, herbal preparations may have a biochemical and
with CIM use by children include parental education beyond high placebo (mind-body) effect.
school; higher household income; coverage by private health insur-
Whole Medical Systems
ance; use of prescription medications; and number of health con-
ditions, doctor visits, or school days missed for illness in the past Whole medical systems, including conventional medicine, are whole-
year. Non-Hispanic white patients are more likely to use CIM than system approaches to treatment, consisting of an underlying the-
Hispanic patients or black patients, although the strength of this ory of healing, standardized training, and diagnostic and treatment
association diminishes when data are adjusted for confounding fac- approaches reflective of the underlying theory. For instance, TCM is
tors. When worry about cost prevents the receipt of conventional based on the theory that illness and symptoms result from yin-yang
medical care, children are more likely to use CIM. The strongest energy imbalances. These energy imbalances are diagnosed through
predictor of CIM use by children is CIM use by a parent; children history and physical examination and treated by altering the energy
whose parents use CIM are 5 times more likely to use CIM. When balance using acupuncture (or other mechanical or thermal stim-
discussing CIM with families who are using it for their children, uli), herbs, and lifestyle changes (eg, diet, sleep, physical activity).
pediatricians need to be aware that parents may also be using CIM. Mind-Body Therapies
Mind-body therapies are intended to enhance the mind’s ability to
Motivations for Using CIM benefit health. A number of mind-body therapies are integrated into
Complementary and integrative medicine can be used for health conventional medical treatment, such as psychotherapy, group ther-
maintenance; for symptomatic relief, as an adjunct to curative con- apy, imagery, and biofeedback. Others are still considered CIM, such
ventional medical care; for relief from adverse effects of conventional as meditation and hypnotherapy.
medical care; or in place of conventional medical care. Families
choose to use CIM for many reasons. Word of mouth and belief in Biomechanical Therapies
the efficacy of the treatment can be strong motivators. Some par- Biomechanical therapies aim to improve health through physical
ents express a desire for more options and feel a sense of empower- manipulation of the body. This may involve working with mus-
ment in their parental role as a result of CIM use. Complementary cles (as with massage) or spinal alignment (as with chiropractic
and integrative medicine may also be more congruous with a family’s and osteopathic approaches). Massage therapies range from rela-
values, philosophies about health, and understanding of the basis of tively light muscle work to deep tissue massage and may be incor-
disease. Parents may fear the adverse effects of conventional medica- porated into physical therapy to work with muscles and joints.
tions or be dissatisfied with the care their child receives in conven- Spinal manipulation therapies are used commonly in the United
tional medical settings. Families may seek the additional personal States and are most often practiced by chiropractors or doctors
attention afforded by CIM providers. For some families, CIM offers of osteopathy.

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Table 15.1. Selected Examples of Complementary and Integrative Medicine Therapies by Category
Modality Description: At a Glance Licensure and Regulation
Ayurveda Originated in India several thousand years ago and is still used by 80% of the population exclusively or in Ayurvedic medicine is not accredited in the United States, although several states
combination with Western medicine. have accredited Ayurvedic schools.
Health and disease are thought to relate to a person’s constitution (prakriti), which is composed of a unique In India, Ayurvedic medicine can be studied at the bachelor and doctorate levels.
combination of the 3 life forces (doshas). Imbalances between the doshas can lead to disease.
Treatments are tailored to the individual’s unique constitution and are aimed at eliminating impurities, reducing
symptoms, increasing resistance to disease, reducing worry, and increasing harmony in the patient’s life.
Treatments include herbs, vitamins and minerals, massage, yoga, enemas, and specialized diet and lifestyle
recommendations.
Native American Broad term that encompasses the healing traditions of hundreds of indigenous tribes. Has been practiced in There is no government oversight of education of or licensure for Native American
healing North America for >40,000 years. healers.
Can combine religion, spirituality, herbal medicine, shamanic healers, purification activities, and symbolic
rituals in the treatment of medical and emotional problems. Treatments may be individual or may involve the
Whole Medical Systems

entire community.
Treatment is often a slow process that is spread over days and weeks. There is a strong belief in the therapeutic

Chapter 15: Complementary and Integrative Medicine in Pediatric Primary Care

value of the relationship with the healer.
Naturopathy Developed in Germany and the United States in the late 19th to early 20th centuries. Naturopathic physicians (ND or NMD degree) complete a 4-year graduate-
Central belief is that nature has healing power; practitioners view their role as supporting the body’s inherent level program at a naturopathic medical school accredited by the Council on
ability to restore health. Naturopathic Medical Education, which is recognized by the US Department of
Naturopathic practitioners strive to use the most natural, least invasive approach to restore and maintain health. Education. States vary in their licensing of naturopathic physicians and the scope
of naturopathic practice, including the ability to prescribe drugs, perform minor
Treatment modalities used by naturopaths include nutrition, vitamins and minerals, herbal medications,
surgery, and assist in childbirth.
homeopathy, therapeutic massage and joint manipulation, hydrotherapy, exercise, and lifestyle counseling.
The American Association of Naturopathic Physicians (www.naturopathic.org)
is the national professional society of naturopathic physicians.
TCM Originated in China >5,000 years ago; rooted in the Taoist philosophy. Most states license acupuncture but vary in their inclusion of other TCM treatments
TCM practitioners view the human body and mind as an integrated whole in which tissues, organs, and parts in licensure.
function interdependently. Acupuncture and TCM schools are accredited by the federally recognized
Yin-yang theory—the concept of 2 opposing but interdependent forces that shape the world—is a core Accreditation Commission for Acupuncture and Oriental Medicine.
principle. Health and disease relate to balance between yin and yang in the body.
Treatments to restore balance include herbs, massage, acupuncture, diet, and exercises such as tai chi and
qigong.
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PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT
Table 15.1. Selected Examples of Complementary and Integrative Medicine Therapies by Category (continued )
Modality Description: At a Glance Licensure and Regulation
Biofeedback Technique that trains people to improve their health by controlling certain bodily processes that typically The Biofeedback Certification International Alliance certifies individuals who have
happen involuntarily, including heart rate, blood pressure, muscle tension, and skin temperature. met their educational and training standards, which include didactic training,
Biofeedback practice involves attaching the patient to electrodes (or other monitoring devices) that monitor the supervision hours, patient sessions, and case manifestations.
desired process (eg, muscle tension, skin temperature). The therapist leads the patient in exercises designed to Treatment of pelvic floor muscle dysfunction is a subspecialty within biofeedback
assist in controlling the desired variable, while the electrodes provide real-time feedback on the patient’s success. that requires specialized training. Only licensed health care professionals may
Biofeedback is used to treat a variety of conditions, including high blood pressure, headaches, chronic pain, and apply for certification in this specialty.
urinary incontinence.
Hypnotherapy Hypnotherapists use exercises that bring about deep relaxation and an altered state of consciousness, also Most hypnotherapists are licensed MDs, registered nurses, social workers, or
known as a trance. family counselors who have received additional training in hypnotherapy. Several
Mind-Body Therapies

Through hypnosis, people learn how to master their own states of awareness. By doing so, they can affect their national bodies provide training certificates.
own bodily functions and psychological responses. The American Society of Clinical Hypnosis (www.asch.net) is the largest organization
Hypnotherapy has been studied in treatment of a number of conditions, including state anxiety (eg, before of health care professionals using clinical hypnosis and provides hypnotherapy
medical procedures or surgeries), headaches, smoking cessation, pain control, hot flashes in breast cancer training.
survivors, and irritable bowel syndrome. It is also used in managing pain during childbirth.
Meditation and There are many types of meditation, most of which originated from ancient religious and spiritual traditions. There is a broad diversity of meditation practices, each of which may have its own
mindfulness Some of these include mindfulness meditation, transcendental meditation, and Zen Buddhist meditation. training programs and certification policies.
Through meditation, a person learns to focus attention. Some forms of meditation instruct the practitioner to There is no national or state-based accreditation for meditation practitioner
become mindful of thoughts, feelings, and sensations and to observe them in a nonjudgmental way. education or licensing. However, meditation programs may be eligible for con-
Meditation is believed to result in a state of greater calmness, physical relaxation, and psychological balance tinuing education credits toward licensing for health care professionals.
and can change how a person relates to the flow of emotions and thoughts. The University of Massachusetts Medical School Center for Mindfulness in
Meditation has been studied in a variety of populations and is used for general wellness and various health Medicine, Health Care, and Society (www.umassmed.edu/cfm) is a long-standing
problems, including anxiety, pain, depression, stress, insomnia, and physical or emotional symptoms that may mindfulness program associated with an academic institution and is a source of
be associated with chronic illnesses such as AIDS and cancer. training and research on mindfulness and mind-body medicine.
Yoga Yoga practice was developed in ancient India, with fully developed practice appearing around 500 BCE. There There is no government oversight of yoga training or practice.
are numerous branches or paths of yoga. Numerous organizations and training programs in the United States and world-
Derived from the Sanskrit word meaning “union,” yoga strives to connect the body, breath, and mind with the wide offer training.
goal of energizing and balancing the whole person. The Yoga Alliance (www.yogaalliance.org) is the most widely recognized edu-
Yoga practice can be individual or class based and consists of physical postures (asanas), breathing exercises, cational and professional organization for people who teach yoga in the United
and meditation. States. It accredits yoga training programs using a minimum training standard.
Yoga is used for maintaining health and has also been studied in treatment of a wide variety of conditions, Teachers who complete Yoga Alliance–accredited training are eligible to become
including anxiety, arthritis, asthma, cancer, back pain, diabetes, heart disease, pregnancy (when modified for Registered Yoga Teachers.
pregnancy), and chronic headaches.
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Table 15.1. Selected Examples of Complementary and Integrative Medicine Therapies by Category (continued )
Modality Description: At a Glance Licensure and Regulation
Alexander Educational method that emphasizes changing faulty postural habits to improve mobility and performance. To be certified by the American Society for the Alexander Technique
technique Treatment has 2 components: table work (hands-on manipulation) and guided activity, in which the (www.amsatonline.org), practitioners must complete 1,600 hours of training
practitioner observes the person in action and gives verbal, visual, and physical cues to help the person over 3 years.
perform the activity with greater ease. There is no government oversight of training or licensure.
People use the Alexander technique to improve performance in performing arts and sports as well as in treat-
ment of musculoskeletal problems, repetitive stress injuries, and chronic pain.
Chiropractic care Developed in the United States at the end of the 19th century. Chiropractic practitioners must meet the licensing and continuing education
Based on the notion that the relationship between the body’s structure—most notably the alignment of the requirements of the states where they practice.
spine—and its coordination by the nervous system affects health. All states require chiropractors to complete a DC degree at an accredited college
People most often seek chiropractic care for musculoskeletal concerns (back, neck, and shoulder pain), head- The American Chiropractic Association (www.acatoday.org) is the largest
aches, and extremity problems. professional organization representing DCs.
Hands-on spinal adjustment and manipulation is a core treatment in chiropractic care, but treatment can also
Biomechanical Therapies

include heat and ice, electrical stimulation, exercise, and counseling about diet, exercise, and lifestyle.
Feldenkrais A method of somatic education that uses gentle movement and directed attention toward movement Feldenkrais Method practitioners must complete 700–800 hours of training over

Chapter 15: Complementary and Integrative Medicine in Pediatric Primary Care

Method patterns to expand movement options and improve functioning. 3–4 years to apply for certification. Certification and training is governed by the
Treatment can be carried out via verbal guidance in a class setting or with hands-on gentle manipulation. Feldenkrais Guild of North America (www.feldenkrais.com).
People use the Feldenkrais Method to maintain well-being and enhance performance in performing arts and There is no government oversight of training or licensure.
athletics, as well as in the treatment of a variety of conditions, including multiple sclerosis, stroke, cerebral
palsy, and repetitive stress injuries.
Massage therapy Massage therapy has been in use for thousands of years in diverse cultures, including those of China, Japan, Most states have laws regulating massage therapy and typically require a
India, Egypt, and Europe. minimum number of training hours and passage of a national examination.
The term massage therapy encompasses >100 distinct techniques, but the core practice is manipulation of the Massage training can be accredited by state boards or an independent agency.
muscles and soft tissues of the body using the hands and fingers. The National Certification Board for Therapeutic Massage and Bodywork (www.
People use massage for general wellness, as well as to treat a wide range of conditions, including sports ncbtmb.org) certifies massage practitioners who pass a national examination.
injuries, stress, pain, musculoskeletal concerns, anxiety, and depression. The American Massage Therapy Association (www.amtamassage.org) is the
nation’s largest professional organization for massage practitioners.
Osteopathy Developed in the United States at the end of the 19th century. DOs complete a 4-year training program in conventional medicine and osteopathy
Core principle is that disease and illness begin with structural problems in the spine and result in and also complete medical residencies in their specialty of choice.
abnormalities in the function of the nervous system. DOs are recognized for full practice rights in all 50 states once they are licensed.
DOs not only receive conventional medical training but are also trained in osteopathic manipulation The American Osteopathic Association (http://osteopathic.org) is the primary
techniques and craniosacral therapy, both of which involve hands-on manipulation of bones and tissues. professional organization and accreditation agency for DOs in the United States.
People seek care from DOs in conventional medicine and specifically for musculoskeletal conditions.
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Table 15.1. Selected Examples of Complementary and Integrative Medicine Therapies by Category (continued )
Modality Description: At a Glance Licensure and Regulation
Acupuncture Acupuncture is a modality of TCM. The earliest recorded use of acupuncture dates from 200 BCE. Most states require a license to practice acupuncture, although licensing
The core principle of acupuncture is the belief that a particular type of life force or energy (chi) circulates and education standards vary from state to state. Licensure confers the degree of
through the energy pathways (meridians) in the body. Chi maintains the dynamic balance of yin and yang. LAc.
An imbalance of chi can cause symptoms and disease. In 1997, the NIH recognized acupuncture as a mainstream medicine healing
Acupuncture treatment involves stimulation of specific points along the meridians using pressure (acupressure), option with a statement documenting the procedure’s safety and efficacy for
thermal energy (moxibustion), or very fine acupuncture needles. treating a range of health conditions.
Acupuncture has been studied in treatment of a variety of conditions, including chronic pain, postsurgical recov- The American Academy of Medical Acupuncture (www.medicalacupuncture.org)
ery, chemotherapy-related nausea, musculoskeletal problems, headaches, substance use, asthma, and men- maintains a database of licensed physicians who are also trained to
strual problems. perform acupuncture.
Bioenergetic Therapies

Homeopathic Developed by a German physician at the end of the 18th century. Homeopathic remedies are prepared according to the guidelines of the
remedies Based on 2 core principles: the principle of “similars” (“like cures like”) states that a disease can be cured by a Homoeopathic Pharmacopoeia of the United States, which was written into
substance that produces similar symptoms in a healthy person, and the principle of dilutions (“law of minimum federal law in 1938.
dose”) states that the lower the dose of the medication, the greater its effectiveness. Homeopathic remedies The US FDA requires that homeopathic remedies meet legal standards for
are so dilute that few or no molecules of the healing substance remain in the diluent. It is believed that the strength, purity, and packaging, but because they contain little or no active
substance has left its imprint, which stimulates the body to heal itself. ingredients, they are not subject to the same safety and efficacy testing as other
Homeopathic remedies are derived from natural substances that come from plants, minerals, or animals. over-the-counter medications.
If a homeopathic remedy is claimed to cure a serious disease such as cancer, it
needs to be sold by prescription.
Reiki Developed in Japan in the early 20th century. No licensing or government accreditation exists for the training or practice
Based on the idea that a universal energy supports the body’s innate healing abilities. Practitioners seek to of Reiki.
access this energy, allowing it to flow to the body and facilitate healing. Multiple organizations offer training programs and certifications.
During treatment, the practitioner’s hands are placed lightly onto or just above the client’s body, palms down,
using a series of 12–15 hand positions to promote the flow of energy. Each position is held for 2–5 minutes.
People use Reiki for relaxation, stress reduction, symptom relief, and general well-being. It can also be used to
help promote peace at the end of life.
Tai chi Originated in ancient China as a component of TCM. No licensing or government accreditation exists for the training or practice
Rooted in the principles of yin-yang balance and the balanced flow of a vital life force or energy (chi). of tai chi.
There are many different styles, but all involve slow, deliberate movements that flow into each other. A variety of organizations offer training programs and certification in tai chi.
Meditation, deep breathing, and maintenance of good posture during continuous movement are foci of the
practice.
People use tai chi to improve overall fitness, balance, coordination, and agility. It is also used to treat chronic
pain, gout, heart disease, high blood pressure, arthritis, osteoporosis, diabetes, headaches, and sleep disorders.
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BPPCA6e_Ch015_0095-0104.indd 101

Table 15.1. Selected Examples of Complementary and Integrative Medicine Therapies by Category (continued )
Modality Description: At a Glance Licensure and Regulation
Therapeutic touch Developed in the 1970s in the United States. No licensing or government accreditation exists for the training or practice of
Based on the belief that problems in the patient’s energy field that cause illness and pain can be identified and therapeutic touch.
rebalanced by a healer. Multiple organizations offer training programs, including some hospitals.
The technique consists of using the hands to release harmful energy and direct healthy energy for healing pur- Many therapeutic touch practitioners are nurses or other licensed health care
poses. During a session, the hands are held 5–15 cm (2–6 in) away from the body and there is no direct physical professionals.
contact between practitioner and patient.
Nutritional Nutritional approaches are an integral part of many whole medical systems, including Ayurveda, TCM, and Certification of registered dieticians is controlled by the Academy of Nutrition and
approaches naturopathy (see the Whole Medical Systems section in this table). Dietetics (www.eatright.org). Forty-seven states currently regulate licensure and
Numerous specialized diets have been promoted as offering benefits of health maintenance, weight loss, or certification of dieticians.
treatment of disease. Examples of diets that have demonstrated evidence of benefit in adult randomized Dieticians in Integrative and Functional Medicine is a specialty practice group within
controlled trials include the DASH (Dietary Approaches to Stop Hypertension) diet, low carbohydrate diets (such the Academy of Nutrition and Dietetics composed of practitioners who self-identify
as the Atkins diet), the Mediterranean diet, and a plant-based diet. Few specialized diets have been studied in as using an integrative approach to nutrition (https://integrativerd.org).
treatment of the pediatric population.
Dietary Dietary supplements contain ≥1 dietary ingredient, including vitamins, minerals, herbs, or amino acids, or their In the United States, dietary supplements can be sold without being tested to

Chapter 15: Complementary and Integrative Medicine in Pediatric Primary Care

supplements constituents. Herbal medications are considered a subset of dietary supplements in the United States. prove they are safe and effective. They must be made according to good
Biochemical Therapies

Some dietary supplements have been proven to prevent or treat disease (eg, folic acid in the prevention of manufacturing practices.
neural tube defects, calcium and vitamin D in the prevention of osteoporosis), while other claims about dietary In the United States, no organization regulates the manufacture or certifies the
supplements are unproven. labeling of dietary supplements.
The most commonly used dietary supplements are fish oil, Echinacea, flaxseed, ginseng, multivitamins,
vitamins E and C, calcium, and vitamin B complex.
Herbal medicine Herbal medicine refers to using a plant’s seeds, berries, roots, leaves, bark, or flowers for medicinal purposes. In the United States, herbal supplements are classified as dietary supplements and
Plants have been used for medicinal purposes since long before recorded history in a wide range of human cultures. can thus be sold without being tested to prove they are safe and effective. They must
According to the World Health Organization, 80% of the world’s population relies on herbal medications for be made according to good manufacturing practices.
some part of their primary care. Nearly one-third of Americans use herbs. In the United States, no organization regulates the manufacture or certifies the
The most commonly used herbal supplements in the United States include Echinacea, St. John’s wort (known labeling of herbal preparations.
botanically as Hypericum perforatum), gingko, garlic, saw palmetto, ginseng, goldenseal, chamomile,
feverfew, ginger, valerian, evening primrose, and milk thistle.
Herbal remedies are used in conventional medicine as well as a variety of CIM practices, including naturopathy,
TCM, Ayurveda, and Native American healing. Many people also use herbal remedies on their own,
without the advice of a health care professional.
Abbreviations: BCE, before the Common Era; CIM, complementary and integrative medicine; DC, doctor of chiropractic; DO, doctor of osteopathy; FDA, Food and Drug Administration; LAc, licensed acupuncturist; MD, medical doctor; NCCIH, National Center for Complementary and
Integrative Health; ND, naturopathy doctor; NIH, National Institutes of Health; NMD, naturopathy medical doctor; TCM, traditional Chinese medicine.
Information in this table is adapted from the health topics pages at the NCCIH website (https://nccih.nih.gov) and the American Cancer Society “Complementary and Alternative Medicine” page (www.cancer.org/Treatment/TreatmentsandSideEffects/
ComplementaryandAlternativeMedicine/index).
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Bioenergetic Therapies Table 15.2. Guide to Complementary and

Bioenergetic therapies are directed at improving health through Integrative Medicine Treatment Recommendations
altering the body’s energy as it runs through, on, or around the body. Is the Therapy Effective?
Because conventional training does not include the concept of energy Yes No
in this way, bioenergetic therapies tend to draw skepticism from con-
Is the Therapy Yes Recommend Tolerate
ventional medical providers. With increasing scientific evidence of its
Safe? No Monitor closely or discourage Discourage
beneficial effects, acupuncture is purported to affect the body’s vital
energy (chi) as it courses through energy channels called meridians. If there is insufficient evidence to assess safety or efficacy
In 1997, an NIH consensus panel declared the evidence sufficient for wwEstablish a plan with the family for monitoring response to treatment.
integration of acupuncture into a number of conventional treatments, wwDiscourage if adverse reactions develop.
including treatment of chemotherapy-associated nausea and eme- wwTolerate if no adverse reactions develop.
sis, anesthesia-associated nausea and emesis, and pain syndromes.
Homeopathic remedies are theoretically bioenergetic therapies, as the
“ingredients” are successively diluted beyond the point of significant
molecular presence; therefore, it is the resulting change in the energy Assessing Efficacy
of the diluents (sometimes called the “memory of the molecule”) that
Data on the efficacy of CIM treatments are increasingly available
is responsible for the therapeutic effect. In addition, a number of ther-
as CIM becomes a greater focus of research effort and funding in
apies aim to affect energy at or near the surface of the body, including
the United States and worldwide. When searching the medical
therapeutic touch and Reiki, both of which involve a trained practitio-
literature for CIM evidence, international and foreign-language
ner identifying the energy imbalance and directing the patient’s own
articles can be useful, as certain CIM modalities may have been
energy to improve the patient’s energy balance and promote healing.
studied in greater depth or over a longer period in other coun-
Biochemical Therapies tries. As with any research data, it is important to consider study
design, outcome measures, sources of bias, methods of data anal-
Biochemical therapies are intended to improve health through their
ysis, and applicability of results for a given indication, patient, or
biochemical effects and include nutritional approaches, herbal reme-
patient population. With herbal remedies and supplements, it is
dies, and dietary supplements. Nutrition is an important part of many
also important to acknowledge that formulations differ, so effi-
CIM approaches, such as Ayurveda, TCM, and naturopathy, and is also
cacy as reported in a clinical trial may be altered if a different for-
an accepted part of the conventional medicine approach to disease pre-
mulation is used.
vention and treatment. As the body of research on integrative nutri-
tional approaches has grown, some interventions that originated as Assessing Safety
integrative approaches, such as the Mediterranean diet, have become
The huge diversity of CIM makes it difficult to discuss safety in
part of evidence-based conventional medicine. Herbal remedies and
general terms. Each CIM modality has its own unique risk profile.
dietary supplements are conceptually related to the biochemical mech-
However, awareness of broad categories of risk can help physicians
anism of effect of conventional medications but have a few important
prevent bad outcomes.
differences. First, herbal remedies are by nature complex mixtures of
ww Delay of conventional care: In studies of CIM risks for the pedi-
chemicals, so their effects are likely caused by multiple biochemical
atric population, the risk associated with the highest morbidity
reactions. Second, there is currently no federal regulation or oversight
and mortality rates is delay of conventional care. This delay can
of the production of herbs or supplements in the United States, as there
come about for a number of reasons, including a family’s percep-
is with pharmaceuticals, so quality control is extremely variable. Third,
tion that care from a CIM provider obviates the need for conven-
safety concerns are addressed not before they are available commer-
tional care, a family’s inability to afford conventional care, or a
cially to consumers, as with pharmaceuticals, but only after products
family’s belief that conventional care will be harmful. It may be
are on the market and adverse events are reported to the US Food
helpful to contract with families that they will seek conventional
and Drug Administration. Because of the lack of oversight of produc-
care before or in conjunction with CIM care for a new symptom
tion and premarket safety, it is important for practitioners to research
or acute illness to rule out disease processes that require con-
not only the evidence of an herb’s effect and potential adverse effects
ventional treatment.
but also the particular formulation’s production and quality control.
ww Drug-drug interactions: Certain supplements and herbal
remedies may interfere with or alter the effects of conventional
Approaching CIM Use From a medications. Particular attention has been given to substances
Conventional Medicine Perspective that are inhibitors or inducers of hepatic drug metabolism
As with any treatment option, the safety (risks) and efficacy (bene- by the cytochrome P-450 enzymes, with St. John’s wort (used
fits) of a CIM modality must form the basis for therapeutic decision- for depression; known botanically as Hypericum perfo-
making about its use. Table 15.2 can be a helpful guide in directing ratum) and grapefruit juice being the most well-known
physician responses to a particular CIM therapy. examples. See the Resources for Physicians section for online

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 Chapter 15: Complementary and Integrative Medicine in Pediatric Primary Care 103

resources for checking drug-drug interactions with herbal rem- may attribute to it (irrationality given lack of evidence and the threat
edies and supplements. of interference with conventional medical care). This disconnect can
ww Adverse reactions: Allergic reactions, adverse effects, and idio- influence patients and physicians to avoid the subject of CIM.
syncratic reactions are possible with CIM therapies as well as
conventional therapies. Close follow-up is helpful in monitor-
Communication Tips
ing response to therapy.
Effective communication about CIM use is a wonderful oppor-
Regulation and Licensure tunity to improve rapport and better understand a patient and
family (Box 15.1). Communication can be a powerful tool for ensuring
Licensure and accreditation for CIM providers varies from state to
safety and reducing harm, as well as for broadening physician knowl-
state. Chiropractic care, massage therapy, acupuncture, naturopa-
edge and building trust with families. Even if they do not initiate or
thy, and homeopathy have licensing bodies in some states. In states
recommend a CIM treatment, pediatricians can play an impor-
where licensing exists for a CIM modality, it is incumbent on the
tant role by monitoring a child’s response to the treatment over
physician to ensure that any referrals are made to licensed practitio-
time and engaging the family in a discussion about risks and ben-
ners. None of these licenses authorizes the practitioner to practice
efits if adverse reactions arise. Discussion about CIM may also
medicine. Other CIM modalities may have a national organization
bring about a greater understanding of families’ explanatory
that supervises training and certifies practitioners. Although these
models of illness as well as their expectations of health care pro-
are not subject to government oversight, there still may be value
fessionals and their beliefs about conventional medications.
in preferentially seeking out providers who are approved by their
Deeper insight into a family’s health beliefs and values can
national organization, as these providers have had to meet a stan-
facilitate a more successful patient-physician partnership and
dard established by their colleagues.
better patient care.
Regulation of herbal remedies and supplements is the sub-
ject of ongoing scrutiny and debate. Currently, the US Food and
Drug Administration does not regulate production or marketing
of these products, so it is often difficult to verify their composi- Box 15.1. Complementary and Integrative
tion, safety, or efficacy unless they have been independently stud- Medicine Communication Tips
ied. The burden of researching products and manufacturers falls ww Make questions about complementary and integrative medicine (CIM)
to the consumer. use a routine part of the medical encounter.
ww Pose questions about CIM use in an open and nonjudgmental way. For
Communication About CIM Use example, “For me to take the best possible care of your child, it is help-
ful to know about all the ways your family manages health and illness.
Most caregivers of children who use CIM do not disclose this use
Are there any treatments, medications, herbs, or supplements that your
to their pediatrician despite that most report a desire to discuss it.
child uses but we have not talked about yet? Does your child see any
The high rate of nondisclosure is alarming, as it places patients at
other providers for health-related care or treatments?”
risk for drug-drug interactions with conventional medications, robs
ww Explore details of CIM use. Ask families not only what CIM modalities
physicians of the opportunity to monitor for adverse reactions, and
they are using but also why they chose each treatment, how it works,
interferes with the development of trust in the patient-physician
whether they have noticed a difference, and if there have been any
relationship. Reasons cited by patients and caregivers for nondis-
downsides to the treatment.
closure include
ww Validate the family’s desire for health and well-being for their child.
ww Negative experiences with past disclosures to physicians.
ww Do not be afraid to acknowledge the limits of your knowledge. If asked
ww Fear of disapproval or judgment on the part of physicians.
to provide a recommendation about an unfamiliar CIM modality, offer
ww Belief that physicians do not need to know.
to do further research, and revisit the question at a follow-up visit.
ww Lack of time with physicians.
ww Seek out information about CIM, including literature on safety or efficacy.
ww Physicians do not ask.
ww Involve families in the thinking process of comparing risks with benefits
Many of these barriers can be addressed by the physician.
of a CIM intervention or modality. Provide evidence-based advice when
Physicians may be reticent to discuss CIM, as evidenced by the fact
possible. If there is little or no evidence to support or discourage use of a
that CIM discussions are patient initiated in most cases. Physicians
CIM modality, share this information with families.
may worry about legal liability if there is a bad outcome. They may
ww Make a plan with families to monitor their child’s response to treatment,
fear conflict with families over use of CIM. They may be afraid to
including measurable outcomes (eg, symptom relief, increased quality of
reveal their lack of knowledge about CIM and may worry that this
life) and any adverse effects.
lack of knowledge will threaten a family’s trust in their abilities. They
ww Encourage families to share information about CIM use continually, even
may feel pressured by time constraints and overwhelmed by the need
if they choose to continue a treatment about which you have raised
to acquire new knowledge. There is often a disconnect between the
concerns.
meaning that patients and families attribute to CIM use (more options
ww Document CIM-related discussions in the medical record.
and a greater sense of empowerment) and the meaning that physicians

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 104 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

modalities for providers and patients, results of clinical trials

CASE RESOLUTION supported by NCCIH, lists of ongoing trials, and information about
Explaining that CIM is not your area of expertise, you ask the family to return in training and funding opportunities in the field of CIM.
1 week to discuss this further. After consulting the NCCIH website and conducting
Natural Medicines (Product Information)
a brief review of the medical literature, you find that there are data on efficacy
and safety of CIM therapies for migraine headache prevention, including acu- https://naturalmedicines.therapeuticresearch.com
puncture and self-hypnosis. At the follow-up visit, you discuss these options with Subscription-based site that includes evidence-based information
the family. The girl is interested in both modalities but decides to try acupunc- on CIM modalities as well as online tools to check for drug-drug
ture first. You offer her support for this choice and ask that she keep close track of interactions.
her headaches and return in 1 month to let you know how she is doing with the
acupuncture treatments and her headaches. NSF International
www.nsf.org/services/by-industry/nutritional-products
An independent organization that tests and certifies a wide range of
products and systems, including dietary supplements. Before certify-
Resources for Physicians ing a product, NSF International performs toxicology tests to ensure
CAM on PubMed that the contents match what is on the label and that the product
https://nccih.nih.gov/research/camonpubmed does not contain unhealthy levels of contaminants such as heavy
The NCCIH provides a link to a PubMed search box that is limited metals, pesticides, or herbicides.
to the CIM subset of PubMed. USP
Cochrane Complementary Medicine www.usp.org/dietary-supplements-herbal-medicines
https://cam.cochrane.org/evidence A nonprofit organization that develops and publishes standards
Link to more than 700 Cochrane reviews on CIM interventions. The for strength, quality, and purity of drugs, dietary supplements, and
Cochrane Collaboration is an independent, international network food ingredients. Dietary supplements must meet these standards
of leaders in the health care field who are committed to helping to display a USP Verified Mark on their labels.
health care professionals make evidence-based decisions. Subgroups
devoted to specific topic areas produce systematic reviews of avail- Selected References
able evidence of health-related interventions. The CIM-related sub- American Academy of Pediatrics Section on Integrative Medicine. Mind-body
group is coordinated through the University of Maryland School of therapies in children and youth. Pediatrics. 2016;138(3):e20161896 PMID:
Medicine Center for Integrative Medicine. 27550982 https://doi.org/10.1542/peds.2016-1896
Black LI, Clarke TC, Barnes PM, Stussman BJ, Nahin RL. Use of complementary
ConsumerLab.com
health approaches among children aged 4–17 years in the United States: National
www.consumerlab.com Health Interview Survey, 2007–2012. Natl Health Stat Rep. 2015;(78):1–19
This for-profit organization carries out independent testing of dietary
Clarke TC, Black LI, Stussman BJ, Barnes PM, Nahin RL. Trends in the use
supplements and herbal remedies by request of manufacturers and of complementary health approaches among adults: United States, 2002–2012.
for the benefit of the public. In either case, samples for testing are Natl Health Stat Report. 2015;(79):1–16 PMID: 25671660
obtained not directly from the manufacturer but from the consumer Culbert TP, Olness K. Integrative Pediatrics. New York, NY: Oxford University
marketplace. The ConsumerLab.com seal that appears on vitamin Press; 2010
bottles can be purchased by manufacturers once their product has Kemper KJ. The Holistic Pediatrician: A Pediatrician’s Comprehensive Guide
been tested by ConsumerLab.com. to Safe and Effective Therapies for the 25 Most Common Ailments of Infants,
Medscape Drug Interaction Checker Children, and Adolescents. 2nd ed. New York, NY: HarperCollins; 2002

https://reference.medscape.com/drug-interactionchecker McClafferty H. Integrative Pediatrics: Art, Science, and Clinical Application.

Free website that offers users the opportunity to look up drug-drug New York, NY: Routledge; 2017

interactions and includes herbal supplements as well as conventional McClafferty H, Vohra S, Bailey M, et al; American Academy of Pediatrics
prescription and over-the-counter medications. Section on Integrative Medicine. Pediatric integrative medicine. Pediatrics.
2017;140(3):e20171961 PMID: 28847978 https://doi.org/10.1542/peds.
National Center for Complementary and Integrative Health 2017-1961
at the National Institutes of Health Misra SM, Verissimo AM. A Guide to Integrative Pediatrics for the Healthcare
https://nccih.nih.gov Professional. Cham, Switzerland: Springer International Publishing; 2014 https://
The NCCIH is the federal government’s lead agency for scientific doi.org/10.1007/978-3-319-06835-0
research on CIM. The website provides information about CIM Rakel D. Integrative Medicine. 3rd ed. Philadelphia, PA: Elsevier Saunders; 2012

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 CHAPTER 16

Principles of Pediatric Surgery

Roxanne L. Massoumi, MD, and Steven L. Lee, MD, MBA, FACS, FAAP

CASE STUDY
A 4-month-old boy is evaluated by his pediatrician for 2. What is the role of the primary care physician
swelling in the groin and is diagnosed with a right ingui- in advising patients and parents about surgical
nal hernia. His parents are told that their child will be procedures?
referred to a pediatric surgeon. The parents are con- 3. What is the role of the surgeon in advising patients
cerned about surgery in such a young infant and ask and parents about the surgery?
their pediatrician multiple questions. Is he big enough to 4. What are general guidelines for feeding infants and
have surgery? Will he be able to eat before the surgery? children prior to surgery?
Will he be in pain? Will he need to have blood drawn? 5. What are the risks of general anesthesia in infants
Will he need to be hospitalized? Will he be put to sleep and children?
for the procedure? 6. How long is the hospitalization after surgery?
7. How do physicians prepare children who are about
Questions to undergo surgery?
1. What are the typical questions parents ask if their
8. What laboratory studies are needed prior to
child is undergoing surgery?
surgery?

No matter the age of the patient, the prospect of surgery is gener- parents about the correct diagnosis and advise them that surgery
ally anxiety-provoking for the patient and the patient’s parents. As is not necessary.
with any stressful patient encounter, communication is the key to The parents and children are given a significant amount of infor-
educating, calming, and reassuring patients and their parents. This mation during the surgical consultation. A referring diagnosis is
is particularly true in case in which patients are being referred, either confirmed or not, and a decision is made about whether the
even initially, for surgical consultation. Pediatricians can provide patient requires an operation. If surgery is indicated, it is described
significant support by giving the parents basic information about in detail to the parents and, if age-appropriate, to the patient as well.
surgical care. It is also important for primary care physicians and A thorough review of the risks and benefits of as well as any alter-
surgeons to communicate with each other to provide the high- natives to the procedure is undertaken. An estimate of how long the
est level of care. procedure will take is given. A general discussion on the expected
recovery is conducted, such as whether the procedure will be per-
formed as outpatient or inpatient, where hospitalization occurs, how
Preoperative Care
long the child will miss school, and whether any activity restric-
Initial Consultation tions exist.
When a surgical problem is suspected, the first task of the pri- Parents and patients should be encouraged to compile a list of
mary care physician is to initiate a referral to a pediatric or gen- questions before their first clinic visit to make sure that questions
eral surgeon. The purpose of the initial surgical visit is to confirm are not left unanswered. Parents and patients should also write
the diagnosis and discuss surgical and, if applicable, nonsurgi- down the answers to refer to them at a later time. Typical ques-
cal, treatment options. Surgical procedures are rarely performed tions include the length of time of the procedure itself, the use of
during this consultation, and this fact should be stressed by the anesthesia, and the length of recovery time. If parents agree to
primary care physician prior to the surgical consult. Many chil- proceed with surgery, the surgeon starts the process and paper-
dren and parents mistakenly think that surgery will occur at the work. Often, this is the only visit required before the date of the
time of the first encounter and are therefore unduly anxious. Some operation. However, some hospitals require patients to be seen by
parents even schedule time off from work unnecessarily with the the surgeon within 30 days of the procedure; thus, a return visit
expectation of having to provide postoperative care for their child, may be required if the date of surgery is beyond that time frame.
and they become disappointed and even angry when the surgery Additionally, some hospitals and surgery centers require patients
is not performed. Sometimes the diagnosis of the primary care to undergo a preoperative evaluation by the anesthesia team prior
pediatrician is incorrect, in which case the surgeon can reassure to surgery.

105

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 106 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Preparation of Anesthesia for Infants Laboratory Studies

and Children Laboratory studies, such as complete blood cell count, electrolyte
Anesthesia is extremely safe in infants and children, and the risk panel, and/or blood typing, should be performed if indicated by the
of a poor outcome from general anesthesia is less than 1% at most patient’s underlying comorbidities or current disease process. Blood
institutions. Specialized pediatric anesthesiologists provide anes- products may be needed during or after surgery if there is blood
thetic care for infants and young children. If a pediatric anesthesiol- loss. If blood loss is anticipated, parents or other family members
ogist is not available, anesthetic care for infants and children is often will be given the option to directly donate blood prior to the proce-
limited to a set of experienced general anesthesiologists. Parents dure. Direct donation of blood can take days to weeks depending
and patients often experience anxiety about undergoing anesthesia. on the specific blood bank, however, and it may not be an option
Videos have been created and are available at many institutions to for urgent or emergent operations. Additionally, the cost of directly
help explain the anesthesia process. These videos have been shown donating blood is often the responsibility of the patient. If primary
to increase medical knowledge and decrease anxiety about under- care physicians need laboratory tests performed for other reasons,
going anesthesia. often the necessary blood work can be obtained while the patient
Many types of anesthesia exist, including moderate sedation is under anesthesia.
(ie, conscious sedation), regional anesthesia, and general anes-
thesia. Typically, the type of anesthesia is determined by the sur- Imaging Studies
geon and anesthesiologist; however, the anesthesiologist makes The parents or guardians should bring with them to the surgical
the final decision. Although the least amount of anesthesia is consultation copies of any imaging studies obtained by the primary
desired, all patients must be treated as though they may require care physician. Hard copies or electronic files of the imaging itself
general anesthesia in case moderate sedation or regional anesthe- as well as the final report by the radiologist are strongly recom-
sia is inadequate to provide the required anesthesia for the indi- mended. If imaging studies are needed for further workup or preop-
vidual operation. erative planning, these studies can be ordered later by the surgeon.
To minimize the risk of aspiration when undergoing general If studies are to be ordered preoperatively by the primary care phy-
anesthesia, the stomach must be empty of food and liquids. Although sician, that physician should contact the surgeon to confirm which
each hospital has its own specific policies, Box 16.1 lists frequently specific studies to order. This communication will avoid obtaining
recommended guidelines for the interval between meals and sur- unnecessary studies or the need to repeat any studies that were not
gery. In general, most infants and children must miss 1 meal before done appropriately.
surgery. Often, it is possible to replace this meal with clear liquids.
All medications should be continued unless otherwise directed Patients Requiring Urgent/Emergent
by the surgeon or anesthesia staff, and they can be taken with a small
Surgery
sip of water on the morning of surgery. It is also recommended that Patients with acute conditions often present first to their pediatri-
the parents or guardians bring a list of the child’s medications on cian or primary care physician. Common conditions include abdom-
the day of the operation so that the surgical staff can review the list inal pain consistent with appendicitis, an abscess requiring drainage,
preoperatively. an incarcerated hernia, or vomiting caused by pyloric stenosis. Such
patients should be directed to the emergency department (ED) for
further workup and management, and the pediatric surgeon should
be notified. Any time a patient is sent to the ED for a possible surgi-
Box 16.1. Guidelines for the Interval
cal issue, it is essential to remind the parents to not give the patient
Between Eating/Drinking and Surgery
anything to eat or drink (ie, nil per os [NPO]). If parents feed patients
in Infants and Children
en route to the ED, treatment will be delayed because of anesthesia-
<6 Months of Age related concerns. The NPO guidelines in Box 16.1 are enforced even
ww 4 hours for human milk/formula in urgent conditions; only in life-threatening emergencies may the
ww 2 hours for clear liquidsa guidelines be disregarded. In most acute situations, patients are
6–36 Months of Age often dehydrated as a result of vomiting, poor oral intake, or third-
ww 8 hours for solids spacing of fluid. Thus, it is essential that patients be adequately resus-
ww 6 hours for human milk/formula citated preoperatively. A standard intravenous (IV) fluid resuscitation
ww 2 hours for clear liquidsa of 20 mL/kg of lactated Ringer injection or normal saline should
be administered, followed by continuous administration of main-
>36 Months of Age
tenance (or slightly higher rate of) fluids. Appropriate antibiotics
ww 8 hours for solids/milk
also should be administered as indicated. Patients with acute appen-
ww 2 hours for clear liquidsa
dicitis should be started on single-, double-, or triple-agent broad-
a
Formula, milk, orange juice, and colas are NOT considered clear liquids. Clear liquids include any spectrum antibiotics depending on their symptoms and the preferences
liquid that can be seen through (eg, water, sugar water, apple juice, Jell-O, broth). of the surgical team. Patients with abscesses should be administered

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 Chapter 16: Principles of Pediatric Surgery 107

antibiotics with excellent gram-positive coverage, with special atten- Controversy persists about the postoperative management of
tion to methicillin-resistant Staphylococcus aureus, given its high former preterm infants after undergoing general anesthesia because
prevalence. Pain medication is given as needed. It is rare that a true they are at increased risk for postoperative apnea. Reasonable rec-
surgical condition is masked by the appropriate dose of pain medica- ommendations for typical outpatient operations include over-
tion; thus, pain medication can be given even if the surgeon has not night admission with apnea monitoring for former preterm infants
yet evaluated the patient. Finally, basic laboratory studies, such as a who were born at 52 weeks’ postconception age or younger. However,
complete blood cell count and electrolyte panel, should be obtained. each institution has its own specific policy, and the exact age for
required admission varies. The decision for admission is also based
Perioperative Care on the patient’s current health and comorbidities and should be
made by the surgeon, anesthesia team, primary care physician,
The final operating room schedule for elective cases is often made
and the patient’s parents. Table 16.1 lists the most common elective
the day before surgery. Thus, the specific time of an elective proce-
and urgent/emergent operations and anticipated postoperative
dure is not known until 1 day prior to surgery. Often the surgeon
hospitalization stays.
can give parents an estimated start time. Families are contacted the
day before surgery to confirm the time of the procedure as well as
when and where to arrive. Patients are typically asked to arrive 1.5
Table 16.1. Common Elective and Urgent/
to 2 hours before the scheduled start time to allow sufficient time
Emergency Operations and Anticipated
to ensure patient safety by completing all necessary steps before the
Postoperative Hospitalization
actual operation.
In general, patients are put under anesthesia when they arrive Length of
in the operating room. For infants and young children, an IV line is Type of Procedure Procedure Hospitalization
placed after the patient is asleep. To minimize anxiety, preanesthetic Elective Inguinal hernia repair Nonea
sedation is administered orally before proceeding to the operating Umbilical hernia repair Nonea
room. Once in the operating room, patients are often administered Cyst excision Nonea
an inhalational anesthetic, followed by placement of the IV line, then
Central venous catheter Nonea
intubation. Some institutions allow parents to accompany their child
insertion/removal
to the operating room and see the child put to sleep with the inha-
lational anesthetic. Orchiopexy Nonea
After the operation is complete, patients are brought to the recov- Laparoscopic cholecystectomy Nonea
ery room. Parents typically are allowed in the recovery room until Lymph node biopsy Nonea
the time of discharge. Criteria for discharge from the recovery room Ostomy takedown 3–4 days
are listed in Box 16.2. For children who have undergone outpa-
Lung resection 3–4 days
tient surgery, careful written instructions are provided to parents
along with dates and times of follow-up appointments as well as tele- Imperforate anus repair 3–4 days
phone numbers for use should problems arise. Children who have Pectus excavatum repair (ie, 4–5 days
undergone inpatient procedures are discharged from the recovery Nuss procedure)
room to the pediatric inpatient service, along with appropriate Urgent/Emergency Laparoscopic or open appen- 1–2 days
orders for nursing personnel. Child life specialists are available dectomy for nonperforated
at many institutions to help acclimate patients to the hospital and appendicitis
perioperative setting. These specialists provide information, com- Laparoscopic or open appendec- 5–7 days
fort, and reassurance, and they offer general positive reinforcement tomy for perforated appendicitis
to patients.
Pyloromyotomy 1 day
Intussusception reduction 1 day
Box 16.2. Criteria for Discharge Bowel resection 5–7 days
After Outpatient Surgery
Bowel obstruction 5–7 days
ww Patient is stable and exhibits age-appropriate vital signs Tumor resection (ie, Wilms 7 days
ww Age-appropriate ambulation tumor or neuroblastoma)
ww Ability to tolerate oral fluids Choledochal cyst or 5–7 days
ww No bleeding portoenterostomy
ww No respiratory distress
Pull-through for Hirschsprung 5–7 days
ww No pain that cannot be controlled by oral medication
disease
ww Age-appropriate alertness
a
Outpatient procedure.

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 108 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Postoperative Care The main reasons to contact the surgeon postoperatively are a
temperature above 38.6°C (101.5°F), worsening or persistent pain,
Parents often call their child’s primary care physician with questions
persistent emesis, and any wound drainage or redness. Additional
postoperatively; thus, it is important that the physician be knowl-
instructions specific to the operation are also provided to the parents.
edgeable about routine postoperative care. Most children recover
from anesthesia and surgery faster than adults. Unless otherwise
specified, such as after an appendectomy for perforated appendi- CASE RESOLUTION
citis, a normal diet can be resumed as soon as the patient has an In this case, the pediatrician reassured the parents that many patients have been
appetite. If patients cannot tolerate a normal diet, a bland diet and referred to this surgeon. The parents were assisted by the pediatrician in crafting
plenty of fluids are recommended. Furthermore, unless otherwise their questions for the surgeon. The surgeon subsequently confirmed the diagnosis
of an inguinal hernia and recommended surgery. All details were provided, and the
specified patients should resume taking all their preoperative med-
parents’ questions were answered. The patient was scheduled for outpatient surgery,
ications the evening of or morning after surgery. and everything went smoothly. Postoperatively, there were no concerns, and the
Almost all surgical wounds are closed with absorbable sutures patient saw the surgeon and pediatrician in the course of standard follow-up care.
and covered with thin adhesive strips. Additional dressings can be
removed 1 to 2 days postoperatively or as needed. By 48 hours post-
operatively, wounds do not need to be covered unless they are open Selected References
or there is drainage. Unless otherwise specified, most wounds can be
American Academy of Pediatrics Section on Hospital Medicine; Gershel JC,
exposed to water (ie, shower, quick bath) 48 hours after surgery. With
Rauch DA, eds. Caring for the Hospitalized Child: A Handbook of Inpatient
the exception of thin adhesive strips, dressings should be removed Pediatrics. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics; 2017
prior to bathing.
Bandstra NF, Skinner L, Leblanc C, et al. The role of child life in pediatric pain
The surgeon provides parents and the child details about activity management: a survey of child life specialists. J Pain. 2008;9(4):320–329 PMID:
limitations and returning to school. Often, heavy physical activity, 18201933 https://doi.org/10.1016/j.jpain.2007.11.004
such as physical education in school or organized sports, should be Jackson HT, Kane TD. Advances in minimally invasive surgery in pediat-
avoided until the surgeon sees the patient after discharge. Although ric patients. Adv Pediatr. 2014;61(1):149–195 PMID: 25037127 https://doi.
heavy physical activity is to be avoided after surgery, patients should org/10.1016/j.yapd.2014.03.011
be encouraged to walk as well as to cough and breathe deeply many Landsman IS, Hays SR, Karsanac CJ, Franklin A. Pediatric anesthesia. In: Coran
times a day to avoid atelectasis. Patients can return to school when AG, Adzick NS, Krummel TM, Laberge JM, Shamberger R, Caldamone A, eds.
they feel ready and have only minimal pain. Pediatric Surgery. 7th ed. Philadelphia, PA: Mosby; 2012:201–226

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 CHAPTER 17

Image Gently Approach

to Pediatric Imaging
Jane S. Kim, MD; Lindsay S. Baron, MD; and Benjamin H. Taragin, MD

CASE STUDY 1 with purulent nasal discharge. Tenderness to palpation

A 15-year-old boy comes to your office reporting back is elicited over the cheeks and forehead.
pain after exertion for the past week. He reports no Questions
significant recent trauma. He plays varsity basketball 1. How does imaging contribute to the diagnosis of a
but has not had any falls during recent games. He is patient’s condition?
otherwise healthy, with no significant medical history. 2. How does the physician determine which imaging
The pain does not prevent him from playing sports or studies are appropriate for an individual patient?
attending school. He has no history of prior episodes of back 3. What is the ALARA principle?
pain. He reports that the pain is relieved by nonsteroidal 4. What information is available for counseling
anti-inflammatory drugs. On physical examination, he has patients about the risks of diagnostic radiation?
left-sided paraspinal focal tenderness in the L3-L4 region. 5. Where can appropriate imaging recommendations
He has limited range of motion twisting to that side. for pediatric patients be found?
6. Does patient history influence the choice of imaging
CASE STUDY 2 studies?
A 10-year-old girl is brought to your office with runny a. Would imaging be appropriate for the patient
nose, congestion, cough, and headache. You saw this in Case Study 1 had he recently experienced
patient 6 weeks ago as well as 4 months ago, when she significant trauma?
had similar symptoms. Her mother reports full compli- b. Would imaging be appropriate for the patient in
ance with the antibiotic regimen you prescribed but Case Study 2? What if her symptoms had been
states that her daughter’s symptoms have never fully new and begun only the week before this lat-
resolved. On physical examination, the child is afebrile est office visit?

Overuse of medical imaging is a growing problem in the United Campaign, with a focus on minimizing the use of diagnostic
States, with financial and medical repercussions. The primary care radiation when possible and using appropriate pediatric imag-
physician must often decide what, if any, imaging is appropriate ing for children in the United States. Because of the widespread
for a particular patient. In many situations, clinical assessment attention and highly concerted efforts of the organizations
and physical examination are adequate for diagnosis. In some involved in this effort, several recent studies have observed
instances, however, imaging provides vital information that can- a slight downward trend in CT use for pediatric patients in
not be adequately obtained from other sources. Patients and their the United States. Because of the substantial variation among
families may also insist on diagnostic imaging based on the belief different imaging practices, however, the opportunity exists for
that the more technologically advanced the evaluation, the bet- continual improvement.
ter the care.
Approximately 370 million studies using diagnostic radia- Basic Concepts
tion are performed annually in the United States (Figure 17.1). Diagnostic radiology examinations that use ionizing radiation
The most dramatic increase has been in the use of computed include plain radiography, fluoroscopy, CT, and nuclear medicine.
tomography (CT). In 1982, 1 million CT scans were performed Ionizing radiation may cause damage to DNA molecules. Children
in the United States. In 2006, 67 million CT scans were per- are more radiosensitive than adults secondary to children’s growth
formed, with an estimated 4 to 7 million of these performed in of rapidly proliferating cells and longer life span. Certain organs are
children. In response, many concerned health professionals more radiosensitive than others, and children in particular are at
and radiologists gathered to create the national Image Gently an increased risk for breast cancer, thyroid cancer, and leukemia.

109

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 110 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Medical All other 0.05 Table 17.1. Comparison of Radiation

0.53 mSv
Dose by Imaging Procedure Type
for Pediatric Patients
Number of Days
Number of of Background
Natural Bkd 2.4 mSv Typical Chest X-rays Radiation for
TOTAL Diagnostic Effective Dose for Equivalent Equivalent
–3.0 mSv
Natural Bkd 2.4 mSv Procedure (mSv)a Effective Dose Effective Dose
Chest x-ray (PA) 0.02 1 72
Consumer products
Occupational 0.005 mSv 0.13 mSv Skull x-ray 0.07 3.5 255

Nuclear power 0.005 mSv

Lumbar spine 1.3 65 159
Natural Bkd
2.4 mSv Upper GI 3 150 366
Barium enema 7 350 852
Medical 3.0 mSv Head CT ≤2 ≤100 243
TOTAL Chest CT ≤3 ≤150 366
–5.6 mSv Abdomen CT ≤5 ≤250 609
a
Natural background radiation is 3 mSv per year.
Abbreviations: CT, computed tomography; GI, gastrointestinal; PA, posteroanterior.
Figure 17.1. Charts showing the United States annual per capita effective Derived from the Image Gently Alliance and the Society for Pediatric Radiology.
radiation dose from various sources. Upper: Chart for 1980. Lower: Chart
for 2006.
Abbreviation: Bkd, background.
Reprinted with permission from Mettler FA Jr, Bhargavan M, Faulkner K, et al. Radiologic and
nuclear medicine studies in the United States and worldwide: frequency, radiation dose,
and comparison with other radiation sources—1950-2007. Radiology. 2009;253(2):
520–531, with permission.

One of the most common ways to measure radiation dose is by

the effective dose, measured in sieverts, which takes into account all
the exposed tissues and the relative radiosensitivity of each organ.
The effective dose also provides a way to compare radiation dose
across different imaging modalities. For example, for children 1 CT
scan of the chest imparts an effective dose equivalent to up to 150
chest x-rays (Table 17.1).
Figure 17.2. Graphic representation of the relative risk of contracting cancer
in atomic bomb survivors. The dashed curves represent 61 standard error for
The ALARA Principle the smoothed curve. The straight line is the linear risk estimate computed
In recent years, increasing attention has been paid to and research from the range 0–2 Sv. Because of an apparent distinction between distal and
done on patient radiation exposure from diagnostic imaging. Much proximal zero-dose cancer rates, the unity baseline corresponds to zero-dose
of this concern stems from data derived from survivors of the atomic survivors within 3 km of the bombs. The horizontal dotted line represents the
bombs used in World War II. The radiation dose to each survivor alternative baseline if the distal survivors were not omitted.
was calculated based on each individual’s distance from ground zero, Reprinted with permission from Pierce DA, Preston DL. Radiation-related cancer risks at
low doses among atomic bomb survivors. Radiat Res. 2000;154(2):178–186.
and more than 50,000 of these patients were followed for more than
50 years. The data showed an increased cancer risk for many of
the survivors. Based on this observation, a mathematical model
was created which states that the risk of cancer proceeds in a linear CT scans performed. This figure is superimposed on the national
fashion without threshold (Figure 17.2). According to this model, cancer incidence of 42%.
even a small amount of radiation increases the risk of cancer. Current This research spurred the founding of the Image Gently
estimates vary concerning diagnostic radiation and the risk of can- Campaign, which stresses a multifaceted approach that is
cer. Based on research by multiple radiation biologists, the American intended to minimize radiation exposure to children. The cam-
College of Radiology (ACR) estimates that for adults, 1 fatal cancer paign has brought together 60 medical and professional orga-
is induced for every 1,000 CT scans performed. The risk is higher nizations representing more than 700,000 imaging specialists
in children, with 1 fatal cancer occurring for every 500 to 1,000 from the fields of pediatrics, radiology, physics, and radiology

BPPCA6e_Ch017_0109-0112.indd 110 2/13/20 5:18 PM

 CHAPTER 17: IMAGE GENTLY APPROACH TO PEDIATRIC IMAGING 111

technology. The as low as is reasonably achievable principle

(ALARA principle) is based on the notion that any amount of

Date
radiation exposure, no matter how small, can increase the chance
of negative biologic effects. The aim of the ALARA principle is to

Exam
balance the goals of minimizing the amount of radiation while
obtaining sufficient imaging quality, resulting in accurate diag-
nostic information. My Child’s
Medical Imaging Record

FOLD HERE
Where Exam Performed
The Role of the Radiologist
The pediatrician can often determine whether any imaging study
is needed based on the history and physical examination. In
some conditions (eg, acute trauma), imaging is important to
assess the nature and extent of the injury. In other conditions,
such as infectious processes (eg, cough, symptoms of sinusitis),
www.imagegently.org
medical management is warranted initially before any imaging
is performed.

Pediatrician: _______________________________________________

Name: _______________________________Date of Birth:__________

Date
Communication and coordination between medical special-
ists and radiologists is often helpful. When in doubt, a call to
the local radiologist, regardless whether that person is a dedi-

Exam
cated pediatric radiologist, can guide the specialist in determining
which test is reasonable for a specific patient. Radiologists have
many means by which to tailor examinations to minimize radi-
ation. The first step often involves asking whether nonionizing

Where Exam Performed

diagnostic examinations, such as ultrasonography or magnetic
resonance (MR) imaging, can be performed to answer the same
diagnostic question. In particular, ultrasonography often can be
used as a screening examination to evaluate conditions that can
be clarified with other studies that involve radiation if needed.
For certain indications, MR imaging is a great alternative to CT;
however, in some cases, the need for sedation and other risks of
MR imaging may outweigh the risks of CT. Radiologists are also
Figure 17.3. Child imaging history card on which parents can record their
required to monitor equipment and study protocols to ensure child’s exposure to imaging studies.
radiation dosage does not exceed the recommended range. All Borrowed with permission from the Image Gently® Alliance.
commercial CT scanners used in the United States include mul-
tiple methods to adjust the radiation dose. A low-dose technique
should be used whenever appropriate. Newer-generation CT scan-
ners use a significantly decreased dose while maintaining imaging CASE STUDY 1
quality. Breast and gonadal shielding can offer additional protec- Case Study 1: The Importance of the Patient History
tion to children. Back pain is a common symptom in active adolescent patients. Many studies have
evaluated the use of imaging in patients with atraumatic back pain. In the absence
of significant trauma, most studies have found that there is little value in imag-
Resources for Physicians and Parents ing otherwise healthy adolescents who present with musculoskeletal pain. If this
patient had experienced significant trauma or demonstrated focal neurologic find-
The ACR has established appropriateness criteria for imaging
ings suspicious for significant injury to the spinal cord or a disk, MR imaging would
of specific medical conditions and presentations; these criteria be the most appropriate examination. Radiographs of the spine are not helpful in
were coauthored by clinicians and radiologists. The criteria are assessing disk disease or central nervous system injury. Radiographs are helpful in
available free online and can be downloaded and incorporated adult patients to evaluate the extent of multilevel disk disease. For this patient, the
into computerized ordering and electronic health record systems physician should obtain a comprehensive history and perform a complete physi-
cal examination to help define the differential diagnosis and assist in the determi-
(https://www.acr.org/Clinical-Resources/ACR-Appropriateness-
nation of the appropriate imaging study. See Resources for Physicians at the end
Criteria). of the chapter for a link to the ACR Appropriateness Criteria for back pain in a child.
The Image Gently website (www.imagegently.org) offers educa-
tional materials for parents to help them understand the risks and Case Resolution
The physician should recommend rest, nonsteroidal anti-inflammatory drugs,
benefits of diagnostic imaging and the associated radiation. The web- and stretching exercises for 4 to 6 weeks. If the pain persists after that time,
site also has printable child imaging history cards for parental use magnetic resonance imaging of the lumbar spine without contrast would be a
to track all diagnostic radiation examinations performed on their reasonable next step in the workup of this patient.
child (Figure 17.3).

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 112 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Image Gently website

CASE STUDY 2 www.imagegently.org
Case Study 2: Symptom Duration and Response to Treatment
Imaging should be obtained in cases of chronic sinusitis that do not respond to
treatment and persist for months without resolution. Computed tomography of
Selected References
the sinuses is useful in the evaluation of the soft tissues, extent of disease, and Bulas D, Goske M, Applegate K, Wood B. Image Gently: improving health
any potential intracranial complications. Computed tomography also provides literacy for parents about CT scans for children. Pediatr Radiol. 2009;39(2):
important anatomic detail should surgery be required. Radiographs have limited 112–116 PMID: 19083213 https://doi.org/10.1007/s00247-008-1101-9
utility in evaluation of sinusitis because of the lack of soft tissue, anatomic detail,
and low sensitivity. This is particularly true in very young children whose sinuses Frush DP. Radiation safety. Pediatr Radiol. 2009;39(Suppl 3):385–390
are not yet well aerated. Imaging is not appropriate in the setting of acute sinus- PMID: 19440758 https://doi.org/10.1007/s00247-009-1215-8
itis in an otherwise well child. See Resources for Physicians at the end of the Goske MJ, Applegate KE, Boylan J, et al. The Image Gently campaign: work-
chapter for a link to the ACR Appropriateness Criteria for child sinusitis. ing together to change practice. AJR Am J Roentgenol. 2008;190(2):273–274
PMID: 18212208 https://doi.org/10.2214/AJR.07.3526
Case Resolution
The physician should order a computed tomography scan of the sinuses, because Lodwick DL, Cooper JN, Kelleher KJ, Brilli R, Minneci PC, Deans KJ. Variation
the patient has signs and symptoms of chronic sinusitis despite treatment. Had in utilization of computed tomography imaging at tertiary pediatric hospitals.
the patient been presenting for the first time with signs and symptoms of sinus- Pediatrics. 2015;136(5):e1212–e1219 PMID: 26504136 https://doi.org/10.1542/
itis, medical treatment would be indicated and imaging would be considered peds.2015-1671
were the patient not to respond to that medical management. Miglioretti DL, Johnson E, Williams A, et al. The use of computed tomography
in pediatrics and the associated radiation exposure and estimated cancer risk.
JAMA Pediatr. 2013;167(8):700–707 PMID: 23754213 https://doi.org/10.1001/
jamapediatrics.2013.311
Resources for Physicians
Pearce MS, Salotti JA, Little MP, et al. Radiation exposure from CT scans in
ACR Resources childhood and subsequent risk of leukaemia and brain tumours: a retrospec-
Appropriateness Criteria tive cohort study. Lancet. 2012;380(9840):499–505 PMID: 22681860 https://doi.
www.acr.org/Quality-Safety/Appropriateness-Criteria org/10.1016/S0140-6736(12)60815-0

Sinusitis
https://acsearch.acr.org/docs/69442/Narrative/
Back Pain
https://acsearch.acr.org/docs/3099011/Narrative/
Radiation Dose Assessment
www.acr.org/-/media/ACR/Files/Appropriateness-Criteria/
RadiationDoseAssessmentIntro.pdf

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 CHAPTER 18

Simulation in Pediatric
Health Care
Tom Kallay, MD

CASE STUDY
An 8-year-old boy presents to your office for a follow-up provides a higher level of care. Although emergency
appointment after an asthma exacerbation. He reports interventions were not required during this situation,
feeling better while at home this morning, but on the you and your staff feel that you could have been better
car ride to your office his chest started “hurting,” and prepared. You decide to take measures to optimize the
he began to experience shortness of breath. During his function of your staff and office environment for the rare
intake he appears to be in a moderate amount of dis- emergency.
tress, with evidence of tachypnea and tachycardia. His
pulse oximeter reports a value of 90%. Your staff admin- Questions
isters nebulized bronchodilator therapy and oxygen, and 1. What is simulation?
a call is made for paramedic transport to the nearby hos- 2. How does simulation apply to health care?
pital. Within the next few minutes the staff becomes 3. What modalities of simulation are available for
concerned that he may need emergent airway support, medical training?
but you are not sure if the equipment is functioning 4. How does one create and deliver simulation
properly since the last time it was used. The paramed- training?
ics arrive and safely transport the child to a facility that 5. Why should a primary care physician use simulation?

Simulation is a powerful tool for learning. In the most general sense, principles of resuscitation. The airway could be obstructed, necessi-
simulation is a learning technique in which people or technology are tating neck hyperextension followed by thrusting the chin forward
used to mimic real-life encounters. These encounters are practiced to insufflate the lungs. An internal spring placed in the chest of the
in simulated conditions, and typically the learner receives perfor- mannequin permitted the practice of cardiac compressions, and sub-
mance feedback as if the learner were in the real situation. sequently, cardiopulmonary resuscitation (CPR) training was born.
Simulation has a long legacy of use for education and evaluation Computer-controlled mannequins were developed in 1967 at
in many high-risk industries. Examples include flight simulators for the University of Southern California. Medical educator Stephen
pilots and astronauts, war games for military personnel, and tech- Abrahamson, PhD, along with anesthesiologist J. Samuel Denson,
nical operations for nuclear power personnel. Simulation provides MD, collaborated with a group of engineers from the Sierra
learners of all levels an opportunity to practice and develop knowl- Engineering and Aerojet General Corporation to create Sim One.
edge and skills without the threat of causing harm to individuals. This prototype device was a computerized, adult-size mannequin
Currently, high-risk industries use robust simulation programs that that featured breathing, heart sounds, functional pupils, and an
are embedded into the culture of training and skill maintenance. anatomically correct airway. It was used for airway management
Because health care is a high-risk industry, it seems intuitive that training for anesthesia residents from the late 1960s through the
simulation would have a place in medical training, with the purpose 1970s, with a noted benefit of training without placing patients at
of improving patient safety and outcomes. risk. Ultimately, however, Sim One did not gain acceptance and the
program was terminated. For the next 3 decades, simulation in
Simulation in Medicine medical education remained relatively dormant.
History
Simulation in medicine began in 1960 with Resusci Anne, a man- The Growth of Simulation in Medicine
nequin designed for mouth-to-mouth resuscitation training. This ...[N]o industry in which human lives depend on the skilled
was a new resuscitation technique conceived by Peter Safar, MD, performance of responsible operators has waited for unequiv-
and James Elam, MD, in 1957, which influenced Norwegian toy- ocal proof of the benefits of simulation before embracing it.
maker Asmund Laerdal to create the mannequin for practicing the —David M. Gaba, MD
113

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 114 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

The Institute of Medicine (IOM) report To Err Is Human: Building can simulate virtually any situation encountered in medicine are
a Safer Health System, published in the year 2000, drew attention available. With the development of not only the technology but the
to the perils of the health care system, highlighting the human and technique of simulation, it has become apparent that for health care,
financial costs of medical errors. The period following the IOM report as in other high-stakes industries, simulation has found its place.
witnessed a resurgence of simulation in medicine as a response to This chapter provides a broad overview of simulation in health
the reinvigorated emphasis on patient safety. Government and med- care—the associated terminology, available resources for health care
ical institutions began to embrace simulation as a means of improv- education, and techniques for providing this mode of training.
ing health practitioner and team performance to improve outcomes.
In 2001, the Agency for Healthcare Research and Quality (AHRQ) Terminology
published an analysis of patient safety practices and dedicated a The term simulator refers to the equipment, such as a simulated arm
chapter to the potential benefits of simulation. for venipuncture, a computerized mannequin that replicates human
With the publication of increasing evidence that simulation physiology, or a virtual reality computer with programming designed
could be applied to patient safety, it began to find its way into to practice laparoscopic surgery. Simulation is an educational
the language of accreditation and certification. The Accreditation technique described by the IOM in 2010:
Council for Graduate Medical Education has embraced simulation The act of imitating a situation or a process through some-
as an effective training method and now mandates that simulation thing analogous. Examples include using an actor to play a
resources be available for programs such as anesthesia and sur- patient, a computerized mannequin to imitate the behav-
gery, and more programs are following suit. In 2008, Accreditation ior of a patient, a computer program to imitate a case sce-
Council for Graduate Medical Education program requirements nario, and an animation to mimic the spread of an infectious
mandated having available simulation resources for 3 out of 159 res- disease in a population.
idency requirements; as of today, that number has grown 10-fold, Simulation-based medical training is a systematically designed
to 30. program that provides information, demonstration, and practice-
A body of pediatrics literature demonstrating the benefits of based learning activities that are supported by the concept of
simulation has been published. This knowledge has been used deliberate practice.
to improve skills ranging from complex resuscitations to lumbar A simulation center is an area designed to provide some or all of
puncture (LP) and has demonstrated applicability to inpatient and the aforementioned modes of simulation. It can range in size from
outpatient settings. a 60 m2 room to a 3,000 m2 building replicating a hospital with
For example, the literature on pediatric outpatient emergen- fully equipped patient rooms, clinics, and operating rooms. Such
cies shows that offices are frequently ill-equipped to manage centers provide opportunities to practice all facets of medicine,
an emergency. In 2000, the American Academy of Pediatrics depending on the learning goals. Spaces can be fashioned to appear
Committee on Pediatric Emergency Medicine published like an emergency department or clinic office and may be wired with
Childhood Emergencies in the Office, Hospital, and Community: cameras and microphones so that learner actions and statements can
Organizing Systems of Care. This resource highlights how prep- be recorded and reviewed for evaluation and feedback. Some centers
aration of the staff, office environment, and community are may use a control room adjacent to the simulation area, separated
crucial for delivering high-quality emergency care and advises by 1-way mirrors, that allows the facilitator to observe and control
that simulated mock scenarios or codes are an essential part of an the scenario without being in the room with the learner. If high-
office emergency preparedness plan. Currently, many resources fidelity mannequins are used, they are controlled from this room
provide the necessary tools for providing simulation education via computer, and facilitators communicate with role players in the
in the field of pediatrics. scenario by 2-way radio. There may be conference areas for viewing
Simulation is increasingly used in the health care field. In 2004, live simulations and debriefing, as well as storage areas for equip-
the Society for Simulation in Healthcare was established by profes- ment. Multidisciplinary centers provide the best opportunity for
sionals who use simulation for education, testing, and research in cross-training among health professionals and building camaraderie.
health care. Members now include physicians, nurses, allied health In situ simulation comprises simulation activities embedded in
and paramedical professionals, researchers, and educators from an actual clinical environment. The advantage of in situ simulation
around the globe, and the society hosts an annual meeting. Since is that medical scenarios can be practiced in the working environ-
2006, the AHRQ has been funding simulation research as part of ment, thereby providing the closest approximation of reality. Such
its safety mission. This research has expanded our knowledge on simulation is excellent for team training, because all members of
how to effectively use simulation in a variety of clinical settings. the medical staff can participate. Another advantage of in situ sim-
Additionally, the AHRQ in collaboration with Society for Simulation ulation is that it provides the ability to test the working environ-
in Healthcare created the Healthcare Simulation Dictionary to ment for conditions that can predispose a person to make an error.
provide uniform terminology and definitions for users of health For example, a prospective randomized controlled trial was
care simulation. In response to demand, the medical simulation performed to evaluate the effect of a simulation-based interven-
equipment industry has blossomed, and currently products that tion designed for emergencies in the pediatric office. Thirty-nine

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 Chapter 18: Simulation in Pediatric Health Care 115

practices were involved, with 20 in the intervention group and 19 such as breathing, heart sounds, and blood pressure. These manne-
serving as controls. The intervention involved 2 mock codes deliv- quins are technology dependent and often expensive and require
ered by the investigators in the office (in situ), where staff responded expertise in maintenance and operation. Mannequins are typically
using their own equipment and local emergency medical services. used for mock codes, which can be applied to almost any health
After the mock code, the investigators debriefed the staff, reviewed care setting, such as an office, inpatient ward, or operating room.
existing emergency equipment, and assisted with developing a Screen-based simulated programs are more affordable and logis-
resource manual designed for emergencies. A post-intervention tically easier than a mock code. Unlike a mock code, which often
survey was distributed 3 to 6 months later that included items on the requires the coordination of more than 1 person, screen-based sim-
following areas: purchase of new emergency equipment or medica- ulated programs can be performed by an individual at any loca-
tions; receipt or updating of basic life support, pediatric advanced life tion with a computer. Software programs are available that contain
support, and advanced life support certification by staff members; libraries of clinical scenarios in which patient history, examination
and development of written protocols for emergencies. The con- findings, image studies, and laboratory tests can be represented
trol group received no intervention and completed the same survey. graphically. Users can select diagnostic and therapeutic options as
Intervention practices were more likely than control practices they work through the case and generate a record of performance.
to develop written office protocols (60% vs 21%; P = 0.02), and Immediate feedback is provided by preprogrammed software or an
staff in intervention practices were more likely to be current on life instructor at a later time.
support certifications by the time of the post-intervention survey Task trainers are three-dimensional representations of body
(118 vs 54; P = 0.02). No significant differences existed between parts that allow the user to improve technique or develop psycho-
the 2 groups in terms of the purchase of new equipment or med- motor skill in many areas, such as intravenous line insertion, LP, or
ications. Satisfaction with the simulation exercise was evaluated bag-valve mask. Task trainers exist for nearly every procedure and
as well. At the time of the post-intervention survey, 90% of staff discipline, with options ranging from preterm neonates to adults.
felt the exercise was useful for their practice, and 95% felt that the Some trainers provide visual, auditory, or printed feedback to the
program should be continued. learner based on the quality of the performance. For example, when
Although in situ simulation is an effective tool, it does have practicing bag-valve mask on a baby head, an airway connected to
shortcomings. In a busy hospital or clinic environment, staff may inflatable lungs allows learners to visualize the effectiveness of their
feel overburdened by having to perform extra tasks during work technique. Task trainers are especially useful for practitioners to
hours that may be perceived as unnecessary. It is the responsibil- gain familiarity with the equipment being used, whether they are
ity of the person organizing a simulation practice session to ensure first learning to use the equipment or refreshing their skills after
that patient care is not compromised during the activity; it is also a period of nonuse.
important to build a culture of patient safety in which staff feel a Virtual reality and haptic systems use the most sophisticated
responsibility to provide excellent individual and team function in computer programming for procedural practice. Virtual reality
a well-prepared environment. refers to the re-creation of environments or objects as a complex,
computer-generated image. Haptic systems provide the capability
Medical Simulation Resources of tactile learning, and these programs can provide detailed feed-
Resources available for simulation in health care can be categorized back on procedural skill based on the kinetic actions of the user.
into 1 of 5 areas: mannequin based, screen based, virtual reality, task Typically, virtual reality and haptic systems are used with a task
trainers, and standardized patients (SPs) (Box 18.1). How these tools trainer; most of the products available are for vascular access,
are used depends on the educational needs. surgical procedures, bronchoscopy, or endoscopy. Evidence shows
Mannequins are lifelike representations of human beings and that virtual reality is superior to traditional training; it is likely that
range in size from a preterm neonate to a full-size adult. The spec- simulation-based medical education will further incorporate virtual
trum of functionality spans from a simple form, such as Resusci reality in the future. Currently, virtual reality programs are relatively
Anne, to a high-fidelity mannequin. A high-fidelity mannequin is cost-prohibitive and limited in scope; however, this may change as
computer driven and has features that represent human physiology, programming and options continue to develop.
Standardized patients have been used in graduate medical
education for years. An SP is an actor playing a role and provides
Box 18.1. Resources for Medical trainees an opportunity to practice communication, physical exam-
Simulation Training ination skills, or history taking. Although SPs typically play the role
ww Mannequin: simple or computer driven of a patient, they can also play the role of a family member or a
ww Screen-based simulation programs fellow health practitioner.
ww Virtual reality programs Hybrid simulation, which combines 2 modes of simulation for
ww Task trainers a more realistic experience, is an excellent opportunity to provide
ww Standardized patients a multifaceted learning experience. For example, when creating a
scenario for a child with diabetic ketoacidosis (DKA), a mannequin

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 116 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

and an SP can incorporate scripts written for DKA as well as for Steps for Delivering Simulation
a mother who must be told that her child has a new diagnosis of Learning
diabetes mellitus. Learners have the opportunity to both apply their
Developing simulation activities requires multiple steps and careful
knowledge in assessing and treating DKA and use their skills in hav-
planning to achieve learning goals (Box 18.2). Health profession-
ing difficult conversations. Afterward, feedback is provided to the
als are often busy and have little spare time, so in addition to mak-
learners about their medical knowledge, decision-making skills, and
ing simulations accessible, the educational product must be sound
communication skills. The term confederate is sometimes applied
and worth the time spent. Learning events that are not well planned
to role players who work together with the instructor in scenarios.
or delivered can have an adverse effect on an individual’s percep-
tion of simulation education, which can have a negative effect on
Technique that person’s learning. The first step in delivering simulation learn-
The Culture of Simulation ing is the creation of a mock code or scenario, followed by deliber-
ate practice, a concept of instruction that is applicable to scenario
This section describes the elements that are needed when designing
or procedural training.
and delivering education using simulation. But before these steps are
illustrated, it is important to discuss the culture of simulation learn- Mock Scenario Development
ing and how it fits with the current culture of medicine. The first step in creating a scenario is to identify what needs to be
Traditional medical education emphasizes the importance of improved; this may be related to medical knowledge, technical
error-free practice, with intense pressure to achieve perfection dur- skill, communication, team function, or a combination of these.
ing diagnosis and treatment. Furthermore, when mistakes are made, Adverse as well as routine events in the office or hospital setting often
the psychological toll for the practitioner cannot be underestimated. provide opportunities for learning and improvement and can set the
It is often said that there are 2 victims in the case of a medical error: educational framework.
the patient and the health professional. Reports of provider depres- The case study provided in the beginning of the chapter, for exam-
sion, substance abuse, and suicide highlight the need for engen- ple, highlights a situation in which a mock code could be helpful in
dering a productive rather than destructive response to medical improving performance. Practicing a mock scenario of anaphylaxis
errors, which is 1 of the central aims of simulation. The technique of can address multiple needs: teamwork, communication, locating
practicing technical skills or decision making skills in an environ- and operating equipment, performing proper advanced life support
ment absent of the “shame and blame” response can assist in devel- techniques, and disposition. After a scenario is developed, it can be
oping healthy reactions to mistakes, in which the factors leading to practiced by the office staff until performance standards are met.
the error are objectively reviewed and analyzed and improvements When writing a mock code or scenario, the first task is to create
instituted. When a mistake is made, the goal is to change the think- succinct learning objectives. Writing clear objectives is an underap-
ing from what the repercussions will be to how health profession- preciated skill and is a crucial part of any educational program. Good
als can better themselves to improve care for their patients. As the objectives are important because they focus teaching and enable
practice of medicine is an art, so is the practice of becoming a bet- the evaluation of the effectiveness of the activity. A helpful way to
ter health professional. start thinking about objectives is to begin with the phrase, “By the
The learning environment for simulation education activities end of this session, the learner will be able to...” followed by the
must connote a safe learning environment in which mistakes can be
made without reproach. In fact, simulation is an area in which it is
desirable for health professionals to make mistakes so that the prac- Box 18.2. Steps for Providing Simulation-based
tice of improvement can occur. It is the responsibility of the facilita- Medical Education
tors to explicitly state this to help create an atmosphere conducive 1. Determine learning needs.
to constructive discussion. 2. Create learning objectives.
Simulation activities also provide an opportunity for health 3. Create learning lesson.
professionals to train together and build effective communication a. Script, if performing mock scenario.
skills and team camaraderie. The training of health professionals— b. Simulated patient history and physical examination, laboratory data,
images, and case progression.
nurses, physicians, medical or physician assistants—generally
c. Consequences of anticipated interventions and disposition.
occurs in parallel, which can have the unintended consequence of d. Identification of required equipment and space.
poor communication between team members. This potentially can e. Identification of role-players.
create an environment that may not always be conducive to fair and 4. Session delivery.
open discussion of mistakes, which is necessary if optimal learning a. Create a safe learning environment.
and improvement are to occur. When delivered with creativity and b. Deliberate practice.
5. Feedback/debriefing.
enthusiasm in a nonthreatening environment, simulation can pro- a. Advocacy inquiry.
vide a venue for building effective relationships between practitio- b. Plan for improvement.
ners while imparting educational benefits.

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learning objective. Objectives should be specific and measurable them and helps them understand the benefits and limitations of the
and use words that are open to few interpretations. For example, simulated clinical setting. Learners must understand whether and
the objective, “By the end of the session, the learner will under- how the case, event, or procedure will later be debriefed (ie, dis-
stand the complications of bag-mask ventilation.”, is open to many cussed and analyzed) and whether the simulation will be video
interpretations, whereas the statement, “By the end of the session, recorded. The instructor must explicitly state that the focus is on
the learner will be able to list the complications of bag-mask venti- learning, not on catching people in a mistake. This helps create an
lation.”, is open to fewer interpretations. environment in which participants feel safe in sharing thoughts and
The learning objective in the first example is vague; it may be feelings about the upcoming simulation and debriefing without fear
answered in multiple ways. Additionally, the instructor does not of being shamed or humiliated.
have a clear standard for how to measure what has been learned. Participants often worry that simulations are designed to expose
The second objective not only focuses the teacher and learner but their weaknesses or humiliate them. To counter these thoughts, facil-
provides a concrete way to measure knowledge by requesting a finite itators should convey the understanding that learners have good
list. Depending on educational needs, typically 2 to 4 objectives are intentions and are trying to do their best but will likely make mis-
sufficient for a session. The use of too many objectives can be over- takes. Learners should be reassured that mistakes are desirable in the
whelming and can have a dilutional effect on the knowledge being simulation environment because they provide focus on areas in need
imparted. Box 18.3 provides examples of words that are open to of improvement, which in turn can result in improved patient care.
fewer or more interpretations. After the scenario is complete, the next step is to debrief the case.
After the learning objectives are established, a script is written This is the time to review the stated objectives; however, the overall
that describes the flow of events and consequences of anticipated goal of debriefing is to allow trainees to explain, analyze, and syn-
interventions. A list of required equipment or props, role-players thesize information and emotional states to improve performance
needed with instructions on how to act, a history and physical exam- in similar situations in the future. This can be challenging, because
ination of the simulated patient, and laboratory tests or images are voicing critical thoughts can result in hurt feelings or defensiveness
also included. It is helpful to write the scenario such that an individ- on the part of the learner. It is the responsibility of the instructor
ual other than the writer can use it for future sessions. Eventually, if to lead a discussion that encourages objective reflection of practice
multiple scenarios are written, a catalog of cases with various clinical without being confrontational.
scenarios can be developed that can serve as a sustainable learning One general framework of inquiry commonly used is: “What
resource. Many templates are available that can be helpful in writing went well? What did not go well? What could we do to improve
a scenario, with varying degrees of complexity. The important thing next time?” This gives participants the opportunity to voice their
is for instructors to use the method that fits their time and needs. opinions and concerns. The techniques helpful for good debrief-
After preparation is complete, the session can be delivered. ings are designed to increase the chances that the learner will pro-
How well the instructor introduces the simulation learning expe- cess what the instructor is saying rather than become defensive.
rience can set the tone for all that follows. Before any simulation Advocacy-inquiry is 1 technique that can be used to help guide
begins, the facilitator helps participants clarify what is expected of debriefing sessions.
Advocacy-inquiry is founded in the theory of reflective practice,
which is a means of analyzing one’s own work practices and exam-
Box 18.3. Words or Phrases Open
ining the foundation of their existence. When incorrect actions are
to More or Fewer Interpretations
performed, the goal of debriefing is to elicit the framework of the
Open to More Interpretation participants’ thought processes, which have been formed by their
ww Know knowledge and experience. There may have been a previous situation
ww Understand in which an instructor provided incorrect information or in which
ww Be able miscommunication occurred that may have had a significant forma-
ww Know how tive effect on the learner’s understanding and subsequent behavior.
ww Appreciate An advocacy is an observation, whereas an inquiry is a ques-
ww Grasp the significance of tion. When the 2 are paired together, instructors state in the advo-
ww Learn cacy their hypothesis and test the hypothesis with an inquiry in
Open to Fewer Interpretations a nonthreatening manner. For example, during a mock code drill
ww List for a patient with asystole, the instructor notices that cardiac com-
ww Recite pressions were not started immediately. During the debriefing, the
ww Demonstrate instructor might say to the learner, “So, I noticed that you were work-
ww Perform ing on providing oxygen to the patient after you noticed the lack of
ww Rank (ie, rank as important) a heart rate” (ie, advocacy). “I was thinking there was possibly an
ww Describe additional maneuver to be done. I’m curious—how were you see-
ing the situation at that time?” (ie, inquiry). Rather than making

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 118 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

a judgmental comment, such as, “Do you realize that it took you participants viewed an educational audiovisual presentation. The
2 minutes before you started chest compressions?”, the instructor is intervention group went on to participate in a simulation-based
using advocacy-inquiry to elicit the learner’s framework of think- deliberate practice session on an infant LP simulator. The primary
ing that guided the learner’s actions. The participant might state, outcome was self-reported clinical success on the first infant LP after
“I was once admonished during my training for not doing things training. Fifty-one residents reported on 32 LP encounters; 94% of
in the right sequence.” Having elicited this description of a prior the intervention group reported success, compared with 47% in the
training experience, the instructor has an opportunity to refine the control group, a marked difference. When the residents were evalu-
thought process of the learner. ated on an observed clinical examination at 6 months, however, no
Just as the delivery of simulated scenarios requires practice, so difference was found between the groups. These findings suggest
does the facilitation of a productive debriefing process. In 2007, that although simulation training with deliberate practice is effec-
Rudolph et al published a good article on learning the technique tive, skills decline over time and continual practice and retraining
of debriefing. are necessary.
Although simulated clinical scenarios and mock codes are com- In adult medicine, a randomized trial with wait-list controls eval-
plex processes, the task of teaching procedures is generally more uated the acquisition of advanced cardiovascular life support skills
straightforward. An excellent framework for teaching procedures among internal medicine residents using a mannequin simulator.
in a simulated environment involves using the concept of deliber- Residents who received the simulation-based medical education
ate practice. with deliberate practice scored 38% higher on a reliable skills eval-
uation than residents in the wait-list control group. After crossover
Deliberate Practice and a deliberate practice session with the control group, the scores
Education research has demonstrated that acquisition of expertise surpassed the performance outcomes of the first intervention group.
in medicine or other fields (eg, professional sports, musical perfor- The authors concluded that deliberate practice, in addition to time
mance, chess) is based on a set of principles governed by the concept and experience, is helpful in achieving competence.
of deliberate practice, which is among the cornerstones of simulation- The concept of close observation by experts with provision of
based medical education. To summarize, deliberate practice dic- constant real-time feedback can be applied to almost any procedure.
tates that the learner will engage in repetitive performance of However, deliberate practice is only 1 tool in achieving procedural
cognitive or psychomotor skills with direct assessment, which provides excellence; continual practice is a key component to maintaining a
the learner with feedback tailored to improve performance. The high level of performance.
elements of deliberate practice are listed in Box 18.4.
Coupled with simulation, deliberate practice has been shown Benefits of Simulation
to improve performance in many medical and surgical specialties.
Despite all the points demonstrating that simulation is a valid teach-
Studies ranging from general pediatrics to vascular surgery have
ing tool for medical training, concerns exist about its role. The time
demonstrated improvements in skills, knowledge, and team func-
taken away from real patient experience, significant effort and time
tion when simulation with deliberate practice is used.
required to create and deliver simulation training, cost of equip-
A randomized, controlled trial evaluated the effectiveness of
ment, and potential for humiliation are all points that cause trepi-
deliberate practice simulation-based training compared with audio-
dation. Furthermore, the question remains whether it is effective.
visual training only for improving infant LP skills among pediatric
Simulation is an evolving science, and a growing body of evi-
residents. After a baseline assessment of LP skill and knowledge, all
dence indicates that it can be effective in improving medical knowl-
edge and skills, team function and communication, and patient
safety and outcomes and that its use can result in hospital cost sav-
Box 18.4. Features of Deliberate Practice ings in terms of return on investment. Health care payers and lia-
1. Highly motivated learners with good concentration bility insurers have noticed simulation as the potential effects on
2. Engagement with a well-defined learning objective or task patient safety are now being realized. For the development of the
3. Appropriate level of difficulty health professional, simulation does not and should not replace
4. Focused, repetitive practice the benefits of real patient experience; however, it can serve as an
5. Rigorous, precise measurements
6. Informative feedback from educational sources (eg, simulators, instructors)
important component in the professional’s education. After an
7. Monitoring, correction of errors, and more deliberate practice individual grasps the technique, simulation education can be pro-
8. Evaluation to reach a mastery standard vided without the use of elaborate and expensive simulators but
9. Advancement to another task or unit instead (with some creativity) with the use of lower-cost materi-
als. The future of simulation in medicine depends on the dedica-
Adapted with permission from McGaghie WC, Siddall VJ, Mazmanian PE, Myers J; American College
tion and ingenuity of the health care simulation community to see
of Chest Physicians Health and Science Policy Committee. Lessons for continuing medical educa-
tion from simulation research in undergraduate and graduate medical education: effectiveness of
that improved patient safety and educational outcomes can be real-
continuing medical education. American College of Chest Physicians Evidence-Based Educational ized using this method. Potential benefits and applications of sim-
Guidelines. Chest. 2009;135(suppl 3):62S–68S. ulation are outlined in Box 18.5.

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 Chapter 18: Simulation in Pediatric Health Care 119

Box 18.5. Benefits and Applications of Simulation Resources for Physicians

Healthcare Simulation Dictionary
ww Avoidance of risks to patients and learners
www.ahrq.gov/professionals/quality-patient-safety/patient-safety-
ww Tailoring of procedural training or scenarios to educational need and
resources/research/simulation-dictionary/index.html
attributes of the learner
ww Practicing of skills and scenarios as many times as necessary Society for Simulation in Healthcare
ww Enhancing transfer of skills from the classroom to the clinical arena www.ssih.org
ww Determination of educational needs
ww Training for optimal team or organizational function Selected References
ww Practicing communication skills
American Academy of Pediatrics Committee on Pediatric Emergency Medicine;
ww Providing opportunity to practice rare critical events
Seidel JS, Knapp JF, eds. Childhood Emergencies in the Office, Hospital, and
ww Assisting in the testing of new equipment or facilities Community: Organizing Systems of Care. Elk Grove Village, IL: American
ww Refresher training for staff at all levels Academy of Pediatrics; 2000
ww Serving as formative or summative assessment tool at all levels Biese KJ, Moro-Sutherland D, Furberg RD, et al. Using screen-based sim-
ulation to improve performance during pediatric resuscitation. Acad
Emerg Med. 2009;16(suppl 2):S71–S75 PMID: 20053216 https://doi.
Conclusion org/10.1111/j.1553-2712.2009.00590.x
Health care simulation is a technique to augment real experiences Bordley WC, Travers D, Scanlon P, Frush K, Hohenhaus S. Office preparedness
with guided, interactive encounters that approximate reality. The for pediatric emergencies: a randomized, controlled trial of an office-based
past 25 years have witnessed tremendous growth in the health care training program. Pediatrics. 2003;112(2):291–295 PMID: 12897276 https://
doi.org/10.1542/peds.112.2.291
training industry, with applications in medical education and patient
safety practices. Simulation provides a training method that does Cohen ER, Feinglass J, Barsuk JH, et al. Cost savings from reduced catheter-
related bloodstream infection after simulation-based education for residents in
not place patients at risk and that can be tailored to meet the needs
a medical intensive care unit. Simul Healthc. 2010;5(2):98–102 PMID: 20389233
of learners at any level. When delivering simulation education, it https://doi.org/10.1097/SIH.0b013e3181bc8304
is important to highlight to participants the goal of improvement
Kessler DO, Auerbach M, Pusic M, Tunik MG, Foltin JC. A randomized trial
and acceptance of errors to connote a safe learning environment. of simulation-based deliberate practice for infant lumbar puncture skills.
Additional work is required to continue to advance this technique Simul Healthc. 2011;6(4):197–203 PMID: 21527870 https://doi.org/10.1097/
to assist in improving the health care system for patients. SIH.0b013e318216bfc1
Lammers R, Byrwa M, Fales W. Root causes of errors in a simulated prehospi-
tal pediatric emergency. Acad Emerg Med. 2012;19(1):37–47 PMID: 22251191
CASE RESOLUTION https://doi.org/10.1111/j.1553-2712.2011.01252.x

You and your staff review what occurred in the case of the child who experi- McGaghie WC, Siddall VJ, Mazmanian PE, Myers J; American College of Chest
enced a severe asthma exacerbation and identify the educational and environ- Physicians Health and Science Policy Committee. Lessons for continuing medi-
mental needs of your practice. You determine that your educational objectives cal education from simulation research in undergraduate and graduate medical
are related to accessing the emergency equipment, having well-defined staff education: effectiveness of continuing medical education. American College of
roles when advanced life support measures are required, and providing effec- Chest Physicians Evidence-Based Educational Guidelines. Chest. 2009;135(suppl 3):
tive bag-valve mask ventilation. You obtain an inexpensive mannequin to serve 62S–68S PMID: 19265078 https://doi.org/10.1378/chest.08-2521
as a model for the child and write the script of the case. You discuss with your Rudolph JW, Simon R, Rivard P, Dufresne RL, Raemer DB. Debriefing
office staff what your plans are and that the goal is to improve office function with good judgment: combining rigorous feedback with genuine inquiry.
in emergent situations. You schedule an appropriate time and deliver the mock Anesthesiol Clin. 2007;25(2):361–376 PMID: 17574196 https://doi.org/10.1016/j.
scenario with the entire office staff. It is discovered that some of the equipment anclin.2007.03.007
is not operational, and some staff members voice a lack of confidence in their
bag-valve mask skills, which they have not had to use for some time. A practice Toback SL, Fiedor M, Kilpela B, Reis EC. Impact of a pediatric primary care
session is held in which staff members can practice the skill of bag-valve mask office-based mock code program on physician and staff confidence to perform
ventilation on the mannequin and receive constructive feedback on technique. life-saving skills. Pediatr Emerg Care. 2006;22(6):415–422 PMID: 16801842
Afterward, you lead a discussion with the group, review the points that require https://doi.org/10.1097/01.pec.0000221342.11626.12
improvement, and develop a plan to rectify the equipment issues, knowledge Wayne DB, Butter J, Siddall VJ, et al. Mastery learning of advanced cardiac life
gaps, and skills gaps. The mock code intervention was universally well received support skills by internal medicine residents using simulation technology and
by the staff, and confidence in the ability to manage emergent situations in the deliberate practice. J Gen Intern Med. 2006;21(3):251–256 PMID: 16637824
office setting was improved. https://doi.org/10.1111/j.1525-1497.2006.00341.x

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 CHAPTER 19

Pediatric Hospital Medicine

Melanie Rudnick, MD, FAAP, and Grant P. Christman, MD, FAAP

CASE STUDY
A 15-month-old girl presents to a community hospital inpatient pediatric service and arranges for ground basic
emergency department with fever, cough, and rhinor- life support transport.
rhea. On initial evaluation, she is found to be in mod-
erate respiratory distress, with decreased air movement
Questions
1. What is the role of hospitalists in inpatient pediat-
and scattered bilateral wheezes and crackles on lung
ric care?
examination. Her oxygen saturation is 87% on room
2. How can hospitalists implement principles of
air and rises to 96% with the application of 1 L/min
family-centered care and evidence-based medicine
of supplemental oxygen via nasal cannula. The physi-
into the clinical care of hospitalized children?
cian diagnoses the patient with bronchiolitis and treats
3. How can hospitalists promote quality improvement
with nebulized albuterol and an oral dose of predni-
and patient safety in the hospital setting?
sone. The hospital has no inpatient pediatric service, so
4. What communication strategies can hospitalists use to
the emergency physician calls the local children’s hospi-
ensure safe transitions of care within the hospital and
tal to arrange a transfer. The emergency physician signs
back to the outpatient medical home after discharge?
the patient out to a hospitalist, who accepts her onto the

Introduction While hospitalists are unable to provide the uninterrupted con-

tinuity of care that PCPs provide in the traditional model, hospi-
Every primary care physician (PCP) must periodically make the
talist systems offer a different advantage: the extended physical
decision to hospitalize a patient who can no longer be cared for
presence of a physician in the hospital who is responsible for the
effectively in the outpatient setting. In 2015, 2.1% of all children and
patient’s care—as much as 24 hours a day, 7 days a week in some
teenagers younger than 18 years, or more than 1.5 million patients,
systems—which is impossible to sustain for a PCP with a busy out-
required hospitalization.
patient practice.
Common reasons for hospitalization include need for close mon-
itoring due to existing or expected clinical compromise, initiation of
an intensive diagnostic workup, expected need for surgical proce- Inpatient Pediatric Care
dures, and administration of treatments that are complex and tech- Every patient presenting for admission to the hospital requires
nology intensive or have a high adverse effect profile. a thorough initial evaluation (history and physical examination)
In the traditional model of providing hospital care, the PCP following a direct verbal handoff from the health professional
admits the patient to the hospital and continues to personally direct requesting the patient be admitted. If admission is requested by
the patient’s care, examining the patient while on rounds in the hos- someone other than the PCP (eg, emergency physician, surgeon
pital daily; writing all orders for diagnostic studies, medications, and following a surgical procedure), the hospitalist should consider
other care; and consulting directly with subspecialists as needed. contacting the PCP to establish a relationship, gather more infor-
Due to economic and structural pressures from a changing health mation about the presenting symptom, and learn more about the
care system, a second model has emerged in which, once the PCP patient’s established medical problems and routine care. Primary
admits the patient to the hospital, further care is coordinated by a care physicians may not always be available, and, as such, it is
pediatrician specializing in inpatient medicine: a hospitalist. helpful if patients have been thoroughly educated on their med-
The term hospitalist was first coined by Wachter and Goldman ical conditions and carry a list of their medications and allergies
in 1996. Although hospitalists existed prior to that time, the preva- with them.
lence of hospitalists has increased significantly in the decades since. Inpatient pediatric care should be patient and family centered,
The Society of Hospital Medicine defines the scope of the hospi- which has been defined by the American Academy of Pediatrics
talist’s work to include the clinical care of acutely ill, hospitalized as “health care that is grounded in a mutually beneficial partner-
patients; education, research, and leadership in the field of hospi- ship among patients, families, and providers that recognizes the
tal medicine; and working to enhance the performance of hospitals importance of the family in the patient’s life.” In the hospital set-
and health care systems. ting, this may take the form of multidisciplinary family-centered
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rounds, a system in which doctors, nurses, and other allied health may warrant deviating from aspects of care recommended by the
professionals make rounds together on hospitalized patients with guidelines. Many of the guidelines used by hospitalists include
their families at the bedside. During rounds, health professionals discharge criteria, which are often dependent on availability of
educate patients and families, hear their preferences, and include outpatient follow-up. This is yet another example of importance
them in the medical decision making process. Some families may of communication with PCPs throughout patient hospitalization.
prefer not to participate in rounds, and this preference should be In addition to providing routine inpatient care, some hospi-
respected as well. If there is a language barrier, in-person, video, talists also provide specialized care, such as procedural sedation,
or telephonic interpreters should be used to facilitate making palliative care consultations, and surgical or medical coman-
rounds, in accordance with the principle of equitability of care. A agement. Hospitalists often play a role in accepting patients
child life specialist may be consulted to help the patient cope with for admission to the inpatient service, facilitating hospital-to-
the hospitalization, including daily rounds and anxiety-provoking hospital transfers, and providing physician-assisted transport.
or painful procedures, by clarifying what is discussed in terms They may also be tasked with determining the appropriate mode
the child can understand and helping the child process or be dis- and level of transport.
tracted from these events through various types of creative play. Due to medical and technological advances leading to
Patient- and family-centered care is complemented by the appli- improved survival rates, hospitalists are increasingly caring for
cation of evidence-based medicine principles to medical decision- the growing population of children with complex medical needs.
making. Questions of diagnosis, prognosis, treatment, or avoidance Providing quality care to these children is challenging due to their
of harm should be clearly defined to facilitate an effective review dependence on medical technologies and multiple medications,
of the medical literature. One way of developing a focused clinical the paucity of applicable clinical evidence to support manage-
question is through the PICO process, wherein the physician iden- ment strategies, and the need for time-intensive and complex care
tifies the applicable patient population, the intervention (ie, treat- coordination. Depending on the resources available to the family
ment, diagnostic test, or prognostic factor), the comparison (ie, and the PCP’s familiarity with the patient (which may be limited
control or placebo group), and the outcome (ie, diagnosis, prog- if the patient has had frequent or prolonged hospital stays), tran-
nosis, or harm to the patient). For example, in formulating a diag- sitioning the patient to the outpatient medical home may be par-
nostic workup for a 5-year-old patient presenting with an asthma ticularly challenging. Specific efforts should be made to ensure
exacerbation, the physician might ask whether school-age chil- a quality handoff so that there are no lapses in care and the PCP
dren with asthma (P) who undergo chest radiography (I) have a fully understands any adjustments made to the care plan during
shorter length of hospital stay (O) than children who undergo no hospitalization.
imaging (C).
Advances in technology have made it possible to consult med-
ical evidence at the point of care—even in the patient’s room dur- Patient Safety and Quality
ing bedside rounds—with the use of laptops, tablets, and in-room Improvement
computers. When searching through primary research studies, such While it is important for individual physicians to engage in clin-
as randomized controlled trials, to answer a clinical question, the ical practice that is family centered and evidence based, the next
physician must determine whether the results are valid and gener- step is to ensure that quality care is being systematically delivered
alizable to the patient under consideration. It may be more efficient at the hospital level, and where it is not, that quality improvement
and just as legitimate to use a summary of the evidence, such as a (QI) programs are initiated. In its 2001 report, Crossing the Quality
systematic review, which may be available through an established Chasm, the Institute of Medicine defined quality health care as
medical journal or a web-based service. Having the PCP as an ally having 6 characteristics: safe, effective, efficient, patient centered,
during the hospital stay may give the hospitalist more insight into timely, and equitable. Opportunities to improve the quality of
how applicable a certain study is to the patient for whom the hos- care delivered by the hospital may be suggested by issues faced in
pitalist is caring. individual patient encounters, quality metrics tracked by hospital
Hospitalists should also make use of clinical practice guide- administration (eg, length of stay, readmissions), or feedback from
lines as a way of providing standardized and evidence-based care. patients, their families, and their PCPs.
The Health and Medicine Division of the National Academies One model for the creation of QI projects is the Plan-Do-Study-
(formerly known as the Institute of Medicine) defines clinical Act cycle. In the Plan phase, the hospitalist identifies a prob-
practice guidelines as “statements that include recommendations lem or aim, defines a change that could be made to address the
intended to optimize patient care that are informed by a systematic problem, and determines how the effectiveness of the change
review of evidence and an assessment of the benefits and harms of will be measured. It is usually necessary to form a team of like-
alternative care options.” In applying clinical practice guidelines, minded physicians, administrators, nurses, and other allied health
hospitalists should consider factors specific to the individual patient, professionals early in the planning process. In the Do phase, the
such as comorbid conditions, limited availability of services in change is implemented, and data are collected about its effec-
specific hospital settings, and patient values and preferences, that tiveness. These data may include process measures (ie, how

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 Chapter 19: Pediatric Hospital Medicine 123

consistently the change is implemented) and outcome measures

Box 19.1. I-PASS Mnemonic for Verbal Handoffs
(ie, the effect of the change on the patient population, hospital
system, costs). In the Study phase, data are analyzed, and suc- ww Illness severity (ie, stable or unstable)
cesses and failures of the program are identified. Finally, in the ww Patient summary (ie, summary statement, events leading up to
Act phase, the team determines whether the change should be admission, hospital course, ongoing assessment, plan)
continued and adopted more widely in the hospital, modified ww Action list (ie, to-do list, timeline, ownership)
in some way, or discarded altogether. Depending on the project, ww Situation awareness and contingency planning (ie, know what’s going
additional Plan-Do-Study-Act cycles may be needed to maximize on and plan for what might happen)
the breadth and depth of the effect. Successes should be shared ww Synthesis by receiver (ie, receiver summarizes what was heard, asks
publicly with the hospital community to promote support for questions, and restates key action items)
further QI efforts.
Physicians should also promote a culture of safety in the hospital,
meaning that the hospital’s processes and workforce are dedicated scheduling of follow-up appointments, and provision of other
to the promotion of patient safety. Adverse events, a term encom- services, such as in-home nursing. The use of written or computer-
passing all injuries caused by medical management, are common ized discharge checklists may help ensure that no element is delayed
and are often caused by preventable medical errors, especially med- long enough to prolong the hospital stay or forgotten altogether.
ication errors (eg, incorrect dosing, administering medications to The final result of the discharge process is the transition of
the wrong patient). Common initiatives to improve patient safety care from the hospitalist back to the PCP and medical home. If
include use of electronic health records and computerized order communication is poor, this transition carries with it the poten-
entry, review of orders by multiple health professionals, promotion tial for significant information loss (eg, the hospital course, new
of sterile technique and handwashing, work-hour limitations to pre- diagnoses, future outpatient care needs such as subspecialty refer-
vent fatigue, and having time-outs before procedures to verify the rals and laboratory testing) and resulting adverse events for the
patient’s identity and the site of the procedure. Hospitals should patient. Ideally, the hospitalist should strive to communicate peri-
hold regular morbidity and mortality conferences to review cases in odically with the PCP throughout the hospitalization, especially
which adverse events occur. These conferences should be confiden- at the time of any important events. Most essential is communi-
tial and focus not on blaming individual health professionals but on cation at the time of discharge, which may take the form of verbal
identifying systemic failures that may be corrected to prevent future updates by telephone, emailed updates, and faxing or emailing of
adverse events. When adverse events occur, patients and their fami- discharge summaries.
lies should be informed in a timely fashion, according to procedures
established by the hospital. Education
A large portion of pediatric residency training occurs on inpatient
Transitions of Care units, and hospitalists are at the front line of pediatric resident edu-
cation. When leading traditional resident-covered inpatient teams,
Inpatient systems in which hospitalists work in shifts typically have
hospitalists may contribute added educational value through direct
physician-to-physician handoffs of patient care responsibility at least
observation of trainee history taking and physical examination skills,
once every 24 hours. Resident work hours in teaching hospitals have
providing frequent and effective feedback, and promoting trainee
been limited to promote patient safety, but this also results in more
autonomy on family-centered rounds. Due to resident duty hour
frequent patient handoffs. If there is inadequate communication,
restrictions, hospitalists are increasingly providing direct patient
handoffs may increase risk for medical errors and adverse events.
care on services without residents or other trainees, and many hos-
Safe handoffs may be promoted by written handoff notes and strat-
pitalists practice in community hospitals without training programs.
egies for effective verbal handoffs. One such strategy uses the I-PASS
In these cases, there may be opportunities to educate non-pediatrician
mnemonic, with the letters standing for a 5-step process in verbal
physicians (eg, surgeons, emergency physicians), nurse practitio-
handoffs (Box 19.1).
ners, and physician assistants in topics related to pediatric care.
The discharge process should begin as soon as the patient is
hospitalized. Goals of the hospitalization, discharge criteria, poten-
tial barriers to discharge, and an anticipated date of discharge Conclusion
should be identified in concert with the patient and family and The use of hospitalists to provide inpatient pediatric care has been on
reviewed during daily rounds. The hospitalist should formulate the the rise over the past 2 decades since the term was first introduced.
discharge plan together with the other health care and allied health Studies of hospitalist systems suggest that the quality of care pro-
professionals involved in the care of the patient, including subspe- vided by hospitalists is equivalent to, and in some ways may improve
cialists, nurses, social workers, and discharge planners. For patients on, care delivered via the traditional model. When attention is paid
with complex needs, issues to address may include procurement of to maintaining communication, hospitalists have the potential to
durable medical equipment, insurance coverage for medications, be partners in care with PCPs and the outpatient medical home.

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 124 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Federal Interagency Forum on Child and Family Statistics. America’s Children:

CASE RESOLUTION Key National Indicators of Well-Being. Washington, DC: US Government Printing
Office; 2017
The patient is admitted to a monitored bed, and, after the initial assessment,
the hospitalist concurs with the diagnosis of bronchiolitis. Based on current Guyatt G, Rennie D, Meade M, Cook D, eds. Users’ Guide to the Medical Literature:
clinical practice guidelines, the hospitalist discontinues the use of systemic A Manual for Evidence-Based Clinical Practice. 2nd ed. New York, NY: McGraw
steroids and bronchodilators. The hospitalist obtains the number of the PCP Hill; 2008
from the family and places a call on the day of admission; during the call, Institute of Medicine. Clinical Practice Guidelines We Can Trust. Washington,
the hospitalist learns that the patient has a penicillin allergy that was not DC: National Academy Press; 2011
reported by the parents during the history and physical examination. The
Institute of Medicine. Crossing the Quality Chasm: A New Health System for the
hospitalist also begins to explore the possibility of initiating a quality improve-
21st Century. Washington, DC: National Academy Press; 2001
ment project in the management of bronchiolitis, which would use the clinical
practice guidelines to create a set protocol for admissions with an order tem- Institute of Medicine. To Err is Human: Building a Safer Health System.
plate in the electronic health record. The patient improves over the subsequent Washington, DC: National Academy Press; 2000
3 days and is transitioned off oxygen. The hospitalist, together with a discharge Leonard MS. Patient safety and quality improvement: medical errors and
planner, reconnects with the PCP, faxes a discharge summary, and arranges a adverse events. Pediatr Rev. 2010;31(4):151–158 PMID: 20360409 https://doi.
follow-up appointment 3 days after discharge at a time when the parents are org/10.1542/pir.31-4-151
available. The patient is seen by the PCP at the scheduled appointment time.
The parents tell the PCP they were told to monitor her respiratory effort, oral Rosen P, Stenger E, Bochkoris M, Hannon MJ, Kwoh CK. Family-centered
fluid intake, and urine output, all of which have been normal. While the parents multidisciplinary rounds enhance the team approach in pediatrics.
have noticed that she still has a runny nose and cough, these symptoms have Pediatrics. 2009;123(4):e603–e608 PMID: 19336351 https://doi.org/10.1542/
improved since discharge from the hospital. peds.2008-2238
Schriefer J, Leonard MS. Patient safety and quality improvement: an overview of
QI. Pediatr Rev. 2012;33(8):353–359 PMID: 22855927 https://doi.org/10.1542/
pir.33-8-353
Selected References
Starmer AJ, Spector ND, Srivastava R, Allen AD, Landrigan CP, Sectish TC;
American Academy of Pediatrics Committee on Hospital Care, Institute for I-PASS Study Group. I-PASS, a mnemonic to standardize verbal handoffs.
Patient- and Family-Centered Care. Patient- and family-centered care and the Pediatrics. 2012;129(2):201–204 PMID: 22232313 https://doi.org/10.1542/
pediatrician’s role. Pediatrics. 2012;129(2):394–404. Reaffirmed February 2018 peds.2011-2966
PMID: 22291118 https://doi.org/10.1542/peds.2011-3084 Zaoutis L, Chang V, eds. Comprehensive Pediatric Hospital Medicine. Philadelphia,
Eichner J, Cooley WC. Coordinating the medical home with hospitalist care. PA: Mosby Elsevier; 2007
Hosp Pediatr. 2012;2(2):105–108 PMID: 24510957 https://doi.org/10.1542/
hpeds.2011-0033

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 CHAPTER 20

Pediatric Genomic Medicine

Moin Vera, MD, PhD, and Henry J. Lin, MD

CASE STUDY
A 4-year-old boy with moderate global developmental Questions
delay is brought to his pediatrician’s office for evaluation. 1. What is microarray technology, and how is it useful
The patient has an unremarkable family history and nor- in pediatric practice?
mal physical examination findings. Previous evaluation 2. How is next-generation sequencing technology
included normal karyotype and fragile X syndrome DNA affecting current practice?
test results. The patient’s parents would like to know 3. What are the limitations of these new technologies?
whether there is anything else that can be done to deter- 4. What is direct-to-consumer genetic testing?
mine the etiology of the delay. In addition, his mother
has recently read about companies that offer multiple
genetic tests to consumers and wonders whether these
tests will be useful as well.

The National Human Genome Research Institute at the National Epidemiology: Human Genome
Institutes of Health describes genomic medicine as the incorporation Anatomy
of an individual’s genomic information into clinical care. In this way,
care involves diagnosis, therapeutic decision-making, health out- Human cells have 2 haploid genomes (ie, 23 pairs of chromosomes,
comes, and policy implications. The Human Genome Project (HGP) 1 pair of most genes), each containing 3 billion base pairs with an
was an extensive, broad-based, and multidisciplinary research effort estimated 20,000 to 25,000 protein-encoding genes, plus a variable
to develop knowledge of biology and disease—leading to the poten- number of copies of the mitochondrial genome. On average, 2 humans
tial for so-called precision medicine, based on genome sequence share 99.9% of their DNA. There are at least 10 million single nucle-
information. The first essentially complete human sequence was otide polymorphisms (SNPs), which are single base changes that are
published in 2003. The cost of sequencing a human genome has present in a substantial percentage of the population (>1%). A small
decreased from approximately $100 million in 2003 to about $1,000 in percentage of SNPs fall within known coding or regulatory regions
2018, according to National Human Genome Research Institute data. of genes and directly influence gene function. The remaining SNPs
Whole exome sequencing (WES) uses next-generation sequencing may have unclear effects on gene function, but they may be inher-
to focus on exons, the regions of the genome that contain the actual ited in recognizable patterns (haplotypes) with other SNPs.
DNA code for making proteins. Overall, the exons amount to about Recently, it has become evident that most human DNA varia-
1.5% of the human genome. Whole exome sequencing costs less than tion is not represented by SNPs. Instead, copy number variants, in
whole genome sequencing, and the functional significance of vari- which DNA segments containing up to several million base pairs are
ants within exons is easier to interpret. Whole exome sequencing or duplicated or deleted, account for a substantial portion of variation
whole genome sequencing is an appropriate study when a single-gene among individuals. Approximately 12% of the genome can exhibit
(or mendelian) disorder is suspected. copy number variation, but the effect of such variation on individ-
Chromosomal microarray analysis—compared with standard chro- ual phenotypes is unknown.
mosome analysis—dramatically increased diagnostic yields for patients Autosomal recessive disorders are caused by pathogenic variants
with intellectual disability, autism spectrum disorder (ASD), and mul- in each of the 2 copies of a disease-associated gene. Carriers of
tiple congenital anomalies. Genetic testing via gene sequencing pan- such disorders, with only 1 variant or disease allele, are typically
els (eg, 20–1,000 genes) and WES have driven diagnostic yields still asymptomatic. Although most autosomal recessive disorders are
further. Direct-to-consumer genetic testing is also available, designed rare, most humans are carriers of several different recessive dis-
to provide information without physician input. Pharmacogenomics, ease alleles. In a 2011 study, an average of 2.8 recessive variants
which promises individualized drug therapy based on genomic data, was observed per person, among 448 genes involved in severe auto-
is moving toward applications to common diseases. somal recessive disorders.

125

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 126 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Pathophysiology: Genotype and which represent identical sequences on both copies of a chromo-
Phenotype Correlations and some. The information may be useful to consanguineous couples,
because it suggests areas where abnormal autosomal recessive dis-
Environment
order genes may be found. In addition, SNP microarray testing can
Genomic data provide information about the genes on which patho- detect some cases of uniparental isodisomy (inheritance of 2 iden-
genic mutations are found. But many diseases are caused by a com- tical copies of a chromosome from 1 parent) and may suggest the
bination of genetic susceptibility and environmental factors. The presence of an imprinting disorder, such as Prader-Willi syndrome.
first examples of such interactions were monogenic conditions, such Although microarray testing has largely replaced standard karyo-
as complement deficiency (which predisposes patients to bacterial typing, microarray testing cannot detect carriers of balanced translo-
meningitis) or mendelian cancer syndromes (which cause extreme cation or patients with mosaicism (when the proportion of abnormal
radiation sensitivity). However, the gene-environment connection cells is <25%–30%). Some deletions or duplications detected by
is now recognized to be more complex. For example, certain envi- microarray testing are benign variants. Therefore, lack of availabil-
ronmental conditions have been found to cause DNA methylation, a ity of parental samples may hinder interpretation of an abnormal
mechanism for gene silencing without changing the DNA sequence. finding in a child.
These so-called epigenetic changes may persist across generations. Next-generation sequencing is a term used to describe methods for
As an example, mothers exposed to wartime famine may birth chil- parallel sequencing of billions of base pairs at relatively low costs. Whole
dren who are predisposed to conservation of energy. When these exome sequencing focuses on the 1% of the genome that encodes pro-
children are fed a typical American diet, they are prone to develop- teins and has been used for clinical testing in the past several years.
ment of obesity and diabetes. Also, fetal cells that persist in maternal A 2013 report shows a diagnostic yield of 25% for this technology in
circulation (fetal microchimerism) may play a role in tumor preven- 250 samples studied. Whole genome sequencing has also become
tion and susceptibility to autoimmune disease. Finally, our bodies clinically available, although perhaps large-scale discovery of varia-
contain more bacteria than human cells, and the interaction of the tion in regulatory elements located outside the coding regions has yet
bacterial and human genomes is thought to play an important role to be fully realized. These technologies have created a major paradigm
in the development of some diseases. shift, by decreasing the time to diagnosis and averting many costly
and invasive procedures, such as muscle biopsy. Improvements in the
Clinical Presentation, History, technology and the bioinformatics used to interpret results are expected
and Physical Examination to increase the use of these tools.
A thorough history and physical examination will continue to be Next-generation sequencing methods have certain limitations
critical components of patient assessment in the genomic age of that should be addressed as technology progresses. Some genes, par-
medicine. Large databases will be needed to correlate human ticularly those with high guanine-cytosine content, are not well cap-
genotypes with corresponding phenotypes defined by the patient’s tured or sequenced with current high-volume technologies. Exome
clinical presentation. Even when genetic and epigenetic sequencing sequencing cannot capture triplet repeat conditions, such as frag-
is commonplace, the only way to measure the effect of the disease ile X syndrome. Microduplications and microdeletions (of exons)
on the individual is by clinical assessment. are not normally detected with current technologies and must be
assessed separately, using a microarray.
Laboratory Tests A patient’s next-generation sequencing results will have thousands
of differences from reference sequences. Bioinformatic algorithms
Microarray testing, which includes a wide range of different technol-
must be used to sift through the data and determine the changes
ogies, has had a dramatic effect on the evaluation of common pediat-
that are potentially relevant to the patient’s condition. Parental test-
ric conditions, such as intellectual disability and ASD. Comparative
ing often provides an essential reference but may not always be avail-
genomic hybridization (CGH) microarray testing uses closely
able. The testing laboratory classifies variants found into 5 categories:
spaced DNA probes to detect chromosomal deletions or duplica-
pathogenic, likely pathogenic, variants of unknown significance,
tions at 100 to 10,000 times the resolution of standard karyotyping.
likely benign, and benign. Family counseling may be limited for vari-
The diagnostic yields for patients with intellectual disability and
ants of unknown significance. Technology will drive down the price
multiple congenital anomalies has increased from 3% to 4% to 15%
of sequencing over time, but the cost of bioinformatics will dominate
to 20%, using this technology. For example, patients with ASD with
the price of these new technologies as the amount of data increases.
normal karyotypes may have microdeletions or microduplications
of 16p11.2. Several relatively common genetic conditions, such as
1p36 deletion syndrome, a form of severe intellectual disability that Direct-to-Consumer Genetic Testing
affects 1 in every 5,000 to 10,000 newborns, have been delineated Several commercial testing companies now market genetic tests
through the use of microarray testing. directly to consumers. These tests purport to provide disease risk
Single nucleotide polymorphism microarray testing has largely information by analyzing multiple SNPs along with common dis-
replaced CGH. It detects the same chromosomal imbalances as CGH, ease mutations. However, the tests may lack sensitivity and specific-
but SNP microarray testing can pinpoint regions of homozygosity, ity, because analyses are based on limited genetic markers without

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 Chapter 20: Pediatric Genomic Medicine 127

family history or phenotype information. Many consumers (and their Future Developments
physicians) are ill-equipped to understand the results, and patients
The huge potential of the HGP now influences several areas of medicine,
of color may have indeterminate results. These companies argue that
including pediatrics. During the next decade, genomics will likely con-
consumers have the right to know their genetic information, and
tinue to revolutionize the diagnosis of rare or previously uncharacterized
some offer genetic counseling services. In 2010, the US Food and
mendelian disorders. The impact of genes on common diseases, such
Drug Administration decided to develop regulations for the sale of
as atherosclerosis, is being investigated through ever larger genome-
direct-to-consumer genetic tests.
wide association studies and polygenic risk scores. Although molecular
diagnoses of previously unidentified diseases have increased over the
Management: Pharmacogenomics past several years, our understanding of disease pathogenesis and treat-
Individualized pharmacological treatment has always been 1 of the ment has not kept pace with the explosion in information. It is unclear
goals of the HGP. Although pharmacogenomics is a fairly young field, how individuals will use genomic information to improve health—
several tests are available that can reduce the risk of an adverse drug whether by altering lifestyles or by use of precision drugs, or both.
reaction. Patients with variant thiopurine methyltransferase alleles In addition, whole exome and whole genome sequencing create
may experience severe toxicity to azathioprine and 6-mercaptopurine. various ethical issues. If a patient presents with heart disease and
Children with a 1555A>G mutation in the mitochondrial genome testing shows an increased risk for Alzheimer disease, should this
are susceptible to aminoglycoside-induced hearing loss, even after information be returned to the patient? It is certain that pediatri-
a single dose of an aminoglycoside antibiotic. Pharmacogenomic cians will need to familiarize themselves with genomic medicine,
treatment of common diseases (eg, asthma) is an active area of inves- because the number of tests—and patients seeking testing—will
tigation and may allow for a more rational choice of drug regimens. exceed the availability of medical genetics specialists.

Glossary
Autism spectrum disorder A medical condition resulting in deficits in social communication and social interaction and characterized by restricted, repeated
interests and behaviors starting in early childhood. Other formal criteria are also used to establish a diagnosis.
Comparative genomic A chromosomal microarray method in which tens of thousands of DNA probes for regions along the genome can be used to detect
hybridization chromosome deletions or duplications at 100 to 10,000 times the resolution of standard karyotyping. Comparative genomic
hybridization has largely been replaced by single nucleotide polymorphism microarray methods. The DNA probes (for binding to
patient DNA) are immobilized on glass slides—called microarrays.
Copy number variant Variants in the structure of the genome having different numbers of copies of DNA segments (usually between 1 kilobase and 5
megabases long). Copy number variants form a large part of human DNA diversity, including causes of some genetic conditions.
Human Genome Project The worldwide effort to determine the DNA sequence of the Homo sapiens genome (3 billion bases). The project was launched in
the United States by the US Department of Energy and the National Institutes of Health. Efforts were also started in France, the
United Kingdom, and Japan. Other countries joined later (eg, Germany, China). The project ran from approximately 1988 to 2003.
A review of the effort stated: “For everyone, this achievement represents a major turning point in our quest to learn how all the
components of the human genome interact and contribute to biological processes and physiological complexity.”
National Human Genome One of the 27 institutes and centers of the National Institutes of Health. It was established in 1989 and is “devoted to advancing
Research Institute health through genome research.”
Single nucleotide The most common and simplest type of DNA polymorphism, in which 1 base is changed to another. These polymorphisms occur
polymorphism roughly every 1,000 base pairs in the genome. Those that occur in or around genes may change the amino acid sequence of the
encoded protein, may produce or remove a stop codon, may impair the usual processing (splicing) of the messenger RNA, may
change how the gene is controlled, or may have no effect at all.
Single nucleotide A chromosomal microarray method that has largely replaced comparative genomic hybridization. It may contain a few million
polymorphism (SNP) oligonucleotide probes (approximately 25 nucleotides long) for regions along the genome. In addition to detecting chromosome
microarray testing deletions and duplications, SNP microarray testing can also detect long regions of homozygosity, indicating uniparental disomy or
consanguinity. The diagnostic utility of chromosomal microarray testing among children with intellectual disability, autism spec-
trum disorder, and congenital anomalies has been estimated to be 10% to 20%.
Whole exome Sequencing all the known coding regions in the genome. The diagnostic utility of WES among children with intellectual disability,
sequencing (WES) autism spectrum disorder, and congenital anomalies has been estimated to be at least 30% to 40%.

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 128 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

Kalia SS, Adelman K, Bale SJ, et al. Recommendations for reporting of second-
CASE RESOLUTION ary findings in clinical exome and genome sequencing, 2016 update (ACMG
SF v2.0): a policy statement of the American College of Medical Genetics
The patient’s microarray testing results show a small microdeletion in chro-
and Genomics. Genet Med. 2017;19(2):249–255 PMID: 27854360 https://doi.
mosome 6. Parental testing indicates that the microdeletion is present in the
org/10.1038/gim.2016.190
patient’s father, who has had normal development. Further testing includes
WES, which shows a potential missense mutation in CASK, a gene on the X chro- Khera AV, Chaffin M, Aragam KG, et al. Genome-wide polygenic scores for com-
mosome that may cause developmental delay. This alteration is not found in the mon diseases identify individuals with risk equivalent to monogenic mutations.
patient’s mother, implying that it is most likely deleterious. The parents receive Nat Genet. 2018;50(9):1219–1224 PMID: 30104762 https://doi.org/10.1038/
genetic counseling about future pregnancies. s41588-018-0183-z
Kuehn BM. Inconsistent results, inaccurate claims plague direct-to-consumer
gene tests. JAMA. 2010;304(12):1313–1315 PMID: 20858870 https://doi.
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Li MM, Andersson HC. Clinical application of microarray-based molecular
National Human Genome Research Institute “The Human
cytogenetics: an emerging new era of genomic medicine. J Pediatr. 2009;155(3):
Genome Project”
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Nussbaum RL, McInnes RR, Willard HF, eds. Thompson & Thompson Genetics
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Adams DR, Eng CM. Next-generation sequencing to diagnose suspected genetic human genome. Nature. 2006;444(7118):444–454 PMID: 17122850 https://doi.
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org/10.1056/NEJMra1711801 Rogers J. The finished genome sequence of Homo sapiens. Cold Spring Harb Symp
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sive diseases by next-generation sequencing. Sci Transl Med. 2011;3(65):65ra4 Schleit J, Naylor LV, Hisama FM. First, do no harm: direct-to-consumer
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Bloss CS, Schork NJ, Topol EJ. Effect of direct-to-consumer genomewide profil- org/10.1038/s41436-018-0071-z
ing to assess disease risk. N Engl J Med. 2011;364(6):524–534 PMID: 21226570 Solomon BD, Hadley DW, Pineda-Alvarez DE, et al; NISC Comparative
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tion in molecular diagnostics. Expert Rev Mol Diagn. 2018;18(6):531–542 PMID: org/10.1542/peds.2011-0080
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org/10.1146/annurev-med-051010-162644

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 CHAPTER 21

Principles of Quality
Improvement: Improving
Health Care for
Pediatric Patients
Bonnie R. Rachman, MD

CASE STUDY
During a routine staff meeting at your group pediatric Questions
practice, it was noted that many of the patients cared for 1. What is quality improvement?
by your practice are behind in their immunizations. The 2. How does assessing the delivery of recommended
reasons for this are unclear, because you and your col- health maintenance relate to quality?
leagues are strong proponents of the timely administra- 3. How is the prevention of medical errors related to
tion of preventive immunizations. You want to develop quality improvement?
a mechanism to determine what factors are resulting in 4. What is the difference between harm and error?
delayed vaccine administration. 5. What factors are associated with medical errors?
6. What is meant by organizational culture?

History “The key is to practice continual improvement and think of manu-

The origin of medical quality improvement (QI) lies not in med- facturing as a system, not as bits and pieces.”
icine but in industry and dates to the 1920s. Walter A. Shewhart, In the 1970s, physician Avedis Donabedian, MD, MPH, proposed
PhD, was an American physicist, engineer, and statistician. In a model for assessing health care quality by describing 7 pillars of
1924, Shewhart presented a memo in which he showed a sche- quality (Box 21.1). He posited that structure is the environment
matic control chart. The diagram and text set forth all the essential in which health care is provided, process is the method by which
principles and considerations involved in quality control. Shewhart health care is provided, and outcome is the result of the care pro-
pointed out the importance of reducing variation in a manufactur- vided. He emphasized the importance of measurement and evalu-
ing process and noted that continual process adjustment in reac- ation of health care quality, ensuring completeness and accuracy
tion to nonconformance actually increased variation and degraded of medical records, observer bias, patient satisfaction, and cultural
quality. He went on to develop data presentation rules that remain preferences for health care.
important today: Data have no meaning apart from their context, In 1991, Paul B. Batalden, MD, and Donald M. Berwick, MD,
and data contain signal and noise. To be able to extract informa- MPP, FRCP, helped found the Institute for Healthcare Improvement
tion, it is imperative to separate the signal from the noise within (IHI), which has resulted in the application of QI science to health
the data. care both in the United States and internationally. The IHI promotes
In the 1950s, Shewhart’s colleague, W. Edwards Deming, PhD, the adoption of measurement and feedback to improve the quality
while working in Japan, taught top management how to improve of health care. The IHI is an easily accessible resource for education
design and, subsequently, service, product quality, testing, and and information related to QI in health care.
sales. Deming, applying Shewhart’s concepts, described the Plan- Historically, QI efforts were focused on “quality assurance.” The
Do-Study-Act (PDSA) method as an approach to QI. He opined, goal was to minimize “defects” as measured by audits. Currently,

129

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 130 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

The Learning from Errors in Ambulatory Pediatrics study aimed

Box 21.1. The Seven Pillars of Quality
to learn the scope of, range of, potential causes of, and possible solu-
ww Efficacy tions to medical errors in pediatric ambulatory care. The study
ww Efficiency found that among 14 pediatric practice sites, 147 medical errors
ww Optimality were reported during the 4-month study period. The largest group
ww Acceptability of errors was related to medical treatment (37%). Of the medical
ww Legitimacy treatment errors, 85% were medication errors. Errors also were asso-
ww Equity ciated with patient identification (22%); preventive care, including
ww Cost immunizations (15%); diagnostic testing (13%); and patient com-
munication (8%). According to the Medical Professional Liability
Association (formerly the Physician Insurers Association of America),
the emphasis is on QI, which focuses on moving the entire perfor- from 2003 through 2012 diagnostic errors accounted for 828 closed
mance curve toward a higher level of performance by identifying and pediatric cases, with an average indemnity of $414,455, and medica-
adopting best practices rather than simply focusing on low perform- tion errors accounted for 113 closed cases, with an average indemnity
ers. Ongoing measurement is vital to sustaining any improvement. of $207,916. According to a systematic review, as many as 10% of all
The process of QI requires careful planning, thorough documenta- pediatric medication orders result in a medication error.
tion, consistent analysis, and open-mindedness to results. In health Many examples exist of errors in inpatient care as well. A study
care, QI consists of systematic and continuous actions that result in of inpatient medication errors found that among 1,000 children in
a measurable improvement in health care services and the health 12 independent children’s hospitals, 1 in 15 children was exposed
status of the targeted patient group. to wrong medications, side effects, or drug interactions. Takata and
Currier reported a rate of 11.1 adverse events per 100 pediatric inpa-
Quality Improvement in Pediatrics tients; 22% of these errors were deemed preventable. In a 2014 report
Quality improvement works to strengthen systems and processes from The Joint Commission, the root causes of more than 80% of
and, in medicine, focuses on patients and the use of data to drive almost 2,400 sentinel events were human factors (eg, staff supervi-
improvement in patient safety and health care. To achieve a differ- sion issues), leadership (eg, organizational planning), communica-
tion (eg, with patients or administrators) and assessment, including
ent level of performance and improve quality, an organization must
timing and/or scope.
be willing to change.
Kurtin and Stucky describe 5 barriers or challenges to high-
The specialty of pediatrics emphasizes health maintenance
quality care in the pediatric and adult health arenas, the first of
and disease prevention. Studies have shown that recommended
which is widespread unnecessary variation in care (Table 21.1). To
targets are not being consistently realized, however. Mangione-
prevent medical error, it is necessary to understand how, when,
Smith et al reported that children received only 46.5% of recom-
and where it occurs; evaluate the system that allowed the error to
mended care for preventive services, acute illness management,
happen; and keep the system open and blame-free (Box 21.2).
and ongoing care of chronic conditions. In 2017, as reported by
Factors associated with risk of error are not the individuals who
the Centers for Disease Control and Prevention, this number had
work in the system but rather the system in which the individuals
not changed. Shaughnessy and Nickel reported that 21% of out-
work. To reduce the risk of error, the language of patient safety must
patient prescriptions in a family medicine practice contained
be understood by all health professionals, and a deeper understand-
at least 1 error. Other investigators found that medication sam-
ing of the principles of systems analysis work models and other
ples were dispensed with inadequate documentation. In a pediat-
solutions must be developed.
ric emergency department in Canada, 100 prescribing errors and
39 medication administration errors occurred per 1,000 patients.
In a sample of new prescriptions for 22 common medications in Definitions
outpatient pediatric clinics, approximately 15% were dispensed Understanding QI and improving patient safety requires familiar-
with potential dosing errors. Even with the use of electronic med- ity with the terminology. The National Patient Safety Foundation
ical records and e-prescribing technology, prescribing errors con- defines patient safety as the avoidance, prevention, and ameliora-
tinue to occur as the result of auto-populate errors and/or choosing tion of adverse outcomes or injuries stemming from the processes
the wrong medications and/or wrong formulations from the drop- of health care. The Health and Medicine Division of the National
down menus. Multiple medication alerts during the prescribing Academies (formerly the Institute of Medicine [IOM]) defines
task distract the clinician, resulting in errors. In addition, these fre- patient safety as freedom from accidental injury.
quent and often irrelevant alerts may cause “alert fatigue.” Outdated An adverse event is injury caused by medical management rather
or incorrect information, such as weight entered as pounds rather than by an underlying condition. Some adverse events are not pre-
than kilograms into the electronic medical records also may result ventable. One example of a non-preventable adverse event is the
in dosing errors. patient who receives the appropriate antibiotic at the appropriate

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 Chapter 21: Principles of Quality Improvement: Improving Health Care for Pediatric Patients 131

Table 21.1. Barriers to High-quality management decisions. Latent errors are more difficult to identify
Health Care and hard to recognize. Health care workers frequently develop work-
arounds to bypass the problem, which often leads to the belief that
Barrier Explanation
the work-around is normal.
Unnecessary variation in care Diagnostic or therapeutic interven- A system is a set of interdependent human and nonhuman
tions performed at the discretion elements that interact to achieve a common aim. In a hospital,
of the ordering physician and not a system may be a unit or a department. Processes are the means
required by the patient’s condition by which care is delivered. Questions related to process include
Gap between knowledge and The time—perhaps many years— the following: Are policies routinely followed? Are evidence-based
practice from publication of a proven new medicine guidelines implemented? Does the transfer of patients
practice in the medical literature to occur in an organized manner? Work models provide a concep-
its use in routine clinical care tual framework for investigating events and processes to ensure
Failure of physicians to understand Challenges to accessing information, the evaluation of all contributing factors. The PDSA method is
and work in the hospital’s complex data, personnel, and/or materials to an example of a conceptual framework. The premise of all work
systems of care facilitate patient care models is that an organized, systematic approach to event inves-
Need to improve patient safety No consistent mechanism in place tigation results in reliable data that may be used to develop a
new system.
Slow adoption and routine use of May be mitigated by the use of clini-
Continuous QI (CQI) is the continued process of reviewing and
practices that can improve clinical cal pathways
improving processes and procedures associated with providing
outcomes and patient safety
goods or services. A CQI approach may involve evaluating struc-
ture, process, or outcome either individually or—because of the con-
siderable overlap that exists between the components and quality
Box 21.2. Factors Associated With Errors of care—as a whole.
in Health Care When evaluating the quality of health by comparing outcomes,
it is important to understand the concept of risk adjustment, which
ww Communication failure
allows statistical adjustment of patient differences (eg, severity of
ww Frequent distractions and interruptions
illness) to make comparisons of outcomes clinically meaningful. Use
ww Inadequate supervision
of risk adjustment enables the translation of statistically significant
ww Medication issues
tests into clinically meaningful results.
ww Limited access to patient information
ww Noisy work environment
ww Lack of 24-hour pediatric pharmacy Errors and How They Occur
ww Emergency situations The pervasiveness of the medical error problem is enormous. In
1999, the IOM published To Err Is Human: Building a Safer Health
System, in which it was reported that medical error accounted for
time in an appropriate dose and who has an allergic reaction to the approximately 44,000 to 98,000 deaths annually in US hospitals.
medication because of the existence of an allergy that was unknown This is more deaths than from AIDS, motor vehicle crashes, or
to both the patient and the staff. This differs from medical error, breast cancer. The types of errors included adverse drug events,
which is the failure of a planned action to be completed as intended improper transfusions, surgical injuries, wrong-site surgeries,
(ie, error in execution) or the use of a wrong plan to achieve an aim mistaken patient identity, and failure to prevent patient suicides.
(ie, error of planning). Outcomes summarize the effectiveness of Most errors occurred in intensive care units, operating rooms, and
care, including adverse events. emergency departments. The annual total cost of these errors was
James Reason, who did extensive work on organization models estimated to be between $17 billion and $29 billion. The cost of
of accidents, further delineates the terminology. A slip is an error additional care was $8 billion. This figure does not account for lost
in execution, that is, the observable action deviates from what was income and productivity. A 2004 report of inpatient deaths esti-
planned. A lapse is an error in execution resulting from a memory mated that 575,000 deaths were caused by medical error between
failure. A mistake is a knowledge-based failure, that is, the plan was 2000 and 2002, approximately 195,000 deaths per year. In 2008, the
performed correctly, but the planned action was wrong for the sit- US Department of Health and Human Services Office of Inspector
uation. An active error (ie, sharp-end error) typically occurs in a General reported 180,000 deaths per year resulting from medical
patient care area by a frontline practitioner; the effects may be felt error among Medicare beneficiaries alone.
immediately. A latent error (ie, blunt error) is the result of a systems- The IOM made many recommendations in their report, includ-
based problem and may relate to poor design, incorrect installation, ing a balanced approach between regulatory and market-based ini-
look-alike packaging, soundalike names, faulty maintenance, or bad tiatives as well as the establishment of a national focus to create

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 132 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

leadership, research, tools, and protocols to enhance the knowledge differentiate? Focusing on harm puts the microscope on the system,
base about safety. It recommended raising performance standards not the individual.
and expectations for improvements in safety through the actions of
oversight organizations, professional groups, and group purchasers Quality Improvement and
of health care. An additional recommendation was to implement Organizational Culture
safety systems in health care organizations to ensure safe practices
“This is the way we do things around here.”
at the delivery level.
Patients expect to receive quality health care without experienc-
In 2001, the IOM published a follow-up report, Crossing the
ing preventable harm. The IOM defined quality in health care as
Quality Chasm: A New Health System for the 21st Century. Safety
“the degree to which health services for individuals and populations
was deemed the key dimension of quality. The report also indi-
increase the likelihood of desired health outcomes and are consis-
cated that only a systems approach would work to improve qual-
tent with current professional knowledge.” One factor that influences
ity in health care; trying harder is an inadequate approach. Per the
quality in health care is organizational culture.
report, a stepwise correction of problems in the system is the key
Many pediatricians are never taught about organizational cul-
to success. Additionally, it indicated a need to overcome the
ture. It is the invisible, powerful dictator of how things are done in
culture of blame, because human error is to be expected. Table 21.2
an organization. Organizational culture is a latent and often uncon-
defines types of errors in health care.
scious set of forces that determines individual and collective behav-
Typically, medical errors are neither isolated events, nor are
ior, ways of perceiving, thought patterns, and values. Hospitals have
they made by a single person. Normally, numerous safe points and
their own cultures. Subcultures exist among physicians and nurses
double checks are built into the process; however, each layer of
as well as within departments (eg, critical care). It may be challeng-
safety has gaps or holes. When these gaps align, the error reaches
ing to understand all the different subcultures within a hospital.
the patient. Standardized approaches can reduce variability and
Activities or processes within a health care organization contain
improve system efficiency. The goal is to make the gaps as small as
2 major components: what is done (ie, what care is provided) and
possible or even eradicate them. Many factors are associated with
how it is done (ie, when, where, and by whom). Quality improve-
medical error, including human factors triggered by interruptions,
ment can be achieved by addressing either component, but address-
fatigue, time pressures, anger, anxiety, fear, and boredom. Mistakes
ing both simultaneously has the greatest effect on QI. The goal of
can result from a wrong plan of action. Mistakes may involve mis-
QI is to enable an organization to achieve the ideal critical pathway,
interpretation of a problem, lack of knowledge, and habitual pat-
which is one that allows proactive and efficient interaction between
terns of thought. A violation is a purposeful rule violation, whether
the care team and patient to achieve optimal health outcomes.
reasoned or reckless. The factors associated with risk are not the
Promoting change is rarely easy. Some people may adapt and
individuals who work in the system but rather the system in which
change appropriately, while others resist and become dysfunctional.
the individuals work.
Making changes requires an environment in which teamwork can
grow. Groupthink must be avoided. The term groupthink was coined
Harm Versus Error by Yale psychologist Irving Janis, PhD; it is “a way of thinking that
The ultimate goal of QI is to reduce patient harm. Early QI efforts people may adopt when they are members of a cohesive or homo-
targeted the elimination of error in the belief that reducing error geneous group; in particular, a group whose members seek una-
would decrease harm. This is not true, however; most medical nimity of thought to the point that they cannot consider alternative
errors never harm patients and may be clinically insignificant (eg, ideas.” It can prevent critical thinking and debate. Without disagree-
1-hour delay in administration of acetaminophen). So why does one ment, creativity can be lost. Negative effects of groupthink include an
illusion of invulnerability, insulated leaders who may be protected
from contradictory evidence, and members who accept confirming
Table 21.2. Types of Errors in Health Care data and reject data that fail to fit their views. Alternatives are not
Error Definition considered, and individuals with conflicting views are discounted
Diagnosis Delayed diagnosis: failure to use indicated tests, use of or demonized. Talented leaders welcome diversity of thought and
outmoded tests or therapy, or failure to act on results of ideas. Leaders must empower team members to have open discus-
monitoring or tests sions and offer ideas.
Treatment Error in the performance of an operation, procedure, or Communication is a key component of organizational culture.
test; an avoidable delay in treatment or failing to respond Communication involves 2 parts: message and meaning. The mes-
to an abnormal test; inappropriate/not indicated care sage is stated; meaning is interpreted and may be interpreted dif-
ferently by different people. Strategies to improve communication
Medication The most frequent type of error, which includes error in
are legion, including methods for remaining calm in high-workload,
the dose; method of using a drug
high-stress situations. Other strategies include increased verbal-
Prevention Failure to provide prophylactic treatment, or inadequate ization, verbalizations that relate to problem-solving, speaking in
patient monitoring or treatment follow-up the first-person plural, readback, and coordinating tasks to the

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 Chapter 21: Principles of Quality Improvement: Improving Health Care for Pediatric Patients 133

right person’s experience level. Good communication encourages be intermittently assessed within and between observers over time
input from team members. Offering positive feedback builds con- to ensure consistency.
fidence, reduces stress, and clarifies ambiguities. Team evaluations Measures must also be valid. A measure is valid if it adequately
provide input on how well team members are communicating represents the attribute of interest. Internal validity is the sound-
with each other and the progression toward achieving team goals. ness of the developed indicator. External validity is the capacity for
Benchmarking against a similar team may provide valuable infor- application of the indicator to a broader population.
mation. An outside consultant may be hired to observe the team Measures must be feasible and usable. Feasibility is the capacity
and provide feedback. Internally, the use of informal, regular meet- to gather measures. For a measure to be usable, the intended audi-
ings to discuss the team’s progress and provide debriefing sessions ence must be able to understand the findings and use them in an
to discuss the team process may be constructive in moving the team appropriate fashion. A measure is usable if it enables the uncover-
toward its goals and ensuring effective communication. All teams ing of meaningful differences between groups.
experience team conflict at some point. Conflict occurs when people Three types of measure are essential to QI: outcomes measures,
come together to resolve a problem, dialogue about care improve- process measures, and balancing measures.
ment, or discuss changing processes to improve care. Team conflict 1. Outcomes measures: Address how the health care services pro-
must be managed effectively so that issues can be resolved while still vided to patients affect their health, functional status, and sat-
providing the highest-quality patient care. isfaction (eg, the percentage of patients who died as a result of
Teamwork has been defined by numerous organizations. In surgery).
2003, the IOM defined teamwork by stating, “All health professionals 2. Process measures: Address the health care services provided to
should be educated to deliver patient-centered care as members of patients (eg, the percentage of patients whose hemoglobin A1C
an interdisciplinary team, emphasizing evidence-based practice, QI level was measured in the past year).
approaches, and informatics.” The IOM defined a multidisciplinary 3. Balancing measures: Evaluate unintended consequences or the
team as one in which members cooperate, communicate, and inte- stability of the system being changed in the project (eg, ensuring
grate care to ensure that patient care is continuous and reliable. that readmission rates are not increasing as a result of reducing
length of hospital stay).
Measuring Quality A balanced set of measures for a QI effort should include at least
1 outcome, 1 process, and 1 balancing measure. After measures are
Data are the cornerstone of QI. Ideally, measurements should evolve
identified, an organization determines its data collection frequency
from the Health and Medicine Division of the National Academies
and sampling. More frequent data collection allows an organization
quality aims (Box 21.3). Improvement measures may not be suffi-
to focus its QI efforts more aggressively.
ciently valid and reliable for public dissemination yet may still be
useful for benchmarking, for identifying best practices, or as part
of QI initiatives. Quality Improvement Models
Adult measures may not be appropriate for pediatrics. Pediatrics After opportunities for performance improvement have been iden-
is unique because of the heterogeneity of patients, including age, size, tified, changes can be made to the underlying system targeted for
diagnoses, and treatment modalities, including medication dosing. improvement. The use of QI models alone or in combination is an
Measures must be reliable. Reliability is the capacity of the mea- effective approach for categorizing potential changes in the organi-
sure to perform similarly under stated conditions over time. In zation’s system and identifying changes that have worked in simi-
health care, reliability and precision are often used interchange- lar settings.
ably. Precision is the capacity of a measurement process to repro- Multiple QI models exist. Both the Care Model and Lean meth-
duce its own outcome. To achieve precision, a reliable system odology provide frameworks for improving patient care, whereas
requires clear and concise definitions of the data fields to be col- the Model for Improvement, the FADE (focus, analyze, develop,
lected. An effective approach is to train data collectors. A robust execute and evaluate) model, and Six Sigma focus on processes.
method for data coding and entry is also required. Reliability must There are 6 fundamental aspects of care identified in the Care
Model, which creates a system that promotes high-quality disease
and prevention management. It does this by supporting produc-
Box 21.3. National Academy of Medicine tive interactions between patients, who take an active part in their
Quality Aims care, and health professionals, who have the necessary resources
ww Safe and expertise.
ww Effective Lean defines value based on what the patient wants. It maps how
ww Patient-centered the value flows to the patient and ensures the competency of the
ww Timely process by making it cost effective and time efficient.
ww Efficient The Model for Improvement focuses on questions to set the aim or
ww Equitable organizational goal, establish measures, and select changes. It incor-
porates PDSA cycles to test changes on a small scale.

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 134 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

The FADE model is separated into 4 broad steps: (1) focus: define entire system to adverse events. Identifying errors is important,
the process to be improved; (2) analyze: collect and analyze data; but focusing solely on errors may lead to “the blame game.” Trends
(3) develop: develop action plans for improvement; (4) execute: may be a barometer for organizational culture as it relates to patient
implement the action plans, and evaluate (ie, measure and monitor safety. Identified errors provide learning opportunities, thereby
the system to ensure success). preventing harm to other patients.
Six Sigma is a measurement-based strategy for process improve- Injury-based patient safety metrics focus on unintended out-
ment and problem reduction. It is completed through the applica- comes, such as catheter-related bloodstream infection, ventilator-
tion of the QI project and accomplished with the use of 2 Six Sigma associated pneumonia, in-hospital cardiac arrest, and death.
models: DMAIC (define, measure, analyze, improve, control), which Improvement efforts are more likely to focus on system vulnerabil-
is designed for use in examining existing processes, and DMADV ities with a high potential for adverse events.
(define, measure, analyze, design, verify), which is used to develop Risk-based patient safety metrics are a means of measuring haz-
new processes. ards or risks. From a systems-based view, errors and injuries are the
result of hazards or risks within the system of care. The focus is on
Error Metrics hazardous conditions that increase the likelihood of downstream
Measuring harm is often done using occurrence reports, which iden- errors. Efforts are centered on 3 core areas: systematic risk iden-
tify 2% to 8% of all adverse events in an inpatient setting. These tification, risk assessment, and risk reduction and/or elimination.
reports may be done with retrospective, concurrent, or trigger-based This approach is proactive and saves counting errors and injuries.
chart review. No standardized methods exist for reporting, investi- The elimination of risk includes the substitution of less risky alter-
gating, or disseminating information related to preventable adverse natives, the development of administrative controls, and individual
events. Historically, the qualitative concept of patient safety has been protection. It incorporates information gathered from the front-
translated into quantitative metrics. Three frameworks that have line bedside practitioner. The focus on risk reduction may decrease
been used include measuring error, measuring patient injuries, and the need to quantify error rates or injury prevalence. This requires
measuring risk. a shift in thinking from counting errors and injuries to proactively
Error-based patient safety metrics are premised on the idea that identifying risk. The goal is to identify systems-level problems that
the goal of medicine is to successfully implement the correct plan may be amenable to QI efforts.
of care. It is an attractive measure of assessing safety because many
errors occur and are somewhat easy to find. Many problems exist Patient Safety and Error Prevention
with error metrics, however, because few measures of error repre-
The Institute for Safe Medication Practices developed a rank order
sent the true rate.
of error-reduction strategies ranging from most effective (ie, forc-
Error rate = (identified errors)/(potential opportunities for that ing functions and constraints) to least effective (ie, education and/
error to occur) or information). In between are strategies (from most to least effec-
The numerator (identified errors) may be hard to obtain because tive), such as automation and computerization, standardization
with reported events, only the errors reported are known, rather and protocols, checklists and double-check systems, and rules and
than the actual number of occurrences. Thus, the error rate is noth- policies.
ing more than a rate of reporting. Education and information dissemination are important and
Another modality that has been proposed to obtain the true helpful in that they increase awareness. Lectures are quickly for-
numerator is chart review. The difficulty with chart review lies in gotten, however, and signs are often ignored. Education and infor-
the number of steps in the process—error occurs, each error is mation dissemination is the least effective means to prevent the
recognized by a health professional, the error is documented by a occurrence of error. Rules and policies are required by several agen-
health professional, the chart is reviewed, the reviewer recognizes cies. Although rules and policies are a good resource, they are not
the event during the chart review, and the error is attributed cor- an effective means of preventing a specific event.
rectly. Ethnographic study (ie, the direct observation of people) is Checklists, double-check systems, and bundles are very effective
subject to the same pitfalls. when they are a routine part of practice, but they are not foolproof.
Other confounders include hindsight bias. To effectively rec- The best example of a double-check system is the surgical sponge and
tify problems, the correct understanding and attribution of events instrument counts done at the end of a surgical case. Checklists and
that created the error must occur. The retrospective analysis of bundles are also being used for other procedures, such as central line
error creates the potential for incorrect or simplistic identification placement. Standardization minimizes the risk of error, and proto-
of causes of events. Subsystem failures are difficult to identify as cols bring a standard approach to a clinical issue. Checklists, bundles,
a contributing cause of an adverse event. The incorrect or inade- and standardization are designed to eliminate variation from prac-
quate attribution of causality creates the potential for misguided titioner to practitioner and patient to patient. Any remaining weak
actions to “solve” the wrong problem. points are prone to the occurrence of error. Automation and comput-
Focusing solely on active errors leaves latent failures unrecog- erization are 2 of the best means of preventing medical error; how-
nized and unaddressed, thus increasing the vulnerability of the ever, there is still a risk of data entry error. Forcing functions and

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 Chapter 21: Principles of Quality Improvement: Improving Health Care for Pediatric Patients 135

constraints is the best way to prevent error through the use of safe- NHS Institute for Innovation and Improvement; 2008. https://webarchive.
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it takes to make the change, the ideas on which to base the design five-havens/deming.htm. Revised April 27, 1998. Accessed March 15, 2019
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the Quality Chasm: A New Health System for the 21st Century. Washington, DC:
During your staff meeting, you decide to use a PDSA cycle to examine the organi-
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fashion. LT, Corrigan JM, Donaldson MS, eds. To Err is Human: Building a Safer Health
Plan: The charts of all patients seen for routine care in the past 3 months are System. Washington, DC: National Academies Press; 2000
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Kozer E, Scolnik D, Macpherson A, et al. Variables associated with medication
ment this change, because it is low cost and easy to put into practice.
errors in pediatric emergency medicine. Pediatrics. 2002;110(4):737–742 PMID:
Study: Three months after implementation, you collect data and discover
fewer missed opportunities for immunization and that your program has had 12359787 https://doi.org/10.1542/peds.110.4.737
some success. Some parents are still resistant to immunizing their children for Kurtin P, Stucky E. Standardize to excellence: improving the quality and safety of
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Mangione-Smith R, DeCristofaro AH, Setodji CM, et al. The quality of ambula- Reason J. Human error: models and management. BMJ. 2000;320(7237):
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 CHAPTER 22

Pediatric Palliative Care:

Principles and Practice
Jori Bogetz, MD, FAAP, and Richard Goldstein, MD, FAAP

CASE STUDY
Jason is a 17-year-old boy with spastic quadriplegia, medical care Jason receives translates into quality of life
severe global developmental delay, seizures, dystonia, for him and his family.
and cortical blindness who is supported by a tracheos-
tomy but is not ventilator dependent. He was born at
Questions
1. What is pediatric palliative care, and how is it
24 weeks’ gestation and had a turbulent neonatal course.
practiced?
Since leaving the intensive care unit at 6 months of age,
2. What children receive palliative care, and what are
he has lived at home, cared for by his family, a loyal
the benefits and barriers to these services?
home nursing team, and his primary care pediatrician.
3. What are some of the essential considerations
Medically he has been fairly stable, with only episodic
when communicating with families of seriously ill
respiratory infections and numerous orthopedic proce-
children?
dures. Over the past 2 years, however, he has spent sub-
4. How do children of different ages understand seri-
stantially more time in the intensive care unit because
ous illness, death, and dying?
of increasing respiratory fragility. With so many hospi-
5. What role do primary care pediatricians have in the
talizations and so little time feeling well at home, his
palliative care of their patients?
parents have begun questioning whether the intensive

The Scope and Practice of Pediatric of palliative care, which is care that incorporates palliative care with
Palliative Care disease-directed treatment throughout a child’s illness and extends
into bereavement (Figure 22.1). Despite its growing acceptance, pal-
Pediatricians develop deep, meaningful relationships with patients liative care is often incorrectly equated with end-of-life and hospice
and their families. Mostly, patient care involves the typical ups and care. Hospice is a program of coordinated services offering comfort-
downs of childhood with acute pediatric illnesses. But some patients centered care at the end of life in the home or community setting.
are challenged by a degree of illness that requires high levels of con- Although hospice and palliative care have a shared philosophy and
tinuous medical management, care coordination, and advocacy. an overlap of many of the priorities of care, palliative care should
These children and their families live with significant uncertainty not be considered only at the end of life, nor is it exclusive of cura-
about the future and a “roller coaster” of health experiences that tive or disease-directed care.
may even involve the child’s death. Along with these difficulties, Palliative care is often thought of as a specialized area of prac-
many pediatricians describe caring for children and families with tice. Subspecialty palliative care (ie, secondary palliative care) is
serious illness as among the most rewarding aspects of their careers. practiced by health professionals with additional training who
Palliative care is a framework that prioritizes the well-being of often provide education and advocacy in the field of palliative med-
patients and families experiencing serious illness. This is accom- icine and provide direct patient care in quite complex clinical sit-
plished through careful symptom management; attention to the uations. Important elements of palliative care can be practiced by
medical, psychosocial, and spiritual needs of patients through mul- other health professionals with trusted longitudinal relationships
tidisciplinary care; and establishing goals of care based on family- and tremendous insights into family dynamics and priorities, how-
centered priorities. The American Academy of Pediatrics states that ever. Primary palliative care is the term used to describe the pallia-
pediatric palliative care should be integrated starting at diagnosis tive care provided by nonsubspecialty health professionals. It is the
and extending into hospice care and bereavement for any child with a most fundamental form of palliative care that patients and their fam-
serious illness. This care should be provided along with that provided ilies receive. The skills of primary palliative care include the abilities
by primary care pediatricians and in conjunction with community- to hold basic discussions about prognosis and goals of care, man-
based teams. These standards are illustrated in the concurrent model age basic pain and symptoms, and address family and sibling issues
137

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 138 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

that a child’s life was lived with identity and value, in contrast to a
life determined by the course of illness alone.
Curative
care
Hospice
Palliative
care Communicating Prognosis, Disclosure,
care
Bereavement
and Decision Making
The care of children with serious illness can be marked by periods
Diagnosis Dying 6-month Death of intense uncertainty and fearful realities. Helping families deter-
prognosis mine the goals of care, appropriate treatment choices, and all aspects
Patients
of planning for the child’s life is facilitated by thoughtful commu-
Families
nication. Research has found, however, that many parents feel they
Disease progression
receive confusing, inadequate, or uncaring communication related
Figure 22.1. Integration of palliative care along with disease-directed to prognosis and treatment. They report often feeling left to reach
treatments and continuing into bereavement. decisions with an understanding of medical details that is different
Adapted with permission from Ferris FD, Balfour HM, Bowen K, et al. A model to from that of their child’s health professionals. Such decisions are
guide patient and family care: based on nationally accepted principles and norms of especially difficult in a setting of misunderstanding, disagreement,
practice. J Pain Symptom Manage. 2002 Aug;24(2):106-23. or, worse, lack of trust. When feeling misunderstood or judged, par-
ents may feel conflicted about stating their true perspective.
that arise related to the illness course. Another important part of To this end, parents benefit from honest, clear communication
primary palliative care is the relationship that remains between the about their child’s illness. This is a difficult task, not only because
clinician and family after a child dies. Both primary and secondary imparting bad news is an uncomfortable and complex task but
palliative care are necessary in helping children and their families also because providing an exact prognosis is fraught with chal-
live as well as possible with serious illness. lenges. The most recent systematic reviews on prognosis predic-
Palliative care for children may be considered in the context of tion have found that physicians are accurate only approximately
4 major categories of serious illness: conditions for which curative 25% of the time and tend to be overly optimistic. Although more
treatment is possible but may fail (eg, cancer with a poor prognosis, experienced health professionals tend to err less, the longer the
complex congenital heart disease); conditions requiring intensive length of a relationship with a patient, the greater the likelihood
long-term treatment aimed at maintaining quality of life (eg, cystic that the prognosis they share will be incorrect. Parents nonethe-
fibrosis, muscular dystrophy); progressive conditions in which less seek a clear disclosure that allows them to have a sense of the
treatment is exclusively palliative after diagnosis (eg, Tay-Sachs future. They interpret hidden or minimal information as evidence
disease, leukodystrophy); and conditions involving severe, nonpro- that the health professional is withholding frightening informa-
gressive disability, causing extreme vulnerability to health compli- tion. In fact, parents who receive more elements of prognostic
cations (eg, holoprosencephaly, extreme preterm birth with severe disclosure are more likely to report communication-related hope,
comorbidities). Many of these conditions are affected by medical even when the likelihood of cure for their child is low. Often the
advances and evolving clinical practices. The use of bone mar- specific details about prognosis are not necessary and instead,
row transplant, gene therapy, and immunotherapy, as well as the generalities about the expected timeline (eg, hours to days, days
increasing application of known therapies such as noninvasive ven- to weeks, weeks to months, months to years) provide sufficient
tilation, have important implications when counseling patients and information for the family’s planning, coordination, and mem-
families. These evolving norms provide opportunities for health ory making. Research has also shown that earlier recognition
professionals to consider categories of illness while simultaneously of a poor prognosis predicts an earlier do-not-resuscitate order,
embracing uncertainty about outcomes for their patients in using decreased use of disease-directed therapies in the last months of
a palliative care framework. life, and an increased likelihood of incorporating the child’s com-
To implement appropriate palliative care, families often need fort as a goal. It allows for some sense of control and expression
an array of different providers, including doctors, nurses, social of values in overwhelming circumstances.
workers, psychosocial health professionals, developmental spe- These difficult conversations can be facilitated through a
cialists, and spiritual supports. They often rely on a hospice or shared understanding about how a child’s health and function
community-based palliative care program, a home nursing agency, have changed over time. When given time to reflect, families can
or their primary care physician to help coordinate care. The care of often recognize these changes. Providing anticipatory care about
children with serious illness may additionally involve representa- feeding, breathing, or mobility changes related to the progression
tives from school, camp, child life services, massage, hippotherapy, of illness can provide a more concrete, tangible basis for action in
developmental therapists (eg, art, occupational, speech, physical), families related to their child’s increased fragility. Uncomfortable
and others. Strong communication and reliable continuity within as it is, anticipating death and addressing it clearly and frankly
the team are essential. Ultimately, the goal of primary and second- allows some children and families to make choices about how to
ary palliative care, as well as hospice care, is to promote the sense spend the time remaining.

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 Chapter 22: Pediatric Palliative Care: Principles and Practice 139

Recent research shows that health professionals can help par- The effects of a serious illness are much larger and more complex
ents feel like “good parents” to their seriously ill children by let- than a physical sensation, however. Suffering was best described by
ting families know that all that can be done for their child is being Eric J. Cassel, MD, as “the state of severe distress associated with
done and not giving families the sense that health professionals are events that threaten the intactness of a person.” This distress comes
“quitting”; respecting parental decisions; providing comfort to the from a threat to any of the multiple aspects of personhood—”the
child and family; demonstrating knowledge of the particular needs lived past, the family’s lived past, culture and society, roles, the
of the individual child/family and that the child is uniquely special; instrumental dimension, associations and relationships, the body,
coordinating care and providing honest, factual information; inquir- the unconscious mind, the political being, the secret life, the per-
ing about spiritual needs; and telling parents that they are seen as ceived future, and the transcendent dimension.” Some authorities
acting as good parents to their child. Table 22.1 outlines a stepwise recommend using terms other than “suffering” to avoid describing
approach to communicating with children and families after prog- persons as victims or with other emotional terms that are sugges-
nostic information has been shared to introduce palliative concepts tive of helplessness. Figure 22.2 shows a sample of the myriad con-
and better understand how to support an individual child and family cerning symptoms that can be interrelated for a child with serious
in these difficult circumstances. Health professionals who support illness, all of which must be considered when thinking about dis-
families in these ways have profound effects on meaning making tress in seriously ill children. In caring for patients with serious ill-
and bereavement after a child’s death. Even when health profession- ness, understanding all aspects of suffering is critical to effective
als are unsure what to say, acknowledging parents as “good parents” care, and listening and reflection are prerequisites to the necessary
can show humility, build trusting relationships, and provide mean- healing presence required to care for dying children and their fami-
ing during an incredibly difficult time. lies. Pain and symptom management are foundational, but the relief
of suffering requires helping a child and family struggle with issues
Suffering and the Power of Hope of meaning and transcendence.
Initially, the topics of suffering and hope can seem to be untechnical Hope evolves from expectations for or belief in a worthy future.
and unprofessional to the practice of medicine. However, it is hard to It carries a sense of trust and resilience. Serious illness can make
imagine concepts more central to the art of healing and medicine’s it difficult to know what to hope for, trust in, or rely on. Research
true purpose. Serious illness and its treatment can cause physical dis- increasingly shows, however, that aspects of hope and resiliency are
tress, and research has shown that dying children have many phys- sustained throughout the disappointments and upheaval associated
ical symptoms, such as pain, fatigue, and dyspnea (see Chapter 14). with serious illness and even death. This process of evolving goals

Table 22.1. Communicating With Children and Families About Palliative Care
Steps and Goals Sample Statements
Step 1: Open I would like to talk about what is ahead with your child’s health and do some thinking and planning in
Goal: Setting a respectful tone of shared decision- advance. Talking about it now allows us to think things through without the pressures that come when
making and asking permission your child is acutely ill and immediate decisions are needed. Would that be alright with you?
Step 2: Assess What is your understanding of where your child is now with this illness? If your child’s health situa-
Goal: Understanding the family’s prognostic aware- tion worsens, what are your most important goals for your child and your family? What are your biggest
ness, hopes, fears, and worries fears and worries about the future with this illness?
Step 3: Align I see these same issues, and I am also worried that your child’s health is getting worse. I wish that
Goal: Sharing emotional understanding of worries and things were different.
wishes with the family
Step 4: Disclose How much have you and your child talked about, that is, how aware is your child of, these issues?
Goal: Understanding how information is shared within How much information do you want, and how much information can your child handle about what is
and with the family likely ahead with this illness?
Step 5: Explore If your child becomes sicker, how much do you think it makes sense to have the child undergo differ-
Goal: Getting to a shared understanding of trade-offs ent treatments for the possibility of gaining more time? Are there specific conditions or states that you
and limits of interventions as perceived by the family would not find acceptable for your child to be in?
Step 6: Close in alliance It sounds like ______is very important to you. Given what is important to you and what we know
Goal: Providing a summary, sharing recommendations about your child’s illness at this stage, I recommend ___________.
for next steps, and expressing non-abandonment Let’s meet again tomorrow to talk some more. I want to make sure I am answering all your questions as
best as I can.
Adapted from Goldstein RD. Eliciting and communicating goals of care for seriously ill patients. EQIPP Course under development, American Academy of Pediatrics, 2018.

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 140 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

to address a family’s palliative care concerns, these practices can help

Sleep pediatricians feel more useful and create deeply meaningful experi-
ences with their seriously ill patients and families.
Another barrier has resulted from the payment model for pedi-
Depression Pain atric palliative care, which historically has been based on Medicare
hospice benefits that require patients to have a prognosis of 6 months
or less and to forego curative care. Understandably, parents find it
quite hard to give up curative efforts for their children. Although
some states offer palliative care programs to remedy this, the Patient
Protection and Affordable Care Act of 2010 removed the prohibi-
Anxiety Mood tion against curative treatment as a hospice benefit by or on behalf
of a Medicaid or Children’s Health Insurance Program eligible child.
Despite these changes, eligibility still requires a physician to certify
the child as likely to die in the next 6 months. It is not uncommon
for children to live beyond their expected prognosis, and in these
Dyspnea Nausea instances children can be re-enrolled to continue receiving hospice
benefits. States are still not required to provide pediatric specific hos-
pice services, however, although doing so is increasingly the norm.
Figure 22.2. Interrelated symptoms of distress in children with serious illness.
Advocacy for increased access to palliative and hospice services for
all children with serious illness is crucial to expanding expert palli-
of care or changing hopes (eg, from hope for cure to hope for time ative supports for these children and their families.
with family or a trip to the beach) occurs naturally, and families
often need clear communication about what to expect to engage in A Child’s Understanding of Death
this process of “regoaling.” In this way, hope can be sustained when Although death itself is an unknown experience, parents want to
a meaningful way forward is elucidated even in the face of a dismal know what their children can understand during the dying process.
diagnosis or the loss of a child. Caregivers, too, wonder what a child knows as they try to anticipate
needs or reckon with enigmatic statements from them. Most would
Barriers to Palliative Care agree that a child’s distress resulting from avoidance of discussions
Parenting a child with serious illness can be frightening, confus- of their fears about their condition and death is especially tragic and
ing, frustrating, and exhausting. A decision to involve palliative care counterproductive.
professionals is in no sense a decision by parents or other health Health professionals should, when possible, communicate with
professionals to stop working tirelessly for the child. It is crucial children about what is happening to their bodies, while respecting
to introduce palliative care with sensitivity and thoughtfulness. the cultural and personal preferences of each family. A developmen-
Often palliative care is inaccurately viewed as an act of abandon- tal understanding of children’s concepts of illness and death can help
ment or “giving up.” Research shows that often health profession- frame these discussions and serve to advise parents in their own con-
als are reluctant to engage palliative supports for families because versations with their children. The understanding of a child’s concept
of perceptions of the family “not being ready” to acknowledge or of death can be extrapolated from Piaget’s work in cognitive devel-
accept a child’s poor prognosis. This is counter to the growing body opment and is based on 4 stages: sensorimotor, preoperational, con-
of research demonstrating that parents generally accept palliative crete operations, and formal operations. These developmental stages
care and believe that it is offered late. This delayed involvement of of understanding of death along with their typical chronologic ages,
palliative care specialists results in not only worse symptom manage- along with sample language to use when talking to children about
ment and quality of life for ill patients, but may also result in shorter illness and death, are shown in Table 22.2. Having limited cognitive
survival. Additionally, children who receive palliative care supports abilities will affect a child’s understanding of these issues. In con-
take fewer medications, undergo fewer invasive interventions, and trast, illness profoundly changes development, and it is not uncom-
are less likely to die in an intensive care unit compared with those mon for seriously ill children to possess a precocious and advanced
who do not receive such care. understanding of certain things, such as the details of their illness
One of the greatest obstacles to palliative care and hospice for or the way to address their symptoms, while simultaneously being
children has historically been lack of trained specialists. This is less mature in other developmental areas.
becoming less of a barrier as more hospitals and communities have Parents can struggle with whether they should talk with their
access to pediatric palliative care specialists and with broader edu- child about the child’s imminent death. Although research supports a
cation, advocacy, and knowledge about the field. Workforce short- bias toward speaking frankly to dying children, each individual situa-
ages are also eased by training in primary palliative care skills for tion should be considered unique and based on the child’s age, cogni-
primary care pediatricians. Despite concerns about the time needed tive development, disease, timeline of disease, parental psychological

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 Chapter 22: Pediatric Palliative Care: Principles and Practice 141

Table 22.2. Ages and Developmental Understanding of Death

Example of Age-Appropriate
Age Group Conceptualization Interventions Language
0–2 years No cognitive understanding of death Maintain routines “Mommy did not want to leave”
Concern for experience of death as Avoid separation from significant others
abandonment and/or separation Presence is more important than words
Understand events in terms of direct
experience
Identify when adults are in distress
2–6 years Believe death is temporary and reversible Use concrete, clear, and simple language “Bobby is dead; that means he won’t
Often blame themselves for the death Avoid euphemisms; use “death,”“dying,” and “died” ever live with us again, but we will
Magical thinking when possible always remember him.”
Clarify question the child is asking
Use repetition and consistent language
Reflect on past experiences with the death and dying
process; pet, grandparent, others
Play-based activities to assist in expressing feelings
6–8 years Death is final and irreversible Clear, realistic information “When someone dies, that means their
Fear death Ongoing discussions about death body is no longer working. Their heart
Concerned for others’ safety Discuss physical details of death stops beating, they no longer need to
eat or sleep, their brain stops thinking,
Believe death is not universal Establish safe space at school where the child can seek
and they no longer feel any pain. They
guidance when necessary
don’t need their body any longer. That
means we will never see them again as
we could before.”
8–12 years Understand death is final, irreversible, Open and honest discussion about one’s own “I, too, am very sad that Katie is sick.
and universal emotions Grandma is going to come take care of
Comprehend biological aspects of death Assure children that they will be informed about you while I go to the hospital with Katie,
Curiosity about death and dying major events of concern but we will have Grandma bring you to
Allow children the choice to participate in activities the hospital if something happens.”
Intellectualize death
surrounding the death, including funeral services and
hospital visits
12–18 years Adult understanding that death is inevitable Support independence and access to peers “If you want to talk, I am here to listen.
Ability to think abstractly and Monitor social media If you do not want to, then I am here if
philosophically Maintain familiar routines or when you do. I can’t imagine what
Curiosity surrounding existential implica- you are feeling right now, and I want
tions of death you know I am here to support you.”

Adapted with permission from Himelstein BP, Hilden JM, Boldt AM, Weissman D. Pediatric palliative care. N Engl J Med. 2004;350(17):1752–1762.

state, and family culture. In studies of parental disclosure to children Pediatric Primary Care at the End
with impending cancer death, parents who talked with their child of Life: Normal and Extraordinary
about their death had no regrets, but among those who did not speak
frankly about imminent death, more than 25% regretted not having Primary care pediatricians can play an important role in the care of
done so. During these difficult times, primary care pediatricians can children with serious illness. The presence of a constant, continuous
provide essential contributions to the care of seriously ill children physician who understands the child, siblings, and family holistically
and their families by serving as a resource for these conversations in the community can contribute profoundly to care as part of the
and providing guidance to families. Collaboration with pediatric medical home. Because caring for these children is a rare experience
palliative care specialists can support pediatricians’ involvement in general practice, the complexities of care can be daunting. Primary
through education and an added layer of support. care pediatricians can benefit from creating a network with others

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 142 PART 2: PRINCIPLES OF HEALTH CARE AND PEDIATRIC MANAGEMENT

involved in the care of the child (eg, home nurses, social workers, the Physician Orders for Life-Sustaining Treatment (POLST) form.
teachers, pediatric subspecialists, hospice) to support their direct care The POLST form differs slightly by US state and serves as a home-
of seriously ill children and families throughout illness and end of life. based do-not-resuscitate order. For instance, emergency medical
For seriously ill children and their families, general pediatric care technicians in the field must resuscitate a child found in cardiac or
from their primary care pediatrician also can be an important affirma- respiratory failure unless orders exist to refrain. After a family has
tion of the normal and can contribute to quality of life. For the child, come to the difficult decision to limit interventions, it is important
routine health care visits, vaccinations, and developmental assess- to advocate for these wishes in all settings and to complete docu-
ments are important, because the rationale for this care is not their ments to communicate these decisions. Asking parents to sign these
disease but the positive and ordinary characteristics of childhood. The forms can be heartbreaking, but framing their completion as a part
focus on development, education and learning, social engagement, of documenting a conversation can be helpful. Reaching out to local
play, and involvement with family and community affirms the whole palliative care specialists to talk through the documents and com-
child and the importance of the child’s life. The primary care pedia- munication strategies beforehand is equally beneficial.
trician is often seen as a trusted advisor who understands the fam-
ily and child in this normal context and thus “knows” them best. The Loss and After
pediatrician is the child’s doctor without qualification. The loss of a child or sibling changes life forever. It begins a pro-
A trusted primary care pediatrician also has a role in complex cess of bereavement, the psychological and spiritual accommodation
disease management. The primary care pediatrician is in the best to death on the part of the child’s family, and grief, the emotional
position to assess the family’s level of understanding and address response caused by the loss. Grief can cause distress and physical and
any gaps in that understanding. Importantly, the pediatrician can emotional pain, but, except in cases of prolongation, it is a normal
help families understand complex medical information, terminol- adaptive human response, not a disease. Anticipatory grief begins
ogy (ie, medical jargon), and other specific medical details. They can with the awareness of impending loss or death in parents and chil-
also help the medical team better understand the patient and family dren with sufficient awareness and cognitive development. Palliative
perspective. The primary care pediatrician can ease the adjustment care attends to the grief reaction before and after death. Assessing
between home and hospital and help ensure more seamless transi- the coping resources and vulnerabilities of the affected family sys-
tions in either direction. Relevant involvement of the pediatrician in tem before death occurs is central to the palliative care approach.
crucial developments requires timely sharing of information among Generally, parental grief is more intense and sustained than other
all members of the team, the generalist, and the specialist. Ideally, the types of grief. Parents may never completely accept the loss of their
primary care pediatrician should participate in hospital-based fam- child. Research suggests that parents who share their problems with
ily and provider team meetings. This is especially important during others during their child’s illness, who have had access to psychological
meetings in which goals of care and medical decision making are support during the last month of their child’s life, and who have had
the focus. Fostering collaboration between the primary care pedia- closure sessions with the attending staff are more likely to resolve
trician and the palliative care specialist is essential. Currently, this their grief. Surviving siblings also grieve. The American Academy
can more readily be accomplished through advances in technology, of Pediatrics recommends that primary care pediatricians reach out
such as telehealth and videoconferencing. to children and families at the time of loss to evaluate their bereave-
When a child is facing the end of life at home, the primary care ment and to understand the personal meaning of their loss and their
pediatrician has additional responsibilities to facilitate care for the process of mourning. Those involved in the care of children and fam-
child, siblings, and family. The primary care pediatrician can work ilies appreciate how meaningful simple things, such as condolence
with the hospice team to continuously fine-tune approaches to pain letters and attendance at funerals or memorial services, are to fam-
and symptoms. Primary care pediatricians can also play a central ilies. Notes remembering the anniversary of the deceased child’s
role in delineating resuscitation orders. Research indicates that par- birthday or helping the family think about developing the child’s
ents consider end-of-life decisions to be the most difficult treatment legacy or a remembrance also contribute to improved bereavement
decisions they face. Thoughtful conversations with a trusted pri- and meaning making after a child’s death. Helping bereaved chil-
mary care pediatrician who has a broad perspective on the medi- dren safely remember their deceased siblings and appreciating their
cal details as well as the family identity and priorities is invaluable. successes as they integrate the loss can be a powerful part of a health
A powerful resource to guide these discussions is the document My care visit and the long-standing caring relationship between primary
Wishes, which offers open-ended prompts with spaces to draw pic- care pediatricians and families. Often primary care pediatricians
tures for young school-age children to better understand their wor- can be in contact with the school and other community organiza-
ries and hopes. Voicing My Choices is another excellent resource for tions that can support bereaved siblings. When necessary, referrals
adolescents and young adults; this resource captures not only who to skilled mental health professionals should be offered, including
the patient is as a person but also the patient’s end-of-life wishes. referrals to bereavement specialists.
Five Wishes can be used when working with adults and is a legally Many resources are available to support primary care pedia-
recognized advance care planning tool when completed correctly. tricians caring for seriously ill children and their families. Helpful
Another important form to consider completing with families is resources include the Palliative Care: Conversations Matter campaign

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 Chapter 22: Pediatric Palliative Care: Principles and Practice 143

(www.ninr.nih.gov/newsandinformation/conversationsmatter/about- Glare P, Virik K, Jones M, et al. A systematic review of physicians’ survival pre-
conversations-matter) and Together for Short Lives (www.together- dictions in terminally ill cancer patients. BMJ. 2003;327(7408):195–198 PMID:
forshortlives.org.uk/about-us/). For families, the Courageous Parents 12881260 https://doi.org/10.1136/bmj.327.7408.195

Network (https://courageousparentsnetwork.org/) is an excellent Goldstein R, Rimer KP. Parents’ views of their child’s end-of-life care: subanal-
resource that describes many aspects of caring for a seriously ill ysis of primary care involvement. J Palliat Med. 2013;16(2):198–202 PMID:
23098631 https://doi.org/10.1089/jpm.2012.0269
child. Most importantly, pediatric palliative care specialists locally
and nationally are available to support the essential work of primary Himelstein BP, Hilden JM, Boldt AM, Weissman D. Pediatric palliative care.
N Engl J Med. 2004;350(17):1752–1762 PMID: 15103002 https://doi.org/10.1056/
care pediatricians in their care of seriously ill patients and families.
NEJMra030334
Working together, the pediatrician and palliative care specialists can
Hinds PS, Oakes LL, Hicks J, et al. “Trying to be a good parent” as defined by
address the needs of these children and their families.
interviews with parents who made phase I, terminal care, and resuscitation deci-
sions for their children. J Clin Oncol. 2009;27(35):5979–5985 PMID: 19805693
https://doi.org/10.1200/JCO.2008.20.0204

CASE RESOLUTION Knapp C, Thompson L. Factors associated with perceived barriers

to pediatric palliative care: a survey of pediatricians in Florida and California.
The hospital-based palliative care team arranged to meet with Jason’s parents.
Palliat Med. 2012;26(3):268–274 PMID: 21680751 https://doi.org/10.1177/
During their conversation, they helped the parents articulate what gave Jason’s
0269216311409085
life meaning, what happiness was for him, and what their hopes were. Jason’s
parents decided they would still opt for ventilatory assistance should he become Kreicbergs UC, Lannen P, Onelov E, Wolfe J. Parental grief after losing a child
compromised by an acute, likely reversible illness but could not imagine cardiac to cancer: impact of professional and social support on long-term outcomes.
resuscitation being consistent with their goals. Therefore, a limitation was placed J Clin Oncol. 2007;25(22):3307–3312 PMID: 17664479 https://doi.org/10.1200/
on cardiac resuscitation, and a POLST form was completed. With the parents’ per- JCO.2006.10.0743
mission, the palliative care team spoke with Jason’s primary care pediatrician and Liben S, Papadatou D, Wolfe J. Paediatric palliative care: challenges and emerging
home nursing team.
ideas. Lancet. 2008;371(9615):852–864 PMID: 17707080 https://doi.org/10.1016/
The palliative care team continues to meet with Jason and his family for each
S0140-6736(07)61203-3
hospital admission and connects regularly with Jason’s primary care pediatrician
for ongoing care transitions and health care needs. Mack JW, Wolfe J, Cook EF, Grier HE, Cleary PD, Weeks JC. Hope and prognos-
tic disclosure. J Clin Oncol. 2007;25(35):5636–5642 PMID: 18065734 https://
doi.org/10.1200/JCO.2007.12.6110
Murphy SL, Mathews TJ, Martin JA, Minkovitz CS, Strobino DM. Annual sum-
Selected References mary of vital statistics: 2013-2014. Pediatrics. 2017;139(6):e20163239 PMID:
American Academy of Pediatrics Section on Hospice and Palliative Medicine 28814547 https://doi.org/10.1542/peds.2016-3239
and Committee on Hospital Care. Pediatric palliative care and hospice care com- Schonfeld DJ, Demaria T; American Academy of Pediatrics Committee on
mitments, guidelines, and recommendations. Pediatrics. 2013;132(5):966–972 Psychosocial Aspects of Child and Family Health, Disaster Preparedness
PMID: 28448256 https://doi.org/10.1542/peds.2013-2731 Advisory Council. Supporting the grieving child and family. Pediatrics.
Bogetz JF, Hauer J. Certainty of decisions: a process-based model for deci- 2016;138(3):e20162147 PMID: 27573086 https://doi.org/10.1542/
sion making for children with severe neurological impairment. Clin peds.2016-2147
Pediatr (Phila). 2018;57(10):1227–1231 PMID: 29113499 https://doi. Voicing My Choices. Five Wishes. https://fivewishes.org/. Accessed July 5, 2019
org/10.1177/0009922817740668
Wolfe J, Grier HE, Klar N, et al. Symptoms and suffering at the end of life in
Feudtner C, Feinstein JA, Satchell M, Zhao H, Kang TI. Shifting place of death children with cancer. N Engl J Med. 2000;342(5):326–333 PMID: 10655532
among children with complex chronic conditions in the United States, 1989-2003. https://doi.org/10.1056/NEJM200002033420506
JAMA. 2007;297(24):2725–2732 PMID: 17595273 https://doi.org/10.1001/
jama.297.24.2725

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 PART 3

Health Maintenance
and Anticipatory
Guidance
23. Neonatal Examination and Nursery Visit.......................147
24. Maternal Perinatal Mood and Anxiety Disorders:
The Role of the Pediatrician.............................................155
25. Newborn Screening..........................................................161
26. Caring for Twins and Higher-Order Multiples...............167
27. Male Circumcision............................................................173
28. Nutritional Needs..............................................................179
29. Breastfeeding.....................................................................187
30. Sleep: Normal Patterns and Common Disorders............193
31. Oral Health and Dental Disorders...................................201
32. Normal Development and Developmental
Surveillance, Screening, and Evaluation.........................211
33. Speech and Language Development:
Normal Patterns and Common Disorders......................221
34. Literacy Promotion in Pediatric Practice........................231
35. Gifted Children.................................................................235
36. Children and School: A Primer for the Practitioner.......241
37. Immunizations..................................................................253
38. Health Maintenance in Older Children
and Adolescents................................................................259
39. Health Care for International Adoptees..........................271
40. Health Care Needs of Children in Foster Care................279
41. Working With Immigrant Children and
Their Families....................................................................285
(continued)

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 42. Well-Child Care for Children With Trisomy 21
(Down Syndrome)............................................................291
43. Well-Child Care for Preterm Infants...............................299
44. Care of Children With Special Health Care Needs............307
45. Injury Prevention..............................................................313
46. Fostering Self-esteem.......................................................319
47. Sibling Rivalry...................................................................325
48. Toilet Training...................................................................329
49. Crying and Colic...............................................................335
50. Discipline...........................................................................339
51. Temper Tantrums.............................................................345
52. Breath-Holding Spells.......................................................351
53. Fears, Phobias, and Anxiety.............................................355
54. Thumb-sucking and Other Habits...................................361
55. Enuresis.............................................................................367
56. Encopresis.........................................................................373

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 CHAPTER 23

Neonatal Examination
and Nursery Visit
Niloufar Tehrani, MD

CASE STUDY
You are performing an examination on a 16-hour-old Questions
newborn who was born at 39 weeks’ gestation to a 1. What aspects of the maternal and birth history are
28-year-old, healthy, primigravida via normal sponta- important to review before performing the neonatal
neous vaginal delivery. No complications occurred at physical examination?
delivery, and the Apgar score was 8 at 1 minute and 9 at 2. What other history is important for a complete new-
5 minutes. The newborn weighed 3,200 g (7 lb 1 oz) and born assessment?
was 50 cm (19.7 in) long at birth, with a head circum- 3. What aspects of the physical examination of new-
ference of 34 cm (13.4 in). The mother received prenatal borns are essential to explain to parents?
care beginning at 10 weeks of gestation; had no prenatal 4. What physical findings mandate a more extensive
problems, including infections; and used no drugs, alco- workup prior to discharge?
hol, or tobacco during the pregnancy. Her blood type is 5. What is the routine hospital course for a normal
O Rh-positive. She is negative for hepatitis B surface newborn?
antigen and group B streptococcus, and she is nonre- 6. What are important points to cover with parents
active for HIV, syphilis, chlamydia, and gonorrhea. The at the time of discharge for a healthy, full term
father is also healthy. newborn?
On physical examination, the newborn is appropri- 7. What laboratory studies, if any, should be per-
ate size for gestational age, with length and head circum- formed prior to discharge?
ference in the 50th percentile. Aside from small bilateral
subconjunctival hemorrhages, the rest of the physical
examination is entirely normal.

The initial newborn physical examination is an important first speak with the parent or parents at a prenatal visit before meeting
encounter with the pediatrician, the newborn, and the newborn’s them in the hospital. The prenatal meeting provides a chance for
parents all establishing relationships with each other. The key pur- parents to interview the physician, as well as the rest of the office
pose of this examination is to assess the status of the newborn and staff, about general policies and procedures for well-child appoint-
detect any underlying medical problems. Relaying this informa- ments, sick visits, and contacting the physician after hours. It also
tion to the parent or parents is essential and answers the question is a time to discuss what will take place at the hospital and explain
foremost in a parent’s mind: “Is my baby ‘normal’?” By perform- the role of allied health professionals (eg, lactation specialists) in
ing a physical examination in the parent’s or parents’ presence dur- the overall care of the mother and newborn. For pediatricians and
ing the first 24 hours after the newborn’s birth, pediatricians can other health professionals, the prenatal visit is a time to gather
play a major role in allaying parental anxiety. The pediatrician’s role vital medical information about the current pregnancy, identify-
includes identifying medical problems or high-risk conditions in the ing any high-risk conditions, and to inquire about any problems
prenatal screening (including ultrasonography), neonatal examina- with previous deliveries. The pediatrician should also review any
tion, and history. Evaluation and treatment, if necessary, can be pertinent family history. In addition, the pediatrician needs to
initiated before discharge from the nursery. note specific needs of the parent or parents, which may include
alternative medicines, cultural rituals, or ceremonies surround-
Pediatric Prenatal Visit ing the birth of a newborn, such as circumcision. Whether the
The prenatal visit during the third trimester is recommended for newborn will be born at home, in a birthing center, or in a typcial
all expectant families. This is the ideal time to establish the med- hospital setting, arrangements should be made to accommodate
ical home and provides the pediatrician with the opportunity to the wishes of the family.

147

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 148 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

The prenatal visit also allows physicians to assess any psy-

Box 23.1. What to Ask
chosocial issues that may negatively influence initial mother-
newborn bonding, such as maternal drug use, no partner involve- Neonatal Examination
ment, absence of a supportive social network, or lack of housing. ww Did the mother receive prenatal care? If so, since what month of gestation?
Any of these issues may necessitate intervention by social services. ww Were all prenatal studies normal?
The social environment that will surround the newborn at home will ww Does either parent have a history of sexually transmitted infections, such as
syphilis, gonorrhea, or herpes?
have a great effect on the child’s future. In addition, this visit provides ww Does either parent have a history of alcohol, tobacco, or illicit drug use?
an opportunity to emphasize the importance of breastfeeding and ww Did the mother take any prescribed or over-the-counter medications routinely
educate parents on its many benefits. Anticipatory guidance issues during pregnancy?
also can be discussed, including positioning the newborn on the ww Did the mother have any complications, such as bleeding or decreased fetal
back to sleep, safe bedding, and child passenger safety. movement, during pregnancy?
ww Were there any indications of a complicated delivery, including administration
of maternal medications?
Neonatal Nursery Visit ww Is there a family history of congenital anomalies or diseases?
At birth, the newborn undergoes a screening examination and risk ww Is the mother planning to breastfeed?
ww Does the neonate have any siblings? If so, what are their ages?
assessment by the nursery personnel when the newborn is stable
ww Is anyone available to help with the new baby or siblings?
after any needed resuscitation. In the absence of abnormalities,
the pediatrician is notified of the birth. The American Academy of
Pediatrics (AAP) and the American College of Obstetricians and
Gynecologists recommend that the pediatrician should evaluate the the occurrence of any unusual circumstances surrounding the deliv-
newborn within 24 hours after birth to identify medical conditions ery should be explained to the parent or parents at the time of the
that need to be addressed and determine the course of newborn examination. For example, the need for a vacuum-assisted or emer-
care. In the delivery room, the newborn is dried, Apgar scores are gent cesarean section may not have been understood. It is important
assigned, and the newborn is placed skin to skin on the mother’s to be prepared to discuss the medical implications of such events.
chest, with a blanket placed over the mother and newborn, to facil- Unexpected events related to the delivery, such as an emergency
itate bonding and early breastfeeding. cesarean section, may be viewed as traumatic by the mother and
If perinatal or delivery complications occur, such as maternal predispose her to perinatal mood and anxiety disorders.
fever, maternal medication during labor and delivery, meconium
Physical Examination
aspiration, asphyxia, preterm birth, obvious newborn malforma-
tions, or distress, the newborn should be supported as necessary Every attempt should be made to perform the neonatal physical
examination at the mother’s bedside. This gives the physician the
and kept on a radiant warmer for further assessment. The physi-
opportunity to meet with the parent or parents and answer all their
cian should be promptly notified to perform the neonatal physi-
initial questions immediately. In certain circumstances, such as pre-
cal examination, evaluating any problem and initiating treatment.
term birth or in cases in which the mother is being treated for a med-
ical complication, it may be impossible to perform an initial bedside
Evaluation
examination. Regardless of the timing of the examination, the phy-
Perinatal History sician or other health professional should discuss the results with
The perinatal history is the beginning of the newborn’s medical his- the parent or parents after the examination has been completed.
tory and is significant in the newborn evaluation. To obtain all the The neonatal examination is performed with the newborn com-
pertinent history, it may be necessary to review the maternal med- pletely unclothed and the newborn’s body temperature maintained.
ical record. Results of maternal laboratory tests, such as blood type; The newborn is assessed for evidence of birth trauma and congenital
rapid plasma reagin status; purified protein derivative; HIV and hep- malformations, and organ systems are assessed for normal physiol-
atitis B status; chlamydial, gonococcal, and group B streptococcus ogy. The newborn’s birth weight, length, and head circumference are
cultures; and toxicology screen results should be noted (Box 23.1). plotted. The physician should bear in mind that these measurements
The pediatrician also should obtain triple screen, prenatal ultraso- are often made in the delivery room and may be subject to error. The
nography, and amniocentesis results, if available. Maternal history, diagnosis of small for gestational age (SGA; weight <10th percentile)
including medical conditions and medications used during preg- or large for gestational age (LGA; weight >90th percentile) can pre-
nancy or delivery, prior deliveries, type of delivery, delivery room dispose a newborn to several medical problems. Temperature, respi-
events, and Apgar scores should be reviewed before performing the ratory rate, and heart rate are reviewed via the nursery record. In a
neonatal physical examination. Given the Zika virus outbreak in the newborn with suspected cardiac or renal anomalies, blood pressure
Americas in 2015 to 2016, it may be necessary to elicit travel his- is also measured. Feeding, voiding, and stooling patterns are also
tory and perform appropriate screening in the mother and possibly available in the nursery record and should be reviewed.
the newborn. Obtaining this information helps focus the examina- The newborn evaluation includes an expanded Ballard score,
tion on findings suggested by previous medical history. Additionally, which includes parameters for neuromuscular and physical maturity

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 Chapter 23: Neonatal Examination and Nursery Visit 149

to accurately estimate gestational age. A late preterm or near-term Ears

newborn (ie, 35–37 weeks of gestation) frequently does not have the The placement, size, and shape of the ear pinnae are noted as well,
physiologic maturity to feed vigorously and maintain body weight, along with any preauricular or postauricular pits or appendages.
glucose, or body temperature. These newborns are at high risk for The presence of a significant auricular abnormality may correlate
readmission for treatment of jaundice, dehydration, and hypogly- with hearing loss or be suggestive of a genetic syndrome and neces-
cemia. They should be closely followed. sitates further evaluation.
The newborn’s overall appearance is noted, particularly for the
presence of any dysmorphic features. The physician should determine Nose
whether the newborn looks normal or has any abnormal facial fea- The nose is checked carefully for patency of the nares. This is easily
tures, such as low-set ears and widely spaced eyes (see Chapter 84). accomplished by occluding airflow from 1 nostril and observing air-
flow from the other. Choanal atresia is an important condition to rule
Skin
out, because newborns are obligate nose breathers until 3 months of
In the newborn, a thick layer of white vernix caseosa composed of age, and choanal atresia may result in respiratory distress. A nasal
sebaceous secretions and epidermal cells is frequently seen. The fracture also can occur during the delivery process. Such fracture
presence and location of any rashes or birthmarks should be care- presents as an asymmetric nose. If the physician presses on the nasal
fully described and pointed out to the parent or parents. The partic- tip and the nares are symmetric, the diagnosis is a deformity that will
ular location of the lesion as well as the pathology of the epidermal resolve. If the nose falls to 1 side, however, the presence of a nasal
lesion may aid in specific diagnosis of it. Skin lesions resulting from septum fracture is probable, and prompt evaluation by an otolaryn-
birth trauma are documented and managed as necessary. Bruising gologist is warranted.
should be differentiated from the benign dermal melanocytosis that
often occurs in the sacral area. Skin color is noted. Cyanosis may Mouth
be indicative of congenital heart disease, the presence of jaundice The oropharynx is examined closely for any defects in the hard or
is suggestive of hyperbilirubinemia, and plethora may be a sign of soft palate. A bifid uvula may be indicative of a submucosal defect
polycythemia. of the soft palate that may be difficult to appreciate without palpa-
tion (see Chapter 85). Common normal findings in the oropharynx,
Head
including Epstein pearls located at the midline on the hard palate
Head size and shape are evaluated, including the size of the fontanels and epithelial cysts along the gum line (ie, Bohn nodules), are also
and position of the sutures. The head may be significantly molded noted. Loose natal teeth should be removed to prevent the possibil-
into a cone deformity secondary to pelvic pressure at the time of ity of aspiration (see Chapter 31). The tongue is evaluated for mac-
delivery. This deformity resolves within days after birth, which may roglossia or a tight lingual frenulum (ie, ankyloglossia), which may
result in a significant change in the head circumference. A cepha- interfere with effective breastfeeding.
lohematoma, which is a subperiosteal bleed that does not cross the
suture line, appears as a unilateral or bilateral discrete lump on the Neck
side of the head. This finding may predispose the newborn to hyper- The neck is palpated for sternocleidomastoid hematomas, masses,
bilirubinemia, and it may take up to 2 months for the condition to torticollis, and skin redundancy. The clavicles are evaluated for frac-
resolve. A cephalohematoma should be differentiated from a caput tures, which are more common in newborns who are LGA or in the
succedaneum, which is scalp edema that crosses the midline and may setting of a delivery complicated by shoulder dystocia.
be ballotable. Edema usually resolves rapidly. Skull fractures may
Chest
occur with birth trauma and may present as a step-off or crepitus
on palpation of the skull. Such fractures are associated with cepha- The chest is inspected for respiratory effort and the lungs auscul-
lohematoma, but treatment is rarely required. Any unusual findings tated to ensure normal breath sounds throughout. The quality of
about the head may be a source of parental concern. the newborn’s cry is assessed as well. Laryngeal webs or a paralyzed
vocal cord may present in the newborn. Chest wall deformities,
Eyes
such as pectus excavatum, are noted. If an absent rib is suspected,
The eyes are evaluated for subconjunctival hemorrhages, colobo- a chest radiograph should be obtained. The presence of breast buds
mas, pupillary reaction, extraocular movements, and presence of red is normal; however, supernumerary nipples are minor malforma-
reflexes. If it is not possible to elicit a red reflex, an ophthalmologic tions, although no treatment is indicated. Widely spaced nipples are
evaluation is essential. Absence of this reflex is suggestive of condi- a common minor malformation in patients with Turner syndrome.
tions such as congenital cataracts or retinoblastomas. The color of
the sclera and the spacing and symmetry of the eyes are also eval- Heart
uated, because abnormalities may be suggestive of an underlying The heart is auscultated to ensure that it is in the proper position
syndrome. For example, upslanting palpebral fissures can be seen on the left side of the chest. Femoral and brachial pulses are pal-
in the setting of trisomy 21, and blue sclerae may be indicative of pated. The physician should listen for and document the presence
osteogenesis imperfecta. of cardiac murmur. The presence of a murmur does not always

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 150 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

indicate complex congenital heart disease. In the first 24 hours, congenital anomalies is highly suspicious for a pathologic cause.
a murmur may be a closing ductus; additionally, newborns can A consultation with specialists in pediatric cardiology should be
have functional murmurs. Peripheral pulmonic stenosis is a com- sought and an echocardiogram obtained.
mon benign heart murmur in the newborn that is characterized
by transmission to the right side and back. The AAP, American Abdomen
Heart Association, and American College of Cardiology recom- It is easiest to palpate the abdomen before feeds. The abdomen is
mend universal screening for critical congenital heart disease by assessed for any masses or organomegaly (eg, polycystic kidneys,
pulse oximetry reading for which the AAP algorithm is most com- hepatosplenomegaly, adrenal hemorrhage) that warrant further
monly used (Figure 23.1). However, every murmur should be eval- investigation. Bimanual palpation may be helpful to identify masses.
uated on an individual basis. If further assessment is indicated, The umbilicus is examined to identify 3 vessels as well as the qual-
4 extremity blood pressures, an electrocardiogram, and a chest ity of the cord. A small, atretic cord can be the cause of low weight
radiograph should be obtained. The presence of abnormal findings in the newborn. Erythema and swelling of the skin around the cord
or a murmur associated with cyanosis, tachypnea, poor feeding, or may be indicative of omphalitis, which is a serious infection.

Measurement #1
Pulse Ox on Right Hand (RH) and One Foot After 24 Hours of Age

RETEST
FAIL PASS
Pulse ox of 90%–94% in BOTH
Pulse ox of 89% or less in Pulse ox of 95% or more in RH or
the RH and foot OR a
either the RH or foot foot AND difference of 3% or less
difference of 4% or more
Action: Do not repeat for between the 2
between the RH and foot
screening, refer for Action: Do not repeat for screening,
Action: Repeat pulse ox
immediate assessment provide normal newborn care
measurements in 1 hour

Measurement #2
Pulse Ox on (RH) and 1 Foot 1 Hr After Measurement #1

RETEST
FAIL PASS
Pulse ox of 90%–94% in BOTH
Pulse ox of 89% or less in Pulse ox of 95% or more in RH or
the RH and foot OR a
either the RH or foot foot AND difference of 3% or less
difference of 4% or more
Action: Do not repeat for between the 2
between the RH and foot
screening, refer for Action: Do not repeat for screening,
Action: Repeat pulse ox
immediate assessment provide normal newborn care
measurements in 1 hour

Measurement #3
Pulse Ox on (RH) and 1 Foot 1 Hr After Measurement #2

RETEST
FAIL PASS
Pulse ox of 90–94% in BOTH
Pulse ox of 89% or less in Pulse ox of 95% or more in RH or
the RH and foot OR a
either the RH or foot foot AND difference of 3% or less
difference of 4% or more
Action: Do not repeat for between the 2
between the RH and foot
screening, refer for Action: Do not repeat for screening,
Action: Do not repeat, clinical
immediate assessment provide normal newborn care
assessment

Figure 23.1. Critical congenital health disease screening protocol.

Abbreviation: pulse ox, pulse oximetry.
Reprinted with permission from Ewer AK, Martin GR. Newborn pulse oximetry screening: which algorithm is best? Pediatrics. 2016;138(5): e20161206.

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 Chapter 23: Neonatal Examination and Nursery Visit 151

Genitalia part of the newborn neurologic examination. The behavior of the

In female neonates, the labia majora may be swollen, but the ure- newborn also can yield information about an intact neurologic sys-
thra and vaginal opening should be visualized to ensure patency. tem. The newborn should respond to sound, fixate on a face, and be
Hymenal tags are a common finding. Clitoral size is noted. Parents capable of attempts at self-consolation.
should be told that a physiologic vaginal discharge and the pres- Normal findings of the nursery physical examination are sum-
ence later of a pink or blood-tinged discharge are a normal neona- marized in Box 23.2. Significant findings should be addressed
tal response to maternal estrogen withdrawal. In male newborns,
the penis is examined for size and length. The foreskin should be
retracted sufficiently to reveal the location of the urethral meatus to Box 23.2. Common Benign Physical
assess for hypospadias. A testicular examination can rule out hydro- Findings in Newborns
celes, inguinal hernias, and cryptorchidism. Atypical genitalia may Skin
be indicative of an underlying disorder, such as congenital adrenal ww Milia
hyperplasia or disorders of sexual differentiation, and necessitates ww Erythema toxicum
further evaluation (see Chapter 107). ww Salmon patch
ww Nevus flammeus
Anus ww Hemangiomas
ww Dermal melanosis
The patency and location of the anus are assessed. Patency is con-
ww Lanugo (ie, body hair)
firmed after the newborn passes meconium, typically by 48 hours ww Vernix
of age. An imperforate anus may be an isolated finding, or it may be
Head
indicative of a syndrome, such as vertebral, anal, cardiac, tracheal,
ww Cephalohematoma
esophageal, renal, and limb syndrome. ww Caput succedaneum
ww Molding
Skeleton
Face
The newborn skeleton is assessed for evidence of skeletal dysplasia.
ww Swollen overall appearance
All long bones are examined for a potential fracture secondary to ww Minor malformations
birth trauma. The neonatal hip examination is important to detect
Eyes
developmental dysplasia of the hip, which is most likely to occur in
ww Swollen eyelids
cases of a breech position in utero. The Ortolani maneuver is per- ww Subconjunctival hemorrhages
formed to detect a dislocated hip. The “clunk” felt when performing
Ears
the examination is the relocation of the femoral head of the affected
ww Preauricular appendages/pits
hip in the joint capsule. In contrast, a “click” may be indicative of ww Folded pinnae
normal perinatal ligament laxity. The Barlow maneuver detects an
Nose
unstable hip that may be at risk for dislocation. A positive result on
ww Flattened nose
either test is indicative of a hip that is or could be dislocated and ww Milia over bridge
warrants an orthopedic consultation. Treatment with a Pavlik har-
Mouth and Throat
ness is initiated until confirmatory testing on ultrasonography is
ww Epstein pearls
reliable at 6 weeks of age (see Chapter 113). The spine is palpated ww Epithelial pearls
completely to the sacrum. Sacral defects, deep sacral pits, or sacral ww Natal teeth
tufts of hair warrant an investigation for conditions such as spina ww Shortened frenulum
bifida occulta. The fingers and toes are counted and assessed for Chest
syndactyly or other abnormalities. The feet may be turned inward, ww Supernumerary nipples
outward, or up and are gently moved to a normal position to ensure ww Breast buds
flexibility. Deformities secondary to intrauterine pressure are com- ww Galactorrhea
ww Pectus excavatum or carinatum
mon. Clubfoot or equinovarus deformations warrant evaluation by
an orthopedic surgeon. Genitalia
ww Females: swollen labia, hymenal tags, vaginal discharge
Neurologic Examination ww Males: hydrocele, undescended testicle (palpated in inguinal canal)
A newborn’s resting position is assessed to evaluate tone. All extrem- Hips
ities should be flexed. The newborn then can be held prone for fur- ww Click or clunk sound
ther evaluation of tone. Newborns are observed for motor activity, Extremities
that is, moving arms and legs symmetrically. Response to sensory ww Feet turned up, in, or out, but malleable
stimulation and deep tendon reflexes should be elicited. Facial move-
Neurologic Examination
ments are closely observed for symmetry. Primitive reflexes, such as ww Primitive reflexes: Moro, grasp, rooting, stepping
suck, rooting, grasp, stepping, and especially Moro, are an important

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 152 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

immediately when noted at the initial nursery examination, to evaluate for malformations of the kidneys; a skeletal survey may
including evidence of hydrocephalus, a ductal-dependent cardiac be helpful as well. More extensive studies are indicated in cases in
lesion, cyanotic congenital heart disease, a diaphragmatic hernia, which an emergent physical finding is discovered.
an abdominal mass, or a possible chromosomal abnormality (eg,
trisomy 13 or trisomy 18), all of which can be life-threatening. Management
The physical conditions associated with trisomy 21 are rarely life- After stabilization in the delivery room, the newborn is thoroughly
threatening, although suspicion of the diagnosis warrants consul- dried and given to the mother for breastfeeding and bonding.
tation with a geneticist as well as evaluation for cardiac, abdominal, If mother and baby are healthy, the newborn should stay with the
and other anomalies (see Chapter 42). mother. Every hospital should encourage rooming-in of the newborn
Laboratory Tests with the mother. The newborn should feed in the delivery room
if breastfeeding or within 2 hours of birth if formula feeding
Few laboratory tests are necessary for the healthy newborn. The
and continue to feed every 2 to 3 hours to prevent hypoglycemia.
only test that all newborns in the United States receive is the state-
The preferred feeding method is breastfeeding, and the mother
mandated newborn screening test prior to discharge from the nursery
should receive sufficient postpartum support to ensure success (see
(see Chapter 25). Heel-stick blood usually is evaluated for inherited
Chapter 29). An initial bath is only necessary to remove blood or
conditions, such as phenylketonuria, galactosemia, hypothyroid-
meconium after the newborn’s temperature is stable. The adminis-
ism, hemoglobinopathies, cystic fibrosis, congenital adrenal hyper-
tration of intramuscular vitamin K to prevent hemorrhagic disease
plasia, and inborn errors of metabolism. The newborn screen varies
of the newborn and the application of ophthalmic antibiotic oint-
by state and depends on the prevalence of a particular disease in a
ment or silver nitrate in the newborn’s eyes to prevent gonorrheal
given region (see Chapter 25). In all states, hearing screening is also
infection is universal. Hepatitis B vaccine is recommended for all
part of the mandated newborn screening. Screening methods may
newborns at birth regardless of mother’s serology; based on mater-
be by automated auditory brainstem response, otoacoustic emission
nal risk factors (eg, history of maternal IV drug use), hepatitis B
testing, or conventional auditory brainstem response. The newest
immune globulin should also be considered.
screening test is the addition of pulse oximetry at 24 hours of age to
Vital signs are monitored every 30 minutes until stable during
rule out transposition of the great vessels, tetralogy of Fallot, hypo-
the transition to extrauterine life (which can last 4–8 hours), then
plasia of the left heart, and other critical congenital heart disease.
every 4 hours. Daily weights as well as strict documentation of void-
Serum glucose testing may be performed for newborns at high
ing and stooling patterns are necessary to monitor the newborn for
risk for hypoglycemia, such as newborns who are SGA or LGA, new-
adequacy of intake and signs of potential dehydration. Weight loss
borns of diabetic mothers, and symptomatic newborns. Evaluation of
is expected, but loss of more than 7% of birth weight requires phy-
hematocrit level is necessary in jaundiced, pale, or ruddy-appearing
sician assessment. Umbilical cord care remains controversial, but in
newborns; SGA and LGA newborns; and twins and multiples. In
developed countries, leaving the cord to dry is sufficient treatment.
cases of ABO or Rh incompatibility, it is important to perform serum
If desired, circumcision is usually performed on the day of
bilirubin and antiglobulin (ie, Coombs) tests. Screening for hyperbil-
discharge (see Chapter 27). The newest guidelines from the AAP note
irubinemia to prevent kernicterus is recommended for all newborns.
that the benefits of circumcision outweigh the risks. Local anesthe-
Bilirubin screening is easily done by a transcutaneous reading; if the
sia is universally recommended for the procedure. After the proce-
transcutaneous reading is elevated, the serum level is evaluated as
dure, parents are instructed to leave the gauze or Plastibell device
well (see Chapter 126).
in place. It will fall off spontaneously. Petroleum ointment may be
Imaging Studies placed on the corona of the penis to prevent it from sticking to the
diaper. The physician should be notified if excessive bleeding or
Routine radiographs are not indicated in neonates whose examina-
oozing occurs (eg, soaking of the diaper with blood) after discharge
tion is normal and should be ordered only if indicated by the exam-
from the nursery.
ination. Some minor malformations, such as pectus excavatum, do
not require radiographic evaluation. Vertebral radiography, ultraso-
nography of the lumbosacral spine, or magnetic resonance imaging Discharge Planning and Counseling
are appropriate in the neonate with a deep sacral pit or sacral tuft of In most cases, mother and newborn should be discharged home
hair in patients in whom spina bifida occulta is suspected. Clavicular together. The recommended hospital stay is 48 hours for a vaginal deliv-
radiographs are indicated in the patient with swelling or pain located ery and 96 hours for a cesarean section. The physician should reexam-
in the clavicular area or in whom an asymmetric Moro reflex is ine the newborn on the day of discharge to identify problems that may
elicited. A chest radiograph, electrocardiogram, and echocardio- have developed and counsel the parent or parents. A bilirubin assess-
gram are warranted for a significant murmur. If hydronephrosis ment is required. This examination is best performed at bedside. The
was detected on prenatal ultrasonography, follow-up renal ultraso- newborn’s physical findings and hospital course should be reviewed
nography should be performed in the neonatal period. If multiple with the parent or parents. If studies other than routine neonatal
anomalies are found, renal ultrasonography should be performed screening were performed, the physician should also share those results.

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 Chapter 23: Neonatal Examination and Nursery Visit 153

Discharge is the best time to review anticipatory guidance issues. Selected References
Topics that should be covered at the bedside include feeding patterns
and what to expect, sleeping and elimination patterns in the new- American Academy of Pediatrics, American College of Obstetricians and
Gynecologists. Guidelines for Perinatal Care. 8th ed. Elk Grove Village, IL:
born, umbilical cord care, bathing the newborn, and safety issues
American Academy of Pediatrics; 2017
(eg, car safety seats, sleeping position, safe sleep environments).
American Academy of Pediatrics Task Force on Circumcision. Circumcision
Guidelines on symptoms of illness and when to call the office or
policy statement. Pediatrics. 2012;130(3):585–586 PMID: 22926180 https://doi.
emergency department also should be addressed. These include a org/10.1542/peds.2012-1989
rectal temperature of 38.0°C or above (≥100.4°F), respiratory dis-
Benitz WE; American Academy of Pediatrics Committee on Fetus and Newborn.
tress, irritability, lethargy, decreased feeding, and evidence of dehy- Hospital stay for healthy term newborn infants. Pediatrics. 2015;135(5):948–953
dration. If parents have any concerns about their newborn, they PMID: 25917993 https://doi.org/10.1542/peds.2015-0699
should be encouraged to call the physician’s office. A follow-up visit Cloherty JP, Eichenwald EC, Hansen AR, Stark AR, eds. Manual of Neonatal Care.
should be arranged at 3 to 5 days of age for any newborn being 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2012
breastfed to give breastfeeding support and to assess the newborn Fernhoff PM. Newborn screening for genetic disorders. Pediatr Clin North Am.
for evidence of jaundice or dehydration. An early follow-up appoint- 2009;56(3):505–513 PMID: 19501689 https://doi.org/10.1016/j.pcl.2009.03.002
ment may also be indicated in a newborn with social risk factors and Karwowski MP, Nelson JM, Staples JE, et al. Zika virus disease: a CDC update
near-term newborns who are at increased risk for complications. for pediatric health care providers. Pediatrics. 2016;137(5):e20160621 PMID:
If the newborn is born via cesarean section or is formula feeding, a 27009036 https://doi.org/10.1542/peds.2016-0621
follow-up visit at 1 week after discharge may be appropriate. Kemper AR, Mahle WT, Martin GR, et al. Strategies for implementing screen-
Early newborn discharge is an option if desired by the mother. ing for critical congenital heart disease. Pediatrics. 2011;128(5):e1259–e1267
The AAP has published guidance recommendations on discharge of PMID: 21987707 https://doi.org/10.1542/peds.2011-1317
the newborn younger than 48 hours. Generally, the history, includ- Lauer BJ, Spector ND. Hyperbilirubinemia in the newborn. Pediatr Rev.
ing social risk factors, physical examination, and hospital course, all 2011;32(8):341–349 PMID: 21807875 https://doi.org/10.1542/pir.32-8-341
should be low risk and the newborn should have been observed for Mahle WT, Martin GR, Beekman RH III, Morrow WR; American Academy of
at least 12 hours. If the newborn is stable and is sent home at less Pediatrics Section on Cardiology and Cardiac Surgery Executive Committee.
than 48 hours of age, a mandatory follow-up appointment should Endorsement of Health and Human Services recommendation for pulse oxim-
be scheduled within 2 days of discharge. The newborn born at home etry screening for critical congenital heart disease. Pediatrics. 2012;129(1):
190–192 PMID: 22201143 https://doi.org/10.1542/peds.2011-3211
should undergo medical evaluation within 24 hours of birth and
again 48 hours after the first evaluation. Hospitalists have been Ramachandrappa A, Jain L. Health issues of the late preterm infant. Pediatr
Clin North Am. 2009;56(3):565–577 PMID: 19501692 https://doi.org/10.1016/
assuming more of the hospital care for newborns in many commu-
j.pcl.2009.03.009
nities. The complete hospital record and results of screening tests
US Preventive Services Task Force. Screening of infants for hyperbilirubinemia
must accompany the newborn to the medical home. This coordi-
to prevent chronic bilirubin encephalopathy: US Preventive Services Task Force
nation of transfer of care can be challenging, but it is essential for
recommendation statement. Pediatrics. 2009;124(4):1172–1177 PMID: 19786451
optimal care. https://doi.org/10.1542/peds.2009-0128
Warren JB, Phillipi CA. Care of the well newborn. Pediatr Rev. 2012;33(1):4–18
PMID: 22210929 https://doi.org/10.1542/pir.33-1-4
CASE RESOLUTION Yogman M, Lavin A, Cohen G; American Academy of Pediatrics Committee
The parents should be advised that the newborn’s weight, length, and head on Psychosocial Aspects of Child and Family Health. The prenatal visit.
circumference are all normal. The examination is reviewed at bedside, and the Pediatrics. 2018;142(1):e20181218 PMID: 29941679 https://doi.org/10.1542/
subconjunctival hemorrhages should be shown to the parents and their benign, peds.2018-1218
self-limited nature explained. Parents should be reassured about all other nor-
mal aspects of the physical examination. The newborn’s blood type from cord
blood should be obtained, because the mother has blood type O Rh-positive.
Routine neonatal screening, feeding, sleeping, elimination, bathing, and safety
should be reviewed. Before discharge, the newborn should receive the hepati-
tis B vaccine and should undergo newborn screening tests and bilirubin assess-
ment. A follow-up appointment should be made for 48 hours after discharge to
follow breastfeeding progress. Results of all newborn screening tests should be
reviewed when available.

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BPPCA6e_Ch023_0145-0154.indd 154 2/14/20 10:41 AM
 CHAPTER 24

Maternal Perinatal Mood

and Anxiety Disorders: The
Role of the Pediatrician
Carol D. Berkowitz, MD, FAAP

CASE STUDY
You are evaluating a 3-week-old boy who is the prod- 2. What are the signs and symptoms of perinatal mood
uct of a 39-week gestation pregnancy to a 30-year-old and anxiety disorders?
gravida 1, para 1 mother who has been breastfeeding 3. What are the risks to newborns of mothers who
the newborn. The newborn’s birth weight was 3,650 g experience perinatal mood and anxiety disorders?
(8.0 lb), and the newborn now weighs 3,380 g (7.5 lb). To older children?
The mother expresses concern about her ability to 4. What is the role of the pediatrician in assessing
breastfeed. She also admits to being exhausted and mothers for perinatal mood and anxiety disorders?
feeling detached from the baby. She is overwhelmed 5. What screening instruments are available to assist in
by being a mom, something she had looked forward to assessing mothers for perinatal mood and anxiety
since she was a little girl. She has difficulty concentrat- disorders?
ing and has no appetite. She asks you if it is normal to 6. What are the risks and benefits of the use of psycho-
feel this way. pharmacology during pregnancy and postpartum if
breastfeeding?
Questions 7. What resources are available to offer to mothers
1. What is the spectrum of perinatal mood and anxiety
who may be experiencing perinatal mood and
disorders?
anxiety disorders?

The term perinatal mood and anxiety disorders (PMADs) is the visits: 2 weeks, 2 months, and 6 months. In addition, women are
preferred nomenclature to denote the spectrum of mental health likely to follow up with their baby’s pediatric appointments more
issues facing mothers (and fathers) related to the pregnancy and than their own. While fatigue is a common report of new mothers as
birth of a neonate. Peripartum depression had been used to encom- well as new fathers, other symptoms, particularly those of impaired
pass a cadre of mental health problems that new mothers may expe- functioning and diminished ability to care for the baby, may suggest
rience, but now there is recognition that mental health issues can be a more significant disturbance.
present preconception, through pregnancy, and into the infant’s first
postnatal year. These issues include not only depression but other
mental health disorders. Despite public disclosures and open dis- Epidemiology
cussions by celebrities such as Brooke Shields and Gwyneth Paltrow, Maternal depression is the number 1 complication of pregnancy,
these conditions are under-recognized, and as a result, many moth- exceeding diabetes and hypertension. The incidence of perinatal
ers go undiagnosed and untreated. The stigma related to mental depression varies with the population studied, with the estimated
health is a barrier to maternal disclosure and seeking help. Too range being from 5% to 25%. Between 15% and 25% of pregnant
often, maternal symptoms are dismissed as fatigue related. The pedi- women experience depressive symptoms while pregnant, and
atrician is in an ideal position to assess a mother for these symp- approximately 13% of women take an antidepressant at some time
toms following the birth of a baby because the pediatrician usually during their pregnancy. Women who experience depressive symp-
sees the mother-baby dyad prior to the obstetric postpartum visit toms during pregnancy are twice as likely to be depressed post-
at 6 weeks following the baby’s birth. The recommended time partum as those with no depressive symptoms while pregnant.
points for screening for PMADs coincide with health supervision The prevalence of PMADs is much higher at 40% to 60% in certain
155

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 156 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

groups, including women in low-income households, certain ethnic

Box 24.1. Perinatal Mood and Anxiety Disorders
minorities (including Hispanic, black, and Native American), preg-
Symptomatology
nant and parenting teenagers, mothers of multiple births, women
who served in the military, and members of the lesbian, gay, bisex- Baby Blues
ual, transgender, questioning/queer, intersex (LGBTQI) community. ww Sadness
The World Health Organization notes that depression is the fourth ww Crying
leading cause of disease burden in the world. In spite of the high ww Mood swings
prevalence across all groups, fewer than 50% of cases of PMADs ww Anxiety
are identified. ww Worrying
Often, there is reluctance on the part of obstetricians or psy- ww First few days after delivery
chiatrists to treat pregnant women with antidepressants because of ww Resolves in 1–2 weeks
concerns about the potential adverse effects of the medications on Peripartum Depression
the developing fetus. These potential effects include fetal malforma- ww Meets Diagnostic and Statistical Manual of Mental Disorders,
tions, cardiac defects, pulmonary hypertension, and reduced birth 5th Edition, criteria for depression
weight. However, untreated PMADs in pregnancy increase the risk ww Depressed mood
of preterm delivery, low birth weight, and a newborn’s own ability to ww Diminished pleasure
regulate emotions and stress. The American College of Obstetricians ww Change in appetite
and Gynecologists and the American Psychiatric Association have ww Change in sleep
issued joint guidelines on the management of pregnant women ww Fatigue
with depression, which include indications for psychotherapy as ww Feelings of worthlessness or guilt
well as psychopharmacology in the pregnant woman. Pediatricians ww Inability to concentrate
are usually not involved in this part of the decision-making pro- ww Recurrent thoughts of death or suicide
cess but should be knowledgeable about the possible complications
Postpartum Psychosis
that the newborn may experience following birth from exposure
ww Severe impairment
to antidepressants or untreated maternal mental illness. The issue
ww Paranoia
of psychotherapeutics in breastfeeding mothers is also relevant to
ww Mood shifts
the pediatrician. Approximately 6% to 10% of fathers experience
ww Hallucinations
peripartum depression, with the highest rates seen between 3 and
ww Delusions
6 months following the birth of the baby. Paternal rates of depression
ww Suicidal/homicidal ideation
are higher when there is maternal depression, and the effect on the
ww Usually requires hospitalization
infant is greater than if the father is not affected. Depressed fathers
have an increased rate of substance use.
There is a wide range of symptomatology that can be categorized
impairment, with paranoia, mood shifts, hallucinations, delusions,
as PMADS (Box 24.1). Baby blues or maternity blues are used to
and suicidal and homicidal ideation. Sometimes there is a history of
describe the very common experience of new mothers, said to affect
a preexisting bipolar disorder. Affected mothers require hospitaliza-
50% to 80% of postpartum women in the first few days after delivery.
tion, in part because the infanticide rate is 4% to 5%.
There is no Diagnostic and Statistical Manual of Mental Disorders,
Other disorders that have been recognized as being pregnancy
5th Edition (DSM-5) categorization of baby blues. Generally, symp-
and birth related or exacerbated include anxiety disorders, panic
toms improve over 1 to 2 weeks and functioning is not impaired,
disorder, postpartum posttraumatic stress disorder, postpartum
although baby blues may herald later depression. Mothers experi-
bipolar disorder, and postpartum obsessive-compulsive disorder;
ence sadness, crying, mood swings, anxiety, and worrying.
5% of mothers report intrusive thoughts of harming their baby.
A diagnosis of peripartum depression meets the criteria of
It is estimated that 400,000 neonates are born to mothers who
depression according to DSM-5, which include a depressed mood,
are depressed. These neonates are at risk for a host of adverse phys-
diminished pleasure (anhedonia), changes in appetite and sleep, psy-
ical and developmental consequences.
chomotor agitation or retardation, fatigue, feelings of worthlessness
or inappropriate guilt, decreased ability to concentrate, and recurrent
thoughts of death or suicide. Suicide is the leading cause of death in Pathophysiology
mothers during the first year postpartum. Technically, symptoms of The precise pathophysiology of PMADs has yet to be elucidated,
depression must begin within 4 weeks of delivery and may persist but sleep deprivation related to the demands of caring for a new-
for 1 year, although the onset is often insidious and does not come born, genetic factors, other biological or inflammatory processes
to medical attention until later than 1 month postpartum. (cytokines), and hormonal changes are felt to be contributing
Postpartum psychosis is less frequent, occurring in 1 to 3 per factors. While there are rapid decreases in the levels of estrogen,
1,000 deliveries within the first 4 weeks after delivery. There is severe progesterone, and cortisol, the hormonal levels noted in mothers

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 Chapter 24: Maternal Perinatal Mood and Anxiety Disorders: The Role of the Pediatrician 157

who experience peripartum depression do not differ from women

Box 24.2. Sample Question From Edinburgh
who do not experience peripartum depression. Perhaps more
Postnatal Depression Scale
significantly, there are a number of risk factors that have been asso-
ciated with its development, including prior history of depression As you are pregnant or have recently had a baby, we would like to know
in general but especially during a previous pregnancy; mood dis- how you are feeling. Please check the answer that comes closest to how
orders, including premenstrual dysphoric disorder; substance use; you have felt IN THE PAST 7 DAYS, not just how you feel today.
alcohol dependence; low socioeconomic status; a history of infer- Here is an example, already completed.
tility; prior perinatal loss (eg, miscarriage, sudden unexpected I Have Felt Happy:
infant death, bed-sharing death); lack of social support or com- ƒƒ Yes, all of the time.
munity network; unintended or unwanted pregnancy; or a fam- ƒƒ Yes, most of the time.
ily history of depression. Other social or familial factors include ƒƒ No, not very often.
marital discord, divorce, and intimate partner violence. Homicide ƒƒ No, not at all.
is the leading cause of death among pregnant women. It is impor- This would mean: “I have felt happy most of the time” during the past
tant to recognize that depression can affect adoptive mothers, who week. Please complete the other questions in the same way.
experience stress and anxiety during the adoptive process, remain
concerned that the birth mother may reclaim her baby, have issues Adapted with permission from Cox JL, Holden JM, Sagovsky R. Detection of postnatal depression.
related to prior infertility, and tend to have less familial support. Development of the 10-item Edinburgh Postnatal Depression Scale. Br J Psychiatry. 1987;150:782–786.
Peripartum depression is less easy to recognize in fathers.
Paternal depression is often covert or masked. Fathers are less likely in 1987 and consists of 10 questions completed by the mother. The
to seek help or have ongoing access to health professionals. They may mother is asked to check the response that comes the closest to how
feel excluded from the mother-baby relationship. Often, they become she has been feeling in the previous 7 days, with there being 4 choices
workaholics as a means of coping with depression. ranging from “often” to “never.” All items must be completed, and
the mother must respond by herself unless she has limited English
Evaluation skills and the questionnaire has not been provided in the mother’s
native language. The maximum score is 30. A score greater than
Mother 10 indicates risk for depression. A positive response to question 10,
Screening for PMADs is within the scope of the pediatric practice. which asks about suicidality by itself, is considered a positive screen-
Studies have shown that mothers are comfortable being queried by ing result. The instrument is available in more than 40 languages.
pediatricians about how they are doing. Some pediatricians express A third instrument is the Patient Health Questionnaire-9
concern about the appropriate strategies for screening and what to (PHQ-9). Like the EPDS, the PHQ-9 includes 10 questions (9 ques-
do if they determine that a mother has symptoms of depression. tions and a follow-up question if any of the first 9 indicated any
It is important to couch any depression screening in language that problems) related to the signs and symptoms of depression. The
makes a new mother feel comfortable and not judged in any way. respondent notes whether the symptoms are present “not at all,”
Postpartum Support International promotes the “universal message” “several days,” “more than half the days,” or “nearly every day.” A
to share with depressed new mothers: You are not crazy; you are not score is then generated based on the mother’s answers to assess
alone; and with the right help, you will get better. her degree of depression. Mothers may be asked to complete these
There are several instruments used to screen for PMADs. A sim- questions while in the waiting area. The PHQ-9 is not limited to
ple tool, called the Patient Health Questionnaire-2 (PHQ-2), begins postpartum mothers but can be used for all patients older than
with a background statement related to depression as a common 18 years. Some electronic health records require the PHQ-9 as part
and treatable condition that often goes unrecognized. There follows of routine annual health screening.
justification citing the recommendation of the US Preventive Services
Task Force that all adults be checked for depression. The question, Infants and Children
“Over the past 2 weeks, how often have you been bothered by any of the Infants and children may experience a number of problems as a con-
following problems?” is asked, with the following 2 categories: “Little sequence of maternal depression. Exposed children may develop dis-
interest or pleasure in doing things,” and “Feeling down, depressed, orders of attachment, particularly insecure attachment, and are at
or hopeless.” For both categories, the following responses are possible: risk for the subsequent development of conduct or other behavior
0, not at all; 1, several days; 2, more than half the days; 3, nearly every disorders. Language development may be adversely affected because
day. A score of 3 or more has a sensitivity of 83% and a specificity of depressed mothers speak fewer words and have fewer social inter-
92% for major depressive episode and indicates the need for a more actions with their children. Maternal depression has also been asso-
extensive assessment to make the diagnosis of depression. ciated with the decision not to breastfeed or the early cessation of
A second instrument is the Edinburgh Postnatal Depression Scale breastfeeding. Classic environmental failure to thrive can be related
(EPDS), which is available on the internet at no cost. A sample ques- to maternal depression and reduced mother-baby interactions. The
tion from the scale is given in Box 24.2. The scale was developed effects of maternal depression can be detected in 2-month-olds who

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 158 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

regard mothers who have depression less frequently, have poorer with sertraline (eg, Zoloft) and paroxetine. Low levels of drug are
state regulation, interact with objects less frequently, and have lower detected in human milk but are not detected in the newborn’s blood.
levels of overall activity than infants of mothers who do not have If the mother is on a different medication, checking the medication
depression. These symptoms are attributed to the notions of the need website may provide additional information. Of note, there is a newly
for reciprocity between the mother and infant: mirror neurons, skill licensed medication, brexanolone, which appears to be effective for
beget skill, and serve and return. The persistent absence of respon- severe postpartum depression. To date there is no information related
sive care disrupts brain development. Long-term effects of maternal to breastfeeding and the use of brexanolone, but treated mothers
depression on child development have been demonstrated in mag- have all been hospitalized because of the severity of their depression.
netic resonance imaging of the brains of such children and increased There are a number of internet sites that are very helpful in pro-
cortisol levels at the time of school entry. Children are characterized viding information about community resources. Postpartum Support
as anxious, wary, and withdrawn. Social skills are noted to be poor. International (www.postpartum.net; 800/944-4PPD [4773]) pro-
Early diagnosis and intervention may prevent the development of vides geographically specific information. The website can be
these problems. accessed even while the mother is in the pediatrician’s office. It
is also important to have an emergency protocol in place, such as
Management calling 911 or a psychiatric emergency team, should a woman
endorse suicidality while in the pediatrician’s office. Having such
The major role of the pediatrician is to assist in the early identifi-
resources and protocols in place can go a long way toward reducing
cation of mothers who are experiencing PMADs and to make the
pediatricians’ anxiety about screening for depression and suicidality.
appropriate referral for more definitive management. Support for
Some perinatal programs offer home visitation or the use of dou-
this recommendation comes from a number of sources, includ-
las (ie, women who support other women through labor and deliv-
ing the 1999 and 2001 reports of the US Surgeon General; Bright
ery and after the birth of a baby in a nonmedical capacity), which
Futures: Guidelines for Health Supervision of Infants, Children, and
assist mothers-to-be as well as recent mothers with the demands
Adolescents; the American Academy of Pediatrics (AAP) National
of birth and parenting. Such programs may offer a preventive and
Resource Center for Patient/Family-Centered Medical Home; and
early intervention approach to peripartum depression by offering
AAP policies on family-centered care. Practices that include the pres-
help and emotional support to new mothers as well as by screening
ence of social workers or mental health specialists can avail them-
for PMADs on a routine basis.
selves of these individuals to assess, counsel, and refer mothers as
Older children who have been affected by maternal depres-
appropriate. The mother may be referred back to her obstetrician
sion may be treated with other modalities that focus on mother
for a visit prior to the 6-week checkup. Alternatively, mothers may
and child to help address attachment disorders. Research on these
be referred to local mental health specialists when such resources
dyadic interventions has demonstrated they are associated with
are known and available.
decreased psychiatric symptoms and significant improvement in
The pediatrician should also be familiar with the possible adverse
functioning of the mother and child. Internalizing and externalizing
effects of psychotropic medications on the fetus and newborn and
child behaviors are reduced when maternal depression, anxiety, or
recommendations about these medications in relation to breastfeed-
other conditions are addressed. Some programs include parent-child
ing. The decision to initiate psychotropic medication ingestion during
interactive therapy and parent-child psychotherapy. The program
pregnancy is made by the obstetrician or psychiatrist caring for the
Circle of Security International involves video-based intervention
mother. There is no antidepressant specifically approved by the US
that focuses on strengthening care giving.
Food and Drug Administration for use during pregnancy. All anti-
depressants cross the placenta and are never category A (no risk).
Animal studies reveal no teratogenic effects of these medications, and Prognosis
the treating physician has to balance the risks of untreated depres- The prognosis for PMADs and their effects on newborns, infants, and
sion versus the use of selected serotonin reuptake inhibitors (SSRIs). children are contingent on early recognition and appropriate inter-
Paroxetine (eg, Paxil) has been associated with abnormalities of the vention. Promoting screening is critical to ensuring a positive out-
right ventricular outflow tract. There is an increased risk of a septal come. Pediatricians still face barriers, however, including the need
defect with the use of any SSRI. It is noted that the risk of preterm to screen for a number of other conditions (eg, parental smoking,
labor increases from 6% to 22% when SSRIs are used, but the risk of interpersonal violence), insufficient time, inadequate training, lack
preterm labor with untreated maternal depression is 20%. Newborns of appropriate resources, and lack of payment for services. A num-
who have been exposed to SSRIs may exhibit poor neonatal adap- ber of models have demonstrated that screening for PMADs can be
tion or neonatal abstinence syndrome. Symptoms include respira- successfully undertaken in a pediatric practice. The Assuring Better
tory distress, irritability, jitteriness, hypotonicity, poor latching and Child Health & Development project has been implemented in
feeding, and, rarely, seizures. The prevalence of persistent pulmo- 28 states and involves the AAP chapters in those states. Pediatricians
nary hypertension increases from 1 to 6 in 1,000. Even if the mother in Illinois, for example, can be paid through Medicaid if they adminis-
is on an SSRI, she should be encouraged to breastfeed. As it relates ter the EPDS. Bright Futures, the health supervision guidelines devel-
to the use of SSRI and breastfeeding, we have the most experience oped by the AAP, endorses assessing parental social and emotional

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 Chapter 24: Maternal Perinatal Mood and Anxiety Disorders: The Role of the Pediatrician 159

well-being. Incorporating questioning into the health supervision Earls MF; American Academy of Pediatrics Committee on Psychosocial Aspects
visits at 1, 2, 4, and 6 months is recommended. Additional informa- of Child and Family Health. Incorporating recognition and management
tion about individual state initiatives can be found at the National of perinatal and postpartum depression into pediatric practice. Pediatrics.
2010;126(5):1032–1039 PMID: 20974776 https://doi.org/10.1542/peds.2010-2348
Academy for State Health Policy ABCD Resource Center website
(www.nashp.org/abcd-resources). Hanley GE, Oberlander TF. The effect of perinatal exposures on the infant: anti-
depressants and depression. Best Pract Res Clin Obstet Gynaecol. 2014;28(1):
37–48 PMID: 24100223 https://doi.org/10.1016/j.bpobgyn.2013.09.001
Mattocks KM, Skanderson M, Goulet JL, et al. Pregnancy and mental health
CASE RESOLUTION
among women veterans returning from Iraq and Afghanistan. J Womens Health
While many of this mother’s symptoms are common, her self-assessment that
(Larchmt). 2010;19(12):2159–2166 PMID: 21039234 https://doi.org/10.1089/
she is unable to function suggests that she is experiencing peripartum depres-
jwh.2009.1892
sion rather than baby blues. You ask her if it is all right to contact her obstetrician
to see if she could be seen sooner. She agrees. When you reach her obstetrician, Olson AL, Dietrich AJ, Prazar G, Hurley J. Brief maternal depression screening at
she schedules the mom to come in the following morning. The obstetrician tells well-child visits. Pediatrics. 2006;118(1):207–216 PMID: 16818567 https://doi.
you she has a therapist in her office who will be able to meet with the mother org/10.1542/peds.2005-2346
at that time. Yonkers KA, Wisner KL, Stewart DE, et al. The management of depression during
pregnancy: a report from the American Psychiatric Association and the American
College of Obstetricians and Gynecologists. Obstet Gynecol. 2009;114(3):703–713
Selected References PMID: 19701065 https://doi.org/10.1097/AOG.0b013e3181ba0632

Choi Y, Bishai D, Minkovitz CS. Multiple births are a risk factor for post-
partum maternal depressive symptoms. Pediatrics. 2009;123(4):1147–1154
PMID: 19336374 https://doi.org/10.1542/peds.2008-1619

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BPPCA6e_Ch024_0155-0160.indd 160 2/13/20 5:25 PM
 CHAPTER 25

Newborn Screening
Henry J. Lin, MD, and Moin Vera, MD, PhD

CASE STUDY
A 1-week-old boy is brought to the pediatrician’s office Questions
for a positive newborn screening test result for congen- 1. What are the proposed benefits of newborn screening?
ital adrenal hyperplasia. The baby was a product of a 2. Which newborn screening tests are most commonly
38-week gestation and was born by normal spontane- performed?
ous vaginal delivery to a 30-year-old gravida 2, para 2 3. How are the results of newborn screening tests
woman with an unremarkable pregnancy. Birth weight reported to physicians?
was 3,300 g (116.4 oz), and the baby is feeding and act- 4. How should a patient with an abnormal newborn
ing appropriately. Family history is unremarkable, and screening result be managed?
the physical examination is normal. 5. What are the most common causes of false-positive
and false-negative results?
6. What are the ethical issues and future challenges
surrounding newborn screening?

Newborn screening programs are designed to identify neonates at disease), mucopolysaccharidosis type I (ie, Hurler syndrome), X-linked
risk for catastrophic outcomes from treatable illnesses. Technologic adrenoleukodystrophy, and spinal muscular atrophy (caused by homo-
advances in the past 50 to 60 years, such as tandem mass spectrom- zygous deletion of exon 7 in SMN1; Table 25.1). The Recommended
etry, have made it possible to test for more than 50 metabolic disor- Uniform Screening Panel also has a list of Secondary Conditions, based
ders from a single blood spot. New techniques in molecular biology, on the earlier recommendations (Box 25.1).
including high-throughput DNA sequencing, allow for rapid diag- Primary care physicians have 3 crucial roles in newborn screening.
nostic testing of conditions such as cystic fibrosis. First, they provide education to parents about the newborn screening
From the inception of newborn screening in the 1960s until 2005, process. Second, they ensure that specimens are drawn under proper
each state in the United States chose a different set of conditions for circumstances and that the results are promptly followed up. Finally,
its newborn screening program, based on disease prevalence, cost, they provide medical follow-up and referral in cases of positive test
availability of treatment, and false-positive rates. In 2005, an expert results. All physicians must have contact information for state new-
panel from the American College of Medical Genetics and Genomics born screening programs and local pediatric subspecialists. Contact
recommended 29 core disorders for which newborn screening was information for these groups is listed in Table 25.2.
most effective, as well as 25 secondary disorders that are in the differ-
ential diagnosis of a core disorder. In 2010, severe combined immu- Epidemiology
nodeficiency (SCID) was added to the core list. Screening for critical
More than 4 million newborns are screened each year in the United
congenital heart disease (by pulse oximetry) was endorsed by the
States. The National Newborn Screening 2006 Incidence Report
American Academy of Pediatrics in 2011. By the end of 2013, all
shows that newborn screening identifies 1 in 3,200 newborns with
states offered testing for the 29 original core disorders, although
a metabolic disorder, 1 in 2,200 with congenital hypothyroidism,
screening for secondary disorders and SCID was variable.
1 in 2,200 with sickle cell disease or a related hemoglobinopathy, and
Since 2015, development of federal recommendations for new-
1 in 29,000 with congenital adrenal hyperplasia. Several disorders
born screening has been the responsibility of the Advisory Committee
are more common in particular ethnic groups. For example, cystic
on Heritable Disorders in Newborns and Children (under the US
fibrosis has an incidence of 1 in 2,500 in whites, and sickle cell dis-
Department of Health and Human Services). The conditions on the
ease has an incidence of 1 in 400 in blacks.
Recommended Uniform Screening Panel include metabolic disorders,
hemoglobinopathies and thalassemias, congenital hypothyroidism,
SCID, hearing screening (see Chapter 88), and critical congenital heart Clinical Presentation
disease. Advances in treatment (eg, enzyme replacement therapy) have Most neonates with disorders detected on newborn screening are
resulted in recent expansion of the panel. As of July 2018, the latest clinically asymptomatic in the first 2 weeks after birth, but oth-
additions to the Core Conditions list were disease type II (ie, Pompe ers may have significant signs and symptoms (see Table 25.1). The
161

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 162 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 25.1. Recommended Uniform Screening Panel Core Conditionsa

Type of Disorder Core Condition Possible Signs and Symptoms in Neonates
Metabolic: Propionic acidemia Lethargy, vomiting, hypoglycemia, ketoacidosis, hyperammonemia,
Organic acid neutropenia, thrombocytopenia
Methylmalonic acidemia (ie, methylmalonyl-CoA mutase) Same as above
Methylmalonic acidemias (ie, cobalamin disorders)
Isovaleric acidemia Lethargy, vomiting, odor of sweaty feet, hyperammonemia
3-Methylcrotonyl-CoA carboxylase deficiency Lethargy, vomiting; may be asymptomaticb
3-Hydroxy-3-methylglutaricaciduria Lethargy, vomiting, hypoglycemia, hyperammonemia, elevated
transaminases
Holocarboxylase synthetase deficiency Lethargy, vomiting, hypoglycemia, ketoacidosis, hyperammonemia
β-Ketothiolase deficiency Lethargy, vomiting; may be asymptomaticb
Glutaric acidemia type 1 Macrocephaly possible; otherwise asymptomaticb
Metabolic: Carnitine uptake defect/carnitine transport defect Lethargy, cardiac decompensation, hypotonia, hypoglycemia, liver dysfunc-
Fatty acid tion; may be asymptomaticb
oxidation Medium-chain acyl-CoA dehydrogenase deficiency Lethargy, coma, sudden death, hypoglycemia, liver dysfunction, arrhythmias,
symptoms similar to those of Reye syndrome; may be asymptomaticb
Very-long-chain acyl-CoA dehydrogenase deficiency Lethargy, cardiac decompensation, coma, sudden death, hypoglycemia;
may be asymptomaticb
Long-chain l-3-hydroxyacyl-CoA dehydrogenase deficiency Lethargy, cardiac decompensation, hypoglycemia, liver dysfunction; may be
asymptomaticb
Trifunctional protein deficiency Same as above
Metabolic: Argininosuccinicaciduria Lethargy, vomiting, seizures, coma, hyperammonemia
Amino acid Citrullinemia type I Lethargy, vomiting, seizures, coma, hyperammonemia
Maple syrup urine disease Lethargy, vomiting, seizures, coma, maple syrup odor
Homocystinuria Asymptomaticb
Classic phenylketonuria Asymptomaticb
Tyrosinemia type I Vomiting, diarrhea, liver dysfunction (jaundice, bleeding, hypoglycemia),
boiled cabbage odor; may be asymptomaticb
Endocrine Primary congenital hypothyroidism May be asymptomaticb; umbilical hernia, enlarged fontanelle, macroglos-
sia, jaundice
Congenital adrenal hyperplasia Virilization in females, salt-wasting crisis
Hemoglobin SS disease (ie, sickle cell anemia) Asymptomaticb, dactylitis
S β-thalassemia (ie, sickle β-thalassemia) Asymptomaticb
SC disease (ie, hemoglobin C sickle cell disease) Asymptomaticb
Other Biotinidase deficiency Lethargy, hypotonia, seizures; may be asymptomaticb
Critical congenital heart disease Hypoxemia
Cystic fibrosis Meconium ileus, intestinal obstruction
Classic galactosemia Lethargy, vomiting, diarrhea, jaundice, hepatomegaly, cataracts, sepsis
(Escherichia coli)
Glycogen storage disease type II (ie, Pompe disease) Hypotonia, hypertrophic cardiomyopathy; may be asymptomaticb
Hearing loss Deafness, speech delay
(continued)

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 CHAPTER 25: Newborn Screening 163

Table 25.1. Recommended Uniform Screening Panel Core Conditionsa (continued )

Type of Disorder Core Condition Possible Signs and Symptoms in Neonates
Other Severe combined immunodeficiencies Recurrent infections
(continuted) Mucopolysaccharidoses type I Inguinal hernia, upper respiratory tract infection; may be asymptomaticb
X-linked adrenoleukodystrophy Asymptomaticb
Spinal muscular atrophy caused by homozygous deletion of Impaired motor function
exon 7 in SMN1
Abbreviations: CoA, coenzyme A; SMN1, survival of motor neuron 1.
a
As of July 2018.
b
Asymptomatic covers the first month after birth and does not exclude very rare case reports of neonatal presentations.
Adapted from Advisory Committee on Heritable Disorders in Newborns and Children. Recommended Uniform Newborn Screening Panel Core Conditions (as of July 2018). Washington, DC: U.S. Department of
Health & Human Services; 2018 www.hrsa.gov/sites/default/files/hrsa/advisory-committees/heritable-disorders/rusp/rusp-uniform-screening-panel.pdf.

presence of such features may require a more urgent work-up or even

Box 25.1. Recommended Uniform Screening
hospitalization. Unfortunately, severe forms of some metabolic dis-
Panel Secondary Conditionsa
orders may cause coma and encephalopathy by 48 hours of age. In
Organic Acid Disorders these cases, newborn screening results are critical, because they will
ww Methylmalonic acidemia with homocystinuria suggest a probable diagnosis and allow early optimization of therapy.
ww Malonic acidemia
ww Isobutyrylglycinuria
ww 2-Methylbutyrylglycinuria Differential Diagnosis
ww 3-Methylglutaconicaciduria Although newborn screening techniques are continually improved,
ww 2-Methyl-3-hydroxybutyricaciduria false-positive and false-negative results may occur. Mislabeled
Fatty Acid Oxidation Disorders specimens, technical errors, and reporting errors can occur in any
ww Short-chain acyl-CoA dehydrogenase deficiency laboratory. Any specimen collected before 12 hours of age is at risk
ww Medium/short-chain l-3-hydroxyacyl-CoA dehydrogenase deficiency for a false-negative metabolic result or a false-positive hypothy-
ww 2,4-Dienoyl-CoA reductase deficiency
ww Carnitine palmitoyltransferase type I deficiency
roidism result. Preterm newborns have a reduced metabolic capac-
ww Carnitine palmitoyltransferase type II deficiency ity and therefore may exhibit higher metabolite levels compared
ww Carnitine acylcarnitine translocase deficiency with levels in full-term infants, which can produce false-positive
Amino Acid Disorders results. Anemia or polycythemia can affect the amount of plasma
ww Argininemia per blood spot, which may lead to false-negative results. Transfusion
ww Citrullinemia type II may alter galactosemia and hemoglobinopathy testing. Neonates
ww Hypermethioninemia receiving hyperalimentation may have increased amino acid and lipid
ww Benign hyperphenylalaninemia levels, especially if the newborn screen is drawn from a central line
ww Biopterin defect in cofactor biosynthesis
(rather than a heel stick). To ensure accurate and uniform testing
ww Biopterin defect in cofactor regeneration
ww Tyrosinemia type II and interpretation, it is imperative that relevant clinical information
ww Tyrosinemia type III be included when the newborn specimen is submitted.
Hemoglobin Disorders
ww Various other hemoglobinopathies Newborn Screening Practices
Other Disorders Parental Education and Consent
ww Galactoepimerase deficiency All parents should be informed of the state screening program at the
ww Galactokinase deficiency prenatal visit or during the initial newborn examination. Written
ww T-cell related lymphocyte deficiencies
materials that explain the screening program are available from state
Abbreviation: CoA, coenzyme A. screening offices. Health professionals are responsible for educating
a
As of July 2018. parents on the method for obtaining the blood specimen, the risks
Adapted from Advisory Committee on Heritable Disorders in Newborns and Children.
and benefits of screening tests, conditions on the screening panel,
Recommended Uniform Newborn Screening Panel Secondary Conditions (as of July 2018).
Washington, DC: U.S. Department of Health & Human Services; 2018 www.hrsa.gov/sites/
and the implications of positive results.
default/files/hrsa/advisory-committees/heritable-disorders/rusp/rusp-uniform-screening- It is important to tell the parent or parents that a positive result
panel.pdf. does not necessarily mean that the newborn has a particular

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 164 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 25.2. Contact Information for State Newborn Screening Programs and Local Pediatric Subspecialists
Contact Type Organization Website
State newborn screening programs National Newborn Screening and Global http://genes-r-us.uthscsa.edu
Resource Center
Geneticists: metabolic and clinical Society for Inherited Metabolic Disorders www.simd.org
American College of Medical Genetics and www.acmg.net
Genomics
Pediatric endocrinologists Pediatric Endocrine Society www.pedsendo.org
Pediatric hematologists American Society of Pediatric Hematology/ www.aspho.org
Oncology
Pediatric pulmonologists Cystic Fibrosis Foundation www.cff.org

disorder. Results must be confirmed by more specific tests. In addi- collection and handling can result in test inaccuracies, delays in
tion, the parent or parents should be advised that in some children reporting results to physicians, and unnecessary repetition of screen-
disorders are missed because of sampling errors, faulty testing, or ing tests. Therefore, all individuals who are involved in the newborn
inadequate accumulation of the abnormal metabolite at the time screening process should strictly adhere to the procedures set forth
of testing. by their state program.
Newborn screening is mandatory in all states, although many
states allow exemptions for religious beliefs or other reasons. In most Reporting of Results
states, parents have given verbal informed consent when practition­ In most states, all results of neonatal screening tests, whether normal
ers have discussed the state-mandated program with them and or abnormal, are reported to the physician of record. Normal results
the parents have agreed to participate. A few states require signed are mailed to the physician for placement in the patient’s medical
consent to opt into the newborn screening program, and several record. Abnormal results are usually reported by telephone or let-
more require consent to disclose identifiable information. Physicians ter, depending on the severity of the potential condition. Results are
should document parental refusal regardless of state laws. also sent to the hospital in which the neonate was born for inclusion
in the medical record. The physician of record is responsible for
Specimen Collection and Handling contacting the parent or parents about the need for confirmatory
Proper specimen collection and handling are essential components testing and, if necessary, referral to an appropriate subspecialist.
of a successful screening program. All newborns should be screened If a newborn is no longer under the care of this physician or if the
before discharge from the hospital, ideally between 24 and 48 hours family cannot be located, state and local public health departments
of age. Screening too early increases the chance of a false-negative can assist in the search for the newborn and family.
metabolic result or a false-positive hypothyroidism result. For this
reason, some states have a mandatory second screen between 1 and Diagnostic and Therapeutic
6 weeks of age, whereas others require a second screen only if the Considerations
first screen is obtained before a specified time (eg, 12 hours, depend- A health care team consisting of a primary care physician and staff,
ing on the state). Late screening increases the sensitivity of meta- state newborn screening office personnel, state newborn screen-
bolic testing but also increases the risk for delayed diagnosis of a ing laboratory, local laboratories, and a subspecialty physician is
life-threatening condition, such as galactosemia. If possible, blood responsible for care and follow-up of patients with positive newborn
samples should be obtained prior to any transfusions or dialysis. screening results. All newborns with abnormal results, whether bor-
If not possible, screening should still be performed as outlined pre- derline or clearly significant, should be evaluated by their primary
viously, and arrangements should be made to repeat the screen at care physician. A complete patient history and physical examina-
an appropriate time. tion as well as a family history should be obtained. Positive results
Several drops of blood are needed to fill each circle on the new- for newborn screening tests often normalize on follow-up testing or
born screening filter paper, and the sample must saturate the paper on a second screen. Therefore, most follow-up evaluations focus on
evenly. The use of needles and glass capillary tubes for blood col- targeted diagnostic tests. Depending on the nature of the suspected
lection is discouraged, because they may cause hemolysis or micro­ disorder, initiation of treatment before confirmatory laboratory
tears in the filter paper. Specimens must be individually air-dried results are known may be appropriate. For example, prophylactic
in a horizontal position to avoid contamination and excessive expo- antibiotics should be administered if sickle cell anemia is suspected.
sure to heat. They should be mailed or preferably, sent by courier, to After confirmatory results have been received, the treatment can be
the laboratory within 24 hours of collection. Inadequate specimen modified or halted.

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 CHAPTER 25: Newborn Screening 165

Potentially life-threatening conditions in the newborn period,

such as galactosemia, maple syrup urine disease, and congenital CASE RESOLUTION
adrenal hyperplasia, are particularly important to evaluate and treat The patient should undergo a full evaluation, including an electrolyte panel,
emergently. Depending on the clinical picture, suspected disorder, because of the possibility of salt-wasting congenital adrenal hyperplasia.
and experience of the physician, telephone consultation with a Confirmatory testing, including measurement of precursor hormones such as
specialist or immediate referral to a regional medical center may 17α-hydroxyprogesterone and subsequent molecular testing, as well as glu-
cocorticoid therapy should be considered in consultation with a pediatric
be necessary.
endocrinologist.
Almost all of the diseases in the current newborn screening
panel are inherited in an autosomal recessive manner. Families
of affected newborns should be offered genetic counseling for
Selected References
future pregnancies, in addition to written information about the
newly diagnosed condition. Depending on the condition, it may Advisory Committee on Heritable Disorders in Newborns and Children.
be necessary to evaluate older siblings. For some conditions, such Recommended Uniform Newborn Screening Panel. Washington, DC: U.S. Department
of Health & Human Services. www.hrsa.gov/advisory-committees/heritable-
as 3-methylcrotonyl coenzyme A carboxylase deficiency, a posi-
disorders/rusp/index.html. Last reviewed February 2019. Accessed March 8, 2019
tive newborn screen may be indicative of an affected asymptom-
Berg JS, Agrawal PB, Bailey DB Jr, et al. Newborn sequencing in genomic med-
atic mother, whose circulating metabolites are also present in the
icine and public health. Pediatrics. 2017;139(2):e20162252. PMID: 28096516
blood of her newborn. https://doi.org/10.1542/peds.2016-2252
Current Issues and Future Challenges Bodamer OA, Scott CR, Giugliani R; Pompe Disease Newborn Screening Working
Group. Newborn screening for Pompe disease. Pediatrics. 2017;140(suppl 1):
Protecting patient privacy is of utmost importance, and most pro-
S4–S13 PMID: 29162673 https://doi.org/10.1542/peds.2016-0280C
grams have laws to protect a patient’s personal information, blood
El-Hattab AW, Almannai M, Sutton VR. Newborn screening: history, current
spots, and genetic information. Great concern exists among par-
status, and future directions. Pediatr Clin North Am. 2018;65(2):389–405
ents and physicians that insurance companies will use newborn
PMID: 29502920 https://doi.org/10.1016/j.pcl.2017.11.013
screening information to discriminate against children and their
Ferreira CR, Gahl WA. Lysosomal storage diseases. Transl Sci Rare Dis. 2017;
families.
2(1–2):1–71 PMID: 29152458
It is indisputable that newborn screening saves lives. Additionally,
Holm IA, Agrawal PB, Ceyhan-Birsoy O, et al; BabySeq Project Team. The
from a purely economic standpoint the cost of newborn screen-
BabySeq project: implementing genomic sequencing in newborns. BMC Pediatr.
ing programs is outweighed by the resulting reduction in mor- 2018;18(1):225 PMID: 29986673 https://doi.org/10.1186/s12887-018-1200-1
bidity and mortality. However, some diseases that are now on the
Kemp S, Huffnagel IC, Linthorst GE, Wanders RJ, Engelen M.
Recommended Uniform Screening Panel, such as lysosomal storage Adrenoleukodystrophy: neuroendocrine pathogenesis and redefinition of nat-
disorders, have extremely costly treatments that may total millions ural history. Nat Rev Endocrinol. 2016;12(10):606–615 PMID: 27312864 https://
of dollars over the lifetime of the patient and produce significant doi.org/10.1038/nrendo.2016.90
morbidity. Cost-benefit analyses will be crucial to shaping future Kolb SJ, Coffey CS, Yankey JW, et al; NeuroNEXT Clinical Trial Network on behalf
newborn screening panels. Pediatricians and other health profes- of the NN101 SMA Biomarker Investigators. Natural history of infantile-onset
sionals must advocate for insurance coverage for newborn screening spinal muscular atrophy. Ann Neurol. 2017;82(6):883–891 PMID: 29149772
panels, especially as hospital charges for newborn testing increase. https://doi.org/10.1002/ana.25101
Several newborn screening tests may detect asymptomatic carrier Levy HL, Watson MS; American College of Medical Genetics Newborn Screening
status in newborns. Although knowledge of a patient’s carrier status Work Group. ACMG ACT Sheets and Confirmatory Algorithms. Bethesda, MD:
may be of some use for childbearing decisions, it has no immedi- American College of Medical Genetics; 2001 www.ncbi.nlm.nih.gov/books/
NBK55827/
ate benefit for the newborn and may inadvertently result in dis-
crimination and stigmatization. The parents of affected newborns, Platt FM. Emptying the stores: lysosomal diseases and therapeutic strategies. Nat
Rev Drug Discov. 2018;17(2):133–150 PMID: 29147032 https://doi.org/10.1038/
who usually are asymptomatic carriers, are presented with the same
nrd.2017.214
dilemma and may wish to forego additional testing concerning their
Stapleton M, Arunkumar N, Kubaski F, Mason RW, Tadao O, Tomatsu S. Clinical
own carrier status.
presentation and diagnosis of mucopolysaccharidoses. Mol Genet Metab.
New challenges will emerge as genetic technology advances 2018;(1–2):4–17 PMID: 30057281 doi: 10.1016/j.ymgme.2018.01.003
toward the idea of using genomic sequencing as the standard
Therrell BL Jr, Padilla CD. Newborn screening in the developing countries. Curr
test for newborn screening. The thought that genomic sequenc- Opin Pediatr. 2018;30(6):734–739 PMID: 30124582 https://doi.org/10.1097/
ing could someday be applied to all newborns seems less remote MOP.0000000000000683
today than it did a few years ago, judging by statements in pub- Wasserstein MP, Caggana M, Bailey SM, et al. The New York pilot newborn screening
lished reviews. The technical feasibility, ethics, legal questions, program for lysosomal storage diseases: report of the first 65,000 infants. Genet Med.
and medical outcomes of genomic testing remain topics for 2019;21(3):631–640 PMID: 30093709 https://doi.org/10.1038/s41436-018-0129-y
dialogue and research. Health professionals may refer to current Zacharias RL, Smith ME, King JS. The legal dimensions of genomic sequenc-
medical literature to stay abreast of this interesting and devel- ing in newborn screening. Hastings Cent Rep. 2018;48(suppl 2):S39–S41 PMID:
oping area. 30133728 https://doi.org/10.1002/hast.884

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BPPCA6e_Ch025_0161-0166.indd 166 2/13/20 5:26 PM
 CHAPTER 26

Caring for Twins and

Higher-Order Multiples
Soina Kaur Dargan, MD, FAAP, and Lynne M. Smith, MD, FAAP

CASE STUDY
An expectant mother visits you. She has been advised by Questions
her obstetrician that a sonogram shows she is pregnant 1. What is the incidence of twin births?
with twins. She asks about care of twins and what spe- 2. What is the difference between fraternal and iden-
cial considerations she should keep in mind as she looks tical twins?
forward to the delivery. In particular, she is concerned 3. What major medical problems may affect twins and
about the feeding schedule and whether she will be able higher-order multiples?
to breastfeed. 4. What developmental and behavioral problems are
associated with raising twins?

With the advent of artificial reproductive therapy 40 years ago, Because of the increased risk of infertility with advanced mater-
the incidence of twins and higher-order multiples has increased. nal age, more couples are choosing to conceive with assisted repro-
Counseling the parents of multiples provides a unique opportunity ductive technologies, including ovulation-stimulating drugs, IVF,
for pediatricians. Much of what is known about caring for multiples and intracytoplasmic sperm injection. More than one-third of twins
comes from work with twins. and more than three-quarters of triplets and higher-order multi-
Parents of multiples often have many questions about the care of ples in the United States resulted from conception assisted by fer-
their children, but they rarely pose them to health care profession- tility treatments.
als. In a study in which 18 out of 29 mothers breastfed their twins, The overall natural prevalence of twin births is about 33 in 1,000.
only 3 received information about breastfeeding from their physi- Twins account for just slightly more than 1% of all births, and 20% of
cians. One mother had been told by her obstetrician that she could neonates born at fewer than 30 weeks’ gestation are twins. The aver-
not breastfeed her twins. Physicians should become knowledgeable age prevalence of monozygotic or identical twins, which is the same
about caring for multiples and the unique challenges they present for all women regardless of race and age, is about 1 in 300 births.
to parents related to feeding, sleeping, and behavior. The incidence of dizygotic or fraternal twins varies among different
groups and by method of conception. In the United States, blacks
Epidemiology and whites have comparable incidences of live-born twin deliveries,
On July 25, 1978, Louise Brown was the first baby born as a result of and both have significantly higher rates than Hispanic women. A
in vitro fertilization (IVF). Since then, improved prenatal care and maternal family history of dizygotic twins correlates most strongly
IVF methods have contributed to the increase in multiple births. with an increased incidence of twins. A family history of monozy-
According to the American Society for Reproductive Medicine, the gotic twins or a paternal family history of dizygotic twins does not
rate of twins has increased more than 75% over the last 40 years increase the likelihood of twins.
in the United States. Twin rates have also increased in Finland,
Norway, Austria, Sweden, Australia, Hong Kong, Japan, Canada, Pathophysiology
Singapore, and Israel, with the highest rate increase in Nigeria. A Higher-order multiples, conceived naturally or via artificial repro-
major contributor to the increase in multiple births is that women ductive technology, may be fraternal or a combination of monozy-
are starting their families later than in previous generations. The gotic twins and fraternal siblings. Monozygotic twins result from
Centers for Disease Control and Prevention reports that from 1980 the splitting of a single egg. They may share a placenta (mono-
to 2009, twin birth rates increased 76% for women aged 30 to chorionic) (Figure 26.1A and 26.1B) and, in rare cases, may also
34 years, nearly 100% for women aged 35 to 39 years, and more share an amniotic sac (monoamnionic). When splitting occurs early
than 200% for women aged 40 years and older. (after several cell divisions of the zygote), each fetus develops its own

167

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 168 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Differential Diagnosis
Diagnosing multiples is not difficult, but physicians should be aware
of the problems these newborns may experience.

Perinatal Complications
A B
Multiple births are associated with a significantly higher risk of
perinatal complications relative to singleton births. The maternal
complication most commonly reported with multiples is pregnancy-
induced hypertension. Maternal preeclampsia rates are higher in
twins and increase nearly 5-fold with triplets. In addition, mothers of
twins who conceived via IVF have a higher rate of preeclampsia than
C D
mothers of twins who conceived naturally. Other maternal com-
Figure 26.1. Variations in placentas in twin births. A, Monochorionic placenta, plications include placenta previa, antepartum hemorrhage, gesta-
cords close together. B, Monochorionic placenta, cords farther apart. tional diabetes mellitus, anemia, uterine atony, and maternal death.
C, Dichorionic placentas, separate. D, Dichorionic placentas, fused. Monozygotic twins are at increased risk for death and cerebral
palsy because of complications such as severe birth weight discor-
chorion and amnion, leading to dichorionic and diamnionic placen- dance and twin transfusion syndrome (TTS). Twin transfusion syn-
tas. Dizygotic twins result from 2 eggs, with each egg fertilized by drome is seen in 10% to 15% of monochorionic pregnancies and
a different sperm. Dizygotic twins have 2 placentas (Figure 26.1C). results from unbalanced blood flow due to vascular anastomoses
Although these placentas may fuse together like 2 pancakes, they within the shared placenta. The diagnosis is suspected by ultra-
are almost always dichorionic and diamnionic (Figure 26.1D). sound when 1 fetus is growth restricted with oligohydramnios and
Ultrasonography at 14 weeks or sooner has been found to be the other fetus has evidence of volume overload with polyhydram-
96% predictive of monochorionic twins and can also predict mono- nios. Both twins are ultimately at risk for fetal hydrops or death.
amnionic twins, anomalies, and syndromes. Without treatment, TTS-induced death of at least 1 twin is as high as
Following birth, many parents want to determine whether twins 80% to 100%. Of additional concern, the death of 1 twin is
are monozygotic or dizygotic. Different-sex twins are almost always associated with neurologic damage or subsequent death of the sur-
dizygotic, although monozygotic twins of different sexes have been viving twin, with 1 in 10 surviving twins developing cerebral palsy.
reported in the literature. This occurs when 1 twin loses a Y chro- Until recently, treatment was drainage of amniotic fluid in the
mosome and becomes a phenotypic female with Turner syndrome twin with polyhydramnios to reduce the risk of preterm delivery.
(XO). Occasionally, the male twin may have an XXY chromosome Endoscopic laser ablation of placental anastomoses has emerged as
complement and have Klinefelter syndrome. the treatment of choice for severe TTS pregnancies diagnosed and
To determine whether twins are monozygotic or dizygotic after treated prior to 26 weeks’ gestation. Laser ablation addresses the
birth, a number of procedures can be undertaken. Visual or patholog- primary pathology and results in an average gestation at delivery
ical examination of the placenta is helpful. About two-thirds of mono- of 33 weeks, which is a significant improvement from the average
zygotic twins have a common chorion and share 1 placenta. Finding 29 weeks with serial amniotic fluid reductions. Laser ablation is not
a single chorion usually means the twins are monozygotic, unless the without risks, however; reported complications include premature
placentas of dizygotic twins have fused together. DNA testing is the rupture of membranes, amniotic fluid leakage into the maternal
preferred method for determining zygosity. Most commercially avail- peritoneal cavity, vaginal bleeding, and chorioamnionitis.
able testing, often referred to as zygosity testing, involves examining One percent of monozygotic twins are monochorionic–
DNA obtained from buccal swabs from each child. Identification of monoamnionic. Although monoamnionic twins have a lower risk
short tandem repeats via polymerase chain reaction is typically accu- of TTS, they are at very high risk for cord accidents. Monoamnionic
rate 99% of the time and is similar to techniques used in forensic pregnancies are monitored closely, and once the fetuses reach via-
medicine. Commercial laboratory charges range from $100 to $200, bility, emergent delivery is indicated if fetal distress is noted to avoid
although prices vary significantly depending on the company and fetal death. Because fetal death significantly increases the risk of cere-
whether zygosity is being tested for twins or higher-order multiples. bral palsy and other neurologic disorders in the surviving twin, it is
Recent research on monozygotic twins has also focused on the no longer recommended to allow fetal death in 1 monoamnionic
effect of epigenetics, or how the environment affects genetics. By ana- twin to lengthen the gestation of the non-distressed twin.
lyzing the DNA of monozygotic twins, researchers are hoping to iden-
tify epigenetic tags that mark a change in gene expression. Although Congenital Malformations
DNA cannot be altered, DNA methylation, which affects the strength Twins and higher-order multiples have an increased risk of anom-
of gene expression, may be a process that, in the future, can be manip- alies. Monochorionic twins have a higher risk of cardiac anomalies
ulated to reverse some complex disorders, such as autism spectrum than dichorionic twins, increasing their need for fetal echocar-
disorder. diograms. Multiples conceived via IVF or intracytoplasmic sperm

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 Chapter 26: Caring for Twins and Higher-Order Multiples 169

injection have increased risk for anomalies and aneuploidy. Evaluation

Disorders of genetic imprinting (eg, Beckwith-Wiedemann syn-
drome, Angelman syndrome) are also increased with intracyto-
History
plasmic sperm injection. These genetic complications are thought A general medical history should be obtained, including a history
to be secondary to the underlying cause of infertility instead of arti- of the pregnancy. Any specific medical concerns should also be
ficial reproductive technologies. Because the costs associated with addressed (Box 26.1).
infertility treatments are substantial, many couples choose not to
Physical Examination
obtain a comprehensive chromosomal analysis looking for deletions
or translocations on themselves prior to using reproductive tech- The initial evaluation of newly born twins involves assessment of
nologies. Nuchal translucency and chorionic villus sampling can gestational age and determination of the presence of any medical
be used to detect and confirm suspected aneuploidy as early as the problems or anomalies (see Chapter 23). Older twins may undergo
first trimester. health supervision visits at which routine as well as specific con-
In addition to increased risks of malformations, multiples are at cerns are addressed.
risk for deformations secondary to crowding, including torticollis, Laboratory Tests
hip dislocation, plagiocephaly, and foot deformities. Monochorionic
Newborns should be assessed for the presence of anemia or polycy-
twins are also at risk for becoming conjoined twins, estimated to
themia with a hemoglobin level determination. Hypoglycemia may
occur in 1 in 50,000 to 200,000 gestations, with more than 50%
occur in the newborn period and should be assessed frequently until
dying in utero or being stillborn and 35% dying within the first
glucose levels are stable. If the twins are preterm, they may have
24 hours after birth. Although monochorionic twins are more com-
many of the problems seen in singleton preterm newborns, such as
monly males, conjoined twins are often female. Conjoining occurs
neonatal respiratory distress syndrome and necrotizing enterocolitis.
because of incomplete splitting of the embryo or after a secondary
Older children should receive an appropriate evaluation for age,
fusion between 2 previously separate embryos. Prenatal ultrasonog-
with a specific focus on any behavioral concerns.
raphy is commonly used to diagnose this condition; more recently,
prenatal magnetic resonance imaging has been used to evaluate spe-
Management
cific anomalies. Due to surgical advances, some parents continue
the pregnancy with hopes the live-born neonates can be separated. The focus of the management of multiples involves counseling
parents about issues related to routine care and anticipated stress
Postnatal Complications of caring for more than 1 newborn simultaneously. Parents often
Approximately 60% of twins and 90% of triplets are born preterm, care for 2 or more children at the same time, but multiples present
increasing the risk of morbidity and mortality. On average, most unique issues related to multiple children who are developmentally
single pregnancies last 39 weeks, twin pregnancies 36 weeks, trip- and chronologically at the same point. Many parents experience
lets 32 weeks, quadruplets 30 weeks, and quintuplets 29 weeks. The anxiety about the upcoming challenges they will face. Baby care
most common cause of preterm delivery in these neonates is pre- books that specifically discuss birthing and raising multiples are
mature rupture of membranes. available. Support groups and websites also provide information
Although growth in twins tends to be normal until 30 to 34 weeks’ for families of multiples.
gestation, growth restriction is commonly associated with multiple Feeding multiple newborns is often an exhausting challenge for
births. Twins conceived by IVF are more likely to be born at a low parents, and consultation with a lactation expert is recommended.
birth weight than spontaneously conceived twins. Smaller twins have The physical demands of feeding multiple newborns are often com-
an increased incidence of hypoglycemia in the newborn period and pounded by women recovering from prenatal complications such
have higher rates of targeted learning deficits and school failures as preeclampsia. Because preterm birth is common, newborns may
during childhood. In addition, children born with growth restric- have an immature suck reflex, making feeding more challenging.
tions have an increased risk for obesity and diabetes in later life. Few multiples are still exclusively breastfed by 4 months of age, and
Selective intrauterine growth restriction (IUGR), when only 1 twin common reasons cited for unsuccessful breastfeeding of multiples
is affected by IUGR, occurs in 10% of monochorionic twins. This
occurs when the twin with IUGR has reversed flow or persistent
absent flow in the umbilical artery.
Neonatal mortality is 4-fold higher in twins and 15-fold higher Box 26.1. What to Ask
in higher-order multiples. Neonatal morbidity is much higher in Caring for Twins
multiple births because of the increased incidence of preterm birth ww Did the mother have any problems during the pregnancy?
and growth restriction. The risk of these complications increases ww How long did the pregnancy last?
with the number of fetuses. The perinatal mortality of monozygotic ww Did the mother take any medications, including fertility drugs?
twins is 8 times that of singletons and 4 times that of dizygotic twins. ww Did the children have any problems after the delivery or in the newborn
Twins and higher-order multiples are also at increased risk for cere- nursery?
bral palsy.

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 170 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

include maternal stress, depression, fatigue, perceiving they were recommended, room sharing, in which infants sleep in the same
producing insufficient milk, and time burden. Despite these obsta- room as parents, is recommended for infants 6 to 12 months
cles, parents should know that breastfeeding is possible even for of age.
triplets and understand the benefits of breastfeeding to the new- Travel can be challenging with twins and higher-order multiples.
borns’ health. It is recommended that mothers begin breastfeed- Planning ahead is the key to making this a positive and construc-
ing as soon as possible to establish their milk supply. An electric tive experience. Having a separate diaper bag in each car and addi-
pump is a useful adjunct to help establish and continue breast- tional car seat adapters may make travel easier, but safety should
feeding. If supplemental nutrition is needed, utilizing a medicine never be compromised. It is important to counsel parents about
dropper, syringe, spoon, cup, or finger feeding instead of a bottle car safety seats. Parents who cannot afford the cost of multiple car
will reduce the risk of newborns developing a preference for an safety seats may resort to placing 1 baby in the car safety seat and
artificial nipple. the others on the seat of the car, a dangerous practice that is illegal
Some options for breastfeeding are outlined in Box 26.2. throughout the United States. Parents should be advised of the need
Sleeping in the same crib is no longer recommended because for car safety seats for all babies.
of concerns about co-sleeping. Approximately 3,500 babies die In the interest of safety, bathing should be done separately until
from sleep-related deaths per year. Although co-sleeping is not babies can sit up.

Box 26.2. Breastfeeding Twins and Higher-Order Multiples

1. Position
a. Babies may be put in a number of positions. Regardless of position, it is important to make sure that the baby’s head and neck are supported just enough
so the baby can adjust its head to breathe during breastfeeding. The mother can be sitting and cradling (across the chest) 1 baby while holding the other
like a football. Alternatively, the mother may cradle both babies so that they are cross-positioned over one another with their legs make an X across the
mother’s lap, or she may have both under her arms in a football position. Breastfeeding pillows designed for feeding multiples are helpful, but several firm
pillows are also sufficient.
b. Maternal positions can be sitting up with pillow back support, reclining, or lying down. To help prevent maternal fatigue, reclining and lying down are preferred.
2. Timing of Feeds
a. Feed both babies at the same time, with 1 on each breast. This method is recommended only after adequate breastfeeding has been established in each
twin, typically not until after 1 week after birth. It is advised that the baby having fewer problems latching be placed on the breast first so that the milk
ejection reflex is already established for the poorer-feeding baby. Because the babies may not drink the same amount, it is important that they feed on the
opposite breast for the next feeding to maximize milk production.
b. Feed the babies at different times. The mother breastfeeds 1 and then the other, starting with the more vigorous feeder. This approach may
pose a problem; both babies may be hungry at the same time, and maternal fatigue may inhibit success because the chance for sleep is
diminished with this method. Some mothers prefer this method because it provides individualized attention and opportunities for attachment with
each baby.
c. Breastfeed 1 baby and bottle-feed the other, which gives the mother a free hand. Most parents use a combination of breastfeeding and bottle-feeding.
A mother can let the first baby feed on demand but awaken the second when the first is done. Eighty percent of parents of twins acknowledge that they
prop the bottle instead of holding it, and they should be counseled against this. There are several effective feeding positions that do not require bottle
propping, and parents should be encouraged to adopt 1 of these positions.
3. Higher-order Multiples
Breastfeeding triplets and higher-order multiples is even more challenging than breastfeeding twins. Mothers of triplets exclusively breastfeeding report they
feed 2 babies, 1 on each breast, and the third follows on both breasts. Because the hind milk has higher fat content, it is important to rotate the feeding order
of the babies. Another popular option is to breastfeed 2 and have the third bottle-feed simultaneously.
4. Maternal Considerations
a. Feeding twins or higher-order multiples may decrease the amount of sleep a mother gets. Because sleep deprivation is associated with maternal depres-
sion and exclusively breastfeeding mothers of multiples report more sleep problems compared with mothers who do not breastfeed or who formula-feed
and breastfeed, these factors should be discussed when offering feeding options to parents. Encouraging paternal involvement with feeding may alleviate
some maternal stress and sleep deprivation.
b. Maternal nutrition plays a key role in quality and quantity of milk produced. Research shows that the higher the nutrition value of the mother’s diet, the
higher the nutrition quality of her milk. According to the Centers for Disease Control and Prevention, the mother should increase her calorie intake by 450
to 500 kcal per baby per day. In addition, a multivitamin may be recommended.

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 Chapter 26: Caring for Twins and Higher-Order Multiples 171

Toilet training is reportedly easier with some twins because 1 less time for individual facilitated play, which is helpful for language
twin learns from the other via modeling or peer pressure. development. If twins communicate with a private language, they
Maintenance of individuality for multiples may be challenging. have an increased risk of language and cognitive delays. Language
Researchers suggest that mothers can bond to only 1 newborn at a delays usually become much less pronounced by mid-childhood.
time, a concept called monotropy. In addition, mothers bond more Attention-deficit/hyperactivity disorder is more common in twins
strongly with the twin who leaves the hospital first. Twins are often but not as common as language delay. The smaller twin has a higher
dressed alike and given similar names because this may facilitate rate of specific learning deficits and school difficulties. The temper
the bonding process. To help with individual development, physi- tantrums multiples display can be severe and are understandable
cians are encouraged to obtain history and examine each child indi- given the heightened need to gain parental attention from their sib-
vidually. Physicians should attempt to distinguish the children from ling. Having twins in the home is a risk factor for child abuse, either
one another independent of parental reminders. Twins should not to a twin or to their siblings.
be referred to as “the twins” but by their respective names. As mul- The risk of cerebral palsy is increased with higher multiples
tiples get older, issues related to classrooms and birthday parties fre- because of the increased rates of preterm birth and IUGR. Fetal
quently arise, and they should be consulted about their preferences. death of a co-multiple and monochorionic placentas are the biggest
Individual birthday parties and gifts should be considered, as some risk factors for developing cerebral palsy.
multiples comment on their disappointment at receiving the same The effect on families of twins and higher-older multiples starts
present for birthdays and holidays. There is no surprise in opening prior to delivery and includes higher risk to maternal health as well
gifts if the other twin opened a gift first. as anticipatory anxiety. Prior to delivery, parents should be made
Whether multiples should be placed in the same or different aware that maternal depression is frequently reported after the birth
classrooms is unclear. Placement in different classrooms or schools is of multiples. The physical stress of caring for multiple newborns
advocated to support each child’s individual development. However, can be overwhelming, and parents often feel isolated at home. The
being in the same classroom ensures the same educational standard incidence of depression is higher in mothers of twins than of sin-
and may be more convenient for families. Research is variable on gletons, with sleep deprivation cited as a contributing factor. It is
whether separate or same classrooms are better or even make a dif- important to suggest that parents of multiples obtain outside aid
ference for twins with regard to emotional and academic outcomes. to decrease fatigue and increase their ability to experience respite
Some studies do suggest that boys do better emotionally when they even for short periods.
are together versus separated. It is currently recommended that In addition to exhaustion and isolation, the increased financial
school districts have a flexible policy addressing school placement demands that accompany multiple newborns can strain the par-
of twins. Online resources are available to assist families and schools ents’ relationship. Mothers often have to leave work earlier in the
in determining the best school placement for twins. Families with pregnancy than women with singleton pregnancies, adding finan-
higher-order multiples are more likely to have children in separate cial burden, especially if they are single or have other children. The
schools because of the higher risk that some of the children will have medical costs associated with preterm birth can be substantial. To
developmental delays. cover for the loss of income at a time of increasing family expenses,
Sibling rivalry is common in all families but is different among the father is often required to work more hours. This increased occu-
co-multiples as well as other siblings within a family. Twins are often pational stress comes at a time when child care demands at home
directly compared to one another, while older siblings may resent have increased significantly. Financial burdens are especially diffi-
the attention paid to the new babies. Because twins often exclude cult with multiples conceived artificially because of the cost associ-
siblings and peers socially, parents are encouraged to schedule times ated prior to birth. This may be a contributing factor to why parents
when 1 twin and sibling are with 1 parent and the other twin is alone of twins conceived artificially have reported less satisfaction than
with another family member. Isolating twins (or 2 triplets from a parents of twins conceived naturally.
third) fosters more sibling interaction and less dependency between Grief is another significant contributor to parental depression.
the twins. These separations are encouraged to begin early in devel- Approximately 15% of children from multiple births grow up as a
opment because the later in development twins are separated, the singleton survivor. Birthday celebrations serve as reminders to the
less agreeable twins are to being apart. parents of the death of the other child. Parents grieving the loss of
Developmental differences among multiples may also contrib- 1 of their twins have comparable grief to those grieving the loss of a
ute to sibling rivalry. The child who is smaller or more delayed may singleton pregnancy. It is imperative that physicians caring for the
become jealous of the co-multiple. Conversely, typically develop- family acknowledge the parents’ grief because family and friends
ing children may become frustrated when the sibling with special often do not acknowledge the parents’ pain if there is a surviving
needs receives more attention than they receive. Parents and family newborn.
should be encouraged to acknowledge and praise individual char- Surviving children born preterm or small for gestational age are
acteristics of each twin. at increased risk of developmental delays. The stress of raising a child
Until age 3 years, language and speech development is delayed in with special needs is also a source of grief because parents mourn
twins relative to singletons. A contributing factor to language delay is the loss of their dreams of having a “normal” child.

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 172 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Prevention Selected References

Regardless if conceived naturally or via artificial reproductive tech- Chauhan SP, Scardo JA, Hayes E, Abuhamad AZ, Berghella V. Twins: prevalence,
nology, it is impossible to guarantee singletons or higher-order problems, and preterm births. Am J Obstet Gynecol. 2010;203(4):305–315 PMID:
multiples. Nonetheless, a maternal family history of dizygotic 20728073 https://doi.org/10.1016/j.ajog.2010.04.031
births increases the likelihood of higher-order multiples. Artificial Cowan JM, Demmer LA. Assisted reproductive technology and preimplan-
reproductive technology often results in higher-order multiples. tation genetic diagnosis: impact on the fetus and newborn. NeoReviews.
However, the number of embryos implanted via artificial repro- 2007;8(3):e127–e132 https://doi.org/10.1542/neo.8-3-e127
ductive technology has decreased following high-profile births of Flidel-Rimon O, Shinwell ES. Breast feeding twins and high multiples. Arch Dis
grand multiples that highlighted the financial difficulties placed Child Fetal Neonatal Ed. 2006;91(5):F377–F380 PMID: 16923939 https://doi.
org/10.1136/adc.2005.082305
on families, as well as the increased risk of developmental delays
in the children. Hansen M, Kurinczuk JJ, Bower C, Webb S. The risk of major birth defects
after intracytoplasmic sperm injection and in vitro fertilization. N Engl J Med.
2002;346(10):725–730 PMID: 11882727 https://doi.org/10.1056/NEJMoa010035
Prognosis
Hay DA, Preedy P. Meeting the educational needs of multiple birth children.
Although multiples have a higher prevalence of perinatal and post- Early Hum Dev. 2006;82(6):397–403 PMID: 16697537 https://doi.org/10.1016/j.
natal problems, appropriate anticipatory guidance, routine health earlhumdev.2006.03.010
maintenance, and treating each child as an individual can help fam- Klock SC. Psychological adjustment to twins after infertility. Best Pract Res Clin
ilies optimize their children’s outcome. Obstet Gynaecol. 2004;18(4):645–656 PMID: 15279823 https://doi.org/10.1016/j.
bpobgyn.2004.04.015
Martin JA, Hamilton BE, Osterman MJK, Driscoll AK, Drake P. Births: final data
CASE RESOLUTION for 2017. Natl Vital Stat Rep. 2018;67(8):1–50 PMID: 30707672 www.cdc.gov/
The mother is advised that breastfeeding is not only possible but recommended. nchs/data/nvsr/nvsr67/nvsr67_08-508.pdf
She is told about the options for timing and positioning of the newborns. Martino D, Loke YJ, Gordon L, et al. Longitudinal, genome-scale analysis of
The issues of family history and child passenger safety are also discussed, and DNA methylation in twins from birth to 18 months of age reveals rapid epi-
anticipatory guidance on the potential stress of raising multiples is given. genetic change in early life and pair-specific effects of discordance. Genome
Biol. 2013;14(5):R42 PMID: 23697701 https://doi.org/10.1186/gb-2013-14-5-r42
Miller P. A thing or two about twins. Natl Geogr Mag. 2012;1:1–6
Resources for Parents Moore JE. Q&As about multiple births. Contemp Pediatr. 2007;24:39–55
American Society for Reproductive Medicine Pharoah PO, Dundar Y. Monozygotic twinning, cerebral palsy and congenital
www.asrm.org anomalies. Hum Reprod Update. 2009;15(6):639–648 PMID: 19454557 https://
doi.org/10.1093/humupd/dmp019
Baby Center Polderman TJ, Bartels M, Verhulst FC, Huizink AC, van Beijsterveldt CE,
www.babycenter.com Boomsma DI. No effect of classroom sharing on educational achievement in
twins: a prospective, longitudinal cohort study. J Epidemiol Community Health.
Center for Loss in Multiple Birth
2010;64(1):36–40 PMID: 20007633 https://doi.org/10.1136/jech.2009.091629
www.climb-support.org
Shinwell ES, Haklai T, Eventov-Friedman S. Outcomes of multiplets. Neonatology.
Flais SV. Raising Twins. 3rd ed. Itasca, IL: American Academy of
2009;95(1):6–14 PMID: 18832859 https://doi.org/10.1159/000151750
Pediatrics, 2019
Sutcliffe AG, Derom C. Follow-up of twins: health, behaviour, speech, language
Luke B, Eberlein T. When You’re Expecting Twins, Triplets, or Quads:
outcomes and implications for parents. Early Hum Dev. 2006;82(6):379–386
Proven Guidelines for a Healthy Multiple Pregnancy. 3rd ed. New PMID: 16690232 https://doi.org/10.1016/j.earlhumdev.2006.03.007
York, NY: HarperCollins Publishers; 2011
Thorpe K. Twin children’s language development. Early Hum Dev. 2006;82(6):
Multiples of America 387–395 PMID: 16690234 https://doi.org/10.1016/j.earlhumdev.2006.03.012
www.multiplesofamerica.org
Neifert M. Dr. Mom’s Guide to Breastfeeding. New York, NY: Penguin
Group; 1998
Raising Multiples
www.raisingmultiples.org

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 CHAPTER 27

Male Circumcision
Jung Sook (Stella) Hwang, DO, FAAP, and Lynne M. Smith, MD, FAAP

CASE STUDY
An expectant mother learns that the sex of her fetus is Questions
male. She visits you prenatally. She talks about circum- 1. What are the benefits of male circumcision?
cision in addition to issues related to breastfeeding and 2. What are the indications for circumcision in older
car passenger safety. Her husband is circumcised. She is children?
unclear about the medical indications for circumcision 3. What are the techniques used to perform circumcision?
and asks your opinion about circumcision in the new- 4. What are the complications of circumcision?
born period. 5. What is the current status of insurance coverage of
circumcision?

Male circumcision, a procedure in which the foreskin of the penis is Circumcision in newborns has been performed in a routine and
removed, has been performed for more than 6,000 years. It is rou- preventive manner, much the same way immunizations are adminis-
tinely performed in certain groups, most notably among Jewish and tered. Primary care physicians should be aware of the risks and ben-
Muslim people. In some other cultures (eg, Australian [Aborigine], efits of the procedure to enable them to counsel parents and make
Polynesian), circumcision is presumably performed to facilitate referrals to consultants should certain medical conditions arise.
intercourse. Circumcision can be viewed as a ritual procedure, but
its role as a medical procedure has long been controversial. Epidemiology
The benefits of male circumcision have been debated for years. In
The prevalence of neonatal circumcision, a procedure that became
the past 20 years, even the American Academy of Pediatrics (AAP) has
increasingly popular in the United States in the 1950s and 1960s,
changed its official position on the medical indications for circumci-
once ranged from 69% to 97% depending on cultural mores. In the
sion. The AAP stated in 1999 and reaffirmed in 2005 that circumcision
United States, the procedure is commonly performed during the
carried potential benefits, although the procedure was not medically
newborn period, and it is the most common surgical procedure
indicated. However, in 2012 the AAP released an updated policy stat-
performed in the country. During the past decade, the circumci-
ing that the health benefits of circumcision outweigh the risks of the
sion prevalence in males aged 14 to 59 years increased from 79% to
procedure. This statement was based on a systematic evaluation of
81%; specifically, 91% in white males, 76% in black males, and 44%
peer-reviewed literature that demonstrated preventive health bene-
in Hispanic males. The estimated prevalence of circumcision for
fits of elective male circumcision, including reductions in the risk of
Australian-born men is 59% (newborn rate estimated 10%–20%);
urinary tract infections (UTIs) in the first year after birth, decreased
in Canada, 32% of men; and in the United Kingdom, 15% of men. A
risks of heterosexual acquisition of HIV and other sexually transmitted
reported 10% of uncircumcised newborn males ultimately require
infections, and a decreased incidence of penile cancer. Additionally,
circumcision as adults because of complications of phimosis and
the statement noted that male circumcision does not adversely affect
balanitis. Uncircumcised males with diabetes are particularly prone
penile sexual function/sensitivity or sexual satisfaction and that com-
to these complications.
plications related to circumcision are infrequent and rarely severe.
Disadvantages of routine circumcision in newborns include
expenses associated with the procedure and the risk of complica- Clinical Presentation
tions; however, some analyses have demonstrated that neonatal male Most often, parents will query their child’s pediatrician about the
circumcision is cost-effective in that it reduces the risk for future advisability of circumcision, and the newborn will not have any clin-
disease. The procedure is sometimes criticized as an archaic and ical symptoms suggestive of a need for the procedure. Older infants
maiming ritual. Female circumcision, which may involve clitori- and children in need of circumcision may present with symptoms of
dectomy or resection and closure of the labia minora or majora, is phimosis, in which the foreskin balloons out on urination; paraphi-
infrequently practiced in Western culture and is not discussed in mosis, in which a retracted foreskin cannot be returned to its nor-
this chapter other than to emphasize that female circumcision has mal position; or recurrent problems of infection or inflammation of
no medical benefit. the foreskin (posthitis), glans (balanitis), or both (balanoposthitis).

173

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 174 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Pathophysiology Table 27.1. Ability to Retract the Foreskin

In uncircumcised males, the foreskin adheres to the glans until in Boys, by Age
approximately age 6 years, after which a gradual, normal lysing of Age Percentage With Retractable Foreskin
the adhesive bands connecting the foreskin to the underportion Birth 4
of the glans occurs. Nonphysiologic phimosis occurs as a result of 6 months 15
scarring of the preputial wing. Lysing of adhesions in an attempt to
1 year 50
treat the phimosis usually results in additional adhesions. If the fore-
skin is retracted and remains in that position, paraphimosis occurs. 3 years 80
6 years 90
Differential Diagnosis
The differential diagnosis relates to conditions that may be man-
aged with circumcision, including phimosis, paraphimosis, and foreskin. In male infants beyond the newborn period, phimosis is
infection and inflammation of the penis. Conditions such as hypo- the major indication for circumcision. Phimosis is normal in chil-
spadias may be mistaken for a partially circumcised penis, because dren up to approximately 6 years of age but is nonphysiologic if
the condition is associated with absence of the ventral foreskin. urination results in ballooning of the foreskin, regardless of age.
A careful patient history will differentiate hypospadias from a When the retracted foreskin acts as a tourniquet in the mid-shaft of
circumcision. the penis, paraphimosis occurs, preventing the return of lymphatic
flow. Paraphimosis is commonly related to traumatic retraction of
Evaluation the foreskin, typically during cleaning or by medical personnel dur-
ing bladder catheterization. Because of this, parents are no longer
History advised to retract the foreskin in an effort to lyse adhesions. The
In cases involving newborns, the history should include the pres- incidence of paraphimosis is increasing in adults secondary to body
ence of any coagulopathies among family members, which would piercing. Newly placed penile rings can cause pain sufficient to pre-
preclude the performance of circumcision. In older infants and vent foreskin retraction.
children, the history should include problems related to voiding, Balanitis, or inflammation of the glans, is not uncommon in
such as ballooning of the foreskin or difficulty initiating the uri- infants. It is frequently associated with Candida infection, and the
nary stream. glans is swollen and erythematous. Posthitis, or inflammation of
the foreskin, is also often secondary to Candida infection. Other
Physical Examination
organisms, including gram-negative microbes, may be associated
The physical examination should assess the genitalia, with particular with balanitis. The presence of recurrent balanitis is an indica-
emphasis on determining whether evidence exists of hypospadias, tion for circumcision. In older males, indications for circumci-
in which the urethral orifice is not located at the tip of the glans. In sion include phimosis, paraphimosis, balanitis, posthitis, and
such a situation, circumcision should be delayed because the fore- balanoposthitis.
skin is used to reconstruct the urethra. In the patient with phimo- Urinary tract infections reportedly occur 10 times more often in
sis, the degree of phimosis also should be assessed. uncircumcised infants than circumcised infants (1 in 100 and 1 in
1,000, respectively). In young uncircumcised boys, UTIs are directly
Laboratory Tests
related to colonization of the foreskin with urotoxic organisms.
Routine laboratory tests are not indicated, although a urinalysis may Pyelonephritogenic, fimbriated Escherichia coli bind to the inner
be obtained in children with a history of a prior UTI. Coagulation lining of the foreskin within the first few days after birth. Other bac-
studies are appropriate in the case of a family history of a bleeding teria preferentially bind to this mucosal surface, including fimbri-
disorder. ated strains of Proteus mirabilis and nonfimbriated Pseudomonas,
Klebsiella, and Serratia species. According to several studies, the
Imaging Studies
rate of UTIs has increased as the rate of circumcision has. A 2012
Imaging studies are not indicated in most children undergoing cir- meta-analysis of 22 studies examining the incidence of UTIs in
cumcision. Such studies are relevant, however, should concern exist males found a 23% lifetime risk of UTI in uncircumcised males
that the urogenital anatomy might be abnormal. compared with 8.8% in circumcised males. Thus, the lifetime risk
of UTI markedly exceeded the 1.5% circumcision complication rate;
Benefits most of these complications were minor. Complications associated
It has been stated that circumcision facilitates penile hygiene by with UTIs have been reported, in particular, bacteremia, menin-
removing the foreskin, which may serve as a repository for bac- gitis, and death.
teria, smegma, and dirt. Ability to retract the foreskin increases The annual incidence rate of penile cancer is 0.58 per 100,000
with age (Table 27.1); thus, penile hygiene is easier to achieve in men in the United States but is 2.9 to 6.8 per 100,000 men in
older children. The term phimosis refers to inability to retract the Brazil. These regional differences are thought to be related to lack

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 CHAPTER 27: Male Circumcision 175

of circumcision of the penis. Only a few isolated cases of cancer to neonates from such a painful procedure. Local anesthesia mini-
of the penis occur in circumcised men. Phimosis is strongly asso- mizes this effect. Parents should be informed about the benefits of
ciated with invasive penile cancer, with other cofactors such as circumcision, including a reduction in the occurrence of UTIs,
human papillomavirus (HPV) infection and poor hygiene possi- sexually transmitted infections, and cancer of the penis and cervix.
bly contributing. Smoking is consistently associated with penile Problems related to the foreskin itself, such as phimosis, paraphimo-
cancer and is further reason to strongly advocate for smoking ces- sis, posthitis, and balanitis, also should be discussed.
sation programs. It is appropriate to tell parents that boys who are not circum-
Cervical carcinoma among the partners of uncircumcised men cised in the neonatal period may need to be circumcised later in life.
is increasingly being reported. In addition, current partners of cir- Parents should be informed about the risks associated with circum-
cumcised men with a history of multiple sexual partners have a cision in newborns, which are discussed later in the chapter. In older
lower risk of cervical cancer than partners of uncircumcised men. individuals, risks of circumcision include hemorrhage, infection,
Circumcision in adolescent boys and men in Uganda was associ- and injuries to the penis and urethra. In addition, parents should
ated with a marked decreased incidence of HPV and human her- be informed that approximately 2% of circumcised neonates require
pesvirus 2 infection. Circumcision has also been associated with a a second circumcision because of inadequate foreskin removal
reduced risk for HIV infections. Three randomized, controlled trials during the first procedure.
conducted in South Africa, Kenya, and Uganda confirmed the find- Research has shown that parents are more influenced by the
ings of observational studies that circumcision is protective against circumcision status of the father, religion, and ethnicity than by
HIV infection. In addition, circumcision was not associated with physician attitude concerning their ultimate decision about circumci-
increased HIV risk behavior. Based on these findings, in 2007 the sion. The 2012 AAP policy statement emphasizes that parents should
World Health Organization stated that male circumcision should consider health benefits and risks in conjunction with their own reli-
be part of a comprehensive strategy for HIV prevention. Since the gious, cultural, and personal preferences, because the medical bene-
World Health Organization made this recommendation, nearly fits alone may not outweigh these other considerations for individual
15 million voluntary male circumcision have been performed for families. Counseling during the second trimester of pregnancy results
HIV prevention in 14 countries of eastern and southern Africa, in no change in parents’ decision about circumcision. Parents of
which is estimated to help prevent more than 500,000 new HIV newborn boys should be instructed in the care of the penis at the
infections through 2030. It remains critical, however, to promote time of discharge from the newborn hospital stay, regardless whether
the practice of safe sex, because circumcision confers only partial the newborn is circumcised.
protection against HPV, human herpesvirus 2, and HIV.
Parents of Infants and Older Children
Risks The need for circumcision in male infants who present with UTIs
Risks related to circumcision are related to complications of the pro- is problematic. Patient evaluation involves an attempt to determine
cedure (Box 27.1) and are discussed in the Management section of the existence of other conditions that may predispose the patient to
this chapter. Elective circumcision should be performed only if the a UTI. Investigators disagree on the need for circumcision after an
newborn is stable and healthy. initial UTI in uncircumcised boys. No evidence definitively indicates
that circumcision at this time decreases the incidence of future UTIs;
Parental Counseling thus, the decision whether to circumcise is based on parental pref-
erence rather than medical evidence. In children who present with
Parents of Newborns significant phimosis or paraphimosis, circumcision is usually recom-
In the newborn period, proper counseling of parents is impor- mended to prevent recurrence of these problems. Medical manage-
tant, including a discussion of the risks and benefits of circumci- ment, including the use of topical steroids for phimosis, may obviate
sion. Opponents to neonatal circumcision cite psychological trauma the need for surgery in some children. Such treatment involves the
daily external application of betamethasone cream from the fore-
skin tip to the corona glandis for 4 to 6 weeks. If the frenulum is tight
and tearing, local anesthetic can be applied and the frenulum can
Box 27.1. Complications Associated
be transected. Patient history, including the duration of symptoms
With Circumcision
and whether the child has had similar episodes in the past, helps in
ww Bleeding ww Penile necrosis formulating appropriate management.
ww Inclusion cysts ww Phimosis
ww Infection ww Repeat circumcision
Management
ww Meatal stenosis ww Skin bridges
ww Meatitis ww Urethrocutaneous fistulae The medical attitude toward circumcision has changed in the past
ww Penile cyanosis ww Urinary retention 40 years, with an initial inclination toward circumcision, followed by a
ww Penile lymphedema ww Wound dehiscence move away from circumcision. The present position on circumcision
as described by the AAP Task Force on Circumcision suggests that

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 176 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

newborn circumcision has potential medical benefits and advantages using tissue glues has been associated with reduced surgical time
as well as disadvantages and risks. When circumcision is considered, and improved cosmetic outcome.
benefits and risks should be explained to parents, and informed con-
sent should be obtained. Parents should be advised that third-party Post-Procedure Care
payers may deny payment for circumcisions, particularly for routine Oral acetaminophen can be administered for apparent pain or irri-
circumcisions in the newborn period. tability. Analgesia is rarely needed after 24 hours. The parent or
caregiver should be advised to watch for and report any bleeding or
Contraindications signs of infection. Mild redness and a yellowish crust may persist
Circumcisions should be performed only in completely healthy for 1 week. To keep the glans from sticking to the diaper, the par-
neonates. Contraindications to circumcision are well defined. Any ent or caregiver should apply a smear of petrolatum to the front of
abnormalities of the penis, such as hypospadias, absence of any the diaper for 1 week.
portion of the foreskin, or chordee, preclude circumcision.
Atypical genitalia and preterm birth are contraindications as well.
Complications
Circumcision should be delayed in preterm and ill term newborns Several complications are associated with circumcision in newborns
until they are ready for discharge from the hospital. Patients with a (Box 27.1). The most common complication is bleeding, which may
personal or family history of bleeding diathesis should not be cir- occur in 0.2% to 8% of patients. Typically, bleeding can be con-
cumcised. Newborns from such families should be assessed for evi- trolled using local pressure. More significant bleeding may require
dence of coagulation problems and, if present, circumcision should local pressure with 1:1,000 adrenaline-soaked gauze or with the use
not be performed. of other topical agents (eg, Surgicel, QuikClot). The second most
common complication is infection, which is reported in up to 8%
Circumcision Procedure of circumcised newborns. The Plastibell device is associated with a
Numerous techniques are used to perform circumcisions. These higher incidence of infection than the Gomco clamp. Local treatment
procedures may involve clamp techniques with the Gomco clamp, is sufficient to manage most infections, although intravenous antibi-
Mogen clamp, or Plastibell device. Any of these techniques is believed otics should be considered because neonatal sepsis and necrotizing
to give comparable results when performed by trained, experienced fasciitis may occur secondary to infection following circumcision.
operators. Formal surgical excision also may be performed, usually Poor cosmetic outcome is another complication of circumci-
in older children and adults. Three guidelines should be followed to sion. Phimosis may occur if removal of the foreskin is insufficient.
reduce the incidence of complications: marking of the coronal sul- Inadequate freeing of the foreskin from the inner preputial epithe-
cus in ink, dilation of the preputial wing, and retraction of the fore- lium may result in a concealed penis, with the shaft retracted back-
skin so that the urethral meatus is visualized to prevent cutting the ward into the abdominal wall. Skin bridges may form between the
meatus. Electrocautery should never be used in conjunction with glans and shaft, resulting in the accumulation of smegma or tether-
metal clamps because of the danger of extensive injury. ing of the erect penis. Most post-circumcision adhesions are reported
Appropriate anesthesia in newborns undergoing circumcision is to resolve at the time of puberty with the onset of masturbation or
the standard of care. The pain and stress of circumcision is evidenced sexual activity.
by changes in neonate state and behavior. Non-pharmacologic tech- In the immediate postoperative period, urinary retention may
niques, such as positioning and sucrose pacifiers, are insufficient occur secondary to tight surgical bandages. This complication can
to prevent procedural pain but may be used in conjunction with be prevented by applying local pressure rather than tight bandages
other forms of analgesia. Local anesthesia is the preferred method to obtain hemostasis. Meatitis and meatal ulcers, which may be
of pain management. Dorsal penile nerve block, which involves the the result of irritation from ammonia or damage to the frenulum
subcutaneous injection of a local anesthetic agent at the base of the artery at the time of circumcision, have been reported in circum-
penis, is effective in reducing pain responses during circumcision. cised males. Inclusion cysts that represent implantation of smegma
Ring block, which involves subcutaneous circumferential injections also have been reported. Additional injuries following circumcision
of a local anesthetic agent around the mid-shaft of the penis, has may include penile lymphedema, urethrocutaneous fistulae second-
also been shown to be effective and avoids the potential complica- ary to misplaced sutures, penile cyanosis, and necrosis secondary
tion of injecting local anesthesia toward the dorsal vessels. Topical to a tight Plastibell device. Wound dehiscence, which involves sep-
application of lidocaine and prilocaine cream (eg, EMLA, Lidopril) aration of the penile skin from the mucous membrane and denuda-
or oral sucrose solution on a pacifier also reduces pain and associ- tion of the penile shaft, may occur more frequently with the Gomco
ated stress but to a lesser extent than local anesthesia. Studies have clamp than the Plastibell device.
shown that blocks seem to be more effective than topical cream or Meatal stenosis appears as a pinhole urethra with an angulated
oral sucrose, and the ring block is better than the dorsal penile nerve or narrow urinary stream and may be associated with enuresis or
block. Infants who receive block anesthesia cry less and have less incontinence. Meatal stenosis is very rare in uncircumcised males;
tachycardia and irritability in addition to fewer behavior changes it is not clear whether circumcision is the direct cause of this rare,
during the 24 hours after the procedure. For older, prepubertal chil- possibly late complication. A chronic inflammatory process result-
dren who require formal surgical excision, sutureless circumcision ing from inadequate post-procedure care, the use of superabsorbent

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 CHAPTER 27: Male Circumcision 177

disposable diapers, urinary ammonia, or diaper dermatitis may con- Fergusson DM, Boden JM, Horwood LJ. Circumcision status and risk of sex-
tribute to the development of meatal stenosis. ually transmitted infection in young adult males: an analysis of a longitudinal
birth cohort. Pediatrics. 2006;118(5):1971–1977 PMID: 17079568 https://doi.
org/10.1542/peds.2006-1175
Prognosis
Kacker S, Frick KD, Gaydos CA, Tobian AA. Costs and effectiveness of neona-
The prognosis after circumcision is excellent, and complications are tal male circumcision. Arch Pediatr Adolesc Med. 2012;166(10):910–918 PMID:
exceedingly rare. 22911349 https://doi.org/10.1001/archpediatrics.2012.1440
Kim HH, Li PS, Goldstein M. Male circumcision: Africa and beyond? Curr
Opin Urol. 2010;20(6):515–519 PMID: 20844437 https://doi.org/10.1097/
CASE RESOLUTION MOU.0b013e32833f1b21
The risks and benefits of circumcision should be discussed with the mother. The Lane V, Vajda P, Subramaniam R. Paediatric sutureless circumcision: a system-
father should be encouraged to participate in the decision making process. If the atic literature review. Pediatr Surg Int. 2010;26(2):141–144 PMID: 19707772
parents elect to forgo circumcision for their son, they should be instructed on https://doi.org/10.1007/s00383-009-2475-y
the appropriate care of the uncircumcised penis, which involves gentle external
washing without retraction of the foreskin. Merrill CT, Nagamine M, Steiner C. Circumcisions performed in U.S. community
hospitals, 2005. HCUP Statistical Brief #45. Rockville, MD: Agency for Healthcare
Research and Quality; 2008. www.hcup-us.ahrq.gov/reports/statbriefs/sb45.pdf.
Accessed March 14, 2019
Selected References Minhas S, Manseck A, Watya S, Hegarty PK. Penile cancer—prevention and pre-
Alanis MC, Lucidi RS. Neonatal circumcision: a review of the world’s oldest and malignant conditions. Urology. 2010;76(2 suppl 1):S24–S35 PMID: 20691883
most controversial operation. Obstet Gynecol Surv. 2004;59(5):379–395 PMID: https://doi.org/10.1016/j.urology.2010.04.007
15097799 https://doi.org/10.1097/00006254-200405000-00026 Morris BJ, Bailis SA, Wiswell TE. Circumcision rates in the United States: ris-
American Academy of Pediatrics Task Force on Circumcision. Circumcision ing or falling? What effect might the new affirmative pediatric policy state-
policy statement. Pediatrics. 2012;130(3):585–586 PMID: 22926180 https://doi. ment have? Mayo Clin Proc. 2014;89(5):677–686 PMID: 24702735 https://doi.
org/10.1542/peds.2012-1989 org/10.1016/j.mayocp.2014.01.001

American Academy of Pediatrics Task Force on Circumcision. Male cir- Morris BJ, Wiswell TE. Circumcision and lifetime risk of urinary tract infection:
cumcision. Pediatrics. 2012;130(3):e756–e785 PMID: 22926175 https://doi. a systematic review and meta-analysis. J Urol. 2013;189(6):2118–2124 PMID:
org/10.1542/peds.2012-1990 23201382 https://doi.org/10.1016/j.juro.2012.11.114

Barkin J, Rosenberg MT, Miner M. A guide to the management of urologic dilem- Schoen EJ. Ignoring evidence of circumcision benefits. Pediatrics. 2006;118(1):
mas for the primary care physician (PCP). Can J Urol. 2014;21(Suppl 2):55–63 385–387 PMID: 16818586 https://doi.org/10.1542/peds.2005-2881
PMID: 24978632 Tobian AA, Serwadda D, Quinn TC, et al. Male circumcision for the prevention
Binner SL, Mastrobattista JM, Day MC, Swaim LS, Monga M. Effect of HSV-2 and HPV infections and syphilis. N Engl J Med. 2009;360(13):1298–1309
of parental education on decision-making about neonatal circumci- PMID: 19321868 https://doi.org/10.1056/NEJMoa0802556
sion. South Med J. 2002;95(4):457–461 PMID: 11958247 https://doi. Van Howe RS. Is neonatal circumcision clinically beneficial? argument against.
org/10.1097/00007611-200295040-00017 Nat Clin Pract Urol. 2009;6(2):74–75 PMID: 19153572 https://doi.org/10.1038/
Brady-Fryer B, Wiebe N, Lander JA. Pain relief for neonatal circumcision. ncpuro1292
Cochrane Database Syst Rev. 2004;(4):CD004217 PMID: 15495086 Warner L, Cox S, Kuklina E, et al. Updated trends in the incidence of circum-
Castellsagué X, Bosch FX, Muñoz N, et al; International Agency for Research on cision among male newborn delivery hospitalizations in the United States,
Cancer Multicenter Cervical Cancer Study Group. Male circumcision, penile human 2000–2008. Presented at: National HIV Prevention Conference; August 26,
papillomavirus infection, and cervical cancer in female partners. N Engl J Med. 2011; Atlanta, GA
2002;346(15):1105–1112 PMID: 11948269 https://doi.org/10.1056/NEJMoa011688 World Health Organization. WHO Progress Brief—Voluntary Medical Male
Dubrovsky AS, Foster BJ, Jednak R, Mok E, McGillivray D. Visibility of the ure- Circumcision for HIV Prevention in Priority Countries of East and Southern
thral meatus and risk of urinary tract infections in uncircumcised boys. CMAJ. Africa. Geneva, Switzerland: World Health Organization; 2017
2012;184(15):E796–E803 PMID: 22777988 https://doi.org/10.1503/cmaj.111372

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 CHAPTER 28

Nutritional Needs
Sara T. Stewart, MD, MPH, FAAP

CASE STUDY
At a routine health maintenance visit, a mother asks if Questions
she may begin giving her 4-month-old daughter solid 1. What are some of the parameters that may be used
foods. The infant is taking about 4 to 5 oz of formula to decide when infants are ready to begin taking
every 3 to 4 hours during the day (about 32 oz per day) solid foods?
and sleeps from midnight to 5:00 am without awaking 2. Up to what age is human milk or infant formula
for a feeding. Her birth weight was 3.2 kg (7 lb), and her alone considered adequate intake for infants?
present weight and length (5.9 kg [13 lb] and 63.5 cm 3. At what age do infants double their birth weight? At
[25 in], respectively) are at the 50th percentile for age. what age do they triple their birth weight?
The physical examination, including developmental 4. What allergy risks are associated with the early
assessment, is within reference limits. introduction of solid foods?

Good nutrition is essential for typical growth and development. The weight by 6 months of age and triple their birth weight by 12 months
physician plays an important role not only in assessing the growth of of age. Children aged 2 years to puberty gain approximately 5 to
children but also in counseling parents about the nutritional needs 10 g/day (0.2–0.4 oz/day). On average, children weigh about 10 kg
of maturing children. The primary care physician should be knowl- (22 lb) at 1 year of age, 20 kg (44 lb) at 5 years of age, and 30 kg
edgeable about key nutritional concepts for children, including typ- (66 lb) at 10 years of age. A rough rule that can be used to estimate
ical growth patterns and assessment of the child’s nutritional status, the expected weight of a child based on age is 2 × age (years) + 10 =
changing nutritional requirements and feeding patterns from infancy weight (kg). A prepubertal child who does not gain at least 1 kg/year
through adolescence, and common feeding and nutritional disorders. should be monitored for nutritional deficits.
Infants and young children grow about 25 cm (9.8 in) during
Growth Patterns and Nutritional the first postnatal year, 12.5 cm (4.9 in) during the second year, and
Requirements of Typical Children 6.25 cm (2.5 in) per year after that until puberty. This is followed by
the prepubertal-pubertal growth spurt. Girls grow 3 to 4 cm (1.2–1.6 in)
Monitoring the growth and nutritional status of infants and children
every 6 months and boys grow 5 to 6 cm (2.0–2.4 in) every
is an integral component of well-child care. The average expected
6 months during this period.
increases in weight, height, and head circumference for the first
several years after birth are listed in Table 28.1.
Feeding Patterns of Infants
The energy and nutritional requirements of children vary with
age. Postnatal growth is most rapid during the first 6 to 12 months
and Children
after birth; hence, caloric and protein needs are very high at this Liquid Foods
time. The average daily energy and protein needs of children from Human milk is generally recommended as the exclusive nutri-
birth to 18 years of age are presented in Table 28.2. ent for newborns and infants during the first 6 months after birth
On average, newborns weigh 3.5 kg (7.7 lb), are about 50 cm and then could be continued along with complementary foods
(20 in) long, and have a head circumference of 35 cm (14 in). They through 12 months of age. However, there are situations in which
lose about 5% to 10% of their birth weight during the first several breastfeeding is not possible for the mother or is contraindicated
postnatal days and usually regain this weight by the age of 10 to because of a disease or medication. Therefore, although breastfeeding is
14 days. During the first several months after birth, weight gain serves the most advantageous for mother and baby and should be encour-
as an important indicator of infants’ general well-being. Failure to gain aged, mothers should never be made to feel inadequate or guilty if
weight during this time may be a clue to a wide variety of problems, they are unable to breastfeed. Human milk or an iron-fortified infant
ranging from underfeeding to malabsorption. Newborns and infants formula provides complete nutrition for infants during the first 4
gain about 30 g/day (1.1 oz/day; roughly 1% of their birth weight to 6 months after birth. During the first postnatal month or 2,
per day) for the first 3 postnatal months and about 10 to 20 g/day newborns and infants take about 2 to 3 oz of formula (approximately
(0.4–0.7 oz/day) for the rest of the first year. Infants double their birth 10 minutes on each breast) every 2 to 3 hours.
179

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 180 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 28.1. Expected Increase in Weight, Height, to mature milk 7 to 10 days after delivery. The nutrient content of
and Head Circumference of Newborns, Infants, human milk of mothers who deliver preterm compared with those
and Children who deliver at term may vary considerably. Individual assessment
may be necessary to determine the appropriateness of human milk
Typical Weight Gain
for preterm newborns.
Age Expected Weight Increase Nutritionally, human milk is uniquely tailored to meet the spe-
0–3 months 25–35 g/day (0.9–1.2 oz/day) cific needs of babies. Human milk provides approximately 20 kcal/oz,
3–6 months 12–21 g/day (0.4–0.7 oz/day) the same as routine infant formulas. Table 28.3 compares the com-
6–12 months 10–13 g/day (0.4–0.5 oz/day) position of human milk and several infant formulas. Human milk
has relatively low amounts of protein compared with cow’s milk (1%
1–6 years 5–8 g/day (0.2–0.3 oz/day)
vs 3%), yet the levels are sufficient to provide for satisfactory growth
7–10 years 5–11 g/day (0.2–0.4 oz/day) of babies. Protein content is highest at birth at 2.3 g/dL, then declines
Typical Height Increase over the first month to 1.8 g/dL, yet it ensures adequate protein sta-
Age Expected Height Increase tus throughout the first postnatal year.
0–12 months 25 cm/year (9.8 in/year) Qualitative differences also make human milk more desir-
able. Casein to whey ratio in human milk is about 30:70, mak-
13–24 months 12.5 cm/year (4.9 in/year)
ing it easier to digest than most infant formulas, which tend to
2 years–puberty 6.25 cm/year (2.5 in/year) have higher casein to whey ratios at 82:18. Whey is more easily
Typical Increase in Head Circumference digested and associated with faster gastric emptying compared
Age Expected Increase in Head Circumference with casein. Newborns and infants who breastfeed tend to digest
0–3 months 2 cm/month (0.8 in/month) their milk more easily, have softer stools, and be less satiated over-
night, requiring more feeds, than those fed formula. Other whey
4–6 months 1 cm/month (0.4 in/month)
proteins, such as IgA, lysozyme, and lactoferrin, all contribute and
7–12 months 0.5 cm/month (0.2 in/month)
help host defenses.
Total increase 12 cm year (4.7 in) in first year Lactose is the major carbohydrate of human and cow’s milk,
but it is present in higher concentrations in human milk. Lactose
in human milk also contributes to softer stool consistency with
Table 28. 2. Energy and Protein Needs of Children
nonpathological fecal flora and improved absorption of minerals.
Age (years) Calories (kcal/kg/day) Protein (g/kg/day) Oligosaccharides in human milk found in the carbohydrate poly-
0–1 90–120 2.5–3.0 mers and glycoproteins have been structurally shown to mimic
1–7 75–90 1.5–2.5 bacterial antigen receptors and may have a role in host defense.
7–12 60–75 1.5–2.5 Fat is the primary source (50%) of calories in human milk. The
fat in cow’s milk, which contains primarily saturated fatty acids,
12–18 30–60 1.0–1.5
is not as well digested by newborns and infants as human milk
fat, which is predominantly composed of polyunsaturated fats.
Because human milk is more easily digested than formula, it Within the last decade, the long-chain polyunsaturated fatty acids
passes out of the stomach in 90 minutes; formula may take up to docosahexaenoic acid (DHA) and arachidonic acid (ARA) have
4 hours. Therefore, during the first 4 to 6 postnatal weeks, breast- been added to most infant formulas to simulate the higher lev-
fed newborns and infants want to feed more frequently (8–12 times els found in human milk. Research continues to be inconclu-
in 24 hours) than formula-fed newborns and infants (6–8 times in sive, however, as to whether DHA and ARA supplementation may
24 hours), with an increased number of nighttime feedings as well. enhance vision and improve growth and cognitive development
By about 3 to 5 months of age, breastfed and bottle-fed infants do not in formula-fed infants.
differ in the number of nighttime feedings, although some breast- Human milk from well-nourished women should provide
fed infants continue to wake out of habit. adequate amounts of all vitamins and other micronutrients.
Most infants 6 months or younger consume about 4 to 5 oz per However, vitamin K, vitamin D, iron, and fluoride are not pres-
feeding every 4 to 5 hours. Under routine circumstances, human ent in sufficient quantities to satisfy all nutritional needs over a
milk is preferred to infant formulas because it has emotional, nutri- prolonged period, and supplementation should be considered. The
tional, and immunologic advantages. Breastfeeding allows infants American Academy of Pediatrics (AAP) recommends that all new-
and mothers to develop a unique relationship that can be emotion- borns receive a prophylactic dose of 0.5 to 1 mg of parenteral vita-
ally satisfying (see Chapter 29). min K in the immediate newborn period to help prevent bleeding
The composition of human milk varies over time. Colostrum, disorders. Even though the vitamin D content of human milk is
the first milk produced after delivery, is high in protein, immu- low compared with cow’s milk, newborns and infants of healthy
noglobulin (Ig), and secretory IgA. Colostrum gradually changes mothers have generally not been observed to develop rickets if

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 Chapter 28: Nutritional Needs 181

Table 28.3. Composition of Human Milk and Select Infant Formulas (Calories: 20 kcal/oz)
Formula Protein Carbohydrate Fat
Human milk 40% casein and 60% whey Lactose Human milk fat
(mature)
Cow’s milk 80% casein and 20% whey Lactose Butterfat
Enfamil NeuroPro Nonfat cow’s milk and whey Lactose Palm olein, soy, coconut, and high-oleic sunflower oils;
DHA, milk fat globule membrane
Similac Advance Nonfat cow’s milk and whey Lactose High-oleic safflower oil, coconut and soy oils, DHA
Enfamil ProSobee Soy protein and methionine Corn syrup solids Palm olein, soy, coconut, and high-oleic sunflower oils
Similac Soy Isomil Soy protein and methionine Corn syrup solids and sucrose High-oleic safflower oil, soy and coconut oils; DHA
Nutramigen with Casein hydrolysate, cystine, tyrosine, Corn syrup solids and cornstarch Palm olein, soy, coconut, and high-oleic sunflower oils;
DHA & ARA tryptophan DHA, ARA
Pregestimil with Casein hydrolysate, cystine, tyrosine, Corn syrup solids, modified MCT, high-oleic safflower, corn and high oleic vegetable
DHA & ARA tryptophan cornstarch, and dextrose oils, DHA, ARA
Similac Expert Care Casein hydrolysate, cystine, tyrosine, Sucrose modified tapioca starch Safflower oil, MCT, soy oil, DHA, ARA
Alimentum tryptophan
Abbreviations: ARA, arachidonic acid; DHA, docosahexaenoic acid; MCT, medium-chain triglycerides.

there is sufficient exposure to sunlight. Newborns require about or longer as mutually desired by mother and infant. Rarely, breast-
1 minute of exposure to sunlight on the face to produce enough feeding is contraindicated (see Chapter 29).
vitamin D. However, adequate sun exposure is difficult to assess,
and there are increasing concerns about the harmful effects of Solid Foods
sunlight. Compared with the previous recommendation of an Supplemental foods may be added to infants’ diets between the
average intake of 200 IU of vitamin D per day, the 2010 Institute ages of 4 and 6 months. Solid foods should be introduced as soon
of Medicine, now known as the Health and Medicine Division as infants require the additional calories and are developmentally
of the National Academies, recommendation calls for an aver- mature (ie, infant can sit and support his or her head and has lost
age intake of 400 IU of vitamin D per day to meet the needs of tongue thrust). Introduction of solid foods prior to 4 months of age
most infants younger than 12 months. Although human milk can interfere with an infant’s ability to take sufficient amounts of
contains less iron than iron-fortified formulas (fortified to human milk or formula to meet nutritional needs. Waiting beyond
about 12 mg/L of iron), the bioavailability of the iron in human 6 months of age to introduce solid foods may increase an infant’s
milk is greater. Breastfed infants do not need iron supplementa- risk of having inadequate iron or zinc intake.
tion until 6 months of age. For children 6 months and older who Infants should first be given cereal grains, fruits, and vegetables,
live in communities with suboptimally fluoridated water, the AAP although 2 to 3 days should separate the introduction of new foods.
recommends systemic (dietary) supplementation. Such supple- Once several of these foods have been tolerated, the early introduction
mentation can be provided through the use of a fluoridated tooth- of subsequent, more allergenic, foods, such as milk, eggs, soy, wheat,
paste twice a day (see Chapter 31). nuts, and seafood, between 4 and 6 months of age may decrease the
Human milk has several immunologic advantages, which are risk of the infant developing food allergies. Factors that indicate
allergy and infection protective, over standard cow’s milk–based infants may be ready for solid foods include current weight twice
formulas. Its allergy-protective characteristics are attributed, in that of birth weight, or about 6 to 7 kg (13.2–15.4 lb); consumption
part, to the decreased intestinal permeability associated with of more than 32 oz of formula per day (if on a formula-only diet); fre-
human milk compared with standard formulas. The host defense quent feeding (regularly more than 8–10 times per day or more often
factors present in human milk include Ig, complement, and cel- than every 3 hours); and perceived persistent hunger after nursing.
lular components (eg, macrophages, neutrophils, lymphocytes). Iron-fortified infant cereal, most commonly rice cereal because
Studies have shown that the incidences of viral and bacterial ill- it does not contain gluten, is usually the first solid food offered to
nesses are lower in exclusively breastfed infants compared with infants. Other single-grain cereals, such as barley cereal or oatmeal,
their formula-fed peers. are also appropriate early supplemental foods. Precooked infant
The AAP recommends exclusive breastfeeding for about cereals can be mixed with a variety of liquids, including human
6 months, followed by continued breastfeeding as complementary milk, formula, water, or infant fruit juices. The vitamin C in juice
foods are introduced, with continuation of breastfeeding for 1 year increases the bioavailability of the iron in the cereal, hence the

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 182 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

recommendation to add it to dry cereal. Initially, the cereal should and nitrites (eg, in vegetables such as carrots, beets, and spinach).
be mixed to a thinner consistency (eg, about 1 tablespoon of cereal to Nitrates and nitrites have been implicated in the development of
2 oz of liquid). It is not unusual for infants to reject their first several methemoglobinemia, especially in infants younger than 6 months.
spoonfuls of cereal because the tastes and textures are new. If they Methemoglobinemia decreases the oxygen-carrying capacity of the
refuse the feeding, it should be stopped and reintroduction of the blood, leading to anoxic injury and death. This is more of a concern
food delayed for 1 week. Once infants have accepted the new taste in rural areas that primarily use well water.
and texture, the mixture should gradually be worked to a thicker Weaning from the breast or bottle to a cup usually occurs by
consistency. By about 7 to 8 months of age, infants should be tak- 12 months of age but may be delayed up to 18 months of age in some
ing 4 to 6 tablespoons of cereal mixed with enough liquid to give children. Homogenized, vitamin D–fortified cow’s milk may be given
the mixture the consistency of mustard. Mixed cereal grains may be at 12 months of age. Fat-free (skim) and low-fat (1%) milk should
given to older infants. not be given before 2 years of age.
Fruits and vegetables may be introduced within a few weeks of
the introduction of cereal. The order is not as important as the need
to add only 1 new food at a time and no more than 1 to 2 new foods Diet of Children and Adolescents
per week. Meats may be introduced after 6 months of age. The caloric and protein needs of children decrease in the second
A wide variety of commercially prepared baby foods designed year after birth, paralleling the decrease in growth rate during this
to be developmentally appropriate and labeled by stage (ie, first, time. Milk intake also decreases and may drop to 16 oz/day by
second, third) are available. The jars of different stages contain the 24 to 36 months of age. Except for increased caloric requirements,
amount of food that an infant at a given age should be able to eat the diet of school-age children and adolescents should be similar to
at 1 sitting. This is not always the case, however, and opened jars of that of normal adults. Evidence that foods eaten during childhood
baby food may safely be stored in the refrigerator for 2 to 3 days. may have long-lasting effects on adult health is increasing, and it is
Infants should not be fed directly from the jar because saliva on the important that children develop healthy eating habits early in life.
spoon mixes with the remaining food and digests it, causing it to liq- Atherosclerosis, osteoporosis, and obesity are some of the diseases
uefy. Vegetables and meats may be offered at room temperature but that may have their beginnings during childhood.
should be warmed slightly for greater palatability. Fruits and desserts The US departments of Agriculture (USDA) and Health and
may be at room or refrigerator temperature. Home-cooked fruits and Human Services (HHS) have replaced the food pyramid with a new
vegetables should be thoroughly washed, pureed, and strained before visual aid—a circular plate on a square mat. Half of the plate con-
giving to infants. Home-prepared foods tend to have a shorter shelf sists entirely of vegetables and fruits, whereas the other half of the
life than commercially bought baby foods because of lack of preser- plate is divided into one-quarter protein and one-quarter grains,
vatives, but some may find that they are more palatable to the infant. with a small side of dairy. It’s a much more visually descriptive
First-stage foods, for infants 4 to 6 months of age, include strained tool that guides how to divide daily meals. The website www.
infant juices, single-grain cereals, and pureed strained fruits and veg- choosemyplate.gov provides individual dietary guidance accord-
etables such as bananas, carrots, and peas. These foods contain no ing to a child’s age, sex, and activity level based on the USDA/HHS
egg, milk, wheat, or citrus, to which some infants may be sensitive. dietary guideline for Americans older than 2 years.
Second-stage foods, for infants about 6 to 9 months of age, are smooth, To promote lifelong heart healthy habits, the American Heart
mixed-ingredient foods, such as mixed vegetables, or meat dinners, Association (AHA) released a statement of dietary recommendations
such as chicken noodle. Third-stage foods, or junior foods, are for for children and adolescents (Box 28.1). It recognizes that children
infants about 9 to 10 months of age who can sit well without support, are often offered nutrient-poor foods that are high in fat and sugar
have some teeth, and have begun self-feeding. These more coarsely and overly processed. The AHA recommendations support USDA
textured foods, such as vegetable and meat dinner combinations, con-
tain a wider variety of nutrients. Finger foods, such as crackers, cheese
wedges, or cookies, can also be introduced by 9 to 10 months of age, Box 28.1. AHA Dietary Recommendations
once infants have developed a pincer grasp. Most infants are eating the for Children and Adolescents
same meals as the rest of the family (table foods) by about 1 year of ww Limit total fats to less than 25% to 35% of total daily calories.
age. Foods that can easily be aspirated, such as raw carrots, nuts, and ww Limit saturated fat to less than 7% of total daily calories.
hard candies, should be avoided until children are older than 4 years. ww Limit trans fat to less than 1% of daily calories.
Baby foods can be prepared at home as long as they are finely ww Remaining fat should come from natural sources of monounsaturated
pureed or strained and contain enough liquid to make them easy for and polyunsaturated fats, such as unsalted nuts and seeds, fish (espe-
infants to swallow. One danger of preparing foods at home is that cially oily fish, such as salmon, trout, and herring, at least 2 times a
sugar, salt, or spices can be easily added to make foods palatable week), and vegetable oils, such as canola oil.
to adults. These ingredients are not necessary for infants. In addi- ww Limit cholesterol intake to less than 300 mg a day or, if you have coro-
tion, homegrown, home-prepared vegetables may be contaminated nary artery disease, less than 200 mg a day.
with high levels of nitrates (eg, because of contaminated well water)

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 Chapter 28: Nutritional Needs 183

guidelines and include eating fruits and vegetables daily while lim- desired physique for their particular sport or weight class. While
iting fruit juice intake, using vegetable oils, using butter instead of there are many nutritional supplements, such as creatinine, carni-
soft margarines, eating whole grain rather than refined grain breads tine, various amino acids, and dehydroepiandrosterone, that claim
and cereals, using nonfat or low-fat milk and dairy products, eating to enhance athletic performance, none has thus far been fully eval-
more fish, and reducing salt intake. uated scientifically. Instead, teenage athletes should be counseled
The AHA also encourages behaviors for parents and caregivers on the importance of a basic nutrient: water. Proper hydration does
that promote healthy habits for the whole family. These recommen- enhance performance and prevents heat injury. Approximately 4
dations ask parents and caregivers to choose mealtimes, provide to 8 oz of fluid for every 15 minutes of exercise is recommended
a social context for eating by having regular family meals, lead regardless of actual thirst. Carbohydrate loading before competi-
by example in their own eating habits, and allow children to self- tion is believed to enhance performance; however, this practice has
regulate food intake and not to force them to finish meals if they no effects on non-endurance events and may confer only a mod-
are not hungry. est effect for endurance events by prolonging time to exhaustion.
Children who consume a varied diet do not need routine vitamin In counseling a teenage athlete, specific questions should also be
supplementation. However, children and teenagers who are consid- directed to elicit any unhealthy practices to maintain or lose weight.
ered picky eaters, as well as children at nutritional risk, may bene- Vegetarianism is gaining popularity among adolescents. Reasons
fit from supplementation. This includes children and teenagers who for choosing vegetarianism are varied, including health benefits,
are anorexic or those who follow fad diets, those with chronic dis- means for weight loss, animal cruelty concerns, and religious beliefs.
eases, those who consume a vegetarian diet, and those with failure It is important to ask vegetarians about their specific restrictions, as
to thrive (FTT). A standard pediatric vitamin-mineral supplement these relate to their nutritional risks. Semi-vegetarians are those who
should contain no more than the dietary reference intakes of its com- avoid red meat but eat fish and chicken in moderation. Lacto-ovo veg-
ponents. Parents should be counseled to teach their children that etarians consume animal products, such as dairy and eggs, but avoid
supplements are not candy and to keep them out of reach. Serious animal flesh. Vegans do not eat any animal products, such as dairy,
overdoses can occur, especially with iron-containing formulations. eggs, honey, or gelatin. Those who follow a macrobiotic diet restrict
Bone health is determined by calcium and vitamin D intake as not only animal products but also refined and processed foods, foods
well as weight-bearing physical activity. Recent data suggest the pos- with preservatives, and foods that contain caffeine or other stimulants.
sibility of other important health benefits throughout life of these A well-planned vegetarian diet can provide all necessary nutrients;
key nutrients and behavior, in addition to bone growth and devel- however, many teenagers experiment with vegetarianism in a non-
opment. The 2010 Institute of Medicine guidelines call for a recom- vegetarian household and require guidance. The nutrients that may
mended daily allowance of 600 IU per day of vitamin D for children be deficient in a vegetarian diet are protein, calcium, vitamin D, vita-
older than 1 year and 1,300 mg of calcium per day for children and min B12, iron, and zinc. Protein intake is usually not a concern for
adolescents 9 to 18 years of age. Unfortunately, calcium intake for lacto-ovo vegetarians because eggs and dairy have high-quality pro-
most US children, particularly adolescents, is generally below the teins. Vegans and macrobiotic followers have a variety of plant-based
recommended levels. Barriers to adequate calcium intake may be protein sources from which to choose, such as legumes, cereals,
due to the preference of sweetened juice and soft drinks over milk nuts, seeds, and fruits. Because vitamin B12 is only found in ani-
as well as lactose intolerance in certain populations. Nondairy cal- mal-based foods, vegans and macrobiotic followers must ensure ade-
cium sources include salmon, white beans, broccoli, and calcium- quate intake via supplements or by consuming vitamin B12-fortified
fortified foods, such as orange juice, breakfast cereals, and soy milk. foods, such as soy and nut beverages and cereals.
Adequate calcium intake can be achieved by eating 3 (or 4 for adoles- Lastly, familiarity with the latest fad diets is an asset for any pri-
cents) age-appropriate servings of dairy products or other calcium- mary care practitioner. These diets are usually restrictive in certain
rich food per day. In children and adolescents who do not consume nutrients and recommend unusual dietary patterns that are incon-
adequate amounts of calcium from dietary sources, a calcium sup- sistent with current USDA guidelines. Although there are some
plement is recommended. This can be in the form of a multivitamin suggestions that these diets work for some adults, there are almost
in the younger child or calcium carbonate tablets with or without no scientific data for children and adolescents. To keep abreast of
vitamin D for the adolescent. the latest fads, refer to the Academy of Nutrition and Dietetics (www.
Adolescence is a period of tremendous physical and emotional eatright.org), which maintains an annual review of such popular
growth, both of which greatly affect nutritional needs and habits. fad diets.
Although their rapid physical growth requires increased energy and
nutrients, the common eating habits of teenagers do not always sup-
Evaluation
port their needs. Teenagers tend to skip meals, eat outside the home,
consume fast food and snacks, and experiment with different restric- History
tive fad diets or various forms of vegetarianism. Nutritional assessment begins with a complete dietary history. The
Teenage athletes also have their own unique nutritional con- dietary assessment should emphasize the quantity, quality, and
cerns. They want to maximize performance while maintaining the variety of foods in the diet. Any special or restricted dietary habits

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 184 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

should be noted (eg, vegetarian diet, vegan diet, occasional veg- also be useful in the evaluation of suspected malnutrition. Screening
etable juicing/fasting). A 3-day food record listing the types and tests that may be used in the evaluation of FTT include thyroid func-
quantities of food eaten throughout the day can be very helpful in tion studies, urinalysis, and bone age (see Chapter 146). More spe-
evaluating dietary history. cific tests, such as serum vitamin levels (eg, folate or vitamin B12
In addition, the child’s routine medical, family, and social his- levels in suspected malabsorption) or hormone assays (eg, growth
tory all may influence nutritional status. For example, the economic hormone levels in the evaluation of short stature), may be obtained
status of families may affect the variety and type of foods they may in certain instances.
be able to purchase, and the level of education of parents influences
their ability to understand the concepts of a healthy diet. Poverty Common Feeding and Nutritional
and ignorance of nutritional needs are among the most common
Problems of Childhood
reasons for malnutrition in children. It is vital that the pediatrician
stress to families the value of healthy food choices at each physi- Several gastrointestinal (GI) problems have been attributed to diet.
cal examination. Family access to food can be estimated by asking A small amount of spitting up is seen in most children, especially
parents about how often the family skips meals during the average during the first 6 months after birth. However, vomiting can be
month. Such information assesses food insecurity within a house- a sign of several disorders, ranging from viral GI tract infections
hold. Specific cultural food preferences and feeding practices should to more severe illnesses, such as pyloric stenosis, urinary tract
also be included in the history. infection, GI obstruction, or inborn error of metabolism (see
Chapter 120). Constipation, which is seen more commonly in
Physical Examination formula-fed than breastfed infants, may be due to insufficient fluid
Weight, length or height, head circumference, weight for length, intake (see Chapter 124). The simple addition of 2 to 4 oz of water
and body mass index should be measured or calculated routinely to an infant’s diet or temporary use of diluted apple or prune juice
and plotted on a longitudinal basis on appropriate growth curves. In may solve the problem.
addition to the charts provided by the Centers for Disease Control Chronic nonspecific diarrhea of childhood, or “toddler’s diar-
and Prevention and World Health Organization, additional charts rhea,” may be seen in infants and children 6 months to 5 years of
are available for special populations for which growth is altered, such age with low dietary fat intake and excessive fruit juice consump-
as infants and children with low birth weight and preterm birth, tion (see Chapter 123). Failure to absorb sugars, especially sorbitol
Down syndrome, Turner syndrome, Williams syndrome, and sev- and fructose, can lead to an osmotic diarrhea.
eral other chromosomal and genetic disorders. Changes in the rate Underfeeding or a diet that is not nutritionally balanced may
of growth over time are more useful than a single measurement in result in FTT (see Chapter 146). The opposite problem, obesity, is
the assessment of nutritional problems. Calculation of the height age among the most common nutritional problems of children in the
(age for which the child’s height is at the 50th percentile), weight United States (see Chapter 155). The prevalence of this condition in
age (age for which the child’s weight is at the 50th percentile), and children 6 to 11 years of age is estimated to be about 20% to 25%.
ideal weight for actual height may be useful when deviations from Finally, the eating disorders anorexia nervosa and bulimia nervosa
reference are noted. are estimated to affect about 1 in 100 adolescent females 16 to 18 years
In addition to the overall impression of nutritional status, cer- of age (see Chapter 64).
tain findings on physical examination may be characteristic of par- The picky eater is a common parental concern in the primary
ticular nutritional disorders. The evaluation of the hair, skin, eyes, care setting. For practitioners who work with the Latinx popula-
lips and oral mucosa, dentition, and musculoskeletal system should tion, the child who “no come nada,” literally translated as the child
be emphasized because the examination of these areas is most likely who “does not eat anything,” is a similar common parental concern.
to show the effects of malnutrition. Muscle wasting; hepatospleno- Parents can be reassured by their child’s normal weight for height
megaly; skeletal deformities; decayed teeth; rough, dry skin; hair or body mass index and growth velocity. They should be counseled
that is easily plucked; and irritability may all be signs of inadequate that it is normal for preschoolers to exert their individuality by lim-
nutrition. iting food preferences, the fact that it may take up to 10 exposures
for a child to accept a new food, the difference between child and
Laboratory Tests adult portion sizes, and the concept that children can self-regulate
Suspected malnutrition or nutrition-related disorders, based on food intake to sustain normal growth and health.
history and physical examination, can be further investigated with Nutritional disorders include malnutrition and deficiencies of
laboratory studies. Tests that may be used in the evaluation of vitamins and minerals. Iron deficiency anemia is 1 of the most com-
anemia, 1 of the most common nutrition-related disorders seen in mon nutrition-related problems seen in children and adolescents.
children, include a complete blood cell count, reticulocyte count, Malnutrition is among the leading causes of childhood morbidity
serum iron, ferritin, and total iron-binding capacity. Investigation and mortality worldwide. Although primary protein-calorie mal-
of suspected malnutrition begins with an assessment of protein sta- nutrition (PCM) is rare in most parts of the United States, surveys
tus, with measures of indicators such as serum albumin, total pro- conducted on pediatric wards have demonstrated that about one-
tein, and transthyretin. Liver function tests and a lipid profile may third of pediatric inpatients with chronic disease have evidence of

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 Chapter 28: Nutritional Needs 185

some degree of PCM. The most common deficits were weight for American Academy of Pediatrics Committee on Nutrition. Pediatric Nutrition.
height below 90% of standard (ie, evidence of acute malnutrition) Kleinman RE, Greer FR, eds. 7th ed. Elk Grove Village, IL: American Academy
and height for age below 95% of standard (ie, evidence of chronic of Pediatrics; 2014
malnutrition). The 2 forms of PCM are marasmus (severe caloric American Heart Association. Dietary recommendations for healthy children.
depletion) and kwashiorkor (inadequate protein intake). Untreated http://www.heart.org/HEARTORG/HealthyLiving/HealthyEating/Dietary-
Recommendations-for-Healthy-Children_UCM_303886_Article.jsp#.W_
PCM can result in impaired growth, poor intellectual development,
s2NS2ZPjA. Reviewed September 2014. Accessed September 2, 2019
and impaired immune functioning.
Bunik M. The pediatrician’s role in encouraging exclusive breastfeeding. Pediatr
Rev. 2017;38(8):353–368 PMID: 28765198 https://doi.org/10.1542/pir.2016-0109
CASE RESOLUTION Centers for Disease Control and Prevention. Growth chart training: introduc-
The infant is ready to begin some solid foods because she is consuming 32 oz tion. https://www.cdc.gov/nccdphp/dnpao/growthcharts/index.htm. Reviewed
of formula per day and continues to be hungry. In addition, she has reached a April 15, 2015. Accessed September 2, 2019
weight of 5.9 kg (13 lb) and has almost doubled her birth weight. The mother is Das JK, Salam RA, Thornburg KL, et al. Nutrition in adolescents: physiology,
counseled to begin feeding her daughter a single-grain infant cereal mixed with metabolism, and nutritional needs. Ann N Y Acad Sci. 2017;1393(1):21–33
formula. (The cereal should be fed by spoon, not given in a bottle.) Within a few PMID: 28436102 https://doi.org/10.1111/nyas.13330
weeks, once the infant is taking the cereal well, other first foods, such as fruits
Diab L, Krebs NF. Vitamin excess and deficiency. Pediatr Rev. 2018;39(4):161–
and vegetables, may be introduced. After she tolerates cereal and several fruits
179 PMID: 29610425 https://doi.org/10.1542/pir.2016-0068
and vegetables, more allergenic foods, such as egg or diluted nut butters, should
be introduced in small amounts to reduce the risk that she will develop a subse- DiMaggio DM, Cox A, Porto AF. Updates in infant nutrition. Pediatr Rev.
quent food allergy. 2017;38(10):449–462 PMID: 28972048 https://doi.org/10.1542/pir.2016-0239
Golden NH, Abrams SA; American Academy of Pediatrics Committee on Nutrition.
Optimizing bone health in children and adolescents. Pediatrics. 2014;134(4):
Resources e1229–e1243 PMID: 25266429 https://doi.org/10.1542/peds.2014-2173
ChooseMyPlate.gov Jasani B, Simmer K, Patole SK, Rao SC. Long chain polyunsaturated fatty
www.choosemyplate.gov acid supplementation in infants born at term. Cochrane Database Syst Rev.
2017;3:CD000376 PMID: 28281303 https://doi.org/10.1002/14651858.
US Department of Agriculture National Agricultural Library Food
CD000376.pub4
and Nutrition Information Center: https://www.nal.usda.gov/fnic
Martin CR, Ling PR, Blackburn GL. Review of infant feeding: key features of
breast milk and infant formula. Nutrients. 2016;8(5):279–289 PMID: 27187450
Selected References https://doi.org/10.3390/nu8050279
Abrams SA. Dietary guidelines for calcium and vitamin D: a new era. Pediatrics. Messina V, Mangels AR. Considerations in planning vegan diets: children. J
2011;127(3):566–568 PMID: 21339264 https://doi.org/10.1542/peds.2010-3576 Am Diet Assoc. 2001;101(6):661–669 PMID: 11424545 https://doi.org/10.1016/
Academy of Nutrition and Dietetics. Eat right. https://www.eatright.org. S0002-8223(01)00167-5
Accessed September 2, 2019 Smith B, Thompson J. Nutrition and growth. In: Kahl L, Hughes HK, eds.
American Academy of Allergy Asthma and Immunology. Prevention of aller- The Harriet Lane Handbook: A Manual for Pediatric House Officers. 21st ed.
gies and asthma in children. https://www.aaaai.org/conditions-and-treatments/ Philadelphia, PA: Elsevier; 2018:570–606
library/allergy-library/prevention-of-allergies-and-asthma-in-children. Accessed US Department of Agriculture. ChooseMyPlate.gov. https://www.choosemyplate.
September 2, 2019 gov. Accessed September 2, 2019

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 CHAPTER 29

Breastfeeding
Karen C. Bodnar, MD, IBCLC, FABM, FAAP

CASE STUDY
A 25-year-old pregnant woman comes to your office Questions
with her 18-month-old for a well visit. When asked, she 1. What is the normal physiology of lactation?
reports that she had a difficult time breastfeeding her 2. What are the benefits of breastfeeding?
first child because of pain; however, she gave pumped 3. What are the contraindications to breastfeeding?
milk for 4 months. She hopes to breastfeed directly 4. What management maximizes a mother’s success at
for at least 6 months with this baby. She would like to breastfeeding?
know what advice you can give her. She expects a nor- 5. How does the pediatrician manage some of
mal delivery, has had no breast surgery, and is not on the common problems that may arise during
any medications; however, she smokes cigarettes occa- breastfeeding?
sionally. She plans to return to work when the baby is
4 months old.

Human milk is the natural food source for human newborns and been the impetus to again promote breastfeeding as the preferred
infants. It provides optimal nutrition and is an immunologically food source for newborns and infants.
active compound that allows for early regulation of an infant’s In the United States today, following the Centers for Disease
immune system and priming of the microbiome. All formulas Control and Prevention Healthy People initiative, which included
are incomplete attempts at replication. Human milk is made of a national agenda calling for an increased rate of breastfeeding,
water, fat, lactose-containing carbohydrates, and protein, as well 83.2% of mothers are initiating breastfeeding. The rate of mothers
as vitamins, immunoglobulins, prebiotics, enzymes, hormones, sustaining breastfeeding to 1 year of age is only 35.9%, however.
and even phagocytes and lymphocytes. It is a dynamic fluid that Rates are lower in low socioeconomic groups and among women
changes in composition as newborns and infants grow. Early with lower levels of education. Ethnic disparities also exist, with
colostrum is high in lactose and protein, composed of casein and the black population having the lowest rates of breastfeeding in
whey, and quite immunologically active. Through lactogenesis, the United States.
it becomes mature milk with much greater quantity and higher In 1991, the World Health Organization and the United Nations
fat content. It thus has a lower concentration of protein but con- Children’s Fund developed the Baby-Friendly Hospital Initiative,
tinues to contain all the immunologically active components of delineating 10 steps to undertake in the hospital to promote suc-
colostrum. cessful breastfeeding. This initiative is used worldwide to improve
breastfeeding rates. The American Academy of Pediatrics published
Epidemiology its policy statement, “Breastfeeding and the Use of Human Milk,” in
Historically, newborns and infants were totally dependent on breast- 2005 and a revised version in 2012. This policy endorses breastfeed-
feeding by their mother or a wet nurse for their survival. When ing and delineates the physician responsibility to promote and sup-
formula feeding was attempted in the 1800s, the mortality rate port it. In 2011, the US Department of Health and Human Services
in exposed newborns and infants was as high as 85%. Thus, the issued The Surgeon General’s Call to Action to Support Breastfeeding.
advantages of breastfeeding were recognized and still promoted in A national imperative now exists to promote breastfeeding.
the early 1900s. Following the advent of pasteurization, cow’s milk
formula became much safer than before pasteurization. Formula Anatomy and Physiology of Lactation
development allowed more mothers to enter the workforce, and The breasts consist of lobules and alveoli where milk is produced, as
breastfeeding rates declined over the course of the 20th century. well as the ductile system, leading to 9 to 15 milk duct openings in
Formula feeding became the norm as companies successfully mar- the nipple. During pregnancy, the breasts enlarge as lobules mature
keted formulas. In 1972, at the nadir of breastfeeding, only 22% of and differentiate in response to estrogen, placental lactogen, pro-
babies were ever breastfed. However, current scientific understand- lactin, and progesterone. The nipples darken, and the surround-
ing of the many benefits of breastfeeding for baby and mother has ing areolas enlarge. In the first 24 hours after delivery, only a small

187

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 188 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

volume of colostrum, approximately 40 to 50 mL total, is produced. Some mothers report feeling uncomfortable breastfeeding in pub-
By 3 to 4 days after delivery, however, increased milk production, lic. Identifying this issue and helping these mothers feel more sup-
termed lactogenesis stage 2, commences. This occurs as estrogen and ported or find privacy when they need it can help them succeed with
progesterone levels drop and prolactin, from the anterior pituitary, breastfeeding. Currently, laws exist in all 50 states and the District of
is increased in response to nipple stimulation. Additionally, oxyto- Columbia that protect a mother’s right to breastfeed in public.
cin is released from the posterior pituitary and causes contraction of Many new mothers have poor family support for breastfeeding,
myoepithelial cells, which squeeze milk from the alveoli. Although and it is necessary to include the entire family in breastfeeding coun-
initial production of milk is not dependent on newborn suckling, seling. The mother will not be successful if her family is encourag-
the more the neonate feeds and the more often the breast is effec- ing her to use formula.
tively emptied, the more milk is produced. If milk is not removed, Some mothers find that their place of employment does not
an autocrine hormone in milk called feedback inhibitor of lactation make accommodations for a breastfeeding mother. It may be that
acts locally within each breast to inhibit milk production. Early effec- the workplace needs to be reminded of laws promoting breastfeed-
tive removal of colostrum, ideally starting within 1 hour of deliv- ing in the workplace. The Patient Protection and Affordable Care
ery, speeds the arrival of increased milk volume, and frequent milk Act includes a provision that the workplace must provide adequate
removal increases supply in the weeks that follow. break time and a private place for nursing mothers to pump for up
to 1 year. Some states offer additional protections.
Benefits of Breastfeeding Hospitals may have practices or policies that interfere with suc-
cessful breastfeeding, including high rates of cesarean section or
For the baby, the benefits of breastfeeding are myriad. Studies have
no rooming-in policy. Physicians should work with their hospitals
demonstrated that breastfed infants have a decreased incidence and
to minimize these potential barriers. Some medical professionals
severity of infectious illnesses, including diarrhea, respiratory infec-
tions, otitis media, bacterial meningitis, and urinary tract infec- become barriers to exclusive breastfeeding because they lack the
tions. They have lower rates of hospitalization and mortality. The knowledge to properly manage problems as they arise.
incidence of otitis media is 100% higher in formula-fed infants
than exclusively breastfed infants. Studies have also demonstrated Contraindications
better performance on cognitive testing among children who were Absolute contraindications to breastfeeding are few. The neonate
breastfed as infants. Among preterm infants fed human milk, the with galactosemia type I as detected by newborn screening and
incidence of necrotizing enterocolitis is also significantly reduced. who therefore is unable to metabolize lactose or galactose may not
Breastfeeding in infancy also reduces the later incidence of atopy, exclusively breastfeed. Newborns can also inherit defects in protein
allergies, asthma, childhood obesity, type 2 diabetes, and even child- metabolism that may necessitate a special diet, precluding breast-
hood cancer. Decreased rates of sudden infant death syndrome feeding. In most states, newborn screening now includes testing for
(see Chapter 72) have also been documented. All these benefits are most of these metabolic disorders.
increased by increasing the length and exclusivity of breastfeeding. Maternal infections prohibiting breastfeeding include active,
For the mother, an immediate benefit to breastfeeding is untreated tuberculosis and HIV. Additionally, if herpetic vesicles are
oxytocin-induced decreased postpartum blood loss and enhanced present on the breast, the mother should not breastfeed from that breast.
mother-infant bonding. Lactation amenorrhea may serve subse-
quently as birth control. Breastfeeding has also been associated with Medications and Drugs of Abuse
quicker return to prepregnancy weight as well as decreased risk Most medications are safe for a mother to use while breastfeeding,
of breast cancer, ovarian cancer, diabetes, hypertension, heart dis- and the risk to the newborn or infant of not breastfeeding often out-
ease, and osteoporosis. Some evidence suggests that breastfeeding weighs the risk of exposure to subclinical doses of the medication
decreases the risk of postpartum depression. in human milk. Each medicine should be reviewed for potential
Societal benefits from breastfeeding include markedly decreased effects on the infant or possible negative effects on milk supply. The
annual health care costs. An estimated savings of $10.5 billion LactMed App is an excellent free resource (https://toxnet.nlm.nih.
annually could be generated if 80% of American families breast- gov/help/newtoxnet/lactmedapp.htm). Chemotherapeutic agents,
fed exclusively for the first 6 months after birth. antimetabolites, radioactive isotopes, and drugs of abuse are all con-
traindicated for breastfeeding. However, a mother with a history of
Barriers to Breastfeeding drug use may benefit from breastfeeding provided her toxicology
Many studies have evaluated barriers to breastfeeding in the United results are closely monitored and are negative.
States. Understanding these barriers is essential to improving breast- The 2018 American Academy of Pediatrics clinical report on mar-
feeding rates. With effective physician, nursing, and peer support, ijuana use during pregnancy and breastfeeding states that because
most mothers should be able to breastfeed successfully. One of the the potential risks of infant exposure to marijuana metabolites are
most important barriers is the lack of knowledge of pregnant women unknown, women should be informed of the potential risk of expo-
about the benefits of feeding mother’s milk. Education beginning at sure during lactation and encouraged to abstain from using any mar-
the first prenatal visit is vitally important. ijuana products while breastfeeding. Although marijuana is legal in

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 Chapter 29 : Breastfeeding 189

some US states, pregnant and breastfeeding women who use mar- to prevent hemorrhagic disease of the newborn, application of oph-
ijuana may be subject to child welfare investigations if they have a thalmic antibiotic ointment or silver nitrate to prevent gonorrheal
positive marijuana screening result. infections, and hepatitis B vaccination are administered to all
newborns shortly after birth (see Chapter 23).
After hospital discharge, the breastfeeding newborn should be
Breastfeeding Management
seen by the physician at day 3 to 5 of age and again at 2 weeks of
The management of breastfeeding should begin in the prenatal age to support breastfeeding. Early assessment may prevent many
period. The US Preventive Services Task Force endorses promotion breastfeeding problems and enables the physician to intervene early
and support for breastfeeding at all health care encounters. The preg- if problems arise, thereby helping to prevent discontinuation of
nant woman should be educated by her pediatrician and obstetrician breastfeeding. New mothers need encouragement and reassurance.
on the benefits of breastfeeding. Her history should be reviewed for At each visit, the neonate should be assessed for weight, feeding
potential contraindications. If none exist, she should be encouraged schedules, voiding and stooling patterns, and jaundice. The mother’s
to breastfeed. If she commits to breastfeeding before the baby is born, breasts should be examined for fullness, engorgement, and nipple
she is more likely to be successful. Involving her partner in these dis- trauma. A feeding should be observed. Mothers who are giving for-
cussions has also been shown to improve breastfeeding success rates. mula supplementation in the first weeks need help improving milk
At the time of delivery, if there are no complications, the neonate transfer and weaning formula supplements. Referral to a lactation
should be dried and placed skin to skin on the mother’s abdomen or specialist and a follow-up appointment in several days is essential.
chest for warmth and contact. Initial Apgar scores can be assigned The infant should exclusively breastfeed until 6 months of age.
during this process. The newborn will find his or her way to the At that time an iron source is needed, and iron-fortified cereal can
breast and latch on. This early breastfeeding experience greatly facil- be given with a gradual introduction of other pureed foods. The
itates further breastfeeding. The neonate should not be separated recommendation is that breastfeeding continue until at least 1 year
from the mother except for medical reasons. If an infant or mother of age or as long as the mother and infant desire.
is not medically stable enough to breastfeed immediately after birth, If a mother plans to return to work, she should be counseled to
hand expression should be initiated within 1 hour of delivery. initiate exclusive breastfeeding and wait until the infant is approxi-
During the hospital stay, the newborn should breastfeed on mately 4 weeks of age before introducing a bottle of expressed milk.
demand on both breasts for as long as she or he wants. Hospital poli- When the mother is separated from the infant, she should pump her
cies for rooming-in greatly facilitate breastfeeding. Generally, the neo- breasts at regular intervals. The milk can be stored at room tempera-
nate should nurse at least 8 times a day. A healthy full-term newborn ture for 4 hours, in the refrigerator for up to 4 days, or in the freezer
has no medical need for formula supplements. Without pacifiers or for up to 6 months (ideal) or even 12 months (acceptable) for later
supplemental feeds, the neonate will learn to breastfeed more quickly. use. Generally, milk should be stored in 2- to 4-ounce bags or con-
The mother should be counseled on appropriate latch-on, positioning tainers that are labeled with the date of expression. When the mother
of the newborn, and manual expression of milk. Breastfeeding sup- is back with the infant, she should put the infant to breast at the
port should be available from all involved hospital staff, and a certified usual interval rather than using the previously expressed milk. Many
lactation consultant can be quite helpful. Early supplementation with mothers can work and breastfeed well past a child’s first birthday.
expressed colostrum should be started for infants who are at high risk Sometimes pediatricians are consulted about weaning the breast-
of breastfeeding problems, such as those who are preterm, multiples, fed infant. There is no age at which weaning must occur, and in
weigh less than 2.7 kg (<6 lb), or feeding poorly for 12 hours, as well many cultures toddlers nurse until age 3 to 4 years. Although some
as for those whose mothers had cesarean section, prior breast sur- infants readily give up the breast, others are more reluctant to do
gery, or previous breastfeeding problems. Early supplementation with so. Lactation consultants may be a valuable resource at this time.
expressed colostrum can decrease excessive weight loss in the infant.
Urine and stool output can be a reliable indicator of the success
Potential Problems
of breastfeeding. A successful breastfeeding neonate should urinate
3 times a day and pass stool 3 to 4 times a day by 3 to 5 days of Attachment
age. By day 5 to 7 of age, the neonate should urinate 4 to 6 times a Latching-on is the first step that is essential for successful breastfeed-
day and pass 3 to 6 stools a day. Weight loss should be monitored ing. Getting some infants to latch may be difficult initially because
and should not exceed 10% of birth weight without further evalua- of sleepiness or fussiness when attempts are made. It is best to start
tion. Mothers whose newborns are taken to the neonatal intensive feedings when the infant exhibits early feeding cues, such as licking
care unit must be helped with pumping or hand expressing their lips or bringing hands to the mouth. Placing the infant skin to skin
breasts within the first hour after delivery. By encouraging initial and in a vertical position on the mother’s chest while she is leaning back
frequent expression of milk, a mother’s milk supply can be estab- can stimulate reflexive infant feeding behaviors. Infants can often
lished even when her newborn cannot directly nurse. maneuver themselves to the nipple with a little help if they are lying
During the first 6 months after birth, the only supplement to on top of their mother as she reclines in bed. The newborn should
breastfeeding that is needed is vitamin D 400 IU daily started in the be positioned with 1 hand on each side of the breast and the chin
first few days after birth to prevent rickets. Intramuscular vitamin K touching the breast and the nipple near the baby’s nose. This allows

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 190 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

the infant to get as much of the areola in the mouth as possible. also be caused in part by interstitial edema and improved by gentle
Causes of poor latching include poor positioning, inverted or flat massage toward the axillary lymph nodes.
nipples, ankyloglossia (ie, tongue-tie), small mandible, engorge- Left untreated, engorgement may result in mastitis, which is breast
ment, or nipple confusion (ie, preference for firm bottle nipples or inflammation with signs of systemic infection, frequently includ-
pacifiers). Management may consist of help with positioning, frenot- ing fever and body aches. If a mother develops mastitis, she should
omy, or use of a supplemental nursing system at the breast. A sup- continue to breastfeed. Emptying the breast is important, but treat-
plemental nursing system is a small tube connected to a syringe or ment also consists of oral antibiotics and rest. Ineffective treatment of
bottle that is slid into the infant’s mouth after the infant is latched mastitis may result in progression to a breast abscess requiring more
onto the breast to give a supplement of expressed milk or formula invasive treatments, such as serial needle aspiration, drain placement,
while the infant breastfeeds. This can provide flow to stimulate suck- or incision and drainage. Incisions should be made parallel to ducts to
ing in a sleepy infant. Occasionally nipple shields are used, but these avoid severing them and away from the areola so the baby can continue
may limit nipple stimulation and cause problems with milk supply. to latch. In cases of frank pus coming from the nipple, the baby should
Thus, mothers who are given shields should be followed closely and not breastfeed until the discharge has resolved. The mother should
encouraged to express milk after feedings to ensure a strong supply. pump the affected breast to empty the milk and support her supply.
In any instance of difficulty with attachment, hand expression or a
pump should be used to ensure frequent effective breast emptying Low Supply
so that milk production continues. The most common reason for early cessation of breastfeeding is
perceived low milk supply. Although 95% of women from devel-
Sore Nipples oped countries are physiologically able to produce sufficient milk,
Breastfeeding should not be painful. If the mother is experiencing many supplement with formula when it is not medically necessary.
pain, the neonate is probably not latching correctly and may be crush- Supplementation may be done because of difficulty with latch or
ing the nipple with his or her gums. This may cause cracked and even unrealistic expectations of newborn behavior causing a lack of con-
bleeding nipples. If the nipple is being compressed the ducts are com- fidence. Formula supplementation leads to decreased frequency of
pressed as well, resulting in poor milk transfer. The nipple may appear breastfeeding, and in time, milk supply decreases if the breasts are
flat or pinched after the feeding. Pediatricians can learn a great deal not emptied by the baby, a pump, or hand expression.
about the status of breastfeeding by checking a mother’s breasts for To encourage exclusive breastfeeding, medical professionals must
fullness and trauma. With a correct latch, neonates have the nipple be able to support a mother’s confidence and ability to breastfeed.
and a significant portion of the areola in their mouth with their lips They must be able to assess milk transfer as well as baby hydration
flanged outward. If a mother experiences pain, the newborn should be and weight gain. When supplementation is necessary, medical pro-
removed from the nipple and attached again. If pain persists, a med- fessionals must enable mothers to express their breasts; often, this
ical professional should evaluate the mother-infant dyad for prob- milk is the only supplement necessary. Any formula supplements
lems. Cracked nipples can be treated with lanolin or hydrogel pads given in the first few days should be of limited volume and should
and repositioning. Mothers should express their milk if the latch is be stopped when mothers experience lactogenesis stage 2. At office
shallow to avoid engorgement and decreased supply. Expressed milk visits, if babies have been given formula unnecessarily, physicians
left to dry on cracked or bleeding nipples has healing properties. In must help families by addressing breastfeeding problems and closely
the setting of significant injury, the mother may need to pump the following the baby’s weight as supplements are decreased and elim-
affected breast for 24 hours while the nipple heals. Occasionally sore inated. In later months, mothers may express concern over shorter
nipples are secondary to a candidal or bacterial infection, atopic der- feeding times, thinking that they have less milk when, in fact, infants
matitis, or Raynaud phenomenon (ie, vasospasm). Raynaud phenom- have become more efficient eaters.
enon can be secondary to trauma, tends to be worse when nipples get In the baby with poor weight gain, a thorough investigation must be
cold, and often is improved with several days of low heat. undertaken to determine whether the difficulty stems from a problem
with the baby, mother’s milk supply, milk transfer, or some combina-
Engorgement/Mastitis tion of these. It is critical to provide the baby with sufficient calories and
Engorgement may occur in the setting of milk stasis for any protect the mother’s supply with pumping during this time. For moth-
reason. The breast appears full, firm, lumpy, and tender. Treatment ers who do lack sufficient milk, more frequent and effective emptying
is to empty the breast, and the newborn or infant is the most effec- of the breast may solve the problem; however, if this is unsuccessful,
tive breast pump. Gentle manual compression along the posterior galactagogues (ie, human milk stimulants) may be useful in some cases.
edge of the glandular tissue may improve emptying. Application of
warm packs or taking a hot shower before feeding can be helpful. Hyperbilirubinemia/Dehydration
Sometimes an electric breast pump expedites emptying, softens the Ten percent of exclusively breastfed infants born via vaginal deliv-
breast, and facilitates baby latching. If it is not possible to empty an ery and 25% of infants born via cesarean section lose more than
area of the breast, such as in cases of a prior surgery that has sev- 10% of their birth weight. Too often, infants are started on for-
ered some ducts, feedback inhibitor of lactation will decrease and mula supplements just as mothers have increasing milk volume.
stop milk production in this area. Postpartum engorgement may For this reason, it is important to check a mother’s breasts at each

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 Chapter 29 : Breastfeeding 191

visit and encourage expression of milk at each feeding if an infant Selected References
requires supplementation. Neonates who lose more than 10% to
12% of their birth weight are at risk for becoming dehydrated and American Academy of Pediatrics, American College of Obstetricians and
Gynecologists. Breastfeeding Handbook for Physicians. Schanler RJ, Krebs NF,
have an increased likelihood of significant jaundice. Increased intake
Mass SB, eds. 2nd ed. Elk Grove Village, IL: American Academy of Pediatrics;
is necessary, and the frequency of feeds should be increased. If a 2014
newborn is not latching on well, expressed milk can be given by
American Academy of Pediatrics, American College of Obstetricians and
syringe. Neonates should be closely monitored, and formula can be Gynecologists. Guidelines for Perinatal Care. Kilpatrick SJ, Papile L, eds. 8th ed.
offered after human milk if milk supply is insufficient. If insufficient Elk Grove Village, IL: American Academy of Pediatrics; 2017
supply is the problem, the mother should be assisted in increasing American Academy of Pediatrics Section on Breastfeeding. Breastfeeding and
her milk supply. Using a breast pump will increase supply because the use of human milk. Pediatrics. 2012;129(3):e827–e841 PMID: 22371471
of increased demand. If the newborn appears significantly jaun- https://doi.org/10.1542/peds.2011-3552
diced, serum levels of bilirubin should be obtained. Physiologic jaun- Brenner M. You can provide efficient, effective, and reimbursable breastfeeding
dice is related to hepatic immaturity with decreased conjugation of support—here’s how. Contemporary Pediatrics. 2005;22:66–76
bilirubin as well as decreased excretion. Breastfeeding babies tend to Feldman-Winter LB, Schanler RJ, O’Connor KG, Lawrence RA. Pediatricians
have increased levels of unconjugated bilirubin. Increased feed- and the promotion and support of breastfeeding. Arch Pediatr Adolesc
ing frequently resolves the problem as hydration status improves Med. 2008;162(12):1142–1149 PMID: 19047541 https://doi.org/10.1001/
and frequency of stooling increases. Occasionally, especially in archpedi.162.12.1142
late preterm neonates, treatment with phototherapy is necessary. Lawrence RA, Lawrence RM. Breastfeeding: A Guide for the Medical Profession.
Interrupting breastfeeding is neither necessary nor helpful. For a 8th ed. Philadelphia, PA: Elsevier; 2016
more extensive discussion of jaundice, see Chapter 126. Ryan SA, Ammerman SD, O’Connor ME; American Academy of Pediatrics
Committee on Substance Use and Prevention, Section on Breastfeeding.
Resources for the Breastfeeding Marijuana use during pregnancy and breastfeeding: implications for neonatal
and childhood outcomes. 2018;142(3):e20181889 PMID: 30150209 https://doi.
Mother org/10.1542/peds.2018-1889
Many hospitals and health care organizations have lactation special- Sachs HC; American Academy of Pediatrics Committee on Drugs. The transfer
ists who can assist nursing mothers and answer questions related to of drugs and therapeutics into human breast milk: an update on selected top-
lactation. Health facilities may loan or rent electric breast pumps to ics. Pediatrics. 2013;132(3):e796–e809. Reaffirmed May 2018 PMID: 23979084
new mothers to help establish a good supply of milk. Some moth- https://doi.org/10.1542/peds.2013-1985
ers may choose to purchase such pumps, especially if they are plan- US Department of Health and Human Services. The Surgeon General’s Call to
ning to continue to breastfeed after returning to work. Breast pumps Action to Support Breastfeeding. Washington, DC: US Department of Health and
usually are covered by health insurance. Human Services, Office of Surgeon General; 2011 PMID: 21452448
Access to information can also be obtained through the inter- U.S. Preventive Services Task Force. Primary care interventions to promote
net, community agencies, or international and national organiza- breastfeeding: U.S. Preventive Services Task Force recommendation state-
ment. Ann Intern Med. 2008;149(8):560–564 PMID: 18936503 https://doi.
tions such as La Leche League International (www.lalecheleague.
org/10.7326/0003-4819-149-8-200810210-00008
org) and the Special Supplemental Nutrition Program for Women,
Wellstart International. Lactation Management Self-Study Modules: Level I.
Infants, and Children. Such agencies provide a resource to the
Naylor AJ, Wester RA, eds. 4th ed. Shelburne, VT: Wellstart International; 2013
health care professional in assisting mothers with breastfeeding.

CASE RESOLUTION
In the case presented, the mother is aware of the many benefits of breastfeeding. You
examine her breasts and note normal anatomy and easily expressed colostrum. The
mother should be encouraged to breastfeed and reassured that she should be able
to make adequate milk with early effective breast emptying. You recommend she
stop cigarette smoking completely for her own health as well as the baby’s; if she is
unable to do so, however, she should be advised that breastfeeding is still superior to
formula. In the hospital, she should request that her newborn be placed skin to skin
with her in the delivery room and continue rooming-in to breastfeed on demand.
If this is not possible, she should initiate hand expression in the first hour after the
birth. Reassure her that the hospital and your office will give her support and guid-
ance with breastfeeding. Even though she is anticipating returning to work, she
should initially nurse exclusively. She can begin to introduce the bottle with pumped
milk at 1 to 2 months of age. Her workplace should provide an area for nursing
mothers to pump and refrigerate the milk. You encourage her to explore the lactation
policies at her workplace and to seek out nursing support groups.

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 CHAPTER 30

Sleep: Normal Patterns

and Common Disorders
Geeta Grover, MD, FAAP, and Thusa Sabapathy, MD

CASE STUDY
During a routine 6-month health maintenance visit, a Questions
mother states that although her 6-month-old son falls 1. How old are most infants when they can begin to
asleep very easily at approximately 10:00 pm every sleep through the night (≥5 hours at a time) with-
night while breastfeeding, he wakes every 2 to 3 hours out a feeding?
and cries until she nurses him back to sleep. A review 2. What factors contribute to frequent nighttime
of the dietary history reveals that the infant is breast- awaking during infancy?
fed approximately every 3 hours and was begun on rice 3. What advice can be given to parents to facilitate an
cereal 2 weeks prior to this clinic visit. His immunizations infant’s sleeping through the night?
are current. The boy has no medical problems, and his 4. What are sleep disturbances experienced by older
physical examination is normal. children and adolescents?
5. What advice can you give parents about helping
children develop good sleep hygiene?

Sleep disorders are common during infancy, childhood, and adoles- ADHD have significant symptoms of impulsivity and hyperactivity
cence. Getting children to go to bed, fall asleep, stay asleep, and stay that can make settling down for bed difficult. In addition, psycho-
in bed can be no small challenge. Parents frequently ask pediatricians stimulant use has been associated with disturbed sleep; interest-
about sleep-related problems at routine health maintenance visits. ingly, however, it also has been shown to have the paradoxical effect
Age-appropriate suggestions on how to help children sleep well are of regulating children with ADHD and getting them ready for sleep.
usually welcomed by parents. An estimated 50% to 80% of children with ASD experience sleep-
related difficulties. In typically developing children, behavioral rea-
Epidemiology sons are the most common causes of insomnia. In children with
Sleep problems occur in 20% to 30% of typically developing children ASD, however, insomnia is multifactorial. In these children, insom-
and are among the most common concerns encountered in pediat- nia is the result of behavioral issues; medical, neurologic, and psychi-
ric practice. Behavioral sleep problems, including bedtime refusal or atric comorbidities; and the secondary effects of medications used
resistance, delayed sleep onset, and prolonged night awaking requir- to manage the symptoms of ASD and the associated comorbidities.
ing parental intervention, are the most common reasons for sleep In most Western countries, children are expected to sleep in
concerns. Inadequate sleep in children negatively affects the quality their own beds. In many cultures, however, it is not uncommon
of life of both the children themselves and their parents. Increased for newborns, infants, and young children to sleep in their parents’
risk for obesity, mood and behavior problems, as well as impaired bed (ie, the “family bed”). Bedsharing with newborns and infants
concentration and academic failure are some of the consequences younger than 10 to 12 weeks is associated with a higher incidence
associated with insufficient sleep in children. of sudden unexpected infant death, especially if the mother smokes.
Higher than normal rates of sleep disturbances are seen in chil- Accidental asphyxia from overlaying or the presence of soft bed-
dren with medical, neurodevelopmental, and psychiatric disorders, ding or overheating may contribute to bedsharing–related deaths.
such as obstructive sleep apnea (OSA), autism spectrum disorder Parents should always be advised about safe sleeping practices (see
(ASD), attention-deficit/hyperactivity disorder (ADHD), intellec- Chapter 72). In older infants and children, co-sleeping is not a prob-
tual disability, anxiety, and depression. An estimated 25% to 50% lem in and of itself, and the decision to co-sleep, like the decision to
of children with ADHD have sleep problems, especially difficulties breast- or bottle-feed, is an entirely personal one. Most newborns
in initiating and maintaining sleep. The relationship of ADHD and and infants who share a bed with their parents have sleep-onset asso-
sleep problems is often complex and multidirectional. Children with ciations that facilitate falling asleep. Therefore, parents who share a

193

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 194 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

bed with their young children commonly must lie down with them hours of the night, with lighter stages of NREM and REM sleep
for 20 to 30 minutes to get them to fall asleep. Several studies have occurring during most of the rest of the night. Although sleep stages
shown that co-sleeping infants are 2 to 3 times more likely to expe- are the same in infants and adults, several differences exist between
rience night awaking than those who sleep alone. Furthermore, the onset and duration of REM and NREM sleep in infants and
newborns and infants who are breastfeeding and bedsharing sleep adults. First, the sleep cycle is shorter in infants than in adults
the shortest periods before awaking. Parents who plan to co-sleep (50–60 minutes and 90–100 minutes, respectively), which means
with their newborns and infants for only a limited period must that infants have more periods of active REM sleep than adults.
develop a clear transition plan, such as ending this practice by 5 to Second, the total amount of time spent in REM sleep decreases with
6 months of age, before infants are old enough to object excessively. increasing age. Full-term newborns spend approximately 50% of
For children with sleep problems, bedsharing is not a good solution. their total sleep time in REM sleep, whereas for preterm newborns
In the absence of preexisting sleep problems or psychological con- up to 80% of total sleep time is spent in REM sleep; this decreases
cerns, however, co-sleeping as a lifestyle choice has not been associ- to approximately 30% by 3 years of age and to 20% by adulthood.
ated with any long-term developmental, behavioral, or psychological Third, infants may have very little REM latency, entering their first
problems in the co-sleeping children. REM cycle very shortly after falling asleep. Adults, in comparison,
generally enter their first REM period approximately 90 minutes
Clinical Presentation after the onset of sleep.
Melatonin, a hormone released by the pineal gland, regulates
Parents may raise concerns about their child’s sleep pattern during
the sleep-wake cycle. It is often called the “Dracula of hormones”
a routine health maintenance visit. However, many parents may
because peak levels occur at night. It has both hypnotic (ie, sleep
not volunteer information about their children’s sleep or may not
promoting) and chronobiotic (ie, sleep phase-shifting) effects on the
appreciate the potential relationship between sleep problems and
daytime behaviors, learning, attention, or overall health. Thus, it is sleep-wake cycle. After melatonin is released into the bloodstream,
important for health professionals to routinely screen children for it is taken into tissues expressing the receptors specific for melato-
sleep disorders. nin and signals the body to prepare for nighttime. The pineal gland
is under the control of the suprachiasmatic nucleus, which resides
in the hypothalamus. When humans are exposed to light, a signal
Pathophysiology
from the retina is sent to the suprachiasmatic nucleus and subse-
To understand disturbances associated with sleep, it is necessary to quently to the pineal gland, thereby suppressing release of melatonin.
understand the physiology of normal sleep and the development of
normal sleep behavior in children. Sleep-Wake Patterns
Sleep patterns follow a normal developmental sequence in chil-
Sleep States dren, and the amount of sleep children need changes with matura-
Normal sleep has 2 distinct states—rapid eye movement (REM) tion (Table 30.1). Through age 12 months, infants sleep 12 to 16 hours
and non–rapid eye movement (NREM) sleep. Rapid eye movement a day. Many infants can sleep through the night (≥5 hours unin-
sleep develops at approximately 29 weeks of gestation and persists terrupted) by age 3 months, and most infants are capable of this by
throughout life. It is an active, lighter stage of sleep that occurs in age 4 months. Brief arousals are a normal part of the sleep cycle at
association with rapid eye movements. Other features of REM sleep all ages, but children should be able to return to sleep on their own
include suppression of muscle tone; rapid, irregular pulse and respi- without requiring parental attention. Children should be able to fall
ratory rate; and body twitches. Dreams occur during REM sleep. asleep on their own by age 4 to 6 months. Otherwise, parental partic-
The pattern of REM sleep noted on electroencephalography (EEG) ipation to fall asleep becomes required at every awaking through-
is very similar to stage 1 NREM sleep. out the night.
Non–rapid eye movement sleep begins at approximately 32 to
35 weeks of gestation. During NREM sleep, pulse and respiratory
rates are slower and more regular and body movements are min- Table 30.1. Total Recommended Amount
imal. Most of the restorative functions of sleep occur during this of Sleep in a 24-Hour Period by Age
state. After the first several months after birth, NREM sleep may be Age Total Number of Hours
divided into 3 stages. Stage 1 includes drowsiness and the begin-
Newborn–12 months 12–16
ning of sleep with slow eye movement. Stage 2 is sleep without eye
movement. Stage 3 is deep sleep (also called slow-wave sleep). Each 1–2 years 11–14
stage represents a progressively deeper state of sleep and has a char- 3–5 years 10–13
acteristic EEG tracing. 6–12 years 9–12
13–18 years 8–10
The Sleep Cycle
Adapted with permission from Paruthi S, Brooks LJ, D’Ambrosio C, et al. Recommended amount of
Rapid eye movement and NREM sleep together make up the sleep sleep for pediatric populations: a consensus statement of the American Academy of Sleep Medicine.
cycle. Typically, the deepest sleep takes place during the first several J Clin Sleep Med. 2016;12(6):785–786.

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 Chapter 30: Sleep: Normal Patterns and Common Disorders 195

One- to 2-year-olds sleep 11 to 14 hours per day, and by age 3 to

Box 30.1. Practical Approach to the Differential
5 years, children sleep a total of 10 to 13 hours per day. One-year-
Diagnosis of Sleep Disorders in Children
olds take 2 naps per day. This typically decreases to 1 afternoon nap
by 18 to 24 months of age. Most children take an afternoon nap until Difficulty Falling Asleep
3 years of age, and some children continue this until 5 years of age. Circadian and Sleep Schedule Disturbances
The amount of nighttime sleep children need continues to gradu- ww Irregular sleep-wake patterns
ally decline, decreasing from approximately 12 hours during the ww Advanced sleep phase
preschool years to approximately 8 to 10 hours during adolescence. ww Delayed sleep phase
Adolescents are often chronically sleep deprived because of a ww Time in bed exceeds sleep requirement
combination of biologically driven processes and modern lifestyle ww Regular but inappropriate sleep schedules without phase shifts (eg, late
demands (eg, digital media use, excessive extracurricular activities). evening naps)
Biologically, around the time of pubertal onset, adolescents begin
Habits, Associations, and Expectations
to experience changes in their circadian rhythm, with delay of sleep
ww Inappropriate sleep-onset associations
onset and wake times by up to 2 hours (ie, sleep-wake phase delay).
ww Bedtime refusal/struggles
It has been suggested that this occurs because of delayed melatonin
ww Poor or inconsistent limit setting
secretion, as well as slower accumulation of sleep drive, resulting in
inability to fall asleep at an appropriate bedtime. In addition, teen- Overstimulation
agers have more demands and expectations in the late afternoon, Psychosocial
including homework and extracurricular activities, that push their ww Separation anxiety
bedtime later. Ultimately, adolescents are not getting the recom- ww Nighttime fears (eg, of the dark, of monsters)
mended 8 to 10 hours of sleep per night, which can have serious ww Family and social stresses
consequences, including impaired driving, academic decline, and Medical
depression. Emerging studies have demonstrated that early middle ww Acute illness
school and high school start times can interfere with total nighttime ww Underlying medical problems
sleep in adolescents. The American Academy of Pediatrics supports ww Medications (eg, antihistamines, stimulants, codeine, anticonvulsant agents)
delaying start times in middle schools and high schools to relieve
Difficulty Maintaining Sleep/Nighttime Awaking
sleep deprivation in adolescents and its associated effects. Discussion
Normal Variation (eg, Breastfed Infant)
of the pathophysiology of sleep is incomplete without inclusion of
Habits, Associations, and Expectations
the effect of electronic media. Use of electronic media in the eve-
ww Inappropriate sleep-onset associations (eg, age-inappropriate night
ning hours can disrupt sleep-wake patterns because of several rea-
awaking for feeding)
sons. Media use may directly displace sleep time. Media content
may cause physiologic arousal, making it difficult to fall asleep and Psychosocial
negatively affecting overall quality of sleep. Additionally, the light ww Nighttime fears
emitted by the devices themselves may disrupt circadian rhythms ww Family and social stresses
by suppressing endogenous melatonin secretion, making it difficult
Medical
to fall asleep at the desired bedtime.
ww Acute illness
ww Underlying medical problem
Sleep Abnormalities
ww Medications
The etiology of sleep disorders can be complex, involving the inter-
action of children’s temperamental characteristics, psychosocial Nightmares
stressors in the home, parental child-rearing philosophies, and the Arousal Disorders
developmental nature of normal sleep states and sleep cycles. ww Night terrors
ww Sleepwalking
ww Sleep talking
Differential Diagnosis
Miscellaneous Sleep Disorders
The differential diagnosis of sleep disorders may be distinguished Intrinsic Sleep Disorders
by problems associated with falling asleep or maintaining sleep (eg, ww Narcolepsy
frequent night awaking) (Box 30.1). Falling asleep may present 2 types ww Sleep apnea (obstructive or central)
of difficulties: problems associated with settling children to sleep ww Restless legs syndrome
and bedtime refusal. ww Periodic leg movements
Inappropriate sleep-onset associations and poor or inconsistent
parental limit-setting are the most common reasons for difficulty Sleep-Wake Transition Disorders
settling infants and children to sleep. Inappropriate sleep-onset ww Head banging
associations in infants are characterized by prolonged night awaking ww Rocking

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 196 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

episodes that require parental participation (eg, holding, rocking, between the child’s inherent biological rhythms and the environ-
feeding) to fall asleep. They have not learned the critical skills of self- ment. Entrainment requires predictable occurrence of time cues,
calming and initiating sleep on their own. Because these infants do such as light and dark, mealtime, and bedtime. A consistent awak-
not have the self-soothing behaviors necessary to fall back to sleep ing time in the morning is among the most important of these cues.
after normal nighttime arousals, they also may experience nighttime Time in bed exceeding actual sleep requirement is a common cause
awaking. Brief arousals are a normal component of sleep. Nighttime of insomnia at any age and occurs when children are expected to
awaking is different because of the need for parental participation to sleep more than is necessary.
resettle the infant. The problem is the difficulty that infants experi- Irregular sleep-wake cycles may occur in children living in cha-
ence falling back to sleep on their own, not the awaking itself. otic environments with irregular mealtimes and sleep-wake sched-
An example of an inappropriate sleep-onset association is the ules. A delayed sleep phase and regular but inappropriate sleep-wake
infant or child who needs to be breast- or bottle-fed to fall asleep. schedule are the most common forms of sleep rhythm disturbance.
These children need to be fed before going back to sleep after nor- Children with delayed sleep phase have a resetting of their circadian
mal nighttime awaking. Although they are developmentally old rhythm; they are not sleepy at bedtime and have excessive morning
enough to receive all nutrition during the day, they have become sleepiness. This is a common problem, because the inherent circa-
conditioned to require nighttime feedings. These children are often dian clock has a cycle closer to 25, not 24, hours. This clock has not
breast- or bottle-fed until they fall asleep and only then placed in the been entrained to a 24-hour schedule in these children.
crib. They are conditioned to require feeding to initiate sleep, and One example of a regular but inappropriate sleep-wake sched-
when they experience normal nighttime arousals, they require the ule is napping at the “wrong” time (eg, a child who regularly naps at
breast or bottle to go back to sleep. Similarly, children who lack the 7:00 pm for 1 hour and then has trouble going to bed at 9:00 pm).
self-comforting and self-initiating skills necessary to fall asleep on Another is seeming confusion between day and night in some
their own will awake, cry, and want to be held, comforted, or enter- infants, who sleep most of the day and stay up most of the night.
tained before they can go back to sleep. Behavioral insomnia result- Night terrors (pavor nocturnus), sleepwalking (somnambulism),
ing from poor or inconsistent parental limit-setting is most common and sleep talking (somniloquism) are all forms of partial awaking
in preschool-age and older children. These children actively resist that occur during deep or stage 4 NREM sleep, most often during the
going to bed with verbal protests and multiple or repeat demands at transition from stage 4 NREM sleep to the first REM sleep period.
bedtime (“curtain calls”). These children may also have difficulty Sleepwalking and sleep talking usually occur during the school-age
settling back to sleep at night, especially if nighttime fears or anxi- years, whereas night terrors begin during the preschool years.
ety contributed to their resistance to going to bed. Night terrors and nightmares may begin during the preschool
Acute illness also may be a cause of sleep disturbances. Children years and may continue throughout childhood. Night terrors are
with otitis media may awake at night because of pain. They may different from nightmares and occur during a different stage of the
continue to experience awaking after the infection has resolved, sleep cycle (Table 30.2). With night terrors, children usually sit up in
however, and require comforting or some sort of attention to fall bed and cry or scream inconsolably for up to 15 minutes. They may
asleep again. appear dazed and have signs of autonomic arousal, such as tachy-
In infants and children between the ages of 9 and 18 months, cardia, tachypnea, and sweating. These children cannot be consoled.
separation and separation anxiety may also affect sleep patterns. When they finally go back to sleep, they do not remember the event
Children may cry when parents leave the room and have difficulty in the morning. Because parents are often frightened by the expe-
settling to sleep. Ability to climb out of the crib or bed can be associ- rience, they may think the child is having a seizure or is having
ated with nighttime awaking in older toddlers. The transition from a
crib to a bed is usually made between 2 and 3 years of age. Children
who can climb out of their cribs or beds may come out of their rooms Table 30.2. Nightmares Versus Night Terrors
repeatedly for drinks of water, trips to the bathroom, or to sleep in
Characteristic Nightmare Night Terror
the parent’s or parents’ bed. Such factors as nighttime fears of the
dark influence sleep behaviors during the preschool years (3–5 years Time of night Late Early, usually within 4 hours of
of age). Children’s growing needs for autonomy and control over bedtime
their environment may result in bedtime refusals during the tod- Sleep stage Rapid eye move- Partial arousal from deep non–
dler and preschool years. ment sleep rapid eye movement sleep
Disorders of the sleep-wake cycle may contribute to sleep sched- State of child Scared but Disoriented, confused, and
ule irregularities. Circadian rhythms govern the regularity and consolable inconsolable
degree of wakefulness and sleepiness. The circadian clock inher- Memory of event Clear recall of dream Usually none
ent in humans is not an exact 24-hour pattern but can be modi-
Return to sleep Reluctant because Easily, unless fully awake
fied or entrained onto one by environmental cues. Parents must
of fear
provide regular and consistent structure, because development
of children’s sleep-wake rhythms is dependent on an interaction Management Reassure child Reassure parents

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 Chapter 30: Sleep: Normal Patterns and Common Disorders 197

an emotional disturbance and may seek medical advice. Although with intermittent pauses or gasps; disturbed sleep; and daytime neu-
attacks may be precipitated by stressful events or fatiguing daytime robehavioral problems. Obstructive sleep apnea syndrome should
activities, night terrors do not indicate excessive stress or emotional be distinguished from primary snoring, which is defined as snoring
disturbance in children’s lives unless they recur. without obstructive apnea, arousals from sleep, or abnormalities in
Nightmares usually occur during the last third of the night dur- gas exchange. Obstructive sleep apnea syndrome not only has the
ing REM sleep, whereas night terrors more often take place during potential to disturb the quality of sleep but also can cause poten-
the early part of the night. Nightmares are scary dreams that may tially serious complications, such as failure to thrive and, in severe
awaken children, who can often remember them. Children usually cases, cor pulmonale.
can be consoled by parents but are reluctant to go back to sleep Restless legs syndrome, or periodic leg movements, is characterized
because of their fears. by uncomfortable creeping or crawling feelings, mainly occurring
Excessive daytime sleepiness can be a symptom of medical prob- in the lower extremities, when the child is resting or inactive and is
lems, such as illness, narcolepsy, sleep apnea, and depression. Viral relieved by movement. The condition may be attributed to growing
illness is perhaps the most common medical cause of such sleepiness pains in younger children (see Chapter 116) and can be associated
in children. Inadequate sleep at night is another potential cause of with delayed sleep onset.
sleepiness during the day. Screening for daytime impairments (eg,
decline in academic performance, inattention) is important in chil- Evaluation
dren suspected of having sleep disorders. Primary sleep disorders, History
such as OSA, have been shown to be associated not only with exces-
Evaluation of children with sleep difficulties begins with a thorough,
sive daytime sleepiness but also with cognitive deficits and impaired
detailed sleep history taken from parents and, if old enough, chil-
attentional capacity.
dren themselves (Box 30.2). A thorough history includes informa-
Narcolepsy, a disorder of excessive sleepiness, is characterized
tion about total daily sleep in 24 hours; daytime sleep and nighttime
by an overwhelming desire to sleep during the daytime despite ade-
quate sleep at night. Symptoms include excessive daytime sleepiness,
cataplexy, sleep paralysis, and hypnagogic hallucination. Cataplexy
is an abrupt loss of muscle tone that usually is precipitated by an Box 30.2. What to Ask
emotional reaction, such as laughter or anger. Sleep paralysis is an Detailed Sleep History
inability to move or speak that occurs as individuals fall asleep or ww Does the child have regular nap times and bedtimes, or do these depend
awaken. Hypnagogic hallucination, which can be visual or auditory, on changing parental schedules?
occurs while falling asleep. Narcolepsy affects approximately 0.05% ww What time does the child go to bed?
to 0.1% of the general population. The prevalence increases to 50% ww What does the child do in the hour before bedtime? Is there a consistent
of family members with a positive family history for the condition. bedtime routine, even on weekends?
The exact genetic basis of inheritance is unknown. The age of onset ww Does the child watch television, play video games, or use the internet or
is usually between 10 and 20 years. Diagnosis is often delayed or mobile telephone in the hour before bed?
missed for months to years in some cases because not all symptoms ww Where does the child sleep (eg, ask about co-sleeping, noise,
may be present initially. Diagnosis is important, because pharmaco- temperature)?
logic therapy with central nervous system stimulants may provide ww Can the child fall asleep without parental participation?
some symptomatic relief. ww Does the child require feeding or fluids at night?
Sleep-related breathing disorders (SRBDs) occur on a spectrum, ww Can you provide a detailed explanation of when the sleep problem
with habitual snoring the least severe form and OSA the most severe occurs relative to bedtime and what the child does?
form. Risk factors associated with the development of SRBDs include ww How often does the sleep problem occur?
obesity, presence of chronic sinus problems, recurrent wheezing, ww How long has the child been having sleep problems?
nasal allergies, family history of OSA, and certain genetic disor- ww How does the parent respond?
ders (eg, Down syndrome, Prader-Willi syndrome). Obstructive ww Does the child snore?
sleep apnea in children is a disorder of breathing during sleep that ww When does the child wake up in the morning? Is the child difficult to awake?
is characterized by prolonged partial upper airway obstruction or ww Is the child sleepy during the day?
intermittent complete obstruction (ie, obstructive apnea) that dis- ww How much caffeine does the child consume, for example, in coffee, tea,
rupts normal gas exchange and sleep patterns. Risk factors for OSA soda, or chocolate?
include adenotonsillar hypertrophy, obesity, craniofacial anomalies, ww Is the child taking any medications?
and neuromuscular disorders. Obstructive sleep apnea syndrome ww Is there a family history of sleep disturbances?
(OSAS) is thought to be secondary to a combination of adenoton- ww Is there any stress within the home resulting from marital or financial
sillar hypertrophy and reduced neuromuscular tone of the upper difficulties that may affect the home environment and cause the child to
airway during sleep. Large tonsils and adenoid alone are not neces- be anxious or stressed?
sarily diagnostic for OSA. Symptoms include nightly snoring, often

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 198 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

sleep; the sleep environment; bedtime rituals and routines; weekday sleep laboratory, may be useful in certain children when significant
versus weekend schedules; snoring; and evening routines, including sleep disturbances, such as nocturnal seizures, narcolepsy, or OSAS,
screen time. The use of a specific screening questionnaire, such as are suspected. Any child with suspected SRBD (eg, history of snoring,
the Children’s Sleep Habits Questionnaire, may facilitate the evalua- abnormal breathing during sleep) should be evaluated for OSAS.
tion. This questionnaire is designed to screen for the most common The use of consumer sleep technology in the form of wearable
sleep problems in children aged 4 to 12 years. It is not intended to devices and downloadable programs on mobile devices for track-
be used to diagnose specific sleep disorders but rather to identify ing sleep cycles and overall sleep quality have become increasingly
children who may require further evaluation. The simple screen- popular. The accuracy of the collected data and the relation of the
ing acronym BEARS (bedtime resistance/sleep-onset delay; exces- data to sleep disorders is unclear, however. It would be advantageous
sive daytime sleepiness; awaking at night; regularity, patterns, and for health care professionals to be aware of the types of available
duration of sleep; and snoring and other symptoms) can be useful as consumer sleep technology applications to further the discussion
an initial screening tool to determine whether further assessment is of sleep-related problems. However, it does not replace a full clin-
necessary. Evaluation should also include an assessment of the child’s ical evaluation.
temperament, psychological well-being, and developmental status.
Children with neurodevelopmental or psychological concerns have
Management
increased rates of sleep disturbances.
Asking about evening screen time is an important component The goal of management is to help children develop a healthy pattern
of the sleep history, because electronic media use can disrupt sleep. of sleeping, not simply to eliminate the immediate problem. Healthy
Increased viewing of media on mobile electronic devices, such as sleep associations include providing a consistent schedule of naps
tablet devices and smartphones, near bedtime has been reported. and bedtime, along with a pleasant bedtime routine. It is important
Seventy-five percent of children have at least 1 technological device to put newborns and infants in their crib while they are relaxed
in their bedroom. Approximately 1 in 10 children 13 to 18 years of and drowsy but not already asleep. This gives them the opportunity
age is awakened after going to bed every night or almost every night to develop skills to put themselves to sleep. If they become accus-
by a telephone call, text message, or email. Emerging data indicate tomed to being fed or rocked until they fall asleep, they will seek the
that screen time in the evening is associated with delayed sleep onset, same means of falling asleep every time they normally awaken dur-
later bedtime, shorter sleep duration, and poor overall sleep quality. ing the night. In addition, overstimulation in the evening may make
The intake of caffeinated substances in the form of coffee and settling to sleep difficult for toddlers or young children. Instead, a
energy drinks is another component of the history to consider in routine such as a bath followed by a story in the child’s bedroom
adolescents experiencing sleep disturbances. Between 30% and 50% with a clearly defined end point when the parent leaves the child in
of adolescents have reported consuming energy drinks, which con- the crib or bed sleepy but awake may help facilitate sleep. Children
tain not only large amounts of caffeine but also large amounts of must learn to fall asleep on their own.
sugar and legal stimulants that can have dangerous side effects (eg, The mainstays of treatment of infants and children with inap-
irregular heartbeat, heart failure, anxiety, dehydration, insomnia). propriate sleep-onset associations is to put them in their crib or
The recommended maximum daily caffeine intake for adolescents bed when they are sleepy but awake followed by systematic ignor-
aged 12 through 18 years is 100 mg, that is, the amount of caffeine ing (ie, “extinction”) when they awake at night. Their last memory
in a single cup of coffee. before falling asleep should not be of a parent holding or feeding
them. If the crying persists, parental contact with the newborn or
Physical Examination infant should be brief and boring (ie, nonstimulating). Scheduled
A thorough physical examination is important to rule out organic awaking is a technique in which the infant is slightly aroused by the
causes of sleep difficulties. Special attention should be paid to the air- parent 15 to 60 minutes before an expected spontaneous awaking in
way and nervous system. Conditions that may alter the sleep-wake an effort to prevent spontaneous awaking. Scheduled awaking may
cycle include acute illness (eg, otitis media), OSA (eg, resulting from be an effective treatment alternative for some infants who awake,
adenoidal or tonsillar hypertrophy), colic, gastroesophageal reflux, cry, and require parental soothing to fall back to sleep. If an infant
and any central nervous system disease or abnormality. awakes for a feeding, the parent should try to stretch the interval
between waking and feeding so that the infant has an opportunity
Laboratory Tests to practice self-calming techniques.
In most cases, a detailed history and physical examination are suf- Implementation of good sleep hygiene practices is an important
ficient to establish the reason for sleep disturbance; laboratory first step for school-age children and adolescents who are experienc-
assessment is rarely necessary. If further evaluation is warranted, ing sleep problems, especially sleep-onset problems. Sleep hygiene
it should be individualized to the child’s clinical presentation. refers to the establishment and maintenance of schedules and con-
Electroencephalography may be useful if a central abnormality, such ditions conducive to healthy, restorative sleep. Good sleep hygiene
as a seizure disorder, is suspected. Polysomnography, which is the practices are listed in Box 30.3. Limiting screen time, especially
simultaneous monitoring of EEG, electrocardiography, chin muscle in the evening, is an important component of good sleep hygiene
tone, eye movements, and respirations during a night of sleep in a practices.

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 Chapter 30: Sleep: Normal Patterns and Common Disorders 199

or psychiatric disorders. It is important to carefully review the

Box 30.3. Good Sleep Hygiene Practices
potential effects of psychotropic medications with regard to
Environment the child’s sleep. Commonly used psychotropic medications in
ww Dark. children that may affect sleep include psychostimulants, selec-
ww Quiet. tive serotonin reuptake inhibitors, and atypical antipsychotic
ww Comfortably cool. medications.
Schedule Medications to manage insomnia include over-the-counter
ww Regular bedtime. preparations and prescription medications. Antihistamines, mel-
ww Regular awaking time. atonin, and herbal supplements are common over-the-counter
ww Naps, if needed, should be early in the day and of a consistent time and options. Antihistamines are generally well tolerated because of
duration. their rapid onset of action and relatively short half-life. However,
potential adverse reactions include “paradoxical” stimulation or
General disinhibition, anticholinergic effects, and development of tolerance
ww No frightening/stimulating television, video games, or stories and no to sedation. Antihistamines are best for short-term situational use
vigorous physical activities in the hour before bedtime. in younger children. Melatonin, which is considered a nutritional
ww Unplug and charge technology devices outside children’s bedrooms to supplement by the US Food and Drug Administration (FDA), has
avoid surreptitious access during sleep hours. both hypnotic and chronobiotic properties. When used as a hyp-
ww Limit caffeine, especially after lunchtime. notic agent to shorten sleep-onset latency, a larger dose is given 30
ww Consistent and calming bedtime routine. to 60 minutes before bedtime, whereas when used as a chronobi-
ww Consistent soothing methods. otic agent, a smaller dose is given 3 to 4 hours before bedtime in
ww Children put to bed drowsy but awake. an effort to shift the circadian rhythm. Currently, the long-term
side effects of melatonin, including its potential for hypothalamic-
gonadal axis suppression, are unclear. The FDA does not monitor
The SPOIL System (social, play, outdoor, independent work, over-the-counter preparations of melatonin, and the actual dose
literacy; www.screenfreeparenting.com) may be helpful in guiding of melatonin may not be the same as listed on the label. Thus, par-
parents on how to fill their child’s time with activities other than ents may want to consider using a pharmaceutical-grade formu-
screen time. The SPOIL System was developed to provide families lation of melatonin to ensure that the tablet contains the stated
healthier options than viewing a screen and includes opportunities amount of melatonin.
for socialization, child-led free play, time in nature, helping with Some children may require referral to a sleep specialist, especially
age-appropriate chores, and education. those children in whom significant sleep disturbances, such as nar-
Specific sleep disorders can be addressed individually. Older tod- colepsy, nocturnal seizures, or OSAS, are suspected.
dlers and preschoolers who delay going to bed or refuse to stay in
their rooms at night need clear, firm limits. These children need con- Prognosis
sistent bedtime routines and nighttime interventions for when they
awake. It may be necessary to install a gate in the bedroom door- It is to parents’ advantage to help their children develop healthy sleep
way to prevent children who refuse to stay in their rooms at night habits rather than ignore the problems and hope that children out-
from moving about the house and potentially hurting themselves or grow them. Children with sleep problems in early childhood are at
disturbing others. Parents of children who have night terrors may increased risk for sleep problems later in life.
require reassurance that their children are not having significant Sleep has an important influence on mood. Emerging data indi-
emotional problems or stressors. A night-light may help alleviate the cate an association between difficulty initiating and/or maintain-
anxieties of preschoolers who are unable to sleep at night because ing sleep with depression in children and adolescents. Additionally,
of their fears of darkness. Disturbances of the sleep-wake schedule sleeping less than 8 hours at night seems to be associated with an
can be corrected over time by gradually shifting children’s schedules increased risk for suicide attempts after controlling for confound-
in the desired direction. For example, in the case of a child with a ing variables.
delayed sleep phase who goes to bed very late, the morning wake-up
time can be progressively advanced approximately 15 minutes per CASE RESOLUTION
day, after which bedtime is progressively advance until the desired The 6-month-old has disordered sleep associations. He has been conditioned
schedule is achieved. to nighttime feedings, although he is old enough not to require them for nutri-
Pharmacologic interventions should be considered only tion. The physician suggests several steps the mother can take to try to solve
after appropriate behavior interventions have been attempted. her son’s sleep problem. She can begin by gradually lengthening the interval
between daytime feedings to 4 to 5 hours. When the baby cries at night, she
Medications for insomnia should be used in combination with a can wait progressively longer before feeding him and then eventually elimi-
behavioral plan that addresses sleep hygiene and unhealthy sleep nate the feedings altogether. He will learn to fall asleep on his own without
practices. It may be necessary to partner with subspecialists when requiring feeding.
treating children with underlying medical, neurodevelopmental,

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 200 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

of insomnia in children and adolescents with autism spectrum disorders.

Selected References
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American Academy of Pediatrics Adolescent Sleep Working Group, Committee peds.2012-0900I
on Adolescence, Council on School Health. School start times for adolescents. Malow BA, Katz T, Reynolds AM, et al. Sleep difficulties and medications
Pediatrics. 2014;134(3):642-649 PMID: 25156998 https://doi.org/10.1542/ in children with autism spectrum disorders: a registry study. Pediatrics.
peds.2014-1697 2016;137(suppl 2):S98–S104 PMID: 26908483 https://doi.org/10.1542/
American Academy of Pediatrics Task Force on Sudden Infant Death Syndrome. peds.2015-2851H
SIDS and other sleep-related infant deaths: updated 2016 recommendations for McClafferty H, Vohra S, Bailey M, et al; American Academy of Pediatrics
a safe infant sleeping environment. Pediatrics. 2016;138(5):e20162938 PMID: Section on Integrative Medicine. Pediatric integrative medicine. Pediatrics.
27940804 https://doi.org/10.1542/peds.2016-2938 2017;140(3):e20171961 PMID: 28847978 https://doi.org/10.1542/peds.2017-1961
Bhargava S. Diagnosis and management of common sleep problems in children. National Sleep Foundation. Melatonin and sleep. www.sleepfoundation.org/
Pediatr Rev. 2011;32(3):91–99 PMID: 21364012 https://doi.org/10.1542/pir. sleep-topics/melatonin-and-sleep. Accessed March 26, 2019
32-3-91
Okami P, Weisner T, Olmstead R. Outcome correlates of parent-child bedsharing:
Byars KC, Yolton K, Rausch J, Lanphear B, Beebe DW. Prevalence, pat- an eighteen-year longitudinal study. J Dev Behav Pediatr. 2002;23(4):244–253
terns, and persistence of sleep problems in the first 3 years of life. Pediatrics. PMID: 12177571 https://doi.org/10.1097/00004703-200208000-00009
2012;129(2):e276–e284 PMID: 22218837 https://doi.org/10.1542/peds.
Owens J; American Academy of Pediatrics Adolescent Sleep Working Group,
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Committee on Adolescence. Insufficient sleep in adolescents and young adults:
Centers for Disease Control and Prevention. The buzz on energy drinks. www. an update on causes and consequences. Pediatrics. 2014;134(3):e921–e932
cdc.gov/healthyschools/nutrition/energy.htm. Accessed March 26, 2019 PMID: 25157012 https://doi.org/10.1542/peds.2014-1696
Common Sense Media. The common sense census: media use by kids age zero to Owens JA. Behavioral sleep problems in children. www.uptodate.com/contents/
eight, 2017. www.commonsensemedia.org/research/the-common-sense-census- behavioral-sleep-problems-in-children#H24. Updated January 14, 2019. Accessed
media-use-by-kids-age-zero-to-eight-2017. Accessed March 26, 2019 March 26, 2019
Hale L, Kirschen GW, LeBourgeois MK, et al. Youth screen media habits and Owens JA, Dalzell V. Use of the ‘BEARS’ sleep screening tool in a pediatric
sleep: sleep-friendly screen behavior recommendations for clinicians, educa- residents’ continuity clinic: a pilot study. Sleep Med. 2005;6(1):63–69 PMID:
tors, and parents. Child Adolesc Psychiatr Clin N Am. 2018;27(2):229–245 PMID: 15680298 https://doi.org/10.1016/j.sleep.2004.07.015
29502749 https://doi.org/10.1016/j.chc.2017.11.014
Owens JA, Mindell JA. Take Charge of Your Child’s Sleep: The All-in-One
Hirshkowitz M, Whiton K, Albert SM, et al. National Sleep Foundation’s Resource for Solving Sleep Problems in Kids and Teens. New York, NY: Marlowe
sleep time duration recommendations: methodology and results summary. & Company; 2005
Sleep Health. 2015;1(1):40–43 PMID: 29073412 https://doi.org/10.1016/j.
Owens JA, Moturi S. Pharmacologic treatment of pediatric insomnia. Child
sleh.2014.12.010
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Keyes KM, Maslowsky J, Hamilton A, Schulenberg J. The great sleep reces- doi.org/10.1016/j.chc.2009.04.009
sion: changes in sleep duration among US adolescents, 1991-2012. Pediatrics.
Owens JA, Spirito A, McGuinn M. The Children’s Sleep Habits Questionnaire
2015;135(3):460–468 PMID: 25687142 https://doi.org/10.1542/peds.2014-2707
(CSHQ): psychometric properties of a survey instrument for school-aged chil-
Khosla S, Deak MC, Gault D, et al; American Academy of Sleep Medicine Board dren. Sleep. 2000;23(8):1043–1051 PMID: 11145319 https://doi.org/10.1093/
of Directors. Consumer sleep technology: an American Academy of Sleep sleep/23.8.1d
Medicine position statement. J Clin Sleep Med. 2018;14(5):877–880 PMID:
Paruthi S, Brooks LJ, D’Ambrosio C, et al. Recommended amount of sleep for
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pediatric populations: a consensus statement of the American Academy of Sleep
LeBourgeois MK, Hale L, Chang AM, Akacem LD, Montgomery-Downs HE, Medicine. J Clin Sleep Med. 2016;12(6):785–786 PMID: 27250809 https://doi.
Buxton OM. Digital media and sleep in childhood and adolescence. Pediatrics. org/10.5664/jcsm.5866
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Shamseer L, Vohra S. Complementary, holistic, and integrative medicine:
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melatonin. Pediatr Rev. 2009;30(6):223–228 PMID: 19487431 https://doi.
Malow BA, Byars K, Johnson K, et al; Sleep Committee of the Autism Treatment org/10.1542/pir.30-6-223
Network. A practice pathway for the identification, evaluation, and management

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 CHAPTER 31

Oral Health and Dental

Disorders
Charlotte W. Lewis, MD, MPH, FAAP

CASE STUDY
The parents of a 9-month-old girl bring her to the office 2. What is meant by “mixed dentition”?
because they are concerned that their daughter has no 3. When should oral hygiene using a toothbrush
teeth yet. Growth and development have proceeded and fluoride toothpaste begin?
normally, and the physical examination is unremarkable. 4. What groups of children are at high risk for dental
caries?
Questions 5. What are the indications for the application of
1. What is the typical first tooth to erupt, and at
fluoride varnish?
approximately what age does that occur?

Healthy teeth allow us to consume a variety of foods, from which from 2011 through 2014 indicate that 36% of children age 2 through
we obtain essential nutrients. Although physicians receive limited 8 years in the United States had experienced caries in their pri-
training about teeth, given the common nature of dental problems mary dentition, and 57% of US children age 12 through 19 years
in children it is important that pediatricians and other pediatric had experienced caries in their permanent dentition. Poverty is
primary care physicians understand and can not only recognize the most important risk factor for caries, and it also affects access
normal and abnormal dental conditions but can implement pri- to professional dental care. In 2015 to 2016, the prevalence of den-
mary and secondary prevention of dental caries and dental inju- tal caries in children age 2 through 19 years in the United States
ries in their practice. Well-child care visits begin in the neonatal increased as family income decreased (Figure 31.1). Among youth
period and continue through the end of adolescence, and at every from families living below the federal poverty level, 56.3% had any
visit opportunities exist to promote oral health and examine oral caries, compared with 34.8% for youth from families with income
structures for timely identification of dental problems. Infancy levels greater than 300% of the federal poverty level. Likewise, the
and early childhood are critical times for the establishment of prevalence of untreated dental caries affected 18.6% of youth from
habits, both good and bad, that have the potential to affect life- families living below the federal poverty level, compared with 7.0%
long oral health. of youth from families with incomes greater than 300% of the fed-
eral poverty level.
Epidemiology Alaska Native/American Indian (AI/AN) children have a mark-
Both dental trauma and dental caries are common in childhood. edly higher prevalence of caries relative to other populations in
Approximately one-third of toddlers and preschool-age children the United States. In 2014, 76% of AI/AN children age 2 to 5 years
and 20% of teenagers experience dental trauma—in young children had experienced at least 1 instance of dental caries, with 47% of all
typically as the result of a fall and in older children most often the AI/AN children in this age group having untreated caries. In con-
result of contact sports. Occlusal abnormalities are also common in trast, during 2011 to 2014, 24% of all US children between 2 and
children. At least 30% of children are estimated to have moderate to 5 years of age experienced caries and approximately 11% of children
severe orthodontic needs. in this age group had untreated caries.
Dental caries is the most common chronic disease of childhood. Toothache, a complication of dental caries, afflicts millions of
Even so, dental decay disproportionately affects low-income indi- US children. In 2007, 14% of 6- to 12-year-olds had experienced a
viduals, resulting in earlier onset of caries, more teeth affected, toothache within the previous 6 months. Toothache disproportion-
more caries-related complications, and ultimately more tooth loss ately affects children who are poor or of minority status, or who have
during adulthood because of caries. Population-based data collected special health care needs.

201

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 202 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

<100% FPG 100–199% FPG 200–299% FPG ≥300% FPG

100

75
Percent

50

25

0
Any caries Untreated caries

Figure 31.1. Percentage of US children, 2 to 19 years of age, with any caries and untreated caries by
family income relative to Federal Poverty Guideline (FPG), 2015–2016.
Adapted from Fleming E, Afful J. Prevalence of total and untreated dental caries among youth: United States, 2015-2016.
NCHS Data Brief. 2018;(307):1–8.

Clinical Presentation Crown Enamel Dentin Pulp

Dental development begins in utero. Subsequently, the teeth
erupt into the lower (ie, mandibular) portion of the jaw and into
the upper (ie, maxillary) jaw. Teeth have a crown and root section
(Figure 31.2). The crown is the visible portion of the tooth. The root Gingival
margin
is that part contained within the socket of the alveolar bone. The
outer hard coating of the crown is the enamel. Beneath the enamel
is the dentin, which is composed of microtubules for transport of
nutrients from the pulp to the outer portions of the tooth. The pulp
contains nerves and vascular structures critical for the health and Cementum
viability of the tooth. Teeth are anchored to the jaw by periodon-
Periodontal
tal ligaments. ligament
Humans have 20 primary or deciduous teeth, which erupt
sequentially between approximately 6 and 34 months of age
(Figure 31.3A). The first primary teeth to erupt typically are the
lower central incisors. These teeth erupt on average at approximately
8 months of age (±1 standard deviation: 6–10 months), after which Alveolar
approximately 1 tooth erupts per month until all primary teeth have bone
erupted, by approximately 24 to 34 months of age. In each quadrant
of the mouth, the normal pattern of primary tooth eruption is that
the incisors erupt first, after which the first molars, the canines (ie,
cuspids), and finally the second molars erupt (Table 31.1). There is
variation in the timing of tooth eruption, and normal tooth eruption Neurovascular
bundle
can vary by 6 months or more from the average age. Occasionally,
the eruption process may be preceded by a bluish discoloration to
the gum, called an eruption hematoma, which is a benign process.
Figure 31.2. Tooth anatomy.
More often, eruption of the primary teeth is associated with more
generalized symptoms, such as fussiness and drooling. These symp-
toms are commonly referred to as teething. Teething does not cause

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 Chapter 31 : Oral Health and Dental Disorders 203

3rd permanent
molar
(wisdom tooth)
2nd permanent
molar
1st permanent
molar
2nd
primary 2nd premolar
molar (bicuspid)

1st 1st premolar

primary (bicuspid)
molar
Cuspid
(canine tooth)

Lateral incisor

A Central incisor B

Figure 31.3. A, Primary dentition. B, Permanent dentition.

Table 31.1. Approximate Ages of Primary Teeth Eruption and Exfoliation in the Upper and Lower Jaws
Erupt Exfoliate
Primary Tooth Name Upper a
Lowerb
Upper Lower
Central incisor 8–12 months 6–10 months 6–7 years 6–7 years
Lateral incisor 9–13 months 10–16 months 7–8 years 7–8 years
Canine 16–22 months 17–23 months 10–12 years 9–12 years
First year molar 13–19 months 14–18 months 9–11 years 9–11 years
Second year molar 25–33 months 23–31 months 10–12 years 10–12 years
a
Upper: Maxillary.
b
Lower: Mandibular.

diarrhea, respiratory infections, or true fever, although these condi- Table 31.2. Approximate Ages of Permanent Tooth
tions may be present coincidentally with tooth eruption in infants. Eruption in the Upper and Lower Jaws
The primary teeth are replaced by the permanent teeth
Erupt
(Figure 31.3B), which begin erupting at approximately 6 years
of age. Permanent and primary teeth are present during the Permanent Tooth Name Upper a
Lowerb
mixed dentition phase, which occurs between 6 and 13 years of age Central incisor 7–8 years 6–7 years
(Table 31.2). During early mixed dentition, the permanent dentition Lateral incisor 8–9 years 7–8 years
looks large and awkward relative to the remaining primary teeth, and Canine 11–12 years 9–10 years
transient malpositioning of the teeth may occur. Normally, the adult
First premolar 10–11 years 10–12 years
mouth has 32 permanent teeth; the last teeth to erupt are the third
molars, commonly referred to as “wisdom teeth,” which emerge at Second premolar 10–12 years 11–12 years
approximately 17 to 21 years of age. First molar 6–7 years 6–7 years
Variations from normal in number of teeth are not uncommon. Second molar 12–13 years 11–13 years
Hypodontia refers to the presence of fewer than normal teeth. The Third molar 17–21 years 17–21 years
most common teeth to be congenitally absent are the third molars, a
Upper: Maxillary.
second premolars, and maxillary lateral incisors. Congenital absence b
Lower: Mandibular.
of a central incisor is distinctly uncommon and should raise con-
cern for the presence of other midline defects. Several teeth may be erupted, known as natal teeth. Teeth that erupt shortly after birth
missing in disorders such as Down syndrome or ectodermal dys- are referred to as neonatal teeth if their eruption occurs in the first
plasia. Anodontia is the congenital absence of teeth. Extra teeth are month after birth. These teeth are usually incisors, and at least 90%
called supernumerary teeth. Children may be born with teeth already represent normal dentition rather than supernumerary teeth. The

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 204 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

presence of natal or neonatal teeth may be familial and, rarely, may Dental fractures may affect the tooth crown, tooth root, and/or the
be suggestive of an underlying syndrome. In addition to variations alveolar bone. Fracture of the crown with no loss of tooth structure—
in number, teeth may also demonstrate variations in color and struc- that is, a crack exists in the tooth but no piece of tooth has bro-
ture resulting from abnormalities in tooth development, trauma, or ken off—is a dental infraction. Usually, these are initially asymp-
extrinsic factors. tomatic. Dental follow up is needed for an infraction because the
The relationship of the maxillary to the mandibular dentition crack may allow passage of bacteria into the pulp, which can result
has functional and aesthetic implications. Malocclusion is an abnor- in pulpal necrosis. Fractures involving the tooth crown, with loss of
mal relationship between the upper and lower teeth and may be tooth structure, are classified depending on the site of the fracture.
developmental, genetic, or environmental in etiology. Children with Fractures through the enamel (Ellis class I) or dentin (Ellis class II)
craniofacial disorders often have significant occlusal problems and are considered uncomplicated dental fractures. Fractures that involve
facial asymmetries necessitating early referral for care by a cranio- the pulp (Ellis class 3), the root, or the alveolar bone are classified as
facial team. Other children may have milder malocclusion requir- complicated dental fractures.
ing orthodontic care. Normal occlusion, or class I occlusion, occurs Caries may occur any time after eruption of the teeth. Early child-
when the maxillary incisors are slightly in front of the mandibular hood caries (ECC), a more general term referring to what in the
incisors and the posterior molars interdigitate (Figure 31.4). Class II past was called “baby bottle tooth decay” or “nursing bottle caries,”
occlusion occurs when the maxillary teeth project too far ante- disproportionately affects children of low socioeconomic status. The
riorly from the mandibular teeth. This may be associated with an pattern of decay seen in ECC is different from that seen in the teeth
overjet, commonly known as “buck teeth,” which can predispose of older children and adults. Typically, ECC first affects the maxil-
to dental injury when children fall. Class III occlusion, or under- lary incisors and spares the lower incisors. This pattern of decay is
bite, occurs when the mandibular teeth are anterior to the maxil- hypothesized to result from prolonged and frequent exposure of
lary teeth. Other common forms of malocclusion include an anterior the teeth to sweet liquids, such as falling asleep with a juice bottle
open bite, whereby the posterior teeth come together but an open- in the mouth, whereby the beverage pools around the upper inci-
ing exists between the top and bottom anterior teeth, and a cross- sors but the lower teeth are protected by the overlying tongue. In
bite, which occurs when some of the upper molars are located inside its earliest stages, ECC appears on physical examination as white,
the lower molars during occlusion. chalky, opaque areas at the gum line (ie, white spot lesions). At this
Dental injuries can be classified into tooth concussion, sublux- early stage, the lesion is potentially reversible if remineralization
ation, luxation, avulsion, and fracture. A concussed tooth follows can occur, such as by applying fluoride varnish to the white spots.
a blow that leaves the tooth tender but not displaced or mobile. In older children, the pit and fissure surfaces of the molars are
Subluxation is loosening of a tooth after injury without displacement. the likely sites of dental decay. Fermentable carbohydrates, partic-
With luxation, or displacement of the tooth from its normal position, ularly those of a sticky nature, become embedded in these surfaces
the tooth is dislodged from its usual location; the tooth may not be and are not easily reached by the bristles of a toothbrush. This allows
mobile at all if it has been forced into adjacent bone. Luxation injuries for prolonged action of acid-producing bacteria and subsequent car-
usually result in damage to the periodontal ligament, threatening the ies formation. As decay invades through the layers of the tooth, it
future viability of the tooth. Intrusion is a form of luxation; it occurs eventually reaches the pulp, resulting in inflammation and necro-
when a tooth is driven into the bone, fracturing the alveolar socket. sis. The infection may then spread around the tooth apex, forming a
An intruded tooth may not be visible at all; alternatively, only the very periapical abscess or fistula. Dental infection can progress to involve
distal aspect of the crown may emerge from the gingiva. Complete the maxilla or mandible and then move into the fascial planes of
loss of the tooth from the socket is referred to as an avulsion. the head and neck, producing abscess, facial cellulitis, or less com-
monly, airway obstruction.

Pathophysiology
Hypodontia may be familial or occur secondary to an underlying
syndrome. However, failure of 1 tooth to erupt is more commonly
caused by another tooth in the path of eruption or insufficient space
in the dental arch. Defects of tooth structure have a variety of causes.
Development of the primary teeth is predominantly subject to pre-
natal influences. The permanent teeth begin to develop in utero and
mineralize after birth, making them susceptible to prenatal and post-
natal exposures. Medications, infection, jaundice, metabolic disor-
Class I Class II Class III ders, and irradiation may adversely affect normal tooth formation
or mineralization. Intrauterine infection, for example, with rubella,
Figure 31.4. Classes of occlusion. Class I is considered most desirable from a cytomegalovirus, or syphilis, may adversely affect tooth structure. A
functional and aesthetic perspective. dental infection involving a primary tooth (eg, a periapical abscess)

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 Chapter 31 : Oral Health and Dental Disorders 205

can damage the developing permanent tooth bud, resulting in a mal-

Box 31.1. Population and Individual
formed permanent tooth.
Risk Factors for Dental Decay
Discolored teeth may be the result of intrinsic or extrinsic factors.
Fetal or early childhood (ie, before age 8 years) exposure to tetracy- Population Risk Factors
cline can cause intrinsic staining of the permanent teeth. Exposure ww Alaska Native or American Indian
to high levels of fluoride during early childhood can cause fluorosis ww Low socioeconomic status
of the permanent dentition. Fluorosis, when it occurs in the United ww Limited access to professional dental care
States, is usually mild, characterized by white striations on the per- Family Risk Factors
manent teeth. Children with mild fluorosis have teeth that are more ww Family history of caries
resistant to dental decay. Teeth more severely affected by fluorosis ww Recent maternal loss of teeth because of caries
may display hypoplastic enamel that is prone to staining; however, ww Parents brush teeth less than twice daily
moderate and severe dental fluorosis is rare in the United States.
Individual Risk Factors
Inherited enamel or dentin defects may cause abnormal color
ww Currently active decay or personal history of caries
of the teeth. These conditions may be isolated to the teeth, as in the
ww Frequent and/or prolonged intake of foods containing fermentable car-
case of dentinogenesis imperfecta, or as part of a systemic disorder,
bohydrates (or frequent/ongoing use of liquid medications prepared
such as congenital erythropoietic porphyria. A single dark tooth is
with sucrose)
usually nonvital or has bled within the tooth structure after dental
ww Presence of visible plaque on the teeth
trauma. Extrinsic staining is superficial and is usually the result of
ww Exposed root surfaces
poor oral hygiene; smoking; chewing tobacco or betel nuts; certain
ww Having a special health care need that increases the risk for caries (eg,
beverages such as coffee, tea, or wine; or medications, such as liq-
cleft lip), interferes with home oral hygiene (eg, oral aversiveness), or
uid iron supplements.
affects ability to obtain regular professional dental care (eg, behavioral
Malocclusion can be caused by tooth crowding, an underlying
difficulties associated with autism spectrum disorder)
craniofacial condition, abnormal jaw growth relationship, or mal-
ww Inadequate exposure to fluoride
positioning of the teeth. One of the more common causes of mal-
ww Reduced salivary flow or xerostomia
occlusion results from prolonged sucking of a digit (usually the
ww History of radiation therapy to head or neck
thumb) or pacifier. The most common types of malocclusion asso-
ww Wearing of orthodontic appliances or prostheses
ciated with digit sucking are anterior open bite, overjet, and pos-
terior crossbite.
Dental decay results from bacterial action on teeth. Streptococcus
mutans and Lactobacillus species, among other bacteria, produce Differential Diagnosis
acids as end products of carbohydrate metabolism. These acids dis- It can be difficult to differentiate tooth staining from a carious lesion,
solve the calcium-phosphate mineral of a tooth’s enamel during the particularly in the tooth with small areas of discoloration on the pit
process of demineralization. If not reversed through remineraliza- and fissure surfaces. If the lesion disappears with cleaning, staining
tion, the tooth’s structure continues to break down until part of it is the etiology. Staining is not always readily removed, however, and
collapses, resulting in a cavity. A balance of caries-promoting and examination by a dentist and radiography can help evaluate such
caries-inhibiting factors is constantly in play. Intraoral factors that lesions. White spots on the teeth also can have multiple etiologies.
inhibit caries include normal salivary production and regular fluo- When the white spot is chalky in appearance and located along the
ride exposure; those that promote caries are frequent intake of carbo- gingival margin, a diagnosis of demineralization should be consid-
hydrates, particularly simple sugars, and xerostomia (ie, dry mouth). ered. This is the earliest stage of dental decay. Small intrinsic defects
A variety of caries risk factors exist, some of which deserve spe- of the enamel may also appear as white spots; however, these are
cific mention. On a population level, children in families with low usually located throughout the crown and not only along the gin-
income and AN/AI children are at high risk for caries (Box 31.1). gival margin, and they usually reflect light like the rest of the tooth
Children with developmental disabilities are also at higher risk than (ie, appearance is not chalky).
the general population, in part because of limited access to qualified
dentists. On a family level, several genes have recently been discov-
Evaluation
ered that influence caries risk. Parental oral hygiene habits, specifi-
cally less than twice daily toothbrushing by parents as well as recent History
maternal tooth loss from caries, increase caries risk in offspring. Dental pathology is diagnosed mainly through history and physi-
On an individual level, inadequate exposure to fluoride, frequent cal examination. The history is most important when evaluating risk
ingestion of sweetened beverages and food, use of medications that for dental caries, a serious dental infection is suspected, or dental
cause xerostomia, a history of caries in the previous 3 years, and trauma has occurred. Symptoms may differentiate the degree and
visible plaque seen on oral examination are all associated with an depth of dental injury or caries involvement of a tooth; however,
increased risk of caries. young children often have difficulty localizing dental pain. A lesion

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 206 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

involving the dentin may produce intermittent pain, especially on and older children, a slightly convex profile is preferable from func-
exposure to temperature change or pressure on the affected tooth. tional and aesthetic perspectives. A concave profile is never normal
After the pulp is involved, pain in the affected tooth may be severe and may be caused by midface underdevelopment or protrusion of
and persistent, possibly awaking the child from sleep. In cases in the mandible. These children usually have a class III malocclusion.
which the neurovascular bundle supplying a tooth is disrupted, how- An overly convex profile may be caused by an overjet or mandibu-
ever, either as the result of trauma or pulpal necrosis from advanced lar retrusion. These children usually have a class II malocclusion.
dental caries, the pain may disappear; treatment is still necessary When dental trauma occurs, the mouth and adjacent structures
in these cases. should first be gently cleaned. On examination, clinicians should
In the patient with dental injury, the history should address the look for facial swelling or tenderness; loose, missing, or fractured
mechanism of injury, the nature of other injuries, whether teeth are teeth; bleeding from the teeth or surrounding gums; and soft tissue
missing, perceived quality of pain, changes in occlusion, and teta- injuries affecting the tongue, frena, mucosa, or palate. The pres-
nus immunization status. ence of any of these findings suggests the need for further evalua-
tion and treatment.
Physical Examination
Many physicians have not traditionally included the teeth and sup- Laboratory Tests
porting structures as part of their routine physical examination. A complete blood cell count and a blood culture should be obtained
However, pediatricians and other health professionals caring for for an ill-appearing child with a dental abscess or facial cellulitis of
children are generally the first, and often the only, health profession- odontogenic etiology.
als to examine a child during the early years of life and therefore play
Imaging Studies
a key role in identification of dental pathology. This is particularly
true in settings in which access to routine professional dental care Dental radiographs are useful in evaluating dental trauma and for the
is limited. Early diagnosis ideally facilitates timely referral to dental presence and extent of dental decay. They can also aid in determining
specialists who provide definitive management. Additionally, chil- whether an unexpectedly unerupted tooth is congenitally absent or
dren may first present to their primary care physician or an emer- has been prevented from erupting. If pathology is suspected, imaging,
gency department when a dental injury has occurred or an advanced particularly panoramic radiography or computed tomography, can be
odontogenic infection is present. useful in evaluating the facial skeleton. If a tooth or tooth fragment is
During each well-child care visit, the clinician should closely missing following dental trauma, radiographic evaluation is neces-
assess the mouth and adjacent structures for the pattern of erup- sary because the missing tooth or fragment may have been aspirated
tion and dental development; the presence of caries, plaque, gingi- or swallowed; alternatively, it may have lodged in the lip or intruded
vitis, and other oral lesions (see Chapter 86); and malocclusion. In into the alveolar socket, nasopharynx, or sinus cavity.
addition to looking for gingivitis, physicians also should examine the
gingiva of children—particularly those with dental decay or tooth- Management
ache—to assess for evidence of periapical fistula or abscess, which Most dental disorders are definitively managed by dentists.
typically requires antibiotic therapy or incision and drainage, fol- Dental professionals are also experts in prevention of dental car-
lowed by a root canal. ies. The American Dental Association recommends that the first
The oral cavity of a young child can be examined most easily dental visit occur by 12 months of age. However, pediatric pri-
with the caregiver and examiner in a knee-to-knee position, with mary care physicians have an important role to play in identifying
the child’s head in the examiner’s lap and the legs wrapped around early and more advanced signs of dental disease and facilitating
the caregiver’s waist. Older children can be examined on the exam- timely referral for professional dental care. Pediatric physicians
ination table or while seated in a chair. It is useful to have a dispos- may also provide initial management for odontogenic infections
able mouth mirror and a good light source. A toothbrush can be used and dental trauma and, in cases in which access to dental care is
to prop the mouth open to allow for examination of the teeth and limited, can provide treatment to halt caries progression. Some
oral cavity. The toothbrush can later be used to demonstrate good dental conditions commonly fall into the realm of the pediatric
toothbrushing techniques. primary care physician. For example, teething symptoms can be
In a child, occlusion is best examined by looking at the child’s alleviated by giving the child a cold teething toy to suck on or
face from the anterior and lateral perspective and watching the with acetaminophen or ibuprofen given orally. The US Food and
child’s front and back teeth as the child opens and then bites down. Drug Administration has issued warnings against the use of ben-
Having the child bite down on a tongue depressor placed horizontally zocaine, viscous lidocaine, and homeopathic teething tablets for
between the child’s upper and lower teeth demonstrates whether a teething because these have been associated with serious or fatal
cant or asymmetry of the occlusion exists. The lateral profile of a side effects in young children.
child’s face can be particularly revealing. Constructing an imaginary Physicians and dentists can apply fluoride varnish to children
line between the bridge of the nose, the base of the nose, and the tip at high risk for caries. Fluoride varnish is both a preventive modal-
of the chin defines the shape of the lateral profile. In preschool-age ity and an agent used to reverse early decay in the form of white

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 Chapter 31 : Oral Health and Dental Disorders 207

spot lesions. Application of fluoride varnish (22,600 ppm fluoride) The shorter the time between tooth avulsion and replacement into
to the teeth can help remineralize enamel and reverse early caries the socket, the better the chance for survival of the tooth. Holding the
lesions. Another product, silver diamine fluoride, which became tooth by the crown (not the root, to avoid damaging the periodontal
available in the United States in 2015, is a solution of silver, amine, ligament fibers), an avulsed permanent tooth should be quickly rinsed
fluoride, and water that is painted onto a caries lesion to arrest more with cold tap water to remove dirt and debris and then manually reim-
advanced active decay before it can progress to a serious infection or planted in the socket. The tooth can be held in place either by the
abscess. At 38% fluoride (44,800 ppm), it is the most concentrated child’s finger or by having the child bite onto a gauze pad or cloth until
fluoride product currently available for caries management. Silver a dentist is seen. If the avulsed tooth cannot be immediately replaced
diamine fluoride offers a nonsurgical alternative to traditional den- into the socket, it should be stored in saline, cold milk, or a commer-
tal restorative surgery, and, as such, is appropriate for individuals cially available tooth-preserving system (eg, those usually stocked by
with untreated caries who are unable to access professional den- paramedics and emergency departments) until definitive care is ren-
tal care and others who may not be able to tolerate more extensive dered. Water is not a desirable transport medium for an avulsed tooth
professional dental treatment, such as young children and special because its low osmolality can result in cellular damage, decreasing the
needs populations. chance that the tooth will survive. A child with an avulsed permanent
In most cases, advanced untreated caries in a child warrants tooth should be seen urgently by a dentist for additional management,
prompt referral to a dental professional. Pulpal involvement and which typically involves splinting the tooth in place.
abscess formation may result if a carious lesion is ignored. In situ-
ations of serious dental infection in which dental care is not imme- Prevention
diately available, antibiotics (ie, penicillin V, clindamycin) and Primary care physicians play an important role in helping fami-
analgesics (eg, ibuprofen) may alleviate symptoms temporarily; how- lies prevent dental problems (Box 31.2). Depending on the child’s
ever, more definitive treatment (ie, root canal, extraction) is necessary
to remove the source of infection and prevent resurgence of symp-
toms and further complications. Admission to the hospital for intra-
venous antibiotics and surgical drainage is usually indicated in cases
Box 31.2. Caries Prevention
in which an abscess has spread to involve the cheek, face, or neck.
Often, injuries to permanent dentition are treated differently Standard Caries Primary Prevention for All Children
from those to primary teeth. When permanent teeth are injured, ww Encourage consumption of optimally fluoridated water (0.7 ppm of fluoride).
emphasis is placed on maintaining tooth viability and prevention ww Initiate toothbrushing with fluoride toothpaste at first tooth erup-
of complications. Even when optimal dental treatment is provided tion. Use a rice grain–size amount of toothpaste before age 2 years
for an injured permanent tooth, however, the tooth may not sur- and a pea-size amount after 2 years. Brush twice daily and do not rinse
vive. Blows to the teeth can result in damage to the periodontal liga- afterward.
ment, resulting in potential neurovascular disruption, pulp necrosis, ww Primary care physician should examine teeth and oral structures at every
abscess formation, or root resorption. Even with prompt manage- well-child care visit.
ment of a displaced permanent tooth, a root canal may ultimately be ww Regular dental visits. The American Dental Association recommends that
necessary. During a root canal, the pulp is removed and the inside of the first dental visit occur by 12 months of age.
the tooth is cleaned, shaped, filled, and sealed. In contrast, when a ww Anticipatory guidance should include the following information:
primary tooth is damaged, protecting the underlying developing per- —— Frequently consuming sugar-sweetened foods and drinks (including
manent tooth is prioritized. For this reason, in some cases, injured 100% juice) increases caries.
or decayed primary teeth may be extracted rather than restored. —— Taking a bottle/sippy cup with any kind of juice or sugar-sweetened
All dental injuries should be evaluated by a dental professional, beverage to bed increases caries.
but certain dental injuries, including Ellis class III fractures, root —— Regularly drinking optimally fluoridated water reduces caries.
fractures, permanent tooth avulsions, and luxations, require urgent —— Twice daily brushing with fluoride toothpaste of at least 1,000 ppm,
dental evaluation and management. Alveolar fractures and more which is all that is commercially available in the United States,
complex facial fractures also require urgent consultation, usu- reduces caries.
ally with an oral surgeon, otolaryngologist, or plastic surgeon. It is Intensive Caries Primary Prevention for Children With a High Risk
important that pediatricians, coaches, school nurses, and parents of Caries
or guardians know how to manage an avulsed permanent tooth, Includes all the standard recommendations as well as the following:
because survival of the tooth depends on immediate and appro- ww Twice-yearly fluoride varnish application beginning at first tooth
priate care in the field. Primary teeth should not be reimplanted, eruption.
because doing so risks damaging the underlying developing per- ww Initiation of regular professional dental care before 12 months of age.
manent dentition. A permanent tooth, however, should be replaced ww For children 5 years and older, chew polyol-sweetened gum for
into the socket, ideally within 5 minutes of the injury, as long as the 10–20 minutes after meals.
child is alert and cooperative.

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 208 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

age, anticipatory guidance should focus on limiting cariogenic food Children should be seen by a dentist for evaluation for sealant place-
and beverages, toothbrushing twice daily with fluoride toothpaste, ment within 6 to 12 months of eruption of their first and second per-
flossing, avoiding all tobacco products, preventing injury, drink- manent molars, typically occurring at approximately 6 and 12 years
ing optimally fluoridated water (where available), and stressing the of age, respectively. School-based sealant programs offer an impor-
importance of regular professional dental care visits. Toothbrushing tant caries preventive modality; such programs are usually provided
with fluoride toothpaste has largely supplanted fluoride drops, in areas with high caries prevalence and limited availability of pro-
because fluoride toothpaste is less expensive, more readily avail- fessional dental care. Regular use of chewing gum sweetened with
able, and most importantly, more effective throughout the entire life polyols, also known as sugar alcohols (eg, xylitol, sorbitol, erythri-
span. Many aspects of oral health anticipatory guidance are also rel- tol), decreases caries incidence, particularly in populations with high
evant to obesity prevention and promotion of overall health. levels of caries. Polyols reduce caries risk through a variety of mech-
Fluoride is the single most important dental decay preven- anisms, including stimulating saliva when chewing gum or sucking
tive modality. Fluoride strengthens teeth and reverses early car- on a lozenge, substituting for sugar, and disrupting bacterial metab-
ious lesions through enhancement of tooth mineralization. The olism as well as biofilm organization and adherence to the teeth. The
composition of the fluoride-containing enamel, fluoride apatite, is American Dental Association recommends that healthy children 5
harder and less acid soluble than the original enamel that it replaces. years of age and older who are at high risk for caries chew polyol-
Increased availability of fluoridated water and fluoride-containing sweetened chewing gum for 10 to 20 minutes after meals.
toothpaste has dramatically decreased the prevalence of dental car- Certain types of malocclusion are preventable. For example, car-
ies in the United States and other countries over the past 50 years. ies and dental trauma can result in premature tooth loss and sub-
Nevertheless, for some in the lay public, fluoride evokes contro- sequent loss of spacing and overcrowding, which then increase the
versy and concerns about adverse health effects, even though a large need for orthodontic treatment. During the first year after birth,
amount of robust research evidence attests to the safety and efficacy nonnutritive sucking on a digit or pacifier helps promote oral-motor
of fluoride for caries prevention. Pediatricians and other health pro- development and self-soothing. Nonnutritive sucking behavior need
fessionals have an important role to play in countering misinforma- not be discouraged up to age 1 year. However, prolonged digit or
tion and educating families about the benefits of community water pacifier sucking contributes to malocclusion. By age 1 year, the bot-
fluoridation and the use of fluoride toothpaste and professionally tle and pacifier should be discontinued, and by age 2 years, efforts
applied fluoride products. should have begun to discourage digit sucking. If children can dis-
Caries preventive practices should begin in infancy and continue continue these practices before age 4 to 6 years, the malocclusion
as lifelong habits; these include twice daily fluoride toothpaste for usually spontaneously reverses. Dentists can use specific devices
toothbrushing beginning at first tooth eruption (using a rice grain– and treatment to help stop digit sucking if other methods, including
size amount of toothpaste until age 2 years and a pea-size amount behavior modification, are unsuccessful (see Chapter 54).
after age 2 years); drinking optimally fluoridated water where avail- Dental injuries can be prevented with appropriate vehicle restraint
able (0.7 ppm is the recommended level of fluoride in drinking water systems, environmental precautions when children are learning to
in the United States); sound dietary practices, including avoiding fre- walk and run, and use of mouthguards during certain sports. The
quent or prolonged exposure to fermentable carbohydrates, espe- National Federation of State High School Associations recommends
cially sucrose; and routine professional dental care. By the age of mandatory mouth guards in high school football, lacrosse, ice hockey,
approximately 7 years, most children have developed sufficient fine field hockey, and wrestling. In the case of wrestling, a mouth guard
motor skills to begin brushing their teeth independently. Until then, is required only if the wrestler has braces. The American Dental
parents and guardians should help their children. It is also at approx- Association recommends mouth guard use for several other sports.
imately 7 years of age that children learn to spit out after toothbrush-
ing. Some parents and guardians worry about the risks of their child
swallowing fluoride toothpaste before they learn to spit. However, by Prognosis
applying the toothpaste to a dry toothbrush, using the appropriate Many dental disorders are preventable either on a primary or sec-
amount of toothpaste, and not rinsing the mouth with water after ondary level. Dentists can restore and repair more advanced dental
brushing, only a small amount of fluoride is swallowed–an amount disease and significant dental trauma, assuming a child has access to
smaller than of prescription fluoride drops. There is no benefit to dental care, and ideally, has a dental home, in which an ongoing rela-
using a “training toothpaste” that does not contain fluoride. tionship exists involving the dentist, child, and parents or guardians.
It is not necessary to floss while spaces still exist between a child’s Pediatric primary care physicians play an essential role in opti-
teeth, as is the usual case in young children who have only primary mizing their patients’ lifelong oral health by incorporating an
teeth. As additional teeth erupt and teeth become closely approxi- examination of the teeth and oral structures into every physical
mated, flossing is important to remove plaque between the teeth that examination, making timely referrals for prevention and in cases
can contribute to caries, gingivitis, and later to periodontal disease. in which pathology is identified, including oral health prevention
Sealants placed on the pit and fissure surfaces of permanent molars as part of each well-child care visit, and advocating for quality pro-
can also be an important defense against caries in these surfaces. fessional dental care for all patients.

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 Chapter 31 : Oral Health and Dental Disorders 209

Dye BA, Mitnik GL, Iafolla TJ, Vargas CM. Trends in dental caries in children and
CASE RESOLUTION adolescents according to poverty status in the United States from 1999 through
2004 and from 2011 through 2014. J Am Dent Assoc. 2017;148(8):550–565.e7
The parents of the infant should be reassured that the absence of teeth in their
PMID: 28619207 https://doi.org/10.1016/j.adaj.2017.04.013
9-month-old daughter is normal. Her first tooth may not appear for a few
months. Provided she is growing and developing normally, there is no cause for Horst JA, Ellenikiotis H, Milgrom PL. UCSF protocol for caries arrest using
concern. silver diamine fluoride: rationale, indications and consent. J Calif Dent Assoc.
2016;44(1):16–28 PMID: 26897901
Keels MA; American Academy of Pediatrics Section on Oral Health. Management
of dental trauma in a primary care setting. Pediatrics. 2014;133(2):e466–e476
Selected References PMID: 24470646 https://doi.org/10.1542/peds.2013-3792

American Dental Association Center for Evidence-Based Dentistry. Fluoride Lewis C, Stout J. Toothache in US children. Arch Pediatr Adolesc Med.
Toothpaste in Young Children for Caries Prevention Clinical Practice Guideline 2010;164(11):1059–1063 https://doi.org/10.1001/archpediatrics.2010.206 PMID:
(2014). https://ebd.ada.org/en/evidence/guidelines/fluoride-toothpaste-for-young- 21041599
children. Accessed September 23, 2019 Lewis CW. Fluoride and dental caries prevention in children. Pediatr Rev.
American Dental Association Council on Access, Prevention and Interprofessional 2014;35(1):3–15 PMID: 24385561 https://doi.org/10.1542/pir.35-1-3
Relations; American Dental Association Council on Scientific Affairs. Using
mouthguards to reduce the incidence and severity of sports-related oral inju-
ries. J Am Dent Assoc. 2006;137(12):1712–1720 PMID: 17138717 https://doi.org/
10.14219/jada.archive.2006.0118

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 CHAPTER 32

Normal Development and

Developmental Surveillance,
Screening, and Evaluation
Geeta Grover, MD, FAAP, and Jeanne Anne Carriere, PhD

CASE STUDY
The parents of a 12-month-old girl are concerned that Questions
she is not yet walking. They report that she sat indepen- 1. How is developmental delay in children defined?
dently at 7 months and began crawling at 8 months. She 2. What are the 5 major domains in which develop-
can pull herself up to stand while holding on to furni- ment is assessed?
ture but is not cruising. Her birth and medical history are 3. How should you advise the parents in the case study
unremarkable. The physical examination is within nor- about the acquisition of gross motor skills, such as
mal limits, and review of your records reveals no con- walking?
cerns on a developmental screening test administered 4. What developmental screening tests could you
at 9 months of age. administer to further assess her development?
5. What is the appropriate next step for the child with
suspected developmental delay?

Development refers to the acquisition of functional skills during Pathophysiology

childhood. Monitoring the growth and development of children is
Development is influenced by biologic and environmental fac-
an integral part of the assessment of pediatric patients. Recording
tors. Biologic factors, such as genetics, preterm birth, exposure
the acquisition of developmental milestones provides a systematic
to drugs in utero, or the presence of chronic disease, may place
approach by which to observe the progress of children over time. For
a child at increased risk for developmental problems and delays.
ease of monitoring, these developmental milestones may be divided
Environmental factors that influence development include parental
into 5 major domains or areas: gross motor, fine motor, language,
attitudes and actions, sociodemographic factors, and cultural and
social-emotional, and cognitive.
societal influences. The quality of parental stimulation may influence
Four principles apply to all aspects of development. First, motor
the rate of acquisition of certain skills, especially cognitive and lan-
development is a continuous process that proceeds in the cepha-
guage abilities in preschool-age children. Poverty and other socio-
locaudal direction and parallels neuronal myelination; therefore,
economic factors may make it difficult for parents to provide their
developmental milestones reflect the maturation of the nervous
children with an optimal environment for growth and development.
system. Second, the sequence of development is the same in all
For example, research has found significant disparities in vocabulary
children, but the rate of development may vary from child to child;
and language processing between infants from families of higher– and
for example, all children must walk before they run, but the age
lower–socioeconomic status (SES) as early as 18 months. Children
at which a child walks or runs varies from child to child. Third,
from families of higher SES tend to have larger vocabularies at 2 years
the rate of attainment of milestones in 1 area may not parallel
of age and score higher on language and cognition testing in elemen-
that in another. Fourth, certain primitive reflexes must be lost
tary school. Processing speed is critical to language and cognitive
before corresponding voluntary movements can be attained (eg,
development, because the faster children process (ie, understand)
the asymmetric tonic neck reflex must disappear before a child
a word they have heard, the more cognitive energy they can put
can roll over).

211

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 212 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

into other parts of communication. Fortunately, regardless of of primitive reflexes or failure of development of postural reactions
SES, parents who consistently engage in language-rich activities can signal developmental problems. Authorities estimate that more
with their infants and young children can help their children learn than 70 primitive reflexes and postural reactions exist. Researchers
more quickly. do not agree on which of these reflexes or reactions are the most
useful in the monitoring of development. The 7 most commonly
Development in Newborns and Infants used primitive reflexes are described in Box 32.1, and select pos-
Normal, full-term newborns enter the world capable of respond- tural reactions are presented in Box 32.2.
ing to visual, auditory, olfactory, oral, and tactile stimuli. They can
be quieted and can even soothe themselves. Newborns can signal Normal Development
needs (eg, crying when hungry or wet), but they have a limited A developmental assessment should include an evaluation of mile-
ability to respond to caregivers, primarily exhibiting disorganized stones in each of the 5 major domains. Gross motor skills are over-
and seemingly purposeless movements when stimulated. The new- all movements of large muscles (eg, sitting, walking, running). Fine
born’s reflexive generalized symmetric movements (eg, arm waving motor skills involve use of the small muscles of the hands, the ability
and kicking) in response to environmental stimuli are eventually to manipulate small objects, and eye-hand coordination. Language
replaced by cortically mediated voluntary actions in older infants skills involve hearing and include understanding and use of language
and children. Additionally, in newborns, certain primitive reflexes as well as nonverbal communication skills. Social-emotional skills
can be elicited by appropriate peripheral stimuli. Eventually, prim-
itive reflexes are replaced by reactions that allow children to main-
tain postural stability in response to a variety of sensory inputs (ie,
proprioceptive, visual, vestibular). Box 32.1. The 7 Most Commonly
Primitive reflexes are mediated by the brain stem; they are invol- Used Primitive Reflexes
untary motor responses that are elicited by appropriate peripheral
stimuli and are present at birth but disappear during the first Moro Reflex
6 months after birth. Normal motor development seems to be related ww Allowing the baby’s head to drop back suddenly results in abduction and
to the suppression of these reflexes (Figure 32.1). Persistence or reap- upward movement of the arms, followed by adduction and flexion. This
pearance of these reflexes may indicate the presence of brain dam- reflex disappears by 3–6 months of age.
age. Postural reactions, which are ultimately smoothly integrated Rooting Reflex
into adult motor function (Figure 32.1), appear between 2 and ww Touching the corner of the baby’s mouth results in lowering of the lower
9 months of age. Postural reactions help maintain the orientation of lip on the same side and movement of the tongue toward the stimulus.
the body in space and the interrelationship of 1 body part to another. This reflex disappears by 3–4 months of age.
The 3 major categories of postural reactions are righting, protec- Sucking Reflex
tion, and equilibrium. ww Placing an object in a baby’s mouth causes vigorous sucking. This reflex
The profile generated by combining primitive reflexes and pos- disappears at approximately 3 months of age.
tural reactions can be used to monitor the course of normal devel- Grasp Reflex
opment and identify cases of problematic development. Persistence ww Placing a finger in a baby’s palm causes the baby to grasp it; the baby
reinforces the grip as the finger is drawn upward. A similar response
is seen in the foot grasp. The palmar grasp reflex disappears by
age 3–4 months and is replaced by intentional grasping by age
Postural reflexes 4–6 months; plantar grasp may be present up to 9–12 months of age.
Placing Reflex
ww Stroking the anterior aspect of the tibia against the edge of a table
Performance

Primitive
reflexes results in the lifting of the baby’s leg to step onto the table. This reflex
Definitive disappears by 2 months of age.
motor actions
Stepping Reflex
ww Holding the baby upright and slightly leaning forward produces
alternating flexion and extension movements of the legs that simulate
walking. This reflex disappears by 2 months of age.
3 6 9 12
Asymmetric Tonic Neck Reflex
Months
ww With the baby lying supine, turning the head to 1 side results in
extension of the extremities on that side and flexion of the opposite
Figure 32.1. Primitive reflex profile. extremities (ie, fencing position). This reflex disappears by 3–4 months
Reprinted with permission from Capute AJ, Accardo PJ, Vining EP, Rubenstein JE, of age and allows for rolling.
Harryman S. Primitive Reflex Profile. Baltimore, MD: University Park Press; 1978:10.

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 Chapter 32: Normal Development and Developmental Surveillance, Screening, and Evaluation 213

reaching for desired objects; by 6 months of age, they can transfer

Box 32.2. Select Postural Reactions
an object from 1 hand to the other. By 7 months, they have a 3-finger
Righting Reactions pincer grasp, and by 9 to 10 months, they have developed the 2-finger
ww These allow the body to maintain normal postural relationships of the grasp, which allows them to manipulate small objects, such as
head, trunk, and extremities during all activities. The different reactions raisins. By 14 months, they begin to scribble, and by 3 to 5 years,
appear at different ages, beginning shortly after birth and occurring up they can copy geometric shapes. Children with early preference
to 12 months of age. for the use of 1 hand over another, especially before approximately
Protection and Equilibrium Reactions 18 months of age, should be assessed for the presence of paresis
Protective Equilibrium Response or other neuromuscular problems. Handedness may manifest by
ww When gently pushed toward one side while in a sitting position, infants 3 years but often is not firmly established until 4 to 5 years of age.
increase trunk flexor tone toward that side to regain their center of
Language Skills
gravity and extend the arm on the same side to protect against falling.
This response usually emerges at about 4–6 months of age. The development of normal speech and language skills is discussed
in Chapter 33.
Parachute Reactions
ww When held in ventral suspension and suddenly lowered (downward Social-Emotional Skills
parachute), infants extend their arms as if to protect themselves from a
These skills enable children to interact and respond to the surround-
fall; similar reactions are seen with forward and backward stimulation.
ing world. Deficits in the development of age-appropriate social
These reactions appear at 8–9 months of age.
skills/social relatedness (eg, social orienting, social referencing,
joint attention, pretend play) are a defining feature of autism spec-
trum disorder (ASD; see Chapter 132). For children on this spec-
involve attachment, socialization, and the ability to regulate emo- trum, the development of social skills is characteristically “out of
tions. Cognitive skills involve the ability to use higher mental pro- sync” with their overall level of functioning. Joint attention is the
cesses, including comprehension, memory, problem-solving skills, inclination to share enjoyment, interests, or achievement with other
critical thinking, and logical reasoning. people, and like other developmental skills, it seems to manifest
Table 32.1 outlines the normal pattern of development for each in graduated stages. Early skills include reciprocal smiling at the
of these domains. The table lists the average age of attainment of sight of a familiar person, followed by later emerging skills, such
these skills as well as the normal ranges as available. Development as the ability to isolate one’s index finger and point with a coordi-
is an orderly and sequential process, and children must proceed nated gaze by 12 to 15 months of age. Lack of joint attention is a
through several stages before any given milestone is attained. core deficit of ASD.
Therefore, the physician should document not only what a child
can do but how the child does it. For example, to sit without sup- Cognitive Skills
port, children first achieve head control. Several stages later they These abilities allow children to think, reason, problem-solve, and
can sit in a “tripod” position with arms extended in front for sup- understand the surrounding environment. Information processing
port, and finally, they sit with the head steady and back straight theories address how individuals acquire, interpret, and remember
without support (Figure 32.2). information and how these abilities develop. Cognitive development
involves gradual changes in these processes. Infants are born with
Gross Motor Skills some sensory and perceptual capabilities. Newborns are drawn to
During the first year after birth, the ultimate goal of gross motor both novel and social stimuli, especially from their caregiver; these
development is walking. The first developmental skill toward this stimuli support further sensory and perception development as well
goal is head control; by 4 months of age there should be no head as social and language development. As children develop, their atten-
lag when a child is pulled to sitting from a supine position. By tion span increases, they more efficiently process information, and
6 months of age, children can sit without support for a few sec- they are better able to plan and direct their actions toward goals.
onds. At 9 to 10 months of age, children can pull themselves to a Additionally, their knowledge base grows and becomes more inte-
standing position, and by 12 to 18 months of age, they can walk. grated. These developments allow them to be more efficient learn-
Children then learn to run, use stairs, hop on 1 foot, and skip— ers as they age.
in that order. A child’s perception and understanding of the world, including
the understanding of pain, disease, and illness, are guided by the
Fine Motor child’s stage of cognitive development. The concept of object perma-
Development of the 2-finger pincer grasp is the major goal of fine nence or object constancy, the realization that objects may exist even
motor development during the first year (Figure 32.3). The hands if they cannot be seen, develops at approximately 7 to 9 months of
primarily remain in a fisted position until 3 months of age. Infants age. The understanding of time comes much later. Children develop
also discover the midline at this age, and shortly thereafter they may the concept of “today” at 24 months of age, “tomorrow” at 30 months,
play with their hands in the midline. At age 4 months, children begin and “yesterday” at 36 months.

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 214 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

A B C

Figure 32.2. Stages in the development of sitting. A, Head control. B, “Tripod sitting.” C, Head steady and back
straight without support.

A
Rake (4 months)

B
Inferior pincer grasp
(7 months)

C
Fine pincer grasp
(9–12 months)

Figure 32.3. Development of the pincer grasp. A, Rake (4 months). B, Inferior pincer grasp (7 months). C, Fine pincer
grasp (9–12 months).

Table 32.1. Normal Pattern of Development

Domain Description Mean Age Normal Range
Gross motor skills Reflex head turn; moves head side to side Newborn 0–3 months
Lifts head when prone 1 month 1–4 months
Lifts shoulders up when prone 2 months 1–4 months
Lifts up on elbows; head steady when upright 3 months 2–5 months
Lifts up on extended hands; rolls front to back; no head lag when pulled to sitting from supine 4 months 3–6 months
position
Rolls back to front 5 months 4–7 months
Sits independently 6 months 5–9 months
Crawls on hands and knees 8 months 6–11 months
Pulls to stand 9 months 6–12 months
Cruises 11 months 9–14 months

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 Chapter 32: Normal Development and Developmental Surveillance, Screening, and Evaluation 215

Table 32.1. Normal Pattern of Development (continued )

Domain Description Mean Age Normal Range
Gross motor skills Walks 12 months 9–17 months
(continued) Walks backward 15 months 13–17 months
Runs 15 months 13–20 months
Kicks a ball 24 months 18–30 months
Walks up and down stairs using railing and putting both feet on each step; jumps with both feet off 24 months —
the ground; throws a ball overhand
Pedals a tricycle; goes up stairs alternating feet without using the railing; balances on 1 foot for 3 years 30–48 months
3 seconds
Hops on 1 foot 2–3 times; gallops 4 years —
Skips; walks down stairs using the railing and alternating feet 5 years —
Tandem walks 6 years —
Fine motor Tracks horizontally to midline 1 month —
Tracks past midline; tracks vertically 2 months 1–3 months
Not fisted for >50% of the time; tracks 180°; visual threat; discovers midline 3 months 3–4 months
Reaches for bright object; brings object to mouth 4 months —
Transfers object from 1 hand to the other 6 months 4–7 months
3-finger pincer grasp 7 months 6–10 months
Neat pincer grasp 9 months 7–12 months
Bangs cubes in midline 9 months 7–11 months
Tower of 2 cubes; scribbles spontaneously 14 months 14–20 months
Drinks from a cup 15 months 10–18 months
Uses a spoon, spilling a little 15 months 12–18 months
Tower of 4 cubes 18 months 17–24 months
Copies vertical and horizontal line; tower of 6 cubes 2 years —
Copies circle; strings beads; imitates a bridge of cubes 3 years —
Copies square; ties single knot; cuts basic shapes with scissors 4 years —
Copies triangle; draws person with 3 parts; writes first name 5 years —
Draws diamonds; draws person with 6 parts; forms letters correctly; creates and writes short sentences 6 years —
Language skills a
Alerts to sound Newborn —
Searches with eyes for sound 2 months —
Cooing (vowel sounds) 3 months 1–4 months
Orients to sound/turns to voice; laughs 4 months 3–6 months
Responds to name 4 months 4–9 months
Babbles (consonants added to vowel sounds) 6 months 5–9 months
Dada/Mama nonspecific 8 months 6–10 months
Understands the word “no” 10 months 9–18 months
Dada/Mama specific 10 months 9–14 months
Follows 1-step command with gesture 12 months 10–16 months
3–5–word vocabulary 12 months —
Follows 1-step command without gesture 15 months 12–20 months
Points to several body parts; points to self 18 months 12–24 months
≥50-word vocabulary (50–300 words); refers to self by name 2 years —
(continued)

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 216 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 32.1. Normal Pattern of Development (continued )

Domain Description Mean Age Normal Range
Language skillsa 2-word phrases; uses pronouns indiscriminately; follows 2-step command with gesture 2 years 20–30 months
(continued) ≥200-word vocabulary (200–1,000 words); uses 3-word sentences; speech intelligible to strangers 3 years —
75% of time; uses pronouns appropriately
Complex sentences using more than 1 action word (eg, “I lost my balloon because I let go”); 100% 4 years —
intelligible speech to strangers although may have articulation errors; points to several colors,
letters and numbers when named; rote counts to 4
Retells short stories with clear beginning, middle, and end; uses ≥2,000 words; produces rhyming 5 years —
words; rote counts to 10
Knows days of the week; describes events in order 6 years —
Social-emotional Regards face Newborn —
skillsb Spontaneous social smile 6 weeks 1–3 months
Discriminates social smile; relates to parent with real joy 6 months —
Displays stranger anxiety; plays peek-a-boo 7 months 6–9 months
Gaze monitoring (follows adult gaze shift when adult looks away) 8 months —
Uses pointing to draw attention to (ie, protodeclarative) or request (ie, protoimperative) an object — 9-12 months
of interest
Joint attention (uses 3-point gaze shifts and follows the gaze of another) — 12–15 months
Shows empathy (eg, looks sad when someone else cries); seeks help from adults 15 months —
Simple pretend play (eg, feeding doll) 18 months 17–22 months
Helps with undressing; washes and dries hands; parallel play 2 years 22–30 months
Undresses self; beginning to take turns 3 years 30–40 months
Toilet training — 24–36 months
Imagines self as different characters; cooperative play — 3–4 years
Dresses without assistance; has a group of friends 5 years —
Cognitive skills: Approaches world through sensations and motor actions; attention drawn to complex or novel stim- — Newborn–2 years
infancyc uli; growing awareness of cause and effect; development of object permanence (ie, awareness that
object exists when removed from view); emergence of symbolic thought
Cognitive Skills: Rapidly developing language skills; limited perspective taking ability; increases in social play; ability — 2–6 years
early childhood to recall past events; growing ability to sustain attention to preferred activities
Cognitive skills: Able to understand that personal thoughts and feeling may differ from those of others; conservation — 6–10 years
middle childhood of mass, volume, and number (eg, realization that amount stays the same if nothing is added or taken
away); ability to perform mental operations if they relate to real objects; begins formal automatization
of basic academic skills (eg, math facts, sight-word recognition); increased ability to focus on important
information and ignore irrelevant information; use of rehearsal as the primary learning strategy
Cognitive skills: Able to attend to a task for an hour or more; beginning ability to engage in abstract thought and — 10–14 years
early adolescence symbolic reasoning; emerging ability to use intentional learning strategies
Cognitive skills: Able to attend to a single task of interest for long periods; extensive and moderately integrated — 14–18 years
late adolescence knowledge; more sophisticated ability to engage in abstract thought and symbolic reasoning;
increasing use of self-regulatory learning strategies (eg, goal setting)
Miscellaneous Concept of today 24 months —
cognitive Concept of tomorrow 30 months —
milestones Concept of yesterday 36 months —
Concept of right and left 7 years —
a
Detailed language milestones are presented in Chapter 33, Table 33.1.
b
For play and social pragmatic milestones, refer to Chapter 33.
c
Cognitive milestones adapted from constructivist theories of Jean Piaget, Lev Vygotsky, and information processing theorists.

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 Chapter 32: Normal Development and Developmental Surveillance, Screening, and Evaluation 217

Developmental Delay thus able to identify and differentiate true deficits and delays from
temporary setbacks.
Children are said to be developmentally delayed if they do not reach
developmental milestones within the expected age range. The age History
ranges for these milestones are broad because of the wide variation
Evaluation of children for suspected delays in development includes
among typically developing children. Children with global develop-
a complete history (Box 32.3). Family history of birth defects, child-
mental delays have delays in multiple domains. Children can also
hood deaths, intellectual disability, speech delay, learning disability,
have a specific delay in 1 area, such as expressive language or gross
and known genetic conditions (eg, fragile X syndrome) should be
motor. Development across domains is often intertwined. Delays
obtained. Perinatal factors that place children at high risk for devel-
in 1 area can affect development in other domains. For example, a
opmental difficulties include a history of maternal drug or alcohol
child with an expressive language delay may also demonstrate delays
use during pregnancy, preterm birth of the child, and congenital
in social development because of limited communicative interac-
infections. Preterm infants are at increased risk for developmental,
tion with peers.
behavioral, and learning disorders compared with children born at
term. Although no formal guideline exists about the specific dura-
Differential Diagnosis tion of time that gestational age correction should be performed for
Three factors are involved in the differential diagnosis of children preterm infants for attainment of developmental milestone relative
with developmental delays: determination of the area or areas of to term infants, most experts recommend correcting for preterm
development in which delay is apparent; if motor delay is evident, status for the first 24 months after birth. Other historical risk factors
determination of whether the condition is progressive or nonpro- for developmental delay include history of seizures, sepsis, or men-
gressive; and assessment for whether developmental milestones ingitis; exposure to lead or other toxins; and poor feeding or growth.
previously achieved are lost or if age-appropriate milestones were Environmental factors, such as stressful home conditions, history
achieved at all. of abuse or neglect, and lack of stimulation, may also contribute to
The child with an early history of normal development who sub- delayed development.
sequently experiences a slowing of developmental progression, often
associated with cognitive delays or seizures, may have a metabolic Physical Examination
defect. The child who attains developmental milestones and subse- Height and weight should be checked. Abnormal growth (ie, height
quently loses them may have a neurodegenerative disease (eg, mul- or weight <5th percentile or head circumference <5th percentile
tiple sclerosis, adrenoleukodystrophy) or a lesion of the spinal cord or >90th percentile) may be a marker for developmental delay. The
or brain. The presence of habitual rhythmic body movements (eg, presence of congenital anomalies (eg, cataracts, hypertelorism, spina
body rocking, head banging) may be a sign of a pervasive develop- bifida) or neurocutaneous lesions (eg, café au lait spots) may be
mental disorder, such as ASD. suggestive of chromosomal anomalies or other genetic diseases.
Cerebral palsy, the classic example of nonprogressive motor Neuromuscular examination should emphasize age-appropriate
abnormality, is a form of static encephalopathy that is character- milestones. Abnormalities in muscle tone (eg, hypotonia, hyperto-
ized by abnormal movement and posture. The type of cerebral palsy nia), bulk, or strength may be clues to the presence of neuromuscular
depends on which area of the brain is injured. Spastic cerebral palsy, disease (eg, muscular dystrophy), cerebral palsy, or Down syndrome.
which is the most common type, is secondary to upper motor neu-
ron injury. The ataxic form of the disease is related to lesions of the
cerebellum or its pathways. Dyskinetic cerebral palsy manifests as Box 32.3. What to Ask
uncontrolled and purposeless movements that often result from a
basal ganglia lesion (eg, athetosis after bilirubin deposition in the Normal Development in the Pediatric Patient
basal ganglia). Onset of symptoms is in infancy or early childhood. ww Has anyone in the child’s family had developmental problems or delays,
The key factor in making the diagnosis is establishing that the motor or been diagnosed with learning disability, intellectual disability, or a
deficits are static and not progressing. known genetic condition?
ww Did the mother use any drugs (illicit or prescription) or alcohol during
pregnancy?
Evaluation ww Did the mother or father use illicit drugs or prescription medications
When evaluating children for possible delays in development, it is prior to conception?
important to remember that a great deal of variation exists in the ww Was the child born preterm?
age of attainment of milestones. Additionally, the rate of acquisi- ww Does the child have a history of seizures?
tion of milestones in 1 area of development may not parallel that in ww Has the child had meningitis or sepsis?
another. Routine and ongoing assessment of a child’s level of devel- ww Does the child have any history of not feeding well or of poor growth?
opment at all periodic health maintenance visits through observa- ww Is the child’s home environment characterized by any stressors (eg, new
tion, history, physical examination, and screening tests allows the sibling, divorce, limited financial resources, homelessness)?
physician to form a longitudinal view of the child. The physician is

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 218 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Laboratory Tests Developmental evaluation (ie, developmental assessment) is per-

formed when surveillance or screening identifies a child as being at
Age-appropriate assessment of the child’s vision and hearing should
high risk for a developmental disorder. The aim is to identify the
be performed if signs of motor, cognitive, or language delays are
specific developmental disorder or disorders affecting the child.
apparent. Chromosomal studies may be conducted if dysmorphic
Developmental evaluation is performed by trained examiners. Unlike
features are noted on the physical examination. Evidence of cog-
developmental screening, developmental evaluation is diagnostic.
nitive or motor delays may warrant metabolic studies (eg, organic
The Bayley Scales of Infant and Toddler Development®, 3rd
and amino acids). Imaging studies of the head, such as magnetic
Edition (Bayley-III®), first published in 1933 by Nancy Bayley, is
resonance imaging or electroencephalography, may be necessary
among the most commonly used developmental evaluation tools. It
if the child has a history of seizures or an abnormal neurologic
has 5 distinct scales yielding scores for 5 developmental domains:
examination.
cognitive, language, motor, social-emotional, and adaptive behav-
Developmental Surveillance, Screening, ior. The Bayley-III may be used for children 1 to 42 months of age.
and Evaluation Especially before age 24 to 30 months, developmental tests such as
Early detection of developmental delays is a responsibility of all pedi- the Bayley-III, which emphasize sensorimotor-based skills, have a
atric health care professionals. Unfortunately, current detection rates poor correlation with later measures of intelligence, which empha-
of developmental disorders are lower than their actual prevalence. size language and abstract reasoning. Thus, developmental tests are
Approximately 20% of children between the ages of 3 and 17 years best used as measures of current developmental functioning rather
will have 1 or more developmental, learning, or emotional disor- than as predictors of future functioning.
der, with 4% to 5% having developmental delays. Without the use Intelligence tests, achievement tests, adaptive skills assessment (ie,
of specific developmental screening tools, only approximately 30% age-appropriate skills necessary to take care of oneself and to inter-
of developmental disabilities are identified; however, with the use of act with others), and behavior rating scales exist that may be used
screening tools this identification rate increases to 70% to 80%. for preschool- and school-age children with suspected behavioral
Developmental surveillance, the ongoing process of monitoring concerns, social-emotional concerns, learning disorders, or devel-
an individual child’s developmental status, should be incorporated opmental disabilities, including intellectual disability. Delays in both
at each health maintenance visit. Surveillance involves eliciting and cognitive skills and adaptive functioning must be present during the
attending to parental concerns about the child’s development and usu- developmental period to make a diagnosis of intellectual disability.
ally involves age-specific queries, such as whether the child is walk- Typical, or normal, development varies significantly; however,
ing, talking, or pointing. Surveillance has been referred to as a sort of certain red flags, or warning signs, of developmental delay exist that
developmental growth chart. Recognition of the child who may be at warrant further monitoring or evaluation. Early detection can result
risk of developmental delays is the goal of developmental surveillance. in early intervention, which improves a child’s chances of healthy
Developmental screening is the administration of a brief stan- or positive outcomes. Language development is discussed in more
dardized tool to identify the child at risk of a developmental dis- detail in Chapter 33, but it is important to note some red flags of lan-
order. Table 32.2 lists frequently used developmental screening guage development that necessitate referral for a full evaluation, which
tools. Screening is based on either parental report or direct obser- include no babbling, no response to name, or no reciprocal gestures
vation and interaction with the child, and use of screening tools can (eg, showing, reaching, waving) by 12 months of age; no single words
increase the accuracy of clinical judgement. Developmental screen- by 16 months of age; and vocabulary of 50 words or less or no non-
ing tools are not diagnostic; rather, they serve as a quick and effec- echolalic 2-word phrases by 24 months. Social-emotional develop-
tive means of identifying children who need further evaluation. A ment typically correlates with language development. A lack of sharing
formal developmental screening should be performed when devel- of vocalizations, back-and-forth smiles, or other facial expressions at
opmental surveillance elicits a risk factor for developmental delay; 9 months of age; a lack of pointing to desired objects by 15 months of
in the absence of established risk factors or parental concerns, the age; a lack of imitating actions by 2 years; or a lack of engagement in
American Academy of Pediatrics (AAP) recommends that health pretend play by 3 years warrants further evaluation. Motor develop-
professionals screen children for general development using stan- ment red flags include an inability to sit by 9 months of age, an inabil-
dardized, validated tools at the 9-, 18-, and 24- or 30-month health ity to stand or bear weight at 12 months, an inability to independently
maintenance visits. These instruments can be administered at other walk by 18 months, frequent falling at 3 years, or poor balance at
times if there are clinical concerns. The AAP further recommends 5 years. Developmental skill regression or marked stagnation in any
that in addition to a general developmental screening tool, an ASD- of the domains is a red flag and warrants further evaluation.
specific screening tool should also be administered at the 18- and
24-month visits. Periodic screening is necessary to detect emerg- Management
ing disabilities as children grow. Screening instruments should have The child with identified developmental delay in 1 or more areas
a broad developmental focus and be brief, inexpensive, valid, and should be referred to the appropriate specialist, agency, state pro-
reliable. A failed screening test should result in a referral for further gram, or public school district (≥3 years of age) for further testing
developmental evaluation. and assessment. Detailed neurologic examination may be necessary

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 Chapter 32: Normal Development and Developmental Surveillance, Screening, and Evaluation 219

Table 32.2. Frequently Used Developmental Screening Instruments

Assessment Type Instrument Patient Age on Administration Languages
Parent report Ages & Stages Questionnaire, Third Edition (ASQ-3) 1 month–5.5 years English
Spanish
French
Arabic
Korean
Vietnamese
Child Development Inventory (CDI) 15–72 months English
Spanish
Infant Development Inventory Birth–18 months English
Spanish
Parents’ Evaluation of Developmental Status (PEDS) Birth–96 months English
Spanish
Vietnamese
Parents’ Evaluation of Developmental Status, Developmental Birth–84-132 months English
Milestones (PEDS:DM) Spanish
Vietnamese
Modified Checklist for Autism in Toddlers (M-CHAT) 16–48 months Translations in >50 languages
Direct observation Battelle Developmental Inventory, 2nd Edition (BDI-2) Birth–95 months English
and interaction with Spanish
the child Bayley Infant Neurodevelopmental Screener (BINS) 3–24 months English
Spanish
Portuguese
Bayley Scales of Infant and Toddler Development®, 1–42 months English
Third Edition (Bayley-III®)
Brigance® Infant & Toddler Screen Birth–23 months English
Spanish

if gross motor delays are identified. Language delays may warrant for- intervention programs can greatly stimulate the developmental
mal hearing and speech-language assessment by an audiologist or a potential of the child. The pediatrician can ensure that children
speech language pathologist. Cognitive impairment requires formal with specific or global developmental delays receive timely sup-
psychological assessment. The child aged 3 years or older and with port and intervention by performing developmental surveillance
developmental delay may qualify for special education support and at each health visit and encouraging parents or caregivers to mon-
services through the child’s public school (see Chapter 36). itor milestones between visits.

Prognosis
CASE RESOLUTION
The pediatrician plays a key role in identifying developmental The parents of the child may be reassured that their child is developing normally for
delay. When delays are observed, the pediatrician should refer her age. Although most children begin walking at approximately 12 months of age,
for further evaluation early. Early identification of children with commencement of walking anytime up to age 18 months is considered to be within
developmental delays is critical because it allows for early inter- normal limits. The AAP recommends that standardized developmental screening be
vention. The prognosis for children with specific or global develop- performed when developmental surveillance identifies high-risk factors for devel-
opmental delay and routinely at the 9-, 18-, and 24- or 30-month health mainte-
mental delays can be greatly improved with participation in early
nance visits. Administration of a formal screening tool is likely not necessary at this
intervention stimulation programs. Identification and manage- visit but can be considered again at the 15-month visit if lack of progression in the
ment of underlying disease (eg, hypothyroidism, infection) also child’s gross motor skills is noted or if any other risk factor is identified at that time.
prevents further damage. Referring families to their state’s early

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 220 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Hamilton SS, Glascoe FP. Making developmental behavioral screening work for
Selected References
school-aged kids. Contemporary Pediatrics. 2010:63–87
American Academy of Pediatrics Council on Children With Disabilities, Section Marks KP, LaRosa AC. Understanding developmental-behavioral screening
on Developmental Behavioral Pediatrics, Bright Futures Steering Committee, measures. Pediatr Rev. 2012;33(10):448–458 PMID: 23027599 https://doi.
Medical Home Initiatives for Children With Special Needs Project Advisory org/10.1542/pir.33-10-448
Committee. Identifying infants and young children with developmental dis-
Roberts G, Palfrey J, Bridgemohan C. A rational approach to the medi-
orders in the medical home: an algorithm for developmental surveillance
cal evaluation of a child with developmental delay. Contemporary Pediatrics.
and screening. Pediatrics. 2006;118(1):405–420. Reaffirmed August 2014
2004;21:76–100
PMID: 16818591 https://doi.org/10.1542/peds.2006-1231
Scharf RJ, Scharf GJ, Stroustrup A. Developmental milestones. Pediatr Rev.
Centers for Disease Control and Prevention. CDC’s developmental milestones.
2016;37(1):25–37 PMID: 26729779 https://doi.org/10.1542/pir.2014-0103
CDC.gov website www.cdc.gov/ncbddd/actearly/milestones/index.html.
Accessed July 10, 2019 Shevell M, Ashwal S, Donley D, et al; Quality Standards Subcommittee of the
American Academy of Neurology; Practice Committee of the Child Neurology
Fernald A, Marchman VA, Weisleder A. SES differences in language process-
Society. Practice parameter: evaluation of the child with global developmental
ing skill and vocabulary are evident at 18 months. Dev Sci. 2013;16(2):234–248
delay: report of the Quality Standards Subcommittee of the American Academy
PMID: 23432833 https://doi.org/10.1111/desc.12019
of Neurology and The Practice Committee of the Child Neurology Society.
Gerber RJ, Wilks T, Erdie-Lalena C. Developmental milestones: motor devel- Neurology. 2003;60(3):367–380 PMID: 12578916 https://doi.org/10.1212/01.
opment. Pediatr Rev. 2010;31(7):267–277 PMID: 20595440 https://doi. WNL.0000031431.81555.16
org/10.1542/pir.31-7-267
Wilks T, Gerber RJ, Erdie-Lalena C. Developmental milestones: cognitive
Gerber RJ, Wilks T, Erdie-Lalena C. Developmental milestones 3: social-emotional development. Pediatr Rev. 2010;31(9):364–367 PMID: 20810700 https://doi.
development. Pediatr Rev. 2011;32(12):533–536 PMID: 22135423 https://doi. org/10.1542/pir.31-9-364
org/10.1542/pir.32-12-533
Zwaigenbaum L, Bauman ML, Fein D, et al. Early screening of autism spectrum dis-
Hagan JF Jr, Shaw JS, Duncan PM, eds. Bright Futures: Guidelines for Health order: recommendations for practice and research. Pediatrics. 2015;136(suppl 1):
Supervision of Infants, Children, and Adolescents. 4th ed. Elk Grove Village, IL: S41–S59 PMID: 26430169 https://doi.org/10.1542/peds.2014-3667D
American Academy of Pediatrics; 2017

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 CHAPTER 33

Speech and Language

Development: Normal Patterns
and Common Disorders
Geeta Grover, MD, FAAP, and Michelle L. Wahlquist, CCC-SLP

CASE STUDY
The parents of a 3-year-old girl bring her to see you. Questions
They are concerned because their daughter has only an 1. What expressive language skills should a child have
8- to 10-word vocabulary, and she does not put words by age 3 years?
together into phrases or sentences. They report that she 2. Approximately how many words should 3-year-olds
seems to have no hearing problems; she responds to her have in their vocabulary?
name and follows directions well. 3. By what age should children’s speech be intelligible
In general, she has been in good health. Aside from to strangers at least 75% of the time?
delayed speech, her development is normal. During the 4. What factors may be associated with delayed speech
physical examination, which is also normal, the girl does development?
not speak. 5. What tests are used to assess children’s hearing,
speech, and language development?

The ability to communicate through language is a uniquely human parents. Normal patterns of language development should be as
skill. It develops in a predictable, orderly sequence, beginning in familiar to pediatricians as all other aspects of child development
infancy with nonverbal forms and eventually progressing to the use (see Chapter 32), thereby allowing for early identification and refer-
of verbal language. When discussing the development of a child’s ral for suspected delays.
communication skills, professionals often use the terms “speech” The development of language skills in a normal sequence but
and “language.” Speech refers to the articulation and production at a slower pace than normal is referred to as language delay; lan-
of speech sounds within the mouth, whereas language involves guage delays may affect only expressive language or both receptive
comprehension and expression; language is the understanding and expressive language (eg, a mixed receptive-expressive language
and use of words, phrases, and gestures to convey intent. Normal delay). An atypical sequence of language skill acquisition is referred
hearing is essential to the development of both speech and to as a language disorder. Children with developmental language dis-
language. orders have persistent and significant limitation in their ability to
Language is often thought of as encompassing 2 components: receive or express language.
receptive language and expressive language. Receptive language
refers to the ability to understand others, whereas expressive
language is the ability to produce communication to convey Epidemiology
meaning to others. Although most people think of language simply The prevalence of specific language impairment in school-age
in terms of only receptive and expressive language, several other children with no hearing loss or obvious genetic or neurologic
critical components of language development must be present for condition is approximately 7%. Speech and language disorders
a child to develop effective communication. These include joint are more common in boys than girls and in children with a family
attention, play, and social-pragmatic language. history of language, speech, or reading disorders. Good evidence
The development of normal speech and language skills is an exists that early language impairment is associated with later
important developmental milestone that is eagerly awaited by difficulties learning to read.

221

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 222 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Clinical Presentation attention, play, receptive language, expressive language, and social-
pragmatic language—which are discussed herein in order of devel-
Lack of response to sound at any age, difficulty following directions,
opmental progression by timing of acquisition (Figure 33.1).
failure to achieve age-appropriate expressive language skills, reduced
Additionally, a brief description of speech sound production, which
eye gaze or gesture, and parental concern about a child’s hearing are
also is important for successful communication, is provided.
the most important signs of hearing or language impairment. Deaf
The foundation of language development begins with eye con-
infants coo normally and may even babble; thus, an infant’s vocal-
tact, social smiling, and the ability to share attention with others,
izing does not preclude hearing loss.
that is, joint attention. Use of eye gaze provides children with their
first experiences with shared meaning, which is crucial to language
Pathophysiology development. Each time children look at their parent, they are pro-
The left hemisphere of the brain is responsible for language skills in vided with language learning opportunities. While looking at their
94% of right-handed adults and in approximately 75% of left-handed parents, children begin to recognize and understand the meaning
adults. Peripheral auditory stimuli are transmitted to the primary audi- of nonverbal communication, including facial expressions and ges-
tory areas in both temporal lobes. Sounds then undergo a series of anal- tures. While watching a parent’s mouth, they observe how speech
yses, primarily in 3 main areas in the left cerebral cortex: the Wernicke sounds are formed. In using eye gaze, children begin to build a rela-
area (ie, auditory association area), which is responsible for language tionship and attachment to their parents, providing future motiva-
comprehension; the Broca motor speech (ie, motor encoding) area, tion to want to communicate. Joint attention is a more advanced
which is responsible for the preliminary conversion of language into form of eye gaze that develops by 12 to 15 months of age. It includes
motor activity; and the primary and supplementary motor cortices, sharing attention by alternating eye gaze between an object of inter-
which control the movements necessary for speech. This complex pro- est, a communication partner, and back to the object. It also involves
cess is responsible for the comprehension and production of language. following the attention of another (eg, following the eye gaze or point
For children to be successful communicators, they must be com- of another person). Without a strong foundation in joint attention, a
petent in all 5 critical domains of language development—joint child will have challenges in all other language learning.

Social-Pragmatic Language
The Social Rules of Conversation
(Initiating Conversation, Staying on Topic, Using/Understanding Body
Language, Perspective Taking, Humor)

Speech Expressive Language

(Articulation)
Fluency What We Express Through Gesture, Spoken Words, or Writing
(Stuttering) (Vocabulary, Grammar, Sentence Structure, Storytelling)
Voice

Receptive Language
Understanding and Processing Language Directed to Us
(Understanding Words, Concepts, and Questions; Following Directions;
Recalling Information)

Play
A Foundation of Language Development
(Important for Development of Sequencing, Problem Solving, Flexibility,
Turn-Taking, Forming New Idea)

Joint Attention
The Earliest Form of Communication
(Using Eye Gaze to Draw Attention to Objects of Interest or
Follow the Attention of Another)

Figure 33.1. Progression of language development through the 5 domains: joint attention, play, receptive language, expressive
language, and social-pragmatic language.

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 Chapter 33: Speech and Language Development: Normal Patterns and Common Disorders 223

Children’s language skills evolve primarily through parent-child Early language exposure through caregiver-child interaction is
interactions such as singing, reading, and play. It is within play that vital for the development of communication, cognitive, and aca-
children learn early vocabulary, language concepts (eg, big, little, demic skills. Earlier research reported that by the time a child is
fast, slow), problem solving, organization, turn-taking, and sequenc- 4 years of age, a difference in word exposure of up to 30 million words
ing, all of which are required for successful language use. may exist between children living in higher socioeconomic environ-
The ability to understand the communication of others is called ments compared with those living in lower socioeconomic environ-
receptive language. Early receptive milestones refer to ability to ments. Newer data support this finding when comparing families
hear and respond to sound (eg, look toward a rattle being shaken), from socioeconomic extremes (ie, top and bottom 2% of families),
whereas later milestones reflect ability to understand spoken words, although they suggest that the gap may be closer to 4 million words
follow directions, recall spoken information, and understand ques- for families in less extreme poverty. Regardless the size, a gap exists
tions. In typical language development, receptive language is more between children living in more privileged environments and those
advanced than expressive language. Children must understand a living in more impoverished environments. Research also suggests
concept before they can verbally express that same concept. that expressive language vocabulary at age 3 years is predictive of
The means by which children express their thoughts and ideas language and reading achievement up to 9 to 10 years of age (see
through gesture, spoken words, and written communication is called Chapter 34). Knowledge of normal play as well as social-pragmatic,
expressive language. Early expressive milestones relate to speech pro- receptive, and expressive language skills is essential to recognition
duction of vowels and simple consonants (eg, cooing, babbling); and identification of developmental delays (Table 33.1). Box 33.1
later, children begin to express themselves with gesture. Eventually, lists “danger signals” that indicate possible delays and serves as a
children use expressive language to convey their intent to others guide for referral to specialists. The American Academy of Pediatrics
through single words; short phrases; simple sentences, including reports that by age 18 months children should have an expressive
grammatical structures (eg, past tense [“-ed”]); and eventually in vocabulary of 10 to 25 words and at age 24 months children should
organized storytelling. be using a vocabulary of at least 50 words. It is important to rec-
Social-pragmatic language refers to the way in which language is ognize that a vocabulary of 50 words at 24 months is not an aver-
understood and used in a social context. It is the “unspoken,” social age vocabulary size, with some children producing fewer words
rules of conversation. Development of social-pragmatic language is a and some producing more words. Rather, the use of 50 words at
long-term process that begins in infancy with a child’s use of eye gaze, 24 months is a minimum single word vocabulary for a child of that
gesture, vocalizations, and single words to communicate with others age. Literature in the field of speech-language pathology suggests
for a variety of reasons (eg, to request, to comment, to protest, to show that, on average, children 24 months of age are able to produce 200
off, to share information). Important early milestones presenting to 300 words, with vocabulary expanding to 1,000 words by age
between 9 and 12 months of age include protoimperative pointing (to 3 years. Thus, if a health professional sees a 24-month-old child who
request) and protodeclarative pointing (to show). Social-pragmatic appears to be “struggling” to reach the 50-word milestone, language
language skills continue to develop and become more refined into late development should be monitored closely. The presence of addi-
adolescence. Later developing skills include understanding and use of tional language concerns or risk factors for language delay or hearing
appropriate body language, initiating and maintaining conversation, impairment warrants referral to a pediatric audiologist and pedi-
staying on topic, taking the perspective of others, and using humor. atric speech pathologist. Children must be able to understand and
Social-pragmatic language deficits are a core feature of autism spec- express at least 50 words before they can begin combining words
trum disorder (ASD). Because ASD currently affects 1 in 59 children, into 2-word combinations. It is important to remember that by age
all health professionals should be mindful of social-pragmatic lan- 3 years, 75% of children’s speech should be intelligible to strangers.
guage development and deficits (see Chapter 132).
For children to use language to communicate effectively, they
must be intelligible to others. Speech sound production, which often Differential Diagnosis
is called “articulation,” refers to how a child uses the structures of the The various causes of delayed language development include hear-
mouth to produce speech sounds. Like language, speech sounds fol- ing loss, disorders of central nervous system processing, anatomic
low a developmental progression. Speech disorders include problems abnormalities, and environmental deprivation (Box 33.2). Although
in the production of speech sounds. Speech disorders may affect birth order (eg, the belief that younger children speak later than
articulation (ie, phonologic disorders), motor planning (ie, child- firstborn children because older siblings speak for them), laziness
hood apraxia of speech), motor strength (ie, dysarthria), fluency (eg, “Don’t give him what he wants when he points. Make him ask
(ie, stuttering), or voice (ie, quality, tone, pitch, volume). By 3 years for it”), and bilingualism are commonly believed to result in speech
of age, a child should be at least 75% intelligible to strangers. By and language delay, these factors have never been proved to have
4 years of age, a child should be 100% intelligible, although speech a contributory role in such delay. For a complete discussion of
production errors (eg, “wabbit” for rabbit) may persist. This reflects hearing loss, refer to Chapter 88.
the “rule of 4s,” that is, 50% intelligible by age 2 years, 75% by age Disorders of central nervous system processing include global
3 years, and 100% by age 4 years. developmental delay, intellectual disability, ASD (see Chapter 132),

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 224 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 33.1. Receptive, Expressive, Play and Social-Pragmatic Language Milestones (Birth to 5 Years)
Milestone Type Skill Mean Age Normal Range
Receptive Alerts to sound Newborn N/A
Orients to sound/turns to voice 4 months 3–6 months
Responds to name 4 months 4–9 months
Understands “no” 10 months 9–18 months
Follows 1-step command with gesture 12 months 10–16 months
Follows 1-step command without gesture 15 months 12–20 months
Points to several body parts 18 months 12–24 months
Follows 2-step command with gesture 24 months 22–30 months
Understands basic spatial terms (eg, in, on, under) 28 months 27–30 months
Follows 3-step, unrelated directions 34 months 33–36 months
Understands basic colors and shapes 42 months 36–48 months
Expressive Cooing (vowel sounds) 3 months 1–4 months
Laughs 4 months 3–6 months
Babbling (consonants added to vowel sounds) 6 months 5–9 months
Dada/Mama nonspecifically 8 months 6–10 months
Dada/Mama specifically 10 months 9–14 months
3- to 5-word vocabulary 12 months —
Immature jargoning (ie, gibberish with inflection) 13 months 10–18 months
Mature jargoning (ie, gibberish with the occasional word) 18 months 16–24 months
10- to 25-word vocabulary 18 months —
≥50-word vocabulary (50–300 words) 24 months (minimum) —
2-word phrases 24 months 20–30 months
Uses pronouns indiscriminately 24 months 22–30 months
States first name 34 months 30–40 months
Uses pronouns appropriately (ie, I, you, we, me, they) 36 months 30–42 months
≥200-word vocabulary (200–1,000 words) 3 years —
75% of speech intelligible to strangers 3 years —
3-word sentences 3 years —
Answers simple “WH” questions (Who, What, Where) — 3–4 years
Complex sentences using >1 action word (eg, “I lost my balloon because 4 years —
I let go”)
100% intelligible to strangers but may still have articulation errors 4 years —
Names letters or numbers — 4–5 years
Says rhyming words — 4–5 years
Tells short stories — 4–5 years
Playa Exploratory play (mouthing, shaking, banging, tapping, and squeez- — 4–10 months
ing toys)
Object permanence (finds object completely hidden under blanket) and 10 months 9–12 months
means-end behavior (pulls string to obtain desired toy)
1-step pretend play (eg, pretends to drink from a cup or feed doll with bottle) 18 months 17–22 months
Imitates housework activities — 18–21 months
Parallel play (ie, sharing play space with another child but not necessarily 24 months —
interacting)

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 Chapter 33: Speech and Language Development: Normal Patterns and Common Disorders 225

Table 33.1. Receptive, Expressive, Play and Social-Pragmatic Language Milestones (Birth to 5 Years) (continued )
Milestone Type Skill Mean Age Normal Range
Playa (continued) Symbolic object use (pretend 1 object is another [eg, a banana becomes — 24–30 months
a telephone])
Multiple-step play (eg, mix cake, bake cake, eat, and wash dishes) and — 36–42 months
imagines self as different characters (eg, firefighter, mom/dad)
Cooperative play (ie, works together with other children for a common — 3–4 years
play goal)
Social-pragmatica Spontaneous social smile 6 weeks 1–3 months
Dyadic joint attention (ie, infant and adult take turns exchanging looks, 8 weeks 6–10 weeks
noises, and mouth movements)
Joint attention (ie, uses 3-point gaze shifts and follows the gaze of another) — 12–15 months
Takes 2 turns in conversation 27 months 24–30 months
Verbally expresses emotional and physical feelings (eg, happy, sad, 30 months 25–36 months
sleepy, hurt)
Takes 4–5 turns in conversation 40 months 36–42 months
Theory of mind (ie, understanding another person’s knowledge, beliefs, 4 years —
intentions, and emotions)
Changes topics appropriately — 4–5 years
Abbreviation: N/A, not applicable.
a
Derived from Westby CE. A scale for assessing development of children’s play. In: Gitlin-Weiner K, Sandgrund A, Schaefer CE, eds. Play Diagnosis and Assessment. 2nd ed. New York, NY: John Wiley & Sons;
2000:15–57.

Box 33.1. Danger Signals in Language Box 33.2. Causes of Delayed Language
Development Development
ww Inconsistent or lack of response to auditory stimuli at any age ww Hearing impairment
ww Regression in language or social skills at any age ww Perinatal risk factors resulting in hearing impairment
ww No babbling by age 9 months ww Disorders of central nervous system processing
ww No pointing or gesturing by age 12 months —— Global developmental delay
ww No intelligible single words by age 16 months —— Intellectual disability
ww No joint attention (ie, following the eye gaze of others) by age —— Autism spectrum disorder
15 months ww Developmental language disorders
ww No 2-word spontaneous phrases by age 24 months ww Disorders of speech production
ww Inability to respond to simple directions or commands (eg, “sit down,” —— Articulation disorder
“come here”) by age 24 months —— Dysarthria
ww Speech predominantly unintelligible at age 36 months —— Verbal apraxia
ww Dysfluency (ie, stuttering) of speech noticeable after age 5 years ww Presence of anatomic abnormalities (eg, cleft lip, cleft palate)
ww Hypernasality at any age ww Environmental deprivation
ww Inappropriate vocal quality, pitch, or intensity at any age

with difficulties in speech production. Children with cleft palate

and developmental language problems. Developmental language dis- characteristically have hypernasal speech secondary to velopha-
orders produce speech or language delays in children in the absence ryngeal incompetence (eg, dysfunction of the soft palate). Speech
of hearing loss, anatomic abnormalities of the vocal tract, intellec- sound production can also be affected as children attempt to com-
tual disability, or global developmental delay. “Late talkers,” that is, pensate for their inability to achieve appropriate velopharyngeal clo-
children with normal comprehension but who simply begin speak- sure while speaking. In addition, conductive hearing loss may result
ing late, have mild developmental language problems. Children who from chronic serous otitis media, which is common in children with
are completely nonverbal have more severe problems. cleft palate. The presence of a submucosal cleft palate, which is char-
Anatomic abnormalities may also result in speech and language acterized by a bifid uvula, diastasis of the muscles in the midline of
delays. Cleft palate is the abnormality most commonly associated the soft palate with intact mucosa, and notching of the posterior

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 226 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

border of the hard palate, should be considered in children without

Box 33.4. High-Risk Indicators for Hearing Loss
an overt cleft palate who have recurrent symptomatic serous otitis
in Children (Birth to 24 Months)
media, hypernasality, and difficulties of speech production.
Environmental deprivation is another cause of delayed language Neonatal Period (Birth–28 days)
development, especially in families in which children are not spo- ww Family history of sensorineural hearing loss (SNHL)
ken or read to. Sometimes additional historical or physical evidence ww In utero infection associated with SNHL (eg, TORCHS [toxoplasmo-
exists of deprivation (eg, profound failure to thrive, physical or sex- sis, other agents, rubella, cytomegalovirus, herpes simplex, syphilis]
ual abuse) or emotional trauma (eg, domestic violence). infections)
ww Anatomic malformations of the head and neck region
Evaluation ww Hyperbilirubinemia at a level requiring exchange transfusion
ww Low birth weight (≤1,500 g [3.3 lb])
History ww Bacterial meningitis
When evaluating children younger than 3 years, pediatricians must ww Low Apgar scores
rely primarily on parental reports of children’s language capabilities ww Respiratory distress (eg, meconium aspiration)
(Box 33.3). Children age 3 years and older usually can be engaged in ww Mechanical ventilation for ≥10 days
conversation during the visit, but younger children are more likely to ww Exposure to ototoxic medications (eg, aminoglycoside antibiotics) for
be uncooperative or remain silent when confronted by strangers or >5 days or in combination with loop diuretics
new situations. Historical factors, such as a family history of child- ww Stigmata of syndromes known to be associated with hearing loss (eg,
hood deafness or speech and language delays, exposure to ototoxic Down syndrome)
agents (eg, aminoglycoside antibiotics), neonatal asphyxia or neo-
Infants and Children (29 days–24 months)
natal intensive care unit stay of more than 5 days, hyperbilirubine-
ww Parental concern about hearing, speech, language, or developmental delay
mia, maternal cocaine abuse, and low birth weight, provide valuable
ww Recurrent or persistent otitis media with effusion for ≥3 months
information in the identification of infants at high risk for delayed
ww Severe head trauma (associated with fracture of the temporal bone)
speech and language development (see Boxes 33.1 and 33.4).
ww Infections associated with SNHL (eg, bacterial meningitis, mumps,
measles)
Physical Examination
ww Neurodegenerative disorders or demyelinating diseases
The physical examination includes a thorough assessment of the ww Any of the newborn risk factors listed previously
head and neck region, including examination of the hard and soft
palate, to assess for the presence of overt or submucous cleft palate. Adapted with permission from Muse C, Harrison J, Yoshinaga-Itano C, et al; American Academy of
Microcephaly may be an indicator for intellectual disability or struc- Pediatrics Joint Committee on Infant Hearing. Supplement to the JCIH 2007 position statement:
tural abnormalities. Abnormalities of the external ear (eg, microtia) principles and guidelines for early intervention after confirmation that a child is deaf or hard of
hearing. Pediatrics. 2013;131(4):e1324–e1349 and Harlor AD Jr, Bower C; American Academy of
may be associated with sensorineural hearing loss. Otoscopic exam-
Pediatrics Committee on Practice and Ambulatory Medicine, Section on Otolaryngology-Head and
ination of the ear is an essential aspect of the examination. A mid- Neck Surgery. Hearing assessment in infants and children: recommendations beyond neonatal
dle ear effusion may be associated with conductive hearing loss. The screening. Pediatrics. 2009;124(4):1252–1263.
tympanic membrane is examined for evidence of scarring or per-
foration, which often occurs secondary to recurrent otitis media.
Pneumatic otoscopy is a subjective assessment of tympanic mem-
brane mobility (eg, compliance) when the membrane is subjected
to a pulse of air. Tympanometry (eg, impedance audiometry) per-
Box 33.3. What to Ask formed in the office can provide objective information about the
Language Development mobility of the tympanic membrane and the presence of middle ear
ww Are the child’s language capabilities age-appropriate? effusions (Figure 33.2). Tympanometry is not a hearing test; rather,
ww Does the parent or do the parents feel that their child has difficulty it is an assessment of middle ear functioning that uses sound energy
hearing? to determine the compliance of the tympanic membrane and pres-
ww Is there any family history of childhood deafness? sure in the middle ear. Physical stigmata of any syndromes that may
ww Is there a family history of speech and language delays? be associated with deafness are noted (Box 33.5).
ww Has the child been exposed to any ototoxic agents (eg, aminoglycoside
Laboratory Tests
antibiotics)?
ww Is there a history of neonatal asphyxia, hyperbilirubinemia, or low birth The advantages of early identification of hearing loss cannot be over-
weight? emphasized. The Joint Committee on Infant Hearing “Year 2007
ww Has the child ever had bacterial meningitis? Position Statement: Principles and Guidelines for Early Hearing
ww Did the mother have a TORCHS (toxoplasmosis, other agents, rubella, Detection and Intervention Programs” recommends that the hear-
cytomegalovirus, herpes simplex, syphilis) infection during pregnancy? ing of all newborns should be evaluated at no later than 1 month
of age. Newborns who do not pass the screening should undergo

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 Chapter 33: Speech and Language Development: Normal Patterns and Common Disorders 227

Box 33.5. Syndromes Commonly Associated

With Hearing Impairment

1.5
Autosomal-dominant Conditions
ww Branchio-oto-renal syndrome
Type A
Compliance (mL)
ww Goldenhar syndrome (ie, oculoauriculovertebral dysplasia)
ww Stickler syndrome
ww Treacher Collins syndrome
0.5 ww Waardenburg syndrome
Autosomal-recessive Conditions
–400 –200 0 +200
ww Alport syndrome
ww Jervell and Lange-Nielsen syndrome
Pressure (mm H2O) ww Pendred syndrome
ww Usher syndrome
Chromosomal Disorders
ww Trisomy 13 syndrome
ww Trisomy 18 syndrome
1.5 Miscellaneous Disorders
ww CHARGE (coloboma, heart disease, atresia choanae, growth and intel-
Compliance (mL)

Type B
lectual disability, genitourinary tract anomalies, and ear anomalies)
syndrome
0.5 ww TORCHS (toxoplasmosis, rubella, cytomegalovirus, herpes simplex, syph-
ilis) syndrome

–400 –200 0 +200 a comprehensive audiologic evaluation at no later than 3 months

Pressure (mm H2O) of age. High-risk indicators for hearing loss serve 2 purposes (see
Box 33.4). First, in areas in which universal newborn hearing
screening is not yet available, these indicators help physicians iden-
tify newborns who should undergo audiologic evaluation. Second,
these risk indicators help physicians identify neonates who require
ongoing medical and audiologic monitoring; normal hearing at birth
1.5 in these children may not preclude the development of later hear-
ing loss (eg, delayed onset).
Compliance (mL)

Type C
Speech and language evaluation begins in the pediatrician’s office,
where parent report inventories may be used to validate parental
concerns and office-based language assessment may be aided by the
0.5
use of screening tests such as the Clinical Linguistic and Auditory
Milestone Scale or the Early Language Milestone Scale. These tests are
used to supplement the clinical history of a child’s language abilities.
–400 –200 0 +200
Children with suspected language delays or disorders should be
Pressure (mm H2O) referred to specialists as early as possible. The first referral should be
to a pediatric audiologist to assess the child’s hearing. Normal hear-
ing is essential to the development of speech and language; thus,
Figure 33.2. Basic tympanometry curves. The Type A curve indicates a if any concerns exist related to the development of either, hearing
normally compliant tympanic membrane (TM). The Type B curve indicates loss must be ruled out. An additional referral should be made to a
little or no motion of the TM and can be seen with middle ear effusion, a speech-language pathologist (SLP) for a complete speech, language,
scarred TM, or a cholesteatoma. The Type C curve indicates negative middle and communication assessment (eg, assessment of pragmatic lan-
ear pressure and may be seen with a resolving middle ear effusion or guage skills) (Box 33.6). An abundance of research supports the ben-
eustachian tube dysfunction. Other variations can occur in these basic curves efit of early intervention in the development of speech and language
that are not illustrated here. skills. If any suspicion or concern exists regarding communication
development, it is best to have an SLP explore these concerns fur-
ther. Taking a “wait and see” approach with families wastes valuable

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 228 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Special education is of prime importance in the manage-

Box 33.6. Role of the Speech-Language
ment of children with language difficulties. The Education for All
Pathologist in the Evaluation
Handicapped Persons Act, which was enacted by the US Congress
and Management of Disorder
in 1975, and the subsequent comprehensive reauthorization of it in
ww Speech disorders affecting intelligibility (eg, difficulty producing speech 1990 and revision of it in 2004 as the Individuals with Disabilities
sounds, deficits in motor-planning for speech production, dysfluency or Education Act, requires that public schools provide individual-
stuttering, voice disorders, resonance disorders). ized and appropriate education for all children with disabilities.
ww Language disorders affecting the ability to understand language or Knowledge of available community resources (eg, special schools)
express oneself verbally or in writing. can aid pediatricians in providing support services, such as special
ww Social communication disorders affecting the ability to communicate for community agencies and support groups for children with disabil-
social purposes. Evaluation and treatment also may address use of eye ity and their parents. Children with language delay in the context of
gaze, joint attention, and play. global developmental delay, cognitive delay, or ASD require prompt
ww Cognitive-communication disorders, including problems organiz- referral to early intervention services.
ing thoughts and ideas, problem solving, planning, and remembering
information. Prognosis
ww Swallowing disorders, including difficulty with feeding and swallowing.
Children with a history of speech and language delays should
be monitored carefully for the emergence of reading difficulties.
Reading is a language-based skill that requires the appreciation of
time that could be used to provide intervention services to reme- subtle differences among speech sounds (eg, phonologic awareness)
diate speech and language deficits. It is not necessary for a child to and the ability to link these sounds to written symbols. The emer-
be using any language expressively to be evaluated or treated by gent literacy model of reading development assumes that reading,
an SLP. Children with speech or language impairments who also writing, and oral language develop concurrently based on interac-
exhibit signs of possible cognitive or social skills impairment should tions in social contexts.
undergo a comprehensive, multidisciplinary developmental assess- Children with speech and language delays require long-term
ment conducted by experts in developmental-behavioral pediatrics, monitoring of academic, emotional, and behavioral functioning.
psychology, speech-language pathology, and occupational therapy. Treated early, speech and language delays and disorders generally
improve over time. The final prognosis is dependent on the nature
Management and severity of the underlying cause and the interventions provided.
The first 3 years after birth are crucial to language development.
Hearing impairment is an important cause of delayed language CASE RESOLUTION
development in young children; thus, the Joint Committee on Infant
The child described in the case history has delayed development of expressive
Hearing 2007 position statement recommends that infants with language skills. At the age of 3 years, she should have a 250-word vocabulary
confirmed hearing loss should receive appropriate interventions by and speak in 3-word sentences; in addition, her speech should be primarily intel-
6 months of age. The history, physical examination, and initial ligible to strangers. Because of the delay, she should be immediately referred for
screening can be used to suggest referral to various specialists (eg, a hearing assessment and speech and language evaluation. Hearing loss is an
important diagnosis to rule out. Simply because her parents report no hearing
audiologists, SLPs, child psychologists, otolaryngologists) for further
problems does not mean she does not have a deficit. She may have learned to
clarification of hearing, speech, or language deficits and treatment. respond to nonverbal cues, or she may hear only some things.
Early identification of hearing impairment and the degree of severity
enable early intervention in the form of amplification of sound, spe-
cial education classes for affected children, and counseling and sup- Selected References
port services for the families of affected children (see Chapter 88).
Language learning occurs best in interactive and responsive envi- American Speech-Language-Hearing Association. The advantages of being
bilingual. ASHA.org website. www.asha.org/public/speech/development/the-
ronments. Parents of infants and young children should be encour-
advantages-of-being-bilingual/. Accessed August 22, 2019
aged to have fun speaking, singing, playing with toys, and reading
American Speech-Language-Hearing Association. How does your child hear
with their children. These reciprocal parent-child interactions, along
and talk? ASHA.org website. www.asha.org/public/speech/development/chart/.
with high verbal parental responsiveness, stimulate children cogni- Accessed March 27, 2019
tively and foster language development.
Bialystok E, Craik FI, Luk G. Bilingualism: consequences for mind and brain.
For bilingual children, the home language should be preserved Trends Cogn Sci. 2012;16(4):240–250 PMID: 22464592 https://doi.org/10.1016/j.
whenever possible. Bilingualism is associated with increased cog- tics.2012.03.001
nitive control in the form of improved executive function skills and Cates CB, Dreyer BP, Berkule SB, White LJ, Arevalo JA, Mendelsohn AL. Infant
mental flexibility. Evidence indicates that bilingualism itself does not communication and subsequent language development in children from low-
cause language delays; however, the risk for language delays exists income families: the role of early cognitive stimulation. J Dev Behav Pediatr.
for bilingual children as it does for monolingual children. 2012;33(7):577–585 PMID: 22947884

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 Chapter 33: Speech and Language Development: Normal Patterns and Common Disorders 229

Fierro-Cobas V, Chan E. Language development in bilingual children: a primer McQuiston S, Kloczko N. Speech and language development: monitoring
for pediatricians. Contemporary Pediatrics. 2001;7:79–98 process and problems. Pediatr Rev. 2011;32(6):230–239 PMID: 21632874
Gilkerson J, Richards JA, Warren SF, et al. Mapping the early language https://doi.org/10.1542/pir.32-6-230
environment using all-day recordings and automated analysis. Am J Muse C, Harrison J, Yoshinaga-Itano C, et al; Joint Committee on Infant Hearing.
Speech Lang Pathol. 2017;26(2):248–265 PMID: 28418456 https://doi. Supplement to the JCIH 2007 position statement: principles and guidelines for early
org/10.1044/2016_AJSLP-15-0169 intervention after confirmation that a child is deaf or hard of hearing. Pediatrics.
Harlor AD Jr, Bower C; American Academy of Pediatrics Committee on Practice 2013;131(4):e1324–e1349 PMID: 23530178 https://doi.org/10.1542/peds.2013-0008
and Ambulatory Medicine, Section on Otolaryngology-Head and Neck Surgery. Nelson HD, Nygren P, Walker M, Panoscha R. Screening for speech and language
Hearing assessment in infants and children: recommendations beyond neona- delay in preschool children: systematic evidence review for the US Preventive
tal screening. Pediatrics. 2009;124(4):1252–1263 PMID: 19786460 https://doi. Services Task Force. Pediatrics. 2006;117(2):e298–e319 PMID: 16452337 https://
org/10.1542/peds.2009-1997 doi.org/10.1542/peds.2005-1467
Hart B, Risley TR. The early catastrophe: the 30 million word gap by age 3. Owens RE Jr. Language Development: An Introduction. 7th ed. Boston, MA:
American Educator. Spring 2003;4–9 Pearson Education, Inc; 2008
Hoff E. Language Development. 5th ed. Belmont, CA: Wadsworth, Cengage Tierney CD, Brown PJ, Serwint JR. Development of children who have hearing
Learning; 2014 impairment. Pediatr Rev. 2008;29(12):e72–e73 PMID: 19047430 https://doi.
Joint Committee on Infant Hearing. Year 2007 position statement: principles and org/10.1542/pir.29-12-e72
guidelines for early hearing detection and intervention programs. Pediatrics. Westby CE. A scale for assessing development of children’s play. In: Gitlin-Weiner K,
2007;120(4):898–921 PMID: 17908777 https://doi.org/10.1542/peds.2007-2333 Sandgrund A, Schaefer CE, eds. Play Diagnosis and Assessment. 2nd ed. New York,
Lee J. Size matters: early vocabulary as a predictor of language and literacy com- NY: John Wiley & Sons; 2000:15–57
petence. Applied Psycholinguistics. 2011;32(1):69–92 https://doi.org/10.1017/ Zebrowski PM. Developmental stuttering. Pediatr Ann. 2003;32(7):453–458
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 CHAPTER 34

Literacy Promotion
in Pediatric Practice
Wendy Miyares, RN, PNP

CASE STUDY
You are seeing a 9-month-old boy for the first time for Questions
a well-child visit. The child has a completely negative 1. How are reading and language developmentally
history and seems to be thriving. The patient’s mother related?
works part-time as a housekeeper, and his father is a 2. What are the consequences of low literacy when
seasonal worker in agriculture. The infant is up-to-date children get older?
on his immunizations. The family history is noncon- 3. How are literacy and health outcomes related?
tributory, but his mother mentions that her 6-year-old 4. What are the components of the Reach Out and
daughter needs to repeat kindergarten. Teachers have Read model?
advised the mother that her eldest daughter is coop- 5. What can pediatricians do to promote literacy
erative, but she has not yet mastered letters and early in families?
reading. Mother says she is not concerned because the
teacher said with “a little more time” her daughter will
be fine.

Early literacy promotion in the office setting is now recognized as 100 billion neurons, which go on to form trillions more connections.
an essential part of pediatric primary care. The American Academy Those connections that are stimulated by frequent use persist, and
of Pediatrics (AAP) and the Canadian Paediatric Society encourage less-used synapses are eliminated as the brain matures. Reading
health professionals to include literacy promotion in the routine clin- aloud and book sharing may help ensure the preservation of brain
ical care of toddlers and young children. It is known from the ever- connections associated with skills such as memory, creativity, com-
expanding evidence of early brain development that reading aloud, prehension, and language. Reading aloud is also a positive nurturing
speaking to babies, singing, and sharing books can permanently activity which in itself promotes other important neuronal con-
change neuronal connections in the brain. These connections forged nections and healthy development. The AAP recommends avoid-
by reciprocal attention and spoken language are important for learning ing television or other electronic media for children younger than
during the school years as well as for the emotional health of the child. 2 years, because young children learn best by interacting with people.
Literacy promotion can begin even before a baby learns to speak Reading aloud exposes children to vocabulary they do not hear
and long before a child is ready to learn to read. Eventual mastery of in daily conversations (eg, 3 bears, beanstalks). Reading aloud also
reading will depend on skills such as language ability, imagination, stimulates the imagination (eg, cows jumping over the moon). In
and familiarity with books and the reading process. Children develop time, children learn that the abstract letters on the page represent
many of these skills in the first few years after birth, even before they words, and they become aware of different, smaller sounds that make
go to preschool. In fact, a child’s language ability by 3 years of age is up words. All these experiences result in reading readiness.
strongly correlated with later academic performance. Parent-child
interactions are crucial, and guidance for parents on literacy promo-
tion activities at home should begin within the first year after birth. Consequences of Low Literacy
Reading aloud to children on a regular basis is among the most Low literacy has significant consequences as children age. Poor
effective means of promoting early literacy and language develop- academic skills are consistently linked with higher dropout rates,
ment. Language skills are the foundation for later reading ability entrance into the juvenile justice system, and unemployment. One-
and are largely dependent on the amount and quality of language third of all juvenile offenders are reported to read below the fourth-
exposure. The architecture of a developing brain is physically altered grade level, and more than 80% of adult prison inmates are high
by experiences during infancy. At birth, a baby’s brain contains school dropouts.

231

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 232 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Literacy level and health outcomes are also intimately related. literacy carries a high financial cost, with billions of dollars spent in
Health literacy is defined as the degree to which individuals can obtain, the United States each year on preventable emergency department
process, and understand the basic information they need to make visits and hospital stays.
appropriate health decisions. Multiple studies have demonstrated that
a low literacy level negatively affects health and well-being. Compared Literacy Promotion in the Medical
with average or above-average health literacy, individuals with limited Office
health literacy have a higher number of visits to emergency depart- The AAP endorses the Reach Out and Read model of early literacy
ments and hospitalizations, and increased morbidity. promotion, and this model is incorporated in the official AAP Bright
Adolescents with low reading ability are more likely to smoke, Futures guidelines for pediatric health professionals.
use alcohol, carry a weapon, and be in a physical fight that results in The Reach Out and Read model has 3 main components:
the need for medical treatment. Conversely, higher levels of literacy 1. Anticipatory guidance: During regular well-child visits, health
are associated with positive health outcomes, such as appropriate professionals encourage parents to read aloud to their young chil-
use of inhaled asthma medication or choosing to breastfeed a baby. dren at home. The advice is age-appropriate, and concrete exam-
Promoting literacy is good medicine not only for the individual ples are provided or modeled by the physician (Table 34.1).
but also for society. Aside from the societal effect of school failure, 2. Books: A new developmentally and culturally appropriate book is
individuals with limited literacy incur medical expenses that are up given by the physician to patients and parents at each well-child
to 4 times greater than patients with adequate literacy skills. Low visit so that parents have the tools to follow the physician’s advice.

Table 34.1. Developmental Milestones of Early Literacy

Patient Age Motor Development Cognition What Parents Can Do
6–12 Months Reaches for books Looks at pictures Hold the child comfortably; face-to-face gaze.
Book to mouth Vocalizes, pats pictures Follow the baby’s cues for “more” and “stop.”
Sits in lap; holds head steady Prefers pictures of faces Point to and name pictures.
Turns pages with adult help
12–18 Months Sits without support No longer mouths right away Respond to the child’s prompting to read.
May carry the book Points at pictures with 1 finger Let the child control the book.
Holds the book with help May make the same sound for a particular Be comfortable with the toddler’s short
Turns board pages (several at a time) picture (labels) attention span.
Points when asked, “Where’s...?” Ask, “Where’s the...?” and let the child point.
Turns the book right side up
Gives the book to an adult to read
18–24 Months Turns board pages easily, 1 at a time Names familiar pictures Relate books to the child’s experiences.
Carries the book around the house Fills in words in familiar stories Use books in routines, including bedtime.
May use a book as a transitional object “Reads” to dolls or stuffed animals Ask the child, “What’s that?” and let the child
Recites parts of well-known stories answer.
Attention span is highly variable Pause and let the child complete the sentence.
24–36 Months Learns to handle paper pages Recites whole phrases and sometimes whole Keep using books in routines.
Goes back and forth in books to find stories Read at bedtime.
favorite pictures Coordinates text with picture Be willing to read the same story over and over.
Protests when the adult gets a word wrong Ask, “What’s that?”
in a familiar story Relate books to the child’s experiences.
Reads familiar books to self Provide crayons and paper.
≥3 Years Competent book handling Listens to longer stories Ask, “What’s happening?”
Turns paper pages 1 at a time Can retell familiar story Encourage writing and drawing.
Moves finger along text Let the child tell the story.
“Writes” own name
Moves toward letter recognition
Adapted from Reach Out and Read. Milestones of early literacy development. www.reachoutandread.org/resources. Accessed February 19, 2020. Used with permission.

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 Chapter 34: Literacy Promotion in Pediatric Practice 233

3. Waiting rooms: Literacy-rich waiting rooms contribute to the lit- see if his mother is watching, the physician can gain information
eracy message. Gently used books for parents to read to their child about fine motor ability and the child’s social interaction in only a
while waiting and displays or information about local libraries are few moments.
encouraged. Where appropriate and feasible, community volun- Children benefit from storytelling and book time in whatever
teers read aloud in waiting rooms, modeling for parents the plea- fashion is most comfortable for the parent. Ideally books should be
sures and techniques of reading aloud to very young children. either in the family’s preferred language or bilingual. It is best for
Reach Out and Read had its origins in an urban clinic that served parents to use the language that is easiest for them, because it is more
a high proportion of low-income families and has always had a spe- important for children to hear a language with rich vocabulary and
cial focus on children growing up in poverty. For complex reasons, complex sentences than to learn English first. If no book is avail-
poverty is a powerful predictor of children’s exposure to language. able in the family’s native language or parents are not confident with
Children in low-income homes are 40% less likely to be read to on their own reading skills, the primary care physician should encour-
a daily basis than children in higher-income households. Pioneer age parents to look at books, name pictures, and talk about what is
researchers Hart and Risley estimate that by age 4 years, children going on in the pictures with their children. Physician knowledge of
living below the poverty line hear 30 million fewer words in total local programs for adult literacy and English as a second language
than those who grow up in higher-income households. is often useful, because parents may ask for resources when literacy
Providing advice to parents is easiest and most effective when a is discussed in the examination room.
book is brought in at the beginning of a visit. That way, physicians
can naturally weave in guidance that is appropriate to the age of the Primary Care Physicians Can Make
child (Box 34.1 and Table 34.1). Advice should be brief and to the a Difference
point, supportive, and part of a general conversation about the child’s
The primary care setting is the ideal venue for literacy promotion.
development and behavior.
Almost all parents will bring in their child for routine examinations
Another advantage of presenting the book early in the visit is
or urgent visits, which provides an opportunity to reach a broad
the amount of developmental and relationship information that can
group, not just those seeking learning experiences for their children.
be observed by the health professional. The book is a tool that can
In fact, 96% of all children younger than 6 years see their physician
speed informal developmental surveillance. If a 2-year-old exclaims,
at least once annually. Each child is seen for multiple visits from a
“Doggie says bow-wow!” there is no doubt she is putting 2 words
very young age, providing repeat opportunities to discuss reading
together. If a 1-year-old uses an index finger to point and looks to
aloud during the critical first years. Suggestions in support of
literacy-focused activities are a logical extension of the advice about
growth and development that parents are already receiving at these
Box 34.1. Anticipatory Guidance for Parentsa visits. Those who provide primary care can review daily activities
in which reading aloud can be incorporated, such as reading traffic
yy Newborns and very young babies need to hear a parent’s voice as much signs, store names, or simple food labels. When parents trust and
as possible: talking, singing, and telling stories are all good. value advice from their primary care physician, early literacy mes-
yy 6-month-olds may put books in their mouths; this is developmentally sages assume greater credibility.
normal and appropriate and is why we give them chewable board books. The effectiveness of this primary care model to promote read-
It is not in any way an indication that the child is too young for a book! ing aloud to young children has been demonstrated in multiple
yy 12-month-olds may point with 1 finger to indicate interest in a picture; research studies. Parents who received even 1 book were much
parents should see this as developmental progress. more likely to read aloud to their children and report reading as
yy 18-month-olds may turn board book pages and may insist on turning a favorite activity of their child. The effect was greatest for the
back again and again to a favorite picture. poorest families, which is an important finding for children who
yy 2-year-olds may not sit still to listen to a whole book but will still enjoy may need this intervention the most. The outcome was similar
looking at individual pages or having parents read them stories bit by for non-English-speaking families, and the findings held even
bit. if the provided book was written in English. Finally, and most
yy 3-year-olds may retell familiar stories and may memorize their favorite encouraging, toddlers and preschoolers who had received care
book. in clinics in which the Reach Out and Read model was used had
yy 4- and 5-year-olds may start to recognize letters or their sounds. They higher scores on language and vocabulary assessments than chil-
can understand and follow longer stories. dren who had not been not served by the program.
yy School-age children will start to be able to read to you—but do not stop Medical professionals who promote literacy and use the Reach
reading to them—and enjoy taking turns. Out and Read model of providing books and advice will help build
stronger bonds between health professional and family. Using clinic
a
Specific guidance can help parents with age-appropriate expectations for how their children will
physically handle and interact with books, and respond to stories.
visits as an opportunity to tailor age-appropriate advice for parents
Adapted from Reach Out and Read. Milestones of early literacy development. www.reachoutandread. and emphasize the importance of reading aloud and the beneficial
org/resources. Accessed February 19, 2020. Used with permission. effects is favorable for all.

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 234 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Berkman ND, Sheridan SL, Donahue KE, et al. Health Literacy Interventions and
CASE RESOLUTION Outcomes: An Updated Systematic Review. Rockville, MD: Agency for Healthcare
Research and Quality; 2011. Evidence Reports/Technology Assessments, No.
You speak to the mother about the benefits of reading aloud to her children.
199. www.ncbi.nlm.nih.gov/books/NBK82444/table/appendixes.app10.
You give the 9-month-old a board book with pictures of baby faces. You demon-
t1/?report=objectonly. Accessed March 11, 2019
strate, showing how the 9-month-old is interested in the pictures and engages
with vocalizations as you describe each page. At the end of the visit you also find National Network of Libraries of Medicine. Health literacy. NNLM.gov website.
a gently used rhyming book that the mother can take home for her 6-year-old https://nnlm.gov/initiatives/topics/health-literacy. Accessed March 11, 2019
daughter. Reach Out and Read. Leyendo Juntos (Reading Together): Literacy Promotion
for Pediatric Primary Care Providers. Boston, MA: Reach Out and Read; 2011.
www.reachoutandread.org/FileRepository/LeyendoJuntosProviderGuide.pdf.
Accessed March 11, 2019
Selected References
Reach Out and Read. What children like in books. Reachoutandread.org
American Academy of Pediatrics. Bright futures. Brightfutures.aap.org website. website. www.reachoutandread.org/FileRepository/WhatChildrenLikeinBooks.
https://brightfutures.aap.org. Accessed March 11, 2019 pdf. Accessed March 11, 2019
American Academy of Pediatrics. Early brain and child development: building
brains, forging futures. AAP.org website. www.aap.org/en-us/advocacy-and-
policy/aap-health-initiatives/EBCD/Pages/About.aspx. Accessed March 11, 2019

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 CHAPTER 35

Gifted Children
Calla R. Brown, MD, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP

CASE STUDY
A 3-year-old girl is brought to your office for well-child The girl is engaging and talkative. She asks ques-
care. Her parents believe that she may be gifted, because tions about what you are doing during the examination
she is much more advanced than her sister was at the and demonstrates impressive knowledge of anatomy.
same age. The parents report that their younger daugh- The physical examination is normal.
ter walked at 11 months of age and was speaking in
2-word sentences by 18 months. She is very “verbal,” has
Questions
1. How are gifted children identified?
a precocious vocabulary, and constantly asks difficult
2. What characteristics are associated with giftedness?
questions such as, “How do voices come over a radio?”
3. What are the best approaches for optimizing the
The girl stays at home with her mother during the day
education of gifted children?
but recently began attending a preschool program
4. What is the role of the pediatrician in the care of
2 mornings a week. She enjoys preschool and plays
gifted children?
well with children her own age. She also likes to play
with her sister’s friends from school.

Addressing caregiver concerns about giftedness provides an excit- Epidemiology

ing intersection of strengths-based pediatric medicine with the
The prevalence of giftedness depends on the somewhat arbitrary
educational system. Because several definitions of giftedness exist,
definitions of giftedness and the varied approaches to the identi-
prevalence rates vary. Identification of gifted children coupled with
fication of gifted children. Traditional screening systems identify
advocacy can promote an optimal educational match for the child;
3% to 5% of students for participation in gifted programs in schools.
however, identification systems currently in use may result in an
Some schools use an alternative system in which 10% to 15% of
underrepresentation of children from socioeconomically disadvan-
children are recognized as above average. With the goal of fostering
taged backgrounds. Pediatricians can help counsel families in the cre-
giftedness in these children, schools offer them enriched programs.
ation of a supportive environment for their children and can advocate
Intellectual giftedness in children has been associated with the
on behalf of children and families within the educational system.
social, economic, and educational background of their families.
Giftedness has been defined in several ways. The psychometric
Factors that correlate with higher IQ scores in children are more
definition of giftedness is based on scores obtained on standard-
years of parental education, higher IQ scores of mothers, increased
ized intelligence tests. The 2 most frequently used cutoff points
family income, smaller family size, and longer intervals between sib-
are 2 standard deviations above the mean (intelligence quotient
lings. Children from low-income and minority backgrounds are less
[IQ] of 130–135) and 3 standard deviations above the mean (IQ
likely to be identified as gifted than other children. Black, Hispanic,
of 145–150). Children with these scores are in the upper 2% and
and Native American students are underrepresented by 30% to 70%
0.1% of the IQ distribution, respectively. Intelligence quotient,
in gifted programs in the United States. In particular, when sin-
which is considered to be fairly stable after 3 or 4 years of age, is the
gle intelligence testing is used to identify gifted children, students
best single predictor of scholastic achievement at all levels, from
from diverse cultural backgrounds are more likely to be excluded
elementary school through college.
from programming opportunities. Some studies have also found
A second definition of giftedness is based on real-life achieve-
that teacher referral–based systems may be more likely to under­
ments or performance of exceptional skills rather than test scores.
identify students from diverse backgrounds if most of the teachers
Children with special talents (other than general intelligence) in
have cultural or ethnic discordance with the student.
areas such as music, mathematics, ice skating, chess, or drama fit
Although research indicates that, as a group, children who are
this description. Other definitions of giftedness recognize motiva-
academically gifted begin to walk, verbalize, and read earlier, this
tion, commitment, perseverance, high self-esteem, and creativity as
area of research is still in its infancy, and conflicting results have
personality traits that allow children with above-average ability to
been reported. Thus, these factors are not useful for predicting gift-
develop exceptional talents. This collective group of traits is often
edness in individual children.
referred to as the “potential to achieve” academic or other success.

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 236 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Evaluation challenged to think and work hard, and develop a pattern of under-
achievement that may be difficult to reverse by the middle grades.
One of the primary goals of child health promotion is developmen-
tal monitoring. The early identification of developmental delays, Special Groups of Gifted Children
which allows for prompt intervention, is among the primary Giftedness is harder to identify in some children. In the child with
purposes of such monitoring. Techniques for developmental assess- physical disability, giftedness is often obscured by the obvious
ment include review of developmental milestones with parents and physical disability, which demands attention. Such children may
discussion of parental concerns, informal observation of children participate in special programs in which their physical needs are
in the office, and formal screening with standardized tests, such as the major concern, to the detriment of their academic or artistic
the Ages and Stages Questionnaires. potential. In addition, poor self-esteem associated with the disabil-
Identification of Gifted Children ity may prevent these children from realizing their potential. To
identify giftedness in the child with physical disability, parents and
Although parents’ first concern is usually to confirm that their child
teachers must make a concerted effort to search for potential and
is developing normally, it is not uncommon for parents to ask if their
encourage its development. Strengthening a child’s capacities may
child is gifted. Such questions are typically motivated by parents’
involve training in the use of a wheelchair or computer or taking
desire to optimally encourage their child’s development. Sharing
frequent breaks to prevent fatigue.
information and observations with parents during developmental
Giftedness also commonly goes unnoticed in children with learn-
monitoring may facilitate parent-child interaction and child develop-
ing disabilities. Exceptional and poor abilities can coexist in a child. In
ment. In a competitive society, the pediatrician should look for signs
fact, an estimated 10% of gifted children have a reading problem, read-
that above-average abilities are the result of undue pressures placed
ing 2 or more years below grade level. Albert Einstein, Auguste Rodin,
on children, such as incessant teaching or overscheduling of time.
and John D. Rockefeller are famous examples of brilliant individuals
Infancy and early childhood may not be the best time to deter-
who had challenges with reading and writing. An extreme example
mine whether a child is gifted. Age of attainment of developmental
of the occurrence of extraordinary and deficient abilities together in
milestones and performance on standardized tests (eg, Bayley Scales
1 individual is the child savant. Affected children possess amazing
of Infant and Toddler Development) during the first 2 years after
abilities in 1 area (eg, music, drawing, mathematics, memory), but
birth are unreliable predictors of intellectual giftedness. Reasons
they exhibit delays in other respects. In addition, they have behavioral
for this lack of reliability may include weaknesses in the tests
problems that resemble autism, such as repetitive behavior, little use
and variable rates of child development that result in transient
of language, and social withdrawal. Learning disabilities may obscure
precocity or delay. Tests that focus on visual memory tasks in
children’s talents, thus preventing fulfillment of their potential.
infants may be better predictors of later academic intelligence,
Conversely, children’s giftedness may mask their weaknesses,
although additional research is required on the efficacy of such tests.
depriving them of needed help. Worst of all, gifted children with
Additionally, many special talents that comprise giftedness, such
learning disabilities may manage to barely “get by” in the regular
as creativity or artistic or musical ability, may not manifest until
classroom setting and fail to receive recognition for strengths or
children are older.
weaknesses. Large differences on intelligence and achievement tests
The determination of giftedness in older children may involve
between scores in different areas, such as language and spatial abili-
several factors (Box 35.1). The early identification of giftedness
ties, may indicate both giftedness and a learning disability. Research
allows for the development of an appropriate educational program
suggests that programs that focus on strengths, not deficits, enhance
that is optimally matched to a particular child’s ability to learn.
self-esteem in gifted children with learning disabilities and can be
Without early identification and intervention, children who are intel-
extremely beneficial in their academic development.
lectually gifted may become disillusioned with school, lose inter-
The identification of giftedness is also difficult in children
est in learning, fail to develop study skills because they are never
who underachieve. Parents may approach the pediatrician with
the following frustrating problem: Their child is doing poorly at
school, although they believe that the child is bright because of the
Box 35.1. Factors Used in the Identification child’s abilities and participation in advanced activities at home.
of Giftedness in Children Underachievement may result from a learning disability; poor self-
ww Intelligence tests esteem; lack of motivation; or the absence of rewards, at home or at
ww Standardized achievement tests school, for succeeding in academics.
ww Grades As previously stated, giftedness is less likely to be recognized in
ww Classroom observations children from families of low income or who are ethnically diverse.
ww Parent and teacher rating scales For school districts that request privately obtained testing for entry
ww Evaluation of creative work in a specific field (eg, poems, drawings, into advanced educational programming, the costs may be prohibi-
science projects) tive for some families. Additionally, many of the tests used to iden-
tify giftedness have been “normed” on white, English-speaking,

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 Chapter 35: Gifted Children 237

middle-class children. Furthermore, studies have shown that rely- Parents should be encouraged to treat children who are gifted the
ing on referrals from teachers who have not had additional train- same way they do their other children. For example, age-appropriate
ing on recognizing giftedness are likely to result in students who do responsibilities and chores should be encouraged. Siblings of gifted
not match typical cultural perceptions of the “gifted child” being over- children may become resentful if attention is centered on their gifted
looked. Students who are learning English as a second language are sibling. They may feel inferior, particularly if gifted children surpass
even more likely to be overlooked for placement into advanced edu- them in school. Tensions may be magnified if gifted children become
cational programming. For new immigrant families, there may be friends with their older siblings’ friends. To preserve a sense of self-
different sociocultural expectations of behavior in school, cross- worth and competence in siblings, the pediatrician should recom-
cultural stress, or symptoms such as posttraumatic stress or depression mend to parents that they set aside special time to spend with each
that may mask giftedness or academic potential. Validation of newer of their children. Parents should encourage other talents (eg, musical
methods of assessment that are less fraught with cultural and socioeco- abilities, athletic abilities) in siblings. Older siblings should receive
nomic bias is ongoing. Until validated tests are available that are sen- the special privileges and responsibilities that come with age, such
sitive to socioeconomic differences, a combination of other means of as staying up later or doing different chores. Any tensions within the
identifying giftedness should be stressed, such as assessment of cre- family should be openly discussed and addressed.
ative work and teacher, student, and perhaps community nominations.
At School
Parents often seek advice from their pediatrician about educational
Management
planning for children who are gifted. A learning environment with
At Home the optimal degree of challenge—hard enough to require new learn-
Loving, responsive, stimulating parenting should be encouraged ing and stave off boredom, but not so hard as to be discouraging—
for all children, including those who are gifted. Parents of gifted is the goal for all children. Parents of young children should select a
children may feel inadequate, fearing that their child is smarter preschool with a flexible program and capable teachers to accommo-
than they are. The pediatrician can provide parental reassurance by date children with precocious skills. Parents of school-age children
telling parents, “You must have been doing something right for your must decide whether acceleration (ie, starting school earlier or skip-
child to have been identified as gifted.” Children’s librarians, period- ping grades) or enrichment (ie, staying in the same grade but supple-
icals written for parents and teachers of gifted children (eg, Gifted menting the regular curriculum) is more appropriate (Table 35.1). The
Child Today), and the local chapter of the National Association for choice of approach is dependent on the particular child.
Gifted Children are good resources for parents.
Parents are often overwhelmed with complex questions from
their precocious preschool-age children about issues ranging from Table 35.1. Acceleration Versus
homelessness and world hunger to theology and the creation of the Enrichment in Gifted Education
universe. The pediatrician should tell parents that they should not Strategy Advantages Disadvantages
be afraid to admit that they do not know all the answers and should
Acceleration May provide suitable Difficult to reverse
work together with their child to find the answers.
academic challenge May have to skip more than 1
The pediatrician may need to warn parents about putting too much
pressure on their gifted children. For example, enrolling children in May have social benefits grade to be properly challenged
multiple classes often leaves little free time for unstructured play. Play Can be offered by all
affords many opportunities for self-learning, interaction with peers, schools
and development of creativity and initiative. Parents of infants, tod- Inexpensive
dlers, and preschoolers should be encouraged to take cues from their Enrichment Classmates are the same May be expensive and inaccessi-
children. If children have a rich environment with plenty of objects and age ble to many families because of
books to explore, diverse experiences, and stimulating interactions, May expose children to tuition and transportation costs
they will develop their own interests. Other educational materials and subjects they would not Inadequate for children who are
special instruction can then be provided in a particular area of interest. otherwise learn highly gifted
Children who are gifted are often mistakenly considered to fit the
Appropriate for children May isolate gifted from non-
stereotype of troubled, socially awkward “nerds.” With the exception
who are mildly gifted gifted children and encourage
of children at the genius extreme (IQ >180), gifted children are gen-
“elite” label
erally more sociable, well-liked, trustworthy, and emotionally stable
than their peers, with lower rates of mental illness and delinquency. Potential for excessive homework
Nevertheless, they have the same emotional needs as other children. if children are required to make
Gifted children may prefer to play with older children whose inter- up the regular class work as well
ests and abilities are closer to their own. This should be allowed as when they miss class to participate
long as these relationships are healthy. in an enrichment program

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 238 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Parents and teachers of gifted children are usually concerned that Homeschooling, either part-time to complement classroom cur-
children in accelerated programs may have problems with social ricula or full-time, is an educational alternative. To decide if this is
adjustment if their classmates are older. Existing evidence suggests, the best choice for the family and the child who is gifted, parents
however, that children who are gifted benefit socially from acceler- who are considering homeschooling are advised to gather as much
ation. Gifted children in accelerated programs participate in school information as possible, including talking to other parents who are
activities (except contact sports) more often than gifted children homeschooling their children, reviewing sample curricular mate-
placed with classmates of the same age. Even when children who rials, and contacting school districts and state departments about
are gifted are placed with children their own age, they tend to make requirements and specific steps.
friends with older children with whom they share more interests. Schools with students from low-income or minority backgrounds
Gifted children also make up any curricular content missed by grade tend to use their limited resources to help students who are doing
skipping. Because the process may be difficult to reverse, accelera- poorly in school, rather than gifted students. In addition, children
tion may not be the best option if doubt exists whether doing so is from low-income or minority backgrounds may experience further
in the child’s best interest. barriers to academic achievement, including lack of informational
Enrichment involves keeping gifted children with same-age class- materials about gifted programs in parents’ native languages and
mates but supplementing the regular curriculum. Regular class difficulty meeting requirements because of parental employment
placement with a teacher who is willing to offer extra work (eg, responsibilities and lack of transportation. Research has shown that
special projects) in addition to grade-level assignments, part-time programs for gifted children from such backgrounds benefit all stu-
programs to supplement regular class work (eg, field trips, foreign dents by creating positive role models and promoting the school
language classes), honors classes that group bright children together as a place for the cultivation of excellence. In addition to providing
for their basic curriculum, and independent study by the family at support for individual gifted children and their families, the pedia-
home are all examples of enrichment programs. These programs may trician may choose to engage in an active advocacy role within the
work well for some children who are gifted, depending in large part community by, for example, promoting diverse educational experi-
on the resources and funding available and the experience, creativ- ences for students of all backgrounds, helping policy makers discover
ity, and enthusiasm of the teachers involved. and remove barriers to participation, and working with commu-
Some enrichment programs may isolate gifted from nongifted nity organizations to educate families from minority or low-income
children, however, and encourage labeling of the gifted students as backgrounds about educational opportunities for their children who
“elite.” If children are required to make up the regular class work that are gifted.
they miss when they are involved in the enrichment program, they
may find themselves overloaded with homework. Prognosis
Often, a combination of acceleration and enrichment programs Children who are gifted are a diverse group, comprising children
is the best option. Acceleration or enrichment alone may be inade- with exceptional academic, artistic, or other abilities, combined with
quate for the brightest children. Acceleration may be insufficient for the creativity and commitment to achieve their potential. Children
markedly advanced children who would have to skip 2 or 3 grades may be gifted in 1 area and average or even below average in another
to be appropriately challenged. The pediatrician should recommend area. The purpose of identifying gifted children is to provide them
that parents work closely with teachers to achieve the best learning with an educational environment to help maximize their potential.
environment for their children. Some factors that should be con- The pediatrician is poised to help children and their families rec-
sidered are age, physical size, motor coordination, emotional matu- ognize that other factors in addition to cognition, including social
rity, personality, and particular areas and degrees of giftedness of development, concepts of self-worth, self-discipline, and resilience,
the child. Acceleration may be a better option for a physically large, are also vitally important to the overall success and well-being of the
outgoing child than for a small one. Gifted children should be asked child. Pediatricians can serve as a resource for parents in raising chil-
what they would like to do. dren who are gifted and helping children and families obtain appro-
When evaluating the suitability of an educational situation for priate evaluation, educational programs, and supportive resources.
their gifted child, parents should watch for certain warning signs. The prognosis for gifted children is excellent.
Excessive homework should not be expected or tolerated, because it
cuts into the child’s time to play and develop socially. The emphasis in
gifted education should be on broadening perspectives, not increas-
ing busywork. If children who are gifted are developing a sense of
CASE RESOLUTION
elitism or peer animosity, the nature and philosophy of the program
The physician should reaffirm the parents’ observations that their child is gifted.
should be questioned. Boredom with schoolwork, not needing to The parents should be encouraged to explore programs in which their daughter’s
study, signs of depression, or symptoms suggestive of school phobia, talents may be fostered, but they also should be advised that even gifted children
such as recurrent abdominal pain or headaches on school mornings, need time for play and unstructured activities.
should prompt investigation into the suitability of the child’s program.

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 Chapter 35: Gifted Children 239

Pfeiffer SI. The gifted: clinical challenges for child psychiatry. J Am Acad
Selected References
Child Adolesc Psychiatry. 2009;48(8):787–790 PMID: 19628996 https://doi.
Davidson Institute. www.davidsongifted.org/Search-Database. Accessed March org/10.1097/CHI.0b013e3181aa039d
14, 2019 Plucker JA, Callahan CM, eds. Critical Issues and Practices in Gifted Education:
Intagliata VJ, Scharf RJ. The gifted child. Pediatr Rev. 2017;38(12):575–577 What the Research Says. 2nd ed. Waco, TX: Prufrock Press; 2014
PMID: 29196518 https://doi.org/10.1542/pir.2017-0088 Ramos E. Let us in: Latino underrepresentation in gifted and talented programs.
Liu YH, Lien J, Kafka T, Stein MT. Discovering gifted children in pediatric prac- J Cult Divers. 2010;17(4):151–153 PMID: 22303650
tice. J Dev Behav Pediatr. 2005;26(5):366–369 PMID: 16222177 https://doi. Shaunessy E, Karnes FA, Cobb Y. Assessing potentially gifted students from
org/10.1097/00004703-200510000-00005 lower socioeconomic status with nonverbal measures of intelligence. Percept
Morawska A, Sanders MR. Parenting gifted and talented children: what are Mot Skills. 2004;98(3 Pt 2):1129–1138 PMID: 15291199 https://doi.org/10.2466/
the key child behaviour and parenting issues? Aust N Z J Psychiatry. 2008;42(9): pms.98.3c.1129-1138
819–827 PMID: 18696287 https://doi.org/10.1080/00048670802277271 University of Connecticut Renzulli Center for Creativity, Gifted Education, and
National Association for Gifted Children. www.nagc.org. Accessed March 14, Talent Development. www.gifted.uconn.edu. Accessed March 14, 2019
2019
Olszewski-Kubilius P, Clarenbach J. Unlocking Emergent Talent: Supporting High
Achievement of Low-Income, High-Ability Students. Washington, DC: National
Association for Gifted Children; 2012

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 CHAPTER 36

Children and School:

A Primer for the Practitioner
Geeta Grover, MD, FAAP, and Jeanne Anne Carriere, PhD

CASE STUDY
An 8-year-old boy is brought in by his parents in early Examination reveals a well-developed and well-
April because his third-grade teacher informed them that nourished boy whose growth parameters are within nor-
he is currently failing in school and may not be promoted mal limits for his age. He appears somewhat anxious in
to the fourth grade. Review of his medical, developmen- the examination room, and when asked about school he
tal, and school histories reveals that he was a colicky infant tells you that he feels he is just not as smart as the other
and continued to be difficult as a toddler. His language children in his class.
skills were somewhat delayed, although not enough to
warrant a full evaluation. His preschool teacher felt that
Questions
1. Should grade retention be considered when a child
he was easily distracted when doing seat work. In kin-
is failing in school?
dergarten, he had some difficulty learning all his letters,
2. What are the potential disadvantages of grade
numbers, and sounds. Early reading was difficult in kin-
retention?
dergarten and first grade but improved by the end of the
3. What are factors to consider when evaluating a child
first-grade year. Second grade was fairly good, except for
for school failure?
continued concerns about inattention and distractibility.
4. What steps should be taken at this time by the par-
By third grade he was struggling more, especially with
ents and the school for the boy in this case study?
writing, and not performing within grade level in several
5. How could early intervention have affected the boy’s
areas. He also continued to be inattentive and distractible
performance?
in his classroom.

Pediatricians are the medical practitioners most knowledgeable of disengagement and school failure. It is imperative that pediatri-
typical and atypical child development. Their routine contact with cians have an understanding of the multiple facets, evaluation, and
young children and their families, as well as their longitudinal management of children’s school readiness needs and the ways in
perspective on their patients’ lives, places pediatricians in a unique which delays or deficits in academic, cognitive, physical, behavioral,
position to evaluate, diagnose, and manage not only children’s medi- and social-emotional development can affect a child’s school engage-
cal needs but also their developmental, social-emotional, behavioral, ment and long-term success. The evaluation and management of
and educational needs. Research has highlighted the importance of educational difficulties requires a multidisciplinary approach; how-
early experiences to optimize development and supported early inter- ever, the pediatrician should have a principal role in monitoring the
vention for children with developmental delays. Traditionally, the critical elements supporting school readiness and providing ongo-
5-year-old health maintenance visit has been regarded as the “school ing guidance, support, and advocacy for patients and their families.
readiness” visit. However, waiting until this visit to address concerns
or provide preventive guidance for educational readiness is too late. Epidemiology
School readiness and the academic, behavioral, and social-emotional Attempting to establish a set of determinants that result in success-
development it entails must be promoted from infancy through early ful learning or that place a child at risk for failure is an oversimpli-
childhood. fication of the complexities of school readiness, school engagement,
During the school-age years, pediatricians should continue to and school failure. School readiness, school engagement, and school
monitor children’s educational progression by inquiring about aca- failure are nonlinear cumulative processes, not solitary events, and
demic, social-emotional, and behavioral development. Parents often a multidisciplinary approach to assessment and management is
turn to pediatricians for advice on their children’s behavioral or required.
academic difficulties at school. Early academic, behavioral, and School readiness is the term used to describe those qualities and
social-emotional difficulties place children at risk for school traits that are considered prerequisites for a child to be ready for
241

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 242 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

school success. When defining school readiness, parents tend to Overall, high school completion rates in the United States have
focus on the pre-academic skills their child has mastered (eg, iden- been slowing rising over the last decade, with 83% of all students
tifying letters and sounds, counting, writing their name). Teachers’ graduating from high school. Graduation rates vary by state, how-
definitions of readiness regularly incorporate social skills and behav- ever, and are lower for children from low-income families and for
ior as well. Factors identified in the literature as affecting school children with disabilities. The overall rate of high school completion
readiness include physical health, motor skill development, social is 76% for low-income students, with some states reporting rates as
and emotional development, language development, adaptive skills, low as 63%. The high school completion rates for children with dis-
and cognitive abilities. In addition, significant additional factors abilities are even more concerning. In the United States, approxi-
exist that readily affect school adjustment and success other than mately 64% of students with disabilities graduate from high school,
a child’s skills and attributes, such as parent-child interactions, with rates as low as 29% for certain states.
access to quality early childhood education, and both positive and Approximately 15% of children in the United States have a devel-
negative life experiences (see Chapters 141 and 142). opmental disability. In the US public school population, 13% of
Language deficiencies and problems with emotional maturity children receive special education services under the Individuals
are cited most often as the factors that most restrict school readi- with Disabilities Education Act (IDEA) of 2004. These services are
ness. Language development and school readiness are intertwined. provided to students with qualifying disabilities, if their disability
Language proficiency provides a strong foundation for the cognitive affects their academic achievement or educational performance. Not
and literacy skills required for school achievement. By providing a all children with a diagnosed disability qualify for or require special
rich language environment from infancy, parents give children a head education support (Box 36.1).
start on school success. Currently, however, more than 1 in 3 American The number of children with diagnosed disabilities is signifi-
children start kindergarten without the language skills necessary to cantly lower than the number of children who experience some level
learn to read. Similarly, with regard to emotional maturity, a recent of adverse environmental, socioeconomic, or stress-inducing condi-
study found that students who entered kindergarten lagging their tions that negatively affect their ability not only to get to school each
peers in social-emotional skills were more likely to experience grade day but be ready to learn on arrival (see Chapters 141 and 142). These
retention, receive special education services, and be suspended or children experience poor educational outcomes when they do not
expelled at least once by the fourth grade. School engagement can be receive comprehensive support, intervention, and services. Youth
broadly defined as meaningful student involvement throughout the who interface with the juvenile justice system have previously expe-
learning environment. Research has shown that school engagement rienced increased rates of academic failure, disengagement from
is associated with positive outcomes, including academic achievement
and persistence, that is, staying in school until graduation. Within
the school research literature, school engagement has 3 components: Box 36.1. Special Education Eligibility Categories
behavioral, emotional, and cognitive. Behavioral engagement is related Under the Individuals with Disabilities Education
to active participation, both in the classroom and the school commu- Act of 2004 for Children and Youth Age 3
nity as a whole. It includes following classroom norms; demonstrating Through 21 Yearsa
good conduct; and being involved in academic, social, and/or extra-
curricular activities. Emotional engagement refers to students’ emo- ww Autism
tional reactions to teachers, peers, academics, and the school as a ww Deafness
whole. Emotional engagement creates a sense of belonging and value ww Emotional disturbance
to the school community. Cognitive engagement is related to students’ ww Deaf-Blindness
investment in learning. It encompasses the problem-solving flexibil- ww Hearing impairment
ity and coping skills students use as well as the hard work they do ww Intellectual disability
to understand and master the curriculum presented to them. ww Multiple disabilities
School engagement is considered crucial to achieving positive ww Other health impairment
academic outcomes and protecting students from school failure. ww Orthopedic impairment
School failure is a multifaceted, epidemiologically complex issue. ww Specific learning disability
Research suggests that health and educational success are intricately ww Speech or language impairment
connected. Compared with non-affected children, children with ww Traumatic brain injury
physical illnesses, mental health concerns, socio-emotional con- ww Visual impairment, including blindness
cerns, behavioral issues, and neurologic deficits are more likely to ww Developmental delaysb
have difficulty learning throughout their school careers. They are
a
To fully meet the definition and eligibility for special education and related services as a “child with
at increased risk of poor attendance, poor achievement, academic
a disability,” a child’s educational performance must be adversely affected as the result of 1 of the
decline, and failure to graduate from high school. Dropping out of 14 categories listed here.
school, which is commonly seen as an event, is in fact a process that b
The Individuals with Disabilities Education Act of 2004 allows each state to determine whether to
often begins with early school failure. use this eligibility category for student age 3 through 9 years.

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 Chapter 36: Children and School: A Primer for the Practitioner 243

school, and/or school disciplinary problems. More than half of such lack those skills. As conceptualized by the National Education Goals
students perform academically below grade level. This population Panel, school readiness encompasses 5 dimensions: physical well-
meets eligibility for special education services at 3 to 7 times the rate being and motor development; social and emotional development;
of their nonincarcerated peers. Approximately 85% of incarcerated approaches to learning; language development (including early
juveniles are functionally illiterate (ie, lacking the literacy skills to literacy); and cognition and general knowledge.
manage daily living and employment tasks that require reading) or Lack of specificity of children’s presenting signs and symptoms
low literate. High school dropouts are 3.5 times more likely than and of parental concerns make it challenging to determine a specific
high school graduates to be arrested in their lifetime and 63% more etiology for school failure. For example, parental concern about a
likely to be incarcerated than their peers with 4-year college degrees. child’s inability to focus may be suggestive of an attention disorder,
Research has not supported retention as an effective remediation a learning disability, a mood disorder, or perhaps all 3. Parental con-
strategy for poor school performance, and many studies have linked cerns can be categorized into 3 broad areas: learning (eg, learning
retention to future school failure. Grade retention rates in the United disability; problems with higher-order cognition, including intellec-
States have declined in the past decade, and currently, approximately tual disability), attention (eg, ADHD), and emotional/behavioral (eg,
10% of students are retained each school year. Most retentions occur anxiety, depression, serious emotional disturbance). Signs of school
in kindergarten, with retention rates between 1st and 12th grade of difficulties are presented in Box 36.3. It is important to look not only
3% to 5%. However, retention rates as high as almost 9% in these at academic skills but also at other components of the educational
grades have been reported for students of color, students of parents experience, such as social and emotional experiences. Additionally,
without a high school diploma, students whose families receive pub- it is important to ascertain the basis for the perception that a child
lic assistance, and students living in the Southern states. Children is failing. It is necessary to determine whether the problem exists
receiving special education services experience retention at a sig- in the eyes of the student, parent, teachers, or everyone involved.
nificantly higher rate, with 32% being retained at some point in Academic achievement across subjects must be assessed, especially
their school career. in the areas of reading, mathematics, and writing. It is also important
Considering that the average child in the United States spends to evaluate students with good skills who fail to perform satisfacto-
approximately 50% of the waking day in a school or a similar learning rily in the areas of writing, planning, organization, project comple-
situation for approximately 12 or 13 years, it follows that a lack of suc- tion, test taking, or classroom participation. Difficulty with academic
cess in these settings will lead to difficulties for much, or all, of adult performance may result in school failure despite satisfactory aca-
life. School difficulties that go undetected, untreated, and under- demic skills. In addition to academic skills and performance, the
treated can result in establishing a lifelong pattern of frustration and development of good social skills and peer relations is equally impor-
failure. For example, children with attention-deficit/hyperactivity tant. Some students have difficulties “fitting in,” which results in a
disorder (ADHD) are 2 to 3 times more likely to drop out of disappointing educational experience despite academic excellence.
high school than their peers without ADHD, and those who attend
college are less likely to graduate than their peers without ADHD
(see Chapter 133). Pathophysiology
Developmentally, support exists for promoting school readiness from
a young age. Research highlights the effect of nurturing relationships
Clinical Presentation and positive experiences on early brain development. Strong neural
Defining school readiness is not an easy task, because the intellec- connections are created and modified by positive reciprocal interac-
tual, physical, social, and emotional development among children tions, creating a solid foundation for learning. Conversely, adverse
of kindergarten age varies tremendously. To confound the concept environments can have harmful effects on healthy brain development.
of readiness, kindergarten expectations and standards have changed The developing brain continues to make new synaptic con-
significantly in the past 2 decades, becoming less socially play based nections and discard underused connections from birth to
and more academically focused. School readiness involves far more approximately age 5 years, well before formal schooling begins.
than adequate pre-academic skills (Box 36.2). Early childhood edu- For example, it is known that children who grow up without being
cators also emphasize the importance of sufficient physical, cogni- read to and with little exposure to books or printed language dur-
tive, language, social-emotional, and behavioral skills to children’s ing their first 5 years are at increased risk for developing reading
success in the formal schooling environment. Current research failure and subsequent school failure (see Chapter 34). School
emphasizes the importance of children’s “learning to learn behav- readiness must be promoted from infancy throughout early child-
iors,” highlighting the role that abilities such as sustained attention, hood; waiting for the 4- to 5-year-old well visit to address school
engaging in goal-directed tasks, impulse control, and emotional reg- readiness is too late. Most children with learning disorders experi-
ulation have on children’s engagement in learning activities and aca- ence language, motor skills, and emotional or behavioral problems
demic achievement. Children who can control impulses, consider well before they encounter difficulties in the classroom. These def-
options, and demonstrate flexible thinking and creativity are better icits are noted by parents an average of 3 years before the disability
able to actively engage in learning opportunities than their peers who is formally identified.

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 244 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Box 36.2. Questions Related to General School Readiness and The Five Domains of School Readiness

General Readiness Questions

ww Have they had any preschool or group child care experience, and how did they respond in these settings?
ww Were any behavioral, developmental, or emotional concerns raised during those group care experiences?
ww Have they been screened for developmental delays, and have any delays been addressed?
Self-regulation and Social-emotional Readinessa
ww Are they able to both express and control their thoughts, feelings, and emotions?
ww Are they able to understand that others have thoughts, feelings, and emotions that are different from their own, and can they express empathy or compassion?
ww Can they take turns, share, and cooperate with others?
ww Can they share and play with other children?
ww Do they play well with age-appropriate peers, or do they seem to consistently prefer to play with younger or older children or adults?
ww Can they self-soothe or are they easily calmed down when they are upset or frustrated?
ww Are they able to separate from parents for several hours?
Physical Health and Motor Readinessb
ww Do they come to school physically ready to learn (eg, well nourished, adequate sleep)?
ww Are their vaccines current?
ww Have vision and hearing been evaluated?
ww Do they demonstrate developmentally appropriate fine motor skills (eg, can they manipulate a crayon/pencil with a correct grasp, turn pages 1 at a time in a
book, or print some letters and numbers)?
ww Do they demonstrate developmentally appropriate gross motor skills, such as balance on 1 foot, hop, or skip?
Language and Communication Readinessc
ww Are articulation errors developmentally appropriate?
ww Is speech understandable to strangers?
ww Can they hold a back-and-forth conversation about everyday topics of interest?
ww Do they use correct sentence structure and speak in complete sentences?
ww Do they use appropriate tenses/pronouns?
ww Can children use language to express their thoughts and feelings or to follow simple oral instructions?
ww Do they use age-appropriate vocabulary?
ww Do they have beginning literacy skills, such as basic book concepts, print awareness, and story sense?
Cognitive Readinessd
ww Do they have general knowledge, such as reciting the alphabet and rote counting, colors, days of the week, letters and numbers names, and basic shapes?
ww Do they have more complex understanding, such as letter-sound associations, spatial relations, number concepts, and 1-to-1 correspondence?
ww Are they able to solve problems, follow the logic in a story, think, and make decisions?
Approaches to Learninge
ww Are they able to focus and sustain attention for a developmentally appropriate amount of time?
ww Are they able to work in a group, with a partner, and independently?
ww Are they able to control their emotions and persist at challenging tasks?
ww Do they demonstrate curiosity and enthusiasm for learning?
ww Can they tend to basic needs independently, such as toileting, washing hands, and asking for help?
a
This domain focuses on children’s social and emotional development, including their interpersonal and intrapersonal skills.
b
This domain covers such factors as health status, growth, and disabilities.
c
This domain focuses on expressive and receptive language skills and literacy development.
d
This domain includes general knowledge as well as gaining knowledge by making connections with objects, events, or people for similarities, differences, and associations. It also includes knowledge about
societal conventions.
e
This domain refers to the ability to use skills to actively engage in learning.
Adapted with permission from National Education Goals Panel. Ready schools. Washington, DC: U.S. Government Printing Office; 1998

Another critical period in brain development occurs during higher-order cognitive tasks that enable attention, self-regulation,
adolescence, when the brain again undergoes a process of synaptic planning, organization, and completion of goal-oriented tasks, all
pruning and myelination that is especially notable in the prefron- of which are necessary to effectively engage in learning.
tal lobe, the area responsible for the executive function skills of rea- Interventions designed to improve children’s school connected-
soning, impulse control, attention, and planning. These skills are ness and prevent later academic problems are most effective when

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 Chapter 36: Children and School: A Primer for the Practitioner 245

a multitiered system of support (MTSS) is often needed to improve

Box 36.3. Factors Related to School Difficulties
student outcomes. School engagement is adaptable and is a function
That May Result in School Failurea
of not only the child but the context, that is, the school environment.
Intrinsic This implies that behavioral, emotional, and cognitive engagement
ww Not able to follow directions, pay attention, or finish a task can be responsive to prevention and intervention.
ww Not able to carry thoughts or ideas to paper School failure has a variety of causes. Both intrinsic (ie, child-
ww Not able to read, write, or spell appropriate to age and educational level related) and extrinsic (ie, environmental-related) causes relating to
ww Requires excessive time to complete homework/excessive parental the home or school environment must be considered (Box 36.3). In
involvement with homework most cases, school failure is the result of a complex interaction between
ww Previously tested but not eligible for special education (eg, child may be a child, family, and school-related variables rather than the result of a
“slow learner”) single factor (eg, ADHD). When evaluating for the etiology of learning
ww Hates school/psychosomatic symptoms disorders, medical problems should always be considered. Neurologic,
ww Has few, if any, friends or change of friends psychological, social-emotional, and behavioral disorders based in
ww Sudden change in behavior
the central nervous system may also be responsible for school failure.
ww Low cognitive skills (ie, intellect)
Unrecognized disorders may be causes of school failure. For example,
ww Specific learning disabilities
children with ADHD often have significant academic underachieve-
ww Attention-deficit/hyperactivity disorder
ment, poor academic performance, and poor educational outcomes.
ww Speech and language disorders
Learning disorders are a broad group of neurodevelopmental
ww Mood and anxiety disorders
ww Low self-esteem, self-concept, and self-determination disorders affecting children’s ability to learn, such as speech and
ww Social-emotional difficulties language impairments, intellectual disability, specific learning dis-
ww Neurodevelopmental delays abilities, and ADHD. Learning disorders are among the most fre-
ww Motor coordination disorders quently diagnosed developmental disorders in childhood.
ww Chronic or serious medical illness (eg, seizure disorder, cystic fibrosis, asthma) Learning disabilities comprise a subset of learning disorders and
ww Vision, hearing, or speech difficulties are neurologically based processing problems that interfere with
ww Poor nutrition learning basic skills, such as reading, writing, and/or math, as well
ww Sleep problems as more complex skills, such as organization, planning, abstract rea-
ww Substance use/abuse soning, short- and long-term memory, and attention. Learning dis-
ww Genetic history (eg, family history of school problems) abilities are the most common learning disorder. Approximately 10%
Extrinsic to 15% of children have learning disabilities, and reading disabili-
ww Serious psychosocial concerns (eg, parental depression, history of abuse ties are the most common type. Children with learning disabilities
or neglect) are the largest group of students receiving special education services
ww Disruption in the family (eg, many moves, divorce, death) in schools; these disabilities vary in degree, nature, and complexity.
ww Poor school readiness/absence of enrichment prior to school entry (eg, A formal definition of specific learning disability was first outlined
early literacy exposure) in federal law in 1975 as part of the Education for All Handicapped
ww Parental or school expectations not commensurate with child’s abilities Children Act, now the IDEA. A specific learning disability is “a disorder
ww School and/or classroom placement (poor “fit” or poor instruction) in one or more of the basic psychological processes involved in under-
ww Grade retention standing or in using language, spoken or written, that may manifest
ww Poor attendance, missed instruction itself in the imperfect ability to listen, think, speak, read, write, spell,
ww Multiple school changes or to do mathematical calculations.” The IDEA of 2004 expanded edu-
ww Language/cultural differences cators’ options for determining whether a child has a learning disabil-
ww Adverse childhood experiences ity by removing the requirement for use of a discrepancy model that
a
School failure is not a single event or a linear process. This list provides examples of factors within
identifies a learning disability as a significant difference between a
individual children and their environments, whether home or school, that could place them at child’s intellectual ability (ie, intelligence quotient [IQ]) and academic
increased risk for school difficulties and failure. achievement. That now-removed requirement was often called the
“waiting to fail” model, because parents and schools had to wait until
they are provided during the preschool years, before a child enters individual children’s achievement fell significantly below their ability
formal K-12 schooling. School engagement stems from learning theo- level before a learning disability could be identified. Currently, chil-
ries demonstrating that learning improves when students are curious, dren can be identified with a learning disability if they do not exhibit
interested, or energized by their schooling and that learning wors- adequate achievement when provided with age- or grade-appropriate
ens when students are bored, indifferent, or disengaged from their learning experiences; do not make progress sufficient to meet age-
school. Levels of student engagement can be seen on a spectrum from or grade-level standards when provided research-based intervention
deeply engaged to resistant. For students with fluctuating engagement (ie, response to intervention); or do exhibit a pattern of strengths and
or true resistance to participation in school and learning activities, weaknesses in academic performance, achievement, or both, relative

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 246 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

to grade-level standards, or intellectual development. Specific learn- changes and prevention and can make the discussion of challenges
ing disabilities do not include “learning problems that are primarily and intervention more comfortable for parents and children.
the result of visual, hearing, or motor disabilities; intellectual develop- School Readiness
mental disorders; of emotional disturbance; or of environmental, cul-
Historically, pediatricians assessed school readiness during the
tural, or economic disadvantage.”
5-year-old health maintenance visit, but currently it is understood
The criteria of learning disorders are listed in Box 36.4. For a more
that the optimization of development and school readiness should
comprehensive list of diagnostic criteria and coding information, refer
be addressed beginning in infancy. The American Academy of
to Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition
Pediatrics (AAP) has highlighted 5 domains of school readiness,
(DSM-5). Similar to IDEA, DSM-5 removed the discrepancy between IQ
which are consistent with the 5 dimensions of school readiness from
and the affected learning area as a requirement for diagnosis. This change
the National Education Goals Panel. These domains include self-
is based on research showing that children who meet and those who do
regulation and social-emotional readiness, physical health and motor
not meet the IQ-discrepancy criterion do not differ in terms of symptom-
readiness, language and communication readiness, cognitive read-
atology, underlying cognitive deficits, or response to effective intervention.
iness, and approaches to learning (Box 36.2). The physician should
Although it is difficult to do, it is important to differentiate stu-
interview the parent and observe the child to assess the acquisition
dents with neurologically based learning problems from those with
or progression toward these readiness skills. Mastery of these skills is
learning problems caused by other factors. For example, a student’s
not necessary for social and academic competence and should never
disability may predispose the individual to learning problems even
be used as exclusion criteria for a child beginning formal schooling,
in highly accommodating environments, whereas inadequate envi-
because public school kindergarten classrooms and curricula are
ronments can result in significant learning problems even in the
designed to accommodate a diversity of skill levels. Providing this
absence of disability. This differentiation is vital to management or
information to families early in a child’s development can help fam-
intervention success. An inadequate environment or a student dis-
ilies make decisions about early childhood programs and, if neces-
ability are best addressed with evidence-based instruction in the def-
sary, early interventions. More than 50% of children younger than
icit areas; focusing only on addressing the academic deficit will not
5 years regularly attend some type of child care or preschool pro-
alleviate comorbid negative social-emotional, behavioral, or envi-
gram; thus, including questions about the quality and experiences
ronmental conditions. Before evaluating for child-related pathol-
within these settings is an important part of the history.
ogy or underlying dysfunction, such as specific learning disabilities,
cognitive delays, language disorders, or mental health disorders, the School Engagement/School Failure
physician should investigate external situations that may be causing When evaluating for school engagement and/or school failure, the phy-
or exacerbating the problem (eg, underperforming schools, lack of sician should elicit information from parents, teachers, and the children
parental involvement, poor student-teacher relationship, mismatch themselves (Box 36.5). A review of present and past report cards pro-
between a child’s ability/disability and the learning environment). vides information not only on academic progress relative to the grade
level standards but also on the child’s behavioral, social-emotional,
Evaluation and classroom adaptive skills. Teacher behavior rating forms, school
History district and state academic evaluations, and results of any school-based
A thorough history is necessary to help the pediatrician assess a psychoeducational evaluations also should be reviewed.
child’s strengths and challenges and formulate a hypothesis for
diagnosis. Research on prevention, resiliency, and social-emotional
development demonstrates that the presence of assets or strengths is Box 36.5. What to Ask
positively linked with improved outcomes in all domains of devel- School Engagement and/or School Failure
opment. Incorporating educational and learning strengths assess- ww Have the child tell you about experience at school. What classes or sub-
ment in the primary care setting can facilitate discussion of positive jects does the child like or dislike and why? Does the child have friends?
Does the child participate in any extracurricular activities?
ww Have any behavioral, social, emotional, or attentional concerns been
Box 36.4. Criteria for Specific Learning Disorder
raised by the child or the classroom teacher?
ww Difficulties learning and using academic skills: word reading; meaning ww How is the child’s academic achievement? Is the child having problems in
of what is read; spelling; written expression; number sense, facts, or all academic areas, or are particular subjects especially difficult whereas
calculation; mathematical reasoning others are not?
ww Academic skills below expected for chronological age; interferes with ww Do concerns exist about educational performance, such as test taking,
academic/occupational performance/activities of daily living project organization, or homework completion?
ww Learning difficulties begin during school-age years or when academic ww Are there attendance issues? How many days of school has the child missed?
demands exceed the patient’s capacities ww Has any testing (eg, psychoeducational evaluation) been performed by
ww Learning difficulties are not secondary to intellectual, visual, auditory, the school district or privately?
mental, or neurological disorders ww Is the child receiving any accommodations or special services (eg,
intervention group, counseling, speech therapy) at school?
Derived from Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.

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 Chapter 36: Children and School: A Primer for the Practitioner 247

Clinical approaches to early identification of children at risk language, visual-spatial, memory skills) and helps identify areas of
for school failure include risk assessments and developmental sur- strength and weakness. Such an assessment can be performed by a
veillance and screening. Children from economically disadvan- professional such as a developmental and behavioral pediatrician
taged backgrounds are at the highest risk for problems in school. or a psychologist. Psychoeducational assessment is also critical in
Developmental surveillance and screening allow pediatricians to the evaluation of children with school failure. Such an assessment
identify children with developmental, behavioral, and emotional includes an evaluation of cognitive abilities, academic achievement
delays and to provide appropriate guidance, as well as referral to across subjects (eg, reading, writing, mathematics), perceptual pro-
early intervention programs (see Chapter 32). Historical factors asso- cessing strengths and weaknesses, social and emotional functioning,
ciated with an increased risk of school-related problems include and the way in which these areas affect a child’s learning. This can
preterm birth, low birth weight, small for gestational age, and mater- be accomplished through the public school system via the Individu­
nal history of tobacco, alcohol, or illicit substance use during preg- alized Education Program (IEP) process, as mandated by federal law
nancy. Developmental risk factors include delays in the acquisition (ie, IDEA) or privately, by an educational psychologist.
of skills, especially those involving language. Medical factors that
may affect school readiness include lead poisoning, iron deficiency
Management
anemia, and failure to thrive. The presence of chronic medical con-
ditions (eg, asthma, diabetes mellitus, seizure disorders) can also According to the AAP policy statement “The Pediatrician’s Role in
affect school performance directly or indirectly via absenteeism, Optimizing School Readiness,” helping children develop the physical,
medication effects, or self-esteem issues. Environmental risk factors social-emotional, cognitive, adaptive, and language skills needed to
include poverty and lower socioeconomic status, parental mental learn should begin at birth. Pediatricians can create a medical home
health issues, domestic violence, substance abuse, or a family his- in which they provide for children’s physical health while also work-
tory of school problems or learning disabilities. ing with families to address developmental, social, emotional, and
behavioral components of school readiness. Empowering parents
Physical Examination with knowledge can give them the confidence to interact positively
In terms of school readiness, the most important aspect of the with their children’s schools and allow them to effectively advocate
5-year-old health maintenance physical examination includes care- for their children’s educational needs.
ful assessment of hearing, vision, speech, and language. Fine and Often, an important role in management is facilitating open com-
gross motor skills, attention and listening skills, ability to follow munication about a child’s needs. Pediatricians must acknowledge
rules and directions, social skills, and self-help skills (eg, asking for the emotions and fear that often are associated with discussing a
help when needed, independently using the restroom and washing child’s delays or disability and must demystify the process of access-
hands) also should be assessed. ing support, interventions, and treatment. For both parents and chil-
The physical examination has a limited but important role in the dren, the process of demystification can include assurances that all
evaluation of school failure. Signs of short attention span, distract- people have strengths and challenges and that resources and sup-
ibility, overactivity, sadness, or anxiety all should be noted. Special port are available. It is important to use an optimistic tone in such
attention should be paid to head circumference, minor congenital discussions. In addition to addressing the needs of families and chil-
anomalies, abnormal facies, and skin lesions suggestive of neurocu- dren, the physician can also serve an important advisory role for the
taneous disorders (eg, multiple café au lait spots, ash leaf macules). school on the educational implications of chronic medical condi-
The pediatrician should evaluate the child’s vision, hearing, and tions and the potential academic, behavioral, and social-emotional
speech and language and conduct any other appropriate evalua- effect of a child’s disability (eg, side effects of medications, appro-
tions as suggested by the history. priate seizure or allergic reaction responses).
Early intervention is the key to success in children’s school readi-
Neurodevelopmental Assessment ness. Pediatricians are in the most opportune role to help families rec-
School readiness assessment begins with routine pediatric surveil- ognize the importance of early brain and child development and how
lance and screening (see Chapter 32). Several brief school readiness they are affected by a child’s environment and experiences. Teaching
tests have been developed for use by pediatricians (eg, Pediatric parents that their child’s learning begins at birth and occurs in all envi-
Examination of Educational Readiness). However, because the reli- ronments not only can support children in meeting developmental
ability and ability of such tests to detect subtle learning disabilities milestones but can help them successfully acquire the curiosity, emo-
or arrive at more complicated diagnoses has not been established, it tional regulation, and problem-solving skills they will need when they
is difficult to recommend any of them for routine use. If a specific begin school. It is important for pediatricians to promote community
school readiness evaluation is warranted, it is best to make a refer- and home activities to enhance readiness skills, refer families to com-
ral to the school district or an independent specialist (eg, develop- munity resources, and support access to early childhood programs.
mental and behavioral pediatrician, educational psychologist) for a During routine well-child visits, the pediatrician should ask fam-
full evaluation. ilies what child care arrangements they have made for their chil-
Neurodevelopmental assessment can help identify the etiology dren and educate them about the importance of high-quality child
of school failure. Neurodevelopmental assessment surveys a child’s care. To maximize quality early learning experiences, pediatricians
abilities across the different areas of development (ie, fine motor, can provide resources through links such as AAP’s Quality Early

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 248 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Education and Child Care from Birth to Kindergarten. For many at- to school disengagement. Retention is the number 1 predictor of stu-
risk children, early childhood education programs such as Head Start dent attrition. Retained students, regardless of race, socioeconomic
and other preschool compensatory programs may come too late. status, attendance, English language status, or parental involvement,
Early intervention programs for younger children and their fami- are more likely to drop out of school than similarly low-achieving
lies that promote good parenting, language stimulation, and learn- students who were promoted to the next grade level. Many teachers
ing through play are valuable and available in many communities. report suggesting retention to allow a child to “mature” or “give the
Reading aloud to children is among the most important gift of time,” although minimal evidence exists that it is beneficial to
parent-child interactions physicians can promote, and it builds repeat a grade without making changes to the curriculum or envi-
the child’s skills required for eventual success in reading (see ronment, or directly addressing the reason for retention. Educational
Chapter 34). Reading promotes language mastery, which is critical alternatives to retention include directly addressing the reason reten-
to school readiness. tion was considered. For academic delays, extra support can be imple-
Children who are at increased risk for school readiness problems mented in the form of targeted academic interventions, differentiated
because of medical, social-emotional, behavioral, or environmen- instruction, classroom accommodations, and, in some cases, special
tal concerns should be monitored carefully. If these children do not education services. Equally as important as remediating the areas of
appear to demonstrate age-appropriate skills, they should be referred weakness is recognizing the child’s areas of strength (eg, the idea of
for further evaluation. Despite early intervention, some children still using children’s strengths to leverage their weaknesses).
may lack age-appropriate school readiness skills. Options for these The pediatrician can assist families with children who are demon-
children include delayed school entry or enrollment in special educa- strating learning, behavioral, and/or social-emotional difficulties that
tional programs. Delaying school entry may not be the best solution are affecting their academic achievement and performance. Public
if children remain in the same environment that failed to produce school systems, as mandated by federal and state laws, have systems
readiness in the first place. In addition, studies have shown that stu- of support and services available for students through both general
dents who are older than their classmates because of delayed school and special education. Pediatricians’ awareness and understanding of
entry have increased rates of behavior problems, substance abuse, and these systems is an important part of management, because they are
other health risk behaviors in adolescence. Instead, it may be best for often the first professional to be made aware of these delays.
these children to enter school along with their same-age peers and, The Every Student Succeeds Act signed into federal law in 2015
if necessary, receive school-based support to address any difficulties. is a reauthorization of the Elementary and Secondary Education
The management of school failure requires a multidisciplinary Act. The law includes a number of provisions to support success for
approach. Information from the school, including school reports, all students by requiring districts and schools to establish a MTSS
teacher conferences and notes, testing that has been completed, and promoting children’s academic, behavioral and social-emotional
input from other school officials, if appropriate, should be requested. learning regardless of their ethnicity, socioeconomic status, pri-
The primary care physician can assist the child and family in working mary language, family history, strengths, challenges, or disability.
with the school system to obtain appropriate services. The primary The MTSS is a schoolwide, data-driven, prevention-based frame-
care physician may also refer the child to appropriate resources in the work for improving learning outcomes for all students through a lay-
community (eg, educational psychologist, developmental and behav- ered continuum of evidence-based practices and systems. Common
ioral pediatrician). Not infrequently, external factors such as social, school prevention frameworks, such as response to intervention
family, and school-based issues may be difficult to alter. Knowledge and Positive Behavior Interventions & Supports, are imbedded
of the educational laws and community services, as well as ongoing into MTSS. Within this tiered system is the recognition that pro-
developmental surveillance and screening by the primary care phy- viding every student the same level of supports, regardless of the
sician, are necessary to help prevent school failure. quality, will not meet every child’s needs. Levels of support are typ-
Grade retention is often suggested as an intervention for school ically divided by the intensity of the services and the number of
failure. However, disparities exist in rates of grade retention based students they are designed to serve. Tier 1 includes foundational
on sex, race/ethnicity, geographic locale, and socioeconomic cir- universal supports, which are evidenced-based practices that sup-
cumstances. Boys, minorities, children born outside the United port the academic, behavioral, and social-emotional success of all
States, and children from homes led by adults with lower levels of students. Examples of tier 1 supports include training all teachers
education are retained at the highest rates. Although grade reten- in differentiated instruction, implementing a school-wide positive
tion has been a common educational practice in US schools, over- behavioral system, conducting mental health screening of the entire
all retention rates have decreased notably in the past 10 years. student body, and adopting an evidence-based language arts curric-
Nonetheless, parents and school personnel may consider retention ulum. Supplemental and intensified services are designed for stu-
as a viable intervention option for children who are struggling for dents who require more academic, behavioral, or social-emotional
academic, behavioral, or social-emotional reasons. Research does support, with the most intensive level of support targeted to stu-
not support retention as an effective remediation strategy, however. dents with the greatest needs. Examples of Tier 2 support for stu-
Data suggest that grade retention has an adverse effect on most dents who have been identified as having academic, behavioral, or
students. In fact, grade retention can diminish the positive outcomes social-emotional struggles may include homework modifications,
of early intervention programs and is a significant contributing factor small group reading intervention, or participation in a social skills

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 Chapter 36: Children and School: A Primer for the Practitioner 249

group. Students who have been identified as needing the most sup- student with a disability. Related services are developmental, correc-
port may receive the most intensive interventions, such as 1-to-1 tive, and other supportive services that are required to assist a child
counseling or an individualized behavior support plan. Special edu- with a disability to benefit from special education. Examples of related
cation services are typically viewed to be tier 2 service. services include transportation, speech and language therapy, occu-
Students with disabilities have the same right to public education pational therapy, nursing services, and behavioral support services.
as students without disabilities. To receive and benefit from that edu- The IDEA requires public schools to provide free and appropriate
cation, students with disabilities may need special education and/or education in the least restrictive environment for all children with a
related aids and services. Pediatricians can help parents of children qualifying disability. That is, special education is provided at no cost
with neurodevelopmental disabilities (eg, intellectual disability, to parents and includes services designed to meet the individual-
mental health disorders, cerebral palsy, learning disorders, autism ized educational needs of the students so they can access their edu-
spectrum disorder) gain access to these services. Special education, as cational program. Students with disabilities must also be educated
mandated by the IDEA, is not a place to which students with disabil- with students without disabilities to the maximum extent appro-
ities are sent. Rather, special education is a broad group of specially priate. Special education services differ for each child, regardless of
designed instruction, services, and supports that address the unique disability, because services, supports, and accommodations are indi-
educational needs of students age 3 through 21 years who have a dis- vidualized to meet each child’s unique educational needs.
ability. In specially designed instruction, the content, methodology, or The IEP process begins when the parent requests an evaluation
delivery of instruction is adapted to address the unique needs of a for special education services (Figure 36.1). A multidisciplinary

• Parent referrals should

Step 1:
always be in writing.
Referral for an Schools must respond
evaluation is to request letter within
made. 15 calendar days.

Step 2:
An assessment • A variety of school-
based professionals
plan is created may be involved in the
and signed by evaluation.
the parent.

Step 3: • The school district has 60

calendar days to complete
A multidisciplinary the evaluation, prepare the
evaluation is report and meet with
conducted. parents to review.

Step 4: • In some states, steps 4 and 5

The IEP meeting are both completed within 60
is held to review days, although IDEA allows an
evaluation and additional 30 days to complete
decide eligibility. step 5.

Step 5:
An IEP, including • Parents must
approve the IEP
goals, services, by signing,
accommodations, before services
and placement is can begin.
created.

• Parents and schools

Step 6: can request an IEP
The IEP is meeting at any time.
reviewed at School must schedule
least annually. within 30 days of
request.

A child’s special
education
eligibility is re-
evaluated every
3 years.

Figure 36.1. The Individualized Education Program process.

Abbreviations: IDEA, Individuals with Disabilities Education Act of 2004; IEP, Individualized Education Program.

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 250 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

evaluation designed to evaluate for special education eligibility and substantially restricts 1 or more major life activity are eligible for
need is conducted. This evaluation may cover a broad range of areas services under Section 504. Examples of school-based major
depending on the referral concerns, including cognitive ability, aca- life activities may include performing manual tasks, speak-
demic achievement, social-emotional and behavioral functioning, ing, learning, working, thinking, and communicating.
adaptive behavior, language development, motor skills, and sensory Students with disabilities who need only reasonable accom-
processing. Eligibility for IDEA is dependent on meeting criteria for 1 modations or modifications to be educationally success-
of the 14 special education eligibility categories (Box 36.1). The IDEA ful within general education are frequently served under this
and state educational codes provide operational definitions for these law. The definition of disability and the way in which it affects a
disability categories. It is important for the medical community to student at school is more broadly defined and has less stringent
understand that educational systems do not use diagnostic criteria of criteria in Section 504 than in the IDEA; thus, Section 504 provides
the DSM-5 or International Classification of Diseases, Tenth Revision. for support and services for students with disabilities who do not
Rather, IDEA eligibility criteria use disability categories and focus on require more comprehensive special education support.
the ways in which a child’s disability affects academic achievement The medical home concept of comprehensive, coordinated care
or educational performance. A multidisciplinary team, including the is particularly useful for children with disabilities. Open commu-
child’s parents, reviews and discusses evaluation results to decide on nication among pediatricians, families, and schools can facilitate
eligibility, identify educational needs, and create an IEP, including shared expertise and knowledge of the unique needs of children
goals, services, accommodations, and placement. Should a child dem- with special needs and can foster implementation of appropriate
onstrate behaviors that affect the child’s or other students’ learning, services to address those needs. Each state establishes its own spe-
behavioral supports such as behavior goals or behavior intervention cial education code and regulations based on IDEA standards. Thus,
plans must be part of the IEP. The IEP is reviewed and revised regu- pediatricians should be familiar with their state laws as well as the
larly, with input from the IEP team members, including the parents federal IDEA so that they can advocate for their patients at the time
and school-based professionals. A distinguishing component of IDEA of school entry or whenever a child with a disability is not succeed-
is that the specific special education eligibility criteria used to qual- ing in school.
ify a child for special education services do not drive goals, services, The pediatrician’s role in advocacy on behalf of the child with a
and placement. Typically, within medical systems a child’s diagno- disability cannot be underestimated. Coordination of care, includ-
sis drives treatment; in the educational system, however, the child’s ing educational services, for children and adolescents with chronic
unique educational needs, regardless of special education eligibil- medical or disabling conditions should include the child’s primary
ity criteria, drive services. This is the individualized part of the IEP. care physician. To effectively coordinate patient care, the pediatri-
Special education services do have certain constraints. The pur- cian must be knowledgeable about federal and state education laws,
view of special education is the provision of educationally relevant establish linkages with early intervention services and parent sup-
services and supports. The purpose of special education is not to port resources, and promote open communication with the child’s
provide the full range of treatment options for a child with disability; family and school-based team. Additional examples of advocacy
rather, special education involves providing services and supports roles for physicians include involvement with assessment team pro-
so that children with disabilities can gain access to and receive bene- cesses at children’s schools, consulting with local school districts,
fit from their education. For example, services such as occupational participation in local or state early intervention interagency coun-
therapy and speech therapy need to relate to educational access and cils, and serving as knowledgeable proponents for improved com-
participation. Health professionals often view medical diagnoses munity and educational services.
and educational eligibility, as well as educationally related ser-
vices and medically necessary services, as the same. They are not.
Although these terms and services overlap, they are not interchange- Prognosis
able, and this can cause confusion and frustration for both medical Promotion of school readiness should be part of early pediatric vis-
providers and families. For example, a child with a medical diag- its. Children with daily exposure to reading, singing, and conver-
nosis of autism spectrum disorder may not meet the special edu- sation have an enormous language and academic advantage over
cational eligibility for a child with autism. However, this child may peers with fewer language and literacy experiences. Early literacy
meet the educational eligibility for speech and language impairment promotion through programs such as Reach Out and Read can edu-
and therefore would be eligible to receive special education sup- cate parents about the importance of reading to their children and
ports and services to address the child’s unique educational needs, enhance children’s exposure to early reading. Quality early child-
regardless of the special education category in which the child was hood education programs also promote children’s school readiness
deemed to be eligible. not only by introducing children to pre-academics but by introduc-
Section 504 of the Rehabilitation Act of 1973 (Section 504) is a civil ing them to the social, emotional, and behavioral requirements for
rights statute that prohibits exclusion of individuals and discrimina- school success. Early academic success is the best predictor of later
tion against people with disabilities in federally funded programs and academic success. Children who begin school with the appropri-
activities, including public schools and many after school programs. ate developmental skills, as well as family and community sup-
Students with a physical or neurologic impairment (eg, ADHD) that port, are prepared for learning. Pediatric primary care physicians

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 Chapter 36: Children and School: A Primer for the Practitioner 251

can serve an important role in improving educational and health Selected References
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on School Health. The pediatrician’s role in optimizing school readiness.
tion for children who are not academically, socially, or behavior-
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ally successful at school. peds.2016-2293
School failure results not from a single factor, but rather from a
American Psychiatric Association. Diagnostic and Statistical Manual of Mental
combination of risk factors. For children with neurologically based Disorders. 5th ed. Washington, DC: American Psychiatric Association Publishing;
learning disorders or other neurodevelopmental disorders, the edu- 2013
cational prognosis depends on the severity of the problems and the Bettencourt A, Gross D, Ho G. The Costly Consequences of Not Being Socially
intensity and timing of the interventions the children receive. With and Behaviorally Ready by Kindergarten: Associations With Grade Retention,
coordinated support from educational, medical, family, and psy- Receipt of Academic Support Services, and Suspensions/Expulsions. Baltimore,
chological sources, many of these students have successful school MD: Baltimore Education Research Consortium; 2016. http://baltimore-berc.
careers. Health impairments can contribute to school failure; how- org/wp-content/uploads/2016/03/SocialBehavioralReadinessMarch2016.pdf.
ever, social, behavioral, and emotional problems often contribute Accessed March 28, 2019
more significantly to academic difficulties. Students who may have Byrd RS. School failure: assessment, intervention, and prevention in primary
endured major or chronic socioeconomic upheaval or environmen- pediatric care. Pediatr Rev. 2005;26(7):233–243 PMID: 15994993
tal or familial trauma often continue to struggle unless they receive Centers for Disease Control and Prevention. Learn the signs. act early. develop-
comprehensive changes in their support system. mental surveillance resources for healthcare providers. CDC.gov website. www.
cdc.gov/ncbddd/actearly/hcp/index.html. Accessed March 28, 2019
The US Department of Education set a goal of having all stu-
dents graduate from high school prepared for college or careers. The Center for Mental Health in Schools at UCLA. Implementation science and inno-
vative transformation of schools and communities. http://smhp.psych.ucla.edu/
transition to college and career can have more challenges for stu-
pdfdocs/implement.pdf. Accessed March 28, 2019
dents with disabilities. On average, students with disabilities who
Center for Parent Information & Resources. Center for Parent Information &
receive special education services earn fewer overall credits, have
Resources hub website. www.parentcenterhub.org/. Accessed March 28, 2019
lower grade point averages, and fail more courses than their general
Chen Q, Hughes JN, Kwok OM. Differential growth trajectories for achievement
education peers. Course failure and credit deficiency are highly pre-
among children retained in first grade: a growth mixture model. Elem Sch J.
dictive of failing to graduate from high school. Outcomes for those
2014;114(3):327–353 PMID: 24771882 https://doi.org/10.1086/674054
who do graduate are also affected. High school students with an
Cortiella C, Horowitz SH. The State of Learning Disabilities: Facts, Trends and
IEP are less likely than their non-IEP peers to have experiences in
Emerging Issues. 3rd ed. New York, NY: National Center for Learning Disabilities;
high school (eg, managing their own bank or credit union account, 2014
driving, holding a part-time job) that are associated with suc-
Donoghue EA; American Academy of Pediatrics Council on Early Childhood.
cess after high school. Additionally, their parents have lower Quality early education and child care from birth to kindergarten.
expectations of them with regard to financial independence and Pediatrics. 2017;140(2):e20171488 PMID: 28771418 https://doi.org/10.1542/
independent living. Research has shown that students with peds.2017-1488
intellectual disability, autism, deaf-blindness, multiple disabilities, Dworkin PH. School failure. In: Augustyn M, Zuckerman B, Caronna EB, eds.
and orthopedic impairments are at the greatest risk for not transi- The Zuckerman Parker Handbook of Developmental and Behavioral Pediatrics
tioning successfully beyond high school. for Primary Care. 3rd ed. Philadelphia, PA: Lippincott, Williams & Wilkins;
Regardless the cause, academic difficulties and school failure can 2011:317–321
have lifelong consequences if not properly diagnosed and addressed. High PC; American Academy of Pediatrics Committee on Early Childhood,
Students with academic, behavioral, and social-emotional strug- Adoption, and Dependent Care, Council on School Health. School readiness.
gles are more likely than their peers to drop out of school or engage Pediatrics. 2008;121(4):e1008–e1015 PMID: 18381499 https://doi.org/10.1542/
peds.2008-0079
in behaviors that are dangerous to their health as adolescents. The
pediatrician can make a significant difference in outcomes for their Jimerson SR, Anderson GE, Whipple AD. Winning the battle and losing the war:
Examining the relation between grade retention and dropping out of high school.
patients’ educational and school success by helping families engage in
Psychology in the Schools. 2002;39(4):441–457 https://doi.org/10.1002/pits.10046
healthy development practices and advocating for appropriate assess-
La Paro KM, Pianta RC. Predicting children’s competence in the early school
ment of and intervention services for children who are struggling
years: a meta-analytic review. Review of Educational Research. 2000;70(4):443–
academically.
484 https://doi.org/10.3102/00346543070004443
Matthews JS, Kizzie KT, Rowley SJ, Cortina K. African Americans and boys:
understanding the literacy gap, tracing academic trajectories, and evalu-
CASE RESOLUTION ating the role of learning-related skills. Journal of Educational Psychology.
2010;102(3):757–771 https://doi.org/10.1037/a0019616
The pediatrician should advise the family to request in writing a psychoeduca-
tional evaluation for special education eligibility from the school. The pediatri- McFarland J, Hussar B, Wang X, et al. The Condition of Education 2018
cian can also institute an evaluation for ADHD by gathering information from (NCES 2018-144). US Department of Education. Washington, DC: National
the family. Center for Education Statistics. https://nces.ed.gov/pubsearch/pubsinfo.
asp?pubid=2018144. Accessed March 28, 2019

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Newman L, Wagner M, Huang T; SRI International. Secondary School Programs Scharf RJ. School readiness. Pediatr Rev. 2016;37(11):501–503 PMID: 27803148
and Performance of Students With Disabilities. A Special Topic Report of Findings https://doi.org/10.1542/pir.2016-0107
From the National Longitudinal Transition Study-2 (NLTS2) (NCSER 2012–3000). Shah RP, Kunnavakkam R, Msall ME. Pediatricians’ knowledge, attitudes,
US Department of Education. Washington, DC: National Center for Special and practice patterns regarding special education and individualized educa-
Education Research; 2011. https://ies.ed.gov/ncser/pubs/20123000/pdf/20123000. tion programs. Acad Pediatr. 2013;13(5):430–435 PMID: 23707687 https://doi.
pdf. Accessed March 28, 2019 org/10.1016/j.acap.2013.03.003
Rimrodt SL, Lipkin PH. Learning disabilities and school failure. Pediatr Rev. Shore R. Ready schools. Washington, DC: The National Education Goals Panel;
2011;32(8):315–324 PMID: 21807872 https://pedsinreview.aappublications. 1998. http://govinfo.library.unt.edu/negp/reports/readysch.pdf. Accessed March
org/content/32/8/315.long 28, 2019

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 CHAPTER 37

Immunizations
ChrisAnna M. Mink, MD, FAAP

CASE STUDY
A 20-month-old boy who emigrated with his family normal other than mild clear coryza and a rectal temper-
from Botswana because his mother is attending gradu- ature of 37.9°C (100.3°F). The physician must determine
ate school at a local university is brought to the office for a what vaccinations may be given to the patient.
checkup. He has his World Health Organization Expanded
Programme immunization card from his homeland show-
Questions
1. What are the different kinds of vaccines?
ing that he received a BCG vaccine at birth; 3 doses of diph-
2. What are the mechanisms of action for live and
theria, tetanus toxoids, and pertussis vaccine at 2, 4, and
inactivated vaccines?
6 months of age; 3 doses of live oral poliovirus vaccine
3. What are the routinely recommended immuniza-
at 2, 3, and 4 months of age; 3 doses of hepatitis B vac-
tions for healthy pediatric populations?
cine at birth and at 2 and 9 months of age; and a mono­
4. What are the considerations for immunizing select
valent measles vaccine at 9 months of age. It is August,
pediatric populations, such as immunocompromised
and his parents plan to enroll him in child care; they are
children?
eager for him to receive any needed vaccines. His parents
5. What are reliable resources for up-to-date informa-
report that he is a healthy boy with no immune problems.
tion about immunizations?
They report that they will be living with his uncle, who has
6. How can a pediatrician address parental vaccine
HIV infection. The boy has had a 3-day history of a runny
refusal?
nose, cough, and tactile fever. His physical examination is

The Centers for Disease Control and Prevention (CDC) consid- infants, and young children as well as education and anticipatory
ered immunizations among the top 10 greatest health accomplish- guidance for parents. Increasing availability of new vaccines tar-
ments of the 20th century, and vaccines continue to play a major geted for older children and adolescents should permit opportunities
role in the improvement of the health of the world’s population. for improved health care delivery to these age groups. Additionally,
In the United States, the incidence of nearly all the pathogens for health professionals who treat adults have a growing appreciation
which there are routine vaccinations has decreased by 95% to 100% for the critical role of vaccinations in protecting their patients as
since the early 20th century. The only exception to this is pertus- well as all members of their patients’ family.
sis, which has undergone an approximately 80% reduction. In 2017,
the immunization rates for children in the United States remained General Principles
high, with 84% to 94% of children aged 19 to 35 months immunized
When planning immunizations for a patient, 2 important factors to
for the 4:3:1:3:3 series (4 diphtheria, tetanus toxoids, and acellular
consider are the health status of the recipient and the type of immu-
pertussis [DTaP]; 3 polio; 1 measles, mumps, and rubella [MMR];
nization to be given. The risks and benefits of using the vaccine in
3 Haemophilus influenzae type b [Hib]; 3 hepatitis B virus [HBV]).
the specific host should be weighed carefully. Vaccines are intended
Worldwide, the percentage of children immunized with 3 doses of
for a host with the capacity for mounting an appropriate immune
diphtheria, tetanus toxoids, and pertussis (DTP) and oral polio vac-
response, who will likely benefit from the protection provided, and,
cines (OPV) and a measles-containing vaccine is at a record high
ideally, who is at little to no risk for adverse effects.
of nearly 85%. Since 1990 the mortality rate has declined for chil-
dren younger than 5 years in every region in the world, which is
directly related to increased rates of vaccinations. Generally, immu-
Types of Immunization: Passive
nizations are safe, well tolerated, and cost-effective, with savings of and Active
$5 to $16.50 for every $1 spent. The 2 major types of immunizations are passive and active.
Another less recognized benefit of vaccinations is that the sched- Passive immunization refers to the delivery of preformed anti-
ule has essentially provided the backbone for routine pediatric care bodies, usually as immune globulin (IG), which may be a general
in the United States, with regular visits scheduled around the rec- formulation or hyperimmune IG developed with high concentra-
ommended intervals for immunizations. These visits have afforded tions of antibodies against a specific disease, such as hepatitis B IG
health professionals opportunities for serial evaluation of newborns, for hepatitis B.
253

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 254 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Delivery of IG may be useful in any of the following 3 settings: in pathogens, including Streptococcus pneumoniae and Neisseria
a host who cannot manufacture antibodies (eg, congenital immuno- meningitidis.
deficiency); as a preventive measure, either pre- or post-exposure,
especially when the host may be unable to mount an antibody Live-Attenuated Vaccines
response (eg, immunocompromised naïve child with acute expo- Live-attenuated vaccines are infectious agents that replicate in the
sure to varicella); or for treatment, in which IG may be used to ame- host to elicit an immune response. The administration is generally
liorate symptoms in the patient in whom disease is already present not intramuscular but by other delivery routes, such as oral, intra-
(eg, intravenous IG for the management of Kawasaki disease). nasal, or subcutaneous. Often the live vaccines are viral, including
With active immunization all, part, or a modified product MMR, varicella-zoster virus (VZV), rotavirus, live-attenuated influ-
(eg, toxoid, purified antigen) of a microorganism is given to the enza virus (LAIV), OPV, and yellow fever vaccines. Two live bacterial
host to elicit an immune response. The intact organisms may be vaccines are available: BCG used against Mycobacterium tuberculo-
inactivated (ie, killed) or live-attenuated (ie, weakened). Usually, the sis and oral typhoid (Ty21a) vaccine.
elicited immune response mimics the response to natural infection A transient suppression of T-cell immunity occurs 2 to 4 weeks
and, ideally, poses little to no risk to the recipient. after measles vaccination. Because of this, when vaccinating with
live viral vaccines, 2 or more live vaccines should be administered
Inactivated Vaccines
at the same time or vaccine administrations should occur at least
Inactivated vaccines may contain inactivated or killed organisms, 4 weeks apart. This principle also holds true for tuberculosis skin
purified components (ie, subunit), or inactivated toxins (ie, toxoids) testing; either the purified protein derivative should be placed at the
of the organism. These vaccines are not capable of replication in the same time as a live viral vaccine, or they should be administered at
host. Most inactivated vaccines are delivered by intramuscular injec- least 4 weeks apart to avoid a false-negative purified protein deriva-
tion. Generally, inactivated vaccines may be administered simulta- tive result because of the transient T-cell suppression. Although this
neously with other inactivated vaccines, as well as live viral vaccines. phenomenon has primarily been studied with measles vaccine, the
The common viral vaccines that are inactivated include inacti- same guidelines should be followed with other live viral vaccines.
vated influenza vaccine (IIV), trivalent and quadrivalent formula-
tions, hepatitis A vaccine (HAV), HBV, inactivated poliovirus vaccine
(IPV), human papillomavirus (HPV), Japanese encephalitis, and Vaccination Schedule
rabies vaccines. Toxoid vaccines that are used routinely include tet- Factors for developing the schedule include the host ability to
anus and diphtheria toxoids alone or in combination with whole- respond (eg, lost maternal antibody), the need for multiple doses
cell or acellular pertussis components (eg, DTaP; DTP; tetanus and (eg, IPV), the minimal intervals needed between serial doses, and
diphtheria toxoids; tetanus toxoid, reduced diphtheria toxoid, the available products (eg, combination vaccines). Each year, a
and acellular pertussis [Tdap]). The acellular pertussis vaccines are synchronized immunization schedule is posted by the American
composed of 1 or more purified antigens of Bordetella pertussis, in Academy of Pediatrics (AAP), American College of Obstetricians
contrast with the whole-cell pertussis vaccines, which are made with and Gynecologists, American Academy of Family Physicians,
killed, whole B pertussis organisms. Diphtheria and tetanus tox- and the CDC Advisory Committee on Immunization Practices.
oids combined with whole-cell pertussis (ie, DTP) vaccines are no Separate immunization schedules are available for children from
longer marketed in the United States but are used in many develop- birth through 18 years as well as for adults 19 years and older. The
ing countries. Other inactivated bacterial vaccines include capsular schedules for the United States are posted each January at https://
polysaccharide (CPS) vaccines, such as the 23-valent pneumococcal www.cdc.gov/vaccines/schedules. Schedules for countries worldwide
polysaccharide vaccine and tetravalent meningococcal CPS vaccine. are available from the World Health Organization.

Conjugate Vaccines
Vaccine Recipients
Capsular polysaccharide antigens are chemically linked to a pro-
tein carrier, which converts the T-cell independent polysaccha- Healthy Pediatric Populations
rides to T-cell dependent antigens. These conjugate vaccines can Routine immunizations on the synchronized schedule are tar-
elicit an immune response, even in young infants. The first CPS- geted for healthy newborns, infants, children, and adolescents. All
protein conjugate vaccine available was for Hib. The Hib bacterium licensed vaccines have undergone review by the US Food and Drug
is covered with a CPS, polyribitol-phosphate. Children younger than Administration (FDA) and have proven safety and immunogenicity
2 years of age are not efficient at mounting antibodies to the polyribitol- or efficacy for the target population. No vaccine is completely free
phosphate CPS; however, with linkage to a protein carrier the of adverse events or provides 100% protection for every recipient,
CPS is immunogenic. Since licensure for infants of the Hib con- however. Every effort should be made to provide immunizations
jugate vaccines in 1991, a more than 98% reduction in Hib disease when the recipient is healthy and has the best chance to mount an
has occurred. With the success of the Hib CPS-protein conjugate optimal immune response without delaying vaccination or risking
vaccines, conjugation techniques have been used for other CPS a missed opportunity.

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 Chapter 37: Immunizations 255

Special-Risk Pediatric Populations (ie, those who cannot make antibodies); however, MMR vaccine may
be indicated for some of these individuals because of the poten-
Although immune responses to vaccinations are likely most
tial risks of natural infection. Receipt of all vaccines, including live
favorable in healthy recipients, a growing segment of the pediatric
viral vaccines, is acceptable for most individuals with complement
population has underlying health problems. Because of congeni-
deficiencies. For abnormal phagocytic function, live bacterial
tal or acquired immune dysfunctions, some individuals should not
vaccines should not be given. For individuals with traumatic or
receive immunizations as directed by the routine schedules. Special
surgical asplenia, vaccination with pneumococcal, meningococcal,
accommodations need to be made for immunizing these individuals,
and Hib vaccines is indicated and should be considered emergently in
such as adjusting the schedule or possibly not administering some
the case of trauma. Chemoprophylaxis also may have a role in
agents. Administration of decreased or partial doses of vaccines is
protection for compromised individuals.
not indicated. Some of the select populations or circumstances that
For children with immunocompromised household contacts, it is
warrant special consideration include immunocompromised sta-
generally acceptable for them to receive MMR, VZV, and oral rota-
tus, immunodeficiency, pregnancy, preterm birth status, low birth
virus vaccines. However, the live viral vaccines of OPV and LAIV
weight status, allergy to egg protein, planned international travel,
should not be given in some settings. In contrast, use of some vac-
patients from other countries, adolescence, and vaccines adminis-
cines, such as IIV, is encouraged to protect the vaccinated individ-
tered in other countries.
uals as well as their compromised contacts.
Immunocompromised Child Pregnancy
The vaccination plan for the immunocompromised child should Pregnancy is associated with some impairment of cell-mediated
be determined by the nature and degree of immunosuppression. immunity. With this decreased immunity, pregnant women may
The health professional should weigh risks and benefits for each not mount protective immune responses to some infectious agents.
child individually, with consideration for some general principles. Thus, in general, live vaccines should not be administered to preg-
For example, live vaccines should not be given to severely compro- nant women; however, the risks and benefits should be weighed for
mised individuals because of the possible risks. In general, inacti- each individual patient. Live-attenuated influenza virus should not be
vated vaccines may be safely administered to nearly all recipients; given to pregnant women. Neither should rubella vaccine be given to
however, immunocompromised individuals may not mount an opti- pregnant women, although no cases of rubella embryopathy following
mal immune response. In this setting, the health professional should inadvertent immunization of a pregnant woman have been reported.
attempt to adjust timing of vaccination to optimize the chance of a Both IIV and Tdap are recommended during pregnancy to pro-
good immune response. Guidelines for immunizing immunocom- vide protection for the mother and the fetus. These vaccines may
promised children and adults have been established by the Infectious be given anytime during pregnancy, although the preferred timing
Diseases Society of America in conjunction with the AAP, CDC, for Tdap administration is 27 through 36 weeks to optimize trans-
and other professional groups and are posted at www.idsociety.org/ fer of pertussis antibodies to the fetus. Pediatricians are often asked
Templates/Content.aspx?id=32212256011. whether administration of live viral vaccines is contraindicated for
children residing with a pregnant household contact; generally, such
Types of Immunodeficiency administration is not contraindicated.
Newborns, infants, and children may have abnormalities of any
aspect of the immune system, which may affect their ability to Preterm and Low Birth Weight Infants
receive vaccinations. Weighing the risks of both the disease and In general, medically stable preterm (<37 weeks of gestation) and
the vaccine and the benefits of protection is essential. A reason- low birth weight infants (<2,500 g [<5 lb 5 oz]) may be immunized
able approach to developing a vaccination plan for children with at the same dose, schedule, and postnatal age as full-term and nor-
immune abnormalities is to consider the mechanism of immune mal birth weight infants.
defense against the vaccine agents; if the needed defense mechanism Special consideration should be given to use of HBV vaccine in
is deficient, immunizing with that agent may not be appropriate. For newborns weighing less than 2,000 g (<4 lb 4 oz) as follows. For
example, cellular immunity is essential in defending against viral the hepatitis B surface antigen (HBsAg)-positive mother or mother
agents. Thus, children with abnormalities of cell-mediated immu- whose status cannot be determined within 12 hours, monovalent
nity, whether primary or acquired, may not be candidates for live hepatitis B vaccine and hepatitis B IG should be administered within
viral vaccines. 12 hours of birth. The birth dose of vaccine does not count as part of
Primary immunodeficiencies are generally inherited, and second- the series; thus, the infant requires 3 additional vaccine doses start-
ary immunodeficiencies are acquired. Examples of acquired immu- ing at 1 month of age. The HBsAg and antibodies should be checked
nodeficiencies include HIV infection, malignancy, and illnesses (eg, after completing the vaccine series, usually at the 9- or 12-month
malnutrition, uremia) as well as those caused by medications (eg, check-up. For the HBsAg-negative mother, monovalent hepatitis B
chemotherapy, immunosuppressive agents). The OPV is contraindi- vaccine is administered to the newborn at 1 month of age (sooner if
cated for individuals with primary humoral immunity abnormalities the newborn is stable for discharge), after which the usual schedule

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 256 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

is followed, such that the infant receives a total of 3 doses. Serologic 18 years of age. Currently, Tdap is licensed only for a single booster
testing is not necessary. dose. Off-label use of additional doses is recommended in special
situations, however, such as during pregnancy and for close contacts
Other Conditions Affecting of infants. With recognition of waning vaccine-induced immunity
Immunization Schedule to pertussis, additional Tdap doses may be routinely recommended
Allergy to Egg in the future.
Children with allergic reactions to egg protein—including severe Meningococcal vaccine and HPV are also indicated for ado-
hypersensitivity—are at low risk for anaphylactic reactions to mea- lescents at the 11- to 12-year-old visit. Four meningococcal vac-
sles, mumps, and influenza (both IIV and LAIV) vaccines. Special cines are available in the United States: 2 CPS-protein conjugate
precautions for immunizing children with egg allergy are no longer vaccine (meningococcal conjugate vaccine [MenACWY]), and
routinely recommended. 2 B meningococcal (MenB) vaccines. The CPS vaccines contain
Yellow fever vaccine may contain egg protein in higher concen- 4 serotypes (A, C, Y, and W-135). The MenACWY-D vaccine
trations than in influenza vaccines and may rarely induce an imme- (Menactra) is licensed for use in persons age 9 months to 55 years.
diate allergic reaction. Guidelines for skin testing and graded vaccine The MenACWY-CRM vaccine (Menveo) is licensed for use in
dosing are provided in the vaccine package insert. persons age 2 months to 55 years. Routine immunization with
MenACWY is recommended at age 11 to 12 years, with a booster
International Adoptees, Travelers, Immigrants, dose at age 16 years. For adolescents who received the first dose
and Refugees between age 13 and 15 years, the second dose may be given at
Travel is not restricted to persons of any particular socioeconomic sta- ages 16 to 18 years (up to 5 years after the first dose); however, no
tus; thus, physicians should inquire about foreign travel in all routine booster dose is needed for teenagers who receive their first dose
clinical visits. In preparing patients for travel, the health professional at age 16 years or older. The MenACWY vaccine is also recom-
should review the child’s record to ensure that all routine vaccines are mended for catch-up dosing for older adolescents who have not
up-to-date. The child should receive vaccinations and other preven- been immunized, as well as for individuals 9 months to 55 years
tive measures (eg, malaria prophylaxis) targeted for his or her desti- of age who are at increased risk of meningococcal diseases. The
nation. An accelerated schedule may be necessary, for example, early 2 MenB vaccines (Bexsero and Trumenba) are prepared using
administration of MMR for infants 6 to 12 months of age traveling different virulence factors of the bacteria. The vaccines are licensed
to a measles-endemic area. Use of IG prophylaxis to prevent HAV is for individuals 10 through 25 years of age and recommended for
recommended for susceptible individuals who are not candidates those at increased risk of meningococcal infection. The vaccines
for active immunization (eg, too young to receive the HAV vaccine, may also be used during MenB outbreaks. The MenB vaccines are
immunocompromised status) traveling to areas with elevated risk not routinely recommended at the 11- to 12-year-old visit.
of hepatitis A. To help ensure healthy travel, the health professional The only HPV vaccine available in the United States is 9-valent
should check the current recommendations for the traveler’s desti- (9vHPV) and is recommended for routine use in adolescents
nation at the CDC Travelers’ Health website (https://wwwnc.cdc.gov/ at 11 to 12 years of age. The vaccine is composed of virus-like
travel) and the World Health Organization International Travel and particles prepared from recombinant L1 capsid protein. The 9vHPV
Health website (www.who.int/ith/en). vaccine (Gardasil 9), contains serotypes 6, 11, 16, 18, 31, 33, 45, 52,
Immigrants, refugees, and international adoptees often have and 58 and it is licensed for females and males age 9 through 26 years
health care issues. Immunization status, underlying health, and to protect against cancers caused by HPV infections. For individu-
possible intercurrent illnesses should be evaluated soon after als younger than 15 years, 9vHPV is administered in a 2-dose regi-
arrival. Many of these children have been in poor living condi- men, with the second dose given 6 to 12 months after the first dose.
tions and exposed to health hazards of environments such as refu- For those age 15 years and older, 9vHPV is given in a 3-dose regi-
gee camps and orphanages. The United States requires proof of the men at day 0, 1 to 2 months, and 6 months.
first dose of vaccines for entry into the country, although exemp- In addition, at adolescent visits (including precollege visits),
tions exist for refugees and adoptees younger than 10 years of age. health professionals should review the patient’s records to ensure
Often these high-risk children have not been immunized or their that all recommended vaccines have been received, inquire about
records are missing. Written, dated, and appropriate records (ie, household contacts of infants or compromised hosts, and provide
patient age, dates, interval, number of doses) may be considered anticipatory guidance for safe and healthy living for the adolescent
valid, and subsequent immunization may resume according to the and parent.
US schedule. Another option, especially in cases in which docu-
mentation is questionable, is to perform serologic studies for anti- Immunizations Received in Other
bodies to vaccine antigens with available valid testing.
Countries
Adolescents Most vaccines used worldwide are produced with adequate qual-
The AAP recommends a routine health visit at 11 to 12 years of age, ity control and may be considered reliable. Healthy immigrants
including receipt of immunizations needed for adolescents. One immunized in countries outside the United States should receive
of these vaccines is Tdap for use as a single booster dose at 11 to vaccines according to the recommended schedule for age in the

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 Chapter 37: Immunizations 257

United States. Only written documentation should be accepted be performed. Additionally, all clinically significant adverse events
as proof of previous vaccination. Written, dated, and appropriate should be reported to the Vaccine Adverse Event Reporting System
records (ie, correct age, dates, interval, number of doses) may be (https://vaers.hhs.gov), which is maintained by the CDC and FDA.
considered as valid, and immunizations may resume according to Adverse event reporting is important because it helps identify pos-
the US schedule. sible unexpected events that were not observed in pre-licensure clin-
Although most globally prepared vaccines are acceptable, con- ical trials.
cern may exist for vaccine potency because of unsuitable storage
and handling. Other concerns include inaccurate documentation Precautions and Contraindications
and inadequate immune response in some children resulting from The Vaccine Information Statement (VIS) and package insert pro-
other factors (eg, malnutrition, underlying illness). If vaccination vide information for health professionals, the non-minor vaccinated
status is uncertain, options include vaccinating with the antigen individual, and the parent(s)/legal guardian(s) of minor vaccinated
in question or, if available, serologic testing. Generally, receipt of individuals about the precautions and contraindications for spe-
additional doses of diphtheria and tetanus toxoids alone or in com- cific products.
bination with a pertussis-containing vaccine (ie, DTP, DTaP, tet- A precaution suggests that careful analysis of risks and benefits of
anus and diphtheria toxoids, Tdap) may result in an increase in the vaccine should be performed; if benefits outweigh risks, the vac-
reactions (especially injection site reactions), and checking anti- cine may be given. A contraindication means that a vaccine should
body titers against diphtheria and tetanus toxoids is encouraged. not be administered. An example of a contraindication is known
Currently, commercially available assays for pertussis antibod- anaphylaxis to any component of the vaccine. Breastfeeding does
ies are of unknown clinically accuracy and testing is not recom- not interfere with oral immunization with rotavirus or OPV vac-
mended. A serological assay developed by the CDC and FDA has cines and is not a contraindication.
been used to confirm the diagnosis of pertussis, especially during Minor illness without fever (temperature ≤38°C [≤100.4°F])
outbreaks. For other vaccines, if the status is unknown, vaccina- should not be considered a contraindication to vaccination.
tion may be performed because extra doses are generally well tol- Temperature above 38°C (>100.4°F) may not be a contraindica-
erated. Additionally, extra doses are less expensive and more time tion, depending on the physician’s assessment of the child, the
efficient than performing serology. Most developing countries do illness, and the particular vaccine. If the child is evaluated early
not have VZV, conjugated pneumococcal, or Hib vaccines; thus, in the disease process and the course is not predictable or the
these should be given as indicated per the US schedule. illness is moderate to severe, delaying immunization is reason-
able. Deferring immunization without appropriate justification can
Adverse Events and Vaccine cause a missed opportunity and may result in inadequate immu-
Information nization of the child.
Adverse Events
As noted previously, no vaccine is completely free of adverse events, Informing Vaccine Recipients and
and known adverse events should be discussed with non-minor vac- Parents and Vaccine Refusal
cine recipients or the parent(s)/legal guardian(s) of the minor vaccine Vaccine recipients and parents should be informed about the risks
recipient. In addition to discussing the risks and benefits of vaccina- and benefits of vaccination and the disease the vaccine is designed
tion, health professionals should include education about the risks to prevent. The National Childhood Vaccine Injury Act of 1986
associated with the natural disease. This is especially important today, requires that parents receive a VIS each time a child receives a vac-
because many individuals have not seen the diseases that vaccines cine covered under this legislation, whether the vaccine was pur-
have been successful in controlling or eradicating. chased with public or private funds. The VISs are available from the
In addition to safety information from the AAP and CDC, the CDC (www.cdc.gov/vaccines/hcp/vis/index.html). Health profes-
manufacturer’s package insert provides information about the rates sionals should document in the patient’s chart the vaccine manu-
of adverse events and contraindications for the specific vaccine. Most facturer, lot number, and date of administration and that VISs were
adverse events observed following routine immunizations are local provided and discussed with the non-minor vaccinated individual
injection site reactions (eg, erythema, swelling, pain) and systemic and the parent or legal guardian of the minor vaccinated individual.
reactions (eg, fever, fussiness). Although most of these adverse events In the United States, proof of immunization is required for entry
are mild and self-limiting, some may be associated with significant into elementary and secondary school. In addition, some child care
dysfunction for the child (eg, not using a limb because of pain). centers and colleges also require vaccines for entry. All states permit
Rarely, serious adverse events may occur following immuni- medical exemptions (eg, immunocompromised child), and most
zation, and these may be associated with permanent disability or states have provision for religious or philosophic exemption for
life-threatening illness. The occurrence of an adverse event after individuals whose beliefs prohibit immunizations. Three states—
immunization does not prove a cause-and-effect relationship of the California, Mississippi, and West Virginia—do not permit personal
vaccine and the event but a temporal relationship. If a vaccine recip- belief exemptions for children attending child care or schools.
ient experiences a serious adverse event, a complete evaluation for Less than 1% of US children are from families who refuse all
all plausible causes, including the role of the vaccine antigen, should vaccines. An increasing number of parents decline some

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 258 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

immunizations for their child, however, despite best efforts

to educate parents about the effectiveness of vaccines and CASE RESOLUTION
the realistic chances of vaccine-associated adverse events. At The boy was appropriately immunized for Botswana recommendations through the
times, parents have a genuine fear of the risks of vaccines with- age of 9 months; however, his immunizations are considered delayed according to
out fear of the natural disease, because many parents have the US schedule. He is past due for the fourth dose of DTaP and the first dose of MMR
not seen the diseases in the current, post-vaccine era. For oth- and VZV vaccines. Additionally, he has not received HAV or any conjugate vaccines.
The dose of monovalent measles vaccine does not change his need to receive 2
ers, their exaggerated fear of vaccine risks results from deeply
doses of MMR and VZV vaccines after age 12 months in the United States. His uncle’s
held family beliefs, or their fears are fueled by biased anti- immune status does not affect his receipt of MMR or VZV vaccines. His current respi-
vaccine information presented in the media without scientific ratory illness is considered mild, and he does not have significant fever (<38°C
support. [<100.4°F]); thus, his illness does not preclude him from receiving immunizations.
A reasonable approach for the health professional is to address During this visit, he may receive DTaP, the first dose of MMR plus VZV (alone
the concerns of parents or guardians in a non-condescending fash- or as a combination MMRV); 1 dose of Hib conjugate vaccine; dose 1 of 2 of conju-
gate pneumococcal vaccine, which should be administered 2 months apart; and
ion, provide education about the known risks and benefits of immu-
dose 1 of 2 of HAV, which should be administered at least 6 months apart. He
nization, and initiate a candid discussion of the risks of the natural is too old to receive the oral rotavirus vaccine. All VISs should be provided. The
infections. The discussion should be documented in the child’s chart patient is scheduled to return in 2 months (ie, in October) for his next dose of con-
and the topic revisited at future encounters. The AAP encourages jugate pneumococcal vaccine and dose 1 of 2 of IIV. He will need a fourth dose of
a presumptive rather than a participatory approach in counseling inactivated poliovirus vaccine before school age.
about vaccinations. With the presumptive approach, instead of ask-
ing the parents or guardians whether they want the vaccines to be
administered, the pediatrician informs them that shots are due. Selected References
Researchers have shown that parents with whom this strategy is American Academy of Pediatrics. Documenting Parental Refusal to Have
used are more likely to accept vaccines. Because of strong convic- Their Children Vaccinated. www.aap.org/en-us/Documents/immunization_
tions that immunization benefits outweigh risks, some health pro- refusaltovaccinate.pdf. Accessed March 22, 2019
fessionals choose not to provide care for children of families who American Academy of Pediatrics. Immunizations: vaccine hesitant parents.
refuse vaccines. www.aap.org/en-us/advocacy-and-policy/aap-health-initiatives/immunizations/
Pages/vaccine-hesitant-parents.aspx. Accessed March 22, 2019
Vaccine Information American Academy of Pediatrics. Active and passive immunization. In:
Information about current immunizations for health profession- Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018–2021
als and laypersons is available from many resources, including Report of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American
Academy of Pediatrics; 2018:1–111
the CDC, AAP, FDA, and World Health Organization (Table 37.1).
American Academy of Pediatrics Committee on Infectious Diseases.
Multiple web-based applications with vaccine information are
Meningococcal conjugate vaccines policy update: booster dose recommendations.
available for smart devices from the AAP and US Department Pediatrics. 2011;128(6):1213–1218. Retired January 2015. PMID: 22123893
of Health and Human Services (ie, CDC, FDA, National Institutes https://doi.org/10.1542/peds.2011-2380
of Health), as well as other professional organizations. Centers for Disease Control and Prevention. Immunization Information Systems
Annual Report (IISAR). www.cdc.gov/vaccines/programs/iis/annual-report-
iisar/index.html. Updated October 9, 2018. Accessed March 22, 2019
Table 37.1. Internet Sources of Vaccine
Centers for Disease Control and Prevention. Recommended Child and Adolescent
Information Immunization Schedule, United States, 2019. www.cdc.gov/vaccines/schedules/
Source Website downloads/child/0-18yrs-child-combined-schedule.pdf. Published February 22,
American Academy of Pediatrics www.aap.org 2019. Accessed March 22, 2019
Centers for Disease Control and Prevention. State vaccination requirements.
Centers for Disease Control and www.cdc.gov/vaccines/schedules
www.cdc.gov/vaccines/imz-managers/laws/state-reqs.html. Updated January 29,
Prevention 2016. Accessed March 22, 2019
Vaccine-Specific Advisory Committee www.cdc.gov/vaccines/hcp/acip- Feldstein LR, Mariat S, Gacic-Dobo M, et al. Global routine vaccination coverage,
for Immunization Practices recs/vacc-specific/index.html 2016. MMWR Morb Mortal Wkly Rep. 2017;66(45):1252–1255 PMID: 29145357
Recommendations https://doi.org/10.15585/mmwr.mm6645a3
Morbidity and Mortality Weekly Report www.ncbi.nlm.nih.gov/ Hill HA, Elam-Evans LD, Yankey D, Singleton JA, Kang Y. Vaccination coverage
pubmed/11848294 among children aged 19-35 Months—United States, 2016. MMWR Morb Mortal
Wkly Rep. 2017;66(43):1171–1177 PMID: 29095807 https://doi.org/10.15585/
US Food and Drug Administration: www.fda.gov/ mmwr.mm6643a3
Vaccines Licensed for Use in the United BiologicsBloodVaccines/Vaccines/ Kroger AT, Duchin J, Vázquez M. General best practice guidelines for immu-
States ApprovedProducts/UCM093833 nization: best practices guidance of the Advisory Committee on Immunization
Vaccine Adverse Event Reporting System https://vaers.hhs.gov/ Practices (ACIP). www.cdc.gov/vaccines/hcp/acip-recs/general-recs/index.html.
Updated October 30, 2018. Accessed March 22, 2019
World Health Organization www.who.int/en
World Health Organization. Global Health Observatory data. www.who.int/gho/
Gavi, the Vaccine Alliance www.gavialliance.org immunization/en. Accessed March 22, 2019

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 CHAPTER 38

Health Maintenance in Older

Children and Adolescents
Monica Sifuentes, MD

CASE STUDY
Before a 13-year-old girl enters a new school, she is 2. What immunizations are recommended for older
required to undergo a physical examination. She has children and adolescents?
not seen a primary care physician in many years and has 3. What laboratory tests should be performed at
been healthy. Currently she has no medical complaints. health maintenance visits? Why?
Her examination is completely normal. 4. What are significant topics to cover for anticipatory
guidance in this age group?
Questions
1. What are the important components of the history
and physical examination in healthy older children
and adolescents?

Older children and adolescents are generally healthy individu- Guidelines for preventive child and adolescent health care have been
als who infrequently visit physicians. If they are seen by a doctor, published by the American Academy of Pediatrics (AAP) in conjunc-
the visits are often for acute complaints, such as upper respi- tion with the Maternal and Child Health Bureau, US Child Health and
ratory infections or sports-related injuries, and are, therefore, Disability Prevention Program, AAP Section on Adolescent Health,
generally problem oriented. Statistics on health maintenance vis- and American Medical Association. Box 38.1 is a brief summary of
its in this age group are not readily available because patients may these guidelines for older children and adolescents.
go to several different sites for health care and often do not receive
consistent comprehensive care at any of these places for a vari- Health Maintenance Visit
ety of reasons. Older children and adolescents seek treatment for The purpose of the health maintenance visit for an older child or
acute and chronic conditions in private offices, urgent care cen- adolescent is to assess their general physical health, mental and psy-
ters, public health clinics, community and school health clinics, chological health, and overall well-being and establish an indepen-
hospitals, and emergency departments. It has been reported that dent relationship between the patient and health professional for
fewer than 50% of adolescents consistently receive a preventive open communication and trust for future visits. Initial questions
health care visit during any given year, and the same percentage asked during this visit should be simple and focused on how the
probably applies to older children as well. patient feels in general about his or her health, physical growth and
This all-too-common practice of inconsistent health care development, and existing relationships with family and friends.
contributes to missed opportunities for anticipatory guidance, health More specific questions can then be formulated depending on the
education, and screening for preventable conditions. Screening tests patient’s responses. In healthy patients, the medical history can be
also can be used to identify treatable conditions such as hyperten- obtained using a questionnaire that parents and children complete
sion, anemia, and tuberculosis. Ideally, older children and adoles- in the waiting room. If this method is used, a separate form should
cents should receive recommended immunizations beginning at be given to the adolescent if they are accompanied by a parent or
11 to 12 years of age; screening for depression; counseling con- guardian. The information is then reviewed at the start of the inter-
cerning sexual activity, contraception, and sexually transmitted view. Chronic medical conditions should be addressed at this time.
infections (STIs), including HIV; reassurance to address their emo-
tional well-being; guidelines for adequate nutrition, sleep hygiene, Medical History
and screen time; education about tobacco, e-cigarettes and vaping, Older children and adolescents should always be questioned directly
illicit drugs, and alcohol; and information about physical fitness and about their medical history (Box 38.2). The parent or guardian
exercise as well as violence and injury prevention. should be encouraged to participate only after the child or adolescent

259

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 260 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Box 38.1. Guidelines for Adolescent Health Maintenance Evaluation

Screening History ww If sexually active

ww Eating disorders —— Urine hCG
ww Gender identification —— Urine or vaginal NAAT for gonorrhea/chlamydia
ww Sexual orientation —— Serum HIV, RPR, hepatitis Cc
ww Sexual activity (consensual and nonconsensual) —— Papanicolaou testb
ww Tobacco, e-cigarettes, or vaping use Anticipatory Guidance and Counseling
ww Alcohol use/abuse ww Parenting/communication
ww Drug use/abuse (CRAFFT questionnairea) ww Pubertal development
ww School performance ww Diet/nutrition, including calcium and vitamin D supplementation
ww Depression ww Exercise
ww Risk for suicide ww Injury prevention
Physical Examination ww Screen time/social media
ww BMI ww Educational or vocational plans/goals
ww Blood pressure ww Lifestyle modifications
ww Comprehensive examination —— Gender and sexual identity
ww Genital examination —— Abstinence
ww Pelvic examinationb —— Safe sexual activity
Universal and Selective Screening Laboratory Tests/Studies —— Other reproductive health issues
ww Snellen test —— Contraception
ww Audiometry —— Avoidance of tobacco, e-cigarettes, vaping, alcohol, and prescription
ww Hemoglobin or hematocrit and illicit drugs
ww Tuberculin skin test or blood test (IGRA) —— Identifying feelings of sadness/anger
ww Cholesterol (If the patient is obese or there is a significant family history of
hyperlipidemia, consider other laboratory tests, such as fasting glucose and
lipid panel.)

Abbreviations: BMI, body mass index; CRAFFT, car, relax, alone, forget, friends/family, trouble; hCG, human chorionic gonadotropin; HIV, human immunodeficiency virus; IGRA, interferon gamma release
assay; NAAT, nucleic acid amplification test; RPR, rapid plasma reagin.
a
See Chapter 63 for information about the CRAFFT questionnaire.
b
A pelvic examination with a Papanicolaou test is recommended within 3 years of the onset of sexual activity (American Cancer Society) or age 21 years (American Congress of Obstetricians and
Gynecologists). For indications for a pelvic examination, see Chapter 58.
c
If patient engaged in injection drug use or young man having sex with men.

Box 38.2. What to Ask

Screening in Older Children and Adolescents ww Does the child or adolescent take any medications, herbs, or supplements
Questions for Patient and Parent (prescribed or over-the-counter) regularly?
ww How has the child or adolescent been doing lately? Does the parent have Questions for Child or Adolescent Alone
any complaints or concerns?
ww Do you have any questions or concerns?
ww How does the child or adolescent like school? How is he or she doing
ww How are things at home? Are there any problems with parents or siblings? Do
academically and socially? What are his or her future goals?
you feel safe at home and school?
ww What activities does the child or adolescent currently participate in, includ-
ww Are you attending school?
ing work?
ww Do you like school? Who do you hang out with at school?
ww Does he or she have any hobbies?
ww Have you ever been truant, suspended, or expelled?
ww With whom does the child or adolescent live?
ww What do you like to do for fun?
ww Are there any significant illnesses in the immediate or extended family,
such as hypertension, diabetes, or cancer? (See Chapter 4 for the rest of the interview.)

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 Chapter 38: Health Maintenance in Older Children and Adolescents 261

has responded to questions or if invited by the child or adolescent to whether or not patients acknowledge that they have skin problems.
assist with the interview. The degree of parental participation also Tattoos, piercings, and signs of abuse or self-inflicted injury (ie, cut-
is influenced by the current cognitive and developmental stage of ting) also should be noted. The oropharynx should be examined for
the patient. any evidence of gingivitis or other signs of poor dental hygiene or
malocclusion. The neck should be palpated for adenopathy and the
Psychosocial History thyroid gland for hypertrophy or nodules, especially in adolescent
The psychosocial component of the interview should be conducted females. The back should be examined for any evidence of scolio-
with older children or adolescents alone as well as together with par- sis, which is important to diagnose during this time of rapid growth.
ents or guardians after the issue of confidentiality has been reviewed Assessment of the pubertal development of the breasts and geni-
(see Box 38.2). General questions about school, outside activities or talia in preadolescent or adolescent females and the genitalia, includ-
hobbies, and family are often less threatening than inquiries about ing presence of pubic hair, in adolescent males is essential. The sexual
friends and high-risk behavior such as tobacco use. More sensitive maturity rating (SMR) (ie, Tanner stage) can then be correlated with
topics relating to drug use, sexuality, gender identification, sexual ori- other signs of puberty, such as the appearance of acne and body odor.
entation, and sexual activity should be addressed confidentially after For example, the adolescent female with SMR 4 breasts and imma-
parents or guardians have left the room. Subjects initially discussed ture pubic hair distribution may have an underlying problem, such
with parents should be reviewed once again with teenagers alone. as complete androgen insensitivity syndrome (also called testicular
A useful tool for conducting the psychosocial interview has feminization syndrome).
been developed and refined by physicians who specialize in pediat­ The abdomen should be palpated for organomegaly and the tes-
rics and adolescent medicine. Known by the acronym HEADSS, ticles for masses, hydroceles, hernias, or varicoceles. Lesions such
it reviews the essential components of the psychosocial history: as warts or vesicles also should be documented. The external female
home, employment and education, activities, drugs, sexuality, and genitalia should be inspected for similar lesions and to document
suicide/depression (see Chapter 4). Additional inquiries should be Tanner stage development. A speculum examination should be per-
made about social media usage, including its influence on sleep formed in females who are sexually active and report vaginal dis-
hygiene. Some authors have suggested that this should be the third charge, unexplained vaginal bleeding, or lower abdominal pain. (See
S in the HEADSS acronym. Chapter 58 for additional indications for a pelvic examination.) A
speculum examination is otherwise not indicated in an asymptom-
Dietary History atic sexually active female. In general, virginal girls with normal
A general dietary history should be obtained, with particular focus pubertal development do not require a speculum examination; gen-
on eating habits, level of physical activity, and body image. Dietary tle inspection of the external genitalia is adequate in most cases, with
restrictions, if any, should be investigated to assess for possible defi- special attention to the SMR and hymenal patency. A rectal examina-
ciencies in minerals and vitamins as well as the presence of disor- tion is generally reserved for patients with chronic abdominal pain
dered eating. Daily calcium, vitamin D, and iron intake should be or other specific acute gastrointestinal symptoms.
reviewed, especially in adolescent females. Adolescent males should
be asked about nutritional supplements. Immunizations
Many recent modifications have been made to the preadolescent/
Family History adolescent vaccination schedule (Table 38.1). As always, health
Significant illnesses, such as hypertension, hyperlipidemia, obe- professionals should verify that patients have completed the primary
sity, and diabetes, in first- and second-degree family members immunization series. If not, they should be given catch-up doses
should be reviewed. Family use of alcohol, tobacco, and illegal as according to the most recent Advisory Committee on Immunization
well as prescribed substances also should be determined. Age and Practices and Centers for Disease Control and Prevention recom-
cause of death in immediate family members should be recorded. mendations. The tetanus and diphtheria toxoids and acellular pertus-
sis (Tdap) (eg, Adacel, Boostrix), human papillomavirus (HPV) (eg,
Medications and Allergies Gardasil 9), and meningococcal conjugate (MCV4) (eg, Menactra,
Prescription as well as nonprescription (over-the-counter) medica- Menveo) vaccines should be given to preteens at the 11- to-12-year
tions, herbs, and supplements should be reviewed along with the visit. The Tdap vaccine has replaced the tetanus/diphtheria booster
indications and frequency of usage. previously given at this age. Pertussis was added to the booster
because immunity to pertussis has been noted to wane 5 to 8 years
Physical Examination after vaccination, and there has been an increasing prevalence of
The height and weight of patients should be plotted on a growth curve, pertussis detected in adolescents and adults with chronic cough in
with particular attention paid to the velocity of growth and body mass many communities. A conjugate vaccine against Neisseria meningiti-
index (weight [kg]/(height [m])2). Blood pressure also should be noted dis (MCV4) was approved by the US Food and Drug Administration
and compared with age- and height-related reference values. in 2005. The Advisory Committee on Immunization Practices rec-
Aspects of the physical examination that are influenced by ommends that MCV4 be given to all 11- to 18-year-olds. Although
puberty should be emphasized. The skin should be carefully there are 3 different vaccines (ie, Gardasil, Cervarix, and Gardasil 9)
inspected for acne and hirsutism; clinicians should offer treatment available that include protection against 2 of the HPV types that

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 262 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 38.1. Recommended Immunization Schedule for individuals 10 years and older who are at high risk for serogroup
Affecting Adolescents B meningococcal disease, such as those with anatomical or func-
tional asplenia or persistent complement deficiencies. Adolescents
Recommended Age (years)
and young adults aged 16 to 23 years also may be vaccinated to pro-
Vaccine Type 11–12 13–18 vide short-term protection during serogroup B meningococcal dis-
Tetanus, diphtheria, Tdap Tdap (catch-up) ease outbreaks. Pneumococcal vaccine should be offered to high-risk
pertussis groups, such as those with chronic lung disease, cyanotic congenital
HPV HPV (2 doses) HPV (catch-up) (2 or 3 doses)a heart disease, and diabetes mellitus. In addition, a Mantoux skin test
Meningococcal MCV4 MCV4 (booster at 16 years) for tuberculosis should be performed if the adolescent resides in a
high-risk environment. A tuberculosis blood test (also called an inter-
Meningococcal Individual clinical decision at age
feron gamma release assay) is preferred if the patient has received
serogroup B 16–23 years if not at increased risk
the tuberculosis or BCG vaccine or has a difficult time returning for
Varicella Varicella 2-dose series a second appointment to look for a reaction to the Mantoux skin test.
Influenza Influenza annually (For complete recommendations, see Chapter 37.)
Abbreviations: HPV, human papillomavirus; MCV4, meningococcal conjugate vaccine; Tdap, tetanus
and diphtheria toxoids and acellular pertussis.
Laboratory Tests
a
2- or 3-dose series depending on age at initial vaccination (see Chapter 37). A hemoglobin level should be obtained to evaluate for anemia.
Modified from Centers for Disease Control and Prevention. Child and adolescent immunization Although previously included in laboratory screening, a urinalysis
schedule (birth through 18 years). https://www.cdc.gov/vaccines/schedules. Reviewed February 5, is no longer recommended to assess for protein, blood, and pyuria
2019. Accessed September 2, 2019. because most abnormal findings resolve spontaneously. Other sug-
gested screening tests include hearing and vision tests and a cho-
cause most cervical cancers (oncogenic types 16 and 18), only the lesterol and lipid profile, once between 9 and 11 years and a second
9-valent product is currently used in the United States. Licensed in time between 17 and 21 years of age.
2015, Gardasil 9 protects against 5 additional HPV types that cause In addition to these laboratory tests, sexually active adolescents
an additional 10% of HPV-associated cancers in the United States. should be screened for STIs. If a pelvic examination is performed, an
While there has been much publicity and some controversy sur- endocervical specimen should be obtained for nucleic acid amplifica-
rounding the HPV vaccine, current recommendations state that all tion testing for gonorrhea and chlamydia. However, if a pelvic exami-
adolescents should begin the HPV vaccination series routinely at nation is not indicated, routine screening for gonorrhea and chlamydia
11 to 12 years of age with the goal of completing the series by age may be performed with a urine or vaginal sample alone using nucleic
13 years. The vaccine is approved for patients as young as 9 years. For acid amplification testing methods. The 2015 recommendations from
those who initiate the series at 9 to 14 years of age, a 2-dose series is the Centers for Disease Control and Prevention state that all sexually
administered rather than the 3-dose series for those who begin vac- active women younger than 25 years should be screened annually.
cination at age 15. Gardasil 9 also should be routinely administered Males should be screened in high-prevalence clinical settings, such as
to young adults through the age of 26 years who have not received adolescent clinics, correctional facilities, or STI clinics; if they are symp-
the vaccine. Ideally, the vaccine should be administered before the tomatic; if they have a history of multiple partners and unprotected
initiation of sexual activity because Gardasil 9 is only preventive and intercourse; or if they are having sex with men. In addition, a rapid
does not treat or cure HPV infection, dysplasia, or cancer that has plasma reagin test for syphilis and an HIV test should be obtained, espe-
already developed in response to HPV exposure. However, regard- cially if another STI is suspected or confirmed. All these tests should
less of previous sexual exposure, the HPV vaccine should be admin- be offered in the clinically appropriate setting after patients have
istered to all adolescents, even if they are already sexually active. received adequate education on STIs, with a follow-up visit scheduled to
Recommendations concerning some of the older, traditional vac- discuss the results.
cines have changed, as have the catch-up schedules. The adolescent
(13 years and older) with no evidence of immunity to varicella should Patient Education
receive 2 doses of the vaccine at least 4 weeks apart. If an adolescent At the conclusion of the health maintenance visit, positive as well
or preadolescent has received only 1 dose of the varicella vaccine, a as negative findings should be reviewed with patients and their par-
second dose should be administered. Routine vaccination against ents or guardians. Depending on the nature of these findings and
hepatitis B also is recommended, regardless of sexual activity, if it the age of the patient, the health professional may initially choose to
has not been administered previously. The 2-dose hepatitis A series address these findings with the patient alone, keeping in mind issues
should be given to all teenagers not previously vaccinated if they of confidentiality. All recommended screening laboratory studies
reside in high-incidence communities. Influenza vaccine should be and immunizations should be reviewed before their administra-
given annually to all infants 6 months and older, children, and ado- tion, including the need for further follow-up. Subsequent vaccine
lescents and to those who come into close contact with individuals doses must be outlined for patients and parents or guardians. The
with high-risk conditions. Two meningococcal serogroup B vaccines timing of the next visit and reasons for this visit should be discussed.
(ie, Bexsero, Trumenba) are currently licensed for use among per- The remainder of the health maintenance visit should be spent
sons aged 10 to 25 years in the United States and are used routinely addressing any specific concerns of patients and parents or guardians,

BPPCA6e_Ch038_0259-0270.indd 262 2/13/20 5:34 PM

 Chapter 38: Health Maintenance in Older Children and Adolescents 263

highlighting health care problems (eg, obesity, high blood pressure),

Box 38.3. What to Ask
and identifying any factors that may be contributing to high-risk
behavior, such as drug or alcohol use. Older children or adoles- Preparticipation Physical Evaluation
cents who are not participating in any deleterious activities should ww What sport(s) does the child or adolescent wish to participate in? Has he
be praised for their positive behavior as well as provided with edu- or she participated in this sport in the past?
cational information such as materials addressing injury prevention
ww Has the child or adolescent ever experienced a sports injury? If so, how much
and sleep hygiene.
time did the athlete refrain from sports activities as a result of this injury?
ww Has the athlete ever experienced a lapse of consciousness or concussion?
Preparticipation Physical Evaluation for
ww Does the child or adolescent have a significant underlying health problem?
School-age and Adolescent Athletes
ww Is the child or adolescent taking any prescribed or over-the-counter
The preparticipation physical evaluation (PPE) is essentially the medications, supplements, or caffeine?
“sports physical” that many schools require for participation in orga-
ww Does the child or adolescent have any allergies?
nized athletic programs. The primary objective of the PPE is to assess
ww Has the child or adolescent ever had syncope or near-syncope, palpitations,
the athlete’s readiness to compete safely and effectively in training
chest pain, discomfort, or shortness of breath during exercise or at rest?
and competition. Ideally, it also should identify athletes at risk of
injury, reinjury, or sudden death, as well as those with an underly- ww Does the child or adolescent have a family history of sudden, early, nontrau-
ing medical condition that may preclude safe athletic participation. matic deaths in a first- or second-degree relative younger than 50 years?
Historically, controversy existed about the appropriate location
for performance of the PPE. Community physicians were often asked syncope, near-syncope, chest pain, palpitations, and excessive short-
to perform limited en masse examinations at schools, or a group of ness of breath or fatigue with exertion.
clinicians was asked to perform the examinations in the gymna-
sium using “stations.” Either way, the patient did not truly receive a Physical Examination
complete physical examination or assessment, and neither approach A complete physical examination should be performed. If cir-
lent itself to privacy. In addition, parents had a false sense of secu- cumstances preclude this, specific attention should be paid to the
rity and believed that their children had received adequate medical eyes, heart, abdomen, skin, and musculoskeletal system. Height,
care. The AAP, in conjunction with other professional organizations, weight, blood pressure, and visual acuity also should be measured.
has developed a monograph that includes guidelines for the PPE. Examination of the eyes is essential to document physiological aniso-
Ideally, primary care physicians should perform the PPE annually coria (different papillary diameters). A thorough cardiac evaluation
in their office during a scheduled visit at least 4 to 6 weeks before for murmurs, abnormal heart sounds, or arrhythmias should be
the beginning of the athletic season. Pediatricians can also use this performed with the patient supine and again standing or straining
required visit as an opportunity to perform an annual comprehensive during the Valsalva maneuver. The abdomen should be palpated for
health maintenance examination on older children and adolescents, an enlarged liver or spleen, especially in the adolescent with a recent
including providing important anticipatory guidance, administer- viral illness that could suggest mononucleosis. In males, the genita-
ing catch-up immunizations, and performing the various screening lia should be examined for sexual maturity in addition to assessing
tests recommended for this age group. for abnormalities, such as atrophy, absence of a testis, or presence
of a testicular mass or inguinal hernia. The skin should be inspected
History for lesions, such as tinea corporis, molluscum contagiosum, scabies,
The most challenging aspect of the PPE is reviewing the past medi- impetigo, or herpes simplex infection.
cal and family history with the athlete to uncover previously unrec- The 2-minute orthopedic examination consists of a head-to-toe
ognized abnormalities of the cardiovascular system that warrant assessment of all muscle groups and joints; any deformities, anom-
further investigation by a cardiologist prior to participating in a alies, or evidence of previous injuries should be noted (Table 38.2).
given sport. Red flags include a history of congenital heart dis- Recent studies suggest expanding this examination to include a more
ease, cardiac channelopathies (eg, long QT syndrome), a history detailed evaluation of areas at high risk of injury, such as the knee,
of Kawasaki disease and associated coronary artery anomalies, ankle, and shoulder.
and a history of myocarditis. The rest of the medical history for the
PPE should focus on previous athletic participation and any cur- Laboratory Tests
rent or past injuries that have required immediate evaluation and Routine laboratory screening tests, except for those performed
subsequent bracing, casting, surgery, or missed practice or play during the general health maintenance visit, are not recommended
(Box 38.3). A standard questionnaire codeveloped by the AAP for for the PPE. Screening young athletes for anemia or proteinuria
this purpose may be used in the office setting. In addition, many has not been found to be particularly helpful. Such screening may
health professionals record the results of the physical examination be useful with highly competitive professional athletes, however.
as well as their recommendations for the degree of athletic partici- Although controversial, some groups believe that electrocardio­
pation on this standard form (Figures 38.1 through 38.4). A review graphy and echocardiography should be routine components of
of systems should specifically include an inquiry about a history of the PPE as well.

BPPCA6e_Ch038_0259-0270.indd 263 2/13/20 5:34 PM

 264 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

■ PREPARTICIPATION PHYSICAL EVALUATION

HISTORY FORM
Note: Complete and sign this form (with your parents if younger than 18) before your appointment.
Name: ________________________________________________________________ Date of birth: _____________________________
Date of examination: _______________________________ Sport(s): _____________________________________________________
Sex assigned at birth (F, M, or intersex): _________________ How do you identify your gender? (F, M, or other): ___________________

List past and current medical conditions. _____________________________________________________________________________

_______________________________________________________________________________________________________________
Have you ever had surgery? If yes, list all past surgical procedures. _______________________________________________________
_______________________________________________________________________________________________________________
Medicines and supplements: List all current prescriptions, over-the-counter medicines, and supplements (herbal and nutritional).
_______________________________________________________________________________________________________________
_______________________________________________________________________________________________________________
Do you have any allergies? If yes, please list all your allergies (ie, medicines, pollens, food, stinging insects).
_______________________________________________________________________________________________________________
_______________________________________________________________________________________________________________

Patient Health Questionnaire Version 4 (PHQ-4)

Over the last 2 weeks, how often have you been bothered by any of the following problems? (Circle response.)
Not at all Several days Over half the days Nearly every day
Feeling nervous, anxious, or on edge 0 1 2 3
Not being able to stop or control worrying 0 1 2 3
Little interest or pleasure in doing things 0 1 2 3
Feeling down, depressed, or hopeless 0 1 2 3
(A sum of ≥3 is considered positive on either subscale [questions 1 and 2, or questions 3 and 4] for screening purposes.)

GENERAL QUESTIONS HEART HEALTH QUESTIONS ABOUT YOU

(Explain “Yes” answers at the end of this form. (CONTINUED ) Yes No
Circle questions if you don’t know the answer.) Yes No 9. Do you get light-headed or feel shorter of breath
1. Do you have any concerns that you would like to than your friends during exercise?
discuss with your provider?
2. Has a provider ever denied or restricted your 10. Have you ever had a seizure?
participation in sports for any reason?
HEART HEALTH QUESTIONS ABOUT YOUR FAMILY Yes No
3. Do you have any ongoing medical issues or
11. Has any family member or relative died of heart
recent illness?
problems or had an unexpected or unexplained
HEART HEALTH QUESTIONS ABOUT YOU Yes No sudden death before age 35 years (including
4. Have you ever passed out or nearly passed out drowning or unexplained car crash)?
during or after exercise?
5. Have you ever had discomfort, pain, tightness, 12. Does anyone in your family have a genetic heart
or pressure in your chest during exercise? problem such as hypertrophic cardiomyopathy
(HCM), Marfan syndrome, arrhythmogenic right
6. ventricular cardiomyopathy (ARVC), long QT
or skip beats (irregular beats) during exercise? syndrome (LQTS), short QT syndrome (SQTS),
7. Has a doctor ever told you that you have any Brugada syndrome, or catecholaminergic poly-
heart problems? morphic ventricular tachycardia (CPVT)?

8. Has a doctor ever requested a test for your

13. Has anyone in your family had a pacemaker or
heart? For example, electrocardiography (ECG)
or echocardiography.

Figure 38.1. Preparticipation Physical Evaluation: History Form.

BPPCA6e_Ch038_0259-0270.indd 264 2/13/20 5:34 PM

 Chapter 38: Health Maintenance in Older Children and Adolescents 265

BONE AND JOINT QUESTIONS Yes No MEDICAL QUESTIONS (CONTINUED ) Yes No

14. Have you ever had a stress fracture or an injury 25. Do you worry about your weight?
to a bone, muscle, ligament, joint, or tendon that 26. Are you trying to or has anyone recommended
caused you to miss a practice or game? that you gain or lose weight?
15. Do you have a bone, muscle, ligament, or joint 27. Are you on a special diet or do you avoid
injury that bothers you? certain types of foods or food groups?
MEDICAL QUESTIONS Yes No 28. Have you ever had an eating disorder?
16. FEMALES ONLY Yes No
breathing during or after exercise?
29. Have you ever had a menstrual period?
17. Are you missing a kidney, an eye, a testicle
30.
(males), your spleen, or any other organ?
menstrual period?
18. Do you have groin or testicle pain or a painful
31. When was your most recent menstrual period?
bulge or hernia in the groin area?
32. How many periods have you had in the past 12
19. Do you have any recurring skin rashes or
months?
rashes that come and go, including herpes or
methicillin-resistant Staphylococcus aureus
Explain “Yes” answers here.
(MRSA)?
______________________________________________________
20. Have you had a concussion or head injury that ______________________________________________________
caused confusion, a prolonged headache, or
______________________________________________________
memory problems?
______________________________________________________
21. Have you ever had numbness, had tingling, had
weakness in your arms or legs, or been unable ______________________________________________________
to move your arms or legs after being hit or ______________________________________________________
falling? ______________________________________________________
22. Have you ever become ill while exercising in the ______________________________________________________
heat? ______________________________________________________
23. Do you or does someone in your family have ______________________________________________________
sickle cell trait or disease? ______________________________________________________
24. Have you ever had or do you have any prob- ______________________________________________________
lems with your eyes or vision? ______________________________________________________

I hereby state that, to the best of my knowledge, my answers to the questions on this form are complete
and correct.
Signature of athlete: ______________________________________________________________________________________________________
Signature of parent or guardian: __________________________________________________________________________________________
Date: ________________________________________________________

© 2019 American Academy of Family Physicians, American Academy of Pediatrics, American College of Sports Medicine, American Medical Society for Sports Medicine,
American Orthopaedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine. Permission is granted to reprint for noncommercial, educa-
tional purposes with acknowledgment.

Figure 38.1. Preparticipation Physical Evaluation: History Form. (continued)

BPPCA6e_Ch038_0259-0270.indd 265 2/13/20 5:34 PM

 266 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

■ PREPARTICIPATION PHYSICAL EVALUATION

ATHLETES WITH DISABILITIES FORM: SUPPLEMENT TO THE ATHLETE HISTORY
Name: _________________________________________________________________ Date of birth: ____________________________

1. Type of disability:
2. Date of disability:
3.
4. Cause of disability (birth, disease, injury, or other):
5. List the sports you are playing:
Yes No
6. Do you regularly use a brace, an assistive device, or a prosthetic device for daily activities?
7. Do you use any special brace or assistive device for sports?
8. Do you have any rashes, pressure sores, or other skin problems?
9. Do you have a hearing loss? Do you use a hearing aid?
10. Do you have a visual impairment?
11. Do you use any special devices for bowel or bladder function?
12. Do you have burning or discomfort when urinating?
13.
14. Have you ever been diagnosed as having a heat-related (hyperthermia) or cold-related (hypothermia) illness?
15. Do you have muscle spasticity?
16. Do you have frequent seizures that cannot be controlled by medication?
Explain “Yes” answers here.
_________________________________________________________________________________________________________________
_________________________________________________________________________________________________________________
_________________________________________________________________________________________________________________
Please indicate whether you have ever had any of the following conditions:
Yes No
Atlantoaxial instability
Radiographic (x-ray) evaluation for atlantoaxial instability
Dislocated joints (more than one)
Easy bleeding
Enlarged spleen
Hepatitis
Osteopenia or osteoporosis
Dif

Numbness or tingling in arms or hands

Numbness or tingling in legs or feet
Weakness in arms or hands
Weakness in legs or feet
Recent change in coordination
Recent change in ability to walk

Latex allergy
Explain “Yes” answers here.
_________________________________________________________________________________________________________________
_________________________________________________________________________________________________________________
_________________________________________________________________________________________________________________
I hereby state that, to the best of my knowledge, my answers to the questions on this form are complete and correct.
Signature of athlete: ______________________________________________________________________________________________________
Signature of parent or guardian: ______________________________________________________________________________________________
Date: _________________________________________________________
© 2019 American Academy of Family Physicians, American Academy of Pediatrics, American College of Sports Medicine, American Medical Society for Sports Medicine, American
Orthopaedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine. Permission is granted to reprint for noncommercial, educational purposes with
acknowledgment.

Figure 38.2. Preparticipation Physical Evaluation: Athletes with Disabilities Form: Supplement to the Athlete History.

BPPCA6e_Ch038_0259-0270.indd 266 2/13/20 5:34 PM

 Chapter 38: Health Maintenance in Older Children and Adolescents 267

■ PREPARTICIPATION PHYSICAL EVALUATION

PHYSICAL EXAMINATION FORM
Name: _________________________________________________________________ Date of birth: ____________________________

PHYSICIAN REMINDERS
1. Consider additional questions on more-sensitive issues.
• Do you feel stressed out or under a lot of pressure?
• Do you ever feel sad, hopeless, depressed, or anxious?
• Do you feel safe at your home or residence?
• Have you ever tried cigarettes, e-cigarettes, chewing tobacco, snuff, or dip?
• During the past 30 days, did you use chewing tobacco, snuff, or dip?
• Do you drink alcohol or use any other drugs?
• Have you ever taken anabolic steroids or used any other performance-enhancing supplement?
• Have you ever taken any supplements to help you gain or lose weight or improve your performance?
• Do you wear a seat belt, use a helmet, and use condoms?
2. Consider reviewing questions on cardiovascular symptoms (Q4–Q13 of History Form).

EXAMINATION
Height: Weight:
BP: / ( / ) Pulse: Vision: R 20/ L 20/ Corrected: Y N
MEDICAL NORMAL ABNORMAL FINDINGS
Appearance
• Marfan stigmata (kyphoscoliosis, high-arched palate, pectus excavatum, arachnodactyly, hyperlaxity,

Eyes, ears, nose, and throat

• Pupils equal
• Hearing
Lymph nodes
Hearta
• Murmurs (auscultation standing, auscultation supine, and ± Valsalva maneuver)
Lungs
Abdomen
Skin
• Herpes simplex virus (HSV), lesions suggestive of methicillin-resistant Staphylococcus aureus (MRSA), or
tinea corporis
Neurological
MUSCULOSKELETAL NORMAL ABNORMAL FINDINGS
Neck
Back
Shoulder and arm
Elbow and forearm

Hip and thigh

Knee
Leg and ankle
Foot and toes
Functional
• Double-leg squat test, single-leg squat test, and box drop or step drop test
a

nation of those.
Name of health care professional (print or type): ___________________________________________________ Date: ___________________
Address: ________________________________________________________________________ Phone: ___________________________
Signature of health care professional: _____________________________________________________________________, MD, DO, NP, or PA

© 2019 American Academy of Family Physicians, American Academy of Pediatrics, American College of Sports Medicine, American Medical Society for Sports Medicine,
American Orthopaedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine. Permission is granted to reprint for noncommercial, educa-
tional purposes with acknowledgment.

Figure 38.3. Preparticipation Physical Evaluation: Physical Examination Form.

BPPCA6e_Ch038_0259-0270.indd 267 2/13/20 5:34 PM

 268 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

■ PREPARTICIPATION PHYSICAL EVALUATION

MEDICAL ELIGIBILITY FORM

Name: _______________________________________________________ Date of birth: _________________________

Medically eligible for all sports without restriction

Medically eligible for all sports without restriction with recommendations for further evaluation or treatment of

__________________________________________________________________________________________________

__________________________________________________________________________________________________
Medically eligible for certain sports

__________________________________________________________________________________________________

__________________________________________________________________________________________________

Not medically eligible pending further evaluation

Not medically eligible for any sports

Recommendations: ___________________________________________________________________________________

__________________________________________________________________________________________________

__________________________________________________________________________________________________

I have examined the student named on this form and completed the preparticipation physical evaluation. The athlete does not have
apparent clinical contraindications to practice and can participate in the sport(s) as outlined on this form. A copy of the physical

arise after the athlete has been cleared for participation, the physician may rescind the medical eligibility until the problem is resolved
and the potential consequences are completely explained to the athlete (and parents or guardians).

Name of health care professional (print or type): __________________________________________ Date: ____________________________

Address: _________________________________________________________________________ Phone: ___________________________

Signature of health care professional: _____________________________________________________________________, MD, DO, NP, or PA

SHARED EMERGENCY INFORMATION

Allergies: ____________________________________________________________________________________________

__________________________________________________________________________________________________
__________________________________________________________________________________________________
Medications: ________________________________________________________________________________________

__________________________________________________________________________________________________

__________________________________________________________________________________________________

Other information: ____________________________________________________________________________________

__________________________________________________________________________________________________

__________________________________________________________________________________________________

Emergency contacts: ___________________________________________________________________________________

__________________________________________________________________________________________________

__________________________________________________________________________________________________

© 2019 American Academy of Family Physicians, American Academy of Pediatrics, American College of Sports Medicine, American Medical Society for Sports Medicine,
American Orthopaedic Society for Sports Medicine, and American Osteopathic Academy of Sports Medicine. Permission is granted to reprint for noncommercial, educa-
tional purposes with acknowledgment.

Figure 38.4. Preparticipation Physical Evaluation: Medical Eligibility Form.

BPPCA6e_Ch038_0259-0270.indd 268 2/13/20 5:34 PM

 Chapter 38: Health Maintenance in Older Children and Adolescents 269

Table 38.2. The 2-Minute Orthopedic Examination different pace. A stepwise graduated return-to-sport program that
was recently updated by the Berlin Concussion in Sport Group allows
Instructions Points of Observation the athlete to gradually progress through 5 stages a day provided
Stand facing examiner. Acromioclavicular joints, general there is no increase in symptoms during exercise. Contrary to pre-
habitus vious reports, prolonged inactivity is known to result in a higher
Look at ceiling, floor, over both Cervical spinal motion symptom level and prolonged recovery. Athletes with more signif-
shoulders; touch ears to shoulders. icant injuries, however, may require several weeks to completely
Shrug shoulders (examiner resists). Trapezius strength recover if the concussion is severe. The short- and long-term effects
of sports-related concussions and repetitive head impacts over the
Abduct shoulders 90°. Deltoid strength
life span of athletes of all ages are still under intense investigation in
Full external rotation of arms. Shoulder motion an effort to create appropriate return-to-play criteria and to reduce
Flex and extend elbows. Elbow motion cognitive, emotional, behavioral, and neurologic consequences.
Arms at sides, elbows flexed to 90°; Elbow and wrist motion Findings discovered during the physical examination, such as
pronate and supinate wrists. severe myopia, strabismus, lens subluxation and stature consistent
Spread fingers; make fist. Hand or finger motion and with Marfan syndrome, a cardiac arrhythmia, or the midsystolic
deformities click of mitral valve prolapse, could preclude the adolescent from
participation in a particular sport. Specific conditions, such as the
Tighten (contract) quadriceps; relax Symmetry and knee effusion; ankle
athlete with 1 kidney, should be evaluated on an individual basis
quadriceps. effusion
by a physician qualified to assess the safety of the particular sport
Duckwalk 4 steps (away from exam- Hip, knee, and ankle motion for the athlete (ie, contact/collision sport vs limited contact sport).
iner with buttocks on heels). Special circumstances to consider during the PPE are amenor-
Back to examiner. Shoulder symmetry, scoliosis rhea and the female athlete, exercise-induced bronchospasm, ana-
Knees straight, touch toes. Scoliosis, hip motion, hamstring bolic steroid use, and eating disorders that may be associated with
tightness certain activities, such as gymnastics, ballet, and wrestling.
Raise up on toes, raise heels. Calf symmetry, leg strength

CASE RESOLUTION
Exclusion Criteria The young adolescent should first be interviewed with the parent and then
alone. Her medical and psychosocial history should be reviewed. A complete
The most common causes of unexpected death during athlet- physical examination should be performed as well as a pelvic examination if
ics include undiagnosed cardiomyopathies, anomalous coronary she is sexually active and has a history of lower abdominal pain, abnormal vag-
arteries, heart valve defects, primary cardiac rhythm disorders, and inal bleeding, or vaginal discharge. If she is sexually active and asymptomatic or
pulmonary hypertension. Although most assessments are within not sexually active, only general laboratory screening tests should be performed
and the results reviewed with the patient. The remainder of the visit should be
reference range, an important part of the PPE is geared toward spent discussing issues such as nutrition, exercise, illicit substance use, sexuality
determining if a patient has risk factors for any of these condi- and sexual activity, and safety. Results of the physical examination and screening
tions. Medical exclusion criteria for athletic participation are based tests should then be discussed with the parent or guardian who accompanied her
on information obtained in the medical as well as family history. to the office. If necessary, a follow-up visit should be scheduled. Otherwise, the
Significant historical clues include a family history of sudden, non- adolescent should be seen annually.
traumatic death; premature coronary artery disease in a first- or
second-degree relative; a history of palpitations, chest discomfort,
or syncope during exercise; and recent, documented infection with
Epstein-Barr virus. Controversy exists concerning when athletes can
Selected References
return to collision sports after infectious mononucleosis. A history American Academy of Family Physicians, American Academy of Pediatrics,
of a recent or suspected sport-related concussion also requires close American College of Sports Medicine, American Medical Society for Sports
monitoring to address when the athlete is cleared to return to par- Medicine, American Orthopaedic Society for Sports Medicine, American
Osteopathic Academy of Sports Medicine. PPE: Preparticipation Physical
ticipate in a given sport. Current clinical experience and neurocog-
Evaluation. Bernhardt DT, Roberts WO, eds. 5th ed. Itasca, IL: American
nitive research on adolescents and concussions support the mantra, Academy of Pediatrics; 2019
“When in doubt, sit them out!” In other words, the athlete should
American Academy of Pediatrics Committee on Adolescence. Achieving qual-
not be pressured to continue to play through injuries or return to ity health services for adolescents. Pediatrics. 2016;138(2):e20161347 PMID:
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rest, physically and cognitively, until their symptoms have improved Bernstein HH, Bocchini JA Jr; American Academy of Pediatrics Committee on
at rest and with exertion, according to the AAP Council on Sports Infectious Diseases. Practical approaches to optimize adolescent immunization.
Medicine and Fitness. The exact amount and duration of rest should Pediatrics. 2017;139(3):e20164187 PMID: 28167515 https://doi.org/10.1542/
follow an individualized course because each athlete recovers at a peds.2016-4187

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Centers for Disease Control and Prevention. Immunization schedules. Table 1. Institute of Medicine, National Research Council. Sports-Related Concussions in
Recommended child and adolescent immunization schedule for ages 18 years Youth: Improving the Science, Changing the Culture. Washington, DC: National
or younger, United States, 2019. https://www.cdc.gov/vaccines/schedules/hcp/ Academies Press; 2014
child-adolescent.html. Reviewed February 5, 2019. Accessed September 2, 2019 McCambridge TM, Benjamin HJ, Brenner JS, et al; American Academy of
Halstead ME, McAvoy K, Devore CD, Carl R, Lee M, Logan K; American Pediatrics Council on Sports Medicine and Fitness. Athletic participation by
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Halstead ME, Walter KD, Moffatt K; American Academy of Pediatrics Council Peterson AR, Bernhardt DT. The preparticipation sports evaluation. Pediatr
on Sports Medicine and Fitness. Sport-related concussion in children and Rev. 2011;32(5):e53–e65 PMID: 21536775 https://doi.org/10.1542/pir.32-5-e53
adolescents. Pediatrics. 2018;142(6):e20183074 PMID: 30420472 https://doi. Strasburger VC, Jordan AB, Donnerstein E. Health effects of media on children
org/10.1542/peds.2018-3074 and adolescents. Pediatrics. 2010;125(4):756–767 PMID: 20194281 https://doi.
Herman-Giddens ME, Bourdony CJ, Dowshen SA, Reiter EO. Assessment org/10.1542/peds.2009-2563
of Sexual Maturity Stages in Girls and Boys. Elk Grove Village, IL: American
Academy of Pediatrics; 2011

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 CHAPTER 39

Health Care for International

Adoptees
ChrisAnna M. Mink, MD, FAAP

CASE STUDY
Jaxon is a 14-month-old boy adopted from Thailand. The parents placed a call to you from the Bangkok air-
His biological mother was a 26-year-old commercial sex port because Jaxon would not stop crying. They report that
worker who entered a maternity house during her preg- on the morning his foster mother left him with them, he
nancy to receive care and relinquish the baby for adop- cried quite a bit but had settled by bedtime and seemed
tion. His mother reported that she was physically and to be adjusting well during the week. Over the 12 hours
sexually abused as a child and became a street child at preceding the telephone consultation, however, he had
age 14 years. She used illicit drugs 5 years previously but not stopped crying, and he refuses to eat. He has been
none since. She identifies the father as a European cus- drooling, and they question if his discomfort is related to
tomer but has no other information. Jaxon was born at teething; they have not noticed any other symptoms of
32 weeks’ gestational age and was placed in an incuba- teething, however. They are gravely concerned that he
tor but did not have any respiratory problems. He has does not like them and is having attachment difficulties.
been in foster care in the home of a Thai family with his
care supervised by an internationally respected adoption
Questions
1. What factors influence the prevalence of interna-
organization. He was selected by his parents at the age
tional adoption?
of 4 months, and they have received monthly progress
2. What are some of the potential health problems of
reports on his growth, development, and medical status.
the international adoptee?
Reportedly, he has had several “colds” and 1 ear infection
3. What is an appropriate medical evaluation for the
but otherwise has been growing and developing well.
international adoptee?
Before departure to pick up Jaxon, his adoptive parents
4. What is the role of the pediatrician in caring for the
met with you to prepare for his arrival.
child and newly formed family?

Although internationally adopted children come from a wide range governing adoption in an attempt to curb corruption, including The
of birth countries, many of their health-related issues are similar. Hague Convention on the Protection of Children and Co-operation
Many of the children have lived in orphanages in impoverished areas in Respect of Intercountry Adoption, which is an international agree-
of the developing world and have incurred the maladies associated ment for standardizing intercountry practices to promote protection
with poverty and deprivation. of children available for adoption.
Many factors influence the choice of international adoption. In
Epidemiology the United States, delays in childbearing and associated infertility
From 1999 to 2012, 242,602 children were adopted internation- have increased the demand for adoptable children. Simultaneously,
ally into the United States; this is nearly the same number as the more readily available birth control and growing acceptance of single
previous 30 years combined. Through the first decade of the motherhood have resulted in a decreased number of infants avail-
21st century, 90% of such children were from Asia, most com- able for adoption. In addition to the shortage of adoptable children
monly China and South Korea; Eastern Europe; and South America in the United States, other factors in the decision to pursue interna-
(Guatemala and Colombia). From 2009 to 2012, China, Ethiopia, and tional adoption include real and perceived risks of domestic adop-
Russia were the 3 leading countries of origin for children adopted tion (eg, failure of birth parents to relinquish rights), reluctance to
into the United States. The number of international adoptions has adopt a child with special needs or with in utero drug exposure,
been declining since 2009, with a nadir in 2017 of only 4,714 adop- and limited availability of children with desired traits (eg, specific
tees entering the United States. This decline is the result of multiple age and ethnicity [often white infants]). The prompt termination
factors, including economic uncertainty in the United States; chang- of rights of birth parents in international adoption is also cited as a
ing geopolitical landscapes in birth countries; and stricter policies factor in this decision.
271

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 272 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

The advent of intercountry adoption in the United States occurred Until the 1990s, most international adoptees were from South
in conjunction with World War II and the large number of orphaned Korea, which had in place an excellent foster care system and health
children in Europe, many of whom were fathered by American sol- care. Since the 1990s, most adoptees come from institutions in poor
diers. The second—and more formalized—wave of intercountry nations without a developed foster care system, resulting in a signif-
adoption occurred with the Korean War. Because of the need to care icant decline in the health and well-being of adoptees.
for unwanted orphans, primarily of mixed ethnicities and fathered
by American soldiers, South Korea established a foster care system Clinical Presentation
and the children became available for adoption to Americans. War
Preadoption
and political turmoil remain factors in the availability of children for
adoption. For example, the fall of communism was a significant fac- Some adoptees become known to the US health professional “only
tor in Russia and other states of the former Soviet Union becoming on paper” during the preadoption stage. The adoptive parent or par-
common birth countries for adoptees in the 1990s and the early part ents may ask their physician for help in assessing the child’s medi-
of the 21st century. Following a diplomatic rift, however, in 2013 the cal status. Often a parent receives a written health report (varying in
Russian government outlawed adoptions to the United States. Early the quantity and quality of information) and photos or videos of the
in the 21st century, because of poverty and political strife, more child under consideration for adopting. The written documents may
children were being adopted from African countries, especially be in a foreign language or not translated by an experienced medi-
Ethiopia. Recently, adoptions from Ethiopia have been stopped, cal translator. Because some countries prohibit international adop-
however, in part because of cultural pride, as well as a highly pub- tion of healthy children, diagnoses may be embellished to improve
licized death resulting from abuse of an Ethiopian child adopted the child’s chances for adoption. Additionally, some medical records
into the United States. contain diagnoses that are nonsensical in US medicine but repre-
Societal values also influence adoption practices. China was sent standard terms used in the country of origin. These inconsis-
among the leading birth countries for adoptees because of the pop- tencies are quite challenging when trying to evaluate the medical
ulation control initiatives of the government mandating that fam- records of potential adoptees. Many physicians may not feel com-
ilies have only 1 child. With this practice and the desire for a male fortable with reviewing medical records given so many limitations;
heir, some newborn girls were abandoned and became available for however, even with all the caveats, review of the records may pro-
adoption. With the 2008 Summer Olympic Games, China had a surge vide valuable insight into the health status of the adoptee.
in national pride and a realization that their future population may During this stage, the physician may provide information for
not include enough girls for the boys to marry. Subsequently, fewer parents and families for preventive health measures to prepare for
infant girls became available for adoption. Currently, China mainly travel to a developing area of the world. The Centers for Disease
permits international adoption of children of both sexes with spe- Control and Prevention (CDC) Traveler’s Health website (www.cdc.
cial medical or developmental needs. gov/travel) is a good resource for physicians and parents. Up-to-
Until 2016, most internationally adopted children were female date information may also be obtained from the World Health
(approximately 56%), in part reflecting the adoption of girls from Organization (WHO) and the US Department of State (Table 39.1).
China, as well as a preference among some adoptive parents (espe- Prospective parents should be informed about the infections that
cially single women) to adopt a girl. Currently, most are male, mainly may occur in international adoptees, and they should receive appro-
reflecting the changes in China’s policies. International adoptees are priate education and preventive measures, including vaccinations
young, with approximately 55% between the ages of 1 and 5 years. (eg, measles, hepatitis A, hepatitis B).
The United States is the birth country of approximately 100 chil-
dren annually adopted into other countries. Absolute statistics for
During the Adoption Trip
US children adopted into other countries are not available because All internationally adopted children are required by the US
the US government does not routinely report the number of exit Department of State to undergo a physical examination before
visas issued for adopted children. These adoptees are often males admission into the country; however, this examination is limited
of African American or mixed ethnicity and are adopted by fami- in scope and performed mainly to rule out severe impairments or
lies in Canada and Western Europe. They are available purportedly certain communicable diseases that may pose a public health threat
because of the low desire for these infants by adoptive parents in (eg, active tuberculosis [TB]). This examination should not be con-
the United States. sidered a complete medical evaluation for an individual child.
The status of the country of origin (eg, war, turmoil, poverty, Some health professionals can provide support for families dur-
societal values) aside, significant overlap exists in the reasons ing travel via e-mail, telephone, and the internet, similar to tele-
that children from foreign countries and from the United States phone consultations performed in general practice.
become available for adoption. The common reasons include
parental substance use, abandonment, chronic neglect, abuse, and Postadoption
domestic violence, all of which often are associated with under- After the adoption, the health status of children on presentation to
lying poverty. the US physician may be quite variable, ranging from well to severely

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 Chapter 39: Health Care for International Adoptees 273

Table 39.1. Websites With Information on International Health, Travel, and Adoption
Resource Information Website(s)
Centers for Disease Control and Prevention Up-to-date information for travelers’ health wwwnc.cdc.gov/travel
Health guidance and immigration process for www.cdc.gov/immigrantrefugeehealth/adoption/index.html
international adoption
US Department of State Up-to-date information for travelers’ risk (eg, https://travel.state.gov/content/passports/english/
civil unrest) alertswarnings.html
Intercountry adoption procedures https://travel.state.gov/content/travel/en/Intercountry-
Adoption/Adoption-Process.html
World Health Organization Health status and recommendations for www.who.int/immunization/policy/immunization_tables/en/
immunizations for each country
Assists with interpreting foreign vaccine
records
US Department of Health & Human Services Adoption information and procedures www.childwelfare.gov
Administration for Children & Families

ill with acute infections or chronic diseases (eg, malnutrition, TB). untreated chronic illness (eg, TB, rickets). Institutionalized chil-
The adopted child should be seen by the physician within 2 to dren may exhibit psychosocial dwarfism and may lose 1 month of
3 weeks of arrival in the United States, or sooner if the child has an linear growth for every 3 to 4 months spent in the orphanage. Delay
acute illness. This 2-week period allows for the child (and parent or in puberty may be observed in adolescents from deprived environ-
parents) to recover from jet lag and become more familiar with each ments, such as orphanages. Precocious puberty may also be seen
other, permitting a better assessment at the visit. If an acute illness among international adoptees.
visit is required, a separate appointment for a comprehensive eval- Immunization records may not be available, may be incom-
uation should be scheduled at a later time. plete, or may be in a foreign language, which hinders assessment
of the vaccination status of adoptees. Many vaccines available in
Health Care Issues the United States are not available in the developing world (eg,
In addition to problems commonly related to poverty and depriva- Haemophilus influenzae type b, pneumococcal conjugates) and,
tion, many health issues are specific to the country or region of ori- thus, children will not have had them. Adopted children immigrat-
gin (eg, increased risk of malaria and other parasites in children ing to the United States who are younger than 10 years are exempt
from the continent of Africa). Adoptees from South Korea have the from the Immigration and Nationality Act regulations requiring
lowest risk for infectious diseases. proof of immunizations before arrival; however, adoptive parents
Generally, health care issues for adoptees are extensive, including are required to sign a waiver that they will comply with US recom-
acute illness (eg, respiratory infections), chronic illness (eg, anemia, mended immunizations after arrival.
malnutrition, poor dental hygiene, TB, asthma, parasite infestation), Psychosocial, emotional, and mental health disorders are some
delayed or unknown immunizations, psychosocial challenges, and of the more challenging problems to assess. The spectrum of men-
impaired growth and development. tal health problems is related to age and previous life experiences of
Some children have assigned birth dates (eg, abandoned infants the child. Children may have experienced physical or sexual abuse
and street children for whom birth dates are not known), and they before placement in an institution, and they may also be subject to
may have small growth parameters, making it difficult to know their abuse by older children or adult caregivers while in institutional
true age and expected development. Developmental delays, most placement. Attachment disorders are among the most concern-
commonly language delay, are frequently identified. Assessment of ing abnormalities for adoptive parents, adoption professionals, and
development may be even more difficult in infants and young chil- health professionals. The fundamentals for learning healthy attach-
dren who are nonverbal and older children who speak their native ments are greatly influenced by early infant-caregiver relationships.
language. Thus, many international adoptees have difficulties bonding, in part
Growth delay is common for adoptees. Many children are mal- because they have not had secure caregiver relationships. Issues of
nourished or exhibit failure to thrive, and these conditions are often attachment and bonding may be especially problematic for chil-
multifactorial in origin, including poor prenatal environment (eg, dren who have resided in an orphanage or had multiple caregiv-
maternal stress, malnutrition, substance abuse), inadequate calo- ers from an early age. Children who have had multiple caregivers
ries, inadequate nurturing, unrecognized genetic or congenital dis- may be indiscriminately friendly, which may pose risks for their
orders (eg, fetal alcohol spectrum disorder; see Chapter 147), and safety. Other common mental health problems include depression,

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 274 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

attention-deficit/hyperactivity disorder, posttraumatic stress dis- recent years, data have emerged to suggest that well-documented
order, abnormal behaviors (eg, self-stimulating behaviors, hoard- immunizations may be considered valid. Written records showing
ing food, sleep disturbances), and oppositional defiant disorder. As the age of the child when vaccinated, date of administration, dose
mentioned previously, communication with the child may be diffi- given, and proper intervals between dosing that are consistent with
cult because of language barriers, causing another obstacle to assess- WHO schedules or are comparable to US schedules may be consid-
ing the child’s mental health. ered acceptable for proof of immunization. (Guidelines for care in
Sensory integration difficulties are increasingly recognized in the absence of vaccine records is discussed in the Management sec-
adoptees. The children may have adverse responses to touch (eg, tion of this chapter.)
new clothing, hugs and kisses, bathing) or textures (eg, new foods). Dietary history is important for assessing the child’s nutritional
Individual senses or all of them (ie, hearing, vision, taste, smell) status. Questions to ask are listed in Box 39.1.
may be notably increased or decreased, and some children have An interim medical history may be available, because many chil-
decreased sensation to physical pain, resulting in an increased risk dren are selected by their adoptive parent or parents several months
for injuries. Dyskinesia in the form of clumsiness or being prone to before immigrating to the United States. The interim medical his-
injury has also been observed. tory may be provided from the orphanage or foster care provider
The most common identified medical issues are infectious dis- through the adoption agency. Parents should be encouraged to solicit
eases, including acute illness (eg, upper respiratory infection, bron- as much information as possible from the child’s caregivers. At a
chitis, otitis, infectious diarrhea) and chronic infection (eg, TB, minimum, this history should include serial growth parameters,
parasite infestations, with scabies and Giardia lamblia common known illnesses, hospitalizations, surgeries, allergies, and immuni-
manifestations of the latter. Because of the lifestyle of their biological zations given while the child was under their care. Parents should
mother and the children’s time residing in institutions, many adoptees also ask caregivers about any food preferences, special fears, toys,
are at increased risk of exposure to infectious diseases, such as syph- or friends from the placement prior to adoption. If the child has a
ilis, HIV, and hepatitis B and C. special “lovey,” the parent or parents should request to bring it with
Preventive care that is considered routine in the United States the child as a transitional object.
is unlikely to have been part of the child’s care and must be per-
formed as appropriate for age. This includes newborn screening Physical Examination
laboratory studies and assessments of hearing, vision, dental, and A complete unclothed physical examination should be performed on
mental health. Anticipatory guidance for new parents should be infants and children of all ages. Because of previous trauma (eg, sex-
incorporated into preadoption encounters and all subsequent visits. ual abuse), however, it may be necessary to perform some parts of
the examination over a series of visits to minimize the possibility of
Evaluation inflicting additional trauma from an examination. All aspects of the
physical examination are essential. Accurate measurements of height,
The initial office visit with the physician should be scheduled for
weight, and, depending on age and size, head circumference should
an extended period because of the complexity of the evaluation
be obtained. Plotting of parameters on the growth curves from WHO
and additional time needed for parental education. If the physi-
or the CDC (compared with birth country) should be used, with few
cian’s schedule does not permit extended visits, ancillary staff (eg,
exceptions. The child should be closely inspected for unusual scars
nurses, dietitians, therapists) may perform parts of the evaluation
or bruises, evidence of fractures (old or recent), rachitic changes, and
and education.
genital or rectal scarring. The skin should be examined for rashes,
Observation of the child’s behavior, development, and interac-
lesions, and a bacille Calmette-Guérin (BCG) scar (typically on the
tions with the adoptive parent or parents and physician is a critical
upper deltoid). Developmental screening should be performed, and
element of the evaluation. Most physicians routinely include such
a more complete developmental assessment should be scheduled at a
observations in their visits, but particular attention to these factors
separate visit (when the child is not distressed or acutely ill). A den-
is necessary for new adoptees. Items to notice include the child’s
tal examination should be included, and referral for a formal dental
demeanor and behavior, such as determining whether the child is
easily engaged or is withdrawn, makes eye contact with the parent
or physician, makes any vocalizations or words (depending on the
Box 39.1. What to Ask
child’s age), plays with toys, is too friendly or is afraid of strangers,
and seeks comfort from the new parent or parents. Dietary History of the International Adoptee
ww What food and formula/milk is the child receiving?
History ww Has the child received adequate calories?
Limited medical information is available from most birth coun- ww Is there known or suspected food intolerance? (For example, lactose
tries, although some exceptions exist (eg, from foster care in South intolerance is more common in Asian ethnicities.)
Korea). Family and birth history are rarely obtainable for adopt­ ww Are there abnormal behaviors associated with food or eating (eg, preoc-
ees. Immunization histories are becoming increasingly available. cupation with food, hoarding, food refusal, gorging)?
Previously, vaccine records were considered unreliable; however, in

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 Chapter 39: Health Care for International Adoptees 275

evaluation likely will be necessary. Screening evaluations of hearing be tested for syphilis (nontreponemal and treponemal antibodies),
and vision should be performed; formal testing may be necessary, hepatitis surface antigen B panel (HBsAg, HBsAg antibody [HBsAb],
depending on the age of the child and ability to cooperate. and hepatitis B core antibody), hepatitis C antibody, and HIV 1 and
2 antibodies. Human immunodeficiency virus polymerase chain
Laboratory Testing reaction may be indicated in some children (eg, those who may not
Laboratory studies should include complete blood cell count, lead make specific antibodies because of malnutrition or immunocom-
levels, and thyroid function testing (iodine is not in many diets in promised status). Stool samples should be sent for ova and para-
Asia) (Box 39.2). Additionally, testing for illnesses associated with site examination and Giardia and Cryptosporidium antigen testing
specific countries of origin, findings on examination (eg, compre- (Box 39.2). Stool for bacterial pathogens (eg, Salmonella, Shigella)
hensive metabolic panel for malnourished child), and as directed should be sent from children from some regions, such as the Indian
by the child’s age (eg, newborn screening for metabolic disorders in subcontinent. Because cytomegalovirus infection is ubiquitous, rou-
newborns and infants) should be performed. Screening tests, if ini- tine testing is not recommended. Testing for acute infection with
tially negative, should be repeated in 3 to 6 months, especially for hepatitis A virus (HAV) by measuring immunoglobulin M anti-HAV
children for whom concerns exist about underlying malnutrition or antibodies should be performed for adoptees from HAV-endemic
of immunocompromised status. areas, because infants and young children may be asymptomatic
Because of the increased risks of exposures, laboratory screening but contagious. Administering HAV vaccine, as recommended in
for infectious diseases should be undertaken. Serum samples should the United States, is not problematic for children who may have had
previous HAV infection.
Tuberculin skin testing (ie, purified protein derivative [PPD])
Box 39.2. Recommended Laboratory Testing should be performed on all children; history of receipt of BCG is
for International Adoptees not a contraindication to skin testing. Bacille Calmette-Guérin vac-
cinations usually are given at birth in most developing nations, and
ww Hepatitis A IgM.
its influence on skin test status is controversial. Generally, BCG
ww Hepatitis B virus serologic testing.
given within the previous 1 to 2 years may contribute to a positive
—— Hepatitis B surface antigen
PPD skin test; however, a positive PPD test is more likely reflective
—— Antibody to hepatitis B surface antigen
of true infection with Mycobacterium with or without active dis-
—— Antibody to hepatitis B core antigen
ease and merits further evaluation. For children 5 years and older,
ww Hepatitis C virus serologic testing.
interferon- release assays (eg, QuantiFERON-TB Gold) are an
ww Syphilis serologic testing.
acceptable screening alternative to PPD testing. Latent TB infec-
—— Nontreponemal test: RPR, VDRL, or ART
tion has been reported in 0.6% to 30% of international adoptees,
—— Treponemal test (MHA-TP or FTA-ABS)
which is not surprising because most adoptees come from areas in
ww HIV 1 and 2 serologic testing.
which TB is endemic.
ww Complete blood cell count with differential and red blood cell indices.
ww Stool examination for ova and parasites (3 specimens).
ww Stool examination for Giardia intestinalis and Cryptosporidium antigen Management
(1–3 specimens).
Counseling for the Transition
ww Additional parasite testing.a
—— Trypanosoma cruzi serology if child is from endemic area Education and preparation for the parent or parents and all family
—— If child has eosinophilia (eosinophil count >450 cells/mm3 with members is a priority. Generally, countries of origin for adopt­
negative ova and parasite stool testing): Strongyloides serology and ees are in the developing world, and parents should prepare for
Schistosoma serology if from endemic area healthy travel for themselves by receiving immunizations and follow-
ww Tuberculin skin test.b ing travel guidelines from the CDC and the US Department of State.
ww Consider antibody testing to select vaccine antigens (if written records Parents should provide consistent structure and boundaries in a
are unreliable).c loving milieu for adoptees. A scheduled regimen may be especially
ww Additional testing: thyroid function tests, lead level, and others as important to previously institutionalized children because it has
directed by history and physical examination (see text). been their way of life, but even children who were not institution-
alized benefit from a predictable routine. Parents should maximize
Abbreviations: ART, automated reagin test; FTA-ABS, fluorescent treponemal antibody absorption; their 1-on-1 interactions with their adoptee while still allowing time
IgM, immunoglobulin M; MHA-TP, microhemagglutination test–Treponema pallidum; RPR, rapid for themselves and other family members—not an easy task with
plasma reagin; VDRL, Venereal Disease Research Laboratories. multiple children. Initially, it may be necessary to temper physical
a
In conjunction with international adoption or infectious diseases specialist.
contact and affection as directed by the child’s tolerance. Parents
b
Repeat at 6 months after initial testing.
c
For children older than 6 months, may check diphtheria, tetanus, and polio; for children older than
should try to enhance bonding and attachment by frequently iden-
12 months, may check measles antibodies. tifying themselves as “Mom” or “Dad.” Other strategies to enhance
Derived from the American Academy of Pediatrics and the Centers for Disease Control and Prevention. attachment include initially limiting contact with individuals outside

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 276 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

the family and not “handing” the child to others, including non- repeating immunizations or checking antibody concentrations to
household family members, because they are strangers to the child. vaccine antigens is an acceptable option. Most areas of the world rou-
Frequent verbal reassurances and talking to the child about the peo- tinely administer BCG, polio (oral), and vaccines containing diphthe-
ple and the new world around the child should be encouraged. In ria and tetanus toxoids with pertussis (often whole-cell) components.
caring for the child, the parent or parents should be encouraged to Receipt of additional diphtheria, tetanus, and possible pertussis
meet the child at the child’s developmental level rather than chron- antigens may be associated with an increased risk of adverse events;
ologic age. measuring antibodies to diphtheria and tetanus is warranted to mini-
mize the risk of adverse events. No US Food and Drug Administration–
Growth and Nutrition approved antibody tests are available commercially for pertussis
When children are identified as malnourished or exhibiting fail- antibodies. Receipt of additional doses of inactivated polio vaccine
ure to thrive, a multidisciplinary treatment plan is recommended, is not usually associated with adverse events. Testing for HBsAb
including the parent or parents, physician, nutritionist, and thera- should be performed on all adoptees, and planning for further
pists (eg, occupational therapist for feeding difficulties). The child immunizations will be based on these results. Hepatitis B vac-
should be offered familiar foods, if that is the child’s preference, and cine will elicit only HBsAb responses; the presence of antibodies to
each meal may include a new, more nutritional offering. Children other hepatitis B antigens is suggestive of natural infection. For mea-
should be offered frequent meals and snacks, because they may sles, mumps, and rubella, various formulations and combinations
not be able to eat much at a single sitting. Foods should be calorie (1- and 2-component more commonly than 3-component) vaccines
dense and, depending on the child’s age and previous dietary are available worldwide. Checking antibodies to these antigens is
history, may include foods such as added butter, cheese, avocado, possible, but it likely may be necessary to administer a US-licensed
and peanut butter. Dietary supplements, such as PediaSure or Boost, combination (eg, measles, mumps, and rubella) to ensure protec-
may be indicated. tion against all 3 antigens. Most of the other vaccines available in
Precocious puberty has been observed more in female international the United States are not available in the developing world (eg,
adoptees than male international adoptees and is thought to be related H influenzae type b, pneumococcal conjugates, varicella, HAV), and
to the rapid improvement of nutrition. If signs of precocious puberty are the child should receive these vaccines as recommended for age per
observed, evaluation by a pediatric endocrinologist is recommended. the US schedule.

Development Infectious Diseases

Because developmental delays are so common among international Any acute identified infectious disease (eg, scabies, otitis) should
adoptees, most of them should undergo developmental assessment. be managed following standard practice, bearing in mind the pos-
Language delays, especially expressive delay, are common; when any sibility that different antibiotic resistance patterns exist in other
such delay is identified, formal audiology testing should be included parts of the world. Thus, if the child does not have the predicted
in the assessment. Performing developmental assessments of lan- response to treatment, consultation with a pediatric infectious
guage poses difficulties, however, because most are conducted in diseases specialist should be considered. For other infectious dis-
English, which is rarely the child’s primary language. Repeating the ease screening noted previously, additional evaluation and possible
testing after a period of adjustment may be helpful. Language bar- management should be initiated promptly (eg, penicillin therapy
riers do not preclude audiology testing. for syphilis) and pediatric infectious diseases consultation may
be considered.
Mental Health For TB evaluation, any positive PPD skin testing merits further
Mental and emotional health problems are common and may manifest investigation for true infection with Mycobacterium tuberculosis. A
early or late or occur as relapses. Initial problems may include sleep compounding problem is that some children may be anergic result-
disorders, fears and phobias, bizarre behaviors, and self-stimulating ing from malnutrition, and a false-negative PPD test result may be
behaviors. Unless the behaviors pose a safety risk, parents should obtained. If malnutrition is suspected, obtaining a chest radiograph
tolerate them because most dissipate with time in a secure environ- should be considered even if the PPD is negative, and consideration
ment. At times of emotional distress, parents may observe a relapse should be given to repeating the PPD when the child’s nutritional sta-
of behavior problems. For milder disorders, physicians may pro- tus has improved. Additionally, routinely repeating the PPD 6 months
vide guidance to parents; however, moderate and severe disorders after the child’s arrival is advisable if the child is from an endemic
merit prompt referral to a mental health professional, ideally some- area, because late exposure to an infected individual is possible.
one with expertise in adoption.
Prognosis
Immunizations Being prepared for the complex needs related to international adop-
If vaccinations are considered valid, as described previously, resum- tion has been identified as the best determinant for parents to con-
ing immunizations per the US schedule (see Chapter 37) is appro- sider the adoption experience as positive, regardless of the number
priate. If immunization status is unknown or incomplete, however, or severity of the medical needs of the adoptee.

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 Chapter 39: Health Care for International Adoptees 277

With improved nutrition and environment, most children After the family returns home, the pediatrician has a role
have significant catch-up growth; however, up to one-third of as primary care provider and in serving as a referral source for
adoptees may have unrecoverable loss in linear growth. In terms specialty care, such as in medical subspecialties, mental health,
of intellectual development, by 1 year of age nearly all children postadoption support services, developmental intervention, and
in orphanages have 1 or more areas of delay. Their progno- nutrition.
sis is generally good, however, with an increase in 2 develop-
mental quotient points per month after arrival in the United Counseling Adoptive Parents About
States. For children who have been in an orphanage for the first Expectations
3 years after birth, the longer the time of subsequent institution- Parents should be informed that it is unlikely that an institution-
alization the greater the negative effect on IQ and development. alized child will emerge from such a situation unscathed. Bearing
Many children have ongoing mental and psychosocial problems, this in mind, parents may make preliminary preparations for treat-
and some disorders do not manifest until children are older, such as ment and rehabilitation for the child. Families should be counseled
attention-deficit/hyperactivity disorder or learning disorders when that it is acceptable to say “no” to a potential adoptee. No one bene-
the child is of school age. Ongoing attachment disorders seem to fits from adoption of children by families who do not have the nec-
occur with greater frequency in children with lower IQs and more essary resources to care for them. Most importantly, parents should
behavior problems than average as well as in adoptive families of be reassured that optimism is appropriate. Being prepared matters!
a lower socioeconomic status.
Assessment of the overall outcome of children who spent 8 or
more months in institutional care in Eastern Europe, when evalu- CASE RESOLUTION
ated at least 3 years after adoption, showed that approximately one-
In the telephone consultation, the parents report that they have not observed
third had multiple serious problems, including IQ less than or equal any injuries and no areas seem tender when they examine Jaxon, per your sug-
to 85, insecure attachment, and severe behavior problems; approx- gestion. They elect to give him some diphenhydramine and fly home with the
imately one-third had a few serious problems but were thought to plan to make an office visit on arrival. Although they are exhausted, they travel
be making progress; and approximately one-third had progressed directly to your office for an acute care visit. They report that Jaxon remained
very well. The best predictors of major problems were greater length inconsolable and that the flight home was miserable for him and everyone
around them. On entering the examination room, Jaxon was screaming and
of time in the orphanage, increased number of children adopted noticeably uncomfortable but trying to find comfort in his father’s arms.
at the same time, younger adoptive mother, lower socioeconomic His temperature was 38.9°C (102°F) axillary, his pulse rate was 144 beats per
status of mother, and father alone selected the child. Although the minute, and his respiratory rate was 30 breaths per minute. His examination was
effect of these risk factors may not be identical for all birth coun- notable for extensive oral and pharyngeal vesicular lesions with erythema, but he
tries, they do provide some insight. Reviewing these risk factors had no labial lesions. He had multiple shotty cervical nodes, and the rest of the exam-
ination was noncontributory. A diagnosis of herpangina was made. He was given a
with adoptive parents during the preadoption period may aid in
dose of ibuprofen, and 15 minutes later he was quiet, able to swallow electrolyte
their decision making. solution, and cuddling in his father’s arms. His parents were reassured that this was
an acute infection and not an indication of poor bonding; in fact, Jaxon was already
Role of the Pediatrician seeking comfort from them. Additional testing for infections included obtaining anti-
bodies to HIV, rapid plasma reagin for syphilis, and hepatitis C and hepatitis B panel,
The role of the pediatrician is not to tell the adoptive parent whom which was especially important because of his biological mother’s history of being a
to adopt; parents are essentially choosing life partners, which is an commercial sex worker. Other testing included complete blood cell count, lead level,
individual decision. The pediatrician can, however, assist the parent and thyroid function. An appointment was made for 2 weeks hence to complete the
or parents in reviewing available medical information, which may assessment, including a developmental assessment; this evaluation was deferred
help the parent or parents make an informed decision about pro- because he was acutely ill at the time of the initial office visit.
ceeding with an adoption.
During the adoption process, the physician must assume an
active role as a child advocate as well as parental advocate and edu- Selected References
cator. This may be the first parenting experience for the adoptive
parent or parents and, thus, education on the basics of child care Albers L, Barnett ED, Jenista JA, Johnson DE. International adoption: medical
and developmental issues [preface]. Pediatr Clin North Am. 2005;52(5):xiii–xv
is necessary, no matter the age of the adoptee. This might include
https://doi.org/10.1016/j.pcl.2005.08.001
feeding and routine care (eg, estimate the number of diapers needed
American Academy of Pediatrics. Medical evaluation for infectious diseases
if traveling to an area with no access to supplies), as well as disci-
for internationally adopted, refugee, and immigrant children. In: Red Book:
pline techniques for a traumatized child. It is necessary to be pre- 2018-2021 Report of the Committee on Infectious Diseases. 31st ed. Kimberlin
pared to care for illnesses because many adoptees are ill at the time DW, Brady MT, Jackson MA, Long SS, eds. Itasca, IL: American Academy of
of the adoption and travel. The parent or parents may be isolated Pediatrics; 2018:176
from their usual support systems while embarking on the new role Barnett ED. Immunizations and infectious disease screening for internation-
of parent, and the pediatrician can facilitate emotional preparation ally adopted children. Pediatr Clin North Am. 2005;52(5):1287–1309, vi PMID:
for that experience. 16154464 https://doi.org/10.1016/j.pcl.2005.06.004

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 278 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Jones VF, High PC, Donoghue E, et al; American Academy of Pediatrics Feigin and Cherry’s Textbook of Pediatric Infectious Diseases. 8th ed. Philadelphia,
Committee on Early Childhood, Adoption, and Dependent Care. Comprehensive PA: Elsevier; 2018:2308–2319
health evaluation of the newly adopted child. Pediatrics. 2012;129(1):e214–e223. US Department of State Bureau of Consular Affairs. Adoption statistics. Travel.
Revised May 2019 PMID: 22201151 https://doi.org/10.1542/peds.2011-2381 State.gov website. https://travel.state.gov/content/travel/en/Intercountry-
Saiman L, Aronson J, Zhou J, et al. Prevalence of infectious diseases among inter- Adoption/adopt_ref/adoption-statistics.html. Accessed July 11, 2019
nationally adopted children. Pediatrics. 2001;108(3):608–612 PMID: 11533325 Weitzman C, Albers L. Long-term developmental, behavioral, and attachment
https://doi.org/10.1542/peds.108.3.608 outcomes after international adoption. Pediatr Clin North Am. 2005;52(5):
Staat MA. Infectious disease considerations in international adoptees and 1395–1419, viii PMID: 16154469 https://doi.org/10.1016/j.pcl.2005.06.009
refugees. In: Cherry JD, Harrison GJ, Kaplan SL, Steinbach WJ, Hotez PJ, eds.

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 CHAPTER 40

Health Care Needs of

Children in Foster Care
Kelly Callahan, MD, MPT; ChrisAnna M. Mink, MD, FAAP; and Sara T. Stewart, MD, MPH, FAAP

CASE STUDY
A 13-year-old girl is brought to your office by her fos- 2 nights previously when asked about school, she began
ter parent for a general physical examination. The foster to cry and ran to her room.
parent states that the girl has been living in her home for On physical examination, the patient is sad appear-
the past 2 weeks. When the child was initially brought ing and quiet, but cooperative. Her weight is in the 25th
by the social worker, she was wearing dirty clothes and percentile and her height is in the 50th percentile for
smelled of cigarette smoke. Neither medical records nor her age. She has poor dentition with multiple dental
immunization records are available for your review, and caries. She has a few basilar wheezes on lung examina-
the teenager is not sure the last time she saw a doc- tion and has scattered bruises on her anterior shins; no
tor. The girl states that she often missed school to help other abnormalities were noted.
care for her sick grandmother. She gets very quiet when
you ask about her family. She states that she misses her
Questions
1. What are the medical, psychological, and behavioral
younger sisters but does not mention anything about
issues that commonly affect children in the foster
her mother. When asked about her mother, she states
care system?
that she does not care to see her because her mother
2. What is the role of the primary care pediatrician
“cares more about her boyfriend than she does me and
in providing a medical home for the child in
my sisters.” The only history known by the foster parent
foster care?
is that the child was failing school because of frequent
3. How does a child’s legal status as a child in foster
absences and that there were extensive amounts of por-
care affect how medical care can be delivered?
nography and drug paraphernalia found in the home at
4. What are the appropriate health care referrals and
the time of removal. The social worker also told the fos-
community resources to access for a patient who is
ter mother that an expired albuterol inhaler was found
in foster care?
in the home with the girl’s name on it. The foster par-
ent states that the teenager seems “sad” all the time, and

The term foster care refers to the temporary placement of a child in care often have a significant number of unmet medical and men-
the home of another caregiver or foster parent because of a threat tal health needs because of complex psychological trauma and lim-
to the child’s safety or well-being in the original home. Placement ited access to health care. These children have rates of medical and
of a child in foster care results from an investigation of the child’s mental health disorders that are higher than those of children from
home environment by child protective services (CPS) and may be equivalent socioeconomic backgrounds who are not in foster care.
arranged via voluntary agreement of the parent or through court Thus, foster children should be considered part of the special needs
sanction. The foster parent may be related to the child (also known patient population.
as kinship care) or may be a nonrelative. For children in voluntary At the time of initial removal, the CPS worker may not be able
placement, the biological parent retains the right to terminate the to obtain a medical history or essential information about current
placement at any time. For those placed by legal sanction, a series of medications for the child. Changes in foster care placement may
court hearings give parents, the child, and the CPS agency the oppor- interrupt continuity of care with a health professional, and frequent
tunity to present their perspectives on the circumstances surround- changes in assigned social services caseworkers can create barriers
ing the allegations as well as their respective views on interventions to communication among biological parents, foster parents, health
to ensure the child has the best home environment. professionals, and caseworkers.
Children in foster care may present to the primary care pedi- Traditionally, those in the general medical community have
atrician soon after placement in foster care or after living with a lacked an appreciation for the complexity of the needs of this patient
foster parent for a long time. In either scenario, children in foster population. Additionally, in part because of low payment rates, it
279

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 280 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

has been difficult to allot sufficient time in a “routine” office visit 18 years of age without attaining permanent placement. Many of
to complete the comprehensive evaluation these children require. these teenagers later report being incarcerated or homeless at some
For those children with identified mental health needs, often few point after emancipation. Twenty-five percent of the children in fos-
psychiatric and psychological resources are available; this is ter care will experience 3 or more placements, which results in fur-
particul­arly true for children younger than 5 years. ther fragmentation of their health care and education. Multiple
These unmet needs have long-lasting effects on the well-being foster care placements are more common for those children with
of the children, even after exiting the foster care system, including behavioral, emotional, or coping problems.
into adulthood. Data have shown that the more adverse childhood
experiences to which a child is exposed (eg, abuse, neglect, paren- Clinical Presentation
tal substance abuse, witnessing domestic violence; see Chapter 142), Medical Issues
the higher the risk for heart disease, suicide, obesity, and other con-
Children in the foster care system have been shown to have high rates
ditions in adulthood, including early death.
of acute and chronic illness at the time of their initial medical evalu-
Because of their complex health care issues and vulnerability
ations after placement (Box 40.1). Thirty percent to 80% of children
to fragmented care and adverse childhood experiences, foster chil-
entering foster care have at least 1 medical concern, with one-third
dren merit a medical home that provides comprehensive, multidis-
having a chronic illness. Common conditions include obesity, asthma,
ciplinary services and medical case management.
vision or hearing problems, neurologic disorders, gastrointestinal
diseases, dental caries, and other inadequately managed chronic ill-
Epidemiology nesses, such as eczema and anemia. Acute infections are also common,
At any point during a given year, 600,000 children in the United States including respiratory tract infections, skin infections, otitis media,
spend time in foster care. Approximately 275,000 children enter the sexually transmitted infections (STIs), and intestinal infestations with
system annually. Reasons for placement, in descending order of prev- parasites. Low immunization rates are a frequent occurrence.
alence, include neglect, physical abuse, psychological or emotional Many children entering foster care have growth delay, with weight,
abuse, and sexual abuse. As a population, children in the foster care height, or head circumference measurement less than the 5th per-
system come from home environments that experience high rates of centile for their age. This may be caused by a combination of factors,
poverty, parental mental illness, parental substance abuse, unemploy- including inadequate nutrition, environmental deprivation, prenatal
ment, adolescent parenthood, frequent involvement with the crimi- alcohol exposure, genetic predisposition, and underlying illness (eg,
nal justice system, and low levels of education. Foster children have HIV infection). Behaviors such as rumination and social withdrawal
high rates of exposure to domestic violence, and many are victims may manifest in children in environments that are chronically stress-
of neglect, physical abuse, and sexual abuse. Their biological parents ful or lack the necessary stimulation and support for a child. (For fur-
often have limited parenting skills; the children experience inconsis- ther discussion of failure to thrive, see Chapter 146.) Overweight (ie,
tent parenting behaviors along with minimal developmental stim- body mass index [BMI] 85%–95% for age) and obesity (ie, BMI >95%
ulation and emotional support. All these factors combine to cause
unpredictable, stressful, and unsafe home environments for these
children, prompting their removal and placement into foster care.
Children in foster care account for 25% to 41% of Medicaid expen- Box 40.1. Medical, Developmental, and Mental
ditures despite representing less than 3% of all enrollees. Health Issues Common to Children in Foster Care
Foster children are of all ethnicities, but children of color are ww Acute infection
disproportionately represented. Children younger than 5 years ww Undiagnosed or inadequately treated chronic illness
comprise nearly one-half of the children in foster care, with those ww Dental caries
11 to 15 years of age a distant second. In the past several decades, ww Growth delay and failure to thrive
an increasing percentage of new entrants into foster care are infants ww Incomplete immunization history
younger than 1 year of age. Many of these infants are exposed to ww Prenatal or perinatal exposure to sexually transmitted infection
substances prenatally and are placed in foster care because of a ww Effects of prenatal substance exposure
combination of factors related to maternal drug use. ww Physical sequelae of physical and sexual abuse
Approximately 70% of children leave foster care within 2 years ww Developmental delay
of placement, with the average stay being 20 months. More than ww Attention-deficit/hyperactivity disorder
one-half of these children are reunited with their biological par- ww Posttraumatic stress disorder
ent or primary caregiver. Six percent remain in foster care for more ww Anxiety
than 5 years, and approximately 35% of all children who leave fos- ww Depression
ter care later reenter the system because of a new CPS report. Since ww Conduct and oppositional defiant disorders
the 1990s, the number of adoptions from foster care has increased ww Attachment disorders
to 20% of those leaving the child welfare system. An additional 8% ww Educational disabilities
of those leaving foster care emancipate out of the system by reaching

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 Chapter 40: Health Care Needs of Children in Foster Care 281

for age) are also common among children in foster care. Depression, developed a secure attachment to a primary caregiver and there-
dysfunctional coping skills, and lack of family connectedness also fore may have difficulty bonding with a foster parent. This prob-
contribute to suboptimal health. lem is further compounded if the child is moved between multiple
Many children placed in foster care have a history of prenatal foster homes, prohibiting development of a healthy attachment to a
exposure to illicit drugs, alcohol, and tobacco. These children have caregiver. In addition to a lack of emotional reciprocity, these chil-
high rates of preterm birth and prenatal or perinatal exposure to dren may exhibit self-stimulatory behaviors or sleep disturbances.
infections such as hepatitis C, hepatitis B, HIV, syphilis, and her- The behavioral difficulties manifested in children in the foster
pes simplex. This risk of exposure to infectious agents is related to care system are often the result of early childhood trauma and toxic
maternal drug use and its frequent association with prostitution, stress. Toxic stress changes the neurobiology of the developing brain
needle sharing, and drug use in sexual partners. and can result in emotional dysregulation, impulsivity, and aggres-
Children who have been placed in foster care may present with sion. These children also may be predisposed to behavioral disorders
physical sequelae of prior physical or sexual abuse. Physical abuse from having been abused or neglected or from having experienced
may result in skin trauma, skeletal fracture, head trauma, abdom- prenatal exposure to drugs or alcohol. Children often experience
inal trauma, and chest trauma. Sexual abuse may result in genital fear, sadness, and a feeling of guilt or responsibility for the family
trauma or symptoms of STIs. Both can result in mental health needs discord that resulted in their removal. Foster placements are invari-
related to toxic stress and/or complex trauma. ably sudden and unexpected, involve the loss of a familiar caregiver,
and are traumatic for children of all ages.
Developmental and Mental Health
Issues Evaluation
A high prevalence of developmental delay, behavioral disorders, It is important for pediatricians to be familiar with the medical
and educational difficulties has been noted in foster children of consent legalities concerning foster children in their geographic
all ages. These disorders are more common in children with a history locales. Specifics vary by locality, but generally, foster parents have
of neglect or abandonment than in those who have experienced other the authority to provide consent for routine medical care. The place-
forms of child maltreatment. Sixty percent of children younger than ment of a child in foster care does not supersede the right of a biolog-
5 years entering foster care have significant developmental delays, ical parent to participate in the medical decision making for his, her,
and 40% of school-age children (age 5 years and older) have school or their child, and many biological parents retain the authority to
difficulties. Speech and language concerns, delayed fine motor skills, consent for medical tests and procedures. Any medical procedure or
and poor social-adaptive skills are common. Foster children are more test that requires specific written consent is likely to require autho-
likely than their peers who are not in foster care to require special rization by the legally recognized parent (eg, child welfare agency,
education, experience multiple school placements, and require grade courts, biological parent). Two common clinical scenarios that fre-
retention. Approximately 30% of children in foster care have quently require consent beyond the foster parent’s authority are HIV
behavioral difficulties, and 17% take at least 1 psychotropic or testing and the administration of psychotropic medications.
antipsychotic medication, a percentage that is significantly higher
than the national average of 5%. Of children taking any psycho-
tropic or antipsychotic drug, a disproportionate number (29%) History
were children placed in foster care, group homes, or residential treat- Children in foster care may be brought for medical evaluation by
ment centers. an authorized caregiver, who may be a foster parent, a relative of
Common mental health issues in the foster care population the foster parent, or the social service caseworker. Often the biolog-
include attention-deficit/hyperactivity disorder, depression, anxi- ical parent is absent from the visit and the child’s medical records
ety, and suicidal ideation resulting from toxic stress. Posttraumatic are not available at the time of the evaluation. Obtaining past med-
stress disorder is common and more prevalent in those who have ical records can be difficult, but the caseworker can be of assistance
experienced or witnessed family violence. Adolescents in foster care in this process. It is important to ascertain the circumstances that
may act out as a manifestation of mental health difficulties, result- prompted a child’s placement in foster care, because it may be nec-
ing in sexual promiscuity, substance abuse, and truancy, as well as essary to modify portions of the evaluation accordingly.
rates of conduct and oppositional defiant disorders that are higher Biological family history is useful to evaluate for the presence
than those in the general adolescent population. of genetic disorders and communicable diseases. A maternal his-
Attachment disorders are more common in the foster care pop- tory of drug use or STIs is helpful in identifying those children
ulation than in the general pediatric population and account for a who may have experienced prenatal exposure to drugs or infec-
portion of the behavioral difficulties in children in foster care. A tious agents.
secure attachment to a primary caregiver is necessary to the devel- Birth history should include any history of prenatal care and com-
opment of emotional security and the sense that one’s needs are plications, such as preterm birth or drug withdrawal. Results of rou-
important. Children who are removed from violent homes or who tine newborn screening (eg, hearing, inborn errors of metabolism,
may have experienced abuse or neglect are likely to have never thyroid function, hemoglobinopathies) should also be obtained.

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 282 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

A complete medical and surgical history should be gathered, prior evaluations by the school system, special education services
including the identification of a regular health professional if the provided, or prior therapeutic services (eg, physical, occupational,
child has one. Older children and adolescents may be able to provide or speech therapy) received through the school district.
some of their own histories. Known medications and allergies should
be documented, and all available immunization records should be Physical Examination
obtained. Documentation of a child’s immunization history is fre- A complete, unclothed physical examination should be performed on
quently unavailable; thus, all possible sources of records, such as each child. For children who are traumatized or particularly fright-
biological parents or school districts, should be identified. Feeding ened, disrobing only the immediate area being examined may help
history and nutritional assessments should include the type and ease their discomfort. For some traumatized children, more than
amount of formula or human milk for infants and types and quan- 1 office visit may be necessary to complete the evaluation. Growth
tities of foods for older children. parameters, including height, weight, and occipitofrontal head cir-
The psychosocial history includes a child’s current and prior cumference (for children younger than 2 years) or BMI (for chil-
foster placements or living arrangements and whether the child dren 2 years and older), should be plotted on a reference chart, and
was exposed to domestic violence, physical abuse, or sexual abuse a close inspection should be performed for signs of prior trauma.
(Box 40.2). Verbal children may also be able to discuss their feel- The child should be assessed for dysmorphic features consistent with
ings about their current foster placement. Adolescents should prenatal alcohol exposure or other genetic syndromes. In children
undergo a confidential screening (eg, home, education and of all ages, the physical examination should include a genital exam-
employment, activities, drugs, sexuality, and suicide/depression ination to assess for signs of trauma. Additionally, sexually active
[HEADSS]; see Chapter 4) to address drug, alcohol, and tobacco females should undergo a pelvic examination if there are any reports
use; issues related to home and school; sexual activity; violence; of abdominal pain, vaginal discharge, or other concerns. (See Chap­
and gang involvement. ters 144–146 for further descriptions of physical findings.)
The developmental history should include the results of prior
developmental assessments as well as a listing of therapeutic or early Immunizations
intervention services received. The behavioral history should also Immunizations should be administered as appropriate for age,
include results of prior assessments, mental health services used, and and if immunization records cannot be obtained in a timely fash-
any psychotropic medications prescribed. The foster parent, biolog- ion (eg, before the next scheduled visit), the child should be consid-
ical parent, or CPS caseworker may be able to provide useful obser- ered unimmunized. In this situation, options include restarting the
vations of the child’s developmental capabilities, behavioral patterns, vaccination series or checking antibody titers for selected vaccine
and social interactions. The biological parent or CPS caseworker may antigens (see Chapter 37 for further information). Other possible
also be able to assist in collecting records from prior assessments. sources for vaccine records include state or county registry, the child’s
Educational history is often not available at the time of the med- previous health professional, or the school previously attended by
ical evaluation. It is helpful, however, to know a child’s history of the child. If available, the biological parent should be asked to assist
with providing any vaccine record as well as other medical records.
All children should also undergo appropriate screening for
Box 40.2. What to Ask
tuberculosis, such as purified protein derivative skin testing or
Psychosocial Concerns interferon-γ release assays.
ww When was the child placed in foster care?
ww Why was the child placed in foster care? Additional Assessments
ww Has the child had prior foster placements? If so, when? Why did All children should undergo mental health and developmental
placement change? screenings and should be referred for comprehensive testing if
ww Was there prior exposure to domestic violence? To physical or sexual abnormalities are noted. Dental screening should be incorporated
abuse? in the physical examination of all children older than 6 months.
ww Does the child have siblings in foster care? Vision and hearing screenings should also be performed on all chil-
ww How does the child feel about the current foster placement? dren old enough to cooperate. A referral for expert vision or hear-
ww How has the child integrated into the foster family? ing evaluation should be made if it is indicated by the history or
ww Has the child previously received mental health services or therapeutic physical examination or the patient cannot complete the screening
services for developmental delay or educational difficulties? procedure, such as infants and children with developmental delay.
ww What is the child’s social service plan (eg, parental visits, reunification,
termination of parental rights)?
Laboratory Tests
ww Does the child have visitation with the biological parents? If so, are visits Routine screening laboratory tests should be performed on foster
monitored or unmonitored? children just as they are indicated for the general pediatric pop-
ww Do the parents have to participate in any classes or training? ulation. For example, hemoglobin level should be checked annu-
ally in all infants, toddlers, and preschool-age children. Serum lead

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 Chapter 40: Health Care Needs of Children in Foster Care 283

levels should be checked according to local guidelines but should be management is necessary to track these referrals and to contact the
checked at least once for a child during the toddler years. A urinal- foster family or caseworker if an appointment is missed.
ysis should be performed as indicated by the history and examina-
tion for the child younger than 2 years of age and at least once for Prognosis
the child 2 years of age or older. Children in foster care, spanning in age from newborns to adoles-
For infants younger than 12 months, especially those with a his- cents emancipating from the system, have a spectrum of mental
tory of prenatal drug exposure or other known risks for maternal health and medical needs. Preliminary studies have documented
STI, rapid plasma reagin, hepatitis C antibody, hepatitis B surface improved physical health status, school performance, and adap-
antigen and surface antibody, and HIV testing should be performed. tive functioning of young children after placement in foster care;
Adolescents who are considered to have at-risk behaviors, however, these studies require replication. The strength of the bond
patients with signs or symptoms consistent with an STI, and chil- between the foster parent and the child, as well as the consistency
dren with a history of sexual abuse that could result in the trans- and predictability of the foster home environment, are significant
mission of infection should be tested for STIs. Often adolescents can to the development of the child’s sense of safety and well-being.
sign their own consent forms for evaluations and treatment related To date, much of the research on the health status of children
to STIs; however, local statutes should be consulted. in foster care has focused on the delineation and description of the
Imaging Studies health-related issues that these children face. More limited evalua-
tion has been done of the different models of health service delivery
If a child has a history or physical findings concerning for physical
to the foster care population as well as of the subsequent medical,
abuse, imaging such as a radiographic skeletal survey, computed
developmental, and emotional outcomes of the children in these
tomography, and magnetic resonance imaging of the head or radio-
different models.
nuclide bone scan may be indicated. (For more specific recommen-
Although a need exists for continued study of health care deliv-
dations, see Chapter 17.)
ery to the foster care population, it is widely accepted that children
in the foster care system benefit from the establishment of trauma-
Management
informed medical homes with case management capabilities and
Given the breadth of health-related needs for this patient pop- physicians who are well-versed in the complex medical and mental
ulation and the frequency with which such patients change fos- health issues that affect this vulnerable population.
ter homes, close case management is an essential component
of health care delivery to this population. Health professionals
should maintain consistent communication with the child’s case- CASE RESOLUTION
worker to ensure that medical and mental health recommenda- This case illustrates many of the common issues that affect children when they
tions are incorporated into the child’s social service plan. Ideally, are placed in foster care. Medical and immunization records are frequently
a child should have a trauma-informed medical home and conti- unavailable to the medical examiner, and chronic medical needs have often gone
nuity with a primary care pediatrician over time. Unfortunately, unmet. This child’s poor hygiene and frequent school absences point to a history
of parental neglect and, given the presence of drug paraphernalia, her mother
changes in home placements often make this difficult. If conti- likely was involved with substance abuse.
nuity of care is broken, incorporation of the health care plan into This child should undergo a thorough assessment for behavioral problems,
a child’s broader social service plan should ensure that medical developmental delays, and education-related disabilities. The child states that
history and medical and mental health recommendations are not she does not want to return to her parents’ care, but she still misses her fam-
lost. The use of electronic health records is a growing practice in ily members. This may be remedied through her child welfare plan, for example,
by arranging for her to remain in contact with her siblings throughout their fos-
many foster care systems and promotes continuity of care, even
ter placement. Because of her experiences in her prior home environment, she
with logistical challenges. has depressive symptoms and is likely to have other unmet mental health needs,
Children and adolescents in foster care require frequent health which require referral to a mental health provider. She also needs evaluation by
visits. The American Academy of Pediatrics recommends a health her new school system to determine her specific educational needs and appro-
screening within 72 hours of placement, a comprehensive medical priate grade placement.
evaluation within 30 days of placement, and a follow-up medical The girl has poor dentition, which is a common finding in the foster care pop-
ulation, and she requires a referral for dental care. The presence of mild wheezing
examination within 60 to 90 days after placement. Because of the in a child who states that she feels fine otherwise is a likely marker for untreated
prevalence of significant medical, social, and mental health issues reactive airway disease and may be a reflection of poor continuity of medical
affecting children in foster care, additional visits are often advis- care prior to her placement. Routine adolescent care should be initiated at this
able. Anticipatory guidance should be provided to the caregiver, and visit, including a thorough history of drug and alcohol use as well as reproductive
age-appropriate issues should be discussed with older children and history. Anticipatory guidance on menstruation, sexuality, and drug use should
be considered. Her vaccines should be updated. She should be scheduled for a
adolescents.
revisit in 2 months for the second human papillomavirus vaccine and to estab-
Children in foster care often need referral to medical subspe- lish an ongoing relationship, monitor her asthma, and talk about any concerns
cialists, dentists, dietitians, speech therapists, occupational thera- she may have.
pists, mental health professionals, and other service providers. Case

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 284 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Stambaugh LF, Leslie LK, Ringeisen H, Smith K, Hodgkin D. Psychotropic

Selected References
Medication Use by Children in Child Welfare. Washington, DC: Office of Planning,
American Academy of Pediatrics. Fostering Health: Health Care for Children and Research and Evaluation, Administration for Children and Families, US
Adolescents in Foster Care. 2nd ed. Elk Grove Village, IL: American Academy Department of Health and Human Services; 2012. Office of Planning, Research
of Pediatrics; 2005 and Evaluation report 2012-33. www.acf.hhs.gov/programs/opre/resource/
American Academy of Pediatrics Council on Foster Care, Adoption, and Kinship nscaw-no-17-psychotropic-medication-use-by-children-in-child-welfare. Accessed
Care; Committee on Adolescence; Council on Early Childhood. Health care July 15, 2019
issues for children and adolescents in foster care and kinship care. Pediatrics. US Department of Health and Human Services Administration for Children and
2015;136(4):e1131–e1140 PMID: 26416941 https://doi.org/10.1542/peds.2015-2655 Families. Administration on Children, Youth, and Families; Children’s Bureau.
Medicaid and CHIP Payment and Access Commission. The intersection of The AFCARS Report. No. 25. www.acf.hhs.gov/cb/resource/afcars-report-25.
Medicaid and child welfare. In: Report to Congress on Medicaid and CHIP. Accessed July 15, 2019
Washington, DC: Medicaid and CHIP Payment and Access Commission; June Wang C, Edelstein SB, Waldinger L, Lee CM, Bath E. Care of the foster child: a
2015:55–87 www.macpac.gov/wp-content/uploads/2015/06/Intersection-of- primer for the pediatrician. Adv Pediatr. 2011;58(1):87–111 PMID: 21736977
Medicaid-and-Child-Welfare.pdf. Accessed July 15, 2019 https://doi.org/10.1016/j.yapd.2011.03.009
Oswald SH, Heil K, Goldbeck L. History of maltreatment and mental health Zlotnik S, Wilson L, Scribano P, Wood JN, Noonan K. Mandates for collabora-
problems in foster children: a review of the literature. J Pediatr Psychol. tion: health care and child welfare policy and practice reforms create the plat-
2010;35(5):462–472 PMID: 20007747 https://doi.org/10.1093/jpepsy/jsp114 form for improved health for children in foster care. Curr Probl Pediatr Adolesc
Schilling S, Fortin K, Forkey H. Medical management and trauma-informed care Health Care. 2015;45(10):316–322 PMID: 26403650 https://doi.org/10.1016/j.
for children in foster care. Curr Probl Pediatr Adolesc Health Care. 2015;45(10): cppeds.2015.08.006
298–305 PMID: 26381646 https://doi.org/10.1016/j.cppeds.2015.08.004

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 CHAPTER 41

Working With Immigrant

Children and Their Families
Ismael Corral, MD, MBA, and Carol D. Berkowitz, MD, FAAP

CASE STUDY
A 7-year-old boy presents with vomiting and clinical Questions
signs of dehydration. The family thinks he has empa- 1. What are the ways in which different immigrant
cho (a Latin American folk illness). You tell the family families view illness and health?
that you suspect that he has viral gastroenteritis. You 2. What are barriers to accessing health care that
want to draw some blood samples for testing and give children in immigrant families face?
him fluids intravenously. The parents are skeptical; they 3. What questions help the physician understand the
refuse the blood work and want to leave, against medical health beliefs of immigrant families?
advice. 4. What are the considerations when interacting with
parents who do not speak English?

The United States is described as a nation of immigrants. Out multigenerational household. Families of immigrant children tend
of a population of 326 million, current estimates are that about to be larger, with 19% having 5 or more children, compared with
43 million, or approximately 13.2% of the current US population, are families of children born in the United States, of which only 14% are
foreign-born citizens or noncitizens. Half of these immigrants are of that size. While some immigrant children are citizens and eligi-
Hispanic, and 65% of Hispanics are of Mexican descent. It is expected ble for safety net programs, their family’s status directly influences
that by 2030, Hispanic children will account for most children liv- whether these children will even access such care. Children of immi-
ing in the United States. During the 1990s, 70% of the overall US grant parents are twice as likely to be uninsured (15%) as children in
population growth was influenced by a wave of recent immigrants, nonimmigrant families (8%). There is also growing concern that the
mostly from Latin America and Asia, and by the children born to health status of some immigrant children, whether foreign born or
these newcomers. The vast growth in the population of children liv- first generation, actually declines after settling in the United States.
ing in immigrant families, whether foreign born (first generation) or
US born (second generation), poses a unique set of challenges. This Demographics of Immigrant Children
is especially the case in 10 major metropolitan areas that are classi- There are 5 general categories of immigrants in the United States,
fied as traditional immigrant destinations, where approximately 48% each benefiting from specific entitlements and services and having
of immigrant children reside. While this chapter focuses on immi- certain legal rights: lawful permanent residents, naturalized citizens,
grant children, children of migrant workers, and children living by refugees/asylees, nonimmigrants, and undocumented immigrants
the United States-Mexico border may face similar issues related to (Box 41.1).
access to quality health care. In 2007, individuals who had become naturalized citizens
Households with immigrant children are more likely to live below included immigrants (32%), lawful permanent residents (29%),
the federal poverty level (FPL) and have at least 1 parent who did undocumented immigrants (29%), refugees (7%), and nonim-
not graduate high school or is not fluent in English. An estimated migrants (3%). From 1980 to 2000, the children of immigrants
31% of mothers and a similar number of fathers in these families increased from 5% to 20% of school-age children, representing
have not graduated from high school. In 2013, 26% of children in approximately 10 million of the estimated 60 million school-age
immigrant families lived below the FPL, compared with 19% of children in the United States.
children whose parents were born in the United States. This has By far the largest category of immigrants is nonimmigrants
gradually increased since 2006, when 22% of children in immi- or temporary visitors. Approximately 3 million children arrive
grant families lived below the FPL. These children are also more each year, mostly from Asia, Western Europe, and parts of North
likely to live in crowded housing (>1 person per room) and in a America, typically accompanying their parents, who are seeking

285

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 286 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

While most pediatricians are not in a position to assist families

Box 41.1. Categories of Immigrants
in their efforts to attain citizenship or legal status, programs such as
in the United States
the National Center for Medical-Legal Partnership (https://medical-
Lawful Permanent Residents legalpartnership.org) may provide necessary advice and services.
ww Carry a green card. There is also an Immigrant Child Health Toolkit available through
ww Noncitizens with permission to permanently live and work in the United the American Academy of Pediatrics (AAP) that provides primary
States. care physicians with immigration-specific resources readily avail-
ww May apply for naturalization after 5 years (or 3 years if they marry a able within their area.
citizen).
ww Group with the most international adoptees. Health Care Needs of Immigrant
Naturalized Citizens Children
ww Born as noncitizens. When the child of an immigrant family presents for care, primary
ww Having met certain English literacy requirements and demonstrating a concerns are about the presence of an infectious disease (from expo-
basic knowledge of civics and a desirable moral character, are granted sures and possible lack of vaccination) or of a hidden genetic or
the same rights as natural-born citizens. ethnic condition (eg, hemoglobinopathies, glucose-6-phosphate
ww However, are not eligible to hold the office of president or vice president. dehydrogenase deficiency). Investigating the health status of a
Refugees/Asylees child, especially from undocumented immigrant families, is par-
ww Granted permission from the US government before entry. amount because these children likely have not seen a doctor prior
ww Fled their country of origin for fear of persecution because of their race, to immigrating. Similarly, nonimmigrants, such as tourists and
religion, social group, or political opinion. other temporary visitors, from many Western or Pacific Rim coun-
ww Many unable or unwilling to return to their country of origin. tries are not required to have a medical examination performed as
ww Those granted permission to remain are deemed asylees. part of their application. Only visas for permanent residency require
ww After a year, both may apply to adjust their status to lawful permanent a health examination to be performed by an approved physician,
resident. and even then, the focus of the examination is to exclude certain
conditions, such as active tuberculosis (TB), HIV, and other severe
Nonimmigrants
physical or mental disabilities. In children, laboratory testing may
ww Carry a visa.
be limited, and proof of vaccination status may be exempted for
ww Granted permission to enter for a specified time and specific purpose
certain groups, such as international adoptees.
(usually to work or study).
Lack of education and poverty, which results in relative food
Undocumented Immigrants insecurity and inadequate, crowded housing conditions, poses an
ww Entered the country illegally or even legally but then violated the terms ominous threat to the health of these same children. Immigrant
of their stay and remained after their visa expired. parents of children born in the United States may be reluctant to
ww Lack proper papers and identification to live in the United States. apply for Medicaid or the Children’s Health Insurance Program for
fear of being considered a public charge (ie, a person dependent on
the government for expenses of living). The Patient Protection and
work. A smaller percentage are students or exchange visitors. While Affordable Care Act of 2010 excluded undocumented immigrants
not technically immigrants, this special group may present for care from health care coverage. Noncitizen children who are lawful per-
with similarly unaddressed health issues depending on their coun- manent residents must wait at least 5 years before they are able to
try of origin. apply for public assistance. While most refugee children may receive
The next largest group, almost a quarter-million children, enter some form of subsidized care, such as Medicaid, many of these ben-
the United States as lawful permanent residents, with most eventu- efits are time-sensitive. Even US-born children of undocumented
ally becoming naturalized citizens. Although most arrive with or to mothers, while legally eligible as US citizens for benefits such as
meet family already residing in the United States, several thousand nutritional assistance programs, may not receive these because of
adolescents immigrate unaccompanied each year; most are female, parental fears of detection and deportation.
and many are married to a naturalized citizen at the time of immi- Physicians should ascertain that the family has adequate hous-
gration. Included in this group are children of refugees or asylees. ing and access to food, 2 basic human needs. The physician should
More than 75,000 individuals arrive each year, most recently from take the following factors into consideration during health super-
the countries of the former Yugoslavia and former Soviet Union, vision visits (Box 41.2): Is the food available in the family’s neigh-
Vietnam, Somalia, and other war-torn regions. Numbers for the borhood consistent with the traditional food that the family desires?
third-largest group, undocumented immigrants, are based primarily Is the food healthy, or does the family live in a food desert where
on estimates. Most are from Mexico or other Latin American states. there is a plethora of fast-food restaurants and a paucity of super-
Some entered the United States legally but have overstayed their markets or grocery stores? The ability to safely attend the local park,
visa or lost their status by committing a crime. walk around the neighborhood, or even go outside the family’s own

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 Chapter 41: Working With Immigrant Children and Their Families 287

low-birth-weight children born to foreign-born mothers, especially

Box 41.2. Risk Factors Affecting the Health
among Hispanic and non-Hispanic black immigrants, compared
of Immigrant Children
with women in their native countries who did not immigrate. This
Social and Economic positive effect related to immigration has been studied less in older
ww Inadequate or crowded housing children because of limited availability of data samples from second-
ww Environmental safety concerns generation children that do not exclude first-generation children.
ww Food insecurity
ww Lack of insurance (because of ineligibility) General Approach to the Initial
ww Lack of access to insurance (eligible but unable or unwilling to apply) Medical Evaluation of an
ww Educational level of household Immigrant Child
Cultural Ascertaining the life history of an immigrant child, including any
ww Dietary preferences relevant travel history, will help determine the extent of the medi-
ww Lack of language fluency or literacy and translation/interpretation issues cal evaluation (Box 41.3).
ww Disparate ideas about the causation and treatment of illness In general, most immigrants from industrialized nations have
ww Expectations of the medical system received comprehensive medical care, and the only issues facing
ww Religious practices with a medical component these children are routine, such as updating immunization status.
ww Lack of access to traditional medical practitioners and treatments In contrast, adoptees, refugees, and undocumented children often
ww Importation of drugs not approved in the United States have received little or no care. While there is no single approach
Physical to the medical evaluation of all immigrant children, some general
ww Carriage of infectious disease and the possibility for repeated exposure principles apply.
via travel or living within one’s immigrant community Any and all existing medical records should be reviewed. Previous
ww Presence of ethnic or genetic variations growth parameters should be obtained, if available. While a com-
ww Lack of vaccination plete translation is not always necessary, if an unusual diagnosis or
ww Poor nutritional status a complicated history exists, use of clinical translational services or
a nearby university’s academic language department may facilitate
Mental Health
the process. Sometimes, records suggest that a diagnosis was con-
ww Negative past living conditions, including exposure to violence or
sidered but not confirmed, and it may be appropriate to reevaluate
natural disaster
the child for any suspected condition.
ww Cultural adjustment (or the process of acculturation or enculturation)
It is important to screen for certain infectious diseases. The most
ww Traumatic separation
common among immigrants are TB, intestinal parasites, hepatitis B,
syphilis, and HIV. In fact, the risk of TB is more than 100 times
greater in immigrant children than in US children. Fifty percent
home should be determined, as any of these factors may interfere of all newly identified cases of TB in the United States are diag-
with treatment plans. In cases in which safety or access is an issue, nosed in immigrants, many within the first 5 years after birth. If
information about local community centers should be provided dur- TB is not clinically apparent (ie, in the incubation period), or test
ing clinic visits. Often, families may not be aware of affordable, local,
easily accessible resources.
It has traditionally been supposed that immigration itself
imposes unique stresses on these children. Mental health challenges Box 41.3. What to Ask
(eg, depression, anxiety, grief) may be related to the reasons for their Evaluating Immigrant Children
migration as well as to the inevitable process of acculturation. Stress ww Are the parents present? Or is there a current guardian who may have
is compounded by the potential loss of the immigrant’s identity, personal knowledge of the child’s social and medical history?
separation from home support systems, or traumatic separation ww In which countries has the child resided and under what circumstances?
from family members either in the United States or during travel; ww Have the child’s living conditions changed dramatically (positively or
inadequate language and literacy skills in a society intolerant of lin- negatively) recently?
guistic weakness; disparities between one’s country of origin and the ww Are there verifiable birth and medical records to be reviewed?
United States in economic, social, and professional standards and Vaccination status? What age is the child? What type of health care did
status; and the psychological and emotional trauma of war, perse- the child receive in his or her home country?
cution, or exploitation in one’s country of origin. ww Has the child undergone any recent procedures or treatment?
Despite these challenges, epidemiological studies indicate that ww Was the child exposed to any potentially toxic occupational or environ-
children in immigrant families experience better outcomes than mental risks?
those in their native countries who did not immigrate, especially ww What educational level did the child achieve?
in some countries. The most striking example is the lower rate of

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 288 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

results are negative but clinical suspicion exists, TB testing should Communicating in a patient’s native language serves to foster
be repeated in 6 months. The AAP Red Book can provide guidance and enhance the physician-patient relationship. Patients often feel
on interpreting tuberculin skin tests in the setting of BCG vaccine more relaxed, confident, and open to sharing their concerns with
as well as advice about alternative tests, if warranted. Intestinal par- their physician. Treatment plans expand beyond a physician-directed
asitic infestations may be present without symptoms. Some clinics plan to a dynamic interaction that empowers the patient to take con-
specializing in immigrant health advocate giving a single dose of trol of the disease.
albendazole as a more cost-effective approach than screening and Providing culturally competent care that considers cultural prac-
diagnosis, but the safety and efficacy of this approach has not been tices and beliefs about child health or illness when devising a treat-
substantiated in children. ment plan is also critical. Questions to ask are provided in Box 41.4
Ethnic or genetic conditions should also be considered. and also include the following: Does the family access healers for
Conditions such as glucose-6-phosphate dehydrogenase deficiency medical problems? What are the family’s views about medication
in immigrants from the Mediterranean, Africa, and Southeast Asia use? Are there hierarchical structures that influence a parent’s accep-
may affect the child’s health in the short term. Others, such as hemo- tance of a physician’s advice (eg, father accepting advice from a female
globinopathies and thalassemia, while common among certain physician, parent accepting advice from a young resident)? Are
ethnic populations, such as those from Southeast Asia, may not there dietary restrictions? In addition, it is important to consider the
be of immediate importance. Overall, anemia is extremely com- influence of socioeconomic status and environmental hazards during
mon among immigrant children, and the diagnostic workup of the development and implementation of a treatment plan. Failure to
these children may uncover an underlying blood dyscrasia trait take all these factors into account may lead to undesired health out-
or lead intoxication. If iron therapy has been initiated empirically, comes and incorrectly labeling the family or patient as noncompliant.
repeat testing after treatment is paramount to ensure a response
and confirm the diagnosis. Understanding the risk of nutri- Conclusion
tional deficiencies based on the country of origin is important. There are numerous barriers that potentially interfere with achiev-
For instance, nutritional disorders, such as rickets and iodine ing optimal health status for immigrant children. Many of these
deficiency, are common in children who are ethnic Chinese and barriers are societal and relate to funding for medical services.
children from the former Soviet Union. Trust is a core component of the physician-patient relationship, and
Immunizations must be administered as appropriate, follow- establishing trust may be more difficult when the physician and
ing the catch-up recommendations of the AAP and the Advisory family have different cultural values and expectations. It has been
Committee on Immunization Practices. Records of previous immu-
stated that in medicine, the sacred trust that develops between a
nizations may be difficult to interpret. Shortened intervals or the
patient and his or her doctor should never be taken for granted.
administration of a vaccination at too early an age may not be readily
The development of trust forms the foundation of the therapeutic
apparent. Fraudulent records, especially among adoptees and chil-
relationship. It provides credibility to the practitioner. The practi-
dren from institutional settings, often exist. In young children, it is
tioner must act in a manner that elicits and fosters trust, and being
generally considered safe and cost-effective to simply give missing
knowledgeable and nonjudgmental about the beliefs and practices
vaccines; however, determining serum immunity through the use of
of patients is pivotal.
titers may be more cost-effective in older children. It is also impor-
tant to remember that children seeking asylum are not required
to meet immunization requirements at time of entry; however, at Box 41.4. What to Ask
time of permanent residency application they must show proof of
immunization. Understanding Cultural Concepts of the Symptoms of Illness
ww What do you call the problem? What is your understanding of the
Cultural and Linguistic Sensitivity problem?
Bridging language differences using professional interpreter ser- ww What do you think caused it?
vices is required to provide adequate patient care. Such linguistic ww Why do you think it started when it did?
services are mandated by agencies such as the Centers for Medicare ww What do you think the sickness does? How does it work?
& Medicaid Services for payment from the federal government. ww How severe is it? How long will it last?
The use of children or hospital maintenance or janitorial staff as ww What are the chief problems the illness has caused?
interpreters is inadequate and potentially violates federal laws, ww What kind of treatment do you think the child should receive? What are
including the Health Insurance Portability and Accountability Act the most important results you expect from this treatment?
of 1996. Professional interpreters are taught to interpret and not ww What do you fear most about the illness? Do you fear the treatment or
carry on additional conversations with the patient or expound on medication?
the questions the physician asks. The physician should address ques- Adapted from Kleinman A. Patients and Healers in the Context of Culture: An Exploration of the
tions directly to and maintain eye contact with the patient, not the Borderland Between Anthropology, Medicine, and Psychiatry. Berkeley, CA: University of California
interpreter. Press; 1981.

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 Chapter 41: Working With Immigrant Children and Their Families 289

Health professionals must work toward creating an atmosphere

of trust so that parents feel comfortable revealing their beliefs, con- CASE RESOLUTION
cerns, and fears. The threat of deportation should be considered You reach an agreement with the family for a community healer to come and
when taking into account whether the patient or family will follow perform a therapeutic massage while allowing you to place an intravenous line
up or adhere to medical recommendations. and administer fluids. The patient is significantly improved after the massage and
It is unwise not to acknowledge the cultural divide that exists intravenous fluids. The family appreciates that you respected their beliefs and
agrees to return for a routine health supervision visit in 2 weeks.
between most pediatric practitioners and the families they serve.
Therefore, pediatricians who are less familiar with the enrichment
provided by a multicultural patient population may be less inclined
to advocate for social reforms that may prove beneficial. Physicians
Selected References
must approach families with an open mind, respect, and a sense of
humility if they are to gain families’ trust, close the gaps that sepa- American Academy of Pediatrics Committee on Infectious Diseases. Red Book:
rate, and promote the well-being of children. 2018–2021 Report of the Committee on Infectious Diseases. Kimberlin DW,
Brady MT, Jackson MA, Long SS, eds. 31st ed. Itasca, IL: American Academy
Children are better served when their families receive infor-
of Pediatrics; 2018
mation about federal, state, and community programs that pro-
American Academy of Pediatrics Council on Community Pediatrics. Providing
vide resources to immigrant families. In addition to health care,
care for immigrant, migrant, and border children. Pediatrics. 2013;131(6):e2028–
pediatricians can provide families with information about edu-
e2034 PMID: 23650300 https://doi.org/10.1542/peds.2013-1099
cational programs, such as Head Start, that are not restricted by
Child Trends [data bank]. Immigrant Children. https://www.childtrends.org/
immigration status. All children, regardless of their immigration
indicators/immigrant-childrenOctober 2014. Accessed September 9, 2019
or socioeconomic status, should receive compassionate, cultur-
Flores G, Brotanek J. The healthy immigrant effect: a greater understand-
ally competent, and linguistically effective care services. Such
ing might help us improve the health of all children. Arch Pediatr Adolesc
care requires that health professionals incorporate knowledge, Med. 2005;159(3):295–297 PMID: 15753277 https://doi.org/10.1001/
attitudes, and skills in cultural and linguistic competence within archpedi.159.3.295
their professional agenda. Grayson J. Serving immigrant families. Virginia Child Protection Newsletter.
The well-being of all children can be advanced through advo- 2011;90:1–20
cacy, especially on behalf of vulnerable children. Such advocacy Guendelman S, Schauffler HH, Pearl M. Unfriendly shores: how immigrant chil-
addresses outreach efforts to children who are potentially eligible dren fare in the U.S. health system. Health Aff (Millwood). 2001;20(1):257–266
for Medicaid and the Children’s Health Insurance Program but are PMID: 11194849 https://doi.org/10.1377/hlthaff.20.1.257
not enrolled, simplifies enrollment for both programs, and expands Jenista JA. The immigrant, refugee, or internationally adopted child. Pediatr Rev.
state funding for those who are not eligible. The medical commu- 2001;22(12):419–429 PMID: 11731683 https://doi.org/10.1542/pir.22-12-419
nity must collaborate with legislators, families, and organizations Mendoza FS. Health disparities and children in immigrant families: a research
representing underserved populations to increase the effective- agenda. Pediatrics. 2009;124(suppl 3):S187–S195 PMID: 19861469 https://doi.
ness of one’s own advocacy effort. All children should receive care org/10.1542/peds.2009-1100F
in a medical home (ie, the establishment of comprehensive, coor- National Immigration Law Center. Guide to Immigrant Eligibility for Federal
dinated, and continuous health care services). This is especially Programs. 4th ed. Santa Monica, CA: National Immigration Law Center; 2002
critical for immigrant children with chronic and mental health Woods T, Hanson D, Saxton S, Simms M. Children of Immigrants 2013 State
care needs. Trends Update. Washington, DC: Urban Institute; 2016

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 CHAPTER 42

Well-Child Care for

Children With Trisomy 21
(Down Syndrome)
Moin Vera, MD, PhD, and Henry J. Lin, MD

CASE STUDY
A 6-month-old girl with trisomy 21 (ie, Down syndrome) on each hand, and mild generalized hypotonia. Her eyes
whom you have known since birth is brought to your have symmetric movement, and her tympanic mem-
office for well-child care. She and her parents have been branes are clear. She has no cardiac murmurs.
doing well, although she has had several episodes of
upper respiratory infections. Her medical history is sig-
Questions
1. What is the prevalence of trisomy 21 (ie, Down syn-
nificant for a small ventricular septal defect, which has
drome) in the general population? What is the asso-
since closed spontaneously, as well as 1 episode of oti-
ciation of maternal age with trisomy 21?
tis media at 5 months of age. Her weight gain has been
2. What are the clinical manifestations of Down
good—along the 25th percentile on the growth chart
syndrome?
for children with Down syndrome. Currently, she sleeps
3. What medical conditions are associated with tri-
through the night and has a bowel movement once a
somy 21 in the newborn period, during childhood,
day. She has received all the recommended immuniza-
and in adolescence? When should screening tests for
tions for her age without any problems.
these conditions be performed?
The infant smiles appropriately, grasps and shakes
4. What is the role of early intervention services for
hand toys, and has some head control but cannot roll
patients with trisomy 21 and their families?
from supine to prone position. Since 1 month of age, she
5. What specific psychosocial issues should be included
has been enrolled in an early intervention program. An
in the anticipatory guidance and health education
occupational therapist visits her at home twice a month.
provided by the physician?
On physical examination, she has typical facial fea-
6. What is the prognosis for the child with trisomy 21?
tures consistent with trisomy 21, a single palmar crease

Trisomy 21, or Down syndrome, is the most common genetic Epidemiology

cause of intellectual disability in children. Primary care physicians
According to the Centers for Disease Control and Prevention, the
are in a unique position to offer affected children specific health
prevalence of trisomy 21 is approximately 1 in 700 live births in
maintenance and anticipatory guidance, help manage intercurrent
the United States. It is estimated that trisomy 21 is responsible
illness and chronic problems, and apply the latest recommenda-
for up to one-third of all cases of moderate to severe intellectual
tions for clinical screening based on risks for conditions associ-
disability. Trisomy 21 has a male-to-female ratio of 1.3:1.
ated with trisomy 21. Additionally, the general pediatrician has
Ninety-five percent of all cases of Down syndrome are caused
the opportunity to develop rapport with children and families,
by chromosomal nondisjunction, and most of these events occur
which is of particular importance because of the complex medi-
in oocytes during meiosis. The probability of maternal chromo-
cal and social implications of raising a child with trisomy 21. An
somal nondisjunction is determined by maternal age. A 25-year-old
important goal of the well-child visit is to provide children with
woman has a 1 in 1,240 risk for having a live baby with Down syn-
trisomy 21 and their families with counseling about educational,
drome, and the risk increases to 1 in 340 at age 35 years and 1 in
social, and financial resources and support to ensure a healthy
98 at age 40 years. It is estimated that approximately one-half of
and productive transition into adulthood.

291

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 292 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

trisomy 21 embryos abort spontaneously. The risk of recurrence of hands, and digits are small and stubby (ie, brachydactyly), and
the nondisjunction type of Down syndrome in subsequent pregnan- the fifth digit may be hypoplastic and turned in (ie, clinodactyly).
cies is 1 in 100 until age 35 years, after which the risk determined A single palmar crease and wide spacing between the first and
by age takes precedence. Other family members generally are not at second toes may be evident. After the newborn period, diffuse
increased risk of bearing children with this type of Down syndrome. hypotonia and developmental delay are universally seen.
Recent advances in first and second trimester screening allow pre-
natal diagnosis of Down syndrome with a sensitivity of 80% to 90%. Pathophysiology
If a prenatal diagnosis of Down syndrome is made and the general
Down syndrome is caused by trisomy 21 resulting from meiotic non-
pediatrician is asked to participate in counseling the family, the pedi-
disjunction (approximately 95% of cases), translocation (3%–4%
atrician should go over with the family the points listed in Box 42.1.
of cases), and mosaicism (1%–2% of cases). A small percentage of
affected children have a chromosomal rearrangement resulting in
Clinical Presentation 3 copies of a portion of chromosome 21.
Newborns, infants, and children with trisomy 21 have a character- Translocations in an affected child are unbalanced and usually
istic appearance (Box 42.2). They may exhibit microcephaly, with occur between chromosome 21 and another acrocentric chromo-
flattening of the occiput and face. The eyes have an upward slant some, most commonly chromosome 14 or 15. Approximately 75%
with prominent epicanthal folds, the ears are small and set low, the of these translocations are de novo rearrangements, and 25% are
nasal bridge is flattened, and the tongue appears large. The feet, the result of a familial translocation. In the latter case, a parent may
be an unaffected carrier of a balanced translocation involving the
long arms of chromosome 21 and another acrocentric chromosome
Box 42.1. Counseling the Family After a Prenatal (ie, robertsonian translocation). Thus, if a child is found to have a
Diagnosis of Down Syndrome translocation, parental karyotypes must be assessed because bal-
anced translocation carriers have an increased risk of Down syn-
ww Review the data that established the diagnosis in the fetus.
drome in future children. Mosaicism implies the presence of 2 cell
ww Explain the mechanism of occurrence and risks for recurrence.
lines—1 normal and 1 with trisomy 21. As might be expected, chil-
ww Review the manifestations of trisomy 21, commonly associated con-
dren with mosaic Down syndrome are usually affected less severely
ditions, and the variable prognosis based on the presence of these
than children with other types of Down syndrome.
conditions.
Research has focused on the role of individual genes, such as
ww Discuss other modalities that confirm the presence of associated anom-
DYRK1A, in the pathogenesis of Down syndrome. It is expected that
alies, such as fetal echocardiography in the case of congenital heart
in the near future, pharmacologic agents that mitigate the effects
disease.
of excess expression of such genes will result in new treatments for
ww Explore treatment options and interventions for associated conditions.
patients with Down syndrome.
ww Offer resources to assist the family with decisions about completing or
terminating the pregnancy.
Evaluation
ww Refer the family to a clinical geneticist or genetic counselor.
Routine health maintenance for newborns, infants, children, and
adolescents with trisomy 21 should include discussion of the same
issues of health education, prevention, and counseling that are
discussed with other patients and their families. The schedule of
Box 42.2. Diagnosis of Trisomy 21 health maintenance visits for newborns, infants, and young chil-
dren with trisomy 21 is essentially the same as that recommended
ww Microcephaly, with flattening of occiput and face
by the American Academy of Pediatrics for other children, whereas
ww Upward slant to the eyes with epicanthal folds
older children with Down syndrome should be evaluated annually.
ww Brushfield spots on the irises
Surveillance and anticipatory guidance related to the additional
ww Small ears and mouth (tongue appears large in relation to the mouth)
medical and psychosocial conditions common among patients with
ww Low-set ears
trisomy 21 is tailored to the main periods in a child’s life: newborn,
ww Flat nasal bridge
infancy and early childhood, and older childhood and adolescence.
ww Broad, stocky neck with loose skin folds at the nape
ww Funnel-shaped or pigeon-breasted chest
ww Small, stubby feet, hands, and digits (ie, brachydactyly); the fifth digit
Newborn Period
may be hypoplastic and turned in (ie, clinodactyly) Verification of the diagnosis of trisomy 21 is perhaps the single most
ww Single transverse palmar crease on each hand important focus of the initial family visit. Sometimes the diagno-
ww Wide space between first and second toes sis has been suspected prenatally because of abnormal biochemical
ww Fair, mottled skin in newborns; dry skin in older children markers and sonographic findings and verified by chorionic villus
ww Hypotonia sampling or amniocentesis (Box 42.1). If no prenatal testing data are
available and the diagnosis is suspected based on clinical findings,

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 CHAPTER 42: WELL-CHILD CARE FOR CHILDREN WITH TRISOMY 21 (DOWN SYNDROME) 293

a karyotype test must be performed while in the nursery and the Bilateral red reflexes and conjugate gaze should be documented to
results reviewed at the 1- to 2-week visit. In some institutions, a exclude congenital cataracts or strabismus. A careful cardiac exam-
fluorescence in situ hybridization test for trisomy 21 may provide a ination must be performed, noting any cyanosis, murmurs, irregu-
more rapid confirmation of the diagnosis (1–3 days). lar heart rates, abnormal heart sounds, or asymmetry of pulses. The
Several conditions associated with trisomy 21 are important to abdomen should be palpated for organomegaly or any masses, and
identify in the newborn period, including hearing loss, congenital patency of the anus should be verified. Ortolani and Barlow maneu-
heart disease (most commonly endocardial cushion defect), duode- vers should be performed for hip laxity. Finally, the newborn should
nal atresia, Hirschsprung disease, congenital hypothyroidism, hip be evaluated for hypotonia.
dislocation, and ocular anomalies (ie, cataracts, glaucoma, strabis-
mus, nystagmus). Hematologic abnormalities include polycythemia, Laboratory Tests
leukemoid reactions that resemble leukemia but resolve during the Karyotyping is an important tool in verifying the diagnosis and
first month after birth, and, rarely, leukemia. assessing the risk of recurrence. Newborn screening laboratory
tests must be reviewed, especially hearing evaluations and thyroid
History screenings. Additionally, a cardiac evaluation for congenital heart
A feeding history is critical because hypotonia often results in diffi- disease should be performed, which may include electrocardiog-
culty swallowing (Table 42.1). A history of vomiting may be indicative raphy, chest radiography, echocardiography, and formal cardiol-
of gastroesophageal reflux or, less commonly, gastrointestinal malfor- ogy referral. A complete blood cell count is indicated to assess for
mation. Constipation may be the first indication of Hirschsprung dis- hematologic abnormalities, including leukemoid reaction, transient
ease or hypothyroidism. A detailed family and social history should myeloproliferative disorder (ie, pancytopenia, hepatosplenomegaly,
also be obtained if this was not done in the hospital. and immature white blood cells), and neonatal thrombocytopenia.

Physical Examination Management

A detailed physical examination should reveal some common fea- The primary care physician should discuss the increased propen-
tures of newborns with Down syndrome (Box 42.2). All growth sity for respiratory infections in children with Down syndrome.
parameters should be recorded and compared with those obtained Other important issues to address include those pertaining to avail-
at birth. The size of the fontanels should be evaluated because of the able resources for children and families, such as early intervention
increased incidence of congenital hypothyroidism in this population. programs and Down syndrome support groups in the community.

Table 42.1. Health Supervision for Children With Down Syndrome

Evaluation Type Assessment Age at Initial Evaluation Timing of Subsequent Evaluations
History and physical Developmental Newborn Same as regular well-child care schedule until age 5 years,
examination Visual and hearing (subjective) then annually.
Laboratory assessment Karyotyping Newborn None.
Thyroid screening Newborn screening 6 months and 12 months, then annually thereafter.
Complete blood cell count Newborn 0–1 month, after which hemoglobin level is assessed
annually.
Celiac screening 2–3 years Measure tissue transglutaminase IgA antibody levels.a
Echocardiography Newborn Condition dependent.
Neck radiography 3–5 years Obtain in symptomatic individuals with pain or neurologic
abnormalities. May be required for Special Olympics entry.b
Consultation Genetics Newborn As needed.c
Cardiology Newborn Condition dependent.
Ophthalmology 6 months Annually until age 5 years, then every 2 years until age
13 years, then every 3 years.
Ear, nose, and throat As needed As needed.
Dental By 1 year Twice per year.
Pulmonary As needed As needed.
a
Lack of expert consensus on evaluation. Some recommend testing every 5 years and others recommend testing only symptomatic individuals.
b
Lack of expert consensus on evaluation.
c
Depending on experience and comfort level of primary care physician.

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 294 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Educational materials, such as pamphlets and books, may also be immunoglobulin A level, at 2 to 3 years of age and every 5 years
supplied at this time. Upcoming appointments with other physicians thereafter. Current American Academy of Pediatrics recommenda-
and allied health professionals should be reviewed. tions include screening only symptomatic children, however.
Children with Down syndrome have an increased incidence
of atlantoaxial instability when screened with routine lateral cer-
Infancy and Early Childhood
vical radiographs with flexion and extension views. Any patient
History with signs or symptoms of spinal cord compression should be eval-
Some additional history-related issues to address include a detailed uated on computed tomography or magnetic resonance imaging
developmental assessment focusing on progress made since the pre- and referred to an orthopedic surgeon or neurosurgeon. The symp-
vious visit, because most affected children have motor and speech tomatic child should be kept out of any sports that involve con-
delays (see Table 42.1). It is important to review any ancillary ser- tact or neck extension, such as swimming, gymnastics, and soccer.
vices, such as physical, occupational, and speech therapy, in antic- Experts agree that careful neurologic screening at health supervi-
ipation of school entry. The parent or parents should provide their sion visits is a much better predictor of serious injury than cervical
assessment of the child’s vision and hearing. A history of recur- radiographs. The current recommendation is to screen only symp-
rent respiratory infections is concerning for recurrent otitis media tomatic children, unless a preparticipation radiograph is required
with the associated risk of hearing loss. Many children with trisomy for events, such as the Special Olympics.
21 experience constipation. A history of snoring and restless sleep
may be indicative of obstructive sleep apnea, and a sleep study Management
may be necessary. Finally, it is extremely important to document Infants with trisomy 21 should undergo all routine screening tests
any history of neck pain, gait changes, increased clumsiness, or and immunizations. Growth and developmental progress should be
other neurologic symptoms that would be indicative of spinal cord reviewed with the parent or parents at the end of each visit, and any
compression resulting from atlantoaxial dislocation. concerns or unmet expectations should be addressed at this time.
Often the developmental delay associated with trisomy 21 is not
Physical Examination apparent to families until an infant is 4 to 6 months of age and not
All growth parameters should be plotted on growth charts specific to achieving the expected milestones of rolling over or sitting. It should
children with Down syndrome (Figures 42.1 and 42.2). Children with also be emphasized to families that the severity of intellectual dis-
trisomy 21 are shorter than other children and may have poor weight ability in trisomy 21 is quite variable, ranging from mild to severe.
gain in their first year. Later in life, obesity unrelated to the syndrome Social function is not necessarily related to IQ, however. If the child is
may become a problem. As with routine well-child visits in other infants not already enrolled in an early intervention program, the physician
and children, a complete physical examination should be performed should emphasize to the parent or parents the positive role of such an
at each patient encounter. Noteworthy aspects of the examination in experience. The availability of support groups for parents and other
infants and children with trisomy 21 are presented in Box 42.3. In par- family members should also be discussed. The role of support groups
ticular, the ear canals of these children are easily collapsed, making it may be especially beneficial to the patient with both Down syndrome
difficult to visualize the tympanic membrane. In some cases, referral to and autism spectrum disorder.
an otolaryngologist may be necessary for an adequate otoscopic exam- In the early childhood years, plans for preschool attendance and
ination. A complete neurologic examination should be performed at future educational opportunities should be reviewed with the fam-
each visit, including an assessment of the severity of hypotonia. ily. The role of discipline and the presence of common behavioral
problems, such as temper tantrums and biting, should be assessed
Laboratory Tests
in preparation for school entry and socialization. Nutrition should
Hearing evaluation should be performed annually, starting with be reviewed, because children with Down syndrome have a reduced
the newborn hearing screening. Developmentally appropriate gross basal energy expenditure and are at increased risk for obesity.
visual screening should be performed at each visit in infants between Nutritional supplements and other alternative medicines have not
6 and 12 months of age, and a formal ophthalmologic examination been proved to have any efficacy in the treatment of patients with
is recommended starting at 6 months of age. Thyroid screening tests Down syndrome.
should be repeated at 6 and 12 months and then annually. As is recommended for all children, a dental referral should be
Children with congenital heart disease should be given anti- made by 1 year of age.
biotic endocarditis prophylaxis for dental or other procedures.
Additionally, these children should be considered for monoclonal
Older Childhood and Adolescence
antibody therapy against respiratory syncytial virus in the winter.
Children with Down syndrome are at increased risk for autoim- History
mune disorders, such as celiac disease, Graves disease, and type 1 School-age children with Down syndrome should continue to
diabetes. Because the signs of celiac disease may be subtle, some visit their primary care physician at least annually. Educational
pediatric gastroenterologists recommend measuring tissue issues should be discussed, including the Individualized Education
transglutaminase immunoglobulin A antibodies as well as an Program and transition from school. Specific medical issues to

BPPCA6e_Ch042_0291-0298.indd 294 2/13/20 5:40 PM

 Chapter 42: Well-Child Care for Children With Trisomy 21 (Down Syndrome) 295

Male subjects—weight Female subjects—weight

18 18

16 95th 16 95th

14 14

50th 50th
12 12
Weight, kg

Weight, kg
10 5th 10 5th

8 8

6 6

4 4

2 2
0 5 10 15 20 25 30 35 0 5 10 15 20 25 30 35
A Age, months B Age, months

Male subjects—height Female subjects—height

100 100

95th
95th
90 90
50th
50th
80 5th 80
5th
Length, cm

Length, cm

70 70

60 60

50 50

40 40
0 5 10 15 20 25 30 35 0 5 10 15 20 25 30 35
C Age, months D Age, months

UK 2002 US 1988 DSGS

Figure 42.1. Curve comparisons for weight in kilograms and length in centimeters for male and female subjects, birth to 36 months of age. Contemporary curves
from the Down Syndrome Growing Up Study (DSGS [solid line]) are compared with those from the US 1988 curves from Cronk et al (dotted line) and the UK 2002
curves from Styles et al (dashed line).
Reprinted with permission from Zemel BS, Pipan M, Stallings VA, et al. Growth charts for children with Down syndrome in the United States. Pediatrics. 2015;136(5):e1204–e1211.

address during the history include visual or hearing deficits; upper cheeks) during adolescence. A cardiac examination is impor-
evidence of hypothyroidism (ie, decreased activity, coarse and dry tant because of an increased risk of mitral valve prolapse and val-
hair, constipation); skin problems, including eczema; and dental vular dysfunction. The sexual maturity rating (ie, Tanner stage) of
problems. A careful nutritional history should also be obtained all patients should be noted and discussed with the parent or par-
because of the propensity for obesity, and the child should be closely ents. A pelvic examination is not indicated as a part of the routine
monitored for signs of obstructive sleep apnea. visit unless concern exists for sexual abuse or a sexually transmitted
infection; however, a testicular examination is important because of
Physical Examination the increased risk for testicular cancer in patients with Down syn-
The physician should continue to plot height and weight measure- drome. Patients who participate in sports and other physical activ-
ments. The skin should be examined closely for xerosis, acne, or ities should undergo a complete neurologic examination to assess
syringomas (ie, multiple papules, often present on the eyelids and for signs of impending atlantoaxial dislocation.

BPPCA6e_Ch042_0291-0298.indd 295 2/13/20 5:40 PM

 296 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Male subjects—weight Female subjects—weight

100 100
95th 95th

80 80

50th 50th
60 60
Weight, kg

Weight, kg
5th

40 40 5th

20 20

0 0
4 8 12 16 20 4 8 12 16 20
A Age, years B Age, years

Male subjects—height Female subjects—height

180 180
95th
160 50th 160
95th
5th 50th
140 140
5th
Weight, cm

Weight, cm

120 120

100 100

80 80

60 60
4 8 12 16 20 4 8 12 16 20
C Age, years D Age, years

UK 2002 US 1988 DSGS

Figure 42.2. Curve comparisons for weight in kilograms and height in centimeters for male and female subjects, 2 to 20 years of age. Contemporary curves
from the Down Syndrome Growing Up Study (DSGS [solid line]) are compared with those from the US 1988 curves from Cronk et al (dotted line) and the UK
2002 curves from Styles et al (dashed line).
Reprinted with permission from Zemel BS, Pipan M, Stallings VA, et al. Growth charts for children with Down syndrome in the United States. Pediatrics. 2015;136(5):
e1204–e1211.

Laboratory Tests gingivitis, periodontal disease, and bruxism (ie, teeth grinding) are
common in these individuals.
Annual thyroid screening for thyroid-stimulating hormone and
thyroxine levels is recommended for all school-age children and
adolescents with trisomy 21, in addition to other routine screening Management
tests. Hearing evaluation should also occur at least once in older The major part of the visit with school-age and adolescent children
children and annually thereafter. Because of the risk of keratoco- should focus on developmental, educational, and vocational antic-
nus, an annual ophthalmologic consultation should be conducted ipatory guidance. Educational placement and future goals should
after the age of 10 years. Additionally, the child with trisomy be developmentally appropriate for the child and acceptable to
21 should be encouraged to continue biannual dental visits, because the parent. Activities requiring socialization and development of

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 Chapter 42: Well-Child Care for Children With Trisomy 21 (Down Syndrome) 297

mortality is the manifestation of symptomatic Alzheimer disease,

Box 42.3. Physical Examination
which occurs in approximately 15% of adults after the fourth decade.
of the Child With Trisomy 21
Many adults with Down syndrome remain asymptomatic, however,
ww Look for dry, sensitive skin and alopecia, which manifests in approximately despite histopathologic evidence of the disease.
10% of children and resolves spontaneously.
ww Monitor the size of the anterior and posterior fontanels, because delayed
closure may be indicative of hypothyroidism.
ww Check for visual abnormalities, such as strabismus, nystagmus, cataracts, CASE RESOLUTION
refractive errors, and blepharitis. The family should be encouraged by the healthy progress of the patient. For
ww Document recurrent serous otitis media. It is estimated that 40%–60% this visit, anticipatory guidance should consist of a review of early interven-
tion services, available resources, and general support services for the patient
of children with trisomy 21 have significant conductive hearing loss and
and her family. The increased risk for upper respiratory infections and otitis
20%–30% have some degree of neurosensory loss. media should be reviewed. Medical screening should include thyroid screen-
ww Examine the oropharynx carefully for delayed dentition, malocclusion, ing, subjective hearing screening, and a formal evaluation by a pediatric
and caries. ophthalmologist. If the results are normal, the next visit should take place
ww Auscultate for stridor, wheezing, or other abnormal breathing that may 3 months hence.
indicate airway anomalies.
ww Perform a thorough cardiac examination and note any evidence of
previously unrecognized congenital heart disease.
ww Palpate the abdomen for distention or organomegaly. Children with Selected References
trisomy 21 have a slightly increased risk of developing acute non-
Bull MJ; American Academy of Pediatrics Committee on Genetics. Health super-
lymphoblastic or acute lymphoblastic leukemia. vision for children with Down syndrome. Pediatrics. 2011;128(2):393–406.
ww Perform a rectal examination in infants or children with a history of Reaffirmed January 2018 PMID: 21788214 https://doi.org/10.1542/peds.
constipation. 2011-1605
ww Evaluate any musculoskeletal abnormalities such as overall hypotonia Cassidy SB, Allanson JE, eds. Management of Genetic Syndromes. 3rd ed.
and joint laxity (most commonly in the knees and hips) that might Hoboken, NJ: John Wiley & Sons; 2010 https://doi.org/10.1002/9780470893159
contribute to overall gross motor delay. Cohen WI. Current dilemmas in Down syndrome clinical care: celiac dis-
ease, thyroid disorders, and atlanto-axial instability. Am J Med Genet C Semin
Med Genet. 2006;142C(3):141–148 PMID: 16838307 https://doi.org/10.1002/
ajmg.c.30102
Eggers K, Van Eerdenbrugh S. Speech disfluencies in children with Down
responsibility should continue to be encouraged; however, these
syndrome. J Commun Disord. 2018;71:72–84 PMID: 29129311 https://doi.
events can be very stressful for parents and other family members.
org/10.1016/j.jcomdis.2017.11.001
Injury prevention should be highlighted as well, especially because
Hankinson TC, Anderson RC. Craniovertebral junction abnormalities in Down
older children are becoming more independent. In early adolescence,
syndrome. Neurosurgery. 2010;66(suppl 3):32–38 PMID: 20173525 https://doi.
prevocational and vocational training within the school curriculum org/10.1227/01.NEU.0000365803.22786.F0
should be reviewed. Additionally, brief discussions about indepen-
Hickey F, Hickey E, Summar KL. Medical update for children with Down syn-
dent living, group homes, transition of medical care, and financial drome for the pediatrician and family practitioner. Adv Pediatr. 2012;59(1):
resources (eg, community-supported employment for young adults) 137–157 PMID: 22789577 https://doi.org/10.1016/j.yapd.2012.04.006
should begin during adolescence. Maranho DA, Fuchs K, Kim YJ, Novais EN. Hip instability in patients with Down
For older children and adolescents with trisomy 21, puberty, fer- syndrome. J Am Acad Orthop Surg. 2018;26(13):455–462 PMID: 29851695
tility, and contraception are extremely important issues to address https://doi.org/10.5435/JAAOS-D-17-00179
with both the patients and their parents. The patient’s psychosocial Mitra S, El Azrak M, McCord H, Paes BA. Hospitalization for respiratory syn-
development and physical sexual maturation should be discussed, cytial virus in children with Down syndrome less than 2 years of age: a system-
including menstrual hygiene and any foreseeable problems with atic review and meta-analysis. J Pediatr. 2018;203:92–100.e3 PMID: 30266507
its management. Contraception and the potential for victimization https://doi.org/10.1016/j.jpeds.2018.08.006
must be addressed as well, particularly with female patients. Males O’Leary L, Hughes-McCormack L, Dunn K, Cooper SA. Early death and causes
with trisomy 21 usually are sterile, but females have an approx- of death of people with Down syndrome: a systematic review. J Appl Res
imately 50% chance of having children of their own with Down Intellect Disabil. 2018;31(5):687–708 PMID: 29573301 https://doi.org/10.1111/
jar.12446
syndrome.
Santoro SL, Bartman T, Cua CL, Lemle S, Skotko BG. Use of electronic health record
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Prognosis PMID: 30154119 https://doi.org/10.1542/peds.2017-4119
Individuals with trisomy 21 often can live well past the age of Simpson R, Oyekan AA, Ehsan Z, Ingram DG. Obstructive sleep apnea in patients
50 years, unless they are born with a congenital heart lesion, which with Down syndrome: current perspectives. Nat Sci Sleep. 2018;10:287–293
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15583773 https://doi.org/10.1177/000992280404300902 Zemel BS, Pipan M, Stallings VA, et al. Growth charts for children with Down
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 CHAPTER 43

Well-Child Care
for Preterm Infants
Soina Kaur Dargan, MD, FAAP, and Lynne M. Smith, MD, FAAP

CASE STUDY
A 10-week-old girl was discharged from the neonatal and, according to the family, is becoming progressively
intensive care unit 2 weeks previously, where she had more alert. She sleeps on her back in a crib.
resided since birth. She was the 780 g (27.5 oz) prod- The infant’s weight gain has averaged 25 g (0.9 oz)
uct of a 26-week gestation born via spontaneous vagi- per day. The remainder of the physical examination is
nal delivery to a 32-year-old primigravida. The perinatal normal, with the exception of dolichocephaly and esotro-
course was complicated by premature rupture of mem- pia of the left eye.
branes and maternal amnionitis. Several aspects of the
neonatal course were significant, including respiratory
Questions
1. What constitutes well-child care in preterm infants?
distress that required surfactant therapy and 2 weeks
2. What are the nutritional requirements of preterm
of endotracheal intubation; a grade 2 intraventricular
infants in the months after discharge from the
hemorrhage diagnosed at 1 week after birth; hyperbil-
hospital?
irubinemia, which was treated with phototherapy; sev-
3. What information must be considered in the
eral episodes of apnea, presumably associated with the
nutritional assessment and developmental screen-
preterm birth; and a history of poor oral intake with slow
ing of preterm infants?
weight gain.
4. What immunization schedule is appropriate for
The parents have a few questions about her feeding
preterm infants? Do they require any special
schedule and discontinuing the apnea monitor, but they
immunizations?
feel relatively comfortable caring for their daughter
5. What specific conditions or illnesses are more likely
at home. She is feeding well (2 oz of 22 cal/oz post-
to affect preterm infants than term infants?
discharge formula for preterm infants every 2–3 hours)

Preterm birth is defined as birth before 37 completed weeks of is essential to the optimal heath and developmental outcome of the
gestation. However, the increased frequency of adverse neonatal patient who was born preterm.
outcomes in neonates born at 37 and 38 weeks’ gestation led the
American College of Obstetricians and Gynecologists to redefine Epidemiology
optimal delivery as 39 weeks’ gestation to eliminate nonmedically In the United States, the Centers for Disease Control and Prevention
indicated deliveries prior to this time. Because advances in neona- (CDC) reports that preterm birth rates decreased from 2007 to 2014,
tal care have resulted in improved survival, an increased demand in part because of a decline in the number of births to teenagers and
exists for skilled primary care physicians who can care for the pre- young mothers. However, since 2016, preterm births are once again
term infant. Providing primary care for these infants is an impor- on the rise, the cause of which is largely unknown.
tant and challenging task and often requires coordination of medical, The preterm delivery rate is highest for black women and low-
developmental, and social services for multiple chronic conditions. est for white women (14% and 9%, respectively). The increase
Because preterm infants are at increased risk for impaired growth in preterm births has occurred among late preterm newborns
and developmental delay, longer well-child visits may be necessary (>34 weeks’ gestation), who comprise 70% of preterm births.
to evaluate their nutritional and developmental progress and assess Reasons for the increased preterm delivery rates include increased
how families have adjusted to caring for them at home. Primary use of artificial reproductive technologies (see Chapter 26), increased
care physicians must learn to manage these and many other com- interest in elective cesarean section, and increased maternal age.
plex issues while providing families with comprehensive anticipa- Although most preterm newborns are delivered at greater than
tory guidance. Providing a medical home in which care is accessible, 34 weeks’ gestation, these neonates are at increased risk for morbidity
comprehensive, continuous, culturally sensitive, and family oriented and mortality compared with neonates born at term. Additionally,

299

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 300 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

many neonatologists routinely resuscitate neonates born at 23 weeks’

Box 43.1. Neonatal Intensive Care Unit
gestation, a gestation with a survival rate of approximately 25%. Very
Discharge Summary Information
low-birth-weight (VLBW, <1,500 g [<52.9 oz]) and extremely low-
birth-weight (ELBW, <1,000 g [<35.3 oz]) newborns are at risk for 1. Birth weight, gestational age, and significant prenatal and perinatal
cerebral palsy, respiratory disease, hearing and vision problems, and information, including delivery room details
intellectual disabilities. Furthermore, learning disabilities, attention- 2. Overview of the hospital course by system, including significant ill-
deficit/hyperactivity disorder, borderline to low IQ scores, psychiat- nesses, events, surgical procedures, and pertinent radiographic and
ric disorders or abnormalities in executive function, and visuomotor other diagnostic studies
integration occur in more than 50% of VLBW infants, thereby com- 3. Nutrition information and present feeding regimen
plicating post-discharge care for these children. In 2007, the Institute 4. A list of current medications, including dosing intervals and, if appro-
of Medicine (now the National Academy of Medicine) estimated the priate, the latest serum drug levels
annual societal cost of preterm birth at $26 billion and individual 5. Immunizations administered during the hospitalization
family cost of $2 million. 6. Pertinent laboratory and diagnostic data, such as most recent hemo-
globin level, newborn screening results, ophthalmologic and hear-
Pathophysiology ing screening information, neurosonography and magnetic resonance
imaging results, and highest serum bilirubin level
In utero, the placenta serves several functions, including promot-
7. Discharge physical examination, including most recent height, weight,
ing and regulating fetal growth, providing nutrients, and preventing
and head circumference
infection by acting as a barrier. Delivery before 39 weeks of gesta-
8. Parental or other social concerns throughout patient’s stay in the
tion halts this process, and these immature organs have functional
neonatal intensive care unit
limitations. Causes of preterm delivery can be maternal, the most
9. Problems remaining at discharge
common of which is pregnancy-induced hypertension, or fetal, the
10. All follow-up appointments
most common of which is premature rupture of membranes result-
ing from chorioamnionitis. Regardless of cause, these infants have
special needs that should be addressed at every primary care visit.
Box 43.2. What to Ask
Evaluation Well-Child Care for Preterm Infants
The purpose of the health maintenance visit for preterm infants is ww How much did the infant weigh when discharged from the hospital?
the same as for other healthy children: to provide consistent pre- ww Is the infant fed mother’s milk, or is the infant formula-fed? Is the infant
ventive health care and education for patients and their parents. on any special formula?
Preterm status, however, places children at risk for additional med- ww How often and how much does the infant feed? How long do feedings
ical and neurodevelopmental conditions. Compared with children take?
born full term, children born preterm have an average of nearly ww Does the infant have any feeding problems (eg, pain, vomiting, gastro-
3 times the number of well-child visits in the first year after birth esophageal reflux)?
and more hospital readmissions. ww Does the infant take dietary supplementation of vitamins and minerals?
ww What developmental milestones has the infant reached? Does the infant
History roll over? Smile? Sit up?
Almost one-third of preterm newborns are not brought in for their ww Does the infant seem to hear and see?
first scheduled outpatient appointment. Because failure to attend ww Who cares for the infant? Is the primary caregiver getting enough rest?
follow-up appointments has been associated with a higher rate ww Does the infant have regional services and if so, which ones and how often?
of developmental delay, discussions about the importance of ww Where and in what position does the child sleep?
follow-up appointments are ideally initiated with the parent or care- ww Does the parent or caregiver have any concerns about growth, development,
giver before the infant is discharged from the neonatal intensive or nutrition?
care unit (NICU). ww Is the infant on an apnea monitor and/or caffeine? Has the infant experi-
It is imperative to review the entire medical history and hospital enced any apneic episodes?
course before the initial visit and then discuss it with the family at
the appointment. Ideally, the NICU should provide a discharge sum-
mary that includes the information listed in Box 43.1. infant’s current weight versus discharge weight. Infants younger
Any significant complications or concerns should be discussed than 6 months should gain an average of 20 to 40 g (0.7–1.4 oz) per
with parents or caregivers at the earliest opportunity to assess their day. To ensure continued weight gain, many preterm infants are
understanding of these issues and their expectations for improve- discharged from the NICU on a 24-hour feeding schedule, which
ment. Specifically, growth, nutrition, and developmental issues requires that they be fed at least every 3 hours. The necessity for this
should be addressed at each visit (Box 43.2). Adequate or desir- practice should be reevaluated during the first few weeks following
able weight gain should be explained to caregivers in terms of the discharge after infants have demonstrated adequate weight gain.

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 Chapter 43: Well-Child Care for Preterm Infants 301

Developmental history is a critical component of the health The genitalia of all preterm infants should be examined closely
maintenance visit. Parental expectations and observations should for inguinal hernias. Inguinal hernia repair is often deferred until
be noted, and any developmental concerns should be evaluated. The 60 weeks’ corrected gestational age unless incarceration risk is
adjusted developmental age should be calculated by subtracting the deemed high or the family lives far from a pediatric surgeon. Surgery
number of weeks the infant was born preterm from the infant’s cur- is deferred because of concerns about adverse neurodevelopmen-
rent chronologic age in weeks. The adjusted age should then be used tal outcomes in children exposed to general anesthesia in the first
for all formal and informal developmental assessments. The impor- months after birth, the high rates of postoperative apnea, and the
tance of correcting for preterm status until children are 2 years of age occasional spontaneous closure in the first year after birth without
must be emphasized when discussing developmental progress and intervention. If a hernia is surgically repaired under general anes-
giving anticipatory guidance to parents or caregivers. It is impor- thesia, the infant should be monitored for apnea for up to 24 hours
tant to know if the patient is receiving any regional services, which postoperatively. The male scrotum should be examined for cryp­
ones, and how often so an accurate recommendation can be made torchidism, because at term gestation only 25% of testes are in the
if it is necessary to increase these services. scrotum of males born preterm. By 1 year of age, more than 90% of
testes are intrascrotal. A careful evaluation for developmental dys-
Physical Examination plasia of the hip should be performed until children are ambulatory,
A complete physical examination should be performed at each and hip ultrasonography (US) should be performed at 6 weeks of
visit to monitor the status of associated medical conditions. All age for all breech deliveries (see Chapter 113).
growth parameters (ie, weight, height, and head circumference) A thorough neuromuscular examination is essential in children
should be plotted on the growth chart for preterm newborns until born preterm. Increased muscular tone, asymmetry, and decreased
approximately 50 weeks’ postmenstrual age and adjusted for pre- bulk should be noted along with the presence of any clonus or asym-
term status on standard growth charts until age 2 years. Because metry of deep tendon reflexes. Inappropriate reflexes, such as a per-
catch-up head growth generally precedes catch-up weight and sistent Moro reflex or fisting beyond 4 months of age, should also be
length, preterm infants may appear to have disproportionally large documented. Other abnormalities (eg, scissoring, sustained clonus)
heads. The onset of accelerated head growth may begin within in the neurologic examination may become more apparent with age.
a few weeks after birth (36 weeks’ postconception) or as late as The detection of subtle early findings is important so appropriate
8 months adjusted age. Average daily weight gain in grams per intervention services can begin as soon as possible.
day should also be calculated and discussed with the parent or
caregiver at every visit. Laboratory Tests
The size and shape of the head must be evaluated, especially if the In addition to the standard screening tests performed on all
infant has a history of intraventricular or intracranial hemorrhage healthy infants and children during health maintenance visits,
or hydrocephalus. An increase in head circumference of more than several laboratory studies are important for preterm infants.
2 cm (0.8 in) per week should be cause for concern in these infants. Such tests include a hemoglobin test and reticulocyte count to
In infants who have undergone neurosurgical treatment for hydro- assess for anemia; electrolytes in infants with BPD on diuretics to
cephalus, ventriculoperitoneal shunt and tubing may be palpated. detect abnormalities; and serum calcium, phosphorus, and alka-
The head must also be evaluated for positional plagiocephaly, a con- line phosphatase levels in infants with documented metabolic
dition caused by lying in the same position for prolonged periods bone disease of prematurity.
of time. Visual abnormalities, such as strabismus, must be carefully Pulse oximetry is indicated for oxygen-dependent infants as well
ruled out by both physical examination and history because up to as those presenting with respiratory symptoms greater than baseline.
20% of preterm infants may have an ophthalmologic problem (see Results from newborn screening tests, including auditory and oph-
Chapter 91). Oropharyngeal abnormalities, such as a palatal groove, thalmologic examinations, should be reviewed and repeated as indi-
high-arched palate, or abnormal tooth formation, may occur as a cated. Cranial US should be reviewed with caution, because nearly
result of prolonged endotracheal intubation. Baseline intercostal, 40% of infants born weighing less than 1,000 g (35.3 oz) with nor-
substernal, or subcostal retractions; wheezing; stridor; and tach­ mal head US findings develop cerebral palsy or developmental delay.
ypnea in former preterm babies with moderate to severe broncho- Additionally, infants with grade 1 or 2 intracranial hemorrhage are
pulmonary dysplasia (BPD), a form of chronic lung disease, should at increased risk for developmental delay. Brain magnetic resonance
be documented. Infants with BPD have increased susceptibility to imaging should be considered in infants born at less than 30 weeks’
pulmonary infections leading to rehospitalization and may continue gestation or in any infant with a concerning abnormal neurologic
to exhibit poor lung function through adolescence. examination or abnormal rate of head growth.
Chest and back scars secondary to the placement of chest tubes
or patent ductus arteriosus ligation should be noted. Adult female
breasts may be affected if scarring occurs on or close to breast tis- Management
sue. The umbilicus may appear hypoplastic as a result of umbilical Well-child care in relatively healthy preterm infants has 2 compo-
catheter placement and suturing. Scars on the distal extremities from nents. One is the provision of routine health care maintenance for
intravenous catheters and cutdowns may be evident. infants and appropriate developmental anticipatory guidance for

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 302 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

parents or caregivers. The other component involves the incorpo- Nutrition

ration into each visit of treatment for chronic conditions that are
According to the AAP Committee on Nutrition, the average daily
sequelae of preterm birth. Health care maintenance should include
energy requirement for most hospitalized healthy preterm new-
the psychosocial well-being of the family, nutrition counseling,
borns and infants is 105 to 130 kcal/kg/day to achieve adequate
developmental surveillance, immunizations, and assessment of
growth. Energy requirements vary by individual infant depend-
vision and hearing in addition to standard screening tests discussed
ing on associated chronic conditions, such as BPD or malabsorp-
previously. Outside resources concerning developmental delay can
tion. Key nutrients, such as calcium, vitamin D, and phosphorus,
be reviewed with parents or caregivers. Care related to chronic
are critical to the management of bone health in preterm infants.
conditions includes adjusting medication doses, such as diuretic
Growth is considered adequate in the newborn weighing more
therapy; weaning from supplemental oxygen; and discontinuing the
than 2,000 g (>70.5 oz) if weight gain is more than 20 g (>0.7 oz)
apnea monitor.
per day and length and head circumference increase 0.7 to 1.0
Anticipatory Guidance cm (0.3–0.4 in) per week. Feeding difficulties are common in
preterm infants and result in hospital readmission secondary to
Before hospital discharge, parents or guardians should be given
failure to thrive.
anticipatory guidance that caring for a NICU graduate is challeng-
At 40 weeks’ corrected age, preterm newborns usually are smaller
ing (Box 43.3). The American Academy of Pediatrics (AAP) recom-
than term newborns. Adequate nutrition in the first year after birth
mends parental education as 1 of 6 critical components of discharge
is critical, because catch-up weight gain is unlikely to occur after
planning for any high-risk neonate and includes not only educat-
age 3 years. Because human milk is insufficient for providing ade-
ing parents about the patient’s care but also identifying an addi-
quate protein and micronutrients to preterm newborns, fortifier is
tional caregiver who can assist with the demands of caring for the
added to human milk.
child at home. Preterm newborns have poorly organized sleep-wake
If they are not receiving human milk, children born at 34 weeks
cycles, resulting in more frequent awaking than term newborns.
or sooner or weighing less than 1,500 g (<52.9 oz) usually are dis-
Additionally, preterm newborns have immature suck-swallow coor-
charged home on a nutritionally enhanced transitional formula (eg,
dination, causing them to feed more frequently and for longer peri-
Similac Expert Care NeoSure, Enfamil EnfaCare). The calcium, phos-
ods. Colic is reported twice as frequently in VLBW infants compared
phorous, and caloric content of these 22 cal/oz transitional formulas
with infants born at term, and many are described as having diffi-
are between levels found in standard preterm and term formulas.
cult temperament until past their first birthday. Preterm infants also
Use of preterm enriched formulas usually is continued until the child
develop gastroesophageal reflux more often than full-term infants,
reaches the 5th to 10th percentile on the growth chart or is 1 year of
which presents with such symptoms as irritability, respiratory prob-
adjusted age. A meta-analysis showed limited evidence to demon-
lems, and postprandial vomiting.
strate that nutrient-enriched formulas improve growth rates after
The medical costs associated with the care of preterm infants
discharge relative to standard term formula.
often strain the family finances at a time when many women reduce
Formula-fed infants require multivitamin supplementation until
their work schedules. Additional stressors include uncertainty about
750 mL/day of formula is consumed. Preterm infants fed moth-
the long-term outcome of the child, guilt many women feel after
er’s milk may benefit from continuing multivitamin solution and
delivering prematurely, and anxiety about future pregnancies. The
supplementation of oral iron drops as long as human milk is the
difficulty of caring for these complicated, challenging children results
predominant source of nutrition. Vitamin D supplementation is
in significant parental stress and can interfere with parents’ ability
recommended for all infants fed mother’s milk. Because preterm
to properly bond with their babies. Health professionals should be
newborns are at increased risk for metabolic bone disease of pre-
alert to signs of a parent feeling overwhelmed, because a higher
maturity, soy formulas are to be avoided because of the low phos-
caregiving burden is associated with an increased incidence of child
phate content.
welfare reports, such as child neglect.
The introduction of solid foods is appropriate after an infant has
developed acceptable oral-motor skills for swallowing solids. Recent
studies have suggested that 20% of parents of preterm infants report
Box 43.3. Physician Support and Education feeding problems at age 2 years. Cow’s milk should not be introduced
of Parents of or Caregivers until 12 months’ adjusted age.
for Preterm Infants
ww Understand parental/caregiver expectations.
Developmental Surveillance
ww Legitimize parental/caregiver fears. Informal and formal developmental surveillance should include
ww Be a source of support and encouragement. referral to an early intervention program, particularly for ELBW
ww Provide consistent, honest information. infants, because routine screening tests are not sensitive enough
ww Assume the role of the overall coordinator of care. to detect subtle neurodevelopmental abnormalities (Box 43.4;
ww Provide referrals to outside resources, including respite care. see Chapter 32). The Individuals with Disabilities Education Act
Part C guarantees early intervention programs for infants and

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 Chapter 43: Well-Child Care for Preterm Infants 303

family’s ability to pay. Because many developmental issues do

Box 43.4. Typical Speech, Play,
not manifest until school age, frequent developmental assess-
and Physical Development
ments and enrollment in preschool are especially important for
In addition to standardized tests, the following guidelines provide develop- infants born preterm.
ment milestones useful for early detection: The AAP recommends that autism spectrum disorder (ASD)–
By 3 Months’ Adjusted Age, the Infant specific screenings be completed for all childrenat 18 to 24 months
ww Coos or vocalizes other than crying of age. The prevalence of ASD has been reported to be 8% in chil-
ww Visually tracks a moving toy from side to side dren born at less than 26 weeks, which is much higher than that for
ww Attempts to reach for a rattle held above chest the general population. Preterm newborns have higher scores on
ww Pushes up on arms the Modified Checklist for Autism in Toddlers, with greater social-
ww Lifts and holds head up communication difficulties and autistic-like behaviors. These
increased scores may be reflective of the increased incidence of
At 6 Months’ Adjusted Age, the Infant
neurodevelopmental delays in individuals born preterm, making
ww Begins to use consonant sounds in babbling and uses to get attention
screening for ASD more challenging.
ww Reaches for a nearby toy while on tummy
ww Transfers a toy from 1 hand to the other while lying on back Immunizations
ww Reaches both hands to play with feet while lying on back Routine immunization schedules recommended by the CDC
ww Uses hands to support self in sitting Advisory Committee on Immunization Practices should be
ww While standing with support, accepts entire weight with legs followed. The administration of any vaccine is determined by
At 9 Months’ Adjusted Age, the Infant the patient’s chronologic or postnatal age, not gestational age.
ww Increases variety of sounds and syllable combinations in babbling Standard doses and intervals should also be used (see Chapter 37).
ww Looks at familiar objects and people when named Several studies have shown an adequate serologic response
ww Explores and examines an object using both hands despite a history of prematurity. Absolute and relative contra-
ww Turns several pages of a chunky book at once indications to specific vaccine components or to live vaccines
ww Imitates others in simple play for preterm infants are identical to published guidelines for
ww Sits and reaches for toys without falling term infants and children. In addition to following the recom-
At 12 Months’ Adjusted Age, the Child mendation that all infants 6 months and older be vaccinated
ww Meaningfully uses “mama” or “dada” against influenza, the pediatrician should strongly urge that all
ww Responds to simple commands (eg, “come here”) household contacts of preterm newborns be vaccinated. Two
ww Produces long strings of gibberish in social communication doses of influenza vaccine are administered 1 month apart,
ww Finger feeds self with subsequent immunization the following year requiring
ww Uses thumb and pointer finger to pick up tiny objects only 1 dose. Because the rotavirus vaccination utilizes a live
ww Pulls to stand and cruise along furniture virus, it is recommended only for clinically stable preterm
ww Stands alone and takes several independent steps newborns if they are 6 to 14 weeks’ chronologic age at the
time of hospital discharge or have already been discharged from
At 15 Months’ Adjusted Age, the Child the NICU.
ww Has a vocabulary consisting of 5–10 words If adequate weight gain has been established, it is safe to admin-
ww Helps with getting undressed ister hepatitis B vaccine to medically stable infants weighing less
ww Walks independently and seldom falls than 2,000 g (<70.5 oz) as early as 30 days of age or at hospital dis-
ww Squats to pick up toy charge, whichever comes first. All neonates born to mothers who
At 24 Months’ Adjusted Age, the Child are positive for hepatitis B surface antigen should receive hepatitis
ww Uses 2- to 4-word sentences B vaccine and hepatitis B immunoglobulin (HBIG) within 12 hours
ww Walks up and down stairs holding on for support of delivery. When maternal hepatitis B status is unknown, the neo-
ww Builds a tower of 4 blocks or more nate should be vaccinated for hepatitis B within 12 hours of birth. In
ww Identifies basic body parts this situation, if status remains unknown, the neonate weighing less
than 2,000 g (<70.5 oz) should receive HBIG within 12 hours after
Derived from Pathways Awareness Foundation. Assure the Best for your Baby’s Physical Development.
birth and those weighing 2,000 g (70.5 oz) or more should receive
https://pathways.org/topics-of-development/milestones/ as well as Centers for Disease Control and
Prevention. Developmental milestones. CDC.gov website. http://www.cdc.gov/ncbddd/actearly/
HBIG within 7 days after birth.
milestones/index.html. Accessed July 16, 2019. The CDC recommends that all pregnant women receive the teta-
nus toxoid, reduced diphtheria toxoid, and acellular pertussis vaccine
toddlers with disabilities up to age 3 years. Children born weigh- to help protect their newborns against pertussis. Maternal vacci-
ing less than 1,200 g (<42.3 oz) are automatically eligible, but nation results in higher concentrations of pertussis antibodies in
any child with suspected developmental delay is entitled to an newborns compared with unvaccinated mothers, possibly resulting
evaluation to determine eligibility for services regardless of a in increased protection against pertussis during the critical period

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 304 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

between birth and the first diphtheria and tetanus toxoids and acel- The AAP recommends universal hearing screening for all
lular pertussis vaccine dose. newborns. Neonatal intensive care unit graduates account for
Because respiratory syncytial virus (RSV) causes increased approximately 50% of all newborn hearing screening failures,
morbidity and mortality in NICU graduates and infants with con- and severe sensorineural hearing loss occurs in up to 10% of
genital heart disease, administration of the RSV-specific immuno- ELBW infants. Screening is recommended immediately before
globulin palivizumab (eg, Synagis) is recommended every month discharge, and repeat testing is recommended if the child devel-
from November through March (Table 43.1). A documented infec- ops speech delays.
tion with RSV is not an indication to discontinue passive immu-
nization, because multiple strains may circulate during the RSV Other Potential Problems
season. Preterm infants exposed to mechanical ventilation and prolonged
exposure to oxygen are at risk for BPD. These infants are at increased
Assessment of Vision and Hearing risk for respiratory illness, especially during the winter. Parents or
Follow-up visits for visual and auditory sequelae of prematurity caregivers should be informed of this risk and counseled about
must be arranged at the health maintenance visit. An initial symptoms such as tachypnea and wheezing associated with a sim-
ophthalmologic screening examination should have been per- ple upper respiratory infection. Physicians should have a low thresh-
formed between 4 and 9 weeks of age in infants weighing less old for considering a diagnosis of pneumonia in these infants even
than 1,500 g (<52.9 oz) or born less than 30 weeks’ gestation, irre- in the absence of classical symptoms.
spective of oxygen exposure. Infants between 1,500 and 2,000 g The primary care physician should also keep in mind that pre-
(52.9–70.5 oz) or greater than 30 weeks’ gestation with an unsta- term infants are at increased risk for sudden unexpected infant death
ble clinical course should also be screened. The frequency and (SUID; see Chapter 72). The AAP recommends that all infants sleep
need for repeat examinations are determined based on initial find- in the supine position. In the NICU, neonates often are placed on
ings. Regardless of the presence of retinopathy of prematurity, their stomach if they have respiratory difficulties and on their side if
preterm infants are at increased risk for strabismus, myopia, they have symptomatic gastroesophageal reflux. Neonatal intensive
amblyopia, and glaucoma and must undergo an ophthalmo- care unit personnel need to begin placing these babies on their backs
logic examination between 4 and 6 months of age. Because birth in anticipation of discharge. The physician should counsel the parent
before 28 weeks’ gestation is associated with an increased risk of or caregiver about safe sleep practices, that is, placed on the back in
retinal detachment later in childhood and early adult life, long- a crib without blankets, pillows, or other objects. Parents and care-
term follow-up to detect and manage late-onset retinal detach- givers should also be reminded that bedsharing is not recommended
ment should be considered. and is highly associated with SUID from accidental asphyxia.
Home apnea monitors are not associated with the prevention
of SUID and should be reserved for infants who are considered to
Table 43.1. Indications for the Use of Palivizumab
have extreme cardiorespiratory instability or are being discharged
for Respiratory Syncytial Virus Prophylaxis
on oral caffeine. Discontinuation of caffeine and home monitoring
Indication Age at Onset of RSV Season
may be considered at 42 weeks’ postmenstrual age and when signif-
Preterm infant with chronic lung dis- <24 months icant apneic events have ceased, whichever comes later.
ease requiring medical management with Preterm survivors who were critically ill can be particularly at
oxygen, a bronchodilator, diuretics, and risk for developing vulnerable child syndrome because their parents
corticosteroids may continue to perceive them to be fragile and vulnerable. Features
Hemodynamically significant congenital <24 months of this syndrome include abnormal separation difficulties for mother
heart disease with cyanosis and moderate and child, sleep difficulties, parental overprotectiveness and overin-
to severe pulmonary hypertension dulgence, lack of appropriate discipline, tolerance of physical abu-
Significant congenital abnormalities of <12 months siveness by the child toward the parent, and excessive preoccupation
the airway or neuromuscular disease that with the child’s health. Serious behavioral problems may arise as a
compromises handling of respiratory result of such parent-child interactions, and recent studies suggest
secretions a correlation between higher parental perception of child vulnera-
Born ≤28 weeks of gestation <12 months bility and worse developmental outcomes. Primary care physicians
must be cognizant of early signs of this syndrome and should try
Born 29–31 weeks 6 days of gestation <6 months
to prevent its occurrence by reassuring parents or caregivers about
Born 32–34 weeks 6 days of gestation with <3 months the child’s well-being. After vulnerable child syndrome is suspected,
at least 1 of 2 risk factors, whether attend- connecting the child’s history of critical illness with ongoing paren-
ing child care or with a sibling younger tal concerns is important because many parents are unaware that
than 5 years of age in the home current concerns may stem from their unresolved anxiety. Every
Abbreviation: RSV, respiratory syncytial virus. effort should be made to normalize the family’s schedule after the

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 Chapter 43: Well-Child Care for Preterm Infants 305

infant is stable and to encourage parent-child interactions unre- American Academy of Pediatrics Committee on Fetus and Newborn.
lated to health care. Hospital discharge of the high-risk neonate. Pediatrics. 2008;122(5):1119–
1126. Reaffirmed May 2011 PMID: 18977994 https://doi.org/10.1542/
peds.2008-2174
Prognosis American Academy of Pediatrics Task Force on Sudden Infant Death Syndrome.
Neurodevelopmental impairment is a concern for physicians The changing concept of sudden infant death syndrome: diagnostic coding shifts,
who care for preterm infants. Risk factors for developmental controversies regarding the sleeping environment, and new variables to con-
delay include postnatal steroid exposure, necrotizing enteroco- sider in reducing risk. Pediatrics. 2005;116(5):1245–1255. Reaffirmed May 2008
litis, BPD, small for gestational age status, and maternal preg- PMID: 16216901 https://doi.org/10.1542/peds.2005-1499

nancy-induced hypertension. Chorioamnionitis is a risk factor for Ballantyne M, Stevens B, Guttmann A, Willan AR, Rosenbaum P. Transition
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term. Extremely low birth weight infants and those born between
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20 and 25 weeks’ gestation are at significant risk for developmen-
Bonamy AK, Holmström G, Stephansson O, Ludvigsson JF, Cnattingius S. Preterm
tal issues. Surviving infants born at less than 26 weeks’ gestation
birth and later retinal detachment: a population-based cohort study of more than
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learning difficulties, including lower reading and mathematics Prevention of rotavirus gastroenteritis among infants and children: recommen-
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abnormally low range, and 30% have attention-deficit/hyperactivity Fierson WM; American Academy of Pediatrics Section on Ophthalmology;
disorder. Although individuals born preterm experience higher rates American Academy of Ophthalmology; American Association for Pediatric
of chronic medical and neurodevelopmental problems, their self- Ophthalmology and Strabismus; American Association of Certified Orthoptists.
perception of health and well-being in adolescence has been found to Screening examination of premature infants for retinopathy of prematurity.
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be the same as those of their normal birth weight peers. Additionally,
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increased systolic blood pressure, insulin resistance, and impaired
Hack M, Flannery DJ, Schluchter M, Cartar L, Borawski E, Klein N. Outcomes
glucose tolerance have also been reported in VLBW adults. Parents
in young adulthood for very-low-birth-weight infants. N Engl J Med.
should also understand that long-term follow-up information may
2002;346(3):149–157 PMID: 11796848 https://doi.org/10.1056/NEJMoa010856
not accurately reflect the outcome for their child because these ado-
Hovi P, Andersson S, Eriksson JG, et al. Glucose regulation in young adults with
lescents were treated before subsequent advances in prenatal and
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neonatal care. https://doi.org/10.1056/NEJMoa067187
Institute of Medicine Committee on Understanding Premature Birth and
Assuring Healthy Outcomes. Preterm Birth: Causes, Consequences, and
CASE RESOLUTION Prevention. Behrman RE, Butler AS, eds. Washington, DC: National Academies
The current feeding schedule for the infant should be continued because appro- Press; 2007 https://www.ncbi.nlm.nih.gov/books/NBK11362/
priate weight gain has occurred. Iron and multivitamin supplementation is recom- Johnson S, Wolke D, Hennessy E, Marlow N. Educational outcomes in extremely
mended until the infant is consuming 750 mL/day of formula. Discontinuation of preterm children: neuropsychological correlates and predictors of attainment.
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(40 weeks) and has been event-free. The first set of immunizations should be 87565641.2011.540541
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testing should be arranged to take place in 1 to 2 months, and the parents should
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LaHood A, Bryant CA. Outpatient care of the premature infant. Am Fam
Physician. 2007;76(8):1159–1164 PMID: 17990838
Lapillonne A, O’Connor DL, Wang D, Rigo J. Nutritional recommendations
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NEJMoa073059

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 CHAPTER 44

Care of Children With Special

Health Care Needs
Clare Kasper, MD

CASE STUDY
A 5-year-old girl with a physical disability is brought to tendon contracture and currently is ambulatory with the
your office for her first visit for a routine physical exam- use of ankle-foot orthoses. She has a neurogenic bladder
ination for school entrance. She was the result of a full- and requires intermittent catheterization. She also has
term pregnancy complicated by an elevated screening chronic constipation that is managed with a bowel regi-
a-fetoprotein and subsequent fetal ultrasonography men. Her cognitive function is age-appropriate.
that demonstrated a lumbar myelomeningocele and She will be entering a school program for the first
no hydrocephalus. Delivery was by elective cesarean time since moving to this community and has not estab-
section, with an Apgar score of 9 at both 1 minute and lished care with any specialists.
5 minutes, to a 25-year-old gravida 1, para 0–1 mother.
The mother used no illicit drugs, alcohol, or any other
Questions
1. Why is early identification and intervention impor-
medications during pregnancy but was not on vitamins
tant for newborns, infants, and children with special
or folate supplementation at the time of conception. At
health care needs?
delivery, a low lumbar spinal malformation was noted,
2. What role do primary care physicians play in the care
with no other malformations. The quadriceps muscles
of children with special health care needs?
were strong, but the feet demonstrated a rocker-bottom
3. What are the appropriate referrals and resources for
deformity.
families of children with special health care needs?
Shortly after birth, the myelomeningocele mal-
4. What specific psychosocial issues should be
formation was closed by neurosurgery. Later, the girl
addressed whenever children with special health
underwent orthopedic surgical release of Achilles
care needs visit their primary care physician?

Children and youth with special health care needs (SHCN) have Early identification of a health condition by a physician can result
physical, developmental, emotional, or behavioral conditions that in appropriate, definitive treatment of many diagnoses. In some
require special health-related services. These conditions must last instances, early intervention may even prevent secondary condi-
longer than 1 year and result in 1 of 5 consequences: the need for tions (eg, early management of hearing loss with hearing aids may
prescription medications; the need for increased medical care; minimize speech abnormalities). Even when such corrective treat-
compromised mental health or limited educational ability; the ment is not available, prompt identification improves children’s long-
need for special therapy; or the need for counseling. Children term outcome and allows families to obtain appropriate resources
with SHCN are defined by the International Classification of for their children. Through early intervention, newborns, infants,
Functioning, Disability and Health as disabled if they are limited and children with irreversible conditions can be introduced to med-
from doing what children of the same age can do. Most informa- ical, educational, and psychosocial services available in the com-
tion on children with SHCN combines the group with and the munity that serve to help these children maximize and reach their
group without disabilities. Conditions experienced by children full potential.
with SHCN can range from mild to severe, depending on the nature
and extent of them and their effect on daily living. Frequently, care
requirements of families and health professionals for children with Epidemiology
a diagnosis of SHCN are dramatically increased. For parents, the According to a national survey, an estimated 11.2 million chil-
diagnosis of a condition in their child can be initially overwhelm- dren in the United States have SHCN, affecting 23% of households
ing and disappointing. Support of the parents is essential as they with children across all racial, ethnic, and socioeconomic groups.
transition from disappointment to acceptance and assume the role Approximately 65% of children with SHCN reportedly have more
of facilitator of their child’s treatment plans. than 1 medical condition, including attention-deficit/hyperactivity
307

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 308 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

disorder, asthma, autism spectrum disorder (ASD), cerebral palsy, Pathophysiology

developmental delay, diabetes, and epilepsy. A single sensory dis-
Pathophysiology of a medical condition is completely dependent on
ability, such as deafness, affects approximately 3.5% of children;
the specific diagnosis. Conditions may be secondary to an embry-
blindness occurs in 1% of children. The prevalence of cerebral
ologic defect such as myelomeningocele, or to an infection such as
palsy is 1.5 to 2 per 1,000 live births. Advances in medical tech-
congenital cytomegalovirus (CMV), which interferes with cochlear
nology, as well as improved survival of low-birth-weight newborns
development and results in hearing loss. Etiologies are often multi-
and children with malformations, have increased the number of
factorial. For some conditions, such as ASD, the pathophysiology is
children living with such disabilities. Environmental exposures
unknown and may have several different etiologies.
have increased the incidence of chronic medical conditions, such
Special health care needs may be classified as acquired or con-
as asthma.
genital and static or progressive. Cerebral palsy, for example, is a
The presence of SHCN has a profound effect on the health and
group of nonprogressive neuromotor disorders resulting from a cen-
education of affected children. Studies show that children with
tral nervous system insult prenatally or within the first 2 years after
SHCN have 1.5 times more doctor visits and spend 3.5 more days
birth. It is characterized by abnormal motor movements and pos-
in the hospital than children without these conditions. Children
turing and may be accompanied by other problems as well. Causes
with SHCN miss twice the number of school days and are twice
of cerebral palsy include preterm birth, low birth weight, asphyxia,
as likely to repeat a grade compared with children without medi-
prenatal abnormalities (eg, placental insufficiency), congenital infec-
cal conditions.
tions (eg, toxoplasmosis, CMV), and biochemical abnormalities (eg,
In addition, numerous associated conditions occur more com-
severe hyperbilirubinemia). Other causes are environmental (eg, in
monly in children with SHCN. These include intellectual devel-
utero exposure to alcohol) and genetic (eg, inborn errors of metab-
opmental disorders, growth failure, and nutritional problems.
olism). Severe postnatal injuries or infections also may result in
Problems with dentition, respiratory infections, and bowel and blad-
cerebral palsy (eg, abusive head trauma, meningitis). However, an
der continence also may occur. Significant emotional disturbances
estimated 25% to 50% of cases of cerebral palsy have no discern-
may occur as children adapt to their condition.
ible cause.
Clinical Presentation
Diagnosis
Children with SHCN can present in a variety of ways depending on
The term SHCN is used in a broad sense to include conditions that
their diagnosis. Many physical disabilities may be readily appar-
require additional medical care and supervision, as defined by the
ent at birth on the newborn examination or newborn screening.
US Department of Health and Human Services Maternal and Child
Some diagnoses, such as cerebral palsy, may be detected later, as
Health Bureau. As previously noted, children with SHCN may have
the motor impairment becomes more evident. Following the child’s
significant physical, sensory, or developmental disabilities that may
development at routine health maintenance visits is essential for
result from preterm birth, congenital infections (eg, CMV), or expo-
early detection of developmental delay. Implementing the Bright
sure in utero to alcohol or illicit substances. Some children are born
Futures program from the American Academy of Pediatrics (AAP)
with congenital malformations or inborn errors of metabolism that
can help in early detection of many conditions. Children with SHCN
mandate special diets and occupational or physical therapy. Other
also may present with chronic illness (eg, asthma, diabetes) or with
children with SHCN include those with chronic medical conditions,
specific complaints (eg, poor vision or hearing). Alternatively, they
such as asthma, obesity, and sickle cell anemia. All these medical
may present with a more general concern, such as growth failure
conditions alter lifestyle, require increased medical care (includ-
(Box 44.1). Behavioral problems or difficulties in school may pre-
ing subspecialty care), increased medication usage, and increased
cipitate the initial visit.
use of community services compared with the general pediatric
population.

Box 44.1. Diagnosis of Children With Physical

and Sensory Disabilitiesa Evaluation
History
ww Growth failure
ww Microcephaly When initially evaluating children with newly diagnosed SHCN,
ww Abnormal neurologic examination, including hypertonicity, spastic health professionals should first determine any specific paren-
diplegia or quadriplegia, and brisk deep tendon reflexes tal concerns. A complete medical history should be obtained,
ww Developmental delay including information about the pregnancy and birth. The his-
ww Speech or hearing deficit tory also should include any possible exposures as well as signif-
ww Visual deficit icant infections. General screening questions about development
ww Physical malformations are important to ask to assess the child’s developmental progress
(Box 44.2). Specific questioning is warranted if parents are con-
a
May not be present in all children. cerned about delayed development or if any of their responses

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 Chapter 44: Care of Children With Special Health Care Needs 309

hearing loss, the evaluation of middle ear effusion or infection in

Box 44.2. What to Ask
the unaffected ear should be prioritized. If a child has a tracheos-
Children With Special Health Care Needs tomy or gastrostomy tube, inspection of the site is essential at each
ww Were there any perinatal complications, such as premature rupture of visit to look for erosions or skin infections.
membranes or fetal distress? Overall, for most children with SHCN, the neurologic examina-
ww Was the child born prematurely? If so, how long did the child remain in tion is extremely important (Box 44.3). An age-appropriate devel-
the hospital and for what reasons? opmental assessment is also an essential part of the examination at
ww Was the child exposed to any toxins (eg, alcohol, illicit drugs) in utero? all health maintenance visits.
ww Is there any history of infection during the perinatal period or infancy?
ww What developmental milestones has the child mastered? Laboratory Tests
ww Is the child attending school or some type of early intervention program? Laboratory evaluation of newborns, infants, and children with phys-
ww How does the child get there? ical or sensory disabilities depends on their specific conditions. Not
ww What does the child do on returning from school? all patients need a costly array of diagnostic procedures. A chro-
ww Who feeds and bathes the child? mosomal karyotype or comparative genomic hybridization using
ww Can the older child use the toilet without assistance? peripheral blood is helpful in children with suspected genetic dis-
ww How is the child sleeping? Does the child take naps at school and home? orders (eg, abnormal facies, a major anomaly, developmental delay).
ww Has respite care been arranged for the family? Routine testing for fragile X syndrome should be strongly consid-
ww Does the caregiver seem overwhelmed or excessively tired, especially ered in all boys with intellectual and developmental disabilities and
one who is caring for a child with multiple disabilities? in girls with a family history of it. Other testing should be specific
ww Do other family members help care for the child? for the suspected diagnosis (see Chapter 84).
ww Is extended family available to help with siblings? Metabolic screening for inborn errors of metabolism should be
ww Are there any other people with disabilities in the family? performed on children with intellectual and developmental disabil-
ww Does the family receive any financial assistance for care of the child? ities and any of the following symptoms: intermittent vomiting or
lethargy, loss of developmental milestones, or seizures. Such screen-
ing is not needed in the routine evaluation of children with devel-
indicate that their child is not attaining age-appropriate devel- opmental delay and no other symptoms.
opmental milestones. A screening test for visual acuity (Snellen eye chart) and hearing
In cases of children with known sensory or physical disabilities, (ie, audiogram) should be performed in all children with suspected
families should be asked directly at each visit about daily activities and sensory deficits, even mild ones. For infants and toddlers, a brain
the child’s ability for self-help skills. Because many children with dis- stem auditory evoked response or behavioral audiogram is a more
abilities are also on daily medication for seizures or other chronic condi- appropriate screening test for hearing (see Chapter 88). A visual
tions, it is important to ask about the presence of any drug side effects. evoked response can be performed to test vision.
Any behavioral or emotional problems in the child should be Psychometric testing may be helpful in certain school-age chil-
identified. Additionally, an overall assessment of family dynamics dren to assess intellectual function. Electroencephalography is indi-
should be made. It is important to inquire about the relationships cated in all patients with a history of seizures or seizure-like episodes.
between children with SHCN and their siblings, as well as the effect
these children have on the parents’ marriage or relationship. Imaging Studies
Brain imaging studies, such as magnetic resonance imaging of the
Physical Examination head, can be informative in the setting of suspected intrauterine
In general, a complete physical examination, including an oral health infection, intraventricular hemorrhage, or genetic disorders with
assessment and a detailed neurologic assessment and neuromotor
examination, should be performed at each visit. Height, weight, and
head circumference should be plotted on the growth chart and com- Box 44.3. What to Ask
pared with previous measurements. Failure of adequate growth as Neurologic Examination of the Child With Special Health Care Needs
measured by any of these parameters should be examined closely. ww Are normal primitive reflexes (eg, Moro, rooting) present in neonates?
For example, microcephaly, nutritional problems, and growth fail- ww Do newborns and infants appear to visualize and track objects appropriately?
ure are not uncommon in children with cerebral palsy. Depending ww Are there any abnormal movements of the trunk or extremities at rest?
on the specific diagnosis, the examination should focus on physi- ww Is muscle tone normal?
cal findings associated with the particular condition. For children ww Is any hypertonicity or hypotonicity evident?
with physical disabilities such as congenital or acquired amputa- ww Is any asymmetry of the upper and lower extremities apparent?
tions, for instance, assessment of the skin that comes in contact with ww Are deep tendon reflexes normal and symmetrical?
prosthetic devices is a pertinent aspect of the physical examination. ww Have all primitive reflexes been extinguished in older children?
Pressure sores may be found in nonambulatory children with cere- ww Is the gait appropriate for age?
bral palsy. In children with sensory disabilities, such as unilateral

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 310 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

associated developmental delay and even isolated global develop- organized treatment plan and entry into an early intervention pro-
mental delay. Electromyography can be used to differentiate cere- gram to some children with disabilities. To qualify, children must be
bral palsy from a congenital myopathy. diagnosed with an eligible condition, such as cerebral palsy, epilepsy,
ASD, or global developmental delay. In addition, newborns and
Management infants considered to be at risk for developing disabilities qualify
Caring for children with SHCN can be rewarding yet challenging for for assistance (eg, preterm newborns with bronchopulmonary dys-
primary care pediatricians. The pediatrician should be cognizant plasia and intraventricular hemorrhage). For children who do not
that these patients will take increased practice time and paperwork qualify, similar services can be coordinated on an individual basis
to ensure that they receive all services necessary. Case management by the physician’s office or the school district.
services are an essential aid to their management. The primary care Children with severe physical and sensory disabilities often are
pediatrician must supervise acute and chronic medical care, provide cared for by many medical subspecialists in addition to the primary
anticipatory guidance, monitor growth and development, coordi- care pediatrician. Referrals to pediatric orthopedic surgeons, plastic
nate subspecialty involvement, make educational referrals, and offer surgeons, geneticists, ophthalmologists, otolaryngologists, child neu-
community services. Sometimes a subspecialty physician will pro- rologists, and psychologists may be necessary. Special comprehensive
vide comprehensive care if the diagnosis (eg, cystic fibrosis) encom- clinics, such as those for craniofacial or spina bifida, are established
passes most of the child’s health care needs. Although care should be in some children’s referral centers to facilitate care of patients, with
individualized to each patient, general guidelines have been devel- multiple subspecialists forming a multidisciplinary team in the same
oped for the provision of pediatric services to newborns, infants, clinical setting. In addition, speech and language, occupational, and
and children with SHCN. The guidelines include recommendations physical therapists are often an integral part of the medical team.
for establishing a medical home with the primary care pediatrician, Initial and ongoing therapeutic services provided by each of these
medical services, suggestions for parental involvement, assistance individuals must be monitored periodically to assess the progress and
from community agencies, and fulfillment of specific federal require- overall effectiveness of the treatment plan. Ideally, services should be
ments for educational opportunities for children with disabilities. coordinated so that children and parents miss a minimum number
Pediatricians who care for children with SHCN should be familiar of school days and workdays (eg, Saturday and after-school appoint-
with the principles of care published by the AAP and should incor- ments, visits to several practitioners on the same day). All informa-
porate these principles into the overall treatment plan. Pediatricians tion from diagnostic studies and initial evaluations should be shared
also should be knowledgeable about the rights of such individuals among each of the health professionals. The AAP has produced a
as established by various legislation, including the Americans with Building Your Medical Home toolkit (https://medicalhomes.aap.org),
Disabilities Act and the Individuals with Disabilities Education Act which can be very helpful to the primary care pediatrician.
Part C. Coordination of intervention services and the use of adaptive The primary care physician also should offer counseling to par-
or assistive technology are essential components of management. ents on ethical issues pertaining to their child’s condition. These
issues include palliative care decisions, decision making in critical
General Considerations care situations, limiting nonbeneficial interventions at the end of
The major role of primary care pediatricians who care for chil- life, and advance directives. Because of the pediatrician’s relationship
dren with SHCN is 4-fold: provide primary medical care; serve as with the family, that physician may be in the best position to engage
the patient and family advocate in evaluating therapeutic options; parents in conversations concerning these topics. Coordinated con-
inform families of available community resources; and most impor- ferences with the primary care physician, specialists, social workers,
tant, serve as a proactive coordinator of care. The first task of the and the family may be effective in counseling.
pediatrician is to establish the diagnosis and recognize any comor- The family has a vital role in caring for the child with SHCN, and that
bidities. Whether the diagnosis is an obvious physical malforma- care can frequently be stressful and emotionally draining. Family sup-
tion or one that is not readily apparent, physicians are placed in the port and counseling should be readily available. The sociocultural con-
challenging position of breaking the news to families. Parents should text of the family and needs of any other children in the family should
be informed of the diagnosis as soon as possible, but care should be be considered when the care of the child with SHCN is addressed.
taken to refrain from discussing the prognosis, especially if it is still With improved medical care and services, 90% of children with
unknown. The cause of any disability and the possible complications SHCN reach adulthood. Transition into adult services can be chal-
of the condition should be reviewed with parents. The primary goal lenging. Preparation for this transition should begin as early as
is to help children with disabilities reach their full potential. 11 years of age to ensure success. The goal should be independence
Health professionals are in a unique position to establish a treat- to the degree that is possible. The individual should be prepared for a
ment plan with families that includes medical, psychosocial, and work environment, if possible, and financial independence. The fam-
educational services. A multidisciplinary team should be assem- ily and child should be evaluated to determine whether the child is
bled that includes the pediatrician, a member of the school system, capable of independent living or will need support in a group home
a social worker, and a representative of an early intervention pro- or an institution or to remain with the family. If necessary, guard-
gram. Federally funded, nonprofit, regional centers can provide an ianship issues must be addressed. A successful transition to adult

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 Chapter 44: Care of Children With Special Health Care Needs 311

health professionals must be ensured, and insurance coverage must Maintaining good oral hygiene is another challenge, because
be arranged. The Patient Protection and Affordable Care Act of 2010 some children with disabilities do not clear oral secretions well and
has greatly improved insurance coverage for these patients, includ- retain food in their mouths, predisposing to cavity development. In
ing extending the age of coverage for dependent children until age addition, many children are treated with anticonvulsant agents and
26 years, preventing exclusion from coverage based on preexisting antibiotics that can cause gingival and enamel dysplasia. Abnormal
conditions, and prohibiting lifetime caps on medical expenses. oromotor coordination, tone, and posturing also contribute to the
development of oropharyngeal deformities, such as high-arched pal-
Psychological Concerns ate and overcrowded teeth. As with other children, fluoride supple-
Children with SHCN have more mental health issues than their mentation and consistent preventive dental care are recommended.
peers. These conditions include behavioral problems, depression, Bowel and bladder continence is important to attain for sev-
anxiety, low self-esteem, peer relationship problems, school perfor- eral reasons. It allows children to function in a socially acceptable
mance problems, and absenteeism. Children with SHCN that include fashion, provides independence, and prevents the development of
a disability have even higher rates of psychological comorbidity. It complications such as recurrent urinary tract infections, diaper der-
is important that primary care physicians screen for these disorders matitis, and decubitus pressure sores. Behavior modification tech-
and refer patients for counseling when appropriate. niques coupled with positive reinforcement are associated with the
complete or partial success of bowel training.
Economic Concerns
The cost of care for children with SHCN is a significant financial Community Resources
burden for as many as 40% of these families in the United States. Optimal care for children with SHCN depends on maximum use of
Families with an affected child are more likely to have a single community agencies and resources. An assessment of parental and
income, have a single parent, live in poverty, and have poor-quality patient needs should first be performed and prioritized. The appro-
housing. Economic evaluation should be performed by caseworkers priate resources should then be identified for individual children.
to determine the eligibility of children with SHCN for financial sup- Early intervention services should be used. Primary care physicians
port through Supplemental Security Income and medical insurance may need to help determine the appropriateness of specific ser-
under Medicaid. Caseworkers can help families apply for the appro- vices for patients and families. Emphasis should be placed on inte-
priate assistance or to other programs, such as the Supplemental grating each child into support services. Parent-to-parent support
Nutrition Assistance Program and the Low Income Home Energy and sibling support groups can be quite helpful in relieving stress,
Assistance Program, which can help extend a family’s resources. helping parents understand the diagnosis, and avoiding feelings of
It is apparent that children with SHCN from low-income fami- isolation. Support groups for siblings as well as parents are avail-
lies have reduced access to health, educational, and social services. able. Respite care to give parents a break from caregiving and in-
Recent studies have been undertaken to determine the best strategy home health service programs also should be investigated. In-home
to improve access to services and availability of services for under- hospice care is available for children near the end of life; it can be an
served communities. Early intervention services can reduce the eco- excellent support for the patient and family. Physicians should act
nomic burden of these patients and their families. as liaisons between all agencies. Case conferences are occasionally
necessary to review the progress of individual children with each
Specific Medical Conditions member of the health care team.
Several medical conditions commonly occur in children with mod-
erate to severe disabilities. While providing children with compre- Education
hensive well-child care, general pediatricians also can address and By federal law, every child with a disability is entitled to an educa-
manage these conditions. tion. Every effort should be made to enroll children with disabilities
Problems with adequate nutrition, which usually result from in conventional schools and provide opportunities for socialization
insufficient caloric intake, manifest in the form of growth failure. at an early age. Structured independence and mainstreaming
Depending on the degree of disability and extent of oropharyngeal children with disabilities in classes with children without disabilities
dysfunction, the placement of a nasogastric or gastrostomy tube may can be quite productive for all the children involved regardless of
be necessary. Caloric needs may be 10% to 50% higher than that of disability status.
children without SHCN. Mainstreaming may not be available in some areas, and the sever-
Respiratory illness is not uncommon among children with SHCN. ity of some disabilities may preclude attendance at a regular school
Close observation and conservative management of viral illnesses are campus. Several other educational possibilities can be considered,
often necessary. Aspiration pneumonia is likely to occur, especially and each case should be evaluated on an individual basis. Options
in children with severe developmental delay resulting from poor include special education classes in designated schools (full- or
handling of oral secretions or severe oral dyspraxia. To help mini- part-time attendance), special education classes in regular schools
mize respiratory infections, these children always should be admin- (full- or part-time attendance), a mix of part-time special educa-
istered influenza vaccine during the winter months. tion classes and part-time regular classes, or homeschooling. The

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 312 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

decision can be facilitated through the development of an Individualized American Academy of Pediatrics Council on Children With Disabilities.
Education Program by a multidisciplinary team at the school. Supplemental Security Income (SSI) for children and youth with disabilities.
Parents and primary care physicians are encouraged to participate Pediatrics. 2009;124(6):1702–1708. Reaffirmed February 2015 PMID: 19948637
https://doi.org/10.1542/peds.2009-2557
in this evaluation (see Chapter 36).
Anderson D, Dumont S, Jacobs P, Azzaria L. The personal costs of caring for a
child with a disability: a review of the literature. Public Health Rep. 2007;122(1):
Prognosis 3–16 PMID: 17236603 https://doi.org/10.1177/003335490712200102
The prognosis for children with SHCN is dependent on the diagno- Burdo-Hartman WA, Patel DR. Medical home and transition planning for
sis, the severity and extent of any disability, medical and supportive children and youth with special health care needs. Pediatr Clin North Am.
intervention, and the child’s environment. It may be impossible to 2008;55(6):1287–1297, vii–viii PMID: 19041458 https://doi.org/10.1016/
establish a prognosis at the time of diagnosis. Children may adapt j.pcl.2008.09.004
differently to the same diagnosis. It is important to establish real- Houtrow AJ, Okumura MJ, Hilton JF, Rehm RS. Profiling health and health-
istic goals to determine the best intervention. With comprehensive related services for children with special health care needs with and without
care, many affected children can lead productive, independent lives, disabilities. Acad Pediatr. 2011;11(6):508–516 PMID: 21962936 https://doi.
org/10.1016/j.acap.2011.08.004
and maximal potential can be realized in all cases.
Kogan MD, Strickland BB, Newacheck PW. Building systems of care: findings
from the National Survey of Children With Special Health Care Needs. Pediatrics.
2009;124(suppl 4):S333–S336 PMID: 19948596 https://doi.org/10.1542/
CASE RESOLUTION peds.2009-1255B
Although the girl’s medical condition of low lumbar spina bifida seems sta- Kuo DZ, Houtrow AJ; American Academy of Pediatrics Council on Children
ble, the physician should inquire about any ongoing problems or concerns. A With Disabilities. Recognition and management of medical complexity.
complete examination should be done. Routine screening laboratory tests and Pediatrics. 2016;138(6):e20163021 PMID: 27940731 https://doi.org/10.1542/
immunizations required for school entry should be performed. The family should peds.2016-3021
be evaluated for financial stability and support services. The mother should be
Kuo DZ, Turchi RM. Best practices: kids with special healthcare needs.
placed on folate supplementation for prevention of recurrence in future children.
Contemporary Pediatrics. 2010;27:36–40
The patient should be referred to the local spina bifida clinic for comprehensive
specialist care by orthopedists, urologists, and physical therapists. Integration Lipkin PH, Okamoto J; American Academy of Pediatrics Council on Children
into the regular classroom should be recommended. The school should be con- With Disabilities; American Academy of Pediatrics Council on School Health.
tacted to arrange for adaptive physical education and for intermittent cathe- The Individuals with Disabilities Education Act (IDEA) for children with spe-
terization by school nursing personnel. A follow-up visit is scheduled with the cial educational needs. Pediatrics. 2015;136(6):e1650–e1662 PMID: 26620061
primary care physician in 2 months to review the child’s integration into services. https://doi.org/10.1542/peds.2015-3409
Norwood KW Jr, Slayton RL; American Academy of Pediatrics Council on
Children With Disabilities; American Academy of Pediatrics Section on Oral
Health. Oral health care for children with developmental disabilities. Pediatrics.
Selected References 2013;131(3):614–619 PMID: 23439896 https://doi.org/10.1542/peds.2012-3650

Adams RC, Levy SE; American Academy of Pediatrics Council on Children With Okun A. Children who have special health-care needs: ethical issues. Pediatr Rev.
Disabilities. Shared decision-making and children with disabilities: pathways 2010;31(12):514–517 PMID: 21123514 https://doi.org/10.1542/pir.31-12-514
to consensus. Pediatrics. 2017;139(6):e20170956 PMID: 28562298 https://doi. Perrin JM, Bloom SR, Gortmaker SL. The increase of childhood chronic con-
org/10.1542/peds.2017-0956 ditions in the United States. JAMA. 2007;297(24):2755–2759 PMID: 17595277
Adams RC, Tapia C; American Academy of Pediatrics Council on Children With https://doi.org/10.1001/jama.297.24.2755
Disabilities. Early intervention, IDEA Part C services, and the medical home: collab- Perrin JM, Gnanasekaran S, Delahaye J. Psychological aspects of chronic health
oration for best practice and best outcomes. Pediatrics. 2013;132(4):e1073–e1088. conditions. Pediatr Rev. 2012;33(3):99–109 PMID: 22383512 https://doi.
Reaffirmed May 2017 PMID: 24082001 https://doi.org/10.1542/peds.2013-2305 org/10.1542/pir.33-3-99
American Academy of Pediatrics; American Academy of Family Physicians; Strickland BB, van Dyck PC, Kogan MD, et al. Assessing and ensuring a com-
American College of Physicians-American Society of Internal Medicine. A con- prehensive system of services for children with special health care needs: a pub-
sensus statement on health care transitions for young adults with special health lic health approach. Am J Public Health. 2011;101(2):224–231 PMID: 21228285
care needs. Pediatrics. 2002;110(suppl 3):1304–1306 PMID: 12456949 https://doi.org/10.2105/AJPH.2009.177915
American Academy of Pediatrics Council on Children With Disabilities. Care US Department of Health and Human Services, Health Resources and Services
coordination in the medical home: integrating health and related systems of care Administration, Maternal and Child Health Bureau. The National Survey of
for children with special health care needs. Pediatrics. 2005;116(5):1238–1244 Children With Special Health Care Needs Chartbook 2009–2010. Rockville, MD:
PMID: 16264016 https://doi.org/10.1542/peds.2005-2070 US Department of Health and Human Services; 2013

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 CHAPTER 45

Injury Prevention
Sarah J. Atunah-Jay, MD, MPH, FAAP, and Iris Wagman Borowsky, MD, PhD, FAAP

CASE STUDY
A 16-year-old girl was brought to the emergency depart- 3. What is TIPP and how should it be used when coun-
ment after being rescued from her submerged vehicle. The seling families?
girl was texting a friend while driving and crashed into a 4. What are some general guidelines for effective
pond. After several weeks in the intensive care unit, she injury prevention counseling?
was transferred out for rehabilitative care from her injury. 5. How does a child’s age affect the advice offered to
a family?
Questions
1. How pervasive are childhood injuries?
2. What are different approaches to injury prevention?
How could this particular injury have been prevented?

Traditionally, unintentional injuries have been called “accidents.” The than 21 years in the United States. The most common causes of
problem with this term is that it implies unpredictability, carrying with nonfatal injuries in children are falls, followed by injuries from
it connotations of chance, fate, and unexpectedness. The perception being struck by or against something, overexertion, motor vehicle
that injuries are chance occurrences that cannot be predicted or pre- occupancy, cuttings/piercings, and bites/stings.
vented has been a major barrier to progress in injury prevention and Several epidemiological factors are associated with higher
the study and control of injury as a scientific discipline. According to rates of pediatric injuries, including sex, race, income status, and
the modern view of injury, accidents must be anticipated to be pre- family stressors (eg, death in the family, new residence, birth of a
vented. Specialists in injury prevention have tried to replace the word sibling). There is a bimodal age distribution of injuries, with new-
accident with injury and have developed the idea of reducing injury borns/infants and adolescents at greatest risk. Males are more
risk. Thus, injuries are not random events at all; they occur in pre- likely than females to die from injuries and slightly more likely to
dictable patterns determined by identifiable risk factors. For exam- experience injuries. Females are more likely than males to expe-
ple, if a 16-year-old is texting while driving, which has been shown to rience sexual assault. American Indian/Alaska Native and black
be a dangerous driving distraction, the resulting injury can hardly be children have higher rates of total injury-related deaths than other
called an accident. On the contrary, the injury is entirely predictable. racial and ethnic age-matched populations. Geography influ-
ences injury rates; drowning deaths tend to be higher in coastline
Epidemiology states (ie, Alaska and California) or states with a higher num-
Unintentional injuries are the leading cause of death among peo- ber of swimming pools (eg, Texas), and injury-related deaths are
ple aged 1 to 44 years in the United States. In 2016, unintentional higher in rural areas and may be related to decreased access to
injuries claimed the lives of more than 18,000 Americans 21 years emergency medical care.
and younger. Suffocation is the third leading cause of death among
newborns and infants; drowning is the leading cause of death among Strategies for Injury Prevention
1- to 4-year-olds; and motor vehicle crashes are the leading cause of Efforts to prevent injuries have shifted from changing the behav-
death among 5- to 21-year-olds. Fires and burns are another major ior of individuals to modifying the environments in which injuries
cause of unintentional injury-related death in young people. occur. William Haddon, MD, a medical epidemiologist, devised 2
Intentional or violence-related injuries are also a major cause of useful frameworks for developing injury prevention strategies: the
mortality in young people. Suicide is the second highest cause of Haddon matrix and a list of 10 countermeasures to prevent injuries
death among 10- to 21-year-olds. Homicides are the third highest or reduce the severity of their effects.
cause of death among 15- to 21-year-olds and fourth highest cause
of death among 1- to 14-year-olds. Most suicides and homicides Haddon Matrix
are firearm related. This matrix relates 3 factors (host, vector, and environment) to the 3
In addition to deaths, in 2016, nonfatal injuries led to almost phases of an injury-producing event (pre-event, event, and post-event).
9 million hospital emergency department visits in people younger The 3 factors interact over time to produce injury. Table 45.1 shows
313

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 314 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Table 45.1. The Haddon Matrix Applied to Motor 7. Modify relevant basic qualities of the hazard (eg, place padded
Vehicle Crash Injuries carpets under cribs; require guns to have safety locks; develop
inter-vehicle communication systems).
Phase Host (Human) Vector (Vehicle) Environment
8. Increase resistance to damage from the hazard (eg, train and
Precrash Driver vision Brakes Speed limit condition athletes; make structures more earthquake-proof;
Driver impairment Tires Road curvature use flame-retardant sleepwear).
(eg, alcohol, drugs) Speed Road signs 9. Limit the damage that has already begun (eg, use fire extin-
Distracted driving guisher; begin cardiopulmonary resuscitation).
Crash avoidance
(eg, telephone, 10. Stabilize, repair, and rehabilitate injured individuals (eg,
equipment and
text) develop pediatric trauma centers and physical rehabilitation
technology
programs; improve emergency medical services).
Crash Use of safety belts Vehicle size Median barriers
Haddon’s work serves as a practical guide for thinking about
Use of age- Airbags Laws about use ways to prevent injury. It emphasizes the importance of consider-
appropriate car of car safety seats ing injuries as a result of a sequence of events, with many oppor-
safety seats and safety belts tunities for prevention. The shift of emphasis away from changing
Postcrash Age Fuel system EMS personnel human behavior to preventing injury is particularly appropriate for
Physical condition integrity training injuries in children because inhibiting children’s curiosity is imprac-
tical as well as undesirable.
Abbreviation: EMS, emergency medical services.
Adapted from the National Committee for Injury Prevention and Control. Injury prevention:
meeting the challenge. Am J Prev Med. 1989;5(3 suppl):1–303.
Passive and Active Interventions
Interventions to prevent injuries can also be categorized as passive
a Haddon Matrix of motor vehicle crash injuries. The precrash phase
or active. Passive or automatic strategies protect whenever they are
describes elements that determine whether a crash will occur; the crash
needed, without the action of parents or children. An example is the
phase describes the variables that influence the nature and severity
automobile airbag that automatically inflates to cushion occupants
of the resultant injury; and the postcrash phase describes the factors
during a crash. Other examples of automatic strategies are water
that determine the degree to which the injury is limited and repaired
heater temperatures set to 48.9°C (120°F) or lower, not having guns
after the crash occurs. By describing the “anatomy” of an injury, the
in the home, and the use of energy-absorbing surfaces under play-
Haddon Matrix illustrates the numerous characteristics that determine
ground equipment. In contrast, active interventions require action
an injury and the many corresponding strategies for interfering with
to become effective, such as in the case of nonautomatic safety belts,
the production of an injury.
which require individuals to “buckle up” every time they enter an
Haddon’s list of 10 countermeasures to prevent injuries or reduce
automobile. Supervision of swimming children is another example
the severity of their effects are as follows:
of an active injury prevention strategy.
1. Prevent creation of the hazard (eg, stop producing poisons, toys Some strategies are partially automatic, requiring some action
with small parts, and non-powder firearms; do not participate in by individuals. Smoke detectors can be very effective in preventing
dangerous sports; support community centers that engage chil- injury and death in house fires, but roughly one-third of smoke detec-
dren in safe after-school activities). tors do not have working batteries. Batteries should be changed once
2. Reduce the amount of the hazard (eg, package drugs in nonle- a year and ideally tested once a month. As might be expected, the
thal amounts; reduce speed limits). greater the effort required for children to be protected, the smaller the
3. Prevent the release of the hazard (eg, use child-resistant caps chance that protection occurs. Therefore, whenever possible, passive
for medications, toilet locks, and safety latches on cabinets and measures are preferable because they are the most effective.
drawers; pass and enforce distracted driving laws; implement Several approaches have been used successfully to prevent child-
restrictions on handgun purchases; counsel families who keep hood injuries, including engineering, education, legislation, and
guns to store them unloaded in a locked case, with the ammu- enforcement. An engineering intervention, the car safety seat, is
nition locked separately). extremely effective (Table 45.2). When used correctly, child safety
4. Modify the rate or spatial distribution of release of the hazard seats in passenger cars reduce the risk of death by 71% for infants
(eg, require airbags in cars; use child safety seats and safety belts; and 54% for toddlers aged 1 to 4 years. Booster seats reduce injury
make poisons taste bad). risk by 59% for children aged 4 to 7 years compared with safety belts
5. Separate people from the hazard in space or time (eg, make side- alone. Unfortunately, studies indicate that between one-third and
walks for pedestrians, bikeways for bicyclists, and recreation two-thirds of car safety seats are used incorrectly. To address this,
areas separated from vehicles). newborn care units often have car safety seat education programs,
6. Separate people from hazards with material barriers (eg, use and some require possession of an infant car safety seat prior to
bicycle helmets and protective equipment for athletes; install hospital discharge. Police departments and private motor compa-
fences around swimming pools; build window guards). nies hold free public events to teach and manually check appropriate

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 Chapter 45: Injury Prevention 315

Table 45.2. Pediatric Car Safety TIPP—The Injury Prevention Program was developed in 1983 by
Seat Guidelinesa the American Academy of Pediatrics (AAP) to firmly establish injury
prevention as a cost-effective standard of care for pediatricians.
Type of Car
The AAP suggests that health professionals focus their safety coun-
Age Group Safety Seat General Guidelines
seling on a few topics targeted to individual risk factors (eg, age,
Term newborns/ Rear facing Rear facing as long as possible, until sex, location, season of the year, socioeconomic status of family).
infants they reach the highest weight or Table 45.3 shows the age-specific counseling schedule of TIPP, which
height allowed by their seat. indicates the minimum topics to cover at each visit. Specific pre-
Toddlers/ Rear facing and Rear facing as long as possible. ventive measures should be reinforced at each visit. Areas of injury
preschoolers forward facing All children who have outgrown prevention guidance recommended for adolescents include traffic
their rear-facing seat should use a
forward-facing seat with a harness
until they reach the highest weight
or height allowed by their seat. Table 45.3. TIPP—The Injury Prevention Program
School-age Belt-positioning When weight exceeds limit for car Safety Counseling Schedule for Early
children booster seat safety seat. Use until adult safety and Middle Childhood
belt fits correctly (usually at 4’ 9” Visit Introduce Reinforce
and between 8 and 12 years of age). Birth–6 months Rear-facing car safety Safe sleep
Older children Safety belts When old enough and large enough seat, fall risks, burn
to use the vehicle safety belt alone. prevention, smoke
a
All children younger than 13 years should be restrained in the rear seats of vehicles. alarm use, choking/
Adapted from Durbin DR, Hoffman BD; American Academy of Pediatrics Council on Injury, Violence, suffocation prevention
and Poison Prevention. Child passenger safety. Pediatrics. 2018;142(5):e20182460.
6–12 months Drowning prevention, Safe sleep, rear-facing car
poisoning risks, safety seats, fall risks, burn
strangulation hazards prevention, smoke alarm
car safety seat use. In addition to engineering and education, pas- use, choking/suffocation
sage and strict enforcement of child restraint laws are essential to prevention
compliance. All 50 states and the District of Columbia have child
1–2 years Firearm hazards Poisoning risks, fall risks, burn
restraint laws (www.iihs.org/topics/seat-belts#laws). Nevertheless,
prevention, smoke alarm use,
loopholes still exist, such as exemptions in some states for safety
drowning prevention, rear-
belt use if older children are riding in rear seats; for safety belt use
facing car safety seats
in school buses, taxis, and police vehicles; and if all safety belts are
already in use. Such exemptions reinforce parental misconceptions, 2–4 years Play equipment safety Fall risks, firearm hazards,
particularly that the lap of an occupant (ie, the “child crusher” posi- burn prevention, smoke
tion) is a safe position. alarm use, poisoning risks,
car safety seats
Counseling by Pediatricians 5 years Bike safety, street Firearm hazards, car safety
safety, water safety, seat or belt-positioning
Although the existence of significant gaps in parental knowledge
fire safety booster seat and safety
about injury prevention has been clearly established, studies have
belt use
shown that pediatricians spend surprisingly little time counseling
parents about childhood safety. One survey found that only 42% 6 years Safe swimming Fire safety, firearm hazards,
of caregivers of children younger than 15 years who had a medical bike safety, street safety,
visit in the past year recalled receiving injury prevention informa- water safety, car safety seat or
tion. Another survey found only 15% of patients presenting with belt-positioning booster seat
an unintentional injury reported receiving injury prevention coun- and safety belt use
seling. Reasons for limited discussion of safety issues may include 8 years Sports safety Water safety, bike safety,
lack of emphasis on preventive medical care in medical schools and firearm hazards
pediatric training programs, inadequate time or payment, and lack 10 years “Rules of the road” Firearm hazards, sports safety,
of perceived self-efficacy or effectiveness. Research, however, has while biking water safety, safety belt use,
shown that injury prevention counseling in primary care settings bike safety
is effective, resulting in increased knowledge and improved safety Adapted from the American Academy of Pediatrics Council on Injury, Violence, and Poison
practices. Parents report that they would listen to physicians much Prevention. TIPP—The Injury Prevention Program: A Guide to Safety Counseling in Office Practice.
more than any other group about child safety. Itasca, IL: American Academy of Pediatrics; 2019

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 316 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

safety (eg, safety belts, alcohol use, motorcycle and bicycle helmets), ww Research suggests both benefits and risks of media use for the
water safety (eg, alcohol use, diving injuries), firearm safety, sports health of children and teenagers. Parents and pediatricians
safety, and distracted driving. can work together to develop a Family Media Use Plan (www.
Connected Kids: Safe, Strong, Secure is a violence prevention tool healthychildren.org/MediaUsePlan) that considers children’s
introduced by the AAP in 2006 to augment TIPP. Acknowledging developmental stages to individualize an appropriate balance for
that injury and violence prevention are intertwined, it uses an media time and consistent rules about media use (2016).
asset-based approach to engage parents in understanding and ww Pedestrian injuries are a significant traffic-related cause of mor-
fostering healthy child development. An emphasis is put on sup- bidity and mortality. Emphasis should be given to community-
port and open communication to promote emotional and phys- and school-based strategies to reduce exposure to high-speed
ical safety. and high-volume traffic, and to promote improvements in vehi-
Health professionals should involve parents and patients in cle design, driver manuals, driver education, and data collec-
educational efforts (eg, have a bicycle helmet in the office for tion to reduce pediatric pedestrian injury (Reaffirmed 2019).
children to try on). Safety counseling is most effective if limited ww The absence of guns from homes and communities is the most
to 2 or 3 topics per visit. Advice should be well defined and prac- reliable and effective measure to prevent firearm-related injuries
tical rather than general information (eg, write the Poison Help in children. The AAP supports a number of specific measures to
number on the phone; never leave children unattended in water). reduce the destructive effects of guns, including the regulation of
Advice should be tailored to each family after exploring individ- the manufacture, sale, purchase, ownership, and use of firearms;
ual situations through open-ended questions (eg, “Where does a ban on semiautomatic assault weapons; and the strongest pos-
your baby spend awake time during the day?”; “What do you think sible regulations of handguns for civilian use (Reaffirmed 2016).
is the biggest safety risk for your child?”). Health professionals ww Drowning is a leading cause of injury-related death in children.
should be aware of different levels of health literacy and confirm Pediatricians should provide specific targeted messages by age,
understanding rather than rushing through a prepared statement. sex, risk of drowning, alcohol or drug use, water competency,
Access to interventions should be considered, such as cost and and geographical location. Children with special health care
accessibility of helmets and child safety seats. Whenever possi- needs should have tailored anticipatory guidance related to water
ble, pediatricians should coordinate their educational efforts with safety (2019).
current community injury prevention efforts (eg, bicycle helmet ww Injury is the leading cause of death in children 1 to 18 years of age
campaigns, handgun regulation). in the United States. The unique needs of injured children must
be integrated specifically into trauma systems and disaster plan-
Recent Recommendations ning at the local, state, regional, and national levels. Pediatric
injury management should include an integrated public health
The AAP has multiple safety recommendations. Following are newer
approach from prevention through prehospital care, to emergency
and revised recommendations:
and acute hospital care, to rehabilitation and long-term follow-
ww Health equity is fundamental to child safety. Children should up, as indicated (2016).
be protected from injury within their built environment and ww Children exposed to intimate partner violence are at an increased
provided with access to quality, patient-centered, and culturally risk of being abused and neglected and are more likely to develop
effective medical care (Reaffirmed 2013). adverse health, behavioral, psychological, and social sequelae later
ww All children should be restrained in a rear-facing–only or con- in life. It is recommended that pediatricians receive training on
vertible car safety seat used rear facing as long as possible. the identification, assessment, and documentation of abuse; inter-
Importantly, nearly all currently available convertible car safety ventions to ensure patient safety; culture and values as factors that
seats have weight limits for rear-facing use that can accommo- affect intimate partner violence; applicable legal responsibilities;
date children 35 to 40 lb (15.9–18.1 kg) (2018). and violence prevention (Reaffirmed 2019).
ww Motor vehicle crashes are the most common cause of mortality ww The overall death rate attributable to sleep-related infant deaths
and injury for adolescents and young adults in developed coun- remains high. Recommendations for a safe sleep environment
tries. Now present in all 50 states, graduated driver’s license include supine positioning, the use of a firm sleep surface, room
programs introduce driving in a staged manner of increasing sharing without bed sharing, and the avoidance of soft bedding
risk and responsibility. The AAP recommends that pediatricians and overheating (2016).
know their state laws addressing teenage drivers, encourage ww Sport-related concussions are a major health concern in young
seat belt use, help parents identify acute or chronic medical or athletes. Although all concussions cannot be prevented, reduc-
behavioral risk factors that might affect their teenager’s driv- ing the risk through rule changes, educational programs, equip-
ing ability, discourage distracted driving, encourage restrictions ment design, and cervical strengthening programs may be of
on nighttime driving and limits on number of passengers, and benefit. Health care professionals should have an understanding
counsel teenagers about the dangers of driving while impaired of their individual state’s laws regarding return to play after a
(2018). concussion (2018).

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 Chapter 45: Injury Prevention 317

Additional policies and guidelines can be found at www.

aappublications.org/search/%20subject_collection_code%3A100 CASE RESOLUTION
using the key words “injury prevention.” Your experience with this case prompts you to become more involved in advocacy
about adolescent drivers and accident prevention. You have the opportunity
Pediatricians as Advocates to engage families in injury prevention as well as to influence the individu-
als who manufacture the products and pass the laws that affect children’s risk
As advocates for child safety, pediatricians can play a major of injury.
role in injury prevention outside the clinical setting. Pediatricians Some of the factors that influenced the injury and outcome include a
have started community programs or provided support to ongo- significantly increased risk of fatal crash for adolescent drivers, evidence that
ing programs. Safe Kids Worldwide (www.safekids.org) is an graduated driver licensure laws and other restrictions on young drivers reduce
deaths, passage and enforcement of laws pertaining to cell phone use in cars,
organization made up of safety experts, educators, corporations,
vehicle design, road conditions, availability of emergency rescue services and
foundations, governments, and volunteers whose mission is to access to specialized pediatric care, and pediatrician counseling to caregivers and
prevent childhood injury through education and advocacy. Safe adolescents advocating family driving rules.
Kids and other issue-specific organizations, such as the Children’s
Defense Fund (www.childrensdefense.org) and Everytown for
Gun Safety (https://everytown.org), can provide resources to sup-
Selected References
port pediatricians in local or national projects and provide ave-
nues for legislative participation. Through community pediatrics American Academy of Pediatrics Council on Injury, Violence, and Poison
programs (www.aap.org/commpeds), the AAP provides grants to Prevention. Injury, violence, and poison prevention. https://www.aap.org/en-us/
advocacy-and-policy/aap-health-initiatives/Injury-Violence-Poison-Prevention/
pediatricians and pediatric residents who want to create innova-
Pages/default.aspx. Accessed September 4, 2019
tive community projects promoting child health.
Centers for Disease Control and Prevention, National Center for Injury
Legislative advocacy is an exciting opportunity for health profes-
Prevention and Control. Injury prevention & control. https://www.cdc.gov/
sionals to effect wide-reaching change. States have enacted laws cov- injury/index.html. Reviewed August 28, 2019. Accessed September 4, 2019
ering many aspects of injury prevention, including car safety seats,
Centers for Disease Control and Prevention, National Center for Injury
poison centers, cribs, playgrounds, amusement parks, protective Prevention and Control. Welcome to WISQARS. https://www.cdc.gov/injury/
gear for sports, swimming pools, and school buses. Dramatic reduc- wisqars. Reviewed August 6, 2019. Accessed September 4, 2019
tions in injuries often follow safety legislation. For example, infant Chen J, Kresnow MJ, Simon TR, Dellinger A. Injury-prevention counseling
walker-related injuries have decreased by 76% since the introduc- and behavior among US children: results from the second Injury Control and
tion of the ASTM International F977 Consumer Safety Performance Risk Survey. Pediatrics. 2007;119(4):e958–e965 PMID: 17403833 https://doi.
Specification for Infant Walkers in 1997 and the introduction of org/10.1542/peds.2006-1605
stationary activity centers as alternatives to mobile infant walk- Gittelman MA, Pomerantz WJ, Schubert CJ. Implementing and evaluating an
ers. Other examples of product-related legislation include federal injury prevention curriculum within a pediatric residency program. J Trauma.
crib standards of 1974 mandating close spacing of vertical slats to 2010;69(4 suppl):S239–S244 PMID: 20938317 https://doi.org/10.1097/
reduce the risk of entrapment, the Child Safety Protection Act of TA.0b013e3181f1ed63
1994 requiring toy safety labels on any balls with a diameter less Hammig B, Jozkowski K. Prevention counseling among pediatric patients
than 1-³⁄4 in (4.44 cm), window blinds manufactured with tassels presenting with unintentional injuries to physicians’ offices’ in the United
instead of loops and children’s clothing without drawstrings to pre- States. Prev Med. 2015;74:9–13 PMID: 25668219 https://doi.org/10.1016/j.
ypmed.2015.02.001
vent strangulation, and toy boxes manufactured with safer lids and
air holes in case a child is trapped within. Similarly, in December Kann L, McManus T, Harris WA, et al. Youth Risk Behavior Surveillance—United
States, 2017. MMWR Surveill Summ. 2018;67(8):1–114 PMID: 29902162 https://
2010, the US Consumer Product Safety Commission voted to ban
doi.org/10.15585/mmwr.ss6708a1
drop-side cribs, citing the recall of more than 9 million cribs over
Li L, Shults RA, Andridge RR, Yellman MA, Xiang H, Zhu M. Texting/emailing
the previous 5 years and the entrapment and death of 30 babies over
while driving among high school students in 35 states, United States, 2015.
the previous 10 years. The ban went into effect in June 2011 and pro- J Adolesc Health. 2018;63(6):701–708 PMID: 30139720 https://doi.org/10.1016/j.
hibits the sale of such cribs even at garage sales. jadohealth.2018.06.010
Pediatricians can heighten awareness about the magnitude of Peden M, Oyegbite K, Ozanne-Smith J, et al. World Report on Child Injury
childhood injuries through calls or letters to legislators, testify- Prevention. Geneva, Switzerland: World Health Organization; 2008. https://www.
ing about the benefits of specific safety legislation, or partnering who.int/violence_injury_prevention/child/injury/world_report/en. Accessed
to introduce new legislation. Pediatrician legislative involvement September 4, 2019
is critical to ensuring evidence-guided local and national injury US Consumer Product Safety Commission. https://www.cpsc.gov. Accessed
prevention. September 4, 2019

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 CHAPTER 46

Fostering Self-esteem
Richard Goldstein, MD, FAAP

CASE STUDY
A 4-year-old girl is brought to the office for her annual “Sit up straight,” “Stop fidgeting,” and “Act your age.”
physical examination. She has been healthy. The mother The mother rolls her eyes as she says, “She doesn’t know
is concerned that her daughter is shy and does not seem how to act.”
eager to play with other children. She does not attend
child care or group activities outside the home, and she
Questions
1. What is self-esteem?
spends most of her time with her mother, grandmother,
2. How do parents or other caregivers affect the
and 7-year-old sister, with whom she gets along well.
development of their child’s self-esteem positively
Both parents work outside the home.
and negatively?
The girl’s medical history is unremarkable with the
3. What role does discipline play in the development
exception of an episode of bronchiolitis at 8 months of
of self-esteem?
age. She has reached all her developmental milestones
4. How does illness affect self-esteem?
at appropriate ages, speaks clearly in sentences, can
5. What suggestions can primary care physicians give
dress herself without supervision, and can balance on
parents and other caregivers to help foster positive
1 foot with no difficulty.
self-esteem in children?
Her physical examination is entirely normal. At
times during the visit, her mother sharply tells her to

A mother worries about her spouse’s sarcasm with their son. A father moments of achievement, self-esteem is also demonstrated by con-
worries that indiscriminate praise at school inflates his child’s sense fidence that difficulties, failures, and disappointments are tolerable
of her abilities while setting her up for “a rude awakening.” As the and can be accommodated. Self-esteem is essential to a child’s well-
pediatrician walks into an examination room, a parent whispers being and influences the development of relationships and iden-
that he wants to discuss their child’s obesity away from the child. An tity during childhood and adulthood. It is grounded in the fact that
urgent care visit is scheduled to discuss the persistent bullying of a success with other people is fundamental to a sense of who we are.
child in school. Embedded in these scenarios and countless others Whether a child’s self-estimate is inflated, overly negative, or
is a concern that a child’s self-esteem is malleable and fragile and accurate is not of importance to the concept of self-esteem; no
that its preservation is crucial to a child’s success. What should a pri- “objective yardstick” exists. In this regard, it is important to under-
mary care pediatrician know about how a child’s self-concept affects stand that a difference exists between high self-esteem and nar-
the child’s thoughts, feelings, and behavior? cissism. A child can have a healthy self-regard without a sense of
entitlement, grandiosity, or feelings of superior worth. This distinc-
Basic Concepts tion is important when critically reading research finding correla-
Parents often use the term “self-esteem” to describe their child’s tions between bullies and high self-esteem, for example; studies also
confidence, implying a sense of agency and feelings of self-worth. find high self-esteem in those who intervene on observing bullying
In fact, self-esteem is a social psychological construct; it is the prod- behavior. Alternatively, a child’s disfigurement or disability should
uct of how an individual understands the effects of that individual’s not preclude that child from possessing feelings of positive self-
actions (ie, agency) and how the individual believes those actions esteem. Self-esteem is a phenomenologic construct, and its impor-
are seen by others (ie, self-worth). Agency, or self-efficacy, is a child’s tance lies in how a child’s self-concept shapes that child’s actions.
confidence in his, her, or their capacity to successfully complete Self-esteem requires the development of certain cognitive abili-
tasks or accomplish goals. Self-worth is the assessment that what a ties, but it is also based on experiences with parents, peers, and other
child thinks, wants, and does is important. High self-esteem often is caregivers. Preschool-age children become much more indepen-
accompanied by a sense of self-respect, purpose, and self-awareness, dent and spend more time away from primary caregivers compared
whereas low self-esteem typically is associated with self-questioning, with younger children. This newly acquired independence, however,
defensiveness, and disproportionate self-criticism, even when does not remove the need for attention, interest, and approval from
receiving positive feedback. Although it may be most apparent in their parents. Agency must be nurtured, and not simply controlled,

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 320 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

to support self-esteem. Opportunities to demonstrate the compe- physicians should bring the parent’s or parents’ focus to this impor-
tence of children can be recognized in the autonomy that appro- tant aspect of their child as a normal part of anticipatory guidance.
priate parenting supports and heard as a source of pride in parents Pediatricians and health professionals are uniquely positioned to
taking note of it. Educating parents about what is developmentally offer specific recommendations for fostering self-esteem that may
correct can be important. Competent play among preschoolers, for affect the lives of their patients. During health maintenance visits
example, may involve playing alongside other children and may not with the child, these health professionals should model respect for
necessarily consist of cooperative play (eg, helping each other in the child’s self-concept and highlight the growing capabilities found
addition to playing together). in the child’s interactions.
The emergence of self-esteem can be framed in terms of Erik
Erikson’s conceptualization of a child’s social development. In the
“industry versus inferiority” stage, the task of a child is to demon- Research
strate the child’s efficacy and for those efforts to be acknowledged Research on self-esteem examines global self-esteem, that is, a mea-
and appreciated. The joy of autonomy and initiative are suffused sure of the overall assessment of the self, and domain-specific self-
with a need to live up to expectations; for example, for a young esteem, that is, measures of self-assessment related to performance
daughter part of feeling that she is a big girl is feeling that she is a and attributes (eg, academic competence, physical appearance). The
good girl. The first stirrings of conscience and confidence to manage most widely used measure for self-esteem, the Rosenberg Self-Esteem
measured responsibility occur in this stage. The complex interac- Scale, assesses global self-esteem. One difficulty in the self-esteem
tion of temperament, developmental stage, family security, parental literature is that interventions meant to address specific performance
style of discipline, sibling and peer interactions, and school expe- areas are sometimes assessed with global self-esteem measures and
riences coalesce in the experience of a uniquely competent child. vice versa. It is no surprise that a child’s general sense of self is insuf-
For her caregivers, the desire to encourage a kind of fearlessness is ficient to improve the child’s performance on a spelling quiz.
balanced by the very real need to keep the child safe and appropri- Some proportion of self-esteem is biologically rooted. Twin stud-
ate. All these aspects of her life contribute to the development of ies indicate that approximately 40% of variability in self-esteem can
her competence, autonomy, and relatedness and animate her self- be explained by genetic factors. However, self-esteem also is known
esteem. The development of self-esteem can be seen when children to have cultural underpinnings that seem to especially resonate in
at the age of 5 years begin to experiment with identity roles, when contemporary American culture. Whether the prominence of self-
they demonstrate an awareness of social comparison at age 7 or esteem is somehow rooted in the US national identity is unknown,
8 years in their peer play and activities, and most clearly when they but it is clear that the importance of self-esteem is not universally
develop a sense of global self-esteem at approximately 8 years of shared in all populations. For example, it is hard to detect self-esteem
age. Research also concludes that a general decline in self-esteem as a motivating factor in more collectivist cultures, such as Japan.
occurs from childhood through adolescence. It has been suggested This stands in broad contrast to its importance in US classroom
that social comparisons and increased awareness of the perspec- reform or claims by psychologists first introduced in the 1990s that
tives of others cause adjustments to a growing child’s self-efficacy “self-esteem has profound consequences for every aspect of our exis-
and self-worth. This is intensified during adolescence, when physi- tence.” The proper conceptualization and importance of self-esteem
cal changes and increased academic and social complexity test chil- remains a matter of debate.
dren’s sense of who they are. At first glance, it would appear that an association exists between
Much of a child’s self-concept is established and reinforced by level of self-esteem and important health outcomes. Credible
those around them, especially primary caregivers. Although impor- research concludes that high self-esteem predicts decreased rates
tant activities occur when the child is alone and engaged in indi- of depression and increased happiness. Adolescents with high
vidual pursuits, much of a child’s self-concept develops in a context self-esteem have better mental and physical health as well as higher
of relatedness. This extends beyond the family during school years, graduation rates and are less likely to have a criminal record. Higher
when peers and teachers assume a more influential role in the con- self-esteem predicts improved persistence when confronting
tinued development and reinforcement of self-esteem. The develop- failure. Lower levels of self-esteem are associated with increased
ment of self-esteem is transactional, built by the responses children rates of obesity, drug abuse, and tobacco use. It is uncertain, how-
receive to their increasing initiative and abilities, resting on a foun- ever, whether the demonstrated outcomes associated with levels
dation of security. A secure interpersonal environment is essential of self-esteem are caused by those levels of self-esteem. For
to exploration and correction. This transactional nature is at the example, researchers are still trying to determine whether self-
core of self-esteem interventions and their enthusiasm for effects esteem results in higher performance or better performance
on individuals as well as society. results in higher self-esteem. Causation can also be considered
All parents hope that their children will develop a positive self- in the context of known correlations between self-esteem and
concept that will aid them throughout their lives. Unfortunately, academic performance and motivation, task performance, aggres-
discussions related to self-esteem usually are held as a result of a cri- sion, sex and/or gender differences, ethnic differences, and health
sis or an observation by worried parents or teachers. Primary care outcomes.

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 Chapter 46: Fostering Self-esteem 321

Whether interventions promoting enhanced self-esteem result effectance-promoting feedback, and freedom from demeaning cri-
in improved outcomes is debatable. Research seems to have tipped tique; parenting that promotes autonomy rather than control; and
the scales toward benefit from such programs when the programs a secure interpersonal environment of relatedness are all essential
include 3 specific elements: attributional feedback (ie, helping chil- contributors to intrinsic motivation and, by extension, self-esteem.
dren attribute outcomes to effort), goal feedback (ie, promoting real-
istic, attainable goals), and contingent praise (ie, praise based on Optimal Challenges
effort and improvements in performance). This seems to under- Successful parenting creates opportunities for children to safely
score the central importance of examining domain-specific inter- extend their boundaries while ensuring their feeling of personal
ventions and self-estimation. control. An overly critical or controlling style of parenting constrains
the emergence of a child’s young sense of competence and, by exten-
sion, affects the child’s self-esteem. Being handed a scribble draw-
Parental Guidance
ing by a preschooler and told what it represents is emblematic. An
Illness and Difficult Family and Social appreciative, interested, positive response to the disordered marks
Challenges is sustaining, whereas disinterest and immediate correction or crit-
Physical and psychiatric illnesses threaten children’s sense of who icism is undermining. Inconsistent or harsh parenting and author-
they are, undermining their self-efficacy and self-worth. Children itarian as opposed to authoritative parenting styles detrimentally
can be left uncertain of their standing, expecting failure or feeling affect levels of self-esteem.
that they are inferior. Opportunities may exist for trusted physicians It may be important to discuss the difference between encour-
to help affected children and their families find realistic, achievable agement and pressure with parents, and many parents may benefit
goals that affirm the children’s sense of agency. It is often beneficial from an introduction to the concept of “positive communication.”
to help a child and family shift the narrative away from preexisting To support positive self-esteem, children need encouragement at all
ideals and toward what is possible and of value. levels of their development (Box 46.1). This is communicated ver-
A child’s self-esteem can be particularly vulnerable in cer- bally and nonverbally and should be distinguished from overt pres-
tain social and familial situations. A child may respond to nega- sure. For example, a first grader learning to spell should be praised
tive experiences in school or at home with feelings of shame and for early, if flawed, application of phonemes, such as spelling “win-
worthlessness. Marital conflict, divorce, or the abuse of a parent ter” as “wntur,” and may not need correction at that point. Generally,
may negatively influence the self-esteem of children who may feel children should be given room to experiment and develop at their
complicit in or responsible for the problem. In such circumstances, own rate and should not be coaxed into activities before they are
parents are often concerned about the effect on the child, which pro- ready or judged too harshly for earnest, early attempts.
vides an opportunity to work together to minimize negative effects. When pressure occurs, it may be the result of parents having
Honest, open communication between the physician and the par- unrealistic expectations of how a child learns and develops or an
ent or parents reinforces the need for sensitive support of the child improper sense that lack of success is caused by laziness or a char-
and helps the parent or parents set priorities or rehearse how they acter flaw. Inadvertent pressure can be detrimental and can cause
will talk with the child about a given social or familial situation. tremendous frustration when, for example, a parent tries to lead a
(For in-depth discussion of divorce in particular, see Chapter 149.) toddler into toilet training when the child has shown few signs of
The issue of self-esteem is important in the context of man- readiness. Pressuring young children to give up pacifiers or another
aging “new morbidities.” The diagnoses of obesity, attention- security object without giving thought to the child’s readiness may
deficit/hyperactivity disorder, and learning disabilities can have in also be quite anxiety provoking to them.
common the taint of personal judgment and the threat of under-
mining self-worth. Frank language that is sensitive to a child’s
self-esteem has a role in the disclosure of diagnosis, in addressing Box 46.1. Encouraging Self-esteem
the reactions of both parent and child, in determining a realistic
treatment plan, in acknowledging the frustrations and shame that Don’ts
come with slow progress, and in the framing of ultimate outcomes. ww Have negative expectations.
Parents and patients will benefit from careful modeling by the pedi- ww Focus on mistakes.
atrician of how to represent and talk about the problem. ww Expect perfection.
In the clinical setting, it can be challenging to provide prac- ww Overly protect children.
tical advice that reflects research in this area. One helpful model Dos
for understanding self-esteem is the self-determination theory, in ww Show confidence in children’s abilities.
which a child’s general self-concept is understood as an organiza- ww Build on children’s strengths.
tion of complex, hierarchically interrelated components. Self-esteem ww Value children as they are.
is the evaluative aspect of self-concept and is linked to intrinsic ww Stimulate independence.
motivation. Research has demonstrated that optimal challenges,

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 322 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Children may not know what is best for their ultimate develop- The purpose of active listening is to hear the child’s message and
ment, but well-intentioned pressure can be harmful as children grow understand its meaning. The success of active listening rests on a
older. Forcing children to participate in rigid, structured play and, centered approach to important parenting moments; parents should
sometimes, classes or lessons does not foster individual creativity or strive to be attentive and present at these moments. They should stop
independence. The acquisition of technical skill is only part of learn- what they are doing and look directly at their children. They should
ing, and the absence of intrinsic motivation can lead the child to feel be aware of nonverbal cues, such as body posture and facial expres-
that the effort is meaningless or inauthentic. Children ought to, but sion. If children are having trouble understanding their feelings, the
should not merely, do what parents have arranged. Encouragement parent should repeat what they hear them saying, for example, “It
to explore and occasionally take risks should build on their sounds like....”
“islands of competence” or areas of strength. Mistakes should be put A nonjudgmental response that is validating and nondismissive
into the context of a sincere effort and understood in terms of what should be the goal of communication between primary caregivers
can be learned rather than as a humiliation. Pressuring children to and children. The basic message should be stated simply, clearly,
do things “right” or “perfectly” discourages normal, healthy, risk- and at a level that respects the maturity of the child. Words should
taking effort and can hinder future participation in activities unless be easily understood and spoken in a moderate tone, especially in
they are certain they can succeed. cases in which discipline is necessary. Facial expression and body
A variation on this theme is overprotection, in which the parent language should also be consistent with the message parents or care-
controls the child’s environment to reduce any risk of failure or dis- givers are trying to convey.
comfort to the child. This generally comes at the cost of sacrificing Many parenting courses teach primary caregivers to use the “I”
the experience of novel achievement and developing the competence method of communication, which requires that parents explain
to work through frustrations and disappointments. Indiscriminate, their feelings to children rather than blame them for their actions.
unanchored praise is a corollary to this. Parents should be cautioned This approach is believed to be less threatening and demeaning
against always speaking in the superlative to their child and avoiding for children, especially in situations requiring discipline. The “I”
acknowledgment of any shortcomings. Children are adept at seeing method has 4 recommended steps: statement of behavior or sit-
the world as it is. A failure to see themselves reflected realistically by uation to be addressed (eg, “When you...”); statement in specific
parents or caregivers may feed a sort of insecurity in which despite terms of how one feels about the effect of the situation on oneself
the glowing language of parents, the children feel uncertain of their (eg, “I feel...”); statement of reason (eg, “Because...”); and state-
actual worth. It robs children of opportunities to make accommo- ment of expectations (eg, “I would like...,” “I want...”). Using this
dations. Both overprotection and indiscriminate, unanchored praise approach, a parent might state, “When you speak so unkindly, I feel
have a negative effect on self-esteem. upset because I would never speak with you in that way. It makes
you seem like the kind of person that you are not. I would like you
Effectance-Promoting Feedback and to speak with me in the way we all try to speak with you, no mat-
Freedom From Demeaning Critique ter how angry you feel.”
Specific means of communication that support self-esteem include Parents should be encouraged to reflect on the complex relation-
active listening, use of positive language, discarding “labels,” use ship between discipline and self-esteem, and they should be ques-
of encouragement rather than pressure, and use of the “I” method tioned about their impressions of their child’s independence and
of communication (Box 46.2). The pediatrician can coach parents competence. Promoting the growth of healthy autonomy should be
that a child’s behavior should not be expected to follow a rigid code a goal for all parents. As children grow, so too does their ability to
of conduct at all times, but rather that the goal is a “good enough” act responsibly and maintain greater self-control. This central insight
environment in which well-intended parents make a consistent effort is an important means of helping parents conceptualize discipline
to be mindful of how they interact with their children. A useful way as more than simply punitive.
to set the correct tone with parents and caregivers is with the appli- The poorer outcomes associated with authoritarian parenting
cation of strength-based counseling, in which advice grows out of a underscore the importance of positive language, even when disci-
focus on what parents are doing right. plining children (see Chapter 50). For instance, when children are
playing kickball in the house, it is understandable that a parent might
yell, “Don’t play ball in the house!” or “Haven’t I told you before? No
ball playing inside!” Although emotionally honest parenting in a lov-
Box 46.2. Communication That Builds Self-esteem
ing context is the best parenting, parents should be mindful of when
ww Active listening they are speaking in a reprimanding and negative fashion or trans-
ww Use of positive language mitting their frustration. Parents or other adults should attempt to
ww Discarding “labels” tell children clearly what they can do, what the limits are, and the
ww Use of encouragement reason for these rules. For example, a calm, more positive response
ww Use of the “I” method of communication might be, “You have to stop playing ball right now. If you kick the
ball inside, you may break a window or hurt yourself or someone

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 Chapter 46: Fostering Self-esteem 323

else. You may kick the ball outside in the backyard.” Communicating
in a clear manner changes the tone of the interaction from a repri- CASE RESOLUTION
mand for being bad to one in which rules are clarified to a child who The health maintenance visit provides an opportunity for promoting parental sup-
wants to do the right thing. port of the child’s self-esteem. The pediatrician can interview the shy child and dem-
Parents should praise their children for successes and achieve- onstrate the use of sincere general compliments to coax the child’s engagement.
ments. Failures should be put into context whenever possible, The interviewer might invite her to speak about herself (eg, “What are your favor-
ite things?” “What is your favorite color?”), while using humor and enthusiasm to
although the acknowledgment of mistakes can be important. A
engage her in sharing some details. This models interactions, which allows the child
forgotten jacket or a careless job with homework can be frustrat- to demonstrate her autonomy, with the hope that this approach will be repeated by
ing, but neither is worthy of a demeaning critique. Negative labels the parent at home. The health professional can reassure the girl and her mother
have no helpful role. If children hear themselves referred to pejo- that the child’s overall health and development are normal. Additionally, after
ratively by their primary caregivers, not only is an undesirable expressing appreciation for the careful, protective experience the parent has man-
aged for the child, the physician should attempt to normalize the child’s behavior
behavior modeled, but they will feel undermined and wonder about
for the parent. Although it may be important to directly discuss shyness in a devel-
the truth of such statements, no matter how innocuous they may opmental context, whether the girl is shy is not certain, and the parent’s sense of
initially seem to parents. deficiencies should be challenged by a view of developing competencies. Concrete
suggestions should be offered for positive communication, such as minimizing
“don’t” and “no” phrases and being cognizant of how the parent speaks about the
Promoting Autonomy daughter. The parent should be encouraged to think about the child’s temperament
Children do best when they are provided with clear, consistent guide- when considering involvement in multiple school activities but be reminded that
lines for their behavior while simultaneously being encouraged to the parent has not yet seen the child in an independent setting, where she may
negotiate quite well and show sociability not yet evident at home.
do well, pursue their interests, and increase their abilities. They
are more likely to achieve autonomy when they see this behavior
mode­led in the adults around them. Parents and other caregiv-
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single prescription for how to talk with children. Pediatricians have Trzesniewski KH, Donnellan MB, Moffitt TE, Robins RW, Poulton R, Caspi A.
a special role in bringing together all these elements as they advo- Low self-esteem during adolescence predicts poor health, criminal behavior, and
cate for the children under their care and the people those children limited economic prospects during adulthood. Dev Psychol. 2006;42(2):381–390
are becoming. PMID: 16569175 https://doi.org/10.1037/0012-1649.42.2.381

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 CHAPTER 47

Sibling Rivalry
Carol D. Berkowitz, MD, FAAP

CASE STUDY
An 8-year-old boy is brought to the office for an annual Questions
checkup. During the course of the evaluation, his mother 1. What is sibling rivalry?
reports that her son and his 6-year-old sister are always 2. What is the physician’s role in counseling a family
fighting. She says her son hits his sister and pulls her about sibling rivalry?
hair, and nothing she does prevents them from fighting. 3. What is the role of anticipatory guidance in preparing
The boy is a B student and has no behavior problems in older children for the birth of a new sister or brother?
school. The medical history and physical examination are 4. How does birth order and an individual’s sex affect
completely normal. sibling rivalry?
5. What are some of the unique considerations related
to sibling rivalry between stepsiblings?
6. What are some practical suggestions to share with
parents about sibling rivalry?

Sibling relationships are important in helping children shape peer have battling siblings. Television has included the issue of sib-
and, later, adult interactions. Moderate levels of sibling rivalry are a ling rivalry in sitcoms, sometimes trivializing the challenges for
healthy indication that each child is assertive enough to express his parents. However, some shows portray unrealistic compatibility
or her needs or wants. Siblings educate and socialize together and between children. Shows such as Good Luck Charlie and Jessie have
mediate parental demands. Siblings often spend more time interact- shown large families in which conflicts are easily and humorously
ing with each other than with either parent. The sibling relationship resolved. This may perpetuate unrealistic expectations in families.
is characterized by continuity and permanence, but the relationship The 2019 comedy Fighting With My Family relates the true story
is not without turmoil. of a former wrestler and the deterioration and subsequent compe-
Sibling rivalry refers to the competitiveness between siblings tition that develops between a sister and brother and their careers
based on the need for parental love and esteem. The rivalry is often as wrestlers.
characterized by jealousy, teasing, and bickering. The term was Sibling rivalry is a universal phenomenon occurring even in
introduced in 1941 by David Levy, who described it as “a common the animal kingdom. For instance, the firstborn eaglet pushes the
feature of family life.” Alfred Adler, the noted psychologist, described other eaglets out of the nest as soon as they are hatched as a way
siblings as “striving for significance” within the family and noted of ensuring an adequate food supply. Studies in animals show
that birth order had a strong influence on development. Historical variation depending on brood or litter dominance and sex dom-
examples of sibling rivalry include relationships between the bibli- inance (male or female) within the species. Aggressive interac-
cal figures Cain and Abel, Joseph and his brothers, Jacob and Esau, tions are more likely when there are multiple offspring in a single
and Leah and Rachel. Sibling rivalry is also noteworthy in pairs of brood. Among wolves, however, older siblings help to feed and
celebrities, such as actresses Joan Fontaine (Academy Award win- guard younger ones. In humans, the fear of displacement,
ner for Suspicion) and her older sister, Olivia de Havilland (Academy dethronement, and loss of love occurs with the birth of a new
Award winner for To Each His Own and The Heiress). Even after more brother or sister, leading to sibling rivalry. Older children
than 40 years, the turbulence of their relationship has remained fear they are not good enough and that their parents need
legendary, was termed one of the most dysfunctional sibling rela- to replace them with a new offspring. Such feelings lead to a fear
tionships in Hollywood, and may have had its roots in their simul- of abandonment. Jealousy also plays a role, and older children
taneous nomination for an Academy Award in 1942. By 1975, the may be angry with younger siblings for displacing them within
sisters were no longer in communication with each other. Other the family.
siblings whose performances have often been compared include Sibling rivalry frequently has a negative effect on parents because
football players Peyton and Eli Manning, tennis stars Venus and it is hard for them to see 1 of their children hurt, even if it is by a sis-
Serena Williams, and musicians Liam and Noel Gallagher of Oasis ter or a brother. The challenge for parents is to know when, and when
and Ray and Dave Davies of the Kinks. Many Shakespearean plays not, to intervene and what strategies to use to minimize conflicts.
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 326 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Physicians can help by offering anticipatory guidance to all parents challenges on sibling relationships. The unique strengths of each
and specific recommendations to parents who are experiencing such child need to be acknowledged.
individual problems. Issues of sibling conflict change over time. Toddlers are protec-
Sibling abuse is a relatively recent concept that recognizes the tive of their toys and belongings and are particularly upset when a
occurrence of physical, emotional, or sexual abuse of 1 sibling by younger sibling touches their possessions. Sharing is a challenging
another. The aggression may range from very mild to severe. Parents theme of the toddler years. During their school-age years children
may not recognize the intensity of the aggression and may attribute are concerned about equity and fairness. They may be upset by what
negative interactions to sibling rivalry. Physicians should be knowl- they construe as preferential treatment (eg, when a 1-year-old sib-
edgeable about sibling abuse and be able to help parents to differen- ling is not expected to put his or her toys away). Sibling competitive-
tiate between rivalry and abuse. ness is said to peak between the ages of 10 and 15 years. Adolescents,
with their additional responsibilities, including minding younger
Epidemiology siblings, may resent the siblings for imposing on their time. Sibling
rivalry can persist into adulthood, and one-third of adults describe
Sibling rivalry is a universal phenomenon, and a number of factors
their relationship with a sibling as distant or rivalrous. After age
influence its development. Time interval between children affects
60 years, 80% of siblings report being close.
the degree of rivalry, as does the age of the older children. Toddlers
Significant sibling abuse is said to affect 3 in 100 children. Less
who are entering the “terrible 2s” may have a particularly hard
violent abuse is reported to occur in as many as 35 per 100 chil-
time mastering independence and tolerating the presence of their
dren. These figures are reported to cross all socioeconomic levels.
younger sibling. Close spacing results in more problems, particu-
larly when children are fewer than 2 years apart. In such situations,
Clinical Presentation
older children still have dependency needs, often feel less secure,
and experience a need for maternal attention. They stay closer to Parental concerns related to sibling rivalry consist of fighting
mothers, are less playful, and are tenser. Closely spaced children between siblings, including physical violence and verbal abuse, bick-
engage in less spontaneous play, seem angrier, and issue sterner ering, and regression to immature behavioral patterns. Although
commands to their playmates. Sex of a new sibling also influences such immature behavior occurs most often following the birth of
the relationship. There tends to be greater rivalry between same- a new baby, it may also be apparent if 1 sibling is receiving more
sex siblings. Additionally, a child’s temperament affects sibling attention, such as during an illness or after a major accomplish-
relationships. The 3 components of temperament include emo- ment. Regressive behavior includes bed-wetting, drinking from a
tional intensity, activity level, and sociability. It is also important bottle, and wanting to be carried to bed. Substitution behavior, such
to remember that children are egocentric, which, according to as nail-biting in place of biting the new sibling, may occur after the
Erich Fromm, lasts through 8 years of age. This contributes to a birth of a new baby.
child’s willingness to share (including toys and parental attention) Before the birth of a new baby, parents may report that their chil-
and to act unselfishly. dren exhibit temper tantrums, irritability, and solemnness. They
Position in the family also influences sibling rivalry. Middle chil- may mimic the pregnancy by eating a lot and putting a pillow under
dren experience what is referred to as middle-child syndrome; they their clothes. In addition, children may have psychosomatic symp-
lack the prestige of older children and the privileges of younger ones. toms such as stomachaches or headaches. Risk factors for malad-
These children are often the least secure and strive hardest to gain justment following the addition of a sibling include family discord,
affection. Special difficulties may develop if middle children are physical or emotional exhaustion in parents, and housing insecurity.
the same sex as older ones. Middle children grow up to be flexible, Conversely, a good marital relationship and family support facili-
adaptable, and good negotiators. In myths and folklore, youngest tates the adjustment to new siblings.
children are “favorites.” They are often the ones defended by par-
ents when there are bouts of fighting. Differential Diagnosis
Twins rarely present a problem of sibling rivalry; instead, they Dilemmas concerning the correct diagnosis of sibling rivalry
have a problem maintaining their individuality. However, sets of most often relate to the appearance of behavioral changes, such as
twins create problems for older siblings because the older siblings regressive or aggressive patterns after the birth of a new sibling.
are not as unique as the pair of twins. For example, a child who was previously toilet trained may become
Stepsiblings also present a unique problem in sibling rivalry. incontinent of urine. Although urinary tract infection may be con-
Children of divorce frequently feel abandoned by 1 parent and sidered in the diagnosis, a careful history concerning the birth of
in competition for the time and love of the custodial parent (see the sibling reveals the correct etiology.
Chapter 149). Competition with stepsiblings is especially difficult The other issue to consider is whether the sibling rivalry has
if the stepsiblings are in the same home. moved into the arena of sibling abuse. Risk factors for sibling abuse
There are also unique considerations when 1 child has a chronic include the absence of parents from the home, domestic or commu-
or potentially terminal illness or long-term disability. Similarly, nity violence, and children having inappropriate family roles (eg,
being the sibling of a gifted child (see Chapter 35) places unique having to care for younger siblings).

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 Chapter 47: Sibling Rivalry 327

Evaluation purchase a gift for older children that represents a present from the
new baby, such as T-shirts that announce the older sibling’s new
The evaluation of children with suspected sibling rivalry involves
status, such as “big sister” or “big brother.” In addition, older sib-
a history of the problem and parental strategies for addressing the
lings may be given a doll to serve as a baby they can care for. Parents
difficulties. The parent should be particularly queried about 1-to-1
should point out the advantages of being older with comments such
opportunities between parents and individual children. Physical
as, “You can stay up later,” or “You can walk and play with all these
examination and laboratory assessment are noncontributory.
toys.” Frequently, the birth of a new baby is met with regressive
If there is concern for sibling abuse, appropriate additional ques-
behavior in older siblings. Regressive behavior should be addressed
tions include the following: Is one child always avoiding another sib-
with tolerance and a realization that symptoms resolve with time.
ling? Has there been a significant change in a child’s behavior? Does
Once the birth mother goes to the hospital, she should be advised
one sibling always seem to be the aggressor and the other the target?
to maintain contact with older children by telephoning or video chat-
ting. Video chatting will also allow the older siblings to see the new
Management addition to their family. Many hospitals now allow for visitation by sib-
The focus of management is to allow parents to recognize the nor- lings. Currently, hospital stays are so brief (often just 24 hours) that this
malcy of sibling rivalry while helping them define the behaviors that period of separation is much shorter than it was previously. Household
are acceptable or unacceptable within the family context and to recog- changes that may be necessitated by the birth of the new baby, such as
nize when the rivalry has progressed to sibling abuse. Children fight room changes, the substitution of a bed for a crib, and entrance into
more often in families when parents condone fighting and aggres- nursery school, should be made before the arrival of the new baby.
sion between siblings as normal behavior. Likewise, children of par-
ents who are angry may interact with their siblings through anger. Rivalry Between Older Children
Parents should be counseled about this. Parents may not appreciate Physicians need to consider individual parenting techniques when
their child’s fear of loss of parental love as the basis of sibling con- counseling parents of older children. Parents who compare 1 child
flicts. They should be reminded that many children think, “If I am so with the other may foster contentious behavior, and those who strive
good, why do I have to be replaced?” Parents should be prompted to to treat all children equally may inadvertently perpetuate rivalry.
empathize by imagining how they would feel if their spouse brought Children need to feel that they are unique rather than ordinary. For
home another mate, even if they were reassured about being loved. example, parents who buy both children the same presents may think
Physicians can also help parents address sibling rivalry by having they are preventing rivalry from developing, but they are actually
them consider their treatment of children in terms of uniqueness depriving each child of a sense of uniqueness. The harder parents try to
versus uniformity and quality versus inequality. In general, parents be uniform, the more vigilantly children may look for inequality. Each
should be advised to set the ground rules for acceptable behavior. child needs a parent’s undivided attention and time alone together.
Such rules include no hitting, punching, hairpulling, name-calling, Siblings also need time apart from each other, and they should
cursing, or door slamming. There may be a neutral area in the home be encouraged to hold separate playdates and individual activities.
that can be set aside for arbitration should disagreements arise. Not all children in a family need piano lessons and soccer practice.
Moving to a neutral area also allows for some time to cool off. Parents Individuality and uniqueness are important. The more agreeable a
should be reminded that children who are hungry, tired, or bored are parent-child relationship is, the more agreeable a sibling-sibling rela-
more easily frustrated and may start fights more readily. tionship is because each child has good self-esteem. Practitioners should
recommend uniqueness and quality in each parent-child relationship.
Birth of New Siblings Parents sometimes have to contend with sibling rivalry between
Parents may notice behavioral changes in their children before older children. Physicians should reassure parents that these older
the birth of a new sibling. These changes depend on the age of the children should be allowed to vent their negative feelings toward
children and presence of other siblings. Children should be told each other. For example, if a girl refers to her brother by saying,
about the upcoming birth. The timing depends on the children’s “I hate him,” the parents should respond by validating these emo-
age; younger children do not need much lead time. Some studies tions and saying something like, “It sounds as if he’s done some-
have evaluated the inclusion of older children in the birthing pro- thing to really annoy you.” Parents should also be advised not to take
cess. The results of these studies vary, but they suggest that chil- sides. They should examine how they usually respond to squabbling
dren younger than 4 years need their mother for emotional support between siblings. Is one child’s name always called first during a fight?
and are concerned about her physical exertion during the birthing Do they perpetuate sibling rivalry by using certain nicknames (eg,
process. Some older children may also want to distance themselves “turkey brain”) or other derogatory terms? Parents should assume that
from the actual events. both parties are at least partially guilty and should not allow them-
Physicians should suggest that older children be involved in plan- selves to be drawn into the fight as referees. Parents can respond to a
ning for the arrival of the new baby as a means of minimizing their request for arbitration with a statement such as, “I wasn’t here when
feelings of exclusion. For example, they can help purchase clothes or things started, so I don’t know who is right or wrong.” The parents
prepare the baby’s room. Physicians should also suggest that parents should also advise siblings that they do not have to be friends with

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 328 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

one another, but they should not hurt each other’s feelings. Positive, Sibling Abuse
authoritative parenting should be encouraged (see Chapter 50).
Addressing sibling rivalry can reduce the risk of the rivalry progress-
Anticipatory guidance helps parents anticipate conflictual situ-
ing to abuse. As noted previously, setting ground rules, spending
ations, such as who sits where during long car rides and who holds
time with individual children, and modelling conflict-solving skills
the remote control. Family meetings can be held to determine the
and nonviolent behavior are positive preventive measures.
ground rules that may avoid such battles. If conflicts arise, children
If violence does occur between siblings, parents should separate the
should be allowed to work out a solution by themselves, with the stip-
children immediately and clearly state that such behavior is unaccept-
ulation that the parents will solve the problem if the children do not
able. Children should be given a cooling off period, and then parents
reach an agreement. If fights between siblings have recurrent themes
should convene a family meeting. Parents should encourage children
(eg, which television shows to watch [who controls the remote] or
to discuss their feelings and devise solutions if similar situations
video games to play), parents can devise a weekly schedule. Failure
arise in the future. If violent behavior continues, the family should
to abide by the schedule means both children forfeit the activity. If
be advised to seek professional family and mental health services.
borrowing is the source of disputes, children who borrow from their
siblings should leave collateral, which gets given back when the bor- Prognosis
rowed item is returned. Box 47.1 lists suggestions for parents who Although sibling rivalry may last for years, most siblings become
are seeking advice about fighting between children. good friends as adults. Occasionally, mental health services are
Siblings of Children With Special Health needed, especially if the sibling conflict has led to marital discord,
Care Needs there is concern about physical or severe emotional harm, or there is
evidence of another psychiatric disorder, such as depression. Learning
Nearly 1 in 5 children in the United States is a child with special
how to negotiate with one’s siblings enables children to develop skills
health care needs (see Chapter 44). Caring for such children places
to collaborate with peers and colleagues as adolescents and adults.
increased demands on parents and their resources, and there is
less parental attention or time available for unaffected siblings.
Integrating the unaffected sibling into the families’ care plan and CASE RESOLUTION
activities can be empowering for children and positively influence The mother should be advised not to serve as a referee. She should learn how to
their self-esteem. As with all children, time alone between unaffected validate each child’s feelings about the other. The physician can help her by talk-
children and parents should be strongly encouraged. Support groups ing to her son about his feelings. The mother should be advised to have a dis-
for siblings of children with special health care needs have been cussion with her children during which each child has the opportunity to define
demonstrated to help youngsters cope and deal with their often- areas of conflict and the means to resolve them. The mother has the right and
responsibility to prohibit physical fighting and encourage verbal dialogue.
conflicted feelings of anger at the special attention their brother or
sister receives and their guilt about being healthy.
Selected References
Adams MM. Sister for Sale. Grand Rapids, MI: ZonderKidz; 2002
Box 47.1. Coping With Rivalry Between Siblings: Alderfer MA, Long KA, Lown EA, et al. Psychosocial adjustment of siblings of
Physicians’ Advice to Parents children with cancer: a systematic review. Psychooncology. 2010;19(8):789–805
PMID: 19862680 https://doi.org/10.1002/pon.1638
Dos
Anderson JE. Sibling rivalry: when the family circle becomes a boxing ring.
ww Allow children to vent negative feelings. Contemp Pediatr. 2006;23:72–90
ww Encourage children to develop solutions.
Benhaiem S, Hofer H, Kramer-Schadt S, Brunner E, East ML. Sibling rivalry:
ww Anticipate problem situations. training effects, emergence of dominance and incomplete control. Proc Biol Sci.
ww Foster individuality in each child. 2012;279(1743):3727–3735 PMID: 22719032 https://doi.org/10.1098/rspb.2012.0925
ww Spend time with children individually. Faber A, Mazlish E. Siblings without Rivalry: How to Help Your Children Live
ww Compliment children when they are playing together. Together so You Can Too. New York, NY: W.W. Norton & Co; 1998
ww Tell children about the conflicts you had with your siblings when you Goldenthal P. Beyond Sibling Rivalry: How to Help Your Child Become Cooperative,
were children. Caring and Compassionate. New York, NY: Henry Holt and Company; 2000
ww Define acceptable and unacceptable behavior. Hoffman KL, Kiecolt KJ, Edwards JN. Physical violence between siblings: a the-
Don’ts oretical and empirical analysis. J Fam Issues. 2005;26(8):185–200 https://doi.
ww Take sides. org/10.1177/0192513X05277809
ww Serve as a referee. Nolbris M, Abrahamsson J, Hellström AL, Olofsson L, Enskär K. The experience
ww Foster rivalry. of therapeutic support groups by siblings of children with cancer. Pediatr Nurs.
ww Use derogatory names. 2010;36(6):298–304 PMID: 21291046
ww Permit physical or verbal abuse between siblings. Okun A. Children who have special health-care needs: ethical issues. Pediatr Rev.
2010;31(12):514–517 PMID: 21123514 https://doi.org/10.1542/pir.31-12-514

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 CHAPTER 48

Toilet Training
Jung Sook (Stella) Hwang, DO, FAAP, and Lynne M. Smith, MD, FAAP

CASE STUDY
A 2-year-old boy is brought to the office for a well-child Questions
visit. His mother, who is about to begin toilet training 1. When should the physician begin discussing toilet
her son, asks your advice. The mother says that by the training with parents?
time her daughter was 2 years old she was already toi- 2. What factors help determine a child’s readiness to
let trained, and she wants to know if training her son will begin toilet training?
be any different. The boy was the product of a full-term 3. Is toilet training in boys different from toilet
pregnancy and a normal delivery. He has been in good training in girls?
health, and his immunizations are current. He is devel- 4. What are some of the methods used to toilet train
opmentally normal, uses some 2-word phrases, and has children?
been walking since the age of 13 months. His physical
examination is normal.

The age at which toilet training is carried out is culturally deter- The renewed interest in earlier toilet training in the United States
mined. Some cultures train children at a very early age. For exam- has been attributed to 3 societal factors: the lower cost and increased
ple, among the Digo, an East African tribe, some children between options for child care and schooling associated with children after
2 and 3 months of age are conditioned to urinate or defecate when they are toilet trained, concerns about contagious illnesses (eg, hep-
placed in certain positions. In the United States, the cultural empha- atitis and infectious diarrhea in child care facilities in which diapers
sis is on the learning aspects of toilet training rather than the condi- are changed), and the adverse environmental effects of nonbiode-
tioning aspects. Training based on the learning aspects focuses on gradable disposable diapers.
the cognitive development of children and children’s readiness to Generally, girls are trained a bit earlier than boys, but only by a
learn the complexity of the task. matter of a few months. Additionally, younger siblings often require
Toilet training is potentially a rewarding and frustrating expe- less time to achieve daytime continence than firstborn children.
rience for children and parents alike. Parents may have unrealistic Most children (80%) are trained simultaneously for bladder and
expectations of their child’s capability or may be quite intolerant of bowel control. Approximately 12% are trained first for bowel con-
normal accidents that occur in the training process. It is important trol, with approximately 8% trained first for bladder control. Girls
for the physician to introduce the topic of toilet training early on to achieve nighttime continence at a younger age than boys.
prevent these unrealistic expectations. Refusal by a child to toilet
train or accidents related to toilet training are often cited as a pre- Pathophysiology
cipitating event for child physical abuse. It is recommended that the Toilet training involves the ability to inhibit a normal reflex release
physician introduce to parents the issue of toilet training and pro- action and then relax the inhibition of the involved muscles. For
vide anticipatory guidance by the time a child is 18 to 24 months of the process to be successful, a certain degree of neurologic and
age to help parents develop reasonable expectations. biological development is essential. Although a recent literature
review found no consensus on which or how many readiness signs
Epidemiology are ideal to start toilet training, several factors affect a child’s toilet
The age at which children are toilet trained varies depending on training readiness. Myelination of the pyramidal tracts and con-
social considerations and pressures. Before the 1920s, the approach ditioned reflex sphincter control are necessary. Voluntary con-
to toilet training in the United States was permissive. After this atti- trol is evidenced by myelination of the pyramidal tracts by age
tude changed, the training methods became more rigorous, requir- 12 to 18 months. Conditioned reflex sphincter control occurs by
ing that children be trained at an earlier age. In 1947, only 5% of 9 months of age, and voluntary cooperation occurs between 12
children in the United States were not trained by 33 months of age, and 15 months of age. In assessing the neurologic development
but by 1975 this figure had increased to 42%. Currently, approxi- of children, walking is viewed as 1 of the milestones that indi-
mately 25% of typically developing US children are daytime toilet cate motor readiness for toilet training. Appropriate motor skills,
trained at 24 months of age and 98% by 36 months of age. including getting to the bathroom, being able to remove clothing,
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and sitting on the toilet, are also key skills required for success- Stool-related accidents may be associated with chronic consti-
ful toilet training. pation and overflow incontinence or with congenital megacolon (ie,
Toilet training depends on physiologic and psychological readi­ Hirschsprung disease; see Chapter 56). Stool toileting refusal occurs
ness. Cognitive development is assessed by a child’s ability to fol- when a child is trained to urinate in the toilet but refuses to defe-
low certain instructions and understand what the potty is used for. cate in the toilet for at least 1 month. Although many parents per-
Two years of age has been suggested as the appropriate age to ini- ceive stool toileting refusal as insignificant, it is often associated with
tiate toilet training in most children given that the developmental developing encopresis, constipation, painful bowel movements, and
and physiologic skills necessary for successful toilet training begin delayed completion of toilet training. If a child has persistent con-
maturing at this time. Toilet training usually takes 2 weeks to stipation, the child may develop megacolon and may not be able
2 months to master. Achieving nighttime continence is often separate to sense a full rectum, thereby causing overflow of loose stools. A
from daytime continence. Although opinions about nighttime wet- complete history and physical examination are required to differ-
ting are culturally dependent, it is considered normal in the United entiate functional constipation from organic causes. The physician
States up to 6 years of age. should recognize and address functional constipation early to avoid
A child’s temperament can also affect the success of toilet train- acquired megacolon, because it takes 3 to 12 months to treat mega-
ing. Children who struggle with inflexibility, are less persistent, or colon caused by chronic constipation. Children who prefer to stand
have a more negative mood often experience delays in toilet train- in a corner to defecate should be commended for recognizing their
ing. Unlike physiologic or psychological readiness, temperament is physiologic urge. However, parents should be aware that children
not likely to change after a 2-month delay in training; understand- who hide while passing stool in their diaper are more likely to exhibit
ing a child’s temperament can better assist parents in supporting stool toileting refusal and be constipated. Successful management of
their child through the process of toilet training. In addition to the constipation may decrease the incidence of toileting refusal.
child’s temperament, the child’s emotional readiness is influenced
by parental attitudes and parent-child interactions.
Evaluation
Differential Diagnosis History
The differential diagnosis of toilet training difficulties focuses on Typically developing toddlers should be assessed for their physio-
factors that contribute to a delay in acquisition of skills. The phy- logic and psychological readiness to initiate toilet training, as well as
sician should look for associated symptoms, such as dysuria, a for any underlying medical conditions that may affect their ability
weak urinary stream, constantly wet underwear, or fecal soiling to learn toileting skills at the customary age. The physician should
when assessing a child who continues to manifest signs of urinary provide anticipatory guidance to parents about toileting readiness.
or stool incontinence. Additionally, it is important to determine Affirmative answers should be obtained to the following 3 questions:
if children are essentially toilet trained but are having intermit- 1. Does the child exhibit bladder control as evidenced by periods
tent accidents. of dryness that last up to 2 hours and facial expressions that
Dysfunctional voiding involves an abnormal voiding pattern show the child’s physiologic response to the elimination process?
stemming from a problem with the bladder filling or emptying. Such 2. Does the child have the motor skills necessary to get around?
voiding is characterized by urine leakage, an increase in urgency, This essentially involves the child’s ability to walk and remove
and an increase in frequency, and it often results in frequent uri- their clothing.
nary tract infections (UTIs). The most common cause of isolated 3. Does the child have the cognitive ability to understand the task
daytime wetting in previously trained children is UTI (see Chapter at hand?
112). Although UTI is not associated with age at the onset of toilet Cognitive ability can be assessed by giving a child 10 one-step tasks
training, earlier toilet training is associated with later onset of UTIs. to determine whether the child can complete at least 8 of the 10 tasks
No association exists between toilet training methods and dysfunc- (Box 48.1). The ability to carry out these tasks does not ensure a willing-
tional voiding. Chemical urethritis may also be associated with uri- ness to be toilet trained, however. When language readiness is apparent
nary incontinence. Stress incontinence, which has also been called (ie, use of 2-word phrases and 2-step commands), training can com-
“giggle incontinence,” may result in wetting. Urgency incontinence mence. In addition to language readiness, understanding of the cause
occurs when children delay going to the bathroom and then are and effect of toileting, desiring independence, and having sufficient
unable to hold urine any longer. Some children have ectopic ure- motor skills and body awareness are helpful for successful training.
ters, which can empty into the lower portion of the bladder, vagina, Stress in the home may negatively affect a toddler’s ability to mas-
or urethra and cause a constant dribble of urine. Labial fusion with ter the task of toilet training. The physician might counsel a family
vaginal reflux of urine may also be associated with daytime wetting. to delay toilet training if the family has moved recently, the birth of
Urine pools behind the fused labia or labia that do not separate suf- a new baby is expected, or a major family crisis has occurred, such
ficiently to allow natural egress during voiding, and when the child as a death or serious illness.
stands up the urine exits. The child with a neurogenic bladder may The child who has had difficulties with toilet training must
also have symptoms of daytime enuresis. undergo a similar assessment.

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 Chapter 48: Toilet Training 331

When toilet training the typically developing child, a parent

Box 48.1. Requests or Imperatives Used to Help
should be advised to take the approach outlined in Box 48.2.
Assess Toilet Training Readiness
Additional suggestions involve allowing children to have
ww Bring me the ball. their undergarments off and keeping the potty chair near them
ww Go to the door. during play.
ww Sit on the chair. Children who use the potty successfully should be rewarded.
ww Pick up the doll. Rewards can be in the form of verbal comments such as “Mommy
ww Open the door. is so proud of you,” hand clapping, or the use of star charts that can
ww Give the pen to your mom. be redeemed for rewards. Children can be consulted about what
ww Put the ball on the table. rewards they like. The AAP recommends using praise rather than
ww Put the doll on the floor. treats for reinforcement. Punishment should not be used, particu-
ww Take off your shoes. larly physical punishment. One report indicates that 15% of parents
ww Open the book. of clinic patients believe that spanking their children for accidents
is acceptable. Parental disapproval of accidents can be articulated;
however, parents should understand that accidents may continue
to occur for months. Regression, that is, the state of being no lon-
Physical Examination
ger willing or able to execute toilet training skills, may also occur
A physical examination should be performed to rule out under- with stressors, such as a recent move, a new sibling, or divorce.
lying problems, such as spina bifida occulta, which may be asso- Consistent parental reminders and a consistent schedule can assist
ciated with a neurogenic bladder. A neurologic examination may with eliminating regression. It is important to normalize regression
reveal nerve damage impeding muscles from relaxing or tight- and encourage parents to focus on regression as a temporary step
ening at the right time. A careful examination of the genitalia is on the way to being successfully toilet trained.
important in the child with urinary incontinence to determine The second method, described by Azrin and Foxx, is parent-
if conditions such as labial fusion, meatal stenosis, or posterior oriented and uses intense operant conditioning methods and a
urethral valves are evident. In the child with a problem related to potty doll to assist with learning. The 6 components of the method
passage of stool, the physician should check for an abdomen with include increasing the amount of fluids consumed; regularly sched-
feces-filled intestines, which is a sign of obstipation. Additionally, uled times for toileting, starting with 15-minute intervals; posi-
a rectal examination should be considered to determine the pres- tive reinforcement for asking about, approaching, or sitting on
ence of hard or impacted stools or any other abnormalities in the the potty chair; lowering/raising pants and successful urination
anal area. or defecation in the potty chair; time-out from positive reinforce-
ment/cleanliness training following accidents; and teaching the
Laboratory Tests
child to differentiate between wet and dry pants, checking for dry
Although a laboratory assessment is not indicated in the normal tod- pants every 5 minutes. Although this method has been effective
dler who is being toilet trained, diagnostic studies may be appropri- in training children, including those individuals with intellectual
ate in the child who is having problems with training. In the older disabilities, it is not recommended for training typically develop-
child, urinalysis may show evidence of a UTI. ing children. A child with autism spectrum disorder who does not
understand the social reward system recommended by the AAP
Management may benefit from the intense conditioning methods described by
The physician should help parents understand the appropriate Azrin and Foxx.
approach to the toilet training process. Unfortunately, most par- Two additional conditioning methods are available that are less
ents do not obtain the necessary advice from physicians. In 1 report, commonly used in North America. The first is assisted newborn
no parents attending a clinic and only 7% of parents in a private toilet training, which begins when the newborn is 2 to 3 weeks
practice received advice about toilet training from their physician. of age. The baby is placed over a toilet after feeding or if showing
Therefore, it is important for the physician to initiate the discussion signs of elimination and is rewarded with food or affection for
early enough to prevent the development of any problems. voiding or passing stool. The second method focuses on elimina-
Two main contrasting styles of toilet training exist. The first tion communication and begins at birth. The parent learns baby’s
method, which is endorsed by the American Academy of Pediatrics body language, noises, and elimination patterns. The parent also
(AAP), is a child-oriented approach that stresses the child’s physio­ makes noises, such as the sound of running water, to associate
logic and psychological readiness to toilet train. The 3 versions of with voiding while the newborn is placed in positions to facilitate
this approach are the Brazelton method, the AAP guidelines, and elimination. Interest in these conditioning methods has increased
the Dr. Spock method, all of which use the same components with in the past few years.
only minor variations. All involve gradual training and emphasize A child may use training pants, which are thickened underwear,
the use of rewards rather than punishments. or disposable diaper-like underwear, rather than diapers. Standard

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 332 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Box 48.2. Toilet Training Approach for the Typically Developing Child

1. Teach children the appropriate vocabulary related to the toilet training process. This could include words such as “pee,”“poop,”“urine,”“stool,”“dry,”“wet,”
“clean,”“messy,” and “potty.”
2. Tell children what the purpose of the potty is. Placing the contents of a soiled diaper into the toilet can educate them on the purpose of the potty. Generally,
a child potty chair should be purchased. The potty chair has several advantages over the toilet. Parents can encourage children to decorate the potty and put
their names on it. The potty can be kept in a place where children spend much of their time, not necessarily in the bathroom. Children can sit on the potty and
have their feet on the floor, which is more physiologically sound and gives them a greater sense of security. Parents should suggest that children stand fully
clothed in the bathroom as an initial step in encouraging their use of a potty. Children should be allowed to sit on the potty with their clothes on for approx-
imately 1 week before the process of toilet training begins. Next, children should sit on the potty without their undergarments, but no attempt should be
made to catch stool or urine. Boys should learn to urinate in the seated position, because they may otherwise resist sitting for defecation.
When away from home, the potty chair should be packed to maintain the established routine. It is important that all individuals caring for the child
(eg, grandparents, babysitters) understand the parent’s or parents’ plans for toilet training.
Toilet adapter rings can be used if the family is resistant to using a potty chair or if the transition from a potty chair to the toilet is likely to be stressful, as
is true for many children with autism spectrum disorder. Adapter rings fit directly onto the toilet and do not require emptying, as do separate potty chairs.
They require that the toddler climb up on the toilet, and they need to be removed for others to use the toilet. A step stool should be used to aid in climbing
onto the toilet and to provide more leverage while defecating.
3. Encourage cleanliness and dryness by changing children frequently. Parents should ask their children whether they need to be changed using the appropri-
ate vocabulary. This phase is important to continue as the toilet training process proceeds. Some parents mistakenly do not change their soiled children as a
means of punishing them for having accidents. This gives children a confusing message about the need for cleanliness.
4. Explain to children the connection between dry pants and going to the potty. Children should understand that dry pants feel good and that they can keep
their pants dry by going to the potty.
5. Help children understand the physiologic signals for using the toilet. Parents can facilitate this by observing children’s behavior around the time of elimina-
tion and making comments such as, “When you jump up and down like that, Mommy knows you have to go to the bathroom.”
6. Children must have the physiologic ability to postpone the “urge to go.” This usually occurs when children are capable of delaying voiding for at least 2 hours.
Parents then can initiate toilet training by taking children to the bathroom at 2-hour intervals. Additionally, children should sit on the toilet immediately after
naps and 20 minutes after meals. Children should not be left on the toilet for more than 5 minutes and should be permitted to get up if they want. While sit-
ting on the potty, they can be entertained with reading a story or playing games. It is helpful to have designated toys or books enjoyed only when the child is
sitting on the potty. Parents are encouraged to rotate the toys so toilet training continues to be interesting to the child.

underwear is promoted as advantageous because it feels different raising their hands or receiving other reminders. Child care offers
from diapers and encourages the use of the potty. Using a bigger the advantage of peer modeling and peer pressure during the toilet
size or snipping the waistband facilitates children’s ability to remove training process. Potty chairs should not be used in child care set-
their underpants and can be recommended. tings because of the risk of infectious diseases.
Some children seem fearful of certain aspects of the toilet train- Children with special needs generally encounter more obstacles
ing process, including fear of falling into the toilet, which can be cir- when mastering toileting. Communication delays, less developed
cumvented with the use of a potty or toilet adapter rings, and fear motor skills, sensitivity to stimulation, and preference for routine
of the noise of the flush. Allowing a child to flush the toilet without are just a few of the additional challenges that make it difficult to
using it may dispel the fear. Toilet phobias can cause children to hold ascertain whether the child is ready to toilet train. Although incon-
their urine until the last moment, resulting in wetting, or cause chil- tinence was once thought to be inevitable for children with special
dren to hurry and not fully empty their bladder, resulting in pos- needs, it is important for parents to understand that continence can
sible infections. Some children become fascinated with flushing or be achieved but that expectations need to modified (ie, it may take
unrolling toilet paper, and parents should discourage children from until age 5 years to achieve, and standard toilet training methods
wasting water or paper. rarely are successful).
Modeling is 1 of the major components of toilet training children. Medications have a limited role in toilet training. Although some
Children should be allowed to enter the bathroom with parents and physicians recommend the use of drugs to increase bladder capacity,
even sit on the potty chair as a parent sits on the toilet. Some children such drugs should not be used because they do not assist children
who are quite strong-willed and independent, coupled with perfec- with the toilet training process. However, children who are consti-
tionist parents, may have problems with toilet training. pated may require stool softeners, such as mineral oil, polyethylene
Special considerations must be made for children attending child glycol solution, or magnesium citrate, or the addition of fiber bulk
care. Children should have open bathroom privileges; that is, they to their diet as well as increased fluid consumption to facilitate the
should be permitted to leave the room to go to the bathroom without passing of stool. The child who seems to have particularly challenging

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 Chapter 48: Toilet Training 333

toilet training problems should be seen by the physician on a weekly Colombo JM, Wassom MC, Rosen JM. Constipation and encopresis in child-
or biweekly basis until the child exhibits improvement. hood. Pediatr Rev. 2015;36(9):392–402 PMID: 26330473 https://doi.org/10.1542/
Parents can also be referred to the many books on toilet training, pir.36-9-392

particularly if problems arise. Many books are written for children Klassen TP, Kiddoo D, Lang ME, et al. The effectiveness of different methods of
to help them understand their body and the elimination process. toilet training for bowel and bladder control. Evid Rep Technol Assess (Full Rep).
2006;(147):1–57 PMID: 17764212
Videos are also available to help children with toilet training. A child
may benefit from the opportunity to practice with dolls designed to Mota DM, Barros AJ. Toilet training: methods, parental expectations and asso-
ciated dysfunctions. J Pediatr (Rio J). 2008;84(1):9–17 PMID: 18264618 https://
wet or poop after being fed.
doi.org/10.2223/JPED.1752
Taubman B, Blum NJ, Nemeth N. Children who hide while defecating before
Prevention they have completed toilet training: a prospective study. Arch Pediatr Adolesc
If a child demonstrates resistance to toilet training, the process Med. 2003;157(12):1190–1192 PMID: 14662572 https://doi.org/10.1001/
should be delayed for 1 to 2 months. The child who learns how to archpedi.157.12.1190
withhold needs additional time to learn how to relax the sphincter Vermandel A, Van Kampen M, Van Gorp C, Wyndaele JJ. How to toilet train
when sitting on a potty. It is important for parents to avoid an aggres- healthy children? a review of the literature. Neurourol Urodyn. 2008;27(3):
siveness/resistance struggle, because this may become the source of 162–166 PMID: 17661380 https://doi.org/10.1002/nau.20490
future bowel problems, including constipation. The child who is reg- Weissman L. Toilet training: how to foster success and manage pitfalls. Consultant
ular, particularly the individual who has a bowel movement at the for Pediatricians. 2012;11(10):307–315
same time every day, is more easily toilet trained. Creating a reg-
Parents and Children
ularly scheduled toilet sitting session can assist with reducing the
incidence of or stopping withholding. American Academy of Pediatrics. Guide to Toilet Training. 2nd ed. Wolraich ML,
ed. Elk Grove Village, IL: American Academy of Pediatrics; 2016

Prognosis Azrin NH, Foxx RM. Toilet Training in Less Than a Day: A Tested Method for
Teaching Your Child Quickly and Happily! New York, NY: Gallery Books; 2019
All typically developing children are eventually toilet trained. The Bennett HJ. It Hurts When I Poop! A Story for Children Who Are Scared to Use
age at which this occurs varies and is significant only if it restricts a the Potty. Washington, DC: Magination Press; 2007
child from participating in school. Berger S. Princess Potty. New York, NY: Cartwheel Books; 2010
Berry R. It’s Potty Time for Boys. Carlsbad, CA: Smart Kids Publishing; 2011
Foote T. My Potty Reward Stickers for Boys: 126 Boy Potty Training Stickers and
CASE RESOLUTION Chart to Motivate Toilet Training. New York, NY: Tracy Trends; 2006
The mother in the case history should be advised that this is a good time to ini-
Foote T. My Potty Reward Stickers for Girls: 126 Girl Potty Training Stickers and
tiate the toilet training process. She should be told that boys, as a group, are
Chart to Motivate Toilet Training. New York, NY: Tracy Trends; 2006
successfully toilet trained at a later age than girls. Her son can be assessed to
determine whether he can follow at least 8 of 10 instructions (Box 48.1). If the Frankel A. Once Upon a Potty—Boy. Richmond Hill, ON: Firefly Books; 2014
boy can do so, the mother should be given the stepwise approach to initiating Frankel A. Once Upon a Potty—Girl. Richmond Hill, ON: Firefly Books; 2014
the toilet training process. The mother should be informed it may take months
Gomi T. Everyone Poops. New York, NY: Scholastic; 1993
to years to achieve nighttime continence after daytime continence is achieved.
Hochman D, Kennison R. The Potty Train. New York, NY: Simon & Schuster; 2008
Katz K. A Potty for Me! New York, NY: Little Simon; 2005
Selected References Mack A. Toilet Learning: The Picture Book Technique for Children and Parents.
Boston, MA: Little, Brown and Company; 1978
Physicians
Mayer G, Mayer M. The New Potty. New York, NY: Random House Books for
Blum NJ, Taubman B, Nemeth N. Relationship between age at initiation Young Readers; 2003
of toilet training and duration of training: a prospective study. Pediatrics.
Miller V. On Your Potty. Cambridge, MA: Candlewick Press; 2000
2003;111(4):810–814 PMID: 12671117 https://doi.org/10.1542/peds.111.4.810
Patricelli L. Potty. Somerville, MA: Candlewick Press; 2010
Chen JJ, Ahn HJ, Steinhardt GF. Is age at toilet training associated with the pres-
ence of vesicoureteral reflux or the occurrence of urinary tract infection? J Urol. Pinnington A. Big Girls Use the Potty! New York, NY: DK Publishing; 2008
2009;182(1):268–271 PMID: 19450811 https://doi.org/10.1016/j.juro.2009.02.137 Rogers F. Going to the Potty. New York, NY: Puffin Books; 1997
Choby BA, George S. Toilet training. Am Fam Physician. 2008;78(9):1059–1064 Smith DC, McClure D. Monkey Learns to Potty. Knoxville, TN: PottyMD, LLC; 2015
PMID: 19007052
Colaco M, Johnson K, Schneider D, Barone J. Toilet training method is not related
to dysfunctional voiding. Clin Pediatr (Phila). 2013;52(1):49–53 PMID: 23117239
https://doi.org/10.1177/0009922812464042

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 CHAPTER 49

Crying and Colic

Geeta Grover, MD, FAAP

CASE STUDY
The parents of a 2-week-old neonate bring their son to Questions
the emergency department because he has been cry- 1. What is the normal crying pattern in newborns and
ing persistently for the past 4 hours. He has no history of young infants?
fever, vomiting, diarrhea, upper respiratory tract infec- 2. What is colic?
tion, or change in feeding. The newborn is breastfed. 3. What conditions are associated with prolonged
On physical examination, the neonate appears well crying in newborns and young infants?
developed and well nourished. His weight is 3.37 kg 4. What are key factors in the history of crying
(7.4 lb), which is 0.20 kg (0.4 lb) more than when he was newborns and infants?
born. Although he is fussy and crying, he is afebrile with 5. What tests or studies, if any, are indicated in crying
normal vital signs. The remainder of the physical exami- newborns and infants?
nation is within normal limits. 6. What are a few of the management strategies that
can be used by parents to soothe their crying or
colicky newborns and infants?

Crying is an important method of communication between babies

and caregivers; it is nonspecific, however, and many stimuli (eg,
hunger, fatigue, pain) can provoke the same response. Parents
report that they can discriminate among various types of cries
in their babies. Crying can be divided into 3 categories: normal
or physiologic crying, excessive crying secondary to distress (eg,
hunger) or disease, and excessive crying without an apparent cause
(eg, colic).
The difference between normal and excessive crying may be more
qualitative than quantitative. Some investigators have used the mne-
monic “PURPLE” to characterize crying during early infancy focus-
ing on the qualities that make the crying particularly frustrating
to caregivers: P, peak pattern (increases weekly until 2 months of
age); U, unexpected bouts of crying; R, resistance to soothing mea-
sures; P, pain-like facial grimacing; L, long periods of crying; and
E, evening clustering. Deciding whether crying is excessive varies
based on parental expectations and thresholds. Expressed paren-
tal concern about extreme crying or fussiness requires attention.
Figure 49.1. Illustration of a baby exhibiting characteristic physical signs of
If parents complain that newborns and infants cry inconsolably or
colic, such as crying, flexed elbows, and clenched fists.
continuously as well as excessively, the crying may have an under-
lying organic etiology. Crying with no organic etiology or definable
and infants may appear to be miserable during an episode of
cause is often attributed to colic.
colic, they are otherwise healthy, eat well, and demonstrate good
Colic is a poorly understood, benign, self-limited condition
weight gain.
in which healthy infants experience paroxysms of inconsol-
able crying. It manifests as unexplained crying in newborns
and infants that usually occurs in the late afternoon or evening. Epidemiology
During an episode of colic, babies cry and may draw the knees Qualitatively, excessive crying is any amount of crying that concerns
up to the chest or rigidly stiffen the legs, flex the elbows, clench or worries parents. Quantitatively, definitions of excessive crying have
the fists, and turn red (Figure 49.1). Although neonates been based on the results of Brazelton’s study of normal newborns

335

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 336 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

and infants. Excessive crying begins at 2 weeks of age (median daily Differential Diagnosis
crying time, approximately 2 hours per day), peaks at 6 weeks of
An acute episode of excessive crying may be secondary to disease
age (median daily crying time, approximately 3 hours per day), and
(eg, fever, otitis media). An organic etiology should be suspected
decreases to less than 1 hour per day by 12 weeks of age. More
in newborns and infants who present with inconsolable crying of
crying occurs during the evening hours, especially between ages
acute onset. Box 49.1 lists the most common causes of acute, unex-
3 and 6 weeks.
plained, excessive crying in newborns and infants. Some conditions
Although many neonates and infants exhibit a relatively
similar pattern of fussiness that peaks at approximately 6 weeks
of age, those with colic tend to be inconsolable for longer periods Box 49.1. Common Causes of Acute, Unexplained,
and cry with greater intensity. Colic affects 10% to 20% of new- Excessive Crying in Newborns and Infants
borns and infants younger than 3 months. Colic affects both
males and females equally and has no correlation with gesta- Idiopathica
tional age (eg, full-term vs preterm), type of feeding (eg, breast ww Colica
vs bottle), socioeconomic status, or season. Postpartum depres- Infectious
sion and abusive head trauma have been associated with colic ww Otitis media
and the stressfulness of infant crying. Colic usually begins at 2 to ww Urinary tract infection
3 weeks of age, peaks at 6 to 8 weeks of age, and resolves by 3 to ww Stomatitis
4 months of age. In general, symptoms of colic last for more than ww Meningitis
3 hours per day, for more than 3 days per week, and for more than Gastrointestinala
3 weeks’ duration (ie, rule of 3s). ww Constipation
ww Anal fissure
Clinical Presentation ww Gaseous distention
Colicky babies are otherwise healthy newborns and infants ww Peristalsis problems
younger than 3 months who cry or fuss inconsolably for extended ww Reflux
periods, usually during the afternoon or evening. Typically, the ww Pyloric stenosis
crying resolves within a few hours. ww Intussusception
Trauma
Pathophysiology ww Corneal abrasion
Crying is a complex vocalization that changes during the first year ww Foreign body in the eye
after birth as babies develop. In the first few weeks after birth, crying ww Hair tourniquet syndrome
is a signal that newborns are experiencing a disturbance in homeo- Behaviorala
static regulation (eg, hunger, discomfort). As babies mature and ww Overstimulation
begin to differentiate internal from external stimuli, crying may ww Persistent night awakening
also be an indication of too little or too much environmental stim- Drug Reactions
ulation. During the second half of the first year, as infants mature ww Immunization reactions (previously common with diphtheria-tetanus-
neurologically and gain voluntary control over vocalizations, cry- pertussis vaccine)
ing can be an expression of different affects (eg, frustration, fear). ww Neonatal drug withdrawal (eg, narcotics)
Various explanations for the etiology of colic have been proposed,
Child Abuse
but the cause remains unknown. Some authorities believe that colic
ww Long bone fracture
may not be a pathologic entity but instead may be simply an extreme
ww Retinal hemorrhage
variant of normal crying. Proposed causes of colic include cow’s milk
ww Intracranial hemorrhage
protein or lactose intolerance, abnormal intestinal peristalsis, alter-
ations in fecal microflora, gastrointestinal immaturity resulting in Hematologica
incomplete absorption of carbohydrates and resultant excessive gas ww Sickle cell crisis
production, increased serotonin secretion, poor feeding technique, Genitourinary
and maternal smoking or nicotine replacement therapy. Recent stud- ww Incarcerated hernia
ies have demonstrated increased levels of fecal calprotectin, a marker ww Testicular torsion
of colonic inflammation, in infants with colic. Others have proposed Cardiovascular
that colic is caused by problems in the interaction between babies and ww Arrhythmia (eg, supraventricular tachycardia)
their environment, specifically their parents. This interactional theory ww Congestive heart failure
requires not only excessive crying on the part of the newborn or infant ww Anomalous left coronary arterya
but also an inability of the parents to soothe the crying baby. More
than 1 of these factors may contribute to the pathogenesis of colic. a
May present as acute or recurrent episodes of excessive crying.

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 Chapter 49: Crying and Colic 337

occur in a more chronic or recurrent pattern, particularly if the con- may present with crying. Even when the initial history and physi-
dition is not treated. cal examination are nondiagnostic, a serious underlying condition
The differential diagnosis of newborns and infants who expe- (eg, intracranial hemorrhage, drug ingestion) should be suspected
rience recurrent episodes of excessive crying or recurrent night in babies who persist in crying inconsolably. Such newborns and
awakening associated with crying is focused more on behavior and infants may warrant an extended period of observation or a more
temperament. Colic, neonatal abstinence syndrome, or difficult extensive workup that includes laboratory assessment.
temperament (eg, extreme fussiness) may cause recurrent crying.
Recurrent night awakening and difficult temperament are discussed Imaging Studies
in Chapters 30 and 51, respectively. Radiographic studies may be necessary in some situations (eg, long
bone radiographs for newborns and infants with long bone ten-
Evaluation derness on palpation, cases of suspected child abuse). Computed
A thorough patient history and physical examination usually provide tomography of the head should be performed on infants with ret-
clues to the diagnosis in instances of acute onset of crying. inal hemorrhage.

History Management
The focus of the history should be on determining the presence Management of excessive crying is determined based on identifica-
of any associated symptoms. Additionally, circumstances sur- tion of the cause. Underlying organic conditions (eg, urinary tract
rounding the crying (eg, occurrence during day or night) should be infection, fractures) should be managed. The cornerstone of man-
ascertained (Box 49.2). agement of colic is parental reassurance and support, practical sug-
gestions of feeding and handling techniques (eg, cuddling, holding),
Physical Examination
and education about the benign, self-limited nature of colic.
A thorough physical examination is required for accurate diag- If symptoms persist and parents desire additional management,
nosis. Red flags in the evaluation of crying infants include fever, further treatment is individualized based on patient history, physi-
lethargy, and abdominal tenderness or tenseness. The following cal examination, and family characteristics. Common management
aspects of the examination warrant particular attention: techniques are listed in Box 49.3. First-line interventions that may
1. Careful inspection of the skin after all clothing has been removed be considered include changes in feeding techniques and/or sooth-
to look for any suspicious bruises or marks ing techniques, both of which address some of the potential etiolo-
2. Palpation of all long bones to detect occult fractures gies of colic, including swallowed air and overstimulation. Currently,
3. Examination of all digits and the penis to check for hair tourni- data on probiotic supplementation, dietary changes, various medi-
quets (ie, single strands of hair wrapped around digits or the penis) cations, and complementary and alternative medical therapies are
4. Examination of the retina for retinal hemorrhages, which may insufficient for routine recommendation; however, such options may
be indicative of prior head injury be considered on an individual basis after a discussion of the poten-
5. Eversion of the eyelids to check for ocular foreign bodies tial risks and benefits. Changes in sensory input (eg, soothing sounds
6. Fluorescein staining of the cornea to look for corneal abrasion. or motions) may resolve crying and soothe colicky babies. Soothing
techniques include using a pacifier, swaddling, shushing, swinging,
Laboratory Tests
rocking, rubbing the abdomen, holding the infant or placing the
With the exception of urinalysis, most screening laboratory tests infant in a front carrier, providing white noise, and giving a warm
likely are not useful unless indicated by the patient history and
physical examination. Afebrile infants with urinary tract infection
Box 49.3. Management of Colic
ww Parental reassurance
Box 49.2. What to Ask
ww Parental education
The Newborn or Infant With Crying or Colic ww Alteration in techniques of newborn/infant feeding and handling
ww Is this the first time the newborn/infant has cried inconsolably, or does —— Increased carrying
this happen on a recurring basis? —— Responding quickly to crying
ww Has the newborn/infant had a fever? ww Alteration of sensory input to the newborn/infant
ww Does the newborn/infant have any cold symptoms, vomiting, or ww Prevention of swallowed air from passing through the pylorus
diarrhea? ww Probiotics
ww Is the newborn/infant having any difficulty feeding? Is the newborn/ ww Dietary modifications
infant formula-fed or breastfed? ww Medication
ww Has the newborn/infant had a recent fall or accident? ww Complementary and alternative medicine therapies (after discussion of
ww What do you do when your newborn/infant cries? potential risks and benefits)

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 338 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

bath. Parents should be encouraged to experiment with various tech- newborns and infants have an increased likelihood of stopping
niques, because the success of any given method may vary from 1 breastfeeding early and also are at increased risk for postpar-
episode of colic to the next. Changes in feeding techniques to min- tum depression. Excessively fussy babies are at increased risk for
imize the passage of swallowed air through the pylorus also can be child abuse.
useful. Such techniques include feeding in an upright position and
limiting the period of sucking at the breast or bottle to approximately
10 minutes, after which time greater amounts of air are swallowed
relative to the amount of milk or formula ingested. Some newborns CASE RESOLUTION
and infants eat very fast and swallow a lot of air. Burping these babies The newborn is experiencing an acute episode of unexplained crying. Despite
a normal physical examination, he was observed for 1 hour in the emergency
every 5 to 10 minutes during feeds may help alleviate discomfort
department because his crying persisted. A septic workup was done, which
caused by excessive air swallowing (ie, aerophagia). Decreasing the resulted in the diagnosis of a urinary tract infection.
size of the opening of a nipple for a bottle-fed baby may reduce the
amount of air swallowing.
Data from several recent clinical trials indicate that in breastfed
infants, use of the probiotic Lactobacillus reuteri DSM 17938 may Selected References
decrease the duration of crying. A similar decrease in crying time
has not been demonstrated in formula-fed infants given L reuteri. Barr RG, Rivara FP, Barr M, et al. Effectiveness of educational materials
Data on dietary changes, such as the use of hypoallergenic diets designed to change knowledge and behaviors regarding crying and shaken-
baby syndrome in mothers of newborns: a randomized, controlled trial.
by breastfeeding mothers, are inconclusive but suggest that such
Pediatrics. 2009;123(3):972–980 PMID: 19255028 https://doi.org/10.1542/
changes may have some therapeutic benefits. Similarly, the use of peds.2008-0908
partially, extensively, or completely hydrolyzed infant formulas also
Brazelton TB. Crying in infancy. Pediatrics. 1962;29:579–588 PMID: 13872677
seems to have beneficial effects on the symptoms of colic. The switch
Chau K, Lau E, Greenberg S, et al. Probiotics for infantile colic: a randomized,
to soy-based formulas is generally not recommended because soy
double-blind, placebo-controlled trial investigating Lactobacillus reuteri DSM
can be an allergen. Generally, these dietary modifications should 17938. J Pediatr. 2015;166(1):74–78 PMID: 25444531 https://doi.org/10.1016/j.
be reserved for newborns and infants with additional symptoms of jpeds.2014.09.020
allergy (eg, wheezing, rash) or intolerance (eg, vomiting, diarrhea, Cohen GM, Albertini LW. Colic. Pediatr Rev. 2012;33(7):332–333 PMID:
hematochezia, weight loss). 22753793 https://doi.org/10.1542/pir.33-7-332
Various medications, including anticholinergic agents, motility- Freedman SB, Al-Harthy N, Thull-Freedman J. The crying infant: diagnostic test-
enhancing agents, proton-pump inhibitors, barbiturates, laxatives, ing and frequency of serious underlying disease. Pediatrics. 2009;123(3):841–
and antiflatulence agents, have limited success and are best avoided. 848 PMID: 19255012 https://doi.org/10.1542/peds.2008-0113
Currently, antiflatulence agents (eg, simethicone) are prescribed most Herman M, Le A. The crying infant. Emerg Med Clin North Am. 2007;25(4):1137–
commonly. Despite lack of scientific evidence to support their effi- 1159, vii PMID: 17950139 https://doi.org/10.1016/j.emc.2007.07.008
cacy, anecdotal reports from parents indicate that they are effective. Iacovou M, Ralston RA, Muir J, Walker KZ, Truby H. Dietary management of
Complementary and alternative medical therapies may be con- infantile colic: a systematic review. Matern Child Health J. 2012;16(6):1319–1331
sidered. Limited amounts (eg, 1–2 oz per day) of herbal remedies PMID: 21710185 https://doi.org/10.1007/s10995-011-0842-5
(eg, chamomile tea) can be administered if parents report satisfac- Johnson JD, Cocker K, Chang E. Infantile colic: recognition and treatment. Am
tion and no evidence exists of adverse effects. Evidence is insufficient Fam Physician. 2015;92(7):577–582 PMID: 26447441
to support the recommendation of physical therapies such as chiro- Perry R, Hunt K, Ernst E. Nutritional supplements and other complemen-
practic or osteopathic manipulation, infant massage, or acupuncture. tary medicines for infantile colic: a systematic review. Pediatrics. 2011;127(4):
However, they may be considered on an individual basis after a dis- 720–733 PMID: 21444591 https://doi.org/10.1542/peds.2010-2098
cussion of the potential risks and benefits with the parents. Finally, Radesky JS, Zuckerman B, Silverstein M, et al. Inconsolable infant crying
physicians should encourage parents to respond to their baby’s cries and maternal postpartum depressive symptoms. Pediatrics. 2013;131(6):
quickly and carry the baby as much as possible (eg, at least 3–4 hours e1857–e1864 PMID: 23650295 https://doi.org/10.1542/peds.2012-3316
per day). Parents should be advised that it is not possible to “spoil” Savino F, De Marco A, Ceratto S, Mostert M. Fecal calprotectin during
babies younger than 4 months and that the baby’s behavior may treatment of severe infantile colic with Lactobacillus reuteri DSM 17938:
a randomized, double-blind, placebo-controlled trial. Pediatrics. 2015;135
improve with increased parental responsiveness.
(suppl 1):S5–S6
Waddell L. Management of infantile colic: an update. J Fam Health Care.
Prognosis 2013;23(3):17–22 PMID: 23724767
The physician should understand and should reassure the par- Wessel MA, Cobb JC, Jackson EB, Harris GS Jr, Detwiler AC. Paroxysmal
ent or parents that the natural history of persistent crying dur- fussing in infancy, sometimes called colic. Pediatrics. 1954;14(5):421–435
ing infancy is one of resolution over time. Mothers of colicky PMID: 13214956

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 CHAPTER 50

Discipline
Carol D. Berkowitz, MD, FAAP

CASE STUDY
A 3-year-old boy is being threatened with expulsion Questions
from preschool because he is biting the other children. 1. What is the definition of discipline?
His mother states that he is very active and aggressive 2. What are the 3 key components of discipline?
toward other children. In addition, his language devel- 3. What is meant by parental monitoring?
opment is delayed. She is at her wits’ end about what to 4. What are 4 different parenting styles?
do. The birth history is normal, and the mother denies 5. What strategies can parents use to discipline
the use of drugs or cigarettes, but she drank socially children?
before she realized she was pregnant. The medical and 6. What are the guidelines for using time-out?
family histories are noncontributory, and the physical 7. What is the relationship between corporal punish-
examination is normal. ment and child abuse?

Discipline can be defined as an educational process in which coupled with parental discipline to promote desirable behaviors and
children learn how to behave in a socially acceptable manner. The eliminate undesirable ones.
word is derived from disciplinare, meaning to teach or instruct. It
involves a complex set of interactions of attitudes, models, instruc- Anticipatory Guidance: Talking
tions, rewards, and punishments. Discipline is not synonymous With Parents About Discipline
with punishment, which denotes a negative consequence to one’s
Practitioners can assist parents by giving them guidance about
actions. The goal of effective discipline is to help children gain self-
appropriate childhood discipline related to routine and problem
control and respect for others and to learn behavior that is appropri-
development and to counsel about the scope of monitoring. The age
ate for given situations. It also serves to ensure a child’s safety in the
and temperament of the child are important factors to consider. In
environment. Proactive discipline is action taken by parents to
addition, pediatricians can educate parents about corporal punish-
encourage good behavior, and reactive discipline is parental action
ment, especially as the major method of discipline. Pediatricians
following misbehavior. To be effective, child discipline must have
also have a role in advising against corporal punishment in schools.
3 components: a learning environment with a positive, supportive
While most states have banned corporal punishment in the school
parent-child relationship; a refined strategy for teaching and rein-
setting, 19 states, mostly in the south, still permit it.
forcing desired behaviors; and a defined strategy for decreasing or
extinguishing undesired behaviors. Children thrive in a support-
ive environment in which they are praised for socially appropri- Parenting Styles
ate behavior and are able to participate in the responsibilities and Diana Baumrind is credited with delineating a classification of par-
activities of the household. Appropriate discipline teaches a child enting styles, which are known as Baumrind’s parenting typology and
empathy and to consider how other children feel when they are hit consist of 4 distinct categories. Authoritarian parenting focuses on
or teased. Parents, however, may be more focused on eliminating specific rules and the belief that the rules should be followed with-
unwanted behaviors and may bring these specific concerns to their out exception. Children are not encouraged to participate in decision-
child’s pediatrician. making or problem-solving. Children are punished for their mistakes
Parental monitoring relates to the oversight of children’s activities and, as a consequence, self-esteem may be negatively affected.
at home, in school, and in the community. The extent and form of Authoritative parenting, however, encourages participation
parental monitoring varies with the age of the child. Parental mon- of children in decision-making and focuses on positive discipline
itoring occurs when parents ask their children, “With whom are strategies and reinforcing desired behaviors. Rules and conse-
you going to be? Where are you going? What will you be doing?” quences do exist, but children play a participatory role. Children’s
Parental oversight involves children’s access to and use of the feelings are considered, children learn empathy, and high self-esteem
internet and social media (see Chapter 7). Inadequate parental is fostered.
monitoring has long-term sequelae, including an increased Permissive parenting is also referred to as indulgent or lenient
incidence of risk-related behaviors. Parental monitoring must be parenting. Rules are rarely enforced, although children may be
339

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 340 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

threatened, such as, “If you do that again, you will be grounded. in English and Spanish that presents videos of hypothetical scenarios
I really mean it this time!” Children may feel anxious because they and has parents select from a list of options how they would man-
are uncertain about the boundaries that might separate them from age the behavior. The program helps augment parents’ repertoire of
harmful decisions they make on their own. responses to their child’s behaviors.
Uninvolved parenting occurs when parents may be more involved
in their own lives and have less interest in or time with their chil- Corporal Punishment
dren. They are unaware of their children’s progress in school, their The relationship among harsh punishment, use of corporal punish-
children’s interests, or their children’s friends. ment, and child abuse has been addressed in a number of studies.
These categories suggest mutually exclusive parenting styles, but The American Academy of Pediatrics has published extensively on
many parents use all styles for truly effective parenting. There are the issue of corporal punishment and highlighted how approval of
times when parents may have to say, “Because I said so” (authori- corporal punishment as an acceptable means of disciplining children
tarian), and other times when they say, “Go ahead; it’s fine with me has significantly decreased in recent years. It is of interest that the
if you want to try that” (permissive). UN Convention on the Rights of the Child (1989) endorses banning
Regardless of parenting style, it is important to encourage parents corporal punishment and promoting positive discipline. Slapping,
to establish a positive interactive environment with verbal commu- smacking, spanking, kicking, shaking, and throwing are all enumer-
nication, monitoring children’s behavior and commending desir- ated, as are other punitive measures. Data support physical disci-
able behavior, ignoring trivial problems, and consistently applying pline as being associated with subsequent aggressive behavior on the
predetermined consequences for misbehavior. Psychologist Marshall part of toddlers. There are also data linking corporal punishment
B. Rosenberg promotes the concept of compassionate communica- with adverse childhood experiences (see Chapter 142). Scolding
tion, using the analogy of the language of the giraffe, which is a (yelling) is sometimes equated with harsh verbal abuse, especially
language of requests, versus the language of the jackal, one of if it is pervasive and may escalate to physical punishment. Receiving
demands. Identifying feelings is integral to the language of giraffes. harsh verbal abuse before 13 years of age has been linked to adoles-
Rules should be simple, clear, and established ahead of time. cent behavioral and mental health issues.
Frequently, physicians fail to inquire about children’s behavior.
Unless parents bring up the topic, discipline is not routinely dis-
cussed during the physician visit. On average, physicians spend Common Problem Behaviors
only 90 seconds per visit on anticipatory guidance and counseling. Common behavioral problems can be placed in 5 major categories.
However, a survey of mothers in a physician waiting room showed 1. Problems of daily routine. Such problems include the refusal of
that up to 90% were concerned about 1 aspect of behavior. Sixty children to go about their daily activities, such as eating, going
percent of mothers surveyed found physician advice quite helpful. to bed, awakening at a certain time, and toilet training.
The American Academy of Pediatrics recommends anticipatory 2. Aggressive-resistant behavior. Such behavior is characterized
guidance about discipline at each health supervision visit between by negativism and includes temper tantrums and aggressive
9 months and 5 years, and studies report that physicians counsel responses to siblings and peers. Some undesirable behavior can
parents about discipline about 40% of the time. Such counseling is place children or those around them in danger or at risk for injury.
especially important to help parents understand the value of appro- 3. Overdependent or withdrawal behavior. This behavior is
priate discipline in shaping their children’s self-esteem. Information typical of children who are very attached to their parents These
about discipline in the media may be confusing and contradictory children find separation difficult, especially when beginning
and often supports the unfounded approaches of nonprofessionals. preschool.
Starting when a child is 5 years old, physician-parent discussions 4. Overactivity or excessive restlessness.
should include the notion of monitoring. 5. Undesirable habits, which include thumb-sucking, nail-biting,
Early in the physician-parent relationship, physicians may throat clearing, and playing with genitals (see Chapter 54).
express their interest in behavioral problems by saying, “I am inter- Some of the listed behaviors are age appropriate, and physicians
ested not only in your child’s physical well-being but also in his can help parents by counseling them about stage-related behavior,
[or her or their] growth and development and how he [or she or such as oppositional behavior in a 2-year-old and independence-
they] gets along with friends and family.” They may then question dependence conflicts in a 3- to 5-year-old. Parents may be more
parents about how children spend their days. During subsequent tolerant of a particular behavior if they understand what is typi-
visits, pediatricians may say, “Parents of children of [child’s name]’s cal at a given age. Just because something is typical, however, does
age frequently worry about discipline. I wonder if you have any con- not mean that it should be tolerated. Physicians can suggest to the
cerns.” In making these inquiries, the physician may establish what parents means of dealing with age-appropriate behavior (eg, plac-
factors, such as religious or ethnic beliefs, or family influences are ing breakable objects out of reach of toddlers).
shaping parents’ decisions about discipline. Certain tools exist that Some behavioral problems reflect differences in childhood
can assist the primary care physician with counseling parents. One temperaments. Temperament is the biological predisposition to a
such model, Play Nicely, involves a multimedia educational program style of behavior. William B. Carey, MD, has compiled a series of

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 Chapter 50: Discipline 341

temperament scales to assess children and adolescents of differ-

Box 50.1. What to Ask
ent ages. For example, some children are shy and have a hard time
adjusting to new situations. If parents anticipate such problems, they Discipline
are often less angry when difficulties arise. Parental expectations can ww What does the child do that the parents wish the child would not do?
vary with a child’s sex. Boys may be permitted to act a certain way ww What do the parents do to stop unwanted behavior?
(“He’s all boy!”) that would be disapproved of in girls. Physicians can ww Does the child usually obey the parents?
discuss such expectations at health supervision visits. ww What does the parent do if the child does not mind the parent?
Physicians can also be particularly helpful in detecting and ww When and where does most of the unwanted behavior occur? Does it
advising parents about disparities in the achievement of different occur mainly if a child is tired?
developmental skills. Some children acquire motor skills before ver- ww Which parent is responsible for disciplining the child?
bal skills, yet parents expect their children to be equally versatile in
speech and movement.
follow-up question could be, “What do you do if your child doesn’t
Psychophysiology mind you?” This may help initiate a discussion related to parenting
style and whether physical punishment is used to achieve adherence
All behaviors are modified by the responses and reactions of other to parental rules. If parents have specific concerns, such as oppo-
individuals. The basic premise of discipline is to discourage unwanted sitional behavior, they should be questioned about the strategies
behavior and to encourage desired behavior. This is accomplished by they have used in their effort to discipline their children. It may also
using techniques that are based on conditioning modalities. be useful to ask parents how they were disciplined as children to
Several factors contribute to an increased incidence of behavioral better understand their personal experience.
problems in children. Ten percent to 15% of all preschool-age chil-
dren are raised in challenging or disrupted family situations. Homes Physical Examination
may be affected by divorce, death, separation, violence, parental sub- Children’s behavior should be assessed during the office visit. A
stance use, mental illness, or extreme poverty (see Chapter 141). general physical examination is useful to check for any signs of phys-
Parental inexperience may also be a factor. In addition, families may ical abuse as well as to evaluate children’s well-being. A developmen-
have fewer social contacts than they once did because of greater tal assessment is also helpful because it may delineate disparities in
mobility within society. As a result, families face greater social the achievement of certain skills. Some overly active children may
isolation and less availability of extended family. warrant a more extensive psycho-developmental assessment to look
for findings consistent with ADHD. Behavioral checklists may be
Differential Diagnosis used to evaluate children’s temperament.
In addition to providing anticipatory guidance about discipline,
consider whether a specific behavior represents typical child- Laboratory Tests
hood behavior or an abnormality in behavior that warrants more No specific laboratory or imaging studies are indicated for children
specific intervention. Between 8% and 18% of behavioral dis- with discipline problems. Diagnostic studies are indicated if child
turbances may deserve physician intervention. More intensive abuse is suspected (see Chapter 144).
management may be necessary for problems related to aggressive-
resistant behavior and hyperactivity. Overactive behavior, which Management
may exist as part of attention-deficit/hyperactivity disorder Physicians should assist parents in establishing appropriate guide-
(ADHD), may be a sign of a significant underlying problem that lines for disciplining their children and reinforce the role of parents
warrants 1-to-1 intervention or the use of neuropharmacological as the source of authority (Box 50.2). It is important for parents
agents (see Chapter 133). to realize that total freedom, which may be seen in families with
Occasionally, children will be seen and noted to have bruises that permissive parenting, may result in poorly controlled anxiety.
were sustained during physical punishment. Although physical pun- Children mimic behavior, and parents should act as role models.
ishment is not illegal, the presence of significant bruises or injuries If parents have temper tantrums when they are frustrated, their
may warrant a report to child protective services. children may act in a similar manner. Consistency is also impor-
tant. A system with a limited number of enforced rules is better
Evaluation
than one with many different rules. In families in which both par-
History ents are working, especially if they have overlapping time sched-
The key component in the evaluation process is the assessment of ules, consistency is sometimes difficult to attain, particularly if
the means parents use to discipline children (Box 50.1). To obtain children are supervised by non-parental caregivers. Likewise, if
this information, physicians may simply ask parents, “How do parents are divorced and children are moving between 2 different
you get your child to mind you?” This question is designed to households, rules may vary depending on which parent is caring
lead to a discussion of how parents interact with their children. A for the child (see Chapter 149).

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 342 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

children and for boys and correlates with parental attributes such
Box 50.2. Advice for Parents About Discipline
as age, education, socioeconomic status, and religious orientation.
ww Set rules. Spanking is often employed when other methods of discipline
ww Set limits. have failed to abort the unwanted behavior. Spanking tends to
ww Define consequences. clear the air and get the punishment over with rather than produc-
ww Be consistent. ing a lingering guilt. To be truly effective, however, physical pun-
ww Ignore trivial problems. ishment must immediately follow the act. The “wait until Daddy
ww Compliment desirable behavior. comes home” approach is less effective because of the lack of
ww Take time out when angry. temporal association. In addition, spanking tends to become
situation-specific so that children associate a particular action
with being spanked. This learning does not generalize to other sit-
Physicians should emphasize to parents that it is best to avoid
uations. Spanking can teach children to be afraid of adults rather
power struggles. Children engaged in a struggle can often win
than to respect them.
because in some situations they have final control (eg, refusing
Spanking, as well as other forms of physical punishment, can
to eat). Children should always be given the opportunity to gra-
damage the parent-child relationship and have a long-term effect
ciously back out of a situation and save face. It is easier to avoid
on a child’s self-esteem. Parents may feel remorseful after spanking
situations that lead to head-on confrontations than to grace-
a child, and some acknowledge that they spank out of anger and
fully emerge from them once the confrontation has occurred.
frustration and question the efficacy of this modality. Most pedi-
Physicians can commend parents on appropriate handling of dif-
atricians discourage spanking as a means of disciplining children.
ficult situations, validate parental ability to handle their children
Differentiation of physical punishment from child abuse can be
appropriately, and guide parents on alternative strategies if there
difficult. In general, physical punishment using objects (eg, belts)
is a need to do so.
and spanking on parts of the body other than the buttocks or
Physicians should remind parents that forestalling undesirable
thighs is unacceptable. Punishing a child by making them engage
behaviors is easier than treating behavioral disorders once they arise.
in physical exercise (eg, 200 jumping jacks) is also inappropriate.
Reasoning is a useful modality, but it is unrealistic to expect infants
Spanking may be a precursor of later physical violence and subse-
and toddlers to have the cognitive skills to understand adult reason-
quent abuse. Again, parents act as role models. Children should
ing or to consistently respond to verbal commands or reprimands.
never be allowed to hit their parents. This makes children feel
Discipline should not only discourage bad behavior but also rein-
extremely insecure.
force good behavior.
Devices that are marketed for child discipline and inflict physical
Following are 5 examples of reactive discipline: redirection,
pain on a child, such as a sudden sting (modified stun gun), are
spanking, scolding, ignoring, and time-out and removal of privileges.
obviously never appropriate.
Redirection Scolding
Redirection is a simple and effective method in which the par- Scolding involves the excess use of reasoning and explanations and
ent removes the problem and distracts the child with an alterna- is used by most parents as part of the discipline process. In fami-
tive. This technique is frequently used to remove some object (eg, a lies in which communication or interaction is minimal, scolding
valued knickknack) from the hands of an infant, replacing it with or verbal reprimands may result in an initial increase in inappro-
a toy. Parental patience, ingenuity, and enthusiasm facilitate this priate behavior because this is the only way children receive any
approach. This approach is also important in teaching children attention. Verbal reprimands are more effective if used infrequently.
what is acceptable behavior. For instance, one cannot draw on the Verbal reprimands should not be used during time-outs because
wall, but one can draw on a piece of paper. Children also respond they reinforce undesirable behavior.
to making tedious routines into a game. For example, children can Scolding, because of its negative focus, can be damaging to
be challenged to see who can get their clothes on faster or who can children’s self-esteem. Scolding may be equated with yelling, verbal
brush their teeth first. Parental creativity and energy often avert abuse, and harsh parenting practices. Scolding would be categorized
confrontations. by Rosenberg as “jackal language.”
Spanking Ignoring
Spanking involves inflicting physical pain, which can be success- Ignoring represents the opposite of explaining and reasoning. This
ful in bringing about the immediate cessation or a decrease in form of discipline is difficult to use successfully because parents must
problem behavior. Spanking is highly prevalent as a form of disci- totally ignore children’s behavior. If even the least flicker of recogni-
pline, with between 70% and 94% of parents reporting using the tion occurs, activity increases. A brief initial increase in unwanted
practice. However, only 6% of pediatricians have a positive atti- behavior, a so-called response burst, may occur with ignoring. This
tude toward spanking. Spanking is used more often for younger disciplinary method works better in younger children.

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 Chapter 50: Discipline 343

Time-out and Removal of Privileges devices, or loss of driving privileges. The privilege must be some-
thing of value to the child for this method to be effective.
Time-out, the form of discipline most often recommended, refers
Parents who report inappropriate behavior should be asked
to time away from positive reinforcement. In sports, teams call a
to keep a record of children’s behavior for 1 to 2 weeks. This
time-out to rethink what they are doing and to replan their strate-
helps determine the nature of the behavior (eg, whether it is
gies. Children are placed in a neutral or boring environment when-
age-appropriate) and what is motivating it. Parents should be
ever they engage in inappropriate behavior. The time-out technique
encouraged to talk to their children in a reasonable manner and
can be used to discourage undesired habits as well as inappropriate
to verbalize what they think children are feeling. They might say
behavior. For example, parents may say, “You can suck your thumb,
something like this: “It’s terrible to be 3 years old and get so upset.
but you may only do it in such and such a room.” This type of dis-
You feel that you can’t always get things you want. Once you grow
cipline is better than ignoring, especially if “ignored” children are
up, you will be in charge. I am really sorry it is so hard for you
receiving attention from siblings and peers. Children should under-
right now.” Physicians should tell parents that it is important to
stand the rules ahead of time and why the behavior is unacceptable.
set limits for children and to avoid threatening, judging, and con-
Once this is accomplished, time-out may occur without any warning.
stantly criticizing children. Frequent threats, such as, “If you don’t
A timer should be used, and children should stay in the time-
stop hitting your sister, you’ll get a time-out,” that are not car-
out area for 1 minute per 1 year of age. An appropriate area must be
ried out undermine the entire discipline process. To help foster
selected. This area should be fairly boring, so children’s rooms with
compassionate communication, parents should ask themselves,
their toys and electronic devices may not be appropriate. A laundry
“If someone said this to me, how would I feel?” Many parents
room, a corner, or a specific chair may be better. If children act unac-
have themselves been disciplined only with spanking and phys-
ceptably during the time-out, the timer should be reset. If children
ical punishment and know no other means, and the advice that
have to go to the bathroom during the time-out, they are allowed
physicians offer is valuable.
1 trip. After they return from the bathroom, the timer is reset.
There are models that can help parent improve their parent-
The use of the time-out method is not always easy. If the inap-
ing skills. Triple P is an evidence-based proven parenting pro-
propriate behavior occurs in the morning when children are getting
gram, as are HealthySteps (www.HealthySteps.org) and Help Me
ready for school, time-out just encourages children’s delaying tactics.
Grow (https://helpmegrownational.org). Pediatricians are encour-
“Beat the buzzer” is a better idea that may be used in such situations.
aged to be familiar with these resources and be able to share them
With “beat the buzzer,” the timer is set. For example, if children are
with families.
dressed before the timer goes off, they may be rewarded for the behav-
ior by being allowed to go to bed half an hour later, but if the buzzer Prognosis
“beats” them, they have to go to bed half an hour earlier.
Children raised in a supportive environment that teaches respect
Inappropriate behavior away from home presents an even greater
for others and self-control grow up as caring adults. Children
challenge. These situations can be dealt with in numerous ways,
who have been exposed to excessive physical punishment show
particularly if the behavior problem involves temper tantrums.
aggressive behavior later. The physician is in an excellent position
When children are crying or screaming uncontrollably, it is best to
to influence parenting practices and child well-being.
remove them from the embarrassment of the situation. This “man-
ual guidance” often occurs in a supermarket, where children select
something that parents do not wish to buy. Parents can often cir-
cumvent this problem by walking into the supermarket and say- CASE RESOLUTION
ing, “If you are good during the whole trip, then I will get you Further history should be elicited about the mother’s disciplining techniques. It
something at the checkout counter.” If children still have temper is also significant that the child’s speech is delayed. The boy’s ability to articu-
tantrums, they should be removed from the area and brought to a late his feelings may be limited, and a formal speech and hearing assessment is
warranted. The preschool should be advised that the evaluation is underway.
neutral place, such as the automobile or a restroom, and allowed
A report from the preschool concerning the boy’s behavior is requested.
to finish their crying and screaming. Inappropriate behavior can
also be managed by “marking” time-out. This consists of putting
a mark with a colored water-soluble marker on the child’s wrist
every time he or she engages in an inappropriate behavior. When Selected References
the child returns home, the marks are totaled, and the time-out
Afifi TO, Ford D, Gershoff ET, et al. Spanking and adult mental health impair-
method is used. Once again, separating the undesirable behavior
ment: the case for the designation of spanking as an adverse childhood
from the disciplinary consequence may limit the effectiveness of experience. Child Abuse Negl. 2017;71:24–31 PMID: 28126359 https://doi.
marking time-out. org/10.1016/j.chiabu.2017.01.014
Removal of privileges is a strategy applied with older children. American Academy of Pediatrics Committee on School Health. Corporal
Classically, this involves grounding a child, prohibiting television punishment in schools. Pediatrics. 2000;106(2):343 PMID: 10920163 https://
or video games, limiting the use of cell phones or other electronic doi.org/10.1542/peds.106.2.343

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 344 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Rosenberg MB. Nonviolent Communication: A Language of Life. 2nd ed. Encintas, Sege RD, Siegel BS; American Academy of Pediatrics Council on Child Abuse
CA: Puddledancer Press; 2003 and Neglect and Committee on Psychosocial Aspects of Child and Family Health.
Runyan DK, Shankar V, Hassan F, et al. International variations in harsh child Effective discipline to raise healthy children. Pediatrics. 2018;142(6):e20183112
discipline. Pediatrics. 2010;126(3):e701–e711 PMID: 20679301 https://doi. PMID: 30397164 https://doi.org/10.1542/peds.2018-3112
org/10.1542/peds.2008-2374 Taylor CA, Fleckman JM, Scholer SJ, Branco N. US pediatricians’ attitudes,
Sanders MR, Kirby JN, Tellegen CL, Day JJ. The Triple P-Positive Parenting beliefs, and perceived injunctive norms about spanking. J Dev Behav Pediatr.
Program: a systematic review and meta-analysis of a multi-level system of par- 2018;39(7):564–572 PMID: 29894363
enting support. Clin Psychol Rev. 2014;34(4):337–357 PMID: 24842549 https:// Vittrup B, Holden GW, Buck J. Attitudes predict the use of physical pun-
doi.org/10.1016/j.cpr.2014.04.003 ishment: a prospective study of the emergence of disciplinary practices.
Scholer SJ. Parental monitoring and discipline in middle childhood. Pediatr Pediatrics. 2006;117(6):2055–2064 PMID: 16740848 https://doi.org/10.1542/
Rev. 2009;30(9):366–367 PMID: 19726704 https://doi.org/10.1542/pir.30-9-366 peds.2005-2204

Scholer SJ, Hudnut-Beumler J, Dietrich MS. A brief primary care intervention

helps parents develop plans to discipline. Pediatrics. 2010;125(2):e242–e249
PMID: 20083523 https://doi.org/10.1542/peds.2009-0874

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 CHAPTER 51

Temper Tantrums
Geeta Grover, MD, FAAP, and Peter Jinwu Chung, MD, FAAP

CASE STUDY
During a routine office visit, the parents of a 3-year- Questions
old boy express concern about his recent behavior. They 1. At what age are temper tantrums common in children?
report that whenever he is asked to do something he 2. What aspects of child development contribute to
does not want to do, he throws a “fit.” He cries fiercely, temper tantrums?
falls to the floor, bangs his hands on the floor, and kicks 3. How do parents’ reactions encourage or discourage
his feet until his parents give in. He often displays such temper tantrums?
behavior at bedtime or mealtime if he is asked to turn 4. What appropriate management strategies may help
off the television or eat foods that he does not want. control problematic tantrums?
He has 2 to 3 such episodes per week. The parents state 5. What factors or aspects of problematic tantrums
that their home life has not changed, and the boy’s may indicate underlying pathology?
teacher reports that he displays no such behaviors at 6. What referrals, if any, are appropriate for the man-
preschool. agement of temper tantrums?

Temper tantrums are common, normal, age-related behaviors in words, such as children with developmental delays, especially those
young children. To a certain extent, oppositional behaviors such as with speech and language delays or with an autism spectrum disor-
negativism, defiance, and tantrums are part of the normal progres- der (ASD), are more likely to continue to have tantrums. Boys and
sion toward self-reliance and independence. Toddlers need to assert girls are affected equally. Although temper tantrums are unusual in
their freedom and explore their environment, which often puts them school-age children, they often reappear in the form of verbal tan-
at odds with the limitations imposed by society and well-meaning trums during adolescence, when autonomy and independence once
parents. Young children cannot appreciate that rules and limita- again become developmental issues.
tions have been established in the interest of their own safety and
well-being. They see only that their own desires have been thwarted, Pathophysiology
and they may react to this disappointment with intense emotions.
Temperament, or adaptability and emotional style, affects the ease
Children are not simply upset because they cannot have their way. with which children adjust to environmental inputs and their reac-
They are angry and frustrated, and they lose control over their emo- tions to these inputs. Temperament is the “how” of behavior, as
tions. During tantrums, children cry and scream uncontrollably. opposed to the “why.” It is innate rather than learned. Although
They may fall to the floor, bang their heads, kick their feet, pound inherent from birth, temperament may be modified in the early
their hands, and thrash about wildly. Some children may throw years by children’s experiences and interactions. Stella Chess, MD,
things, try to hit one another, or destroy property. and Alexander Thomas, MD, identified 9 major temperamental traits
Such intense displays of anger may be a terrifying experience for based on their study of children’s behavioral characteristics dur-
children and parents. Some children use tantrums to gain attention, ing the 1950s: activity level, rhythmicity (regularity), approach or
whereas others use them to achieve something or avoid doing some- withdrawal, adaptability, intensity, mood, persistence and attention
thing. Recurrent temper tantrums may strain relationships among span, distractibility, and sensory threshold. Three common patterns
parents, children, and other family members. of temperament based on whether a child shows a greater or lesser
degree of each of these traits are easy (high rhythmicity and adapt-
Epidemiology ability with a positive mood), slow to warm up or shy (slow adapt-
Temper tantrums are noted most often in children who are 2 to ability and tendency to withdraw initially in new situations), and
3 years of age, but they may occur any time between the ages of difficult or challenging (low rhythmicity and adaptability, resulting
1 and 5 years. Parental surveys reveal that approximately 20% of in negative and intense reactions to the environment).
2-year-olds, 18% of 3-year-olds, and 10% of 4-year-olds have Appreciation of children’s temperament allows parents to antic-
at least 1 tantrum per day. Most children can express their feelings ipate and understand their children’s reactions, thereby affording
verbally by 3 to 4 years of age, at which point temper tantrums begin them the opportunity to rethink how the parents interpret and
to taper off. Children who cannot express their feelings well with respond to their children’s behaviors. Ultimately, this knowledge
345

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 346 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

allows parents to guide children in ways that respect their individ- are signs of problematic tantrums (Box 51.1). These tantrums may
ual differences. Temperament does not excuse children’s unaccept- result from factors that are beyond the child’s control, such as
able behaviors, but it does provide some insight into the origins parental problems, school difficulties, or health-related conditions
of problematic behaviors, such as tantrums. For example, allow- (Box 51.2). For example, the child with unrecognized hearing loss
ing extra time in the morning for the high–activity-level child with may be performing poorly at school and resort to tantrum behav-
high distractibility and low attention span to get ready for school ior in frustration. Marital discord or domestic violence may create
may avoid the daily negative interactions between parent and child. anxiety for a child, which may manifest as frequent or destruc-
A discussion of temperament is not complete without noting that tive tantrums. Additionally, problematic temper tantrums may be
ultimately it is the “goodness of fit” between parental and child tem- a symptom of an underlying psychiatric or neurodevelopmental
peraments that is the key issue. What may appear to be a behavioral condition.
problem may in fact be a mismatch between parental and child tem-
peraments (eg, a high-energy and high-intensity child may be quite Differential Diagnosis
challenging for the slow to warm up or shy parent). It is important Temper tantrums are readily recognizable because of their classic
to help parents appreciate that such mismatches in temperamental pattern in which a child becomes frustrated, reacts physically, and
traits between themselves and their children do not necessarily rep- cries or screams.
resent problems in their children’s character.
Understanding the child’s level of maturity and the developmen-
tal tasks normally associated with the toddler and preschool years, Box 51.1. Features of Problematic Tantrums
which is when temper tantrums most often occur, facilitates further
understanding of tantrum behavior. Young children who are explor- ww Tantrums that persist or get worse beyond 4–5 years of age
ing the world and developing a sense of autonomy think primarily ww Frequent tantrums (>5 per day)
in egocentric terms. They view reality from their own perspective ww Tantrums lasting more than 15 minutes
and are unable to appreciate the perspective of other individuals. ww Persistent negative mood or behavior in intervals between tantrums
Only as they mature and enter school do they learn to recognize ww Recurrent tantrums at school
the position of others and begin to develop a sense of morality—of ww Destruction of property during tantrums
right and wrong. Toddlers may become frustrated or angry because ww Harm to self or others during tantrums
of their lack of control over the world, their inability to communi- ww Other behavioral problems (eg, sleep disorders, aggressive behaviors,
cate, or limitations of their cognitive and motor abilities, which do enuresis)
not allow them to accomplish desired tasks. Unlike adults, who can
verbalize frustrations or simply walk away from unpleasant situa-
tions, young children have neither the sophisticated ability to artic-
Box 51.2. Underlying Causes
ulate their feelings nor the freedom to walk away. Therefore, they
of Problematic Tantrums
may react to disappointments with temper tantrums. With cogni-
tive and emotional maturation, children should gradually learn to Parent-related Factors
exhibit more emotional control and/or use language to express them- ww Marital discord
selves. If caregivers consistently reinforce tantrum behavior, how- ww Abusive behavior toward children
ever, such as by “giving in,” this maturation process may be delayed. ww Domestic violence
Temper tantrums may be classified as normal or problematic ww Substance abuse
based on their cause, frequency, and characteristics. Normal tan- ww Depression
trums can simply be demands for attention or signs of frustration, ww Inappropriate parental expectations
anger, or protest. In the interval between tantrums, the child’s dispo- Child-related Factors
sition and mood are normal. The well-behaved 3-year-old boy who ww Developmental or learning disabilities
has an occasional tantrum after the birth of a sibling, the girl age —— Hearing loss
2 years 6 months who throws a tantrum to express frustration —— Speech and language delays
because no one understands what she is trying to say, and the 2-year- —— Autism spectrum disorder
old boy who cries uncontrollably because he cannot complete the ww Mood disorders (eg, depression, disruptive mood dysregulation disorder)
puzzle he started or run fast enough to keep up with his 4-year-old ww Disruptive behavior disorders (eg, attention-deficit/hyperactivity disorder)
brother are all examples of normal tantrums. A typical reason for ww Trauma-related impairment (eg, posttraumatic stress disorder)
an avoidance-type tantrum is not wanting to go to bed at bedtime. ww Temperament (eg, high persistence and intensity of response and slow
All types of tantrum are more common when children are tired, ill, adaptability)
or hungry, because their ability to cope with disappointment and ww Illness
frustration is limited under these circumstances. —— Unrecognized illness (eg, otitis media, sinusitis)
Frequent tantrums (>5 per day) and tantrums that result in —— Chronic or recurrent illness
destruction of property or physical harm to the child or others

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 Chapter 51: Temper Tantrums 347

Evaluation language and social skills) is warranted. For a more complete dis-
cussion of ASD, refer to Chapter 132.
History Children with ADHD may react impulsively and lack the execu-
Obtaining a thorough history is essential (Box 51.3). Frequency of tive function skills necessary to regulate their emotional responses,
temper tantrums, circumstances that provoke them, a description which can precipitate problematic tantrums. Parent and teacher
of actual tantrums, and parental reaction must be ascertained. In feedback should be solicited about behaviors of inattention and
some instances, this reaction may provide insight into why tantrums hyperactivity. For a more complete discussion of ADHD, refer to
recur. Parental expectations should be assessed as well. Expectations Chapter 133.
that are inappropriate for children’s age and developmental matu- The child with an internalizing disorder, such as anxiety or
rity may create unnecessary tensions between parents and children depression, may exhibit generalized irritability or overreactivity
and result in tantrum behavior. Factors associated with problematic with frequent temper tantrums. In the child with depression irrita-
tantrums should also be assessed (Box 51.2). bility, rather than sadness, may be the presenting mood symptom.
It is important to remember that physicians usually see chil- Adolescent depression is discussed in Chapter 66.
dren whose tantrums are frequent, severe, or cannot be con- The child with exposure to trauma can present with behav-
trolled by parents. First, the pediatrician must determine whether ioral regression (eg, recurrence of temper tantrums) or changes in
any underlying pathology may be contributing to the behav- mood. If the severity or frequency of temper tantrums has recently
ior and, if so, what parental or child factors may be provoking increased, the pediatrician may screen for trauma by asking a simple
it. Second, the pediatrician must differentiate between normal question, such as “Since your last visit, has anything really scary or
and problematic tantrums (Box 51.1). Identification and reme- upsetting happened to your family or your child?” Cases of suspected
diation of the cause of problematic tantrums are the first steps traumatic exposure are best referred to a mental health professional
toward cure. for evaluation and treatment. Adverse childhood experiences and
Problematic temper tantrums can be a presenting symptom trauma-informed care are discussed in Chapter 142.
of an underlying neurodevelopmental or psychiatric condition, For the child with severe temper outbursts, the health profes-
especially if the tantrums persist or worsen after 4 to 5 years of sional should ask about characteristics suggestive of disruptive
age despite the development of typical expressive language skills. mood dysregulation disorder (Box 51.4). Referral to a subspe-
Conditions associated with problematic temper tantrums include cialist for further evaluation and treatment may be indicated.
ASD, attention-deficit/hyperactivity disorder (ADHD), internaliz- For more details about diagnostic criteria, see Diagnostic and
ing disorders, exposure to trauma, disruptive mood dysregulation Statistical Manual of Mental Disorders, Fifth Edition.
disorder, and disruptive behaviors. Disruptive behaviors, such as oppositional defiant disorder and
The child with ASD may have problematic temper tantrums conduct disorder, may present with severe temper tantrums in young
related to the core deficits of the disorder, such as rigidity, difficulty childhood. High intensity of defiance, aggression to people and/or
with change, and perseverative interests (eg, a child who lines up animals, self-harm, destruction of property, and difficulty recover-
toys may get upset if the arrangement is disrupted). In such situa- ing from tantrums occur more commonly in disruptive behavior
tions, careful history about the “trigger” for the tantrum as well as disorders and warrant referral to a subspecialist.
an investigation into the child’s developmental history (especially
Physical Examination
A thorough physical examination is appropriate, as is a develop-
Box 51.3. What to Ask mental assessment to determine if the child exhibits findings such
as speech delay or behavioral signs consistent with ASD or ADHD.
Temper Tantrums
Typically, the physical examination is normal.
ww How often does the child have temper tantrums?
ww What circumstances provoke the tantrums?
ww How does the child behave during the tantrums? What does the child do?
Box 51.4. Characteristics of Disruptive Mood
ww How does the child behave in the interval between tantrums?
Dysregulation Disorder
ww How do the parents react to the child during the tantrums? What do
they do or say? Temper Outbursts
ww Are parental expectations consistent with the child’s developmental ww Are verbally and/or behaviorally disproportionate to the situation
stage? ww Are developmentally inappropriate
ww Have there been any changes at home or school (eg, birth of a sibling, ww Occur ≥3 times per week
new school)? ww Occur in a child who is irritable or angry between tantrums
ww Is the child having any other behavioral or development-related ww Last for a defined time period
problems (eg, enuresis, sleep difficulties)? ww Are not related to other mental health issues such as depression,
ww Are there any other signs or symptoms of an underlying psychiatric or medication, medical, or neurological conditions
neurodevelopmental condition?
Derived from Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.

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 348 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Laboratory Tests can say, “Me angry.” Physicians should emphasize that it is impor-
tant for parents to remain calm during children’s temper tantrums.
No laboratory tests are indicated in the assessment of the child with
Shouting and spanking indicate to children that parents are also out
temper tantrums. Routine tests that are age-appropriate and sug-
of control. Children feel more secure if the adults around them are
gested as part of health maintenance are appropriate.
calm and in control.
Different types of temper tantrum may require specific treatment
Management and management strategies. A parent can prime a child by outlin-
Health maintenance visits are an ideal time to provide anticipatory ing expectations before common triggers occur, such as explain-
guidance on tantrums and discuss strategies to prevent or minimize ing before going to into a store that they are not going to buy a
this behavior. Parents typically report that their children become defi- toy. Parents should be supportive of a child who is having a tan-
ant and difficult to manage during the “terrible twos.” At the 12- and trum resulting from frustration or fatigue by letting the child know
15-month visits, the physician should alert parents that this period is that the parent understands. The child’s energy should be redirected
approaching and remind them that it is a normal part of development. into activities the child can do well. Parents should be encouraged
Preventive strategies should be discussed, such as childproofing the to praise positive behavior, for example, completing tasks properly or
home to minimize unnecessary conflicts. Additionally, parents can managing anger in an acceptable fashion. They should ignore some
give young children frequent opportunities to make choices, such as tantrums, such as those for the purpose of attention seeking or want-
which color shirt to wear or which of 2 foods to have for lunch. These ing something. The child with no audience has no need to perform.
opportunities allow children to exercise independence and autonomy Time-outs may also be used in such situations (see Chapter 50).
in a positive rather than a negative manner. The physician can pro- Parents should not give in to children’s wishes, because doing so may
vide reassurance that this unpleasant stage will pass; children even- reinforce tantrum behavior. Physical movement of children to where
tually become more cooperative and agreeable. Punishment is not they belong may be necessary if they are refusing to do something
the solution to temper tantrums (see Chapter 50). (eg, bed for the child who is refusing to go to sleep at bedtime) or
A parent may inadvertently reinforce negative behaviors by in danger of hurting themselves. Holding children who are raging
primarily giving attention to the child during the moments the child may give them a sense of security and help calm them. If temper
is misbehaving. Setting aside special time between parent and child tantrums occur outside the home, it may be necessary to accom-
on a regular basis gives children a close connection with parents pany children to a quiet, private place, such as an automobile, until
without having to misbehave. Similarly, time-in is a strategy in which they calm down. Distracting children by suggesting another activity
the parent, upon noticing that the child’s behavior is beginning to or pointing out something of interest in the environment may also
escalate, spends 5 to 10 minutes soothing and comforting the child interrupt the unwanted behavior. Corporal punishment is associated
in an effort to mitigate the emerging negative behavior. with increased aggressive behavior and loses potency with repeated
Parents should be advised that helping children learn self-control administration. The American Academy of Pediatrics strongly dis-
and how to manage anger are keys to managing temper tantrums. To courages striking a child, including spanking.
expect that a child will never become angry is unrealistic. Instead, If parents continue to struggle with their child’s temper tantrums,
children should be taught how to vent their anger and frustration referral to a parenting program should be considered (Table 51.1).
in an acceptable manner, such as articulating their feelings or hit- Several parenting programs have demonstrated efficacy in decreas-
ting a designated punching bag or pillow. As children mature, their ing disruptive behaviors and temper tantrums and may be available at
ability to verbalize their feelings increases, but even young children community centers, parent support centers, and mental health facilities.

Table 51.1. Programs for Parents of Children With Temper Tantrums

Name of Program Child Eligibility Comments
The Incredible Years Different groups for infant, toddler, preschool, and Parenting classes provided in small group setting
(www.incredibleyears.com) school age (up to 12 years) Course duration is 12–20 weeks
Includes children’s group for older children
Programs also available for teacher training
Triple P—Positive Parenting Program Birth–16 years of age Range of services, including public seminars, telephone
(www.triplep.net) Multiple delivery methods to match specific needs consultation, targeted counseling, small group learning
Separate modules available for children with sessions, online instruction, and 1-to-1 consultation
disabilities, medical concerns, and divorce
Parent-Child Interactive Therapy International Treatment modality for children with attention- Averages 14 sessions lasting 1–2 hours each
(www.pcit.org) deficit/hyperactivity disorder, oppositional defiant
disorder, and conduct disorder

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 Chapter 51: Temper Tantrums 349

Degnan KA, Calkins SD, Keane SP, Hill-Soderlund AL. Profiles of disruptive
CASE RESOLUTION behavior across early childhood: contributions of frustration reactivity, physi-
ological regulation, and maternal behavior. Child Dev. 2008;79(5):1357–1376
The child seems to be having normal, age-appropriate tantrums. The boy’s tantrums
PMID: 18826530 https://doi.org/10.1111/j.1467-8624.2008.01193.x
occur when he is asked to do something that he does not want to do. In these situ-
ations, the parents should try to ignore the tantrums as much as possible and not Harrington RG. Temper tantrums: guidelines for parents. Naspcenter.org web-
give in to the child’s wishes. site. www.naspcenter.org/parents/tantrums_ho.html. Accessed July 17, 2019
Hong JS, Tillman R, Luby JL. Disruptive behavior in preschool children: distin-
guishing normal misbehavior from markers of current and later childhood con-
duct disorder. J Pediatr. 2015;166(3):723–730.e1 PMID: 25598304 https://doi.
org/10.1016/j.jpeds.2014.11.041
Selected References Ogundele MO. Behavioural and emotional disorders in childhood: a brief over-
American Academy of Pediatrics. How to understand your child’s tempera- view for paediatricians. World J Clin Pediatr. 2018;7(1):9–26 PMID: 29456928
ment. HealthyChildren.org website. www.healthychildren.org/English/ages- https://doi.org/10.5409/wjcp.v7.i1.9
stages/gradeschool/Pages/How-to-Understand-Your-Childs-Temperament.aspx. Potegal M, Davidson RJ. Temper tantrums in young children: 1. behavioral com-
Accessed July 17, 2019 position. J Dev Behav Pediatr. 2003;24(3):140–147 PMID: 12806225 https://doi.
Barlow J, Bergman H, Kornør H, Wei Y, Bennett C. Group-based parent training org/10.1097/00004703-200306000-00002
programmes for improving emotional and behavioural adjustment in young chil- Potegal M, Kosorok MR, Davidson RJ. Temper tantrums in young children: 2.
dren. Cochrane Database Syst Rev. 2016;(8):CD003680 PMID: 27478983 https:// tantrum duration and temporal organization. J Dev Behav Pediatr. 2003;24(3):
doi.org/10.1002/14651858.CD003680.pub3 148–154 PMID: 12806226 https://doi.org/10.1097/00004703-200306000-00003
Beers NS. Managing temper tantrums. Pediatr Rev. 2003;24(2):70–71 PMID: Wilson HW, Joshi SV. Recognizing and referring children with posttraumatic
12563041 https://doi.org/10.1542/pir.24-2-70-a stress disorder: guidelines for pediatric providers. Pediatr Rev. 2018;39(2):
Belden AC, Thomson NR, Luby JL. Temper tantrums in healthy versus depressed 68–77 PMID: 29437126 https://doi.org/10.1542/pir.2017-0036
and disruptive preschoolers: defining tantrum behaviors associated with clin- Zahrt DM, Melzer-Lange MD. Aggressive behavior in children and adolescents.
ical problems. J Pediatr. 2008;152(1):117–122 PMID: 18154912 https://doi. Pediatr Rev. 2011;32(8):325–332 PMID: 21807873 https://doi.org/10.1542/
org/10.1016/j.jpeds.2007.06.030 pir.32-8-325

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 CHAPTER 52

Breath-Holding Spells
Geeta Grover, MD, FAAP, and Peter Jinwu Chung, MD, FAAP

CASE STUDY
A 15-month-old girl is brought to the office because Questions
of parental concern about seizures. In the past month 1. What are breath-holding spells?
she has passed out momentarily 3 times. Each episode 2. What is the differential diagnosis of breath-
seems to be precipitated by anger or frustration on her holding spell?
part. Typically, she cries, holds her breath, turns blue, 3. What, if any, laboratory studies are indicated in the
and passes out. Each time she awakens within a few sec- evaluation of breath-holding spells?
onds and seems fine. The medical history and family his- 4. What measures can be taken to prevent breath-
tory are unremarkable, and the physical examination is holding spells? Are anticonvulsant agents necessary?
entirely within normal limits. 5. What are the effects of breath-holding spells on
family functioning?
6. What, if any, are the long-term sequelae of breath-
holding spells?

Breath-holding spells (BHSs) are a benign, recurring condition of spontaneously. Other disorders in this heterogeneous group include
childhood in which anger or pain produces crying that culminates syncope, migraine, cyclic vomiting, benign paroxysmal vertigo, par-
in noiseless expiration and apnea. The frequency of BHSs, which oxysmal torticollis, sleep disorders (eg, narcolepsy, night terrors,
are involuntary phenomena, is variable and ranges from several epi- somnambulism), and shudder attacks.
sodes a day to only several episodes per year. Although the spells are The 2 major types of BHS are cyanotic and pallid. Approximately
innocuous, they usually provoke fear and anxiety among parents and 60% of children with BHS have cyanotic spells, 20% have pallid
caregivers because children often turn blue and become limp. The spells, and 20% have both types. Most commonly, affected
diagnosis usually can be made on the basis of a characteristic history children experience several spells per week. Approximately 15%
and description of the episode; however, the possibility of seizures of children with BHSs have complicated features. Complicated
should be considered. BHSs are defined as a typical BHS followed by seizure-like activity
or rigid posturing of the body. Unlike the postictal period of
Epidemiology epileptic seizures, prolonged periods of lethargy or drowsiness
Breath-holding spells occur in approximately 5% of all children following spells are uncommon.
between ages 6 months and 6 years, but they are most common in chil- Pallid spells are similar to cyanotic BHSs with some exceptions.
dren between 12 and 18 months of age. Most children with BHS will Pallid episodes are more commonly provoked by minor injury, pain,
have experienced their first episode by 18 months of age and nearly or fear rather than frustration or anger; the initial cry is mini-
all will have done so by 2 years. Although BHSs have been described mal prior to apnea and loss of consciousness; and children become
in children younger than 6 months, occurrence in such young infants pale rather than cyanotic. In pallid BHSs, children often lose con-
is uncommon. Boys and girls are affected equally. Approximately 25% sciousness or tone after only a single gasp or cry, whereas in the
of patients have a positive family history for BHSs. cyanotic form, the period of apnea prior to loss of consciousness
is much longer.
Clinical Presentation
The typical clinical sequence of the major types of BHSs is described Etiology
in the Pathophysiology section of this chapter and in Box 52.1. After Although the spells are triggered by identifiable stimuli, they are
a spell, the child may experience a short period of drowsiness. involuntary phenomena. It is believed that loss of consciousness in
the cyanotic and pallid forms is caused by cerebral anoxia. The mech-
Pathophysiology anisms of the 2 types of BHS are different. The processes involved in
Breath-holding spells may be classified as 1 of the nonepileptic cyanotic BHS are not clear. Proposed mechanisms include centrally
paroxysmal disorders of childhood. These recurrent conditions, mediated inhibition of respiratory effort and altered lung mechanics,
which have a sudden onset and no epileptiform focus, resolve which may inappropriately stimulate pulmonary reflexes, resulting
351

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 352 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Box 52.1. Diagnosis of Breath-Holding Spells Box 52.2. Differential Diagnosis

of Breath-Holding Spells
ww Identifiable precipitating event or emotion
ww Brief duration Central Nervous System
ww Color change, if present, prior to loss of consciousness and rhythmic ww Seizures (ie, epilepsy)
jerking of extremities ww Occult or overt brain stem lesions (causing dysfunction within the
ww Rapid restoration of full activity pontomedullary area)
ww Normal neurologic examination ww Benign paroxysmal vertigo
Cardiovascular
ww Cardiac arrhythmia (eg, long QT syndrome)
in apnea and hypoxia. In the pallid form, the pale coloration and ww Syncope (orthostatic or vasovagal)
loss of tone are thought to result from vagally mediated severe bra-
Miscellaneous
dycardia or asystole. Pallid spells have been spontaneously induced
ww Gastroesophageal reflux/Sandifer syndrome
in the electroencephalogram (EEG) laboratory using ocular com-
ww Cataplexy (ie, transient loss of muscle tone associated with narcolepsy);
pression to trigger the oculocardiac reflex. Vagally mediated brady-
rare before adolescence)
cardia or asystole lasting more than 2 seconds has been produced
ww Central or obstructive apnea
by this maneuver.
ww Factitious disorder imposed on another (ie, Munchausen syndrome
An association between iron deficiency anemia and BHSs has
by proxy)
been recognized for many years but is poorly understood. It may be
related to the importance of iron in the function of various enzymes
and neurotransmitters in the central nervous system or because
children with anemia have decreased cerebral oxygenation, mak-
Box 52.3. What to Ask
ing them more susceptible to BHSs.
Although children with BHSs have not been shown to have Breath-Holding Spells
higher rates of psychiatric conditions compared with their other- ww What happened before the episode?
wise healthy peers, they are more likely to exhibit difficult tempera- ww Was the child crying?
mental traits, including increased intensity of reactions and general ww What was the child’s color before and during the episode?
states of anger, annoyance, and/or irritability. Mothers of children ww Was the child lethargic after the episode?
with BHSs have been shown to have higher rates of anxiety, depres- ww Does the family have a history of breath-holding spells?
sion, stress, and family functioning problems than mothers of chil-
dren without BHSs. Breath-holding spells are also associated with
stressful life events during pregnancy, although the mechanism and
directionality of this association is not known. of the suspected breath-holding episode. The sequence in which the
events occurred may help differentiate BHSs from epileptic seizures.
Differential Diagnosis
Physical Examination
The differential diagnosis primarily includes seizures and syncope
The child should undergo a complete physical examination, includ-
secondary to cardiac arrhythmia or a vasovagal episode. Although
ing a thorough neurologic evaluation. Focal neurologic signs or
vasovagal syncope, like BHS, may be provoked by fear or pain, it is
evidence of structural lesions, such as meningomyelocele or hydro-
uncommon in children younger than 12 years. Three factors may
cephalus, may be suggestive of a diagnosis other than BHSs.
help differentiate BHSs from true epileptic seizure activity. First,
spells usually are provoked by some upsetting event or emotion, Laboratory Tests
unlike seizures, which generally lack a recognizable precipitating
If the history is consistent with BHSs and the physical examination
event. Second, episodes are brief in duration and are followed by
is normal, laboratory evaluation is usually unnecessary. Because of
rapid restoration of full activity. Third, color change precedes loss of
the association of BHSs with iron deficiency anemia in some chil-
consciousness and rhythmic jerking of the extremities, whereas in
dren, it is appropriate to determine a hemoglobin level. An EEG
the typical epileptic seizure, convulsive activity and loss of muscu-
may be obtained if the physician is concerned about the possibil-
lar tone usually precede change in color. Box 52.2 shows the differ-
ity of epileptic seizures. The interictal EEG is normal in the child
ential diagnosis of BHSs.
with BHSs, whereas it is often abnormal in the child with epilepsy.
In both forms of BHS, during attacks the EEG shows generalized
Evaluation
slowing followed by flattening; this pattern is characteristic of cere-
History bral anoxia. It is unusual to capture a BHS during the EEG, however.
History alone may be diagnostic (Box 52.3). A family history of Simultaneous EEG and video recordings can be quite useful in help-
BHSs should be obtained. It is essential to record a detailed history ing to distinguish BHSs from seizures, especially in the child with

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 Chapter 52: Breath-Holding Spells 353

frequent episodes. An electrocardiogram may be obtained if there age, and 90% resolve by 6 years of age. Neither pallid nor cyanotic
is any question about cardiac arrhythmia (eg, long QT syndrome). BHSs are associated with an increased risk for developing epilepsy,
although children with pallid BHSs do have an increased incidence
Management of developing syncopal attacks in adulthood.
Management of BHSs includes parental support and reassurance.
Breath-holding spells may be extremely frightening for parents to
witness, especially if the episodes are routinely associated with loss CASE RESOLUTION
of consciousness or seizure-like activity. Parents should be told of the
The child has a history and physical examination suggestive of BHSs. The girl’s
involuntary nature of the attacks and cautioned against reinforcing episodes are consistent with cyanotic BHS. The episodes are preceded by an iden-
the spells by giving in to the child’s wishes. They should be advised tifiable emotion, brief in duration, and followed by a rapid recovery of normal
to avoid unnecessary confrontations with the child. It is impossi- consciousness and activity. Assessment of the hemoglobin level revealed mild
ble to ensure that the child will never be frustrated or injured, how- iron deficiency anemia. The child received iron therapy, and the parents were
reassured about the benign nature of BHSs.
ever. Instead, parents should be encouraged to address the episodes
in a matter-of-fact manner and continue using age-appropriate dis-
cipline. They should be reassured that the long-term prognosis is
excellent. Research has demonstrated that psychoeducation about Selected References
the disorder can reduce the level of anxiety, depression, and stress
experienced by the caregivers; therefore, the physician may wish to Abbaskhanian A, Ehteshami S, Sajjadi S, Rezai MS. Effects of piracetam on pedi-
atric breath holding spells: a randomized double blind controlled trial. Iran J
consider screening caregivers for mental health concerns and refer-
Child Neurol. 2012;6(4):9–15 PMID: 24665274
ring them to the appropriate supportive services.
Anderson JE, Bluestone D. Breath-holding spells: scary but not serious.
For a subset of children with iron-deficiency anemia and BHSs,
Contemporary Pediatrics. 2000;17:61–72
iron therapy may be effective in the management of cyanotic and
Benbadis S. The differential diagnosis of epilepsy: a critical review. Epilepsy
pallid BHSs. More recent research has demonstrated that iron sup-
Behav. 2009;15(1):15–21 PMID: 19236946 https://doi.org/10.1016/
plementation, even in the absence of iron deficiency/insufficiency,
j.yebeh.2009.02.024
may be effective in reducing the frequency of BHSs.
Carano N, Bo I, Zanetti E, Tchana B, Barbato G, Agnetti A. Glycopyrrolate
Referral to a neurologist, cardiologist, or psychiatrist may be
and theophylline for the treatment of severe pallid breath-holding spells.
considered for the child with frequent episodes or for complex Pediatrics. 2013;131(4):e1280–e1283 PMID: 23509162 https://doi.org/10.1542/
cases. Pharmacologic therapy usually is not necessary, but atro- peds.2012-0182
pine sulfate may be considered in the treatment of children with DiMario FJ Jr. Prospective study of children with cyanotic and pallid breath-
frequent pallid BHSs because of the anticholinergic action of atro- holding spells. Pediatrics. 2001;107(2):265–269 PMID: 11158456 https://doi.
pine. Anticonvulsant agents are not effective. Successful cardiac org/10.1542/peds.107.2.265
pacemaker implantation has been performed for complex cases of Eliacik K, Bolat N, Kanik A, et al. Parental attitude, depression, anxiety in moth-
pallid BHSs with severe and frequent spells associated with seizures, ers, family functioning and breath-holding spells: a case control study. J Paediatr
life-threatening bradycardia, or asystole. Case reports have noted the Child Health. 2016;52(5):561–565 PMID: 27089451 https://doi.org/10.1111/
successful management of pallid BHSs with fluoxetine or a combina- jpc.13094
tion of glycopyrrolate and theophylline in small cohorts of patients. Jain R, Omanakuttan D, Singh A, Jajoo M. Effect of iron supplementation
Finally, several blinded, randomized controlled trials performed in children with breath holding spells. J Paediatr Child Health. 2017;53(8):
outside the United States have demonstrated efficacy of piracetam in 749–753 PMID: 28568906 https://doi.org/10.1111/jpc.13556
the management of BHSs, although this medication is not approved Kelly AM, Porter CJ, McGoon MD, Espinosa RE, Osborn MJ, Hayes DL. Breath-
for any use by the US Food and Drug Administration. holding spells associated with significant bradycardia: successful treatment with
permanent pacemaker implantation. Pediatrics. 2001;108(3):698–702 PMID:
11533339 https://doi.org/10.1542/peds.108.3.698
Prognosis
Walsh M, Knilans TK, Anderson JB, Czosek RJ. Successful treatment of pallid
Breath-holding spells resolve spontaneously in most children by breath-holding spells with fluoxetine. Pediatrics. 2012;130(3):e685–e689 PMID:
5 to 6 years of age. Approximately 50% of cases resolve by 4 years of 22869831 https://doi.org/10.1542/peds.2011-1257

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 CHAPTER 53

Fears, Phobias, and Anxiety

Carol D. Berkowitz, MD, FAAP

CASE STUDY
A 5-year-old girl is brought into the office by her mother, Questions
who complains that her daughter has been afraid to sleep 1. What are normal childhood fears and when do these
alone since the occurrence of an earthquake. The house fears commonly occur?
did not sustain any significant damage, but the entire 2. What strategies are used to deal with these fears?
family was awakened. The mother says that the girl 3. What are phobias? What are social phobias?
has become more timid. As nighttime approaches, she 4. What is school phobia, and how is it best handled?
becomes particularly fearful. She will not stay in her 5. What are common anxiety disorders in children and
bed, and she is comforted only by sleeping with her par- adolescents?
ents. In addition, the girl has begun bed-wetting since 6. How can families deal with childhood disturbances
the earthquake, and the mother wonders whether she that emerge after natural and artificial disasters?
should put her daughter in diapers. The physical exam-
ination, including vital signs, is normal, except for the
observation that the child is very clingy and whiny.

Fears are normal feelings that cause emotional, behavioral, and phys- they may be incapacitating. Anxiety refers to fear without a defin-
iological changes that are essential for survival. Fears are associated able source. It is characterized by a physiological response and may
with psychological discomforts, such as a negative, unpleasant feel- be perceived as a vague feeling of uneasiness, apprehension, and
ing. Children may develop fears in response to actual events (eg, foreboding of impending doom. A child may experience an anxi-
earthquakes) or as a result of the temporal association of 2 events ety problem where there is significant but not severe distress and
(eg, seeing a scary movie on a rainy day and then becoming afraid an anxiety disorder when the distress is excessive or functioning is
of rain). Some fears seem to be innate, and others seem to be devel- impaired. Fifteen percent to 20% of youths have anxiety disorders,
opmental. Children fear different things at different ages. For exam- the most prevalent psychiatric condition in children and adoles-
ple, school phobia is sometimes particularly problematic in young, cents. Girls are twice as likely to develop anxiety disorders as boys.
school-age children. Worry is the cognitive manifestation of fear There is often a positive family history of anxiety disorders, which
and anxiety. is felt to be related to a genetic predisposition and environmental
Phobias are overwhelming, intense, highly specific, and often factors. Children with autism spectrum disorder have an increased
irrational fears. The Diagnostic and Statistical Manual of Mental incidence of anxiety and anxiety-related disorders (see Chapter 132).
Disorders, 5th Edition, defines a phobia as excessive anxiety accom- Different strategies are useful for dealing with different fears. It is
panied by worry occurring more often than not, for at least 6 months important for parents not to trivialize these fears or reinforce them
and associated with 1 or more of the following: restlessness, easy but to empower children to deal with them.
fatigability, difficulty concentrating, irritability, tense muscles, and It is also important to realize that parents sometimes foster fears
disturbed sleep. Childhood phobias can be divided into 5 catego- by using threats with children, such as, “The doctor will give you a
ries: animals (eg, spiders, snakes, dogs), natural environment (eg, shot unless you eat your spinach,” or “The boogeyman will get you.”
heights), medical related (eg, doctors, dentists, injections), situa- By fostering fears, the parents are also fostering dependency. Parents
tions (eg, flying), and other (eg, loud noises, rain, thunder). Specific lack the imagination that children have and find it difficult to under-
phobias are often managed by avoidance and may not present to stand the degree of fear that children experience.
the physician for treatment. Social phobias (also called social anx- The opportunities for primary care physicians to counsel families
iety disorders) are specific to social situations that arouse intense about childhood fears has increased over the past decades related
concerns about humiliation or embarrassment. Fear of speaking to a number of catastrophic events, such as the terrorist attacks
in public may represent a social phobia. Selective mutism involves of September 11, 2001 (9/11), hurricanes Katrina and Sandy, the
children who are able to speak but are unable to do so in certain earthquake in Haiti, the tsunami in Sri Lanka, and tornados in the
settings, such as school. This probably represents 1 form of a social midwestern United States. Acts of violence, such as multiple mass
phobia. When these fears are combined with avoidance behavior, shootings, often at schools (eg, Columbine, Sandy Hook, Virginia

355

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 356 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Tech, Marjory Stoneman Douglas High School), also create fear and immediacy. These children are afraid of the death of their parents or
anxiety in children and adolescents who witness these events on tele- the burning of their home. They also fear war, growing up (expressed
vision and through messages posted on social media. It is important as “How will I know what to do?”), going into the next grade, being
to recognize the pervasiveness of mental health sequelae following alone or kidnapped, and the divorce of their parents. Children in this
disasters and the factors that influence the prevalence of these dis- age group are often reluctant to bother their parents with their fears,
turbances. One percent of children in New York, NY, lost a relative and they can easily misinterpret parental concerns when they over-
on 9/11. There is a greater risk of mental health sequelae if there hear parental conversations. Separation anxiety, which may mani-
are poor social supports or a prior history of psychopathology or if fest as school phobia and may be referred to as separation anxiety
the child is fearful or shy by nature. Natural disasters have a lesser disorder, may occur in school-age children. The prevalence is esti-
effect than intentional ones. While many of the recent disasters have mated at 3.2% to 4.1%, although up to 50% of third graders report
been acute and unexpected, there are children who are continuously separation anxiety symptoms. Separation anxiety disorder is defined
exposed to what has been called “process trauma” in the form of war, as developmentally inappropriate, excessive anxiety precipitated by
detention of children and families seeking political asylum in the actual or anticipated separation from home or family. Affected chil-
United States, and child abuse. dren develop physical complaints (eg, stomachaches) on school days.
The parent-child relationship may be disturbed or made insecure
Epidemiology (eg, marital discord, maternal illness), and the child is fearful of leav-
ing the parent alone. Childhood school phobia and parental history
Fears follow a developmental pattern (Box 53.1). Neonates are
of panic attacks and agoraphobia may be associated.
believed to have no fear, although young infants whose faces are
Fears during adolescence relate to social functioning, such as
covered with a blanket struggle to toss off the blanket. Infants who
public speaking or talking to members of the opposite sex. Older
are 6 months of age exhibit what is known as stranger anxiety in
response to unfamiliar persons, places, or objects. To combat this children are also concerned about school failure and physical injury.
anxiety, infants seek refuge with a parent. Stranger anxiety becomes They have many of the same fears expressed by school-age chil-
equated with separation anxiety and reaches a peak at 2 years of age. dren, although phobias are uncommon. Social phobia is a distinct
Children between 6 months and 2 years of age are also frightened entity and is different from shyness, as reported in a recent study
by loud noises and falling or quickly moving objects. of adolescents. Social phobia is a potentially impairing psychiatric
Children between the ages of 2 and 5 years are in what is termed disorder. Overall, phobias occur in less than 1.7% of the general pop-
the age of anxiety. They fear many things, including animals, aban- ulation but are reported in 13% of children with other emotional or
behavioral problems.
donment, loud noises, and darkness. Children in this age group are
Anxiety disorders are rare in childhood but more common dur-
particularly fearful of physicians, hospitals, and getting hurt. Young
ing adolescence. They may include panic attacks, which involve the
children are afraid of those who are physically disabled, who rep-
sudden onset of intense fear or discomfort associated with physio-
resent bodily injury, and monsters and scary movies. They some-
logical symptoms such as palpitations and shortness of breath. Fear
times displace their anger onto monsters and witches and attribute
about a panic attack may lead to agoraphobia (ie, the avoidance
to these imaginary characters the bad feelings they are experiencing.
of going away from home). Posttraumatic stress disorder (PTSD)
Children in this age group have strong imaginations, which makes
involves a set of symptoms that recurs after a person has experi-
it difficult for them to differentiate fantasy from reality.
enced a traumatic event. Symptoms include intense fear, helpless-
Children between 6 years of age and adolescence tend to have
ness, or a sense of horror. The person reexperiences the trauma,
more abstract thoughts, and their fears are less relevant to physical
avoids circumstances that are reminiscent of the trauma, and is in
a state of hyperarousal. It is estimated that 5% of men and 10% of
women have a lifetime prevalence of PTSD.
Box 53.1. Common Fears During Childhood
Pathophysiology
ww Neonates: no fears
ww 6 months–2 years: separation anxiety, loud noises, quickly moving Fear has its basis in a series of psychophysiological reactions, which
objects, the dark are mediated through a series of neurotransmitters. The reaction is
ww 2–5 years (ie, age of anxiety): animals, abandonment, loud noises, often referred to as the fright/flight response and is critical for sur-
darkness, physicians, hospitals, getting hurt, monsters, witches, ghosts, vival. The response is regulated through the limbic system. Elevated
storms levels of certain transmitters, such as -aminobutyric acid and nor-
ww 6 years–adolescence: death (parental death), parental divorce, natural epinephrine, are associated with feelings of anxiety. Excess sero-
and artificial disasters, growing up, school performance (going into the tonin has also been related to anxiety disorders.
next grade), war Studies on the neurobiology of pediatric anxiety disorders dem-
ww Adolescence: social situations, school performance, health, public onstrate dysfunction in the amygdala prefrontal-based circuits. The
speaking amygdala is responsible for the initiation of the central fear response
and is noted to be “overactivated” in magnetic resonance imaging of

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 Chapter 53: Fears, Phobias, and Anxiety 357

individuals with anxiety disorders. The prefrontal area helps regu- children, needles represent possible mutilation. When asked to rep-
late amygdala activity. Other areas of the brain have also been impli- resent needles in drawings, children often portray needles as larger
cated in anxiety disorders in youth. than themselves and very pointed. They comment that needles are
sharp (eg, “Needles can make you pop, just like a balloon”; “Needles
can also take out all your blood until you die”). In addition, chil-
Differential Diagnosis
dren are preoccupied with what happens to their blood. One young-
The challenge for physicians is to assess the etiology of the fear and ster commented, “They check out your blood to see if it’s good or
to differentiate normal fears from those that may be signs of unusual bad, and if your blood is bad, then it means that you need to have
stresses or signs of psychopathology. Appropriate fears represent more tests.” Another youngster thought that physicians were doing
a real reaction to a real danger. As a rule, children are more resil- a “blood taste” rather than a blood test.
ient than adults and recover more rapidly from traumatic events. Hospitalization raises other issues concerning parental sepa-
However, children are prone to inappropriate fears, which may ration as well as painful procedures. As children adjust to hospi-
develop for a number of reasons. talization, they progress through 3 stages: protest, during which
Inappropriate fears may occur because of operant conditioning, they complain about the hospital and cry; despair, during which
in which a conditioned stimulus becomes associated with another they have given up hope that their parents will return; and detach-
object. Fear of the other object becomes reinforced through this ment, during which they seem to be adjusting but actually have
association. Inappropriate fears may also develop in a child whose distanced themselves from their parents. Unrestricted visitation
parent has the fear (modeling) or through witnessing a fearful event by family members and involvement of child life specialists miti-
in the media (informational). True phobias represent neuroses and gates much of the distress.
may occur in more than 1 family member.
School phobia, also called school refusal, may occur under 3 dis-
tinct conditions. Not uncommonly, young children who are entering Evaluation
school for the first time are frightened. This fear is a normal compo- Physicians should explore the area of childhood fears and phobias
nent of separation anxiety, which usually resolves within a few days at routine health supervision visits, even if parents do not have spe-
of starting school. This is also referred to as adaptive anxiety. In con- cific concerns. Sometimes parents are embarrassed by children’s
trast, older children may experience school phobia because they are fears (eg, the fear of an older child to sleep without a night-light;
truly afraid of a school situation. They may fear a teacher, violence, the fear of dogs, which may preclude the child from visiting cer-
or a bully. To avoid the problem, children may actually request to tain friends). Parents may not report children’s fears unless these
change classrooms or schools. It is important to talk with children fears seem to be unusually intense. Practitioners may ask children,
to find out what is behind their fear of school. “What is the scariest thing you can think of?” If children are hav-
Some children who seem fearful of school, however, are actu- ing difficulty providing details, physicians may ask them to name
ally concerned about parental separation (ie, separation anxiety). things that other children fear or to complete the sentence, “I feel
Frequently, these children enjoy school and miss it when they are afraid when....” Alternatively, practitioners may suggest things that
absent. Absences occur when children’s feelings of separation from other children may fear: “Do the kids you know seem to be worried
parents are so intense that they do not allow them to function well about kidnapping?”
in school settings. Children are worried that something bad will Several instruments have been used to assess the level of anxi-
happen to them or to their parents when they are apart. This sepa- ety in children. These include the Multidimensional Anxiety Scale
ration anxiety disorder may result from parental illness or parents’ for Children, 2nd Edition; Spence Children’s Anxiety Scale; and Screen
fostering dependency in children. Children then see parents as vul- for Child Anxiety Related Disorders. The latter instrument is in the
nerable and are uncomfortable about leaving them alone. To qual- public domain and readily available. It includes statements for chil-
ify as an anxiety disorder, the symptoms must last at least 4 weeks. dren (eg, “I get scared if I sleep away from the house”) that are then
School phobia is the third leading cause of school absenteeism scored “Not True or Hardly Ever True” (0), “Somewhat True or
after transient illness and truancy. Fifty percent of children with Sometimes True” (1), and “Very True or Often True” (2). There is a
school phobia have other problems, including depression (28%), separate page for parents that includes similar statements framed
tantrums (18%), sleep disturbances (17%), obsessive-compulsive as, “My child gets...”, rephrasing the statement that their child rated.
behavior (11%), other fears (10%), enuresis (3%), and learning dis- Scores are added up and the total score, plus the items that scored
abilities (3%). Overall, school phobia has a good prognosis, although high, help distinguish the nature of the anxiety; a score of greater
adolescents do not do as well as younger children, and individuals than 25 indicates an anxiety disorder, with subcategories including
with a higher IQ have a poorer outcome. Twenty percent of parents panic disorder or significant symptoms, generalized anxiety disor-
of children with school phobia have a diagnosable psychiatric disor- der, separation anxiety disorder, social anxiety disorder, and signifi-
der. Issues of parent-child dependency are often a concern. cant school avoidance. Another instrument is a book called, What to
Another type of childhood fear concerns physicians and hos- Do When You Worry Too Much: A Kid’s Guide to Overcoming Anxiety.
pitals. Children have many concerns about what happens to them This book suggests a number of strategies (eg, setting up a worry-
at the doctor’s office. They are particularly fearful of needles. To ing time, not worrying if it’s not the designated time) in addition

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 358 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

to discussing the origin of different worries (eg, “How do worries Parents may feel helpless because they do not know how to deal
get started?”). with children’s fears. Physicians should give them the necessary
information. Children’s fears should not be trivialized. Even if the
History fears are unfounded, they should be validated. When discussing fears
The evaluation of children with specific fears demands a careful his- with children, parents should always provide physical comfort and
tory that provides information about situations in which children are help children develop a sense of safety and security. In general, chil-
fearful (Box 53.2). Physicians should consider fears within a devel- dren should be questioned about whether they are fearful about a
opmental context because many childhood fears are normal and situation. The following 2 examples illustrate the proper handling
experienced by all children. It is also important to look at changes of fears in children:
in the family situation. Children sometimes develop what seem to If children are visiting the dentist for the first time, it is appropri-
be fears but in fact are behaviors designed to manipulate other fam- ate for parents to ask, “Are you afraid?” If children reply, “Yes, a lit-
ily members. For instance, young children who sense marital dis- tle bit,” parents can say, “Almost everybody is afraid. Tell me what it
cord may insist on sleeping with their parents as a way of ensuring is you’re afraid of. Fear is a normal emotion, and I’m glad you told
that the parents are together rather than separate. me about it.”
Parents of children who express fear of imaginary characters can
Physical Examination reassure children that they do not exist. In addition, parents can tell
A routine examination is warranted, but findings are usually normal. children what the parents would do if such characters did exist. For
Such an evaluation, however, is particularly important if present- instance, the father of a little girl who was afraid of witches told her,
ing complaints include symptoms such as abdominal pain, head- “There are no such things as witches. But if there were, and they
ache, or palpitations. came into your room, I would punch them in the nose and punch
them in the stomach and beat them up, and then there would be no
Laboratory Tests
more witches to hurt you.” For those who would opt for a less violent
As a rule, laboratory tests are not required unless the symptoms approach, the parent could state: “I would tell any witch who came
suggest an organic etiology, such as hyperthyroidism, as the cause into your room, ‘STOP! Go away. No witches allowed in here.’ And
of palpitations. the witch would run away, and I would slam the door!” By doing this,
parents establish the reality of the situation and then also create a
Management plan to deal with the problem should it actually happen.
Management of the fear or phobia is determined by the degree to Parents can also help to limit or reduce children’s fears by min-
which children are incapacitated. As a general rule, children should imizing their exposure to fear-provoking situations such as televi-
be empowered to conquer their fears. Children’s books that address sion shows or scary movies. These programs can be particularly
the issues of certain fears can help achieve this empowerment; for frightening for some children, who should not watch them with-
example, The Berenstain Bears in the Dark discusses specific wor- out adult supervision. Minimizing exposure to television is particu-
ries such as fear of lightning and thunder. These books often explain larly important following a disastrous event. The recurrent images of
the basis of such natural phenomena in easy-to-understand terms. planes flying into buildings on 9/11 were interpreted by children as
Books also normalize particular fears and show how 1 character is repeated different attacks. Watching the nightly news can be anxiety
fearful. Parents can recreate some of the sounds that children fear. provoking not only for children but for their parents. Even if a fam-
For example, children who are afraid of the noise the wind makes ily chooses not to watch the evening news, “breaking news” includ-
are shown a teakettle from which hot steam blows through the whis- ing graphic images often appears on cell phones and other electronic
tle, creating the same noise as the wind. For fears about nuclear war, devices automatically, intruding during the school day.
empowering children to become active, such as joining a nuclear When dealing with children who have school phobia because
protest group, may be useful. of problems in school, it is important to determine if a change in
school would be appropriate to facilitate their school attendance.
This may be particularly appropriate in children whose schools are
Box 53.2. What to Ask
plagued with violence.
Fears and Phobias Cognitive-behavioral therapy (CBT) is reported to have the
ww What fear does the child have? Exactly what does the child fear? highest rate of success for dealing with anxiety-related conditions.
ww Under what circumstances was the fear originally expressed? Did any Cognitive-behavioral therapy includes psycho-education, somatic
changes in the child’s life occur around the time that the fear appeared? management (eg, relaxation techniques), cognitive restructuring
ww Under what conditions is the fear currently expressed? (ie, modifying negative thoughts), and exposure methods, including
ww How long has the child had the fear? desensitization. The goal of the therapist is to teach the child alter-
ww How does the fear affect the daily living of the child and family? native ways of viewing the feared object and of coping with the fear
ww What has the family done to help the child deal with the fear? itself. Social Effectiveness Therapy for Children and Adolescents is
geared to specifically address social phobia (social anxiety disorder).

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 Chapter 53: Fears, Phobias, and Anxiety 359

Studies have demonstrated it is more effective than placebo and Prognosis is good for children with true phobias, with 100% reso-
superior to fluoxetine on certain measures of social functioning. lution of monosymptomatic phobias. More significant anxiety disor-
Mindfulness-based psychotherapies have also been incorporated ders may persist into adulthood, at which time similar management
into the management of anxiety disorders. The focus is on the devel- involving CBT and medications may be indicated. Persistent anxi-
opment of mindfulness skills to help mitigate the symptoms asso- ety disorders are associated with increased morbidity, including an
ciated with anxiety. increased risk for self-injurious behavior and suicide. Early recogni-
Medications such as antidepressants, anxiolytics, sedatives, and tion and appropriate management can significantly affect the prog-
beta blockers have an unsubstantiated role in managing phobic dis- nosis of this common pediatric disorder.
orders in children but may be indicated in other conditions, such
as anxiety disorders.
When school phobia is linked to separation anxiety, a pro- CASE RESOLUTION
gram of desensitization or habituation (graded exposure) is recom- The girl’s fear of sleeping in her bed was triggered by a significant environmen-
mended. Desensitization may involve the participation of parents tal event. Although earthquakes are uncontrollable, the girl can be empowered to
cope with manageable aspects of an earthquake as much as possible. She should be
in the classroom for a time. When children acclimate and can
assured that in the same situation, many adults probably would also fear sleeping
tolerate some separation, mothers move to another area in the alone. The parents should stock a box with shoes, a flashlight, a radio, and water and
school, such as the principal’s office. Next they go outside the place the box under the child’s bed. In addition, they may have their daughter get
school grounds. As children reestablish a sense of well-being in into her bed and then shake it, simulating the jiggling that she would experience
spite of the separation, the mothers gradually move farther and during an earthquake. The girl should also practice getting out of bed and stand-
farther away. This solution is somewhat problematic for mothers ing in the doorway. To combat the child’s fear of separation during times of natural
disaster, the parents should reassure their daughter that they will all be together.
who work outside the home. There is some research to suggest
that children adjust more readily if they resume school immedi-
ately without the gradual withdrawal of their parent. Children
with significant school phobia may need the assistance of child Selected References
psychologists or psychiatrists. Physicians
Phobias may be treated using the concept of flooding, which con- American Psychiatric Association. Diagnostic and Statistical Manual of Mental
sists of rapid, prolonged exposure to the feared item. For example, a Disorders. 5th ed. Washington, DC: American Psychiatric Association; 2013
child who is afraid of dogs is exposed to a friendly, docile, small dog Bagnell AL. Anxiety and separation disorders. Pediatr Rev. 2011;32(10):
while in the company of the child’s parents. Alternatively, system- 440–446 PMID: 21965711 https://doi.org/10.1542/pir.32-10-440
atic desensitization, during which children are exposed to the feared Beidel DC, Turner SM, Young B, Paulson A. Social effectiveness therapy for chil-
objects over a series of weeks, coupled with relaxation techniques, is dren: three-year follow-up. J Consult Clin Psychol. 2005;73(4):721–725 PMID:
also used. Phobias usually require the help of mental health special- 16173859 https://doi.org/10.1037/0022-006X.73.4.721
ists. Selective serotonin reuptake inhibitors (see Chapter 134) have Benun J, Lewis C, Siegel M, Serwint JR. Fears and phobias. Pediatr Rev.
been found to be beneficial in the management of certain anxiety 2008;29(7):250–251 PMID: 18593756 https://doi.org/10.1542/pir.29-7-250
disorders in children and adolescents. They are noted to be effec- Burstein M, Ameli-Grillon L, Merikangas KR. Shyness versus social phobia
tive for panic disorders, social phobia, generalized anxiety disor- in US youth. Pediatrics. 2011;128(5):917–925 PMID: 22007009 https://doi.
der, obsessive-compulsive disorder, and PTSD. Benzodiazepines are org/10.1542/peds.2011-1434
safe and generally used on a short-term basis. Sedation is a frequent Compton SN, March JS, Brent D, Albano AM V, Weersing R, Curry J. Cognitive-
side effect, and there is the potential for misuse, tolerance, and drug behavioral psychotherapy for anxiety and depressive disorders in children and
dependence. Propranolol lessens the peripheral autonomic nervous adolescents: an evidence-based medicine review. J Am Acad Child Adolesc
system symptoms of social phobias and may be used for specific Psychiatry. 2004;43(8):930–959 PMID: 15266189 https://doi.org/10.1097/01.
chi.0000127589.57468.bf
instances. Combined therapy involving CBT and medication is ben-
eficial in some patients. Hanna GL, Fischer DJ, Fluent TE. Separation anxiety disorder and school refusal
in children and adolescents. Pediatr Rev. 2006;27(2):56–63 PMID: 16452275
Children who must undergo hospitalization benefit from a pre-
https://doi.org/10.1542/pir.27-2-56
hospital visit, when possible. This visit familiarizes the child with
Hoge EA, Bui E, Marques L, et al. Randomized controlled trial of mindful-
the facilities and explains proposed procedures. Many hospitals have
ness meditation for generalized anxiety disorder: effects on anxiety and stress
child life specialists who ease the adjustment of children as well as reactivity. J Clin Psychiatry. 2013;74(8):786–792 PMID: 23541163 https://doi.
their parents to the hospital stay. org/10.4088/JCP.12m08083
King NJ, Muris P, Ollendick TH. Childhood fears and phobias: assessment
Prognosis
and treatment. Child Adolesc Ment Health. 2005;10(2):50–56 https://doi.
Most childhood fears resolve with time, nurturing, and reassurance. org/10.1111/j.1475-3588.2005.00118.x
Most fears last only several weeks, and then new fears may develop. Wehry AM, Beesdo-Baum K, Hennelly MM, Connolly SD, Strawn JR. Assessment
As a rule, specific fears should not last longer than 2 years, and the and treatment of anxiety disorders in children and adolescents. Curr Psychiatry
younger the child, the shorter the duration of the fear. Rep. 2015;17(7):52 PMID: 25980507 https://doi.org/10.1007/s11920-015-0591-z

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Williams TP, Miller BD. Pharmacologic management of anxiety disorders in chil- Berenstain S, Berenstain J. The Berenstain Bears Visit the Dentist. New York, NY:
dren and adolescents. Curr Opin Pediatr. 2003;15(5):483–490 PMID: 14508297 Random House; 1981
https://doi.org/10.1097/00008480-200310000-00007 Huebner D, Matthews B. What to Do When You Worry Too Much: A Kid’s Guide
to Overcoming Anxiety. Washington, DC: Magination Press; 2006
Parents and Children
Mayer M. There’s a Nightmare in My Closet. New York, NY: Puffin Books;
Berenstain S, Berenstain J. The Berenstain Bears and the Bully. New York, NY: 1992
Random House; 1993 Ziefert H, Brown R. Nicky’s Noisy Night. New York, NY: Puffin Books; 1986
Berenstain S, Berenstain J. The Berenstain Bears in the Dark. New York, NY:
Random House; 1982

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 CHAPTER 54

Thumb-sucking
and Other Habits
Carol D. Berkowitz, MD, FAAP

CASE STUDY
A 5-year-old boy is brought to the office because of Questions
thumb-sucking. His mother claims that she has tried 1. What are common habits in children?
nearly everything, including tying his hands at night and 2. What is the significance of transitional objects?
using aversive treatments on his thumbs, but nothing 3. What are the consequences of common habits in
has worked. She reports that her son has been teased at children?
school and has few friends. He is in good general health, 4. What are strategies used to break children of habits?
and his immunizations are up-to-date. 5. How are benign habits differentiated from self-
His growth parameters are at the 50th percen- injurious behaviors?
tile. Except for a callus on the right thumb, the physical
examination is normal.

Habits are defined as somewhat complicated, repetitive behaviors after birth, and 90% of newborns show hand-sucking behavior by
that become automatized, fixed, and carried out easily and effort- the age of 2 hours. Forty percent of children between the ages of
lessly. They are different from tics, which are rapid, repetitive muscle 1 and 3 years, 33% of children between the ages of 3 and 5 years, and
twitches involving the head, face, or shoulders. Tics are also referred 25% of children at the age of 5 years still suck their thumbs. Some
to as habit spasms (see Chapter 130). Children have many habits children suck fingers rather than thumbs. Other oral behavior may
that are characteristically discouraged, such as thumb-sucking, involve lip sucking, lip biting, and toe sucking. Lip sucking and bit-
nail-biting, skin picking, nose picking, hairpulling (trichotilloma- ing begin at about 5 to 6 months of age and occur in about 90% of
nia), rocking, biting other children, and teeth grinding (bruxism). infants. It is unusual for these actions to persist as habits. Toe suck-
Some habits, such as pica (the ingestion of nonfood substances), are ing is noted in infants who are 6 to 7 months of age and is reported
potentially harmful. Children engage in most of these habits because in 80% of typically developing infants.
of their soothing potential. In recent years, cutting, a form of self- Trichotillomania is a disorder once believed to be uncommon
injury in adolescents, has received attention. While not a habit in a but now thought to affect 8 million Americans (about 5 in 1,000).
traditional sense, cutting is described by teenagers as a way of deal- The term, first coined in 1889 by French dermatologist Hallopeau, is
ing with stress and alleviating anxiety. One-third of children use derived from the Greek thrix (hair), tillein (pull), and mania (mad-
transitional objects for comfort. Blankets or favorite toys are tra- ness). The condition is an impulse control disorder in which alopecia
ditional transitional objects that represent an age-appropriate cop- develops from compulsive hairpulling. Hairpulling may involve hair
ing strategy. Most transitional objects are stroked, and the stroking from the head, eyebrows, eyelashes, or pubic area. Trichotillomania
often occurs in association with thumb-sucking. Transitional objects is reported from infancy into adulthood. In young children, boys
sometimes present a problem because children experience distress and girls are equally affected, but in older children and adolescents,
if these objects are lost or misplaced or need cleaning. females outnumber males. In preschool-age children, trichotillo-
mania is viewed as benign, similar to thumb-sucking. When the
Epidemiology condition appears in older children (most common age of onset is
Thumb-sucking probably represents the most common habit of chil- between 9 and 13 years) the condition is more likely to persist into
dren and is also reported in other primates, including chimpanzees. adulthood. The disorder is not associated with comorbid psycho-
Up to 90% of children engage in this habit at some point. Prenatal pathology, but there may be some association with mood disor-
ultrasonography has demonstrated in utero thumb-sucking in some ders or attention-deficit/hyperactivity disorder. There is a condition
fetuses. The median age for the onset of hand sucking is 54 minutes in infants, called “baby trich,” in which infants pull their mother’s

361

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 362 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

hair when they are being held or nursed. This is considered typical Teeth grinding (bruxism) is reported in 5% to 15% of chil-
exploratory behavior. dren and frequently occurs during sleep. Boys are more commonly
Rhythmic movement habits are stereotypical, repetitive behaviors affected than girls, and the disorder seems to regress later in life. It
that usually occur in infants younger than 1 year. Based on paren- is reported with increased incidence among children with develop-
tal reporting, rhythmic movements are noted in up to 15% to 20% mental delays, including those with autism spectrum disorder. The
of the population. Rhythmic movements include rocking (about cause is unknown, although it may be associated with malocclu-
19% of infants), when infants rock back and forth; jouncing (5%– sion in some children. There is some evidence that sleep bruxism in
10%), when they move in an up-and-down manner on their hands childhood is associated with an increased incidence of exposure to
and knees so that the whole crib rocks; head rolling (8%); and head secondhand smoke. The disorder may contribute to temporoman-
banging (5%). Rhythmic movements are seen more commonly in dibular joint dysfunction and pain.
boys; the male to female ratio is 3:1. These habits usually occur with Self-injury has been reported in up to 20% of adults with intel-
a frequency of 60 to 80 movements per minute, often when infants lectual disabilities. Autism spectrum disorder and the absence of
are tired, and last for less than 15 minutes before they fall asleep. In a speech are the highest associated risk factors. Nail-biting, head bang-
recent study that used home videosomnography to assess the occur- ing, and self-biting are frequently described associated behaviors.
rence of sleep-related rhythmic movements in more than 700 infants Severe self-injury related to biting is seen in Lesch-Nyhan syndrome.
and toddlers, the prevalence was significantly less, at only 2.87%.
Rhythmic movements have sometimes been referred to as sleep tics. Clinical Presentation
These tics are reported in 20% of children, most often between the ages
Children with common habits, such as thumb-sucking or rhythmic
of 6 and 10 years. As a rule, tics are 3 times more common in boys than
movements, may be brought to the physician with these particu-
girls. They tend to be noted with increased frequency in children who
lar complaints because the parent wants advice about stopping the
are shy or overly self-conscious or have obsessive-compulsive tenden-
behavior. Other children may present with consequences of hab-
cies. Tics usually occur when children are under stress.
its, such as alopecia (trichotillomania), paronychia (nail-biting), or
Biting, an aggressive habit noted in toddlers, may be related to
lead intoxication (pica). Whitlow (infection of the tip of the finger;
teething. It occurs more often in children with delayed language
also called felon) and, rarely, osteomyelitis of the distal phalanx have
development.
also been reported with nail-biting. Osteomyelitis should be con-
Nail-biting (onychophagia) is deemed to be a sign of internal ten-
sidered in a nail-biting child who presents with an abscess of the
sion and affects 10% to 40% of children. Nail-biting begins between
finger. Hairpulling can be associated with hair ingestion, also
the ages of 3 and 6 years, and the peak age is 13 years. One-third of
referred to as trichophagia. Trichobezoars that can complicate
adolescents bite their nails, but 50% of these adolescents break the
trichotillomania associated with trichophagia may present with
habit by the time they reach adulthood. When nail-biting persists
gastric outlet or bowel obstruction. Symptoms then include abdom-
into adulthood, it may be considered an oral compulsive disorder
inal pain, anorexia, early satiety, nausea, vomiting, halitosis, and
and classified under obsessive-compulsive and other disorders in the
weight loss (Box 54.1).
Diagnostic and Statistical Manual of Mental Disorders, 5th Edition
(DSM-5). The family history for nail-biting is often positive. Identical
Pathophysiology
twins are concordant for the condition in 66% of cases. In contrast,
the incidence in dizygotic twins is 34%. Children engage in habits to reduce stress and provide comfort.
Nose picking, noted in children and adults, is reported in more Thumb-sucking is related to nonnutritive sucking. Although the
than 90% of individuals. In general, adults and older children limit initial purpose of sucking is nutritional, the pleasure associated
nose picking to when they are unobserved, but younger children will with sucking reinforces the behavior. Infants who are served from a
pick their noses in public. There are no sex-based differences in the
prevalence of nose picking. Nosebleeds are the most common com-
plication of nose picking (see Chapter 90). Box 54.1. Diagnosis of Habits in
Pica is defined as the ingestion of nonfood, nonnutritive prod- Pediatric Patients
ucts. The peak prevalence of pica is between the ages of 1 and 3 years. Childhood Habits
The prevalence is increased in children of lower socioeconomic sta- ww History of a habit
tus, and the behavior occurs in 10% of children who present with ww Callus on thumb or fingers
lead poisoning. To meet the DSM-5 criteria, pica must persist for ww Short, chewed nails
longer than 1 month at an age when eating such objects is develop- ww Alopecia
mentally inappropriate and not part of a culturally sanctioned prac- ww Lead intoxication
tice. The word pica is derived from the Latin word for the magpie ww Iron deficiency anemia
(picave), a bird attributed with eating anything. Patients with pica ww Tooth surface loss
may prefer specific substances to ingest, such as ice (pagophagia), ww Masticatory muscle hypertrophy
soil (geophagy), or stones (lithophagia).

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 Chapter 54: Thumb-sucking and Other Habits 363

cup from birth develop no interest in sucking. Humans and other characterized by severe, frequent, and multiple tics (see Chapter 130).
primates spend more time in nonnutritive than in nutritive sucking. These tics are also often vocal and consist of sounds such as hiss-
Monkeys use a 5-point hold, with 2 hands, 2 feet, and mouth (holding ing, barking, grunting, or coprolalia (repeating profanities). Some
on to their mother’s nipple) for attachment. Universal thumb-sucking rhythmic habits may be mistaken for seizures but can be easily dis-
is noted even in orphan monkeys, and sucking is thought to be an tinguished because of the stereotypical, repetitive nature of the
important aspect of environmental adaptation. Nonnutritive suck- behavior.
ing occurs even in the absence of fatigue, hunger, or discomfort and Trichotillomania usually has a classic physical appearance that
has a purpose in itself—to provide comfort and be self-soothing. The has been referred to as tonsure (Friar Tuck) pattern baldness, with
maximum intensity of sucking occurs at 7 months of age. For older baldness around the vertex of the head. Unilateral temporal baldness
children (≥3 years), sucking is also a way of coping with boredom. is also a consequence of trichotillomania. The differential diagnosis
In bottle-fed infants, thumb-sucking seems to commence when of trichotillomania includes alopecia areata, tinea capitis, syphilitic
feeding stops. Some infants, described as “type A,” seem to be satis- alopecia, and androgenic alopecia (see Chapter 136). Broken hairs
fied only when their thumb is in their mouth. As infants spend more of variable length usually characterize alopecia secondary to tricho-
time engaged in motor activity, they spend less time thumb-sucking. tillomania. Other disorders in the differential diagnosis include trac-
Placid infants who cry less also do less sucking. Some studies have tion alopecia, related to tight braids or hair brushing; atopic eczema;
shown that thumb-sucking is less common in breastfed infants and seborrheic dermatitis; hypothyroidism; systemic lupus erythema-
that thumb-suckers as a group feed less frequently (every 4 hours tosus; and dermatomyositis. When trichotillomania is associated
rather than 3 and for 10 minutes rather than 20). with a trichobezoar and signs of gastric outlet obstruction, the dif-
Nail-biting is related to thumb-sucking, a form of oral gratifica- ferential diagnosis includes neuroblastoma, lymphoma, and gas-
tion, and children may progress from thumb-sucking to nail-biting. tric carcinoma.
The pattern of nail-biting usually involves placement of the hand in Cutting is not a benign habit, but it can provide stress relief,
the vicinity of the mouth, tapping of the fingers along the teeth, quick a feature of many benign childhood habits. It is usually associ-
spasmodic bites with the fingers around the central incisors, and the ated with a wide range of mental and behavioral health issues,
removal and inspection of the hands. Other oral habits, such as pen- including depression; anxiety; eating disorders, especially
cil gnawing, gum chewing, lip biting, and nail picking, are related bulimia nervosa; a history of prior sexual abuse; and obsessive-
activities, as is nose picking. The cause of teeth grinding is unclear compulsive symptoms. The mechanism by which cutting alleviates
but may be related to malocclusion. stress and anxiety has not been elucidated, but the role of endog-
Rhythmic movements are kinesthetically pleasing and soothing enous endorphins has been suggested. Cutting is felt not to rep-
and a means of autostimulation. The etiology of hairpulling is less resent suicidal behavior, but some studies differentiate the site
apparent. The DSM-5 defines trichotillomania as chronic hairpull- of cutting as predictive of suicidality: Wrist cutters, as opposed to
ing often associated with hair ingestion. In recent years, investigators arm cutters, have a higher rate of suicidal ideation and attempts.
have linked trichotillomania to disorders of serotonin reuptake and All cutters are at greater risk for suicide than the general popula-
placed it in the category of obsessive-compulsive behavior. Some indi- tion. Management generally involves referral to a mental health
viduals who engage in trichotillomania have abnormal findings on specialist and the use of psychotherapy. Other forms of self-injury
head positron emission tomography. Although the etiology of tricho- are reported with increased frequency among children with devel-
tillomania is unclear, affected children share certain features, which opmental disabilities, including autism spectrum disorder (see
have been characterized as fiddling SHEEP (sensation, hands, emo- Chapter 132).
tion, environment, perfectionism). The overriding factor is a need for
tactile stimulation. Pica, which is also considered abnormal, may be
Evaluation
associated with intellectual disability, environmental deprivation, or
inadequate nutrition, particularly iron deficiency. It may also have a History
cultural basis. Geophagy (ie, ingesting earth substances such as clay) Children who present with thumb-sucking, nail-biting, and
was related to the ingestion of kaolin, found in clay, in individuals teeth grinding usually do not require an assessment other than a
of west African origin. Such clay had antidiarrheal properties and routine health supervision history and physical examination.
helped treat dysentery and other intestinal conditions. This practice The history should determine the specific circumstances when
persisted in Georgia, which, following the slave trade, had a large pop- the habit is manifest. Is the habit more likely to emerge when the
ulation of descendants from west Africa. Adolescents with pica may child is tired or stressed? Habits must also be evaluated in the
experience stress relief when they ingest certain nonfood products. context of the child’s developmental level and home situation.
Understanding the effect of the habit on the child and family is
Differential Diagnosis important. Children who present with movements that resemble
The differential diagnosis of most habits is not difficult. Tics or tics should be carefully questioned about the frequency and dura-
habit spasms should be differentiated from Tourette syndrome, tion of the tics, the effect of the tics on their behavior, and whether
which is a neurologic disorder, reported in 1 in 3,000 children, coughing is associated with the tics (sign of Tourette syndrome).

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 364 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

The occurrence of obsessive-compulsive mannerisms should also The growth of these small hairs is uniform because children are
be noted (Box 54.2). unable to pull them out. Head shaving may not be acceptable, how-
ever, to the parent or child. Disorders such as syphilis and collagen
Physical Examination vascular diseases can be ruled out using appropriate laboratory stud-
A routine physical examination should be performed. The physical ies. Fungal infections can be differentiated by the use of appropriate
examination may reveal the sequelae of the habit, such as thumb cultures. A Wood light examination may reveal fluorescence noted
calluses, candidal infection of the nails, or evidence of malocclu- with certain fungal infections.
sion with an overbite (Figure 54.1). Children with suspected tricho- Children who present with pica should be evaluated for the pres-
tillomania should undergo a thorough assessment of their scalp in ence of iron deficiency anemia and lead poisoning. If the history
an effort to differentiate other causes of alopecia (see Chapter 136). involves geophagy, testing stool or blood for parasites may be war-
A careful neurologic examination should be performed in children ranted, especially if a complete blood cell count reveals eosinophilia.
with tics, and referral to a child neurologist may be indicated in chil-
dren with suspected neurologic disorders. Management
In general, parents should be queried about what they have done
Laboratory Studies to decrease their child’s engagement in the habit. The management
Routine laboratory studies are not needed in children diagnosed of childhood habits should be tailored to the specific habit and the
with common habit disorders. Studies are indicated if the chil- associated symptoms. For older children, self-monitoring and relax-
dren have experienced complications from the habit. For instance, ation training may be helpful as alternative means of coping with
if osteomyelitis is suspected in a nail-biting child, magnetic reso- stress. The issue of thumb-sucking versus the use of pacifiers can
nance imaging would be the study of choice. be addressed by anticipatory guidance. Pacifiers, which were previ-
Children with trichotillomania should be evaluated for the disor- ously discouraged, are now believed to have some advantages over
ders listed previously. An easy evaluation process for trichotilloma- thumb-sucking. A report from the American Academy of Pediatrics
nia involves shaving the hair in the middle of the area of baldness. noted a decrease in the incidence of sudden infant death syndrome
in infants who used a pacifier. With pacifiers, the risk of dental dis-
turbances is lower because the pacifiers are softer and are accom-
Box 54.2. What to Ask panied by a plastic shield that puts counter pressure on the teeth.
Pacifiers are also detachable and cleanable.
Childhood Habits
Pacifiers can be lost, however. Parents should be advised not
ww What about your child’s habit concerns you?
to attach a pacifier to the child’s shirt with a string because of risk of
ww Is your child experiencing any adverse consequences (eg, being teased
strangulation. For children who are pacifier dependent and unable to
at school) as a result of the habit?
go back to sleep if they lose their pacifier at night, multiple pacifiers
ww What have you done to discourage your child from engaging in the habit?
can be placed in the crib to make finding one easier. For infants who
ww Does the habit interfere with your child’s routine activities?
desire pacifiers because they complete their feeding in less than
ww Can you identify stressors in your child’s life?
20 minutes, a nipple with a smaller hole can be used or the cap can be
ww Is your child comforted by the habit?
screwed on the bottle more tightly to prolong the time spent in nutri-
tive sucking. Dental problems may develop when pacifiers are used
upside down, all day long, or after the eruption of permanent dentition.
It is suggested that parents do not try to stop thumb-sucking
behavior until children have reached the age of 4 years. Dental prob-
lems in late thumb-suckers include anterior open bite, increased
horizontal overlap (protruding upper incisors), intruded and flared
upper incisors, lingually flipped lower incisors, and warped alveolar
ridge. When thumb-sucking persists to school age, tongue thrust is
noted, as are articulation problems, specifically with consonants s, t,
d, n, z, l, and r. The physician should reassure parents that children
who stop sucking their thumbs prior to the eruption of the second-
ary dentition are not at risk for poor dentition.
Numerous devices have been proposed to help with the cessation
of thumb-sucking, but reported success has been variable. The use of
arm restraints, particularly at night, is not recommended and may
result in rumination. Bitter paints seek to reduce thumb-sucking
by subjecting children to a bitter, aversive taste. This medication
Figure 54.1. Anterior open bite associated with thumb-sucking. consists of 49% toluene, 19% isopropyl alcohol, 18% butyl acetate,

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 Chapter 54: Thumb-sucking and Other Habits 365

11% ethyl cellulose, and 0.3% denatonium benzoate. A 3/4-oz bottle is outlet into the small intestine, a phenomenon referred to as Rapunzel
toxic if ingested in its entirety. Application of aversive tasting chemi- syndrome. Gastric hair balls can be dissolved enzymatically or with
cals are used less frequently. Nocturnal application is needed if chil- the installation of a cola soda through a nasogastric tube. If such
dren suck their thumbs during the night. The principle of retraining, maneuvers fail, they are removed endoscopically or through sur-
in which thumb-sucking becomes a duty and children are required to gery. Pharmacological therapy for trichotillomania is not routinely
suck all 10 fingers one at a time, has also been recommended. Some recommended for the management of affected children. In adults,
recommend that elastic bandages be put on the hand of nocturnal selective serotonin reuptake inhibitors, clomipramine, bupropion,
thumb-suckers. Problems associated with thumb-sucking include and risperidone have been used. There are currently several mobile
sore thumbs, calluses, and candidal infections. Dentists may fash- apps that can be used to monitor behavior and assist with treatment
ion a reminder appliance, called a palatal crib or rake, making it strategies. Children whose symptoms have not improved with behav-
difficult for children to suck their thumbs. Such devices are usually ioral interventions should be referred to a mental health professional
applied for a minimum of 3 months. A fixed appliance is preferable for additional management.
to a removable one, and treatment should be initiated in spring or Nail-biting also often responds to behavior modification. As is
summer when children are engaged in numerous physical activities. sometimes used to stop thumb-sucking, denatonium benzoate, a
A number of dental devices are available commercially and online bitter chemical compound, can be applied to nails, although results
including TGuard and Hand Stopper or Thumb Sucking Handaid. are variable. Olive oil may be put on the nails to make them soft
They may consist of a plastic covering for the thumb and hand. This so there are fewer jagged edges to bite. Habit reversal therapy is
covering eliminates the pleasurable sensation created by the interac- another recommended modality to reduce biting. The promise of a
tion of thumb, saliva, and mouth. Encouragement works better than professional manicure may be an incentive for young girls to let their
nagging, as a rule, and a reward system is particularly useful in chil- nails grow. Recently, smart watches, as well as other devices worn on
dren who are 5 to 6 years of age. Parents may be referred to books the wrist, have been used to track hand movements and alert the
such as Thumbs Up, Brown Bear and encouraged to talk to their chil- individual to the biting. In the children’s book, The Berenstain Bears
dren about how good it feels not to suck their thumbs. A star chart and the Bad Habit, collecting pennies is suggested as a habit sub-
and diary are also useful. Sometimes, telling children something like, stitution for nail-biting.
“Mommy would be so proud of you if you didn’t suck your thumb now Rhythmic habits are less easy to modify. For the most part, reas-
that you’re such a big girl or a big boy,” is effective. In addition, the surance is all that is needed. The use of metronome-like devices
pressure to stop thumb-sucking becomes greater during the school has had no demonstrable effect. Children older than 3 years who
years. Children who suck their thumbs are regarded by their peers in disturb the family’s sleep with their rhythmic habits may be
first grade as less intelligent, less happy, less likable, and less desirable given mild sedatives, such as diphenhydramine or hydroxyzine.
as friends. A Cochrane review showed that orthodontic appliances Medications to reduce head banging include transdermal clonidine
and psychological intervention, both positive and negative, were suc- and thioridazine (eg, Mellaril). Other maneuvers involve placing the
cessful in stopping thumb-sucking both short- and long-term. crib or bed on carpeting or bolting the crib to the wall to decrease
In children who suck their thumbs and twirl their hair at the the amount of noise from movement.
same time, the hair twirling stops once the thumb-sucking ends. Children who engage in biting behavior should be managed with
The phenomenon is referred to as habit covariance. Hairpulling in behavior modification, including praising of good behavior and
young children often seems to resolve spontaneously but is more time-out for inappropriate behavior. Aversive conditioning involves
problematic in adolescents and adults. Management of trichotillo- the placement of some unpalatable food, such as a lemon or onion
mania usually involves non-pharmacological treatments. In chil- on a necklace, and having the child bite on that object rather than
dren, behavior modification, including putting socks on the hands biting another child. Biting behavior is reported to be extinguished
and the use of time-out for hairpulling, in addition to extra attention with this technique. Another option is the placement of a whistle.
for not pulling the hair, is recommended. Substituting behavior is The child blows the whistle rather than biting the other child.
also encouraged. For instance, children should be advised to sit on Nose picking is a common habit in children and adults. One sug-
their hands, wear gloves, pull rubber bands, or squeeze a ball when- gestion to extinguish or minimize this habit involves letting chil-
ever they have an urge to pull their hair. In older individuals, hair- dren look in a mirror and pick their nose or videotaping the child
pulling may be related to obsessive-compulsive disorders. A form while nose picking. Their reaction is that nose picking looks “gross”
of cognitive-behavioral therapy referred to as habit reversal therapy and the habit may decrease in frequency. Keeping the nasal mucosa
is said to have significant empirical support. This therapy involves moist through the application of lubricant such as petroleum jelly
an understanding of the hairpulling by the patient and then a com- will reduce the presence of dried material in the nose, which often
bination of awareness training, self-monitoring, stimulus control, provides the impetus to pick.
and competing response procedures. Habit reversal therapy is more Iron deficiency related to pica requires iron supplementation.
appropriate for older children and adolescents. Trichotillomania Lead intoxication should be managed with chelation and environ-
may lead to the presence of trichobezoars (hair balls) from swal- mental manipulation. One strategy suggested to reduce pica is to
lowed hair. Sometimes, hair balls can extend through the gastric create a “pica box.” The individual puts substitute substances, such

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 366 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

as popcorn or chewing gum, in the box, and the substitute material Borrie FR, Beam DR, Innes NP, Iheozor-Ejiofor Z. Interventions for the cessa-
is used to satisfy the urge to ingest the desired product. tion of non-nutritive sucking habits in children. Cochrane Database Syst Rev.
2015;(3):CD008694 PMID: 25825863 https://www.cochranelibrary.com/cdsr/
doi/10.1002/14651858.CD008694.pub2/full
Prognosis
Castroflorio T, Bargellini A, Rossini G, Cugliari G, Rainoldi A, Deregibus A. Risk
Most habits are not harmful to children’s health. The major problem factors related to sleep bruxism in children: a systematic literature review. Arch
is social acceptability. Parents should be encouraged to stop a habit Oral Biol. 2015;60(11):1618–1624 PMID: 26351743 https://doi.org/10.1016/
before it becomes ingrained. This can often be done by praising good j.archoralbio.2015.08.014
behavior and encouraging activities during which the unwanted Davidson L. Thumb and finger sucking. Pediatr Rev. 2008;29(6):207–208 PMID:
behavior does not appear. Habits that do not respond to parental 18515338 https://doi.org/10.1542/pir.29-6-207
influence often resolve spontaneously under peer pressure. Flessner CA, Lochner C, Stein DJ, Woods DW, Franklin ME, Keuthen NJ. Age
of onset of trichotillomania symptoms: investigating clinical correlates. J Nerv
Ment Dis. 2010;198(12):896–900 PMID: 21135642 https://doi.org/10.1097/
NMD.0b013e3181fe7423
CASE RESOLUTION
Gogo E, van Sluijs RM, Cheung T, et al. Objectively confirmed prevalence of
It is important for the physician and the mother to empower the boy to stop
sleep-related rhythmic movement disorder in pre-school children. Sleep Med.
thumb-sucking before he finds himself ridiculed by his classmates. He might be
2019;53:16–21 PMID: 30384137 https://doi.org/10.1016/j.sleep.2018.08.021
allowed to suck his thumb at certain times and in certain places (eg, “You can
suck in your room after school for 15 minutes”). Books geared at children and Golomb RG, Vavrichek SM. The Hair Pulling “Habit” and You: How to Solve
parents to help stop thumb-sucking are recommended, and the boy is rewarded the Trichotillomania Puzzle. Silver Spring, MD: Writers’ Cooperative of Greater
for times when he is not sucking his thumb. Washington; 2000
Koç O, Yildiz FD, Narci A, Sen TA. An unusual cause of gastric perfora-
tion in childhood: trichobezoar (Rapunzel syndrome). A case report. Eur
J Pediatr. 2009;168(4):495–497 PMID: 18548272 https://doi.org/10.1007/
Selected References s00431-008-0773-3

Balighian E, Tuli SY, Tuli SS, et al. Index of suspicion. Case 1: persistent fever and Morris SH, Zickgraf HF, Dingfelder HE, Franklin ME. Habit reversal training
cough following episodes of emesis in a 7-year-old girl. Case 2: blurry vision and in trichotillomania: guide for the clinician. Expert Rev Neurother. 2013;13(9):
unilateral dilated pupil in a 14-year-old girl. Case 3: swelling, pain, and erythema 1069–1077 PMID: 23964997 https://doi.org/10.1586/14737175.2013.827477
of the thumb in a 10-year-old girl with habits of nail biting and thumb suck- Panza KE, Pittenger C, Bloch MH. Age and gender correlates of pulling in pedi-
ing. Pediatr Rev. 2012;33(1):39–44 PMID: 22210932 https://doi.org/10.1542/ atric trichotillomania. J Am Acad Child Adolesc Psychiatry. 2013;52(3):241–249
pir.33-1-39 PMID: 23452681 https://doi.org/10.1016/j.jaac.2012.12.019
Beddis H, Pemberton M, Davies S. Sleep bruxism: an overview for clinicians. Tay YK, Levy ML, Metry DW. Trichotillomania in childhood: case series
Br Dent J. 2018;225(6):497–501 PMID: 30237554 https://doi.org/10.1038/ and review. Pediatrics. 2004;113(5):e494–e498 PMID: 15121993 https://doi.
sj.bdj.2018.757 org/10.1542/peds.113.5.e494

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 CHAPTER 55

Enuresis
Carol D. Berkowitz, MD, FAAP

CASE STUDY
A 9-year-old boy who is in good general health is evaluated Questions
for a history of bed-wetting. He is the product of a normal 1. What conditions account for the symptoms of
pregnancy and delivery, and he achieved his developmen- enuresis?
tal milestones at the appropriate time. The boy was toi- 2. What is the appropriate evaluation of children with
let trained by the age of 3 years, but he has never been dry enuresis?
at night for more than several days at a time. Bed-wetting 3. What is the relationship between enuresis and
occurs at least 3 to 4 times a week even if he is fluid restricted emotional stresses or psychosocial disorders?
after 6:00 pm. The boy never wets himself during the day, 4. What management plans are available for enuresis?
has normal stools, and is an average student. His father had 5. How do physicians decide which management
enuresis that resolved by the time he was 12 years old. technique is appropriate for which patients?
The boy’s physical examination is entirely normal.

Enuresis is defined as involuntary or intentional urination in chil- as attention-deficit/hyperactivity disorder (ADHD), anxiety, and
dren whose age and development suggest achievement of bladder depression, that warrant inquiring about.
control. Voiding into the bed or clothing occurs repeatedly (at least
twice a week for at least 3 consecutive months). On average, urinary
continence is reached earlier in girls than in boys, and the diagno- Epidemiology
sis of enuresis is reserved for girls older than 5 years and boys older Enuresis affects 5 to 7 million individuals in the United States. It is
than 6 years. The term diurnal enuresis, wetting that occurs dur- 1 of the most common conditions of childhood, affecting 10% to
ing the day, has been replaced by daytime incontinence. The 20% of first-grade boys and 8% to 17% of first-grade girls. By age 10
International Children’s Continence Society promotes a standard- years, 5% to 10% of boys still are enuretic (1% of US Army recruits
ization for enuresis-related terminology. It prefers the use of the are enuretic). Seventy-four percent of affected children have noc-
term incontinence to denote uncontrollable leakage of urine, inter- turnal enuresis, 10% daytime incontinence, and 16% both. Primary
mittent or continuous, that occurs after continence should have been enuresis affects the majority (75%–80%) of children with enuresis,
achieved. Nocturnal or sleep enuresis refers to involuntary urination and 80% to 85% of these children have monosymptomatic noctur-
or incontinence that occurs during the night. The term primary noc- nal enuresis. Although the overall prevalence of secondary enuresis
turnal enuresis is used when children have never achieved sustained is lower (20%–25%), it increases with age; secondary enuresis makes
dryness, and secondary enuresis is used when urinary incontinence up 50% of cases of enuresis in children 12 years of age.
recurs after 3 to 6 months of dryness. Monosymptomatic noctur- Several epidemiological factors have been associated with enure-
nal enuresis means that nighttime wetting is the only symptom. sis, including low socioeconomic status, large family size, single-
Children who experience urgency, frequency, dribbling, or other parent family, low birth weight, short height at 11 to 15 years of age,
symptoms have polysymptomatic enuresis. Such symptoms may be immature behavior, relatively low IQ, poor speech and coordination,
related to inappropriate muscle contraction, are often associated and encopresis (fecal incontinence; 5%–15% of cases). Enuresis has
with constipation, and are termed dysfunctional elimination syn- been associated with obstructive sleep apnea, in which an increased
drome or bowel/bladder dysfunction. level of atrial natriuretic factor has been reported. Atrial natriuretic
Physicians can be particularly helpful by routinely question- factor inhibits the renin-angiotensin-aldosterone pathway, causing
ing parents about bed-wetting during health supervision visits. diuresis. Correcting obstructive sleep apnea with tonsillectomy or
Many families are otherwise reluctant to bring up this embarrass- adenoidectomy can lead to the elimination of enuresis. Enuresis has
ing concern because enuresis is viewed as socially unacceptable. a familial basis, with 44% being enuretic if 1 parent was enuretic and
It poses particular difficulties if children are invited to sleep away as many as 77% of children being enuretic if both parents were sim-
from home, such as at a slumber party. In addition, enuresis may be ilarly affected. Concordance for enuresis is reported in up to 68% of
associated with other behavioral or developmental problems, such monozygotic twins and between 36% and 48% of dizygotic twins.

367

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 368 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Clinical Presentation or constipation, may be associated with urinary tract infections

(UTIs) and account for secondary enuresis.
A history of enuresis may be obtained as a presenting symptom or
ww Maturational delay. The development of the inhibitory reflex of
elicited by physicians during a health supervision visit. Medical con-
voiding may be delayed in some children, which may contribute
ditions such as encopresis, obstructive sleep apnea (nighttime snor-
to enuresis until the age of 5 years. This is similar to the range
ing), or ADHD may be associated with enuresis (Box 55.1). Children
in which children achieve other developmental milestones. It is
with ADHD have a 30% greater risk of being enuretic compared
unlikely that maturational delay persists as a cause of enuresis
with their peers who do not have ADHD. The physical examination
beyond this age. Experts believe that maturational delay is not
is usually normal.
a reasonable explanation if children can achieve dryness in the
daytime but not at night.
Pathophysiology ww Small bladder capacity. Evidence suggests that some children with
Delayed control of micturition has several possible causes (Box 55.2). enuresis have smaller than normal bladder capacities. Bladder
ww Faulty toilet training may perpetuate diurnal and nocturnal enure- capacity in ounces is estimated as the age in years plus 2. For
sis but is not expected to selectively perpetuate the latter. Parental example, 5-year-olds have a bladder capacity of 7 oz (210 mL).
expectations are believed to play a role in the toilet-training expe- Adult bladder capacity is 12 to 16 oz (360–480 mL). Small blad-
rience. Parents who allow children to sleep in overnight diapers der capacity is associated with diurnal frequency or incontinence.
or pull-ups may delay the achievement of nighttime dryness, but ww Sleep disorder/impaired arousal. The relationship between enure-
it is unlikely that the use of diapers or pull-ups causes noctur- sis and sleep has been the focus of numerous studies, some with
nal enuresis. Poor toilet habits, particularly infrequent voiding conflicting results. It has been suggested that children with enuresis
are in “deep sleep” and do not sense a full bladder. This is often the
parent’s perception of their child’s sleep pattern. However, studies
Box 55.1. Diagnosis of Enuresis in Pediatric Patients
have shown that enuresis occurs during all stages of sleep, partic-
Enuresis ularly in the first one-third of sleep and in transition from non-
ww Bed-wetting rapid eye movement (non-REM) stage 4 to rapid eye movement
ww Wet underwear (REM) sleep. During this period, body tone, respiratory rate, and
ww Old enough to be toilet trained heart rate increase, and erection and micturition occur. Studies
ww Bowel and bladder dysfunction suggest that the arousal center in the brain fails to respond (ie,
ww Precipitating problem, such as diabetes or urinary tract infection the child does not awake) to full bladder sensation. Children with
ww Encopresis enuresis do not seem to sleep more deeply than other children.
ww Attention-deficit/hyperactivity disorder However, children with enuresis may have diminished arousal
ww Family history of enuresis during sleep. In one study, 40% of children with enuresis, com-
pared with only 8.5% of children without enuresis, did not awaken
to an 80-dB noise. Other studies highlight the association of noc-
turnal enuresis with fragmented sleep, a lower proportion of
Box 55.2. Causes of Enuresis
motionless sleep, and more nighttime awakenings.
Primary Enuresis Recent studies suggest a correlation between nocturnal enure-
ww Faulty toilet training sis and periodic limb movement disorder. This disorder consists
ww Maturational delay of involuntary movement of the lower extremities (ie, knee, hip, or
ww Small bladder capacity ankle) during non-REM sleep. Periodic limb movement disorder
ww Sleep disorder/impaired arousal is related to dopamine-depletion, leading to the disinhibition of
ww Allergens spinal-cord motor and sensory reflexes. Dopamine deficit may
ww Nocturnal polyuria/relative vasopressin deficiency affect the micturition center in the brain, leading to increased
ww Dysfunctional bladder contraction bladder contractions; hence, the association with enuresis.
Secondary Enuresis ww Allergens. No evidence confirms the notion that exposure to
ww Urinary tract infection certain foods (eg, food additives, sugar) contributes to enuresis.
ww Diabetes mellitus However, some parents believe that bed-wetting is decreased if
ww Diabetes insipidus certain foods, such as sodas and sweets, are eliminated from the
ww Nocturnal seizures diet. The ingestion of caffeine-containing beverages may exac-
ww Genitourinary anomalies erbate nocturnal enuresis through the diuretic effect of caffeine.
ww Sickle cell anemia ww Nocturnal polyuria/relative vasopressin deficiency. Research has
ww Medication use shown that although children who do not have enuresis exhibit
ww Emotional stress a diurnal variation in arginine vasopressin (AVP) secretion, this
rhythm is disturbed in some children with enuresis, resulting in

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 Chapter 55: Enuresis 369

nocturnal polyuria. In addition to regulating urine formation,

Box 55.3. What to Ask
AVP also regulates circadian rhythm. Dysregulation of AVP can,
therefore, be associated with nocturnal polyuria as well as dis- Enuresis
turbed sleep. ww Is the enuresis primary or secondary?
ww Dysfunctional bladder contraction. In cases of daytime inconti- ww Is the enuresis diurnal, nocturnal, or both?
nence, contractile disturbances of the bladder affect normal void- ww How old was the child when toilet training occurred?
ing. Children with an “uninhibited bladder” have not learned to ww How old was the child when daytime and nighttime dryness was
inhibit bladder contraction. They may assume a certain posture, achieved?
called Vincent curtsy, in an effort to prevent micturition. Some ww How often does the child urinate and defecate during the average day?
children exhibit uncoordinated, incomplete voiding and the urine ww Is the child’s urinary stream forceful or dribbling?
exits the urethra in a staccato stream. Trabeculations or bladder ww Does the child dribble before or after voiding?
wall thickening may be noted on imaging studies. ww Does the child experience symptoms such as polydipsia, polyuria,
Daytime incontinence can be related to problems with blad- dysuria, urgency, frequency, or problems with passing stool?
der filling and storage or to bladder emptying. Each of these func- ww Who changes the bed and who washes the bedclothes after bed-
tions is under different neurologic control, with filling and storage wetting occurs?
under the sympathetic nervous system and bladder emptying ww Does the child wear diapers or pull-ups or use incontinence pads
related to the action of acetylcholine and the parasympathetic overnight?
system. Effective voiding requires the coordinated effort of these ww Does the child seem to delay using the toilet?
2 phases. Management of daytime incontinence is dependent on ww Does the child assume any unusual or distinct postures to avoid being
which phase is malfunctioning. incontinent?
ww What is the attitude of the family toward the child with enuresis? Are
Differential Diagnosis family members accepting or ashamed?
The differential diagnoses for both primary and secondary nocturnal ww Has the family tried any treatments yet?
enuresis are noted in Box 55.1. A specific organic problem is rarely ww Is there a family history of enuresis?
the cause of primary nocturnal enuresis, although abnormal AVP ww Does the child have other symptoms, such as encopresis, attention-
regulation may affect some children. However, secondary enuresis deficit/hyperactivity disorder, or obstructive sleep apnea?
may result from an organic problem, such as UTI, diabetes melli-
tus, diabetes insipidus, nocturnal seizures, genitourinary anomalies
(eg, ectopic ureter), sickle cell anemia, medication use (eg, diuretics,
theophylline, lithium), or emotional stress. When primary enuresis plotted. Blood pressure should be obtained. The abdomen should
is diurnal and nocturnal, some of these conditions should be consid- be assessed for evidence of organomegaly, bladder size, and fecal
ered. Additional diagnoses include neurogenic bladder, which may impaction. An anal examination should be performed to evalu-
occur in association with cerebral palsy; sacral agenesis; and myelo- ate rectal tone.
meningocele. Some children experience urinary frequency, a benign If possible, physicians should watch children void. Practitioners
self-limited condition characterized by the sudden need to urinate should determine whether children can start and stop micturition
very frequently, often 25 to 30 times a day. The condition occurs most and whether the stream is forceful. Dribbling in girls may indi-
often in children between 3 and 8 years of age, is self-limited, and is cate an ectopic ureter. A more sophisticated approach involves the
felt to be stress related. A urinary diary, noting time and amount of uroflow test, in which the patient voids into an apparatus that electri-
voiding, is sometimes helpful in diagnosing the condition. cally senses the rate of flow. A graph is generated that notes the flow
rate and quantity. This study is most useful in children with diurnal
incontinence. The appearance of the genitalia should be assessed. A
Evaluation
rash in the genital area may be secondary to wetness from urinary
History incontinence. The skin may be macerated, erythematous, or hyper-
A thorough history should be obtained when evaluating children with pigmented secondary to persistent moisture and irritation.
enuresis (Box 55.3). It may be helpful for the child or family to keep a Labial fusion in girls may trap urine, allow reflux into the vagina,
diary recording the episodes of nocturnal enuresis over several weeks and lead to dribbling. Meatal stenosis, epispadias, hypospadias,
to a month. It may be useful to note the time the child ate dinner, what or cryptorchidism may be present in boys. Any of these conditions
was eaten, and the time the child went to bed to help determine if is suggestive of a possible underlying genitourinary anomaly.
there is any discernible pattern or contributing environmental factors. Neuromuscular integrity of the lower extremities should be
evaluated. This may provide a clue to a disorder such as spina
Physical Examination bifida occulta. The presence of some anomaly in the sacral area,
A general physical examination should be performed, with par- such as a sacral dimple or a tuft of hair, may also be a sign of this
ticular attention to certain areas. The pattern of growth should be condition.

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 370 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Laboratory Tests component. Star charts should be used in conjunction with other
management strategies.
Usually the diagnosis of nocturnal enuresis is determined by the
Two treatment modalities are acceptable for managing enuresis.
history. Only a minimal laboratory evaluation is indicated in most
Most studies do not support the use of fluid restriction as a reliable
children with primary enuresis. Urinalysis, including specific grav-
isolated means of controlling enuresis. Some children benefit from
ity, is usually indicated. A complete blood cell count, serum elec-
sequential or combination therapy.
trolytes, and blood urea nitrogen should also be considered. Studies
Conditioning therapy involves the use of an alarm that is trig-
such as urine culture and blood glucose are more often indicated in
gered when children void during the night. Children are awakened
cases of secondary enuresis.
by the sounding of the alarm, and further urination is inhibited.
Some studies suggest that AVP levels be assessed, although this
Eventually, bladder distention is associated with inhibition of the
is challenging because of the instability of the molecule and the
urge to urinate. When conditioning therapy is used for 4 to 6 months,
short half-life (20 minutes). In addition, more than 90% of AVP is
it is associated with a success rate of 70%. If the alarm is used for
bound to platelets. There is a biomarker for AVP, which is a precursor
4 more weeks with sustained dryness, relapses are uncommon.
peptide, copeptin. Copeptin has been used to differentiate central
Because patient cooperation is needed with the alarm system, its
diabetes insipidus from nephrogenic diabetes insipidus and, in a
use is reserved for children age 7 years and older. There are multiple
single study to date, helped differentiate those with severe bed-
different types of alarm systems, including wireless alarms, wear-
wetting from those with milder enuresis. The role in the routine
able alarms, and pad-type alarms. For example, 1 system involves a
evaluation of children with nocturnal enuresis is yet to be defined.
transistor device that contains a small sensor in the underwear and
Imaging Studies an alarm on the wrist or collar. Some of these alarms are watches
In cases in which urinalysis is abnormal, the culture is posi- that resemble devices for measuring steps or heart rate. Most sys-
tive, or genitourinary anomalies are apparent on physical exam- tems now use vibrations so other family members are not disturbed
ination, renal ultrasonography and voiding cystourethrography by loud alarms.
may be warranted. Vertebral radiography or magnetic resonance Overall, conditioning devices have a cure rate of 70% to 85%
imaging is appropriate in the diagnosis of spina bifida. Magnetic and a relapse rate of 10% to 15%. They incur a one-time cost of $50
resonance urography is helpful in girls suspected of having an to $75, although some of the newer systems may cost up to $200.
ectopic ureter. Electroencephalography is indicated if noctur- Conditioning devices may be covered by insurance companies if the
nal epilepsy is suspected. Urodynamic studies to evaluate blad- alarm is prescribed by a physician as a medical device. Approximately
der contractility are controversial but are recommended by some 30% of families discontinue use of the alarm before the recommended
urologists in children who do not respond to traditional therapy period for various reasons. Conditioning without the use of auxiliary
or are suspected of having spina bifida occulta not revealed on alarms may also be undertaken. One proposed method involves insti-
other studies. tuting a self-awakening program. Older school-age children practice
lying in bed during the daytime and simulating the experience of
awaking, sensing a full bladder, and going to the toilet. Another dry-
Management
bed training program involves parents awaking their children first
Primary Enuresis hourly and then at longer intervals over the period of about 1 week.
Family counseling about enuresis should be part of all management Children eventually learn to self-awake. A 92% success rate with a
plans. Issues related to psychosocial stress should be explored, par- relapse rate of 20% is reported with this program.
ticularly in cases of secondary enuresis. Families should be advised Transcutaneous electrical neural stimulation therapy, which
that the wetting is not intentional and that punishing children for has been used successfully in patients with hyperactive bladder
accidents is inappropriate. However, children should be given the and polysymptomatic enuresis, has been tried in patients with
opportunity to help by removing soiled bedding or helping with the monosymptomatic enuresis with some success, but the results are
laundry. Limiting fluids and caffeinated beverages in the evening preliminary.
and having children void before bedtime are recommended steps. Pharmacological agents include tricyclic antidepressants and
Children should be rewarded for dry nights. Star charts, in which a desmopressin. Generally, medications produce a more rapid
sticker or gold star is applied to a calendar for each dry night, have response but have a higher rate of relapse. Tricyclic antidepres-
traditionally been used. Star charts and rewards are part of motiva- sants, especially imipramine, have been successfully used to treat
tional therapy, which is a recommended first-line intervention for nocturnal enuresis, although the mechanism of action is uncer-
younger children (5- to 7-year-olds) who do not wet the bed every tain. The antidepressant action of the drug, its effect on sleep and
night. Success, as defined by a 2-week period of dry nights, occurs arousal, and its anticholinergic properties may all play a role. There
in 25%, and improvement is reported in more than 70%. The exclu- is also some evidence that imipramine increases concentrations of
sive use of these charts without other interventions, however, has antidiuretic hormone. Imipramine may also act partially by reduc-
limited success and suggests that the enuresis may have a volitional ing clearance of solutes and partially by increasing urea and water

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 Chapter 55: Enuresis 371

reabsorption from the kidneys. The bladder capacity of individuals 6 to 12 years of age and 10 mg at bedtime for children older than
with enuresis treated with imipramine may be increased by 34%, 12 years. The response rate is 33%, and the major side effects include
which indicates that the anticholinergic effects of the drug may be drowsiness, flushing, dry mouth, constipation, and hyperthermia.
the most significant. Hyoscyamine sulfate and flavoxate hydrochloride are 2 other med-
Imipramine should not be prescribed for children younger than ications used for daytime incontinence.
6 or 7 years because of potential adverse effects. The recommended Treatment of enuresis in children with small bladder capacities
dosage is 0.9 to 1.5 mg/kg/day. In general, children younger than 8 includes bladder retention training. Such children are fluid loaded
years are given 25 mg 1 to 2 hours before bedtime, and older chil- and asked to delay voiding for 5 to 10 minutes. This strategy is gen-
dren are given 50 to 75 mg. Beneficial results usually occur within erally reserved for children with daytime incontinence.
the first few weeks of therapy. Medication is usually continued for 3 Associated symptoms, particularly constipation and encopresis,
to 6 months to prevent relapses, which are reported in up to 75% of should be adequately addressed.
cases. The drug should be tapered by reducing the dose or using an
alternate-night regimen. Side effects are rare and include insomnia, Secondary Enuresis
nightmares, and personality changes. Acute overdoses are poten- The management of secondary enuresis should focus on the treat-
tially fatal secondary to cardiac complications. The initial cure rate ment of the causal disorder, such as a UTI or diabetes mellitus.
is 10% to 60% with a relapse rate of 90%. The monthly cost of imip-
ramine is about $25 to $30. Because of the potential cardiotoxicity, Prognosis
reboxetine, a newer antidepressant, has been used with equal ben- The prognosis for children with enuresis is good. The spontane-
efit, although the cost may be higher. ous cure rate is 15% per year overall, although those with severe
Desmopressin, an analog of vasopressin, the antidiuretic bed-wetting (ie, 5 wet nights per week) have only a 50% chance
hormone, is another pharmacological agent used for enuresis. of achieving spontaneous remission before adulthood. Medical
Desmopressin most likely works by decreasing nocturnal urine management results in a reduction in symptoms in more than
production. Most patients who respond to desmopressin have a 70% of affected children.
large bladder capacity, large overnight urine volume, and low urine
osmolarity overnight. These patients respond rapidly to desmo-
pressin and become dry within 1 to 2 weeks of the initiation of CASE RESOLUTION
therapy. The medication is taken orally as a 0.2-mg tablet 1 hour The boy has primary nocturnal enuresis. The history of childhood enuresis in the
before bedtime. The dose may be increased by 1 tablet at weekly father is significant. Two management options, behavior modification and treat-
intervals (maximum dose: 0.6 mg). Desmopressin in nasal spray ment with desmopressin or imipramine, can be discussed with the family. The
is no longer recommended for the treatment of enuresis because child’s symptoms will probably spontaneously improve over time.
of the risk of severe hyponatremia, seizures, and even death.
Hyponatremia has also been reported with oral desmopressin
in the setting of high fluid intake sometimes associated with habit Selected References
polydipsia. Fluid restriction is recommended from 1 hour before Alloussi SH, Mürtz G, Lang C, et al. Desmopressin treatment regimens in
until 8 hours after desmopressin administration. While some phy- monosymptomatic and nonmonosymptomatic enuresis: a review from a clin-
sicians recommend a 6-month course of the medication, others ical perspective. J Pediatr Urol. 2011;7(1):10–20 PMID: 20576470 https://doi.
suggest a shorter trial period. If patients achieve a 2-week period org/10.1016/j.jpurol.2010.04.014
of dryness, the dose can be tapered at 2-week intervals. The cure Austin PF, Bauer SB, Bower W, et al. The standardization of terminology of
rate is 40% to 50% with a relapse rate of 90% off medication. There lower urinary tract function in children and adolescents: update report from the
is a biomarker, aquaporin 2, that can assess for clinical effective- Standardization Committee of the International Children’s Continence Society.
ness. The cost of desmopressin is less than when initially recom- J Urol. 2014;191(6):1863–1865.e13 PMID: 24508614 https://doi.org/10.1016/j.
juro.2014.01.110
mended, ranging from $30 to $50 a month, although some insurers
may require prior authorization for its use. Dhondt K, Baert E, Van Herzeele C, et al. Sleep fragmentation and increased peri-
odic limb movements are more common in children with nocturnal enuresis.
Some children do not respond to desmopressin. They tend to have
Acta Paediatr. 2014;103(6):e268–e272 PMID: 24612370 https://doi.org/10.1111/
small bladder capacity, low overnight urine volume, and high urine apa.12610
solute load. These patients frequently have comorbidities that require
Fagundes SN, Lebl AS, Azevedo Soster L, Sousa E Silva GJ, Silvares EF, Koch
management. In other nonresponders, excessive prostaglandin pro- VH. Monosymptomatic nocturnal enuresis in pediatric patients: multi-
duction has been noted, and some of these patients will respond to disciplinary assessment and effects of therapeutic intervention. Pediatr
nonsteroidal anti-inflammatory drugs. Nephrol. 2017;32(5):843–851 PMID: 27988804 https://doi.org/10.1007/
Oxybutynin is an antispasmodic, anticholinergic agent used s00467-016-3510-6
in the management of daytime incontinence or polysymptomatic Mercer R. Seven Steps to Nighttime Dryness: A Practical Guide for Parents of
nocturnal enuresis. The dosage is 5 mg at bedtime for children Children with Bedwetting. Ashton, MD: Brookeville Media; 2011

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 372 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Nalbantoğlu B, Yazıcı CM, Nalbantoğlu A, et al. Copeptin as a novel biomarker Van Herzeele C, Dhondt K, Roels SP, et al. Desmopressin (melt) therapy in chil-
in nocturnal enuresis. Urology. 2013;82(5):1120–1123 PMID: 23958506 https:// dren with monosymptomatic nocturnal enuresis and nocturnal polyuria
doi.org/10.1016/j.urology.2013.05.047 results in improved neuropsychological functioning and sleep. Pediatr Nephrol.
Perrin N, Sayer L, While A. The efficacy of alarm therapy versus desmopressin 2016;31(9):1477–1484 PMID: 27067081 https://doi.org/10.1007/s00467-016-3351-3
therapy in the treatment of primary mono-symptomatic nocturnal enuresis: Van Herzeele C, Walle JV, Dhondt K, Juul KV. Recent advances in managing and
a systematic review. Prim Health Care Res Dev. 2015;16(1):21-31. PMID: understanding enuresis. F1000 Res. 2017;6:1881 PMID: 29123651 https://doi.
24252606 https://doi.org/10.1017/S146342361300042X org/10.12688/f1000research.11303.1

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 CHAPTER 56

Encopresis
Carol D. Berkowitz, MD, FAAP

CASE STUDY
A 7-year-old boy is seen with a report of soiling his but distended, with palpable loops of stool-filled bowel.
underpants. His mother states that he has never been A small amount of stool is present around the anus and
completely toilet trained and that stool-related acci- in the boy’s underpants. Digital examination of the rec-
dents occur at least 2 to 3 times a week, mainly during tum reveals hard stool. The rectal tone is normal, as is the
the day. The boy rarely has a spontaneous bowel move- rest of the physical examination.
ment without assistance. He sits on the toilet for just a
few minutes and passes small, pellet-like stools. His
Questions
1. What is the definition of encopresis?
mother has not previously sought medical care for this
2. What is the difference between retentive and
problem.
nonretentive encopresis?
The boy is quite fidgety during the physical exam-
3. What are some physiologic conditions that contrib-
ination. His vital signs are normal, and his height and
ute to encopresis?
weight are at the 25th percentile. His abdomen is soft
4. What conditions may be mistaken for encopresis?

Encopresis is the voluntary or involuntary repeated passage of stool child has never been completely toilet trained. Secondary encopre-
into inappropriate places (eg, clothing) in children who, based on sis occurs in a child who has had a period of complete continence
their age, should be toilet trained (usually at least 4 years of age, the of stool. Most children with encopresis have the secondary form.
age at which 95% of children have achieved stool continence) and
who exhibit a normal developmental level and who have no primary Epidemiology
organic pathology. One such encopretic event occurs each month for Encopresis is reported in approximately 1.5% of school-age chil-
at least 3 months. The term encopresis, which was coined in 1926 dren, and boys are affected 2 to 6 times more often than girls. This
by Weissenberg and originally was used for children with psycho- sex ratio reverses in the elderly, in which the prevalence of fecal
genic soiling, is similar to enuresis (ie, urinary incontinence). Unlike incontinence is twice as high in females as in males. An associa-
enuresis, however, encopresis rarely occurs at night. Currently, tion between encopresis, enuresis, attention-deficit/hyperactivity
“encopresis” is used in a broader sense to refer to all types of disorder, and autism spectrum disorder is sometimes present.
fecal incontinence. “Functional fecal incontinence” is the currently Approximately 15% of children with enuresis also have encopresis.
preferred term as recommended by the Multinational Working Family history for encopresis may also be positive; 16% of affected
Teams to Develop Diagnostic Criteria for Functional Gastrointestinal children have 1 affected parent (usually the father). An associa-
Disorders. tion between encopresis and child sexual abuse has been reported
Retentive encopresis, also referred to as functional fecal retention in a small number of children. No reported relationship exists
with encopresis or retentive fecal incontinence, occurs in the setting between socioeconomic status, parental age, child’s birth order,
of functional constipation (ie, obstipation), in which chronic rectal or family size.
distention results in the seepage of liquid stool around hard, retained
feces. Sometimes this is called “overflow,” “fecal soiling,” or “pseu-
doincontinence,” because the individual has the potential for bowel Clinical Presentation
control. Onset of symptoms is usually approximately 4 years of age. Children with encopresis have a history of staining of the under-
Between 80% and 95% of cases of fecal incontinence are retentive. pants, which may be hidden in drawers or under beds by embar-
Nonretentive fecal incontinence is characterized by the passage of soft rassed children. Occasionally, parents are unaware of the problem.
stool without colonic distention or retention of stool. Fecal incon- Stool incontinence occurs more frequently at home than in school.
tinence in the absence of constipation is reported in up to 20% of Some children have a history of constipation. Other children may
children with encopresis. There are 2 categories of children with be initially misdiagnosed as having diarrhea and are inappropri-
nonretentive fecal incontinence: those who can control defecation ately placed on antidiarrheal medications, which exacerbate their
but who pass stool in inappropriate places and those who have true problem. Parents may complain that their child exudes a fecal
failure to achieve bowel control. Primary encopresis occurs when a odor, but children are unaware they are malodorous (Box 56.1).
373

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 374 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

Approximately one-half of children with encopresis report abdomi- include change in schedule (eg, overnight school trips with use of
nal pain, which may be vague and nonspecific or severe and crampy. communal bathrooms) and parental separation. Constipation, if
Approximately 30% to 35% experience urinary incontinence or have associated with painful defecation, may contribute to the mani-
a history of urinary tract infections (UTIs). festation of retentive fecal incontinence. With time, the colon dis-
tends and liquid feces seeps around impacted stool (Figure 56.1).
Pathophysiology In 30% to 50% of children anal spasm (ie, anismus) occurs, and
The 3 identified milestones at which a child may be at risk for the contraction rather than relaxation occurs during evacuation of
development of functional constipation are the introduction of feces. In another 40% of children, rectal hyposensitivity is appar-
dietary solids into an infant’s diet, toilet training, and the start of ent, resulting in unawareness of the presence of stool. Some chil-
school. Other factors that may precipitate secondary encopresis dren have an evacuation release disorder in which the presence
of stool does not result in relaxation and stool evacuation. In
such cases, the rectum is chronically distended by stool, water
Box 56.1. Diagnosis of Encopresis
is absorbed, and stool becomes harder and drier. The distended
ww Incontinence of stool rectum cannot sense the presence of the stool. When evacuation
ww Urinary incontinence is attempted, the process is painful, resulting in further reten-
ww Constipation tion (see Chapter 124).
ww Hyperactivity Encopresis has been associated with a short attention span and a
ww Distended abdomen high level of motor activity. Affected children are unable to sit on a
ww Stool-filled loops of bowel toilet for more than a few minutes and do not adequately attend to
ww Lax rectal tone the task of stool evacuation. As a result, they get off the toilet after
ww Soiled clothing or bedding the incomplete evacuation of only small amounts of stool. In some
ww Fecal odor toddlers, constipation is related to the struggle of toilet training and
an unwillingness to sit on the toilet (see Chapter 48).

NORMAL
RECTUM
RECTUM
DISTENDED
WITH STOOL

Stool

Superior
rectal valve

Middle Sigmoid colon

rectal valve

Inferior
rectal valve

Levator
ani muscle
Internal
Rectal
anal sphincter
sinus
Anal valve
Rectal
column

External sphincter

Anal verge

Figure 56.1. Diagram of the rectum, anal canal, and sigmoid colon distended with stool.

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 Chapter 56: Encopresis 375

The etiology of nonretentive encopresis is unclear; however, 40%

Box 56.2. What to Ask
of children with nonretentive encopresis have never been adequately
toilet trained. Comorbid psychiatric disorders as well as a history Encopresis
of sexual abuse have also been reported. Some children who have ww At what age was the child toilet trained?
been chronically sexually abused have lax anal tone, which may con- ww Does the child have spontaneous bowel movements (without enemas or
tribute to fecal incontinence. A small proportion of children with suppositories)? If so, how frequently?
nonretentive encopresis have a history of prior surgery in the rec- ww Does the child have large, dry, hard stools that clog the toilet?
tosigmoid colon for the management of conditions such as congen- ww Does the child pass blood with the stool?
ital megacolon (ie, Hirschsprung disease) and imperforate anus. The ww Did the child pass meconium within the first 24 hours after birth?
child with acute proctitis secondary to cow milk protein allergy or ww Has the child had any surgery in the anogenital area, spine, or bowel?
inflammatory bowel disease may experience fecal incontinence; in ww Is the child taking any medication that can promote constipation, such
this setting often the stool is also blood-tinged. as aspirin, iron, methylphenidate hydrochloride, imipramine, calcium
channel blocking agent, or an anticholinergic agent?
Differential Diagnosis ww Does the child seem to resist the urge to defecate (eg, squeezes legs
together and rocks back and forth)?
The differential diagnosis of encopresis focuses on organic con-
ww Does the child have a history of enuresis or attention-deficit/
ditions associated with chronic constipation. Organic conditions,
hyperactivity disorder?
which account for 5% to 10% of fecal incontinence, include con-
ww What is the pattern of encopresis (ie, is the encopresis primary or
genital megacolon (ie, Hirschsprung disease), disorders of intes-
secondary)?
tinal motility (eg, pseudo-obstruction), disorders of anal tone and
ww When and where does the child soil (eg, nocturnal, at home)?
anal anatomy (eg, imperforate anus with fistula), disorders of the
ww Have changes or stresses occurred in the home or family?
lumbosacral spine (eg, meningomyelocele), previous surgeries (eg,
ww What happens to the child’s stained underwear?
repair of imperforate anus) and neurologic disorders (eg, intellec-
ww What has the family done to manage the problem?
tual and developmental disabilities, hypotonia). Neurofibromatosis,
ww What is the child’s diet? How much milk does the child drink? Does the
lead poisoning, and hypothyroidism are also associated with consti-
child eat fruits and vegetables?
pation. Congenital anorectal anomalies, which occur in 1 in 5,000
live births, are rare.
Most of these conditions can be ruled out on the basis of a care-
ful history and physical examination. In some cases, specific test-
ing, such as anal manometry or rectal biopsy, may be necessary to encopresis have a palpable fecal mass. The rectal area should be
exclude a particular disorder. assessed for rectal tone, anal wink, and the presence of hard stool
(which may be noted in up to 90% of children with encopresis). To
Evaluation elicit an anal wink, the skin adjacent to the anus should be stroked
using a cotton swab. Some children with retentive encopresis may
History
forcibly tighten their anal sphincter and their buttocks in response
The physician should determine the age of onset of fecal inconti- to a digital examination or because they are frequently contracting
nence as well as the age of initiation of toilet training. Generally, their external anal sphincter to prevent the seepage of liquid stool.
affected children are 4 years of age or older. A detailed history of The child with a patulous anus may have a neurologic disorder
the stool pattern should be obtained as well as a dietary history and should undergo a thorough neurologic examination. Rectal pro-
(Box 56.2). The frequency, consistency, and quantity of stools should lapse, if present, may be indicative of chronic constipation or another
also be noted. Additionally, the presence of nocturnal episodes of condition (eg, cystic fibrosis). Perianal fissures may reflect passage
fecal incontinence should be determined. In cases of secondary of large, hard stools. Hemorrhoids are unusual in children and can
encopresis, the duration of prior fecal continence and the occur- be indicative of chronic constipation. Location of the anus should
rence of any events (eg, birth of a sibling, start of school) that may also be noted; anterior displacement may be suggestive of an incom-
have precipitated the episodes of encopresis should be noted. Some plete type of imperforate anus.
children experience the onset of encopresis when they start school. Dysmorphic features should be noted; these may be suggestive
Because of “toilet phobia,” they are unwilling to use the public toilet of a syndrome of which constipation is a feature. Abnormalities
in the school setting. It is important to note that not all children around the anus, such as fistulas, should also be noted. Fistulas
who experience constipation develop encopresis. are found in Crohn disease. The underpants should be evaluated
for the presence of stool, mucus, or pus. A sensory and motor
Physical Examination examination of the lower extremities helps determine if any signs
A comprehensive physical examination is necessary, paying particu- of spinal cord dysfunction are evident. An evaluation of the skin
lar attention to the abdomen to check for the presence of distended, over the spinal area may reveal abnormalities, such as sacral dim-
stool-filled loops of bowel. Fifty percent of children with retentive ples or tufts of hair.

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 376 PART 3: HEALTH MAINTENANCE AND ANTICIPATORY GUIDANCE

The child who does not exhibit abdominal distention may have such as senna derivatives, bisacodyl, or lactulose syrup, which may
soft stool on rectal examination, which is indicative of nonretentive be used in association with stool softeners or lubricants (eg, min-
encopresis. Patulous anal tone is suspicious for spinal cord abnor- eral oil). Use of mineral oil has decreased. The amount of mineral oil
malities or prior child sexual abuse. may be titrated up to ensure success. Some physicians recommend
that mineral oil be given until it oozes from the rectum, after which
Laboratory Tests the amount may be titrated back to a lower level. Magnesium sulfate
Most children with encopresis require few laboratory studies. is also recommended to relieve constipation. Magnesium citrate can
Studies are selected with a focus on eliminating organic causes be used but should be administered cautiously and with the admo-
of encopresis, such as congenital megacolon (Hirschsprung dis- nition to drink plenty of fluids to prevent dehydration. If the degree
ease) or spinal cord anomalies. Urinalysis and urine culture are of retention is more severe, suppositories or enemas may be nec-
recommended in children with fecal impactions to exclude UTI. essary. Occasionally, manual disimpaction is required. Alternative
Encopresis is reported to be an independent risk factor for UTI. methods to manual disimpaction include 2 to 3 sodium phosphate
Thyroid function studies, a lead level, celiac serology, and electro- enemas over 1 to 2 days or 226.8 g (8 oz) of mineral oil a day for
lytes, including serum calcium, have been suggested, particularly 4 days. Pulsed irrigation–enhanced evacuation involves the insertion
in children with refractory constipation. Anorectal manometry, of a rectal tube and the installation of pulses of warm irrigating solu-
which may be used to measure the pressure generated by the anal tion, simultaneously draining rectal contents. If these modalities are
sphincter, may also reveal abnormalities of anal tone or evidence unsuccessful, it may be necessary to admit the child to the hospital for
of aganglionosis. Manometry may also detect dyssynergic defec- oral administration or nasogastric lavage using polyethylene glycol-
tion characterized by failure of the muscle of the pelvis floor to electrolyte solution at 30 to 40 mL/kg/hour until successful evacua-
relax during defecation. The Child Behavior Checklist is useful to tion has occurred. This procedure requires insertion of a nasogastric
determine if certain behavior problems exist. Such problems may tube and 6 to 8 hours of treatment. After the fecal accumulation has
potentiate the encopresis or result from it. been relieved, every effort should be made to keep the child regular.
This can be accomplished with the combined use of toilet retraining,
Imaging Studies stool softeners or laxatives, and enemas or suppositories. Prokinetic
In most children with encopresis, imaging studies are not neces- agents, such as metoclopramide hydrochloride, may also be used.
sary. Abdominal radiographs obtained from children with retentive Dietary manipulation is important to ensure sustained regular
encopresis may reveal a distended, stool-filled bowel. In children passage of stool. Parents should be advised that children require a
with suspected nonretentive encopresis, abdominal radiographs high-fiber diet with fruit juices (eg, pear, peach) and decreased milk
can confirm the absence of constipation. A contrast enema with consumption (<16 oz/day). It has been suggested that a “team and
barium or a hydrosoluble substance is useful if congenital mega- coach” approach is the most successful route and that bowel train-
colon (Hirschsprung disease) or an anorectal malformation is sus- ing be likened to fitness training.
pected. Strictures, which may occur after necrotizing enterocolitis, The toilet retraining process, or “enhanced toilet training,”
are also detected on such radiographs. Electromyography to deter- requires that the child sit on the toilet at least 2 or 3 times a day,
mine whether the innervation of the external anal sphincter is intact usually after meals, for approximately 10 minutes or until the child
is recommended for the child with encopresis who does not respond has had a bowel movement. Some physicians recommend the use of
to routine treatment. an egg timer to ensure that the child spends the appropriate amount
of time on the toilet. Some children are more receptive to time on a
Management toilet if they have access to video games or shows during their time
The management of encopresis is focused on patient and parent on the toilet. Children should be requested to maintain a diary of
education and counseling with the goal of eventual complete rectal their evacuation, which may take the form of a star chart. Stars or
evacuation of stool. Typically, it takes 2 to 6 months to regain mus- other rewards are given for successful bowel movements in the toilet.
cle tone of the anal canal. The child with anorectal malformations If a child skips a day between bowel movements, a suppository
or prior gastrointestinal tract surgery may require additional surgi- may be used (eg, glycerin, bisacodyl). If after administration of the
cal procedures to help them achieve fecal continence. suppository the child still has not had a bowel movement, an enema
Rectal evacuation inevitably requires pharmacologic management may be appropriate. This sequence should be maintained until the
to ensure an adequate cleaning out of retained stools. The decision child is having bowel movements in the toilet and is not soiling for
about which laxatives to use depends on the severity of constipation. at least 1 month. Generally, it is necessary to use stool softeners for
Polyethylene glycol 3350 has a very high success rate in the manage- at least 3 to 6 months. It may be necessary to modify the regimen,
ment of constipation and encopresis and in recent years has become particularly in younger children.
the mainstay of therapy. The medication comes as a powder; gen- Behavior modification and biofeedback, such as using external
erally, 17 g (0.6 oz) is added to juice or water. The medication may anal sphincter electromyography, are 2 other modalities that can be
be given twice a day if there is no initial response. Other modalities used to help manage encopresis. Consultation with a specialist, such
include a high-fiber diet and stool-bulking agents or oral laxatives, as a pediatric gastroenterologist, may be required.

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 Chapter 56: Encopresis 377

Thirty percent of children with encopresis may need psycho- Fishman L, Rappaport L, Cousineau D, Nurko S. Early constipation and toilet
logic consultation. Psychologic intervention in the form of interac- training in children with encopresis. J Pediatr Gastroenterol Nutr. 2002;34(4):
tive parent-child family guidance has been successful when standard 385–388 PMID: 11930094 https://doi.org/10.1097/00005176-200204000-00013

gastroenterologic intervention has failed. The child with evidence Fishman L, Rappaport L, Schonwald A, Nurko S. Trends in referral to a sin-
of child sexual abuse or nonretentive encopresis should be referred gle encopresis clinic over 20 years. Pediatrics. 2003;111(5):e604–e607 PMID:
12728118 https://doi.org/10.1542/peds.111.5.e604
to a psychologist early in the course of therapy. The underlying psy-
chosocial problems of the child with nonretentive encopresis must Har AF, Croffie JM. Encopresis. Pediatr Rev. 2010;31(9):368–374 PMID:
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be adequately addressed before the condition can improve.
Kuizenga-Wessel S, Koppen IJN, Vriesman MH, et al. Attention deficit hyper-
activity disorder and functional defecation disorders in children. J Pediatr
Prognosis Gastroenterol Nutr. 2018;66(2):244–249 PMID: 28742722 https://doi.
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good with appropriate intervention. In 1 study, children who were Levitt MA, Peña A. Pediatric fecal incontinence: a surgeon’s perspective.
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5 minutes were twice as likely as those who could not do so to recover 31-3-91
from constipation and encopresis. It is estimated that approximately Loening-Baucke V. Encopresis. Curr Opin Pediatr. 2002;14(5):570–575 PMID:
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The prognosis for the child with nonretentive encopre- J Pediatr Gastroenterol Nutr. 2004;38(1):79–84 PMID: 14676600 https://doi.
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peds.2013-1580
CASE RESOLUTION
Pashankar DS, Bishop WP, Loening-Baucke V. Long-term efficacy of polyethylene
The boy exhibits typical manifestations of retentive encopresis. His condition
glycol 3350 for the treatment of chronic constipation in children with and
should be managed with the use of laxatives, stool softeners, and toilet retrain-
without encopresis. Clin Pediatr (Phila). 2003;42(9):815–819 PMID: 14686553
ing with a star chart. The possible diagnosis of attention-deficit/hyperactivity
https://doi.org/10.1177/000992280304200907
disorder should be addressed separately but may be contributing to his inabil-
ity to attend to the task of toileting. Peeters B, Noens I, Philips EM, Kuppens S, Benninga MA. Autism spectrum disor-
ders in children with functional defecation disorders. J Pediatr. 2013;163(3):873–
878 PMID: 23522863 https://doi.org/10.1016/j.jpeds.2013.02.028

Selected References Reid H, Bahar RJ. Treatment of encopresis and chronic constipation in
young children: clinical results from interactive parent-child guidance.
Benninga MA, Faure C, Hyman PE, St James Roberts I, Schechter NL, Clin Pediatr (Phila). 2006;45(2):157–164 PMID: 16528436 https://doi.
Nurko S. Childhood functional gastrointestinal disorders: neonate/toddler. org/10.1177/000992280604500207
Gastroenterology. 2016;150(6):1443–1455.e2 PMID: 27144631 https://doi. Setty R, Wershil BK, Adam HM. In brief: fecal overflow incontinence. Pediatr
org/10.1053/j.gastro.2016.02.016 Rev. 2006;27(8):e54–e55 PMID: 16882755 https://doi.org/10.1542/pir.
Borowitz SM, Cox DJ, Sutphen JL, Kovatchev B. Treatment of childhood 27-8-e54
encopresis: a randomized trial comparing three treatment protocols. J Tabbers MM, DiLorenzo C, Berger MY, et al; European Society for Pediatric
Pediatr Gastroenterol Nutr. 2002;34(4):378–384 PMID: 11930093 https://doi. Gastroenterology, Hepatology, and Nutrition; North American Society for
org/10.1097/00005176-200204000-00012 Pediatric Gastroenterology. Evaluation and treatment of functional con-
Burket RC, Cox DJ, Tam AP, et al. Does “stubbornness” have a role in pediatric stipation in infants and children: evidence-based recommendations from
constipation? J Dev Behav Pediatr. 2006;27(2):106–111 PMID: 16682873 https:// ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58(2):258–274
doi.org/10.1097/00004703-200604000-00004 PMID: 24345831

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 PART 4

Adolescent Health
57. Culturally Competent Care for Diverse Populations:
Sexual Orientation and Gender Expression....................381
58. Reproductive Health.........................................................389
59. Vaginitis.............................................................................399
60. Sexually Transmitted Infections......................................405
61. Menstrual Disorders.........................................................417
62. Disorders of the Breast.....................................................427
63. Substance Use/Abuse........................................................437
64. Eating Disorders...............................................................447
65. Body Modification: Tattooing and
Body Piercing....................................................................457
66. Depression and Suicide in Adolescents...........................465

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BPPCA6e_Ch057_0379-0388.indd 380 2/14/20 10:42 AM
 CHAPTER 57

Culturally Competent Care

for Diverse Populations:
Sexual Orientation and
Gender Expression
Ilana Sherer, MD, FAAP; Brittany Allen, MD, FAAP; Joseph H. Waters, MD; and Lynn Hunt, MD, FAAP

CASE STUDY
The mother of an 11-year-old boy makes an appoint- Questions
ment with you to discuss her son’s “behavior problems.” 1. What is meant by gender expression, sexual orienta-
He is the youngest of 4 children and is doing well in fifth tion, and gender identity?
grade, but she is concerned that her son does not like 2. What is the role of the pediatrician in counseling
typical “male” activities. He dropped out of Little League, parents and patients about gender expression,
will not join other sports teams, and prefers riding his sexual orientation, and gender identity?
bike by himself. Additionally, he still likes dressing up in 3. What are some of the consequences of discrimina-
costumes and prefers playing with girls rather than boys. tion against sexual orientation and gender identity
His mother finally mentions that she is worried that her minority populations?
son will be gay and is wondering what she can do to help 4. How can the physician help families support their
him develop “normally.” children who are lesbian, gay, bisexual, transgender,
or queer/questioning (LGBTQ+)?

Introduction gender identity and sexual orientation will come under the care of a
pediatrician during childhood and adolescence, and it is known
Lesbian, gay, bisexual, transgender, or queer/questioning (LGBTQ+)
that adult outcomes are dependent on the level of nonjudgmental
individuals are members of nearly all communities. All pediatri-
support received.
cians will have the privilege of caring for an individual from this
Language concerning sexual orientation and gender identity
population at some point in their career. Youth who are LGBTQ+
is important and constantly changing. In this chapter, the most
are a diverse and resilient population who, when supported, grow
commonly used acronym, LGBTQ+, is used to refer generally to peo-
into healthy, well-adjusted adults. The stigma associated with being
ple with diverse bodies, sexual orientations, and gender identities.
LGBTQ+ can result in a host of adverse health outcomes, however.
Longer or different acronyms are used as well, such as LGBTQIAAP+
The American Academy of Pediatrics position statement on caring
(lesbian, gay, bisexual, transgender, queer/questioning, intersex,
for LGBTQ+ youth clearly states that there is nothing inherently high
asexual, ally, and pansexual; see Table 57.1 for definitions), with the
risk or abnormal about these youth, but that stigma often causes
goal of being more inclusive and acknowledging that certain groups
psychological distress, with a resultant increase in risk behaviors.
have not been well represented by some labels or acronyms. The plus
Stigma may also be encountered in health care settings. Many
symbol at the end of LGBTQ+ is used to indicate that this popula-
LGBTQ+ youth have had negative health care experiences and often
tion includes but is not limited to people who identify as lesbian,
do not “come out” to the health professionals they consult. As a
gay, bisexual, transgender, and queer/questioning, and that a list of
result, pediatricians caring for these youth may not be aware of
letters can never be fully inclusive.
their patients’ identity. Children and adolescents of all variations of

381

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 382 PART 4: ADOLESCENT HEALTH

Table 57.1. Glossary of Terms Concerning Sex, Gender Identity, and Sexual Orientationa,b
Term Definition
Affirmed gender An individual’s true gender identity.
Agender A person who does not identify as having a particular gender.
Ally An individual who supports and stands up for the rights of LGBTQ+ persons and communities.
Asexual The sexual orientation of individuals who feel little or no attraction to others. Having an asexual orientation is
different from choosing to abstain from sex.
Bisexual The sexual orientation of individuals who develop both same-sex and opposite-sex romantic, physical, and emotional
attractions.
Cisgender A person who identifies as and expresses a gender that is consistent with the culturally defined norms of the sex they
were assigned at birth. Used as an adjective.
FTM; affirmed male; trans male/man; Terms used to describe individuals who were assigned female sex at birth but whose gender identity and/or
transmasculine expression is asserted to be more masculine.
Gay An individual whose romantic, physical, and emotional attractions (ie, sexual orientation) are to persons of the same
sex. Often it refers to men, but it may be used to describe a person of any sex with a same-sex orientation. Used as an
adjective.
Gender diverse/gender expansive Umbrella terms used to describe people with gender behaviors, appearances, or identities that are incongruent with
those that are culturally normative for their birth sex. Gender-diverse individuals may refer to themselves by many
different terms, such as transgender, nonbinary, genderqueer, gender fluid, gender creative, gender independent, or
noncisgender. Gender diverse is used to acknowledge and include the vast diversity of gender identities that exists.
It replaces the formerly used term, “gender nonconforming,” which has a negative and exclusionary connotation.
Children who do not yet have language to describe themselves as transgender may be referred to by others by
one of these terms.
Gender dysphoria A clinical symptom characterized by a sense of alienation to some or all of the physical characteristics or social roles
of one’s assigned gender. Gender dysphoria is also the psychiatric diagnosis in the DSM-5 that focuses on the distress
stemming from the incongruence between one’s expressed or experienced (ie, affirmed) gender and the gender
assigned at birth. Previous versions of the DSM included Gender Identity Disorder, which is no longer appropriate
to use but may be found in older research.
Gender expression The diverse means of communicating one’s gender to others, such as through behavior and mannerisms, clothing,
hair, voice/speech, and roles/activities. Such expression may be the result of conscious or unconscious decisions and
may or may not align with social expectations for gender identity or sex assigned at birth.
Gender identity The internal sense of one’s own gender, which may be female, male, a combination of both, somewhere in between,
or neither. Gender identity may or may not align with the social expectations for the sex an individual was assigned
at birth and results from a multifaceted interaction of biologic traits, environmental factors, self-understanding, and
cultural expectations. Gender identity is distinct from sexual orientation.
Genderqueer/nonbinary Terms to describe or name the identity of an individual whose gender identity is beyond or outside the gender binary
categories of man/male and woman/female.
Homosexual An outdated term that refers to same-sex sexual orientation. This term is often considered abrasive and offensive.
Currently preferred terms may include gay, lesbian, or queer, depending on the individual.
Intersex/differences of sex development An umbrella term used to describe the variety of conditions in which an individual’s physical sex characteristics (ie,
external genitalia, internal anatomy, chromosomes, or hormone levels) are considered atypical based on categories of
male and female. These conditions may be apparent at birth or may be diagnosed later. For some affected individuals,
intersex may also be an identity.
Lesbian A woman whose romantic, physical, and emotional attraction (ie, sexual orientation) is to other women. May be used
as an adjective or a noun.
LGBTQ+ Lesbian, gay, bisexual, transgender, or queer/questioning.
MTF; affirmed female; trans female/ Terms used to describe individuals who were assigned male sex at birth but whose gender identity and/or expression
woman; transfeminine is asserted to be more feminine.

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 Chapter 57: Culturally Competent Care for Diverse Populations: Sexual Orientation and Gender Expression 383

Table 57.1. Glossary of Terms Concerning Sex, Gender Identity, and Sexual Orientationa,b (continued )
Term Definition
Pansexual An individual whose romantic, physical, and emotional attraction (ie, sexual orientation) may be to individuals of any
sex or gender identity. Sometimes shortened to “pan.”
Queer Umbrella term used by some individuals to describe having a sexual orientation or gender identity that is beyond or
outside societal norms and expectations. Although “queer” was historically a pejorative term for LGBTQ+ people, some
LGBTQ+ individuals have reclaimed this term to describe their identities; however, it is not embraced by all members
of the LGBTQ+ community.
Sex An assignment that is made at birth, usually male or female, typically based on external genital anatomy but
sometimes on the basis of internal gonads, chromosomes, or hormone levels.
Sexual orientation A term used to describe an individual’s inherent emotional, romantic, or sexual feelings toward other persons in
relation to the sex or sexes to which they are attracted. Examples of sexual orientations include but are not limited to
gay, lesbian, bisexual, pansexual, asexual, heterosexual/straight, and queer.
Transgender An adjective used for an individual who identifies and expresses a gender that differs from the sex assigned at birth
or, more generally, who experiences or expresses gender differently from what people expect. Gender identity is
different from sexual orientation. Transgender individuals can be any sexual orientation, including but not limited to
gay, straight, lesbian, or bisexual. The term “transgender” also encompasses many other labels individuals may use to
refer to themselves.
Abbreviations: DSM-5, Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition; FTM, female to male; MTF, male to female.
a
This list is not comprehensive. It is important to recognize that language used to describe identities changes over time. PFLAG maintains a “National Glossary of Terms” at https://www.pflag.org/glossary.
b
Some definitions adapted from PFLAG. National glossary of terms. PFLAG.org website. https://www.pflag.org/glossary. Accessed July 23, 2019 as well as from Rafferty J; American Academy of Pediatrics
Committee on Psychosocial Aspects of Child and Family Health; Committee on Adolescence; Section on Lesbian, Gay, Bisexual, and Transgender Health and Wellness. Ensuring comprehensive care and
support for transgender and gender-diverse children and adolescents. Pediatrics. 2018;142(4):e20182162.

With proper knowledge, skills, and understanding, every pedia- an identity in between, or neither, whereas gender expression is the
trician can be equipped to help all LGBTQ+ youth grow and thrive way in which individuals present their gender to people around them
(Box 57.1). through outward markers, such as clothing, hair, and mannerisms.
It is important to understand and distinguish between gender iden-
Incidence tity and gender expression. Although gender expression can vary
based on cultural expectations and may be influenced by parental
It is challenging to get an accurate estimate of the number of
and peer support or rejection, gender identity is intrinsic to the self
LGBTQ+ youth in the United States. Large-scale surveys indi-
and cannot be changed by external influences.
cate that approximately 5% of the adult population identifies as
For most people, gender identity coincides with cultural norms
LGBTQ+ or reports being in or having been in a same-sex relation-
associated with their biologic or natal sex; the word used to describe
ship. Younger adults are more likely to identify as LGBTQ+; thus, it
this alignment is cisgender. For some individuals, gender identity
is likely that these percentages will continue to increase over time.
is incongruent with cultural norms associated with their biologic
The prevalence of sexual minority persons is consistent across
sex; the word used to describe this alignment is transgender. Gender
all ethnic and socioeconomic groups. Although less research
identity often develops before puberty, as young as 2 to 3 years of
is available specifically about gender identity, studies show that
age. Many children display variations in gender expression and are
0.17% to 2.7% of youth identify as transgender or gender diverse
gender expansive or gender variant throughout early childhood
in surveys of middle school, high school, and college students in
and into adolescence. Some of these children eventually identify as
the United States.
transgender and some as cisgender. Additionally, some may iden-
tify as nonbinary, genderqueer, gender-creative, or agender, mean-
Gender Identity and Sexual ing that their gender identity is something outside the binary of
Orientation male or female.
Gender identity, gender expression, and sexual orientation are 3 sep- In contrast, sexual orientation refers to an individual’s attrac-
arate concepts that are often confused and sometimes inaccurately tion to others and usually develops in late childhood and adoles-
interchanged (see Table 57.1). Gender identity refers to an individual’s cence. Traditionally, individuals who experience same-sex attraction
internal sense of self as male, female, some combination of both, have been called gay or lesbian, those who experience opposite-sex

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 384 PART 4: ADOLESCENT HEALTH

attraction have been called heterosexual or straight, and those who

Box 57.1. Best Practices for Creating
experience attraction to both sexes have been called bisexual. Like
an LGBTQ+-Friendly Office
all people, those who are transgender can be attracted to a person of
Before the Visit any sex or gender, and they may identify as straight, bisexual, gay or
ww Decorate the office with affirming rainbow stickers and posters. lesbian, or something else. It is also important to note that language
ww Brochures, books, and website images should represent the full around sexual attraction shifts with every new generation, and cur-
spectrum of gender expressions and family structures. rently it is common to hear youth use new terms and language to
ww Intake forms should include space for different family structures and more fully describe the complexities of their sex and gender as well
gender identities. The forms should include questions about preferred as to whom they are attracted.
name and gender marker. Examples of such questions can be found here:
www.ama-assn.org/delivering-care/creating-lgbtq-friendly-practice. Intersex Conditions
ww Electronic medical records should represent the patient’s preferred name
Intersex conditions are important to consider in discussions of sex-
and pronoun.
ual orientation and gender identity, but these states and identities
ww All office staff should be trained to use open-ended language when
should not be conflated. Individuals with intersex conditions, or dif-
discussing family structure and preferred name and/or pronoun.
ferences of sexual development, have bodies that differ from typical
ww Bathrooms should be gender neutral or single stall, and signage should
male or typical female medically defined sex designations. External
indicate this.
genitalia, internal anatomy, chromosomes, or hormone levels are
During the Visit atypical based on categories of male and female. These conditions
ww Provide the same level of care that you would for any patient. Use the may be apparent at birth or may be diagnosed later. Historically, and
American Academy of Pediatrics or the Society for Adolescent Health and sometimes even currently, those in the medical profession have tried
Medicine guidelines. to “correct” these bodies to align individuals’ genital appearance with
ww Use the appropriate pronoun and/or name. typical male or female definitions. These procedures have at times
ww Discuss confidentiality. Concerns about possible disclosure often are resulted in harm, such as removal of tissue important for sexual sen-
mentioned by LGBTQ+ youth as a reason to avoid seeking health care. sation, fertility, and hormone production, or performing a surgical
ww Use open-ended language during the interview. See Box 57.2 for examples sex assignment that does not align with that person’s identity as the
of language to use to ask about sexual orientation and gender identity. individual ages. Many members of the intersex community feel that
ww When asking questions about identity or behavior, explain to the such irreversible elective procedures performed on young children
youth the medical reasoning behind the question. Avoid asking are harmful and unnecessary and put children and adults at risk;
invasive questions only to satisfy curiosity. The physician should avoid instead, individuals with this view would prefer that these intersex
asking the youth to be the physician’s educator about issues of sexuality children be left intact and celebrated as existing on the continuum
and gender. of normal of healthy bodies. Additionally, individuals with certain
ww As for all youth, it is important to know about recommendations intersex conditions have increased rates of identity as a member of
for screening based on sexual risk behaviors and to avoid making a sexual or gender minority population compared with the general
assumptions about risk based on sexual orientation or gender identity. population. Although the medical needs of intersex individuals are
ww Genital examinations can be particularly traumatic for gender diverse different from those of sexual and gender minority groups, advo-
patients. Explain the medical rationale behind the examination and allow cacy for people with intersex conditions shares common themes, val-
the youth as much control over the process as possible. ues, and goals with broader advocacy for the LGBTQ+ community.
ww LGBTQ+ status is only 1 of the patient’s many identities and may not be
the main reason for the patient’s visit. It is important to conduct routine
health maintenance and devote attention to other identities important
Health Consequences of
to the individual patient, such as race, religion, and chronic illness. Discrimination
After the Visit Despite an increase in public dialogue and recent changes in some
ww The physician must be the patient’s advocate and help families and discriminatory laws, LGBTQ+ youth continue to experience stigma-
communities support the child. The physician can provide parents with tization and its consequences. Often, LGBTQ+ youth are the objects
the information offered in Box 57.3. of bullying, isolation, and family rejection. Individuals at particular
ww If referrals are necessary, the primary care physician should call ahead risk are those whose gender expression or identity differs from soci-
to ensure that the specialist or therapist is aware and supportive of the etal expectations; this is especially true for transgender women (ie,
needs of LGBTQ+ youth and families. assigned male sex at birth but with self-identified female or feminine
ww The physician should advocate for LGBTQ+ youth within the school gender identity) and people of color. National surveys of LGBTQ+
district to encourage implementation of support systems, such as peer youth show that verbal, electronic, and physical harassment in schools
organizations and anti-bullying policies. are commonplace and are associated with school avoidance and other
far-reaching consequences. Additionally, persons of sexual and gen-
Abbreviation: LGBTQ+, lesbian, gay, bisexual, transgender, or queer/questioning. der minority groups are targeted in approximately 20% of violent hate

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 Chapter 57: Culturally Competent Care for Diverse Populations: Sexual Orientation and Gender Expression 385

crimes in the United States. The result of these high rates of victim- are LGBTQ+. Because of the harm caused by this ineffective approach,
ization is that the lives of many LGBTQ+ youth have been touched reparative/conversion therapy is now banned in an increasing num-
by violence or the threat of violence. This trauma is compounded for ber of states and municipalities and is opposed by most professional
LGBTQ+ youth who are part of other vulnerable groups, such as peo- organizations, including the American Academy of Pediatrics. Although
ple with disabilities, people of color, and immigrants. such “therapies” are still practiced within some communities, pediatri-
Most LGBTQ+ youth are resilient and develop into healthy adults; cians should actively discourage families from pursuing them.
however, compared with their cisgender heterosexual peers, as a group
they are at increased risk for adverse health outcomes, such as suicide Children With LGBTQ+ Parents
attempts, substance use, and risky sexual behavior. It is important for
As many as 6 million American children and adults have a lesbian,
health professionals to understand the key mediators of these adverse
gay, bisexual, or transgender parent, and combined data suggest
health outcomes. Youth who have been victimized or who perceive dis-
that almost 2 million children younger than 18 years in the United
crimination are more vulnerable to engaging in high-risk behaviors.
States are being raised by at least 1 gay or lesbian parent. Children
Family support is particularly important for persons who are
join families with same-sex parents in a variety of ways, includ-
LGBTQ+. One study showed that LGBTQ+ young adults who experi-
ing adoption, assisted reproductive technologies, or from previous
enced higher levels of family rejection during adolescence were 8 times
heterosexual contact as stepfamilies or blended families. Like all
more likely to report suicide attempts, 6 times more likely to report sig-
families, families led by LGBTQ+ parents are diverse. These fam-
nificant depression, and 3 times more likely to use illegal drugs or engage
ilies are more likely to be composed of racial minorities, include
in unprotected sexual intercourse compared with those who did not
adopted children, include children with disabilities, and have lower
experience family rejection. Conversely, families that are highly support-
household incomes than families of opposite-sex couples. Multiple
ive are more likely to have children who are resilient and well adjusted.
longitudinal and cross-sectional studies indicate that children with
Family acceptance during adolescence is associated with better gen-
same-sex parents do well in domains of social, academic, and total
eral health, self-esteem, and social support in LGBTQ+ young adults.
competence. Many studies have shown that children in families
Youth who are LGBTQ+ are also significantly overrepresented
headed by same-sex parents in 2-parent households have outcomes
among homeless youth. Studies estimate that up to 40% of homeless
similar to children with heterosexual parents.
youth identify as a person of a sexual or gender minority population.
Youth often become homeless resulting from family conflict about their
sexual orientation or gender expression. Sexual and gender minority Important Role of Pediatricians
youth are more likely to suffer negative outcomes associated with living Pediatric health professionals have a unique opportunity to model
on the streets. Physical assault, sexual victimization, substance abuse, acceptance of each patient, provide appropriate risk-reduction
and high-risk sexual behavior are all more common for LGBTQ+ home- counseling, and encourage family support. LGBTQ+ adoles-
less youth than for heterosexual cisgender homeless youth. cents want the same attributes in their health professionals that
other groups of teenagers value, including confidentiality,
Transition and Transgender Youth honesty, respect, competence, and a nonjudgmental approach to
history taking and guidance. To maintain a supportive role, the
Transgender youth may choose to undergo a transition in which
physician must take care to avoid making assumptions. The phy-
their gender expression shifts from 1 gender to another. Transition
sician should not assume genital anatomy, the gender of part-
may be different for every individual and can take months or years.
ners, or family constellation. Neutral language should be used
It may involve social transition, which involves changing one’s name,
until the physician has completed important components of the
pronoun, and gender expression to align with the gender identity;
history (Box 57.2).
medical transition, which can involve hormones or surgeries to phys-
It is also critical never to assume information about a person’s
ically change the body; and legal transition, which involves chang-
sexual practices based on that individual’s stated gender identity
ing name and sex marker on documents, such as birth certificates
or sexual orientation, because people’s identities do not always
and passports. Decisions around transition are often informed by
align with their behavior. In 1 survey, more than twice as many
personal choice, finances, and medical barriers. It is important
youth reported same-sex sexual experiences as those who eventu-
to note that there is no such thing as a “complete transition,” and
ally identified as gay. These data reinforce the important distinc-
neither surgery nor medical intervention is necessary to legitimize
tion between sexual orientation and sexual behavior. A wide range
a person’s identity as transgender or fully male or female.
of sexual behavior exists in teenagers, and sexual identity forma-
tion is a dynamic developmental process. Teenagers who eventu-
“Reparative” or Conversion Therapy ally identify as gay may have had heterosexual sexual contact, and
Care for LGBTQ+ youth involves affirmation and support for their iden- those who identify as straight may have had a same-sex experience.
tities. Historically, some religiously and politically motivated groups For this reason, the pediatrician should be prepared to ask ques-
have used outdated and discredited medical theories to justify trying tions about sexual behaviors as well as identity when evaluating the
to “cure” the natural sexual orientation or gender identity of those who LGBTQ+ pediatric patient.

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 386 PART 4: ADOLESCENT HEALTH

Equally important is to ask for and use the individual’s preferred is acceptable to ask the individual. Health professionals can also
name and pronoun. Pronouns can be “he,” “she,” the singular “they,” show support by avoiding unnecessarily invasive questions about
or other nonbinary pronouns. It is most appropriate to refer genital status.
to people by their gender identity and not by their assigned sex. Many physicians report that they feel unprepared to care for
For example, someone assigned male at birth who identifies as LGBTQ+ individuals. Part of this process involves learning how to
female is called a transgender woman or simply a woman, and ask and how to respond when a youth answers in the affirmative.
she/her pronouns are used. The term transgender is used as an
adjective. It is not a noun, as in “transgenders,” nor a verb, as in
“transgendered.” If pronouns or preferred name are not clear, it
CASE RESOLUTION
The child is displaying behaviors that do not meet his mothers’s expectations for
male gender expression. The pediatrician should let the mother know that gen-
Box 57.2. What to Ask
der expression, sexual orientation, and gender identity are separate and distinct
Gender and that a broad range of normal exists for each of these. Neither sexual orienta-
tion nor gender identity can be predicted from the behaviors described.
ww What is your gender? What pronouns do you use?
Many adolescents go through a period of questioning their sexuality. The
ww Do you consider yourself male, female, both, or neither? child’s mother should be informed that no matter her child’s sexual orientation
ww Some people feel as though there is a mismatch between their sex or gender identity, a major risk factor for engaging in unsafe behaviors in adoles-
assigned at birth and the gender they feel themselves to be. Does that cence is parental rejection. Attempts to change a person’s sexual orientation do
resonate with you? not work and are in fact dangerous; they are associated with significant depres-
sion and thoughts of suicide. For this child to develop normally, he needs sup-
Sexuality portive adults in his life—ideally, his parents—who accept and love him.
ww To whom are you attracted? Particularly because of this child’s social withdrawal, it is important to
ww Do you have a partner or partners? determine if he has been a victim of bullying at school or on sports teams, or if
ww What is the sex of your partner(s)? he is experiencing depression. An appointment for the child should be sched-
ww Are you intimate with your partner? uled, and some time should be spent during the visit without his parents pres-
ent. This will provide an opportunity to evaluate the child’s strengths and note if
ww What parts of your body do you use for intimacy?
he is displaying any signs of anxiety or depression. The sample questions about
gender identity and sexual orientation found in Box 57.2 should be adapted
to the developmental stage of the child. The child should be assured that his
responses to these questions will be kept confidential. The pediatrician could
Box 57.3. Supportive Behaviors That Help Families also facilitate a discussion between the patient and his parents while modeling
Promote the Well-Being of Their Lesbian, Gay, support and acceptance. An ongoing dialogue with the mother will also help
Bisexual, or Transgender Child the pediatrician determine if or when referrals to support and educational
groups, such as PFLAG (formerly parents, families and friends of lesbians and
ww Talk with your child about his, her, or their lesbian, gay, bisexual, gays), are appropriate.
transgender, or queer/questioning (LGBTQ+) identity.
ww Express affection when your child tells you or when you learn that your
child is LGBTQ+.
ww Support your child’s LGBTQ+ identity even though you may feel Online Resources
uncomfortable. Health professionals recognize that patients often come to them
ww Advocate for your child anywhere he, she, or they is mistreated because having already searched online for the answers to their questions.
of an LGBTQ+ identity. What follows is a list of reliable organizations to recommend or refer
ww Insist that all family members respect your LGBTQ+ child. to for more information and support.
ww Bring your child to LGBTQ+ organizations or events.
ww Connect your child with an LGBTQ+ adult role model to show your child For LGBTQ+ Youth
positive options for the future. GLSEN (formerly the Gay, Lesbian & Straight Education Network)
ww If you are part of a faith community, work to make it supportive of www.glsen.org
LGBTQ+ members or find a supportive faith community that welcomes GLSEN strives to ensure that each member of every school com-
your family and LGBTQ+ child. munity is valued and respected regardless of sexual orientation or
ww Welcome your child’s LGBTQ+ friends and partner to your home as well gender identity or expression.
as family events and activities.
It Gets Better Project
ww Support your child’s gender expression.
www.itgetsbetter.org
ww Believe your child can have a happy future as an LGBTQ+ adult.
The website offers hundreds of videos of encouragement, and young
Adapted with permission from Ryan C. Supportive Families, Healthy Children: Helping Families with
people who are LGBTQ+ can see the ways in which love and happi-
Lesbian, Gay, Bisexual & Transgender Children. San Francisco, CA: Marian Wright Edelman Institute, ness can be a reality in their future. Straight allies can visit the web-
San Francisco State University; 2009. site and support their friends and family members.

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 Chapter 57: Culturally Competent Care for Diverse Populations: Sexual Orientation and Gender Expression 387

National Runaway Safeline The mission of this American Academy of Pediatrics section is to
www.1800runaway.org support the health and wellness of LGBT children and their parents/
1-800-RUNAWAY is a confidential and anonymous crisis hotline guardians, families, and health providers; children with variations
for runaway and homeless youth available 24 hours a day, 365 days in gender presentation; as well as LGBTQ+ pediatricians and
a year. trainees.
The Trevor Project Centers for Disease Control and Prevention Lesbian, Gay, Bisexual
www.thetrevorproject.org and Transgender Health Website
The Trevor Project is a national organization focused on crisis and www.cdc.gov/lgbthealth/index.htm
suicide prevention efforts among LGBTQ+ youth. Trained counsel- The perspectives and needs of LGBT people should be routinely
ors are ready 24/7 at 1-866-488-7386. considered in public health efforts to improve the overall health of
every person and eliminate health disparities.
For LGBTQ+ Parents and Their Children
Fenway Institute
COLAGE: People with a Lesbian, Gay, Bisexual, Transgender https://fenwayhealth.org/the-fenway-institute
or Queer Parent The Fenway Institute is dedicated to advancing the skills, atti-
www.colage.org tudes, and knowledge of clinicians and other health professionals
COLAGE is a national movement of children, youth, and adults by providing professional development, educational materials, and
with 1 or more LGBTQ+ parents. COLAGE connects people with resources on LGBTQ+ health topics. The website offers several online
LGBTQ+ parents into a peer support network and offers orga- educational modules for physicians.
nized events for families as well as youth leadership development
GLMA: Health Professionals Advancing LGBTQ Equality (formerly
opportunities.
Gay and Lesbian Medical Association)
Fenway Institute. Pathways to Parenthood for LGBT People www.glma.org
www.lgbthealtheducation.org/wp-content/uploads/Pathways-to- The mission of GLMA is to ensure equality in health care for LGBTQ+
Parenthood-for-LGBT-People.pdf individuals and health professionals. The GLMA website features an
This publication is a readily available resource for information and online provider directory and educational materials.
guidance for potential or current parents as well as health profession-
Society for Adolescent Health and Medicine
als about the various pathways to parenthood for LGBTQ+ people
www.adolescenthealth.org/Resources/Clinical-Care-Resources/
and some of the unique issues faced by LGBTQ+ parents.
Sexual-Reproductive-Health.aspx
For Parents and Others This website offers a variety of information of sexual and reproduc-
tive health information for adolescents categorized by target popu-
Family Acceptance Project
lation, including providers, parents, and teenagers.
familyproject.sfsu.edu
A research-based, culturally grounded approach to help ethnically,
socially, and religiously diverse families decrease rejection and
Selected References
increase support for their LGBTQ+ children. The website offers Baum J, Brill S, Brown J, et al. Supporting and Caring for our Gender Expansive
printable handouts in multiple languages. Youth: Lessons From the Human Rights Campaign’s Youth Survey. Washington, DC:
The Human Rights Campaign Foundation and Gender Spectrum; 2014. Available
Gender Spectrum at https://assets2.hrc.org/files/assets/resources/Gender-expansive-youth-report-
www.genderspectrum.org final.pdf?_ga=2.91064666.1704139424.1563567021-988756860.1563567021.
Gender Spectrum provides education, training, and support to help Accessed July 19, 2019
create a gender-sensitive and inclusive environment for all children Institute of Medicine Committee on Lesbian, Gay, Bisexual, and Transgender
and teenagers. It provides resources to help families, educators, pro- Health Issues and Research Gaps and Opportunities. The Health of Lesbian, Gay,
fessionals, and organizations understand and address the concepts Bisexual, and Transgender People: Building a Foundation for Better Understanding.
of gender identity and expression. Washington, DC: National Academies Press; 2011 PMID: 22013611

PFLAG (formerly Parents, Families and Friends of Lesbians and Gays) Levine DA; American Academy of Pediatrics Committee on Adolescence.
Office-based care for lesbian, gay, bisexual, transgender, and questioning youth.
https://pflag.org
Pediatrics. 2013;132(1):e297–e313 PMID: 23796737 https://doi.org/10.1542/
PFLAG promotes the health and well-being of LGBTQ+ persons,
peds.2013-1283
their families, and friends through support, education, and
Makadon HJ, Mayer KH, Potter J, Goldhammer H, eds. Fenway Guide to Lesbian,
advocacy.
Gay, Bisexual, and Transgender Health. 2nd ed. Philadelphia, PA: American
College of Physicians; 2015
For Professionals
Murchison G, Adkins D, Conard LA, et al. Supporting and Caring for Transgender
American Academy of Pediatrics Section on Lesbian, Gay,
Children. Washington, DC: Human Rights Campaign, American Academy of
Bisexual, and Transgender Health and Wellness Pediatrics, American College of Osteopathic Pediatricians; 2016. Available at https://
www.aap.org/en-us/about-the-aap/Sections/Section-on-LGBT- assets2.hrc.org/files/documents/SupportingCaringforTransChildren.pdf?_ga=2.
Health-and-Wellness/Pages/SOLGBTHW.aspx 98715550.128904594.1532306658-1967744383.1531001057. Accessed July 19, 2019

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 388 PART 4: ADOLESCENT HEALTH

Olson J, Forbes C, Belzer M. Management of the transgender adolescent. Bisexual, and Transgender Health and Wellness. Ensuring comprehensive care and
Arch Pediatr Adolesc Med. 2011;165(2):171–176 PMID: 21300658 https://doi. support for transgender and gender-diverse children and adolescents. Pediatrics.
org/10.1001/archpediatrics.2010.275 2018;142(4):e20182162 PMID: 30224363 https://doi.org/10.1542/peds.2018-2162
Olson KR, Durwood L, DeMeules M, McLaughlin KA. Mental health of transgender Ryan C, Huebner D, Diaz RM, Sanchez J. Family rejection as a predictor of nega-
children who are supported in their identities. Pediatrics. 2016;137(3):e20153223 tive health outcomes in white and Latino lesbian, gay, and bisexual young adults.
PMID: 26921285 https://doi.org/10.1542/peds.2015-3223 Pediatrics. 2009;123(1):346–352 PMID: 19117902 https://doi.org/10.1542/
Perrin EC, Siegel BS; American Academy of Pediatrics Committee on peds.2007-3524
Psychosocial Aspects of Child and Family Health. Promoting the well-being of Sherer I, Baum J, Ehrensaft D, et al. Affirming gender: caring for gender-atypical
children whose parents are gay or lesbian. Pediatrics. 2013;131(4):e1374–e1383 children and adolescents. Contemporary Pediatrics. 2015;32:16–19
PMID: 23519940 https://doi.org/10.1542/peds.2013-0377
Rafferty J; American Academy of Pediatrics Committee on Psychosocial Aspects
of Child and Family Health; Committee on Adolescence; Section on Lesbian, Gay,

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 CHAPTER 58

Reproductive Health
Monica Sifuentes, MD

CASE STUDY
An 18-year-old female college student in good health Questions
comes in for a routine health maintenance visit during her 1. What issues are important to discuss with adoles-
spring break. She is unaccompanied by her parents and has cents at reproductive health maintenance visits?
no complaints, stating that she just needs a checkup. She 2. What are the indications for a complete pelvic
enjoys college, passed all her fall and winter classes, and examination?
has some new friends. She denies tobacco use but says 3. When is a Papanicolaou test indicated as a part of
many of her friends smoke e-cigarettes. She occasionally the reproductive health visit?
drinks alcohol and has tried marijuana once. Although 4. What methods of contraception are most success-
she is not currently sexually active, she is interested in ful in adolescent patients? What factors about each
discussing contraceptive options. Her last menstrual method should be considered?
period, which occurred 2 weeks previously, was normal. 5. What are the legal issues involved in prescribing
She is taking no medications. Her physical examination contraception to minors in the absence of paren-
is entirely normal. tal consent?

Adolescent visits to primary care physicians are relatively infre- Aside from issues of sexual activity, the adolescent also may have
quent by the time teenagers reach puberty. At most, the healthy questions about the progression through puberty. Normal variants
adolescent patient is seen once or twice during high school for in body habitus or certain physical characteristics can be a source
preparticipation sports or camp physicals. If an adolescent is of unnecessary anxiety for the uninformed teenager. Health educa-
not involved in athletics or if activities in which the adolescent tion to alleviate these fears is ideal. The adolescent who is seen for
is involved do not require periodic assessments, such a teenager a health maintenance examination should be allotted extra time
will rarely visit a health professional while in high school except so that topics such as puberty, abstinence, gender identity, sexual
for an acute illness. Therefore, it is extremely important to use any behaviors and activity, STIs, and contraception can be discussed.
interaction with an adolescent as a unique opportunity to provide Additionally, during more acute, problem-oriented visits, the adoles-
anticipatory guidance and health education, particularly reproduc- cent should be encouraged to voice any other concerns he or she may
tive health education. This chapter is largely devoted to a discus- have. Depending on the nature of these issues, follow-up appoint-
sion of the reproductive health of adolescent females. However, ments can be scheduled.
the Evaluation section is divided into 2 sections, 1 for females
and 1 for males. The reader is referred to Chapter 60 for more
information on sexually transmitted infections.
Normal Secondary Sexual
Reproductive health is multidimensional and includes sexuality- Development
related services, screening for communicable infections, anticipa- Puberty begins during early adolescence with the development of
tory guidance, and counseling. Such services should be included secondary sexual characteristics. Because of the tremendous vari-
as a part of the routine health maintenance examination for male ation in the age, duration between pubertal stages, and somatic
and female adolescents for several reasons. The high incidence growth of adolescents, a sexual maturity rating (SMR [ie, Tanner
of sexually transmitted infections (STIs) in this age group, the stage]) is used to describe breast and pubic hair development in
risk of acquiring HIV, and the reality of an unplanned pregnancy females and genital development and pubic hair growth in males
make reproductive health issues increasingly important for teen- (Figures 58.1, 58.2, and 58.3). The average age of menarche in the
agers and young adults. Additionally, adolescents rarely schedule United States is 12.5 years, which for most females occurs during
appointments with primary care physicians prior to the initiation SMR 3 and 4. In contrast, spermarche occurs early in pubertal devel-
of coitus. Experimentation with drugs and alcohol at this time opment in boys, at approximately 13 years of age, with little to no
in their lives also contributes to early, unplanned sexual experi- pubic hair development. Full fertility is generally achieved by age
ences (Box 58.1). 15 years, or mid-adolescence, in most boys and girls.

389

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 390 PART 4: ADOLESCENT HEALTH

Box 58.1. Reproductive Health: Sexual Activity

Statistics on sexual activity among adolescents in the United States have changed over the last decade. Previously, it was reported that 1 in 4 females and 1 in
3 males had had sexual intercourse by 15 years of age. Currently, only 13% of teenagers have ever had vaginal intercourse by age 15, according to the Guttmacher
Institute. Currently, most adolescents are waiting to initiate sexual activity; by their 19th birthday, 7 in 10 teenagers of either sex have had sexual intercourse.
Contraceptive use at first premarital sexual encounter has increased to nearly 80% in adolescent females and 87% in adolescent males; however, unintended
pregnancy and sexually transmitted infections (STIs) continue to be a major public health concern for this age group. Although the pregnancy rate among
teenagers has dropped steadily over the past 10 years, each year nearly 850,000 adolescent females younger than 20 years become pregnant. Most of these
pregnancies are unintended and occur premaritally, especially among certain racial and ethnic minority groups. The outcome of these pregnancies in 15- to
19-year-olds varies. An estimated 50% to 60% of these pregnancies result in live births, 30% end in abortion, and 10% to 15% are miscarried or stillborn.
Unprotected sexual activity among adolescents has several adverse health consequences, the most obvious being teenage pregnancy. Of the adolescents who
continue their pregnancies, preterm birth (<37 weeks’ gestational age) and low birth weight (<2,500 g [5.5 lb]) are 2 of the most frequently reported neonatal
complications. Long-term maternal psychosocial sequelae of adolescent pregnancy include undereducation/school failure, limited vocational training and skills,
economic dependency on public assistance, subsequent births, social isolation, depression, and high rates of separation and divorce among teenage couples.
In addition to unintended pregnancy, the risk of contracting an STI, such as chlamydia, human papillomavirus, herpes, and HIV, is increased. In cases of pelvic
inflammatory disease from gonorrhea or chlamydia, future problems with fertility and an increased risk of ectopic pregnancy can occur. Human papillomavirus, which
is associated with the development of genital warts, cervical dysplasia, and cancer, accounts for approximately one-half of STIs diagnosed in adolescents and young
adults. The prevalence rates of other STIs, such as chlamydia and gonorrhea, are still highest among 15- to 19-year-old females compared with older age groups in
the United States. More alarming, however, is the relationship between AIDS in young adults aged 20 to 29 years and probable exposure to HIV during adolescence.
Many factors have been associated with the initiation of early coitus in adolescents. They include male sex; race/ethnicity; poverty; a large, single-parent
family; previous teenage pregnancy in the household, whether of the mother or a sibling; poor academic achievement; discrepancy between the onset of
physical puberty and cognitive development; peer group encouragement; and problem behaviors, such as drug use. Additionally, religious affiliation and
cultural norms likely influence this decision. The role of hormonal changes during puberty and their influence on behavior remains unknown.
The adolescent with an intellectual disability that may or may not be associated with chronic illness requires special consideration in terms of reproductive health. With
recent advances in medical therapy for conditions such as diabetes mellitus and sickle cell disease, many of these adolescents experience normal pubertal development and
fertility. Like their healthy peers, some begin engaging in sexual intercourse at an early age. Unintended pregnancy and childbirth can exacerbate some chronic illnesses
and can increase health risks significantly for both the adolescent and developing fetus. The genetic implications and specific patterns of inheritance of certain medical
conditions must also be considered. Thus, attention to sexual issues is essential for the adolescent or young adult with chronic medical illness and/or intellectual disability.

1 2 3

4 5

Figure 58.1. Female pubic hair development. Sexual maturity rating 1: prepubertal, no pubic hair. Sexual maturity
rating 2: straight hair is extending along the labia and between ratings 2 and 3, begins on the symphysis pubis. Sexual
maturity rating 3: pubic hair is increased in quantity; is darker, coarser, and curlier; and is present in the typical female
triangle. Sexual maturity rating 4: pubic hair is more dense, curled, and adult in distribution but is less abundant.
Sexual maturity rating 5: abundant, adult-type pattern; hair may extend on the medial aspect of the thighs.

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 CHAPTER 58: REPRODUCTIVE HEALTH 391

1 2 3

4 5

Figure 58.2. Female breast development. Sexual maturity rating 1: prepubertal, elevations of papilla only. Sexual
maturity rating 2: breast buds appear, areola is slightly widened and projects as small mound. Sexual maturity
rating 3: enlargement of the entire breast with no protrusion of the papilla or of the nipple. Sexual maturity
rating 4: enlargement of the breast and projection of areola and papilla as a secondary mound. Sexual maturity
rating 5: adult configuration of the breast with protrusion of the nipple, areola no longer projects separately from
remainder of breast.

1 2 3

4 5

Figure 58.3. Male genital and pubic hair development. Sexual maturity rating 1: prepubertal, no pubic hair, genitalia
unchanged from early childhood. Sexual maturity rating 2: light, downy hair develops laterally and later becomes
dark; penis and testes may be slightly larger; scrotum becomes more textured. Sexual maturity rating 3: pubic hair is
extended across pubis; testes and scrotum are further enlarged; penis is larger, especially in length. Sexual maturity
rating 4: more abundant pubic hair with curling, genitalia resemble those of an adult, glans has become darker.
Sexual maturity rating 5: adult quantity and pattern of pubic hair, with hair present along the inner borders of the
thighs. The testes and scrotum are adult in size.

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 392 PART 4: ADOLESCENT HEALTH

Evaluation Box 58.3. What to Ask

History
Contraceptive Use
The history obtained at a reproductive health visit should include
ww Does the adolescent use condoms never, sometimes, or always?
2 parts: the medical history, which in females focuses primarily on
ww Is any other method of birth control also used?
the gynecologic history, and the psychosocial interview. Regardless
ww Is the adolescent female currently using oral contraceptives?
of the type of visit scheduled, the physician should take a few
—— If so, what particular type is she taking, and how long has she been
moments at the beginning of the interview to address routine health
using this method of contraception?
maintenance issues with the adolescent (Box 58.2). For the female
—— How often does she miss taking the pill? What does she do when
patient, the primary care physician should ask whether she has ever
she fails to take the pill?
undergone a genital or pelvic examination. Additionally, current
—— Does she experience common minor side effects, such as break-
methods of contraceptive use, if any, should be reviewed (Box 58.3).
through bleeding, headache, or nausea?
If the adolescent female has just started taking oral contraceptives,
ww Is the adolescent female using a long-acting progestin, such as
compliance, common side effects, and a review of the more emer-
Depo-Provera? If so, has she experienced irregular bleeding, weight
gent complications of birth control pills is warranted, especially at
gain, hair loss, headache, or acne?
an initial visit. The acronym ACHES (abdominal pain, chest pain,
ww Is there another method of contraception that the adolescent has used
headaches, eye problems, severe leg pain) is useful to remember life-
or might be interested in discussing or starting, such as long-acting
threatening reactions that can be associated with hormonal contra-
reversible contraception?
ceptive use (Box 58.4), although these reactions are uncommon in
ww What does the adolescent know about emergency contraception?
otherwise healthy adolescent girls. If the adolescent is using another

Box 58.2. What to Ask Box 58.4. Danger Signs Associated With
Oral Contraceptive Use
Reproductive Health
For Males and Females A Abdominal pain (severe)
ww How is the adolescent feeling overall? C Chest pain (severe) with shortness of breath
ww Has the adolescent had any recent illnesses or conditions that the health H Headaches
professional should know about? E Eye problems (visual loss or blur)
ww When was the last physical examination performed? Did it include a S Severe leg pain (calf and/or thigh)
genital or pelvic examination?
ww Is the adolescent sexually active?
—— If so, are their sexual relationships with males, females, or both? form of contraception, adherence to and satisfaction with the partic-
—— When was the last episode of vaginal or anal intercourse? ular method should be reviewed along with the respective common
—— Was the last episode of sexual intercourse protected or unprotected? side effects. The physician should specifically inquire if the adoles-
—— Does the adolescent have oral sex? cent desires to continue the same method of birth control or is inter-
—— How old was the adolescent when he or she they began having ested in another method.
sexual relationships? Was it consensual? Coerced? Forced? The remainder of the psychosocial history, otherwise known as
—— How many sexual partners does the patient have currently? How the HEADSS assessment (home, employment and education, activ-
many sexual partners has the patient had in his or her lifetime? ities, drugs, sexuality, suicide/depression), should be completed
ww Is there any history of or ongoing physical or sexual abuse? regardless whether the adolescent is currently sexually active (see
ww Has the adolescent or any of the adolescent’s partners ever been treated Chapter 4). Risk factors for an unplanned pregnancy or uninten-
for a sexually transmitted infection or tested for HIV? tional exposures to STIs should be kept in mind when formulating
a health care plan with the teenager.
For Females Only
ww What was the age at menarche? Physical Examination
ww What was the date of the last menstrual period and the duration and A complete physical examination should be performed on all ado-
amount of flow? lescents, with particular attention paid to SMR, blood pressure, and
—— Are any symptoms, such as cramping, bloating, or vomiting, growth chart. A chaperone should be present during the physical
associated with menses? examination, particularly during the breast and genital examina-
—— Are any of these symptoms incapacitating? Do they cause the tion, even if the patient and examiner are the same sex.
adolescent to miss school or work?
Males
—— Does the mother or do any siblings have similar problems? If so, how
do they manage them, if at all? The genitalia should be examined closely for penile and testicu-
lar size, distribution of pubic hair, and presence of any ulcerative,

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 CHAPTER 58: REPRODUCTIVE HEALTH 393

vesicular, or wart-like lesions. Any urethral erythema or discharge that may indicate an otherwise asymptomatic chlamydial infection
should be noted. Testicular masses require further evaluation. should be noted. If a pelvic examination is indicated, a vaginal dis-
Ideally, the physician should use this opportunity to teach the male charge may be appreciated before inserting the speculum; ideally,
adolescent how to perform a testicular self-examination. however, the cervix should be examined for cervical ectopy, friabil-
ity, and any lesions or discharge from the os. The vaginal mucosa
Females
should also be inspected as the speculum is withdrawn.
Before performing the physical examination, the physician should During the bimanual examination, the cervix should be palpated
determine whether a full-speculum examination is indicated for any cervical motion tenderness. Uterine size and position should
(Box 58.5). This decision should be based on the details of the indi- be appreciated, and adnexal tenderness or masses should be noted.
vidual case and not solely on the basis of sexual activity. With the Because normal ovaries are approximately the size of almonds, many
advent of noninvasive screening methods for STIs, a routine pelvic physicians do not palpate them. A rectovaginal examination is nec-
examination often is unnecessary and not required before initiat- essary to rule out fistulas, especially in the postpartum adolescent.
ing contraception. Most experts now recommend the use of urine or If the physician is unable to perform a vaginal bimanual examina-
vaginal-based nucleic acid amplification tests (NAATs) to screen for tion, a rectoabdominal examination can be done to assess uterine
gonorrhea or chlamydia in lieu of endocervical swabs. If a speculum size and position and the presence of adnexal masses.
examination is indicated, however, proper preparation of the ado-
lescent is imperative. This should include an explanation of the pro- Laboratory Tests
cedure and the physical sensations felt while the speculum is being Because most Chlamydia trachomatis and Neisseria gonorrhoeae
inserted and the endocervical specimen is being obtained. In addi- infections in adolescents are asymptomatic, screening for these
tion to a chaperone, the choice of who should be present during the organisms via noninvasive urine-based or vaginal NAATs is recom-
examination (eg, parent or friend who may have accompanied the mended annually in all sexually active adolescents and more fre-
patient) and a discussion of the desired positioning (ie, supine or quently in those who have a history of unprotected intercourse or a
semi-sitting) are also important points to review with the patient. new sexual partner. If the adolescent has a vaginal discharge or cer-
Additionally, the speculum, specimen swabs, and other equipment vical friability noted on speculum examination, the specimen should
should be shown to the adolescent before she is draped. The goal is be obtained directly from the endocervix. A saline and potassium
to minimize the adolescent’s fears, anxieties, misconceptions, and hydroxide wet mount should also be collected from the symptom-
discomfort about the examination. atic patient. A Papanicolaou (Pap) smear should be performed in
A breast examination should be performed on female adoles- sexually active females who are 21 years or older. Although the Pap
cents, and any breast tenderness, nodularity, or masses should be smear may detect Trichomonas vaginalis or the cytologic changes
noted. This portion of the examination can be used to educate the associated with human papillomavirus infection, routine screen-
patient about the purpose and importance of breast self-examinations ing in asymptomatic adolescents is not recommended. A NAAT
and to document breast SMR. (eg, polymerase chain reaction) for human herpesvirus should be
The external genitalia should be examined in all adolescent performed if painful vesicles are noted on examination. As clinically
females at least once during puberty regardless whether they are indicated, a fresh vesicle can also be unroofed and a specimen sent
sexually active. The SMR and any congenital anomalies, such as for herpesvirus cell culture or NAAT.
asymmetric enlargement of the labia minora or an imperforate Other laboratory tests include a rapid plasma reagin test for syph-
hymen, should be identified. In the sexually active adolescent, the ilis and an HIV screening test in the adolescent identified as high
external genitalia should be carefully examined for warts, ulcers, risk or at least annually. Baseline complete blood cell count, liver
and vesicular lesions. Any urethral erythema, edema, or discharge function tests, cholesterol, and hemoglobin A1C may be indicated
as part of the health maintenance visit but are not required before
starting contraception. A pregnancy test is warranted in the sexually
active female if the physician chooses to begin oral contraceptives or
Box 58.5. Indications for a Complete
another method of hormonal contraception mid-cycle (“quick start”
Pelvic Examination
method) or if menses are late.
ww Pregnancy
ww Request by the adolescent Management
ww Unexplained lower abdominal pain
Reproductive Health Education
ww Persistent abnormal vaginal discharge
ww Unexplained vaginal bleeding All management plans during reproductive health visits should
ww Dysmenorrhea that is unresponsive to nonsteroidal anti-inflammatory include a frank discussion of puberty, gender identity, sexual orien-
drugs tation, sexual behavior, and STIs regardless of current or prospec-
ww Suspected or reported sexual assault tive sexual activity. The adolescent also should be counseled about
ww Perform a Papanicolaou test abstinence as an acceptable choice. Ideally, preventive health care
measures, such as breast and testicular self-examinations, have been

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 394 PART 4: ADOLESCENT HEALTH

reviewed during the physical examination. It is hoped that by encour- should be discussed to maximize effectiveness, minimize discontin-
aging adolescents’ familiarity with these self-examinations, they will uation, and avoid contraceptive failures.
continue to perform them throughout their adult lives. The use of Barrier methods include male and female condoms, which have
posters, plastic models, and electronic or written materials to rein- a typical pregnancy failure rate of 15% when used alone but provide
force the discussion is strongly encouraged. The goal of reproductive protection against many STIs. Nonlatex male condoms are available
health education is to assist adolescents in identifying and commu- for individuals with latex allergies; however, these condoms have an
nicating their thoughts and feelings about sexual abstinence as well increased risk of slippage and breakage. Vaginal spermicides, such
as sexual activity and to aid in the prevention of unintended preg- as nonoxynol-9, are available in a variety of forms (ie, foams, gels,
nancy, young parenthood, and STIs. Prevention programs offered by films, suppositories) and should be used in conjunction with a bar-
schools must be supplemented by open parental communication in rier method because general concerns exist that these products in
the home about sexuality, although this varies considerably by family. high doses can increase the risk of genital ulceration and irritation,
thereby facilitating STI acquisition.
Legal Issues Hormonal contraceptive methods include combination oral con-
The issue of confidentiality is important to consider when providing traceptives (COCs), the transdermal patch, the intravaginal ring,
reproductive health care for the adolescent. Parental involvement injectable long-acting progestin agents, and long-acting reversible
should be strongly encouraged; however, health professionals are contraceptives, such as the subdermal implantable rod and intra-
not required to disclose any confidential information to parents or uterine devices (IUDs). Although previously not recommended but
guardians except in cases of suicidal ideation, harmful intent to oth- now offered for nulliparous teenagers, the levonorgestrel IUD is a
ers, and sexual or physical abuse. In most states, contraceptive ser- highly effective and cost-efficient reversible contraceptive method
vices can be provided to adolescents age 12 years and older without that requires little compliance, making it especially useful for adoles-
specific knowledge or consent of a parent or guardian. In the United cents. Previous reports about increased rates of pelvic inflammatory
States, the complex issue of parental consent and pregnancy ter- disease in teenagers with IUDs are unsubstantiated. The withdrawal
mination varies from state to state and should be reviewed by the method, or coitus interruptus, and natural family planning are inef-
individual health professional based on the state in which that indi- fective methods for adolescents for protection against pregnancy.
vidual practices medicine. All 50 states and the District of Columbia
explicitly allow minors to consent for their own health services for Hormonal Contraception
STIs and do not require parental consent for STI care. Providing Combination oral contraceptives are an effective means of birth
confidential care for adolescents enrolled in private health insur- control for adolescents, with most pills containing 30 to 35 mcg
ance plans, however, remains a difficult issue because many states (“low dose”) to 20 mcg (“very low dose”) of ethinyl estradiol (ie, a
mandate that health plans provide a written statement to the ben- synthetic estrogen) and a progestin. Monophasic pills contain a fixed
eficiary about the services covered and received, including clinical dose of estrogen and progestin throughout the 21-day pill cycle.
services provided confidentially to teenagers. Biphasic preparations contain a lower dose of the progestin com-
ponent during the first 10 days of the cycle but are rarely used in
Pap Smear teenagers. In triphasic pills, the doses of estrogen and progestin, or
The recommendation of the American College of Obstetricians the progestin component alone, are varied 3 times throughout the
and Gynecologists is that cervical cancer screening should begin at cycle. This contraceptive was created to decrease the overall progestin-
21 years of age. After the initiation of screening, a Pap smear for related side effects, such as hypertension, acne, and lipid abnor-
average-risk women age 21 through 29 years should be performed malities, but has not been shown to have any great advantage over
every 3 years. The rationale for not screening teenagers for cervi- a monophasic pill. Most recently, very low-dose monophasic estrogen
cal cancer is that there is little risk in not treating abnormal cervical (20 mcg) pills have been developed to minimize estrogen-related
cytology in adolescents because in this age group 90% to 95% of low- side effects and decrease discontinuation rates. However, 20-mcg
grade lesions, as well as many high-grade lesions, regress to normal estrogen pills may be associated with a higher rate of intermen-
spontaneously. Premature screening can result in an overdiagnosis strual bleeding and less bone mass acquisition than 30- to 35-mcg
of cervical dysplasia and an overtreatment of lesions with poten- pills, especially in young patients. Because of this, monophasic pills
tially harmful procedures, such as excision or ablation of the cervix. with 30 to 35 mcg of ethinyl estradiol are considered the first-line
Because the incidence of cervical cancer is quite low among adoles- therapy for most teenagers who wish to use COC. Specific instances
cents, the benefits of the Pap test are offset by the potential harm may exist, however, in which the lower efficacy of these pills must
of unnecessary procedures and treatments in this young age group. be taken into account and the ethinyl estradiol dose increased to
50 mcg, such as in patients concurrently receiving medications that
Contraceptive Methods increase the metabolism of synthetic steroids (eg, certain anticon-
The appropriate method of contraception should be individualized vulsant agents).
according to the needs and acceptability for each adolescent. The Another COC regimen is 3-month continuous hormonal ther-
risks, benefits, and limitations of the various contraceptive modalities apy followed by 1 week of withdrawal bleeding for young women

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 CHAPTER 58: REPRODUCTIVE HEALTH 395

who prefer to menstruate only 4 times a year, that is, an extended- than 198 lb (>90 kg). Additionally, concerns have been cited by
cycle pill. To reduce the frequency of breakthrough bleeding often the US Food and Drug Administration (FDA) about the risk of
experienced by users of the extended-cycle pill, another product is venous thromboembolic events associated with the patch, although
now available that replaces the placebo pills with 7 days of low-dose conflicting data have been reported. The reader is referred to the
estrogen. article by Trenor et al in Selected References for a comprehensive
Progestin-only pills, referred to as minipills, also are available and review of this topic.
are particularly useful in postpartum and lactating teenage mothers The long-acting injectable progestin depot medroxyprogesterone
and for women with contraindications or an intolerance to estro- acetate (DMPA) (eg, Depo-Provera) is given intramuscularly every
gen. Because ovulation is not consistently inhibited by progestin- 3 months (12–14 weeks) to inhibit ovulation, thicken cervical
only pills, however, they must be taken at the same time every day, mucus, and induce an atrophic endometrium; a subcutaneous for-
because the effect of cervical mucus thickening diminishes in mulation is also available. The most common side effect is irregular
22 hours. menstrual bleeding, especially in the first few months, and eventual
Clear medical benefits associated with COC use include pre- amenorrhea with prolonged use. Weight gain remains a significant
vention of pregnancy, protection against ovarian and endome- issue for some patients, particularly in the adolescent with over-
trial cancers, decreased risk of functional ovarian cysts and benign weight or obesity in whom exaggerated increases in weight occur.
breast conditions, improvement of acne, and decreased menstrual Breast tenderness and mood disturbances occur less frequently than
blood loss and menstrual symptoms, such as dysmenorrhea. The weight gain. In 2004, the FDA issued a black box warning for DMPA
most common side effects of COCs include breakthrough bleed- about possible irreversible bone loss in women with long-term use
ing, nausea, and breast tenderness, which generally resolve after of DMPA and a potential reduction in overall bone mineral den-
3 cycles. Although potential risks of COC include venous throm- sity in teenagers that may contribute to the development of osteo-
boembolic events, hypertension, and changes in the lipid profile, porosis later in life. The FDA therefore recommends that DMPA
actual risks are minimal in most healthy adolescents without a should not be used in adolescents for longer than 2 years; however,
personal or family history of thromboembolic events compared many experts believe that the risk for pregnancy using an inferior
with the morbidity and mortality associated with teenage preg- method of birth control far outweighs the risk for the development
nancy and childbirth. of osteoporosis in a healthy teenager. Ongoing studies suggest that
Other combined hormonal contraceptive methods for the ado- although an adolescent may not increase her bone mineral density
lescent include the vaginal ring (eg, NuvaRing, Annovera) and the while receiving DMPA and does experience bone loss, the effects
transdermal patch (eg, Ortho Evra, Xulane). Approved in 2001, appear to be temporary and reversible with the discontinuation of
the vaginal contraceptive ring is a soft, flexible device that con- DMPA. The adolescent desiring this method of contraception should
tains estrogen and progestin, which is released directly through be made aware of the black box warning and receive adequate
the vaginal wall into the bloodstream. The ring is inserted into calcium and vitamin D and recommend supplementation if the diet
the vagina for 3 weeks, then removed for 1 week to allow for a appears suboptimal.
withdrawal bleed. The ring can also be removed intermittently for A subdermal implant (eg, Implanon, Nexplanon) is available
up to 3 hours and remain effective. Systemic side effects are simi- for young women who desire a long-acting reversible method of
lar to other low-dose combined hormonal methods (eg, headache, contraception and has gained popularity as a good option for most
breast tenderness, nausea, breakthrough bleeding and/or spotting); adolescents because it requires no compliance after insertion. It is
specific local effects include vaginal discharge and discomfort sec- designed to deliver a low, steady dose of continuous progestin for
ondary to local irritation. Sensation of a foreign body and expul- 3 years via a single plastic polymer rod placed below the skin.
sion of the ring during coitus may also occur. The teenager must Formal instruction for insertion and removal is required for the
be comfortable with insertion and removal of the device for suc- health professional interested in providing this form of contracep-
cessful use of this method. tion. The subdermal implant is highly effective; however, as with
The transdermal adhesive patch is a thin, beige, 3-layered plas- other long-acting progestin-only contraceptives, it is associated with
tic patch that contains estrogen and progestin and is applied weekly bleeding irregularities, especially during the first year of use, which
to specific areas of the body (ie, lower abdomen, upper torso, upper may contribute to its early discontinuation.
arm, or buttocks) to complete the application of 1 patch per week Other long-acting reversible contraceptives include levonorgestrel-
for a total of 3 weeks, followed by 1 week patch-free during which releasing intrauterine systems (eg, Mirena, Skyla) and the copper
menses occurs. Although these patches are well tolerated, high IUD (ParaGard). Although previously not recommended for nul-
complete or partial detachment rates in teenagers have been liparous women, including teenagers, Mirena was approved by the
documented. Additionally, an adolescent may have concerns FDA in 2009 for treatment of severe menorrhagia and dysmenor-
about the visibility of the patch. Side effects are similar to those of rhea and is effective for up to 5 years. The Skyla IUD releases a lower
other combined hormonal methods of contraception. Local effects dose of levonorgestrel and is approved for 3 years. An increased risk
include skin irritation, redness, and rash at the site of application. of pelvic inflammatory disease was previously thought to be associ-
Reduced effectiveness has been reported in women weighing more ated with IUDs in teenagers, but this belief is no longer supported by

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 396 PART 4: ADOLESCENT HEALTH

the literature. Side effects of intrauterine systems include headache, Another type of EC, ulipristal acetate (eg, Ella), is a progestin
acne, and breast tenderness as well as irregular menstrual bleeding, receptor agonist/antagonist with a mechanism of action similar
particularly during the first 3 to 6 months of use. Additionally, 50% of to levonorgestrel EC; it primarily works by delaying or inhibiting
women develop amenorrhea after 1 year of intrauterine system use. ovulation. Important differences, however, must be considered
The copper IUD is approved for 10 years of effective long-term con- between ulipristal acetate and progestin-only EC. First and fore-
traception and can be used for emergency contraception (EC) as well. most, ulipristal acetate is more effective than progestin-only pills,
particularly on the fifth day after sex. It also is more effective closer
Emergency Postcoital Contraception to the time of ovulation, when women are at greatest risk of preg-
Emergency contraception (ie, the “morning-after pill”) is an effective nancy. Recent data also suggest that ulipristal acetate may be more
means of preventing unintended pregnancy in adolescents by provid- effective for women with overweight or obesity (body mass index
ing high-dose progestin up to 5 days after unprotected intercourse. ≥26 or weight >74.8 kg [>165 lb]). Finally, ulipristal acetate is
It requires, however, that the health professional educate teenagers available only with a prescription, regardless of age. Pregnancy
about its availability and usage and that teenagers feel comfortable must be excluded before prescribing ulipristal because of the risk
contacting their physician, if necessary, within 72 to 120 hours of of fetal loss if used inadvertently in the first trimester of preg-
unprotected or inadequately protected intercourse. Although EC is nancy. Patients also must be counseled to seek immediate medical
not meant to be used repeatedly as the sole method of contracep- attention if they become pregnant or experience severe lower
tion, it is useful for the unplanned sexual encounter, which is often abdominal pain within 6 weeks after its use, because ectopic preg-
the case with adolescents, or after a failed contraceptive method (eg, nancy can occur. Common side effects of ulipristal acetate include
condom breakage) or sexual assault. Although different EC regimens headache, nausea, and abdominal pain.
exist, the most frequently used EC contains a total dose of 1.5 mg of Regardless of the type of EC used, the patient should be sched-
levonorgestrel in a 1- or 2-dose regimen (eg, Plan B One-Step, Next uled for a follow-up office or clinic appointment 2 to 3 weeks after
Choice One Dose, My Way). Emergency contraception acts primar- using EC so that a repeat pregnancy test can be performed, treat-
ily by delaying or inhibiting ovulation. Levonorgestrel-based EC does ment failures can be identified early, STI screening can occur, and
not interrupt or disrupt an already established pregnancy and is not consistent contraceptive options can be discussed.
an abortifacient. Because levonorgestrel EC is not teratogenic, a preg-
nancy test is not required before its use. Nonhormonal Contraception
The original EC regimen is a combination of high-dose estrogen Numerous studies and clinical experience have shown that nonhor-
and progestin, known as the Yuzpe method; however, nausea and monal methods are less effective in adolescents than in adults. Latex
vomiting occurs in approximately 25% to 30% of patients. To reduce condoms in conjunction with a spermicide have become a crucial
these side effects, 2-pill formulations of progestin-only EC (eg, Plan B, method of contraception since the emergence of AIDS, however.
Next Choice) are available, consisting of 2 doses of 0.75 mg of Although they help prevent transmission of some STIs, such as gon-
levonorgestrel taken 12 hours apart within 3 days of unprotected orrhea, chlamydia, trichomoniasis, and HIV, condoms do not pro-
intercourse. Based on data reported by the World Health tect against human papillomavirus and human herpesvirus infection
Organization, however, this regimen has been modified to take both overall because the genital area is not completely covered. Thus, the
pills at once up to 5 days after unprotected or inadequately protected physician should take time during the office visit to explain these
intercourse. As a result, Plan B One-Step and its generic forms are details and demonstrate proper use of condoms. Risks of condom
now available as a single-pill regimen of 1.5 mg of levonorgestrel, use are minimal, except for allergic reactions to the spermicide, latex,
which may improve adherence. or lubricants. The female condom is not widely used by adolescents
In the United States, progestin-only EC is now available over- but may be helpful in situations in which a male partner refuses to
the-counter without age restrictions and can be purchased from a wear a condom. The inner ring may be inserted into the vagina up to
pharmacy without the need to show identification. Some state laws 8 hours before intercourse, and neither a prescription nor physician
also allow pharmacists to provide EC pills directly to individuals of visit is necessary to obtain a female condom. Some teenagers, how-
all ages without requiring a doctor’s prescription. Emergency con- ever, may be uncomfortable with its insertion and the fact that the
traception is most effective when used within the first 24 hours after outer ring remains on the vulva during vaginal intercourse. Similar
unprotected coitus. Although side effects are less common than with issues are encountered when considering the diaphragm as a con-
the Yuzpe method, the side effects of single-dose progestin-only EC traceptive method for teenagers; therefore, it is not recommended
include nausea, vomiting, breast tenderness, and irregular bleeding as a first-line contraceptive method for most adolescents.
patterns (eg, spotting); shortened interval to menses; and lighter
or heavier menses. Because EC is safe and highly effective in pre- Sexually Transmitted Infections
venting pregnancy, the physician should provide the sexually active All STIs should be managed according to the most recent guidelines
teenager with information about the different types of EC, where published by the Centers for Disease Control and Prevention based
and how to obtain it, and a prescription (for insured patients, if on current epidemiology. See Chapter 60 for detailed discussion of
necessary) at the annual preventive health care visit. the diagnosis and treatment of STIs in adolescents.

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 CHAPTER 58: REPRODUCTIVE HEALTH 397

Recomm Rep. 2016;65(RR-3):1–104. https://www.cdc.gov/reproductivehealth/

CASE RESOLUTION contraception/contraception_guidance.htm. Accessed February 23, 2019

A more detailed history should be obtained about the adolescent’s menstrual Ford C, English A, Sigman G. Confidential health care for adolescents:
history and daily activities (eg, With whom does she spend most of her time? What position paper for the Society for Adolescent Medicine. J Adolesc Health.
does she like to do in her spare time?). Additionally, the indications for a pelvic 2004;35(2):160–167 PMID: 15298005 https://doi.org/10.1016/S1054-
examination should be reviewed because most teenagers are not familiar with 139X(04)00086-2
the new recommendations to delay the Pap smear until 21 years of age. Because Guttmacher Institute. Fact sheet. Contraceptive use in the United States.
the patient does not meet the new criteria for a Pap smear, the pelvic examination Guttmacher Institute website. https://www.guttmacher.org/sites/default/files/
can be deferred. A discussion should follow about barrier and hormonal methods factsheet/fb_contr_use_0.pdf. Published July 2018. Accessed August 9, 2019
of contraception and their role in the prevention of pregnancy and STIs. Particular
Guttmacher Institute. State policies in brief. An overview of minors’ consent
attention should be paid to the use of long-acting reversible contraceptives.
law. Guttmacher Institute website. https://www.guttmacher.org/state-policy/
Emergency contraception should also be reviewed with the patient. Written infor-
mation as well as useful website addresses should be given to the adolescent for explore/overview-minors-consent-law Published February 1, 2019. Accessed
future reference. A follow-up visit should be scheduled for sometime in the next February 23, 2019
few months, especially if the patient decides to begin contraception. Levine SB. Adolescent consent and confidentiality. Pediatr Rev. 2009;30(11):
457–459 PMID: 19884287 https://doi.org/10.1542/pir.30-11-457
Marcell AV, Burstein GR; American Academy of Pediatrics Committee on
Adolescence. Sexual and reproductive health care services in the pediat-
Selected References ric settings. Pediatrics. 2017;140(5):e20172858 PMID: 29061870 https://doi.
org/10.1542/peds.2017-2858
Allen S, Barlow E. Long-acting reversible contraception. an essential guide for
pediatric primary care providers. Pediatr Clin North Am. 2017;64(2):359–369 Murphy NA, Elias ER. Sexuality of children and adolescents with developmen-
PMID: 28292451 https://doi.org/10.1016/j.pcl.2016.11.014 tal disabilities. Pediatrics. 2006;118(1):398–403. Reaffirmed November 2017
PMID: 16818589 https://doi.org/10.1542/peds.2006-1115
American Academy of Pediatrics Committee on Adolescence. Condom use
by adolescents. Pediatrics. 2013;132(5):973–981 PMID: 28448257 https://doi. Pfeffer B, Ellsworth TR, Gold MA. Interviewing adolescents about sexual mat-
org/10.1542/peds.2013-2821 ters. Pediatr Clin North Am. 2017;64(2):291–304 PMID: 28292446 https://doi.
org/10.1016/j.pcl.2016.11.001
American Academy of Pediatrics Committee on Adolescence. Contraception for
adolescents. Pediatrics. 2014;134(4):e1244–e1256 PMID: 25266430 https://doi. Powell A. Choosing the right oral contraceptive pill for teens. Pediatr Clin
org/10.1542/peds.2014-2299 North Am. 2017;64(2):343–358 PMID: 28292450 https://doi.org/10.1016/j.
pcl.2016.11.005
American Academy of Pediatrics Committee on Adolescence. Emergency con-
traception. Pediatrics. 2012;130(6):1174–1182 PMID: 23184108 https://doi. Rome ES, Issac V. Sometimes you do get a second chance. emergency contra-
org/10.1542/peds.2012-2962 ception for adolescents. Pediatr Clin North Am. 2017;64(2):371–380 PMID:
28292452 https://doi.org/10.1016/j.pcl.2016.11.006
American College of Obstetricians and Gynecologists Committee on Gynecologic
Practice. ACOG committee opinion no. 415: depot medroxyprogesterone acetate Rowan SP, Someshwar J, Murray P. Contraception for primary care providers.
and bone effects. Obstet Gynecol. 2008;112(3):727–730 PMID: 18757687 https:// Adolesc Med State Art Rev. 2012;23(1):95–110, x–xi PMID: 22764557
doi.org/10.1097/AOG.0b013e318188d1ec Trenor CC III, Chung RJ, Michelson AD, et al. Hormonal contraception and
Braverman PK, Breech L; American Academy of Pediatrics Committee on thrombotic risk: a multidisciplinary approach. Pediatrics. 2011;127(2):347–357
Adolescence. Gynecologic examination for adolescents in the pediatric office PMID: 21199853 https://doi.org/10.1542/peds.2010-2221
setting. Pediatrics. 2010;126(3):583–590. Reaffirmed May 2013 PMID: 20805151 Tulloch T, Kaufman M. Adolescent sexuality. Pediatr Rev. 2013;34(1):29–38
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Centers for Disease Control and Prevention. Reproductive health. United States Upadhya KK. Contraception for adolescents. Pediatr Rev. 2013;34(9):384–394
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 CHAPTER 59

Vaginitis
Monica Sifuentes, MD

CASE STUDY
An 11-year-old girl is brought to your office with vaginal Questions
itching for 1 week and a yellow discharge on her under- 1. What are the most common causes of vaginal
wear for the past 4 days. The girl reports no associated discharge in prepubescent girls? In pubescent
abdominal pain, vomiting, or diarrhea. She has no uri- girls?
nary problems and denies any history of sexual abuse. 2. What basic history-related information must be
Although she occasionally bathes with bubble bath, she obtained from all females whose chief complaint is
most often takes showers. Except for the vaginal com- vaginal discharge?
plaint, she is healthy, and she takes no medications. 3. What specific methods are used to perform a
The physical examination is notable for a soft, non- gynecologic examination in prepubescent girls?
tender abdomen with no organomegaly. Bowel sounds In pubescent girls?
are audible in all quadrants. The genitalia are sexual 4. What is the appropriate laboratory evaluation
maturity rating (ie, Tanner stage) 2. The labia majora for prepubescent girls who complain of vaginal
and minora and the clitoris all appear normal, and the discharge? For pubescent girls? How does this
hymen is annular in shape with a smooth rim. A scant evaluation differ for pubescent girls who are
amount of yellow discharge, along with minimal peri- sexually active?
hymenal erythema, is noted at the vaginal introitus. The 5. What are the various treatment options for girls
anal examination is normal, with an intact anal wink. with vaginitis?

Vaginal discharge is not an uncommon occurrence in prepubescent causes include respiratory pathogens, such as Haemophilus influen-
and pubescent girls. Primary care physicians are largely responsi- zae, Neisseria meningitidis, and Streptococcus pneumoniae, and enteric
ble for differentiating between a physiologic discharge, or leukor- organisms, such as Escherichia coli, Shigella, and Yersinia enteroco-
rhea, and a pathologic discharge, which occurs, for example, with litica. A positive culture for sexually transmitted pathogens such as
a bacterial or yeast infection. In cases of an abnormal discharge, Chlamydia trachomatis or Neisseria gonorrhoeae is found in approxi-
the possibility of sexual abuse must be considered and investigated mately 5% of children who are evaluated for child sexual abuse. Higher
appropriately (see Chapter 145). Primary care physicians should figures have been reported from select centers and when data from
become familiar with the various causes of vaginal discharge in pre- adolescent victims are included. These organisms are not considered
pubescent and pubescent girls. More importantly, they should be part of the normal flora in prepubescent girls. Vaginal and rectal infec-
comfortable performing age-appropriate gynecologic examinations tions with C trachomatis can be acquired perinatally but usually are
in these patients so that the appropriate treatment can be initiated. not considered perinatally acquired after 2 to 3 years of age.
Vulvovaginitis, a term that often is used interchangeably with Parasitic infections may also cause vaginal symptoms. Twenty
vaginitis or vulvitis, signifies inflammation of the perineal area, often percent of females with a rectal infestation of Enterobius vermicu-
accompanied by vaginal discharge. The discharge may be bloody, laris, the organism known as pinworm, have vulvovaginitis. Affected
malodorous, or purulent, depending on the etiology (Table 59.1). patients often complain of anal pruritus in addition to the vaginal
discharge. Mycotic infections with organisms such as Candida albi-
Epidemiology cans also can cause symptoms in prepubescent girls, although many
Vulvovaginitis is a common gynecologic complaint in prepubescent of these girls have a previous history of recent oral antibiotic use,
girls. Most cases of vulvovaginitis in this age group result from nonspe- diabetes mellitus, immunosuppression, or other risk factors.
cific inflammation; vaginal cultures show normal flora in 33% to 85%
of such cases. The incidence of more specific bacterial causes, such Clinical Presentation
as group A b-hemolytic streptococcus, has been reported in approx- Prepubescent and pubescent girls with vulvovaginitis most com-
imately 10% to 20% of patients. Its occurrence seems to be seasonal, monly present with a vaginal discharge, which may be white, puru-
however, and confirming the diagnosis depends on the use of proper lent (ie, yellow or green), or serosanguineous. Consistency of the
culturing techniques using the appropriate media. Other bacterial discharge can range from smooth and thin to thick and cottage

399

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 400 PART 4: ADOLESCENT HEALTH

Table 59.1. Characteristics and Specific Causes of Vaginal Discharge

Color Consistency Amount Cause
Clear/white Thin Variable Physiologic leukorrhea; bacterial vaginosis
White Cottage cheese–like Moderate Candida
White/yellow Variable Variable Chemical irritation, pinworms, Chlamydia trachomatis, bacterial vaginosis
Yellow/green Thick Moderate–profuse Neisseria gonorrhoeae, foreign body, Trichomonas vaginalis, group A b-hemolytic
streptococci
Bloody Variable Variable Shigella, group A b-hemolytic streptococci, foreign body

cheese-like. The discharge may also be malodorous. In addition, Differential Diagnosis

girls may complain of associated pruritus, erythema, urinary prob-
A vaginal discharge is normal at 2 distinct times in prepubescent
lems such as dysuria and increased frequency, and abdominal pain
girls: shortly after birth, secondary to the effects of maternal estrogen,
(Box 59.1). Sexually active pubescent girls with vaginitis from a
and approximately 6 months to 1 year before the onset of menarche
sexually transmitted infection (STI) (eg, gonorrhea) generally have
(physiologic leukorrhea), which occurs, in most girls, by sexual matu-
a more profuse, purulent discharge.
rity rating (SMR) (ie, Tanner stage) 4. Other causes of vaginal dis-
charge in prepubescent and pubescent girls are presented in Box 59.2.
Pathophysiology
Prepubescent girls are at risk for developing vulvovaginitis for ana-
Evaluation
tomic and physiologic reasons. Unlike pubertal girls and young adult
women, prepubescent girls have no pubic hair and a smaller labial Prepubescent Girls
fat pad to protect the vaginal introitus. The labia minora are small History
and tend to open when girls are in a squatting position, thereby A complete history should be obtained in all girls with a vagi-
exposing the vaginal introitus. The relative proximity of the anus to nal discharge (Box 59.3). Practitioners should inquire about the
the vagina in young girls also contributes to vaginal contamination appearance of the discharge, its duration, and the relative amount.
with enteric organisms. More importantly, poor hygienic practices A profuse, purulent discharge is probably more consistent with 1
(ie, wiping back to front after urination or defecation) can further specific etiology (eg, N gonorrhoeae) than is a scant, thin discharge,
compound the problem. which is suggestive of a nonspecific etiology. The existence of uri-
In addition, the normal physiology of the vaginal epithelium in nary problems also should be determined. Pooling of urine in the
prepubescent girls predisposes to vaginitis and vulvar inflammation. vagina secondary to labial fusion can result in vulvovaginitis in addi-
The unestrogenized vaginal epithelium is relatively thin, immature, tion to a urinary tract infection. Changes in bowel or bladder habits
and easily traumatized. Additionally, unlike the acidic environment and sudden changes in behavior, such as nightmares or inappropri-
of the vagina in adult females, in prepubescent girls the pH of the ate stranger anxiety, also should be noted. Such changes in behav-
vagina is neutral to alkaline, which allows overgrowth of patho- ior warrant a further inquiry into the possibility of sexual abuse,
genic fecal and oropharyngeal bacteria. Local antibody production regardless of the practitioner’s index of suspicion. Depending on the
also may be lacking in the vagina of prepubescent girls. Vulvar skin information disclosed and the age of the patient, a decision might be
is also easily irritated by harsh soaps, medications, chemicals such made to interview the child and parents or guardians independently.
as bubble baths, and tight-fitting clothing or synthetic underwear. Other points to discuss include the type of detergents or soaps used
Girls who are overweight may be particularly susceptible to perineal for laundry as well as for bathing, because these may be irritating.
irritation and subsequent inflammation of the area. Any recent illnesses also should be documented as a possible source
of autoinoculation or, if oral antibiotics were prescribed, as a reason
for the alteration of the normal vaginal flora. Additionally, patients’
hygienic practices should be reviewed. Adolescent patients should
Box 59.1. Diagnosis of Vaginitis in Prepubescent always be interviewed alone (see Chapter 4). In particular, a confi-
and Pubescent Girls dential reproductive history must be obtained, keeping in mind that
ww Nonphysiologic vaginal discharge puberty and sexual activity alter normal vaginal flora.
ww Profuse, malodorous, or purulent vaginal discharge
Physical Examination
ww Perineal erythema
ww Vaginal pruritus or irritation Although the genital examination is the priority, a complete physical
ww Dysuria examination should be performed. Doing so not only allows physicians
to identify other abnormal physical findings, but also alleviates some

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 Chapter 59: Vaginitis 401

Box 59.2. Causes of Vaginal Discharge Box 59.3. What to Ask

in Prepubescent and Pubescent Girls
Vaginitis
Prepubescent Girls Prepubescent Girl
ww Estrogen withdrawal (neonates) ww What is the color of the discharge?
ww Nonspecific vulvovaginitis ww What is the consistency of the discharge?
ww Chemical irritation secondary to soaps and detergents ww How profuse is the discharge?
ww Mechanical irritation from nylon panties or tight-fitting clothes ww Is the discharge malodorous?
ww Foreign body in vagina ww How long has the discharge been present?
ww Poor hygiene ww How often does the discharge occur (ie, is it found on the panties daily)?
ww Pinworms (Enterobius vermicularis) ww Are there any associated problems (eg, dysuria, abdominal pain, enuresis,
ww Yeast infection (eg, Candida) anal pruritus)?
ww Bacterial infection (eg, group A b-hemolytic streptococcus, ww What types of laundry soap or detergent are used?
Staphylococcus species, nonencapsulated Haemophilus influenzae, ww Does the child take bubble baths?
Escherichia coli, Shigella species, Salmonella species, Yersinia) ww Has the child had any recent illnesses or treatment with oral antibiotics?
ww Sexually transmitted infection (eg, gonococcal infection, chlamydial ww Does the child clean herself after using the toilet, or does she require
infection, trichomoniasis, human herpesvirus, human papillomavirus) help?
ww Congenital abnormality (eg, ectopic ureter [local inflammation]) ww In what direction does she tend to wipe after a bowel movement?
ww Acquired abnormality (eg, labial fusion [pooling of urine in vagina]) ww Is there a concern for, or history of, child sexual abuse?
ww Urethral prolapse ww Has there been any recent behavioral changes (eg, nightmares, crying,
ww Systemic illness (eg, scarlet fever, measles, varicella, Kawasaki disease, recurrent abdominal pain)?
Crohn disease) ww What has the family done to manage the discharge?
ww Vulvar skin disease: lichen sclerosis, contact dermatitis, psoriasis, zinc
Pubescent Girl (all of the above questions plus the following)
deficiency
ww What is the reproductive history (menstrual and sexual)?
Pubescent Girls ww Does the adolescent douche or use scented panty liners?
ww Physiologic leukorrhea ww Does the adolescent use or share sex toys?
ww Foreign body in vagina (eg, retained tampon or condom) ww Is there a history of sexual assault or abuse?
ww Yeast infection (eg, Candida) ww If appropriate, is the adolescent using any form of barrier or hormonal
ww Bacterial infection (eg, group A b-hemolytic streptococcus, contraception? When was the last episode of unprotected intercourse?
Staphylococcus aureus) ww How many sexual partners has the patient had?
ww Sexually transmitted infection (eg, gonococcal infection, chlamydial ww Does the patient or partner have any history of a previous sexually
infection, trichomoniasis, bacterial vaginosis) transmitted infection, including hepatitis B or C?
ww Chemical irritation (eg, douches, spermicides, latex [condoms])
ww Local trauma (eg, penile or labial piercings)
used, depending on the comfort level of the practitioner and the
patient. If the labia cannot be spread apart, the girl may have labial
of the anxiety often associated with a genital examination. Because fusion or adhesions. Perihymenal and periurethral erythema should
most vaginal discharges in prepubescent girls result from nonspecific be noted, in addition to any evidence of edema, trauma, or abnor-
vulvovaginitis, visualization of the cervix with a speculum is not indi- mal masses such as urethral prolapse. Hymenal size and appearance
cated. Regardless of the sex of the examiner, a chaperone is required should be carefully evaluated for any evidence of suspected sexual
during the genital examination, particularly in postpubertal patients. abuse (see Chapter 145). The appearance, consistency, and amount
The overall demeanor of the young patient at the onset of the gen- of the vaginal discharge, including the presence or absence of an
ital examination should be noted. Overly compliant, apathetic behav- odor, also should be documented. These findings may vary depend-
ior in children may be a cause for concern for sexual abuse, especially ing on the characteristics and cause of the discharge. Finally, the anus
in the context of a chronic or recurrent purulent vaginal discharge. should be inspected for tone and any evidence of trauma or abnor-
On physical examination, the patient’s SMR must be noted and mal lesions such as genital warts. An anal wink is normally elic-
recorded. In addition, the external genitalia should be examined ited. Although not usually indicated, a rectal examination should be
closely for the presence of any lesions or evidence of erythema. performed to attempt to palpate a foreign body or mass in patients
Chronic changes in labial skin, such as those associated with con- with chronic or bloody vaginal discharge. If the examiner cannot
stant scratching, also should be documented. adequately visualize the perineum, vulva, and vaginal introitus
Girls should be placed in the modified lithotomy or batrachian using the batrachian or knee-chest position and the patient has per-
(ie, frog-leg) position, and the labia majora should be gently spread sistent or significant symptoms, an examination under anesthesia
apart to visualize the hymen. The knee-chest position also can be may be warranted.

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 402 PART 4: ADOLESCENT HEALTH

Laboratory Tests Laboratory Tests

Laboratory studies are often unnecessary in prepubescent girls For pubescent girls who are not sexually active, the laboratory
with a nonspecific, non-bloody vaginal discharge, diffuse vulvar evaluation should be similar to that described for prepubescent
erythema, and no suspicion or history of sexual abuse. A vagi- girls. Sexually active adolescents, however, warrant a more thor-
nal culture for isolation of group A b-hemolytic streptococcus ough evaluation to establish the etiology of their symptoms and
is indicated in girls with an abrupt onset of a serosanguineous investigate for cervicitis. Generally, if a speculum examination is
discharge and a history of a systemic illness. The cellophane tape being performed in these adolescents, a single-swab endocervi-
test may be helpful in diagnosing a pinworm infection in prepu- cal sample should be obtained and sent for gonorrhea and chla-
bescent girls with associated vulvar or anal pruritus. A potassium mydia via NAATs (eg, ligase chain reaction, polymerase chain
hydroxide (KOH) wet mount reveals pseudohyphae if a monil- reaction). Urine or a vaginal swab also should be sent for N gon-
ial infection is present; however, this diagnosis is often made orrhoeae and C trachomatis in those adolescents who refuse a
clinically. pelvic examination or for screening purposes in the asymptom-
If the discharge is purulent, persistent, or malodorous or if atic sexually active patient. Recommendations have changed for
sexual abuse is suspected, vaginal cultures must be obtained for teenagers concerning the age at which cervical cancer screening
N gonorrhoeae and C trachomatis. For suspected cases of sexual (ie, Papanicolaou test) is indicated (see Chapter 58 for details).
abuse or assault, culture remains the standard because of the risk A culture or polymerase chain reaction test for herpes simplex is
of a false-positive result with nonamplified probe tests (eg, direct indicated only if the history of exposure is positive and ulcerative
immunofluorescent smears, enzyme immunoassays) and nucleic or vesicular lesions are present.
acid amplification tests (NAATs). With genital and anal specimens, Other causes of vaginal symptoms can be determined by the pH
false-positive results can occur because of cross-reaction with fecal and microscopic analysis of a fresh sample of the vaginal discharge.
flora. In certain cases, a normal saline wet mount to check for mobile A normal saline wet mount should be made using a sterile, saline-
trichomonads may be indicated. moistened swab, especially if a nonspecific discharge is present or
bacterial vaginosis (BV) is a concern. In addition, motile or static
Pubescent Girls trichomonads may be seen on a wet mount as well as white blood
History cells, although a diagnosis of trichomonas should be made using
NAAT on a vaginal, endocervical, or urine specimen. A 10% KOH
In adolescent or pubescent girls with vaginal discharge, physicians
mount is appropriate in cases of suspected candidiasis. Mixing 10%
should inquire about a history of sexual activity or assault in addition
KOH with the discharge may also produce a “fishy” odor (ie, pos-
to other information that relates to their condition (see Box 59.3).
itive “whiff test”), which is consistent with BV and sometimes a
Questions concerning the possible acquisition of an STI also must
trichomonad infection. Additionally, a vaginal pH greater than 4.5
be asked in a nonjudgmental, confidential manner.
is common with BV or trichomoniasis, although it is not specific.
Physical Examination Commercial kits to evaluate for BV, trichomoniasis, and candida
via nucleic acid probes are available for the office setting in which
Pubescent girls with an uncomplicated history who have never been
pH paper, KOH, and microscopic analysis of vaginal fluids is not
sexually active should be examined in a similar fashion as the pre-
possible. A Gram stain of any purulent material may result in an
pubescent female. The external genitalia should be examined to
early diagnosis of gonococcal cervicitis. Serologies for syphilis also
evaluate the SMR of the patient and the presence of any lesions or
should be obtained in adolescents with suspected (ie, symptomatic)
anatomic abnormalities, although SMR assignment may be challeng-
or proven STIs. In addition, these patients should be offered annual
ing in the adolescent who has shaved her pubic hair. Perihymenal
testing for HIV and syphilis.
or periurethral erythema and hymenal size and appearance should
be noted. In the absence of a mucopurulent or bloody discharge, a
speculum examination is generally not warranted, particularly in Management
the virginal adolescent. The goal of management of a nonspecific vaginal discharge is relief
In addition to an overall physical examination, a complete exter- of the uncomfortable symptoms associated with this type of inflam-
nal genital and speculum examination is indicated in symptom- mation. Prepubescent girls should be instructed to discontinue use
atic, sexually active girls (ie, history of lower abdominal pain, severe of all chemical irritants, including bubble baths and harsh soaps,
dysmenorrhea, vaginal discharge). The purpose of the speculum in the genital area. The physician should recommend once or twice
examination is to visualize the cervix, properly obtain vaginal and daily warm water sitz baths for approximately 1 week or until symp-
cervical specimens, and examine the vagina for lesions (eg, con- toms have improved. A small amount of vitamin A and D ointment
dylomata acuminata). A bimanual examination also should be per- or similar emollient can be used to protect the vulvar skin and
formed to check for cervical motion tenderness as well as adnexal promote healing. In addition, girls should be instructed in proper
masses or uterine tenderness, which are associated with pelvic hygiene (eg, wiping front to back after a bowel movement, appro-
inflammatory disease. priate hand washing). The use of cotton or cotton-crotch underwear

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 Chapter 59: Vaginitis 403

and loose-fitting skirts or pants should be encouraged. Occasionally, Table 59.2. Treatment Recommendations for
a persistent, nonspecific vaginal discharge of more than 2 to Adolescents With Infectious Vaginal Discharge
3 weeks’ duration may benefit from a 10-day course of oral antibio­
Organism Treatmenta
tics such as amoxicillin, amoxicillin-clavulanate, or clindamycin.
Girls with obesity and poor hygiene are especially prone to recur- Neisseria gonorrhoeae Ceftriaxone 250 mg intramuscularly once, PLUS
rences of vulvovaginitis. azithromycin 1 g orally in a single dose
Anticandidal medications, such as clotrimazole or miconazole Chlamydia trachomatisb Azithromycin 1 g orally once, or doxycycline orally
cream, may be prescribed if a monilial infection is present. Empiric 100 mg twice per day for 1 week
treatment with either of these medications may be warranted in Trichomonas vaginalis Metronidazole 2 g orally once, or tinidazole 2 g
children or adolescents with a previous history of oral antibiotic orally once
usage, diabetes mellitus, or other chronic conditions that may alter Bacterial vaginosis Metronidazole 500 mg orally twice per day for
the normal vaginal flora. 7 days; or metronidazole gel 0.75%, 1 applicatorful
Pinworms are treated with 2 oral doses of albendazole 400 mg, intravaginally once per day for 5 days; or clindamy-
or pyrantel pamoate 11 mg/kg per dose (maximum dose, 1 g), cin cream 2%, 1 applicatorful intravaginally at
with the second dose given 2 weeks after the first. Most authori- bedtime for 7 daysc
ties recommend repeat treatment after 2 weeks to kill worms that
Candida albicans Over-the-counter intravaginal agents: 1% clotrim-
may have hatched after the first dose. All household contacts and
azole cream 5 g intravaginally for 7–14 days;
caregivers of the infected person also should be treated with med-
2% clotrimazole cream 5 g intravaginally for
ication and instructed on good hand hygiene to prevent reinfec-
3 days; 2% miconazole cream 5 g intravaginally
tion. Bedding and clothing should be laundered in hot water and
for 7 days
dried in a dryer.
If a retained foreign body is suspected in a prepubescent girl, Prescription intravaginal agents: 4% miconazole
an examination under general anesthesia may be necessary. cream 5 g intravaginally for 3 days; 0.4% tercon-
Alternatively, in a cooperative child practitioners can attempt vagi- azole cream 5 g intravaginally for 7 days; 0.8%
nal irrigation by placing a small feeding tube at the hymenal open- terconazole cream 5 g intravaginally for 3 days;
ing and injecting warm saline. Toilet paper is the most commonly fluconazole 150 mg orally once
retrieved material in prepubescent girls. a
For a complete list of treatment options, refer to Workowski KA, Bolan G; Centers for Disease
Pubescent girls who are not sexually active should be treated in Control and Prevention. Sexually transmitted diseases treatment guidelines, 2015. MMWR
Recomm Rep. 2015;64(RR-3):1–137.
a similar fashion as prepubertal girls. If the discharge is consistent b
May be associated with vaginal discharge, because it causes cervicitis.
with a diagnosis of physiologic leukorrhea, practitioners should c
Oil-based product; thus, condom efficacy may be decreased during treatment.
reassure patients and educate them about other issues related
to puberty (eg, menarche, body odor). Sexually active adoles-
cents with positive vaginal cultures, NAATs, or highly suspicious
vaginal discharges should receive treatment depending on the CASE RESOLUTION
suspected or causal organism (see Chapter 60 for details con- The girl and her parents should be assured that the discharge is consistent with
cerning treatment). Table 59.2 briefly outlines current treatment a nonspecific inflammatory process. She should be instructed to take sitz baths
for 1 week, discontinue bubble baths and the use of soap in the genital area, and
recommendations.
wear loose-fitting clothes and cotton underwear. The girl should be reexamined
Any disclosure of molestation or assault by prepubescent in 1 to 2 weeks for resolution of her symptoms. No laboratory studies or medica-
or pubescent girls must be reported to the appropriate author- tions are warranted at this time.
ities. In addition, abnormal physical findings and positive cul-
tures for STIs in prepubescent and pubescent girls who have never
been sexually active must be reported to law enforcement and
investigated. Selected References
Braverman PK, Breech L; American Academy of Pediatrics Committee on
Prognosis Adolescence. Gynecologic examination for adolescents in the pediatric office
setting. Pediatrics. 2010;126(3):583–590. Reaffirmed May 2013 PMID: 20805151
In most prepubescent girls, vaginitis resolves spontaneously or https://doi.org/10.1542/peds.2010-1564
after appropriate treatment with no permanent sequelae. In con- Dei M, Di Maggio F, Di Paolo G, Bruni V. Vulvovaginitis in childhood. Best
trast, pubescent girls treated for vulvovaginitis or uncomplicated Pract Res Clin Obstet Gynaecol. 2010;24(2):129–137 PMID: 19884044 https://
cervicitis from an STI continue to be at future risk for the develop- doi.org/10.1016/j.bpobgyn.2009.09.010
ment of pelvic inflammatory disease, HIV, and pregnancy because Emans SJ. Vulvovaginal problems in the pre-pubertal child. In: Emans SJ, Laufer
of their high-risk behavior, inconsistent use of barrier contraception MR. Emans, Laufer, Goldstein’s Pediatric and Adolescent Gynecology. 6th ed.
(eg, condoms), and recurrent exposure to STIs. Philadelphia, PA: Lippincott Williams & Wilkins; 2012:42–59

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 404 PART 4: ADOLESCENT HEALTH

Farhi D, Wendling J, Molinari E, et al. Non-sexually related acute genital ulcers in Stricker T, Navratil F, Sennhauser FH. Vulvovaginitis in prepubertal girls.
13 pubertal girls: a clinical and microbiological study. Arch Dermatol. 2009;145(1): Arch Dis Child. 2003;88(4):324–326 PMID: 12651758 https://doi.org/10.1136/
38–45 PMID: 19153341 https://doi.org/10.1001/archdermatol.2008.519 adc.88.4.324
Fortin K, Jenny C. Sexual abuse. Pediatr Rev. 2012;33(1):19–32 PMID: 22210930 Sugar NF, Graham EA. Common gynecologic problems in prepubertal girls. Pediatr
https://doi.org/10.1542/pir.33-1-19 Rev. 2006;27(6):213–223 PMID: 16740805 https://doi.org/10.1542/pir.27-6-213
Jacobs AM, Alderman EM. Gynecologic examination of the prepubertal girl. Syed TS, Braverman PK. Vaginitis in adolescents. Adolesc Med Clin. 2004;15(2):
Pediatr Rev. 2014;35(3):97–104 PMID: 24585812 https://doi.org/10.1542/ 235–251 PMID: 15449843 https://doi.org/10.1016/j.admecli.2004.02.003
pir.35-3-97 Workowski KA, Bolan GA; Centers for Disease Control and Prevention.
Kokotos F, Adam HM. Vulvovaginitis. Pediatr Rev. 2006;27(3):116–117 PMID: Sexually transmitted diseases treatment guidelines, 2015. MMWR Recomm
16510554 https://doi.org/10.1542/pir.27-3-116 Rep. 2015;64(RR-03):1–137 PMID: 26042815
McGreal S, Wood P. Recurrent vaginal discharge in children. J Pediatr Adolesc Zuckerman A, Romano M. Clinical recommendation: vulvovaginitis. J Pediatr
Gynecol. 2013;26(4):205–208 PMID: 22264471 https://doi.org/10.1016/j. Adolesc Gynecol. 2016;29(6):673–679 PMID: 27969009 https://doi.org/10.1016/j.
jpag.2011.12.065 jpag.2016.08.002

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 CHAPTER 60

Sexually Transmitted
Infections
Monica Sifuentes, MD

CASE STUDY
A 17-year-old boy presents with a small red lesion on masses are palpable. Bilateral shotty, nontender, inguinal
the tip of his penis. He noticed an area of erythema a adenopathy is evident.
few weeks previously, but it resolved spontaneously.
He reports no fever, myalgia, headache, dysuria, or ure-
Questions
1. What conditions are associated with vesicles in the
thral discharge. He is sexually active and only occasion-
genital area?
ally uses a condom. He did not use a condom during
2. What risk factors are associated with the acqui-
his last sexual encounter 2 weeks previously, however,
sition of sexually transmitted infections during
because his partner uses oral contraception. The adoles-
adolescence?
cent has never been treated for any sexually transmit-
3. What screening tests should be performed in the
ted infection and is otherwise healthy. His partners are
patient with suspected sexually transmitted infection?
exclusively female.
4. What recommendations about partners of the patient
On examination, he is a sexual maturity rating (ie,
with sexually transmitted infection should be given?
Tanner stage) 4 circumcised male with a 2- to 3-mm
5. What issues of confidentiality are important to
vesicle on the glans penis. Minimal erythema is pres-
address with the adolescent who seeks treatment
ent at the base of the lesion, and no urethral discharge
for a sexually transmitted infection?
is evident. The testicles are descended bilaterally, and no

Many teenagers in the United States have their first sexual experi- sequential or concurrent; inconsistent and incorrect use of condoms;
ence before they graduate from high school. In the 2017 national unprotected sex; experimentation with drugs, including alcohol,
Youth Risk Behavior Surveillance System conducted by the Centers which results in poor judgment concerning sexual activity; mental
for Disease Control and Prevention (CDC), 40% of all students in health issues; poor adherence to antibiotic regimens; and biologic
high school reported having had sexual intercourse, with 3.4% of factors, such as young age at onset of menarche and the presence of
students nationwide reporting sexual intercourse for the first time cervical ectopy in adolescent females. The feeling of invulnerability
before age 13 years. More important, nearly 12% of boys and 8% of and the desire for autonomy that occur commonly during adoles-
girls in grades 9 to 12 reported having had 4 or more sexual partners cence make most sexual encounters spontaneous rather than pre-
during their life. The consequences of sexual activity in adolescents meditated. As a result, preventive measures are forgotten, ignored,
include increased rates of bacterial and viral sexually transmitted or overlooked by individuals of this age group, and the short- and
infections (STIs), unintended pregnancy, and the possible acqui- long-term consequences of their actions are seldom considered.
sition of long-term infections (eg, HIV) in the 15- to 24-year age Other factors that influence STI trends are related to societal norms.
group. Early detection and effective management of these infec- Traditionally, unlike in other industrialized countries, educational
tions, particularly HIV, can greatly enhance the teenager’s current materials and STI services have not been readily available to adoles-
health and overall lifespan and reduce the risk of transmitting HIV cents in some areas of the United States. Many teenagers also have
to others. Because of the complex nature of these consequences, the difficulty accessing comprehensive health care in their communi-
physician must be skilled in and comfortable obtaining a complete ties and are concerned about confidentiality when obtaining medi-
sexual history in the adolescent patient and in diagnosing and man- cal services for sensitive issues. Additionally, the depiction of casual
aging common STIs and must refer individuals with more compli- sexual relationships in the media, music videos, and motion pictures
cated infections to the appropriate subspecialists. may contribute to the glamorization of sex. Advances in technology
Increasing levels of risk-taking behaviors and sexual activity in via unlimited internet access also give teenagers the opportunity to
adolescence directly affect STI trends in that patient population. communicate with peers who were previously unreachable and to
Other influential factors include multiple sex partners, whether access health information that is unfiltered and may be misleading.
405

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 406 PART 4: ADOLESCENT HEALTH

Epidemiology men in their mid-30s. In 2017, however, young men age 20 to 24 years
had the highest rates of syphilis. Studies have shown that people with
The overall prevalence of STIs in adolescents is difficult to estimate
syphilis, as well as other STIs that cause genital ulcers, also are at
because not all STIs are reportable, many infections are asymptom-
increased risk for HIV acquisition.
atic, and collected data may not include specific subsets of the popu-
Human papillomavirus is the most common STI in the United
lation. It has been estimated, however, that more than 50% of all new
States, with the highest infection rates among adolescents and
STIs diagnosed annually in the United States occur among teen-
young adults. Recent studies report a prevalence in sexually active
agers and young adults aged 15 to 24 years. After human papilloma-
adolescents ranging from 30% to 60%, with one-half of new infec-
virus (HPV), Chlamydia trachomatis is the second most common
tions occurring in individuals 15 to 24 years of age. The prevalence
STI in the United States. Chlamydia trachomatis remains the most
of HPV in adolescents varies widely for 2 reasons: infection with
common cause of cervicitis and urethritis in adolescents, with age-
HPV is often latent and generally regresses spontaneously, partic-
specific rates highest among girls and young women 15 to 24 years of
ularly in young adolescents, and HPV is not a reportable condi-
age and young men 20 to 24 years of age. Additionally, studies have
tion. Behavioral and biological risk factors for HPV infection have
shown that certain adolescent subpopulations are at increased risk
been identified and include early age of sexual initiation, unpro-
for chlamydial infection, such as homeless and incarcerated youth,
tected intercourse with multiple sexual partners, the partner’s num-
socioeconomically disadvantaged youth, ethnic minority youth,
ber of sexual partners, a lack of consistent condom use, and a history
teenagers attending family planning clinics, and pregnant adoles-
of another STI, such as genital herpes, which may facilitate HPV
cents. Complications of unmanaged chlamydial cervicitis occur in
acquisition by compromising mucosal integrity. Cigarette use also
10% to 15% of cases and include pelvic inflammatory disease (PID),
increases the risk of infection and HPV-related disease, as does an
ectopic pregnancy, chronic pelvic pain, and infertility. Epididymitis,
altered immune system.
a result of urethral infection, occurs in 1% to 3% of infected males.
Infection with herpes simplex virus (HSV-1 and HSV-2) is under-
Other conditions that may occur in males engaging in receptive
estimated and is the most common cause of genital ulcerative disease
intercourse include proctitis, proctocolitis, and reactive arthritis
in the United States. Most primary episodes in adolescent females
(formerly known as Reiter syndrome).
and young men who have sex with men are caused by HSV-1 and
In 2017, the CDC reported gonorrhea rates to be highest among
recurrent infections by HSV-2.
adolescents and young adults compared with the general popu-
As of 2016, 21% of all new HIV diagnoses in the United States
lation, particularly among teenage girls and young women. The
were among youth. According to the CDC, most of those new diag-
highest rates of gonorrhea reportedly occur in adolescent females,
noses occurred among young gay and bisexual men, particularly
young men in their early 20s, young ethnic minority adults living
young black/African American and Hispanic/Latino gay and bisex-
in the inner city, incarcerated youth, men who have sex with men,
ual men. Because the time from acute HIV infection to immunosup-
and commercial sex workers. Injection drug use, exposure to com-
pression is, on average, 10 years for untreated adolescents, estimates
mercial sex workers, and numerous sexual contacts also contrib-
of asymptomatic or early HIV infection often are based on reported
ute to the risk of infection. The prevalence of gonorrhea in 15- to
cases of AIDS in young adults in their third decade. Most of these
19-year-old girls and young women was 557 per 100,000 population.
individuals are infected through sexual contact or injection drug use.
Boys and young men 15 to 19 years of age had the second highest
Teenage subpopulations who are at particularly high risk for acquir-
rates of gonorrhea (323 per 100,000) compared with men age 20 to
ing HIV are youth who have male-to-male sexual contact; are trans-
24 years, who had even higher rates of gonorrhea (705 per 100,000).
gender; are experiencing homelessness or who have run away; are
Of the more than 1 million cases of PID reported annually in the
users of injection drugs; are incarcerated; are in the foster care sys-
United States, approximately 20% occur in sexually active adoles-
tem; or have been sexually or physically abused. Of note, research
cents. The risk of developing PID is increased several fold in this
has shown that young gay men who have sex with older partners
age group compared with adult women for several reasons: failure to
are at increased risk for HIV infection because the older partner is
use condoms consistently, multiple new partners within the previous
more likely to have had more sexual partners and therefore has an
12 months, and a history of other STIs. Additionally, according to the
increased likelihood of being infected with HIV.
National Survey of Family Growth conducted by the CDC, girls who
initiated vaginal intercourse at younger than 15 years had the high-
est prevalence of PID. Complications of PID, such as tubo-ovarian Clinical Presentation
abscess (TOA) formation, are more likely to occur in adolescents as The adolescent with an STI may consult his, her, or their physician
a result of late presentation, delayed diagnosis, difficulty accessing with specific complaints related to the genitourinary system, such
health care, and nonadherence with prescribed treatment regimens. as painful urination or vaginal discharge. The adolescent also may
Although the rate of primary and secondary syphilis declined report more generalized complaints, such as fever, rash, and malaise,
from 1990 to 2000, the number of cases has since been increasing especially in cases of primary HSV-1 and HSV-2 infection or during
at epidemic proportions, primarily among young men of color who the viremic phase of HIV acquisition (Box 60.1). Additionally, some
have sex with men. During 2005, the incidence of syphilis was high- teenagers use a vague complaint as an opportunity to visit their pri-
est among women in the 20- to 24-year-old age group and among mary care physician with the hope that the physician will inquire

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 Chapter 60: Sexually Transmitted Infections 407

about sexual behaviors. The likelihood that the adolescent will dis-
Box 60.1. Diagnosis of Sexually
close his, her, or their true concern about an undiagnosed infection
Transmitted Infection
is greatly increased if the physician appears genuinely interested
Males and nonjudgmental.
ww Dysuria
ww Urethral discharge or pain Pathophysiology
ww Testicular pain Several biologic factors contribute to the increased prevalence of
ww Presence of any lesions in the genital area, such as ulcers, vesicles, STIs in adolescents, particularly in females. At the onset of puberty,
or warts the columnar epithelial cells in the vagina transform to squamous
ww Nonspecific rash epithelium, while columnar cells at the cervix persist (Figure 60.1).
ww Sexual partner who has a sexually transmitted infection With increasing age, the squamocolumnar junction recedes into the
Females endocervix. In adolescent females, however, this junction, referred
ww Dysuria to as cervical ectopy, often is located at the vaginal portion of the
ww Abnormal vaginal discharge cervix and is relatively exposed, which places these individuals at
ww Intermenstrual or irregular vaginal bleeding particular risk for gonococcal and chlamydial infections. The infec-
ww Dysmenorrhea tious organisms preferentially attach to cervical columnar cells and
ww Dyspareunia infect them. The use of oral contraceptives prolongs this immature
ww Postcoital bleeding histologic state.
ww Lower abdominal pain The cytologic changes observed in cervical cells of adolescents
ww Nonspecific rash with HPV infection are also believed to be age-related. The immature
ww Systemic symptoms, such as fever, nausea, vomiting, or malaise cervical metaplastic or columnar cells seem to be more vulnerable
ww Presence of any lesions in the genital area, such as ulcers, vesicles, to infection and neoplastic changes. Additionally, exposure to other
or warts cofactors (eg, tobacco use, multiple episodes of new HPV infection)
ww Sexual partner who has a sexually transmitted infection is likely to promote the development of squamous intraepithelial
neoplasia and cervical carcinoma. Not all young women exposed to

PREPUBERTAL ADULT

Columnar Columnar
cells cells

Squamous Squamous
cells cells

POSTPUBERTAL

Columnar
cells S-C junction
S-C located near or
junction in endocervix
Squamous
cells

S-C junction
moves caudally

Squamous metaplasia

Figure 60.1. Development of the cervical squamocolumnar (S-C) junction, from puberty to adulthood.

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 408 PART 4: ADOLESCENT HEALTH

HPV develop lesions or progress to squamous intraepithelial neo- normal immunologic barriers to infection, such as altering the
plasia, however, and most do not remain positive for HPV through- vaginal pH and flora and the cervical mucus barrier.
out their lifetime.
The presence of genital ulcers has been shown to facilitate the Differential Diagnosis
transmission and acquisition of HIV. Such ulcers provide a point Most patients with STIs present with 1 of 5 clinical syndromes:
of entry past denuded epithelium. Additionally, it is hypothesized urethritis/cervicitis, epididymitis, PID, genital ulcer disease, or geni-
that many activated lymphocytes and macrophages are located tal warts, all of which are easily diagnosed with the appropriate diag-
at the base of the ulcer and are therefore susceptible to infec- nostic studies (Box 60.2). Other conditions that mimic STIs must
tion by HIV. be considered, however, particularly in certain cases in which the
Pelvic inflammatory disease usually manifests from an ascending adolescent denies sexual activity or in which the disorder does not
mixed polymicrobial infection, often related to an untreated STI of respond to routine medical management. These disorders include
the cervix and vagina. The infection spreads contiguously upward to mucocutaneous ulcers associated with systemic lupus erythemato-
the upper genital tract, resulting in inflammation involving the endo- sus and Behçet syndrome. Often, systemic disorders such as these
metrium, fallopian tubes, and/or ovaries. The most common causal can be ruled out based on the history, although a minimal workup
organisms, which account for more than one-half of the cases of PID may be necessary. Benign oral lesions, such as aphthous ulcers, also
in most series, are C trachomatis and Neisseria gonorrhoeae. Other can be confused with herpetic ulcers. When evaluating an adolescent
organisms include Escherichia coli, other enteric flora, and microbes female with acute lower abdominal pain, it is necessary to rule out
implicated in bacterial vaginosis, such as Mycoplasma hominis, surgical conditions such as appendicitis, ovarian torsion, and ectopic
Mycoplasma genitalium, Ureaplasma urealyticum, Bacteroides spe- pregnancy. In the sexually active male with testicular pain, testicular
cies, and anaerobic cocci. Viruses such as HIV and HSV-1 and HSV-2 torsion must be cautiously considered and thoroughly evaluated
can facilitate the process of this ascending infection by disrupting before a diagnosis of acute epididymitis is made.

Box 60.2. Differential Diagnosis of Sexually Transmitted Infection by Clinical Syndrome

Urethritis Genital Ulcers
ww Neisseria gonorrhoeae ww Treponema pallidum (syphilis)
ww Nongonococcal disease ww HSV-1 and HSV-2
—— Chlamydia trachomatis ww Haemophilus ducreyi (chancroid)
—— Ureaplasma urealyticum ww C trachomatis (lymphogranuloma venereum)
—— Trichomonas vaginalis ww Epstein-Barr virus (infectious mononucleosis)
—— Mycoplasma genitalium Genital Warts
—— Herpes simplex (HSV-1 and HSV-2) virus ww T pallidum (condyloma latum)
—— Yeasts ww Human papillomavirus (condyloma acuminata)
Cervicitis Proctitis
ww C trachomatis ww N gonorrhoeae
ww N gonorrhoeae ww C trachomatis
ww T vaginalis ww T pallidum
ww M genitalium ww HSV-1 and HSV-2
ww HSV (primarily HSV-2) ww Particular to youth who engage in same-sex sexual activity (in addition
Pelvic Inflammatory Disease to the above)
ww N gonorrhoeae ww Hepatitis A and B virus
ww C trachomatis ww Shigella
ww Anaerobes ww Campylobacter
ww Gram-negative rods ww Giardia lamblia
ww Streptococci ww Entamoeba histolytica
ww Mycoplasma hominis Pharyngitis
ww M genitalium ww N gonorrhoeae
ww U urealyticum ww HSV-1 and HSV-2
Vaginitis
ww T vaginalis
ww Candida albicans and other yeast
ww Gardnerella vaginalis

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 Chapter 60: Sexually Transmitted Infections 409

Evaluation asked about the presence of dysuria, urethral discharge, and ure-
thral erythema or pruritus. In females, symptoms such as dysuria
In all sexually active adolescents, a complete medical and psychoso-
and increased urination may have a more gradual onset and are
cial history, including a sexual history, should be obtained confiden-
reported more frequently than other symptoms, such as meatal
tially (Box 60.3). The risk assessment for an STI, particularly HIV,
edema, erythema, or urethral discharge, which are rarely noticed
should be based on a review of actual sexual behaviors rather than
in girls. General urinary symptoms, such as acute urinary frequency
on an adolescent’s stated sexual orientation at the time of the visit. A
and urgency, are uncommon with urethritis, especially in males.
detailed gynecologic history also should be reviewed with females,
More often, urethritis is asymptomatic, and the diagnosis is made
including any recent changes in menstrual bleeding and dyspareu-
by routine annual screening in sexually active adolescents or through
nia. The remainder of the history should focus on the patient’s spe-
known contact with a partner with an STI (eg, C trachomatis,
cific complaint and any associated symptoms. A complete physical
N gonorrhoeae).
examination, including a thorough genital examination, should
To diagnose acute epididymitis, the sexually active male should
be performed. Although diagnostic tests are determined based on
be asked about testicular pain and swelling. Symptoms associated
findings from the history and physical examination, it is impor-
with urethritis also may be present or may have preceded the
tant to remember that many adolescent patients are asymptomatic,
scrotal symptoms.
and screening for common STIs, such as chlamydia and gonorrhea,
should be performed at least annually on all sexually active teenag- Physical Examination
ers through noninvasive urine or vaginal nucleic acid amplification The genital examination in both males and females must be per-
testing (NAAT). Adolescents at increased risk for STI (eg, a history of formed in the presence of a chaperone, regardless of the sex of the
STIs, multiple partners, young men who have sex with men) should health professional.
be screened every 6 months or more frequently depending on their In males, the presence of a urethral discharge and its consis-
risk factors or current symptomatology. tency (ie, mucopurulent or purulent) should be noted. Any other
Urethritis and Epididymitis urethral or genital lesions also should be assessed. The epididymis,
spermatic cord, and testes should be palpated carefully for tender-
History
ness and swelling.
Because infectious urethritis is more common in young men In symptomatic females, a full pelvic examination should be
than in older men, all sexually active adolescent males should be performed after careful examination of the external genitalia for any
ulcerative or wart-like lesions. The urethra should be inspected for
edema, erythema, or any evidence of a discharge prior to insertion
Box 60.3. What to Ask of the speculum. Urethritis is most often caused by C trachomatis,
Sexually Transmitted Infections but HSV-1 and HSV-2 and trichomoniasis can also cause urethritis.
ww Are you currently in a relationship? The presence or absence of a vaginal or endocervical discharge also
ww Are you sexually active? Do you have or have you had oral, vaginal, and/ should be noted during the pelvic examination; however, its absence
or anal intercourse? does not rule out the possibility of an STI.
ww At what age did you begin to have sex? Laboratory Tests
ww Do you have sex with men, women, or both?
Screening tests using urine or vaginal samples are considered
ww How many partners have you had? When was your last contact?
standard of care for detecting gonorrhea and chlamydia, espe-
ww Have you ever been forced to have sex, had sex while under the influence
cially in the asymptomatic sexually active adolescent. These non-
of alcohol or drugs, or exchanged sex for food, shelter, money, or drugs?
culture tests rely on amplification of DNA (ie, polymerase chain
ww Do you or your partner(s) use contraception? What type?
reaction [PCR], ligase chain reaction) and are highly sensitive
ww Do you or your partner(s) use drugs or alcohol?
and convenient for screening teenagers because the test can be
ww Have you or any of your sexual contacts ever been diagnosed with a
performed on a routine patient-obtained urine or a physician- or
sexually transmitted infection? Did you and they undergo treatment?
patient-obtained vaginal specimen. A disadvantage is that these
ww Do you have abdominal pain, dysuria, increased urinary frequency, or
tests may have an increased potential for false-positive results,
hesitancy?
making a definitive diagnosis questionable in a judicial setting
ww Have you noticed any ulcers, blisters, warts, or other bumps in the
(eg, child sexual abuse). In the adolescent population, however,
genital area? Are the lesions painful?
NAATs definitely are more acceptable than tests requiring a direct
ww Have you had any recent systemic symptoms, such as fever, chills, body
urethral swab specimen and should be used to confirm chlamydial
aches, sore throat, or rashes?
or gonococcal urethritis for screening or diagnostic purposes.
ww For females: Do you have a vaginal discharge or itching? Is sex uncomfort-
In cases of persistent symptoms or recurrent urethritis, particu-
able or painful? Do you have bleeding between periods or after intercourse?
larly in males, infection with M genitalium should be considered.
ww For males: Do you have a discharge from your penis? Any testicular pain
Testing for this organism is not currently available in most lab-
or swelling? Any associated burning or itching?
oratories, however.

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 410 PART 4: ADOLESCENT HEALTH

Color duplex Doppler ultrasonography may be necessary to A presumptive diagnosis of mucopurulent cervicitis is made in the
differentiate between epididymitis and testicular torsion in the setting of copious discharge from the cervical os, cervical erosion,
adolescent male. Whether the diagnosis of testicular torsion is or friability. A bimanual examination also must be performed to
questionable or confirmed, an immediate urologic consultation evaluate for cervical motion tenderness (CMT), adnexal masses or
is necessary. fullness, and uterine tenderness.

Cervicitis Laboratory Tests

History According to the CDC, NAATs for N gonorrhoeae and C trachomatis
Cervicitis in females is parallel to urethritis in males. Because cer- are preferred for the diagnostic evaluation of cervicitis and can be
vicitis is a local infection, systemic symptoms may not occur, which performed on vaginal, urine, or endocervical specimens. If a pelvic
makes asymptomatic infections, especially with C trachomatis, quite examination is being performed, an endocervical or vaginal swab
common. A variety of important points related to the patient his- specimen for NAATs can be collected by direct visualization. A wet
tory and associated complaints should be addressed with all sex- mount evaluation to diagnose associated STIs (eg, trichomoniasis) is
ually active female teenagers to rule out other etiologies, however. recommended, especially if the discharge is foul smelling or frothy;
The physician should ask questions such as, “Do you have a vaginal however, immediate specimen evaluation is required for optimal
discharge?”; “What color is the discharge?”; “Is there any odor?”; results. Alternative diagnostic tests for trichomoniasis are also avail-
“Are you experiencing any urinary frequency, urgency, or dysuria?”; able for females; vaginal, endocervical, or urine specimens can be
and “Have you had any nonmenstrual vaginal bleeding or spotting, tested using NAAT. The adolescent who is diagnosed with a new STI
including postcoital bleeding or dyspareunia?” A known exposure to or engaged in other high-risk behaviors, such as unprotected sexual
other STIs must be ascertained as well, which can be discerned with intercourse, multiple sexual partners, inconsistent or incorrect con-
questions such as, “Is your current partner symptomatic or receiv- dom use, or young men who have sex with men, should undergo test-
ing antibiotic treatment?”; and “Do you or your current or any past ing for other STIs as well, such as HIV, syphilis, and viral hepatitis.
partner have a history of an STI?”
Pelvic Inflammatory Disease
More generalized symptoms, such as moderate lower abdominal
pain (acute or chronic) associated with nausea, vomiting, and fever, History
may be indicative of a complication of untreated or undetected cer- When considering a diagnosis of PID, the history should include a
vicitis, such as PID. Additional symptoms associated with dissemi- discussion of known risk factors, such as unprotected sexual activity;
nated gonococcal infection, such as fever, arthritis, and rash, should the number of sexual partners, especially new partners in the previ-
be investigated as well as the involvement of other areas of the body ous 2 months; a previous history of an STI or recent exposure to an
(eg, sore throat). STI; the type and consistency of contraceptive use; and the timing of
the last menstrual cycle, because many young women present with
Physical Examination
PID during the first half of their menstrual cycle. The physician also
After completing the full physical examination, with particular should inquire about other symptoms that are often associated with
attention paid to the patient’s skin, pharynx, hands, fingers, and PID: the onset, duration, quality, and location of abdominal pain;
joints, the physician should focus on the genitourinary examination. urinary symptoms that may be indicative of concomitant urethritis;
In the presence of a chaperone, the sexual maturity rating should intramenstrual bleeding; dysmenorrhea or dyspareunia; abnormal
be noted first, and the external genitalia should be examined for vaginal discharge; right upper quadrant pain; and systemic symp-
the presence of any lesions or inflammation of the perineum. Any toms, such as nausea, vomiting, fever, and malaise. The classic symp-
urethral erythema or discharge also should be noted. The presence toms of PID, which include pelvic or lower abdominal pain, vaginal
of a vaginal discharge can be assessed more completely when per- discharge, fever, and abnormal bleeding, are not always present.
forming the speculum examination, which should be considered in More often, symptoms are nonspecific or vague, and the physician
all sexually active adolescent females with vaginal or persistent lower must maintain a high index of suspicion in any sexually active female
urinary tract complaints. with acute or subacute pelvic or lower abdominal pain.
During the speculum examination, the vaginal mucosa should
be inspected, the presence of a vaginal discharge identified, and the Physical Examination
appearance of the cervical os documented. The physician should Classic signs of acute PID include fever with lower abdominal pain
determine whether a purulent or mucopurulent exudate is coming and tenderness of the cervix, uterus, or adnexa on bimanual exami-
from the endocervix and look for any evidence of cervical friabil- nation. The clinical presentation can vary from vague discomfort to
ity or sustained endocervical bleeding as a swab is gently passed severe pain, however, depending on the particular patient. Therefore,
through the cervical os to perform the NAAT. A cotton swab of the before performing the pelvic examination a thorough physical exam-
vaginal discharge should be collected for a microscopic evaluation ination should be completed to exclude other common causes of
of wet preparations (wet mount), if necessary. Cervical inflamma- lower abdominal pain. This should include a review of the vital
tion must be differentiated from normal adolescent cervical ectopy. signs, evaluating for fever or tachycardia. The blood pressure also

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 Chapter 60: Sexually Transmitted Infections 411

must be reviewed carefully because hypotension can occur with a diagnosis. A NAAT for N gonorrhoeae and C trachomatis should be
ruptured ectopic pregnancy, which may present with similar symp- sent on the endocervical or vaginal swab specimen obtained dur-
toms, including abdominal pain and vaginal bleeding. The abdomen ing the pelvic examination.
should be assessed for tenderness and guarding. The location of the Other laboratory studies to obtain include a complete blood cell
pain is particularly relevant because certain acute surgical condi- count with differential and a sedimentation rate or C-reactive pro-
tions, such as appendicitis, ovarian torsion, and ectopic pregnancy, tein level, although in some cases the laboratory tests are normal. A
are important considerations in the differential diagnosis of PID. urine pregnancy test should be performed to exclude the possibility
Additionally, right upper quadrant pain is consistent with perihep- of a concomitant intrauterine or ectopic pregnancy. Additionally, a
atitis (ie, Fitz-Hugh–Curtis syndrome), which can occur with a gon- urinalysis and urine culture should be obtained in the patient who
orrheal or chlamydial infection. In the presence of a chaperone, the reports dysuria. A serologic test for syphilis and HIV testing also
speculum examination should be performed, looking for a muco- should be offered to the adolescent. Testing for viral hepatitis is rec-
purulent endocervical exudate or any evidence of cervicitis (eg, cer- ommended in the patient who has not been immunized against hep-
vical friability or erosion). The cervix should be carefully palpated atitis B or who has exposure to injection drug use, thereby placing
for any evidence of CMT and the uterus for tenderness or adnexal the patient at risk for hepatitis C.
masses on bimanual examination. Laparoscopy may be performed to make a definitive diagnosis of
PID or to obtain cultures directly from the fallopian tubes in cases
Laboratory Tests
in which the diagnosis is equivocal or the patient is not improving
The diagnosis of PID is based on clinical findings and a high index on standard antimicrobial therapy. Immediate consultation with a
of suspicion, after other causes for pelvic or lower abdominal pain gynecologist is necessary for these challenging cases.
have been excluded. Previous CDC criteria for the diagnosis of PID
included 3 major components: lower abdominal pain, CMT, and Imaging Studies
adnexal tenderness. Current recommendations outline 1 or more Transvaginal ultrasonography may help exclude diagnoses such as
minimum clinical criteria and 5 additional criteria to support the ectopic pregnancy or ovarian torsion and can aid in the detection
diagnosis (Box 60.4). Particularly when evaluating an adolescent of complications associated with PID, such as TOA. Fluid in the
for PID, early conservative treatment and maximum sensitivity for cul-de-sac may be evident on ultrasonography; however, it is not
subtle clinical findings are paramount to avoid a delayed or missed specific for the diagnosis of PID.

Genital Ulcers
History
Box 60.4. Diagnosis of Pelvic
Inflammatory Disease Likely the most important information to obtain from the adolescent
with genital ulcers is whether the ulcers are painful. A painless chan-
Pelvic or Lower Abdominal Pain and ≥1 of the Following Minimum cre on the penis in males, around the mouth, in the oropharynx, or
Criteria on Pelvic Examination
in females on the external genitalia is consistent with primary and
ww Cervical motion tenderness
secondary syphilis. If the lesions are painful or are associated with
ww Uterine tenderness
a grouped vesicular eruption, HSV-1 and HSV-2 is the likely cause.
ww Adnexal tenderness
The presence of systemic symptoms, such as fever, chills, headache,
Additional Findings or malaise, also is important to discern because these symptoms can
ww Oral temperature >38.3°C (>101°F) occur with a primary infection with HSV-1 and HSV-2 or second-
ww Abnormal cervical mucopurulent discharge or cervical friability ary syphilis. Generalized complaints, however, are associated with
ww Presence of abundant numbers of white blood cells on saline microscopy secondary syphilis in only 50% of cases. A history of adenopathy,
of vaginal fluid whether localized or generalized, also must be noted, along with
ww Elevated erythrocyte sedimentation rate or C-reactive protein level dysuria, which may be present in females with HSV-1 and HSV-2.
ww Laboratory documentation of cervical infection with Neisseria Additionally, a history of a viral-like illness accompanied by a rash
gonorrhoeae or Chlamydia trachomatis warrants further investigation because a diffuse maculopapular rash,
Specific Criteria for the Diagnosis especially on the palms and soles, is a classic sign of secondary syph-
ww Inflammatory tubal mass or tubo-ovarian complex seen on magnetic ilis. Because a nonspecific rash also can occur with primary HIV
resonance imaging or transvaginal ultrasonography, or evidence of infection, all possible exposures to other STIs should be reviewed
pelvic infection (ie, tubal hyperemia) on Doppler ultrasonography with the teenager.
ww Histologic evidence of endometritis on endometrial biopsy Physical Examination
ww Laparoscopic abnormalities consistent with pelvic inflammatory disease
All adolescents who present with chancres, or ulcers, should undergo a
Derived from Workowski KA, Bolan GA; Centers for Disease Control and Prevention. Sexually trans- complete physical examination. The skin, including the palms and soles,
mitted diseases treatment guidelines, 2015. MMWR Recomm Rep. 2015;64(RR-3):1–137. should be examined closely for any dull red to reddish-brown macular

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 412 PART 4: ADOLESCENT HEALTH

or papular lesions. The oropharynx should be carefully checked Genital Warts

for chancres or blisters. All lymph nodes should be palpated History
for pain, enlargement, or induration. Suppurative or fluctuant
Risk factors for HPV infection should be assessed in all sexually
nodes often are associated with chancroid caused by Haemophilus
active adolescents regardless of their immunization status. These fac-
ducreyi. The genital examination should focus on the appear-
tors include a history of multiple sexual partners, age younger than
ance of the lesions, with the following questions in mind: Are
25 years, a previous history of STIs, lack of consistent condom use,
the lesions ulcerative, clustered, and painful (ie, HSV-1, HSV-2,
pregnancy, altered immune response, and tobacco use. Symptoms
or chancroid)? Are they associated with grouped vesicles on an
may include pruritus, pain, or dyspareunia, but often the condition
erythematous base (ie, HSV-1 or HSV-2)? Is tender inguinal ade-
is asymptomatic. The adolescent may palpate a lesion on the exter-
nopathy present (ie, HSV-1, HSV-2, or chancroid)? Or does the
nal genitalia, report local irritation, or note bleeding from larger,
patient have a single, painless, indurated chancre with a clean
traumatized lesions, depending on lesion location.
base and a sharply defined, slightly elevated border (ie, syphilis)?
The lymph nodes associated with this syphilitic type chancre often Physical Examination
are nontender but enlarged. Other less common STIs produce
A complete pelvic examination should be performed on all sexually
genital ulcers that often are painful and deep and may be accom-
active female adolescents who report lower abdominal pain, vaginal
panied by some purulence. Adenopathy can be quite impressive
discharge, or intramenstrual or postcoital vaginal bleeding, or who
with chancroid and lymphogranuloma venereum, as in the case
express concern with “something they felt” in the genital area. Before
of C trachomatis.
the speculum is inserted, the external genitalia should be carefully
Laboratory Tests inspected for lesions. Genital warts (ie, condylomata acuminata)
The necessary diagnostic tests depend on the patient’s clinical most commonly appear on squamous epithelium as irregular pol-
picture. However, all adolescents who present with a painless ypoid masses with an irregular, cauliflower-like surface that may
ulcerative lesion should undergo dark-field microscopy for syph- coalesce into larger lesions. Usually these are located at the poste-
ilis or direct fluorescent antibody testing of the lesions, which rior introitus, labia minora, and the vestibule in females; they may
is also available and quite specific. Nontreponemal serologic tests be found on the cervix and in the vagina as well.
for syphilis, such as the Venereal Disease Research Laboratories In males, warts are found more commonly on the circumcised
(VDRL) or rapid plasma reagin (RPR) tests, should be done if penile shaft, glans, or corona, as well as under the foreskin of the uncir-
direct examination is not available or the clinical appearance cumcised penis. They also may appear as flat, flesh-colored papules
of the ulcer is nonspecific. In cases of early primary syphilis in on the scrotum and anus. Because the anus is a common location for
which the VDRL or RPR may not be reactive initially, the test lesions, it should be inspected carefully in males and females. Intra-
should be repeated in 1 week. Treponemal tests, such as the flu- anal warts are seen in persons who have receptive anal intercourse, but
orescent treponemal antibody absorption test, should be used they also can occur in men and women with no history of anal sexual
only as confirmatory tests and not for screening purposes. The contact. Condylomata plana are subclinical lesions that are not grossly
physician must keep in mind that false-positive nontreponemal visible but are apparent on colposcopy and histology.
testing can occur in some clinical conditions, such as pregnancy, Condylomata lata are flat, flesh-colored warts that occur in moist
autoimmune disease, and HIV. In these instances, confirmatory areas (ie, anus, scrotum, vulva) and may be associated with other
treponemal testing is required. signs of secondary syphilis.
Viral cell culture and PCR are the preferred diagnostic tests
for individuals who seek treatment for painful genital ulcers or Laboratory Tests
other mucocutaneous lesions likely caused by HSV-1 and HSV-2. The diagnosis of genital warts often is made by visual inspection and
Samples should be obtained from the base of a fresh unroofed generally does not require confirmation by biopsy. A biopsy may be
vesicle. Herpes simplex virus-1 or HSV-2 serology for HSV-1 and required in specific instances, however, such as with an uncertain
HSV-2 is generally not helpful. diagnosis, in the case of a lesion that is not improving or that worsens
Because of the well-described correlation between genital ulcers with standard therapy, for an atypical lesion, in the immunocompro-
and HIV, it is recommended that all patients with a diagnosis of mised patient, or for warts that appear pigmented, indurated, bleed-
syphilis be tested for HIV as well. Conversely, annual syphilis screen- ing, or ulcerated. A definitive diagnosis of HPV infection is based
ing is not indicated for all teenagers but is currently recommended on detection of viral nucleic acid or capsid protein. Tests that detect
for the adolescent with multiple sexual partners; a history of injec- specific types of HPV DNA are available but are not routinely rec-
tion drug use; a history of a male having sex with males; or a previ- ommended because this information does not alter the clinical man-
ously diagnosed STI. Additionally, the seroprevalence of syphilis in agement of the condition, except in extraordinary circumstances.
the health professional’s particular community or specific patient Historically, suspicious areas of white epithelium identified by
population should be taken into account when contemplating more the application of 3% to 5% acetic acid or a specific vascular pattern
frequent screening. consistent with HPV infection were biopsied. This procedure is no

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 Chapter 60: Sexually Transmitted Infections 413

longer recommended, however, because prospective studies have about the symptoms of PID and advice on when to seek additional
shown that many cervical and anogenital squamous intraepithelial medical care.
lesions in adolescents resolve spontaneously if left untreated. For this Antibiotic recommendations and dosing schedules for the inpa-
reason, the American College of Obstetricians and Gynecologists tient treatment of PID are noted in Box 60.5, as well as in Chapter 59
and the American Cancer Society currently recommend that rou- (Table 59.2) and Chapter 145 (Table 145.1) for other infections. The
tine screening for cervical cancer should be performed starting at outpatient management of PID in adolescents with mild to moderate
21 years of age. Cervical Papanicolaou testing is no longer recom- disease is supported by the 2015 CDC sexually transmitted diseases
mended by any major medical organization for individuals younger treatment guidelines; however, individual physician judgment of
than 21 years, with the exception of adolescents with HIV infection, the teenager’s ability to adhere to an outpatient regimen is crucial to
for whom screening is warranted 1 year after onset of sexual activ- prevent short- and long-term sequelae. For additional details about
ity because of the high rate of progression of abnormal cytology. specific conditions and therapies, consult Selected References at
All teenagers should be offered STI screening. Nucleic acid ampli- the end of this chapter, specifically the CDC publication “Sexually
fication tests for the detection of gonorrhea or chlamydia can be per- Transmitted Diseases Treatment Guidelines, 2015.” To avoid reinfec-
formed on urine, endocervical, or vaginal specimens. Additionally, tion, patients and their sex partners should avoid sexual intercourse
a serum nontreponemal antibody test for syphilis (RPR or VDRL) for at least 7 days after all parties have been adequately treated and
is indicated. An HIV antibody test should be offered to all adoles- symptoms have resolved.
cents who are deemed at risk. In addition to antimicrobial therapy, treatment of the adolescent
A urinalysis for asymptomatic hematuria is indicated in males with an STI should include preventive services and counseling on
with visible condylomata. Its presence is indicative of a urethral or risk reduction in a nonjudgmental and developmentally appropri-
meatal lesion. ate manner. Additionally, the patient’s primary language, culture,
sexual orientation, sexual practices, and age should be taken into
Management account. Adolescents with a first-time STI and those with recurrent
STIs should be educated about disease transmission, consequences
Although the details of health care delivery for adolescents differ by of delayed treatment, and methods for the prevention of acquiring
state, all 50 states and the District of Columbia allow health profes-
sionals to evaluate and treat adolescents for an STI without paren-
tal consent, except in unusual circumstances. Routine laboratory Box 60.5. Parenteral Regimens for the Inpatient
screening for common STIs is recommended at least annually in Treatment of Pelvic Inflammatory Disease
all sexually active adolescents and more frequently for those with
additional risk factors. Gonorrhea, chlamydia, syphilis, chancroid, Parenteral Regimen A
and HIV/AIDS are reportable diseases in every state, and a positive Cefotetan, 2 g IV every 12 hours
laboratory result is the impetus for reporting. Notification of all sex or
partners within 60 days of the onset of symptoms or diagnosis of Cefoxitin, 2 g IV every 6 hours
infection or, if greater than 60 days, the last sexual partner, is gen- plus
erally anonymous and carried out by local public health officials. Doxycycline,a 100 mg PO or IV every 12 hours
Contacts are informed that a partner has been diagnosed with an STI Parenteral Regimen B
and are instructed to be evaluated and receive appropriate treatment. Clindamycin 900 mg IV every 8 hours
Adequate and timely treatment of all sexual partners of patients plus
diagnosed with an STI is extremely important to prevent reinfec- Gentamicin loading dose IV or IM (2 mg/kg body weight), followed by a
tion. Because partners often are asymptomatic and do not seek treat- maintenance dose of 1.5 mg/kg every 8 hours. (Single daily dosing
ment, however, and because persistent and recurrent infection rates 3–5 mg/kg may be substituted.)
are reported to be particularly high among adolescents, expedited The above regimens are continued for 24–48 hours after the patient
partner therapy (EPT) is now advocated in many states. Expedited improves clinically. On discharge from the hospital, doxycycline is contin-
partner therapy is the practice of treating the sex partners of indi- ued orally for a total of 14 days. Clindamycin, 450 mg PO 4 times a day, can
viduals with specific STIs without an intervening formal medical be used as an alternative to complete 14 days of treatment.
evaluation or professional prevention counseling. An extension of When tubo-ovarian abscess is present, clindamycin or metronidazole
EPT, patient-delivered partner therapy, is another practical means of with doxycycline is recommended rather than doxycycline alone.
providing patients with medications or prescriptions for their
presumed infected partners. Care must be taken when treating Abbreviations: IM, intramuscularly; IV, intravenously; PO, orally.
a
Doxycycline should be administered orally when possible because of the pain associated with
the female partners of men with gonorrhea or chlamydia with-
IV infusion.
out an examination because of the potential for undiagnosed PID Derived from Centers for Disease Control and Prevention. Sexually Transmitted Diseases Treatment
in the female partner. Per CDC guidelines, in addition to provid- Guidelines, 2015. Atlanta: U.S. Department of Health and Human Services; 2015. https://www.cdc.
ing medications, female partners should also receive instructions gov/std/tg2015/clinical.htm. Accessed April 3, 2019.

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 414 PART 4: ADOLESCENT HEALTH

new and recurrent infections (eg, abstinence, condom use, limiting

the number of sexual partners, modifying sexual behaviors, asymp- CASE RESOLUTION
tomatic viral shedding, pre-exposure immunization [eg, Gardasil 9, A diagnosis of HSV-1 and HSV-2 infection can be made clinically. The diagnosis
Cervarix]). The risk for acquiring other specific STIs, such as HIV, can be confirmed by unroofing a vesicle and gently swabbing the ulcer to exam-
also should be addressed. The physician should spend additional ine for HSV via PCR testing. The adolescent should be counseled about HSV and
time with the adolescent with recurrent STI and explore the reasons its general mode of transmission, the natural history of primary versus recurrent
infection, and the role of antiviral agents. A urine sample also should be sent for
why preventive methods have failed in the past. Several factors are
NAAT for the detection of gonorrhea and chlamydia. A serum RPR or VDRL test
extremely important to consider, including an untreated or undis- should be performed as well as a thorough assessment for HIV risk. Testing for
closed partner, poor sexual judgment secondary to concomitant HIV should be strongly encouraged, allowing the patient time to ask questions
substance use or abuse, and nonadherence to previously prescribed or decline testing. The adolescent should be given a return appointment for 1 to
treatment regimens. 2 weeks hence to review laboratory results, discuss possible treatment options
for recurrent HSV-1 and HSV-2 infection, and explore risk-reduction behavior.
Patient retesting in 3 months is recommended for gonorrhea,
Depending on the results of the remainder of the STI screening tests, the adoles-
chlamydia, and trichomonas infections, even if the physician is cent should be followed every 3 to 6 months.
certain the patient has been treated with an appropriate antibiotic
regimen. Most posttreatment infections are the result of reinfection
rather than treatment failure and occur because the sex partner did
not receive treatment or from the initiation of sexual activity with a
new infected partner. Because repeat infections can increase the risk Selected References
for long-term complications associated with PID and because the Berlan ED, Holland-Hall C. Sexually transmitted infections in adolescents:
rate of reinfection in adolescents is so high, the CDC recommends advances in epidemiology, screening, and diagnosis. Adolesc Med State Art Rev.
retesting all individuals in 3 months. 2010;21(2):332–346, x PMID: 21047032
Centers for Disease Control and Prevention. Expedited partner therapy. Centers
Prognosis for Disease Control and Prevention website. https://www.cdc.gov/std/ept/default.
htm. Reviewed September 20, 2018. Accessed August 9, 2019
The prognosis is good in most cases in which STIs are diagnosed
Centers for Disease Control and Prevention. Expedited Partner Therapy in the
and managed in a timely manner, especially with the judicious use
Management of Sexually Transmitted Diseases. Atlanta, GA: US Department of
of observed single-dose oral therapy for uncomplicated chlamydial Health and Human Services; 2006
infection, which is the most common bacterial STI in adolescents.
Centers for Disease Control and Prevention. Pre-exposure prophylaxis (PrEP)
Acute complications of PID include TOA and Fitz-Hugh–Curtis for HIV prevention. Centers for Disease Control and Prevention website.
syndrome, which can result in an unanticipated surgical proce- https://www.cdc.gov/hiv/pdf/PrEP_fact_sheet_final.pdf. Accessed August 9,
dure or prolonged hospitalization. Long-term consequences of PID 2019
are chronic abdominal or pelvic pain, ectopic pregnancy, and tubal Centers for Disease Control and Prevention. Sexually Transmitted Diseases
infertility. Early administration of appropriate antibiotics and pre- Treatment Guidelines, 2015. Atlanta: U.S. Department of Health and Human
vention of recurrent infection is crucial to minimize the manifesta- Services; 2015. https://www.cdc.gov/std/tg2015/clinical.htm. Accessed April 3,
tion of these long-term sequelae. 2019
Currently, no pharmacologic agent exists for eradicating HSV-1 Centers for Disease Control and Prevention. Sexually Transmitted Disease
and HSV-2, although acyclovir, famciclovir, and valacyclovir are use- Surveillance 2018. Atlanta, GA: US Department of Health and Human
ful in managing the signs and symptoms of primary herpes and Services; 2019. https://www.cdc.gov/std/stats18/default.htm. Accessed
reducing the incidence and duration of recurrences. The long-term October 16, 2019

safety of HSV treatment with valacyclovir or famciclovir for longer Centers for Disease Control and Prevention. Youth Risk Behavior Surveillance
than 1 year is unknown. Daily suppressive therapy to reduce the System (YRBSS). https://www.ncbi.nlm.nih.gov/pubmed/29902162 Accessed
August 28, 2019
frequency of outbreaks may be warranted in patients with more
than 6 outbreaks per year. Because recurrences often decrease over Comkornruecha M. Gonococcal infections. Pediatr Rev. 2013;34(5):228–234
PMID: 23637251 https://doi.org/10.1542/pir.34-5-228
time, the need for suppressive therapy should be assessed annually.
The prognosis for HIV is variable, depending on the individual’s Emmanuel PJ, Martinez J; American Academy of Pediatrics Committee on
Pediatric AIDS. Adolescents and HIV infection: the pediatrician’s role in promot-
disease progression at the time of diagnosis and adherence to anti-
ing routine testing. Pediatrics. 2011;128(5):1023–1029. Reaffirmed September
retroviral therapy. The advent of new preventive therapies, such as 2015 PMID: 22042816 https://doi.org/10.1542/peds.2011-1761
HIV pre-exposure prophylaxis, has significantly reduced the risk
English A. Sexual and reproductive health care for adolescents: legal rights and
of HIV infection in individuals at substantial risk (eg, those exposed policy challenges. Adolesc Med State Art Rev. 2007;18(3):571–581, viii–ix PMID:
to HIV through sex or injection drug use). When taken daily and 18453235
consistently, a combination of emtricitabine and tenofovir (eg, Goyal M, Hersh A, Luan X, Localio R, Trent M, Zaoutis T. Are emergency
Truvada, Descovy) can reduce the risk of permanent infection by departments appropriately treating adolescent pelvic inflammatory disease?
up to 92%. These medications should be used in combination with JAMA Pediatr. 2013;167(7):672–673 PMID: 23645074 https://doi.org/10.1001/
other medications to manage established HIV infection as well. jamapediatrics.2013.1042

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 Chapter 60: Sexually Transmitted Infections 415

Haamid F, Holland-Hall C. Overview of sexually transmitted infections in Trent M. Status of adolescent pelvic inflammatory disease management in the
adolescents. Adolesc Med State Art Rev. 2012;23(1):73–94 PMID: 22764556 United States. Curr Opin Obstet Gynecol. 2013;25(5):350–356 PMID: 24018871
Marcell AV, Burstein GR; American Academy of Pediatrics Committee on https://doi.org/10.1097/GCO.0b013e328364ea79
Adolescence. Sexual and reproductive health care services in the pediat- Wangu Z, Burstein GR. Adolescent sexuality: updates to the sexually transmit-
ric setting. Pediatrics. 2017;140(5):e20172858 PMID: 29061870 https://doi. ted infection guidelines. Pediatr Clin North Am. 2017;64(2):389–411 PMID:
org/10.1542/peds.2017-2858 28292454 https://doi.org/10.1016/j.pcl.2016.11.008
Siqueira LM. Chlamydia infections in children and adolescents. Pediatr Rev. Zuckerman A, Romano M. Clinical recommendation: vulvovaginitis. J Pediatr
2014;35(4):145–154 PMID: 24692154 https://doi.org/10.1542/pir.35-4-145 Adolesc Gynecol. 2016;29(6):673–679 PMID: 27969009 https://doi.org/10.1016/j.
Trent M. Pelvic inflammatory disease. Pediatr Rev. 2013;34(4):163–172 PMID: jpag.2016.08.002
23547062 https://doi.org/10.1542/pir.34-4-163

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 CHAPTER 61

Menstrual Disorders
Monica Sifuentes, MD

CASE STUDY
A 16-year-old girl presents with a 9-day history of vagi- Her body mass index is at the 50th percentile. The physi-
nal bleeding. She has no history of abdominal pain, nau- cal examination, including a pelvic examination, is unre-
sea, vomiting, fever, dysuria, or anorexia, and she reports markable except for minimal blood noted at the vaginal
no dizziness or syncope. Her menses usually lasts 4 to introitus.
5 days and, in general, occurs monthly. Her last menstrual
period was 3 weeks ago and was normal in duration and
Questions
1. What menstrual disorders commonly affect
flow. Menarche occurred at 14 years of age. She is sex-
adolescent girls?
ually active, has had 2 partners, and reportedly uses a
2. What factors contribute to the manifestation of
condom “most of the time.” Neither she nor her current
menstrual disorders, particularly during adolescence?
partner has ever been diagnosed with or treated for a
3. What relevant menstrual history should be obtained
sexually transmitted infection. She has no family history
from the adolescent?
of blood dyscrasia or cancer, has no history of chronic
4. What options are available for managing primary
illness, and takes no medications.
dysmenorrhea?
On physical examination, she is in no acute distress.
5. How is abnormal uterine bleeding managed in the
Her temperature is 36.9°C (98.4°F). Her heart rate is
adolescent patient?
100 beats/min, and her blood pressure is 110/60 mm Hg.

Gynecologic concerns and symptoms are common reasons for (eg, endometriosis) occur in approximately 10% of female adoles-
adolescent girls to visit their primary care physician. The challenge cents and young women with severe dysmenorrhea.
for the pediatrician is to differentiate between an organic etiology, Prevalence estimates concerning premenstrual syndrome (PMS)
a functional condition, and psychogenic symptoms. When this can- are difficult to assess because most studies in adolescents are retro-
not be readily done or if the physical examination is equivocal, mul- spective, and self-reports can be unreliable and misleading. In these
tiple diagnostic procedures may be performed, often with variable studies, between 20% and 30% of older adolescents report signifi-
results. Additionally, many pediatricians are uncomfortable eval- cant PMS-type symptoms. An estimated 20% to 40% of adult women
uating gynecologic problems in adolescents and performing pel- experience PMS symptoms sufficiently bothersome to impair daily
vic examinations, which contributes to this diagnostic dilemma. functions, and 5% to 10% have debilitating symptoms that warrant
The purpose of this chapter is to review some of the more common the diagnosis of premenstrual dysphoric disorder (PMDD). Other
gynecologic conditions affecting adolescent girls and to highlight menstrual problems in adolescents include abnormal uterine bleed-
the significant historical and physical findings associated with each ing, primary and secondary amenorrhea, and vaginal discharge.
problem. For a discussion of the infectious conditions that cause Several factors contribute to the occurrence of menstrual dis-
pelvic pain, see Chapter 60. orders in adolescence. The average age of menarche in the United
States remains at 12.5 years (range: 9–16 years), although the age
Epidemiology of onset of puberty has decreased in some racial groups and in chil-
The overall prevalence of menstrual disorders during adolescence dren with obesity. Bleeding may be irregular or prolonged initially in
is estimated to be 50% in the United States, with the most common young adolescents because most early menstrual cycles are anovu-
gynecologic symptom being dysmenorrhea, or painful menstru- latory and irregular, especially during the first few years after men-
ation. At least 70% to 90% of women have some pain associated arche. Bleeding problems may resolve after ovulatory cycles are
with menses; the extent of discomfort varies. Although most men- established; however, menstrual symptoms, such as lower abdomi-
struating women report mild to moderate discomfort, severe dys- nal pain, breast tenderness, headache, bloating, and vomiting, may
menorrhea occurs in 10% to 15% of women and has been reported predominate. Early sexual activity among adolescents and associ-
to be responsible for significantly limiting activities of daily liv- ated sexually transmitted infections (STIs) also may contribute to
ing, including school attendance, participation in athletics, and the presence of certain gynecologic conditions in this age group,
socialization with peers. Uterine anomalies or pelvic abnormalities particularly vaginitis, abnormal uterine bleeding, and pelvic pain.

417

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 418 PART 4: ADOLESCENT HEALTH

Clinical Presentation usually do not occur until 2 to 3 years after menarche, although 10%
to 20% of cycles remain anovulatory as long as 5 years after menarche.
The adolescent with a menstrual disorder may present in a variety of
It has been reported that girls with earlier menarche establish regular
ways. Specific symptoms include heavy menstrual bleeding, irregu-
ovulatory menstrual cycles more rapidly than girls with later menarche.
lar periods, and painful menses, and more general symptoms include
One-quarter of females begin menstruating when they reach sex-
fatigue, dizziness, and syncope (Box 61.1). The adolescent with PMS
ual maturity rating (SMR [ie, Tanner stage]) 3 of sexual matura-
may experience mood swings, stress, and nervousness accompanied by
tion, but approximately two-thirds do not menstruate until they
abdominal bloating and pain before menses. Additionally, the adoles-
reach SMR 4 breast and genital development. Several other processes
cent or her parent or guardian may have questions or concerns about
occur before the onset of menstruation. Thelarche, or the beginning
delayed pubertal development and primary or secondary amenorrhea.
of breast development, takes place approximately 2 to 3 years before
Pathophysiology menarche, and growth acceleration usually begins approximately
1 year before thelarche.
Puberty and the Normal Menstrual
Cycle Dysmenorrhea
Figure 61.1 depicts the menstrual cycle, which typically lasts for 21 to Dysmenorrhea often is accompanied by other symptoms, such
35 days, with a mean length of approximately 28 days. Normal duration as nausea, vomiting, diarrhea, fatigue, bloating, low back pain,
of menses is 4 to 7 days. Blood loss is usually 30 to 40 mL per cycle; most and headaches. It can be classified as primary or secondary. Primary
women do not lose more than 60 mL per cycle. Regular ovulatory cycles dysmenorrhea occurs in the absence of any pelvic pathology,

(ng)
0.8 Progesterone

0 0
74
200
(pg/mL)

42
100 Estrogen

(mIU)
0
LH
22
(mlU)

10
14
FSH
6
1 7 14 21 28
Days

Growing follicle and ovulation Development and degeneration

of corpus luteum

Menstrual Proliferative Secretory

phase phase phase

Figure 61.1. The normal ovulatory menstrual cycle.

Abbreviations: FSH, follicle-stimulating hormone; LH, luteinizing hormone.

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 Chapter 61: Menstrual Disorders 419

Box 61.1. Diagnosis of Menstrual Disorder Box 61.2. Differential Diagnosis of Common
in the Adolescent Patient Menstrual Disorders
Primary Dysmenorrhea Secondary Dysmenorrhea
ww Painful menstruation ww Endometriosis
ww Lower abdominal pain associated with menstruation, usually worse on ww PID
the first few days of bleeding ww Uterine myomas, polyps, or adhesions
ww Associated back pain ww Adenomyosis
ww Pain sometimes accompanied by nausea, vomiting, fatigue, headache, ww Ovarian cysts or tumors
bloating, and diarrhea ww Presence of an intrauterine device
ww Symptoms begin 6–12 months after menarche ww Cervical stenosis or strictures
Abnormal Uterine Bleeding ww Congenital malformations (ie, septate uterus, imperforate hymen)
ww Prolonged bleeding (>8 days) or Excessive Uterine Bleeding
ww Excessive bleeding (>6 tampons/pads per day) or ww Ovulatory dysfunction: physiologic anovulation
ww Frequent uterine bleeding (≤21 days) ww Complications of pregnancy: spontaneous/threatened/incomplete
ww No demonstrable organic etiology abortion, ectopic pregnancy, hydatidiform mole
ww Normal laboratory studies, with the possible exception of anemia ww Infections of the lower and upper genital tract: endometritis, PID,
Primary Amenorrhea cervicitis/vaginitis
ww No spontaneous menstruation in a girl of reproductive age ww Blood dyscrasia and thrombocytopenia: von Willebrand disease, ITP,
ww Absence of menarche by age 15 years in a girl with normal pubertal leukemia, platelet defects, aplastic anemia
development or ww Endocrine disorders: hypothyroidism and hyperthyroidism, hyperprolactinemia,
ww Absence of menarche by age 13 years in a girl with no secondary sexual late-onset 21-hydroxylase deficiency, Cushing or Addison disease, PCOS
development or ww Vaginal anomaly: carcinoma
ww Absence of menarche within 1–2 years of reaching full sexual matura- ww Cervical/uterine abnormalities: endometriosis, polyp, hemangioma,
tion (sexual maturity rating 5) rhabdomyosarcoma
ww Ovarian abnormalities: primary ovarian failure, tumors, cysts
ww Systemic/chronic illness: IBD, malignancy, SLE, diabetes mellitus
ww Foreign body: retained condom or tampon, IUD
comprising 90% of adolescent menstrual pain, and most commonly
ww Medications: aspirin, anticoagulants, hormonal contraception, andro-
occurs in older adolescents after ovulatory cycles are established.
gens, chemotherapy
Secondary dysmenorrhea refers to painful menses associated with
ww Trauma or sexual assault (ie, high vaginal laceration)
some underlying pelvic pathology, such as pelvic inflammatory dis-
ease (PID), endometriosis, ovarian cysts or tumors, Müllerian anom- Amenorrhea (Primary and Secondary)
alies, or cervical stenosis. A complete list of causes of secondary ww Pregnancy
amenorrhea can be found in Box 61.2. Endometriosis is the most ww Systemic abnormalities: endocrinopathies (hypothyroidism, Cushing syn-
common cause of secondary dysmenorrhea in the adolescent. drome), chronic diseases (IBD, sickle cell disease), poor nutrition (anorexia
Numerous studies have shown that cell membrane phospholip- nervosa), obesity, intense exercise, stress, drugs (opiates, valproate)
ids, endometrial prostaglandins, and leukotrienes play a role in the ww Hypothalamic lesions: tumors, infiltrative lesions (TB, CNS leukemia)
pathogenesis of primary dysmenorrhea. After ovulation, fatty acids ww Pituitary lesions: prolactinoma, drugs causing elevated prolactin (eg,
build up in the phospholipids of the cell membrane in response to marijuana, cocaine), cranial irradiation
the production of progesterone. Arachidonic acid as well as other ww Ovarian failure: gonadal dysgenesis (ie, Turner syndrome); autoimmune
omega-6 fatty acids are released after the onset of progesterone with- failure associated with diabetes mellitus, adrenal insufficiency, thyroid
drawal before menstruation. A cascade of prostaglandins and leu- disease, and celiac disease; radiation- or chemotherapy-induced oopho-
kotrienes is initiated in the uterus during menses, which results in ritis; galactosemia
an inflammatory response. Prostaglandin F2a, which is produced ww Congenital abnormalities of the reproductive tract: imperforate hymen,
locally by the endometrium from arachidonic acid, is a potent vaso- transverse vaginal septum, absence or abnormality of the uterus, complete
constrictor and myometrial stimulant that causes uterine contrac- androgen insensitivity syndrome (complete or partial receptor defects),
tions, resulting in tissue ischemia and pain. Prostaglandin E2a causes Mayer-Rokitansky-Küster-Hauser syndrome
hypersensitivity of the pain nerve terminals in the uterine myome- ww Androgen excess: PCOS, benign ovarian androgen excess
trium. The cumulative effect of these prostaglandins may cause
Abbreviations: CNS, central nervous system; IBD, inflammatory bowel disease; ITP, idiopathic
the pain of primary dysmenorrhea. Hormonal and endocrine fac-
thrombocytopenic purpura; IUD, intrauterine device; PCOS, polycystic ovary syndrome; PID, pelvic
tors also may play a role in the etiology of primary dysmenorrhea, inflammatory disease; SLE, systemic lupus erythematosus; TB, tuberculosis.
because ovulatory cycles with estrogen and progesterone are neces-
sary for development of the condition.

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 420 PART 4: ADOLESCENT HEALTH

Most cases of primary dysmenorrhea begin 1 to 2 years after age 16 years in the girl with otherwise normal pubertal devel-
menarche, and symptoms gradually increase until patients reach opment, an absence of menarche by age 14 years in the girl with
their early 20s. Parity and advancing age are associated with a no secondary sexual development, and an absence of menarche
decrease in symptomatology. within 1 to 2 years of reaching SMR 5 pubic hair. Causes of pri-
mary amenorrhea range from congenital anatomic anomalies to
Abnormal Uterine Bleeding genetic and endocrine conditions. Because many of these disorders
Abnormal uterine bleeding (formerly called dysfunctional uterine can be diagnosed and treated earlier than 16 years of age, how-
bleeding) is abnormal or excessive endometrial bleeding in the ever, guidelines have been modified to address when menstrual
absence of any pelvic pathology. Menstruation is considered exces- conditions should be evaluated. Current guidelines encourage a
sive if the cycles are short (≤21 days) and the bleeding is prolonged more proactive medical evaluation for girls who lack menses by
(>8 days). Although ovulatory dysfunction is the most common age 15 years or more than 3 years after the onset of secondary sex-
cause of abnormal or excessive uterine bleeding in adolescents, it is ual development. Additionally, absence of secondary sexual char-
a diagnosis of exclusion. Other causes of abnormal bleeding should acteristics by age 13 years is considered abnormal (Box 61.3). A
first be investigated by obtaining a thorough history, performing detailed discussion of each etiology that causes primary amenor-
a complete physical examination, and obtaining laboratory stud- rhea is beyond the scope of this chapter; see Selected References
ies as indicated. for more information.
Excessive uterine bleeding typically is the result of anovulatory, Secondary amenorrhea is a state of 3 or more consecutive months
immature menstrual cycles. In adolescents, 50% of menstrual cycles of amenorrhea in the girl who has already established menstrua-
are anovulatory within the first 2 years after menarche. If menarche tion. The most common cause of secondary amenorrhea is preg-
occurs later in adolescence (ie, at SMR 5), the interval from anovu- nancy, which must be ruled out in all adolescents presenting with
latory to ovulatory cycles reportedly lasts even longer. Most cases this symptom, regardless of their acknowledgment of sexual activity.
of abnormal uterine bleeding in adolescents are thought to result Other causes include systemic illness, significant change in weight,
from the delayed maturation of the hypothalamic-pituitary-ovarian stress, intense physical exertion, eating disorders (eg, anorexia ner-
axis. Normally, a positive feedback mechanism manifests with ris- vosa), and certain medications, such as phenothiazines, glucocor-
ing estrogen levels, resulting in a surge in luteinizing hormone ticoids, and heroin. Polycystic ovary syndrome is another common
and follicle-stimulating hormone, which triggers ovulation. The cause of secondary amenorrhea in young adult women, but often it
progesterone-producing corpus luteum then stimulates develop- is characterized by a wide range of menstrual irregularities, includ-
ment of the secretory endometrium, with subsequent shedding ing abnormal uterine bleeding, oligomenorrhea, and amenorrhea
after approximately 14 days if no fertilization occurs (ie, menses). of perimenarcheal onset.
With anovulation, progesterone-producing corpus luteum is absent;
thus, no development of a secretory endometrium occurs. Estrogen
thus remains unopposed, and proliferative endometrium continues Box 61.3. Menstrual Conditions That May
to accumulate. When the tissue can no longer maintain its integrity, Require Evaluation
it sloughs. Additionally, without progesterone the normal vasospasm
Menses That:
that helps limit endometrial bleeding does not occur. As a result,
ww Have not started within 3 years of thelarche
bleeding is prolonged, frequent, and heavy.
ww Have not started by 13 years of age with no signs of pubertal
Premenstrual Syndrome development
ww Have not started by 14 years of age with
Premenstrual syndrome refers to a group of physical, cognitive,
—— Signs of hirsutism or
affective, and behavioral symptoms that occur 1 to 2 weeks before
—— A history or physical examination suggestive of excessive exercise
menses, that is, during the luteal phase of the menstrual cycle, and
or eating disorder or
resolve within 4 days after the onset of menstruation. Various mech-
—— Concerns about an outflow tract obstruction or anomaly
anisms have been proposed, including an increased sensitivity to the
ww Have not started by 15 years of age
normal cyclic fluctuations in steroid hormones and releasing factors
ww Are regular, occurring monthly, then become markedly irregular
and alterations in central neurotransmitters, such as endorphins,
ww Occur more frequently than every 21 days or less frequently than every
-aminobutyric acid, and serotonin. The exact etiology remains
45 days
unknown, however, despite multiple studies with a focus on pin-
ww Occur 90 days apart even for 1 cycle
pointing the cause of this complex condition.
ww Last longer than 7 days
Amenorrhea ww Require frequent pad/tampon changes (soaking more than 1 every
1–2 hours)
Amenorrhea is the lack of spontaneous menstruation in women
of reproductive age. Similar to dysmenorrhea, it can be classi- Adapted with permission from American College of Obstetricians and Gynecologists Committee on
fied as primary or secondary. Traditionally, primary amenorrhea Adolescent Health Care. Menstruation in girls and adolescents: using the menstrual cycle as a vital
was defined by the following criteria: an absence of menarche by sign. Committee Opinion No. 651. Obstet Gynecol. 2015;126:e143-6.

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 Chapter 61: Menstrual Disorders 421

Vaginal Discharge Box 61.5. What to Ask

Vaginal discharge can be normal (ie, physiologic), nonspecific, or
related to a particular bacterial or viral pathogen. A thin, white dis- Menstrual Disorders
charge, known as leukorrhea, occurs approximately 6 to 12 months Dysmenorrhea
before the onset of menarche. A purulent, malodorous, or bloody dis- ww What is the pain like?
charge is considered abnormal, however. Etiologies are varied and may ww Does it always occur with menses?
be infectious, inflammatory, or traumatic (see Chapters 59 and 60). ww When did the pain first begin in relation to menarche?
ww How frequently does the pain occur?
Differential Diagnosis ww How long does the pain last?
ww Are any other symptoms associated with the pain (eg, nausea, vomiting,
The differential diagnosis of common menstrual disorders is
diarrhea, headache)?
extensive and can be found in Box 61.2. Other etiologies of pelvic
ww Do you miss a lot of school or work as a result of painful menses?
pain include mid-cycle menstrual disorders (eg, mittelschmerz), uri-
ww Do the painful menses interfere with other activities?
nary tract infection, complications of unmanaged or inadequately
ww What do you do for the pain? Have you tried any medications or comple-
managed PID, and abdominal conditions, such as inflammatory
mentary or alternative remedies?
bowel disease and irritable bowel syndrome. Psychogenic pain also
ww Does a maternal or sibling history exist of painful menses?
should be considered as a cause of recurrent pelvic pain and may
ww Does a family history exist of endometriosis?
be secondary to depression, anxiety, a history of sexual abuse, or
another psychological condition. Abnormal Uterine Bleeding
ww What was the age at menarche?
Evaluation ww Do you have any symptoms of anemia (eg, fatigue, dizziness, shortness
of breath)?
History
ww Have you experienced any syncopal episodes?
A thorough medical and family history should be obtained from the ww Do you have any history of blood loss in the urine or stool?
adolescent girl with a suspected menstrual disorder because many non- ww Do you have any evidence of a bleeding disorder (eg, easy bruising,
gynecologic conditions can affect menses. A complete psychosocial bleeding from the gums or nares)?
assessment, including a private, confidential sexual history, also should ww Do you have any symptoms of pregnancy (eg, breast tenderness, morn-
be performed without the parent or guardian present. The acronym ing nausea or vomiting, fatigue)?
HEADSS (home, employment and education, activities, drugs, sex- ww Have you knowingly been exposed to a sexually transmitted infection?
uality, suicide/depression) serves as a useful tool when interviewing ww Has your weight or diet changed markedly?
adolescents (see Chapter 4). ww Are you using any medications, such as aspirin, oral contraceptives, long-
Most of the interview should focus on the gynecologic history acting progestational agents, psychotropic medications, or anticoagulants?
(Box 61.4), including pattern of menstrual bleeding and any associ- ww Do you have a history of a systemic illness, such as systemic lupus erythema-
ated symptoms (Box 61.5). With primary dysmenorrhea, pain that tosus, diabetes mellitus, or renal disease? Do you have a history of trauma?
ww Is the bleeding cyclic in nature?
ww Does breakthrough bleeding occur throughout the cycle?
Box 61.4. Gynecologic History ww Does a family history exist of bleeding disorders, type 2 diabetes
ww Age at menarche mellitus, polycystic ovary syndrome, or thyroid disease?
ww Date of last menstrual period Amenorrhea
ww Regularity of menses ww Have you ever had a period?
ww Duration and pattern of bleeding ww Have you noticed any other changes associated with puberty (eg, breast
ww Amount of flow (ie, number of pads and/or tampons used per day and development, pubic hair, growth spurt)?
amount of saturation) ww Do you have any other symptoms, such as galactorrhea, weight loss, or
ww Associated menstrual symptoms, such as bloating, headache, lower hirsutism?
abdominal pain, and cramping ww Have you experienced significant changes or stressors in your life (eg,
ww Maternal and sibling gynecologic history parental divorce, new school)?
ww Treatment of menstrual symptoms ww How often do you exercise?
ww Sexual activity (consensual and nonconsensual) ww What is your typical diet, or do you have any dietary restrictions (eg,
ww Age at debut vegan, vegetarian)?
ww Number of partners and ages ww Are you taking any medications, including contraception?
ww Date of last sexual encounter ww Do you have a history of headaches or visual changes?
ww Protected versus unprotected vaginal or anal intercourse ww Does the review of systems reveal anything suggestive of a chronic
ww Current method of contraception illness, such as Crohn disease?

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 422 PART 4: ADOLESCENT HEALTH

radiates to the anterior thighs or the lower back is not uncommon, presence of a normal vagina, uterus, and adnexa. Typically, a spec-
although other emergent etiologies, such as ovarian torsion, must be ulum examination is not necessary for an adolescent with no prior
explored if the patient presents acutely. The color of the blood may sexual intercourse who is reporting abnormal uterine bleeding or
be helpful when assessing excessive uterine bleeding. Brown or dark simple primary dysmenorrhea. If the sexually active teenager is
blood may be associated with a cervical obstruction or endometri- asymptomatic, screening tests for STIs, particularly chlamydia and
osis, whereas red or pink blood occurs with most other conditions. gonorrhea, can be performed noninvasively using a voided urine
More important, the timing of the bleeding is extremely significant. sample or self-collected vaginal swab, and the pelvic examination
Cyclic bleeding beginning at menarche is more consistent with the can be deferred. In the adolescent who is sexually active and has
presence of a blood dyscrasia. In contrast, breakthrough bleeding abnormal vaginal discharge, intermenstrual bleeding, history of
throughout the cycle may be indicative of an infection, endometri- dyspareunia, or lower abdominal pain, however, a complete pelvic
osis, or a polyp. The passage of blood clots on rising in the morning and bimanual examination is mandatory to evaluate for PID and its
is not uncommon secondary to the vaginal pooling of blood while complications (eg, tubo-ovarian abscess).
the patient is supine. Clots throughout the day, however, are not nor-
mal and require further investigation. Laboratory Tests
A thorough review of systems also should be performed, paying The performance of laboratory studies depends on the specific men-
particular attention to recent weight changes, systemic illnesses, strual symptoms. No laboratory studies initially are necessary for
and chronic symptoms. primary dysmenorrhea because the diagnosis usually is based on a
classic clinical history and normal physical examination. The same is
true for PMS. The laboratory evaluation for primary amenorrhea is
Physical Examination dependent on the presence or absence of associated secondary sexual
A complete physical examination, including an evaluation for any characteristics. For more information on the diagnostic evaluation
stigmata associated with a systemic illness, must be performed in of primary and secondary amenorrhea, refer to Selected References.
the adolescent girl with a suspected menstrual disorder. The patient’s In the patient with abnormal uterine bleeding, baseline studies
height and weight should be plotted on the growth chart and com- must include obtaining hemoglobin or hematocrit. Other initial lab-
pared with previous measurements. Depending on the SMR, the oratory studies should include a complete blood cell count to evalu-
health professional can then determine if the teenager should have ate the red cell indices and platelets, a reticulocyte count, and a urine
experienced her expected growth spurt. Body mass index, calculated pregnancy test. Further diagnostic studies depend on the severity
by dividing weight in kilograms by height in meters squared, also of the anemia and findings on history and physical examination.
should be calculated and compared with previous values, especially These may include coagulation studies (eg, prothrombin time/partial
in girls with amenorrhea. Vital signs, including orthostatic mea- thromboplastin time), erythrocyte sedimentation rate, and thyroid
surements, are especially important to review in the patient with function tests. Other tests for the evaluation of a blood dyscrasia,
excessive uterine bleeding or amenorrhea as the result of restrictive such as a von Willebrand factor (measured with ristocetin cofactor
eating (eg, hypothermia, severe bradycardia, orthostatic hypoten- activity and antigen), factor VIII, and fibrinogen, should be per-
sion). The skin should be inspected for any evidence of androgen formed in the patient who presents with severe anemia, especially
excess (eg, hirsutism, acne), insulin resistance (acanthosis nigri- at menarche or shortly thereafter. The studies should be performed
cans), bruising, pallor, or petechiae. The thyroid gland should be in consultation with a pediatric hematologist and obtained before
palpated for masses or any evidence of hypertrophy, and the abdo- the administration of any required blood transfusions or hormonal
men and suprapubic area also should be palpated for tenderness, treatment (eg, estrogen-containing medications), which may affect
organomegaly, or masses. the results of certain assays. If the patient is sexually active and a
In the presence of a chaperone, the SMR of the breasts should be pelvic examination is performed, an endocervical specimen should
noted and compared with pubic hair development, particularly in the be obtained for nucleic acid amplification testing for Chlamydia
adolescent with primary amenorrhea. Other signs compatible with trachomatis and Neisseria gonorrhoeae. Nucleic acid amplification
gonadal dysgenesis include webbed neck, broad shield-like chest, testing of the urine or a vaginal swab to screen for gonorrhea and
short fourth metacarpal, and an increased carrying angle of the chlamydia are also available (eg, ligase chain reaction, polymerase
arms. The presence or absence of galactorrhea also should be noted chain reaction). Follicle-stimulating hormone, luteinizing hormone,
by gently squeezing each nipple. With the patient in the lithotomy or prolactin, thyroid-stimulating hormone, testosterone, free and total
frog-leg position, the external genitalia should be carefully inspected testosterone, and dehydroepiandrosterone sulfate studies should be
for clitoral size (normal clitoral glans width, 2–4 mm) and patency of performed in the patient with a history of chronic anovulation or in
the hymen via gentle separation/traction of the labia majora. Passing whom androgen excess is suspected (eg, polycystic ovary syndrome).
a saline-moistened cotton swab gently through the vaginal introi-
tus can help determine vaginal length as well as the presence of a Imaging Studies
transverse or longitudinal vaginal septum. A bimanual vaginal or Transabdominal or transvaginal pelvic ultrasonography can be help-
rectoabdominal examination may be performed in the adolescent ful in the patient with excessive uterine bleeding or amenorrhea if
with primary amenorrhea who is not sexually active to ensure the a mass is suspected or palpated on physical examination. Complex

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 Chapter 61: Menstrual Disorders 423

congenital obstructive anomalies, such as a longitudinal vaginal sep- Table 61.1. Nonsteroidal Anti-inflammatory
tum with hemi-obstruction, cervical agenesis or stenosis, or a par- Drugs Used in the Management of
tially obstructing uterine septum, may require magnetic resonance Primary Dysmenorrhea
imaging if pelvic ultrasonography is inconclusive. Plain radiogra-
Generic Sample Trade Name(s) Dosage
phy is not indicated. Magnetic resonance imaging of the head may
be indicated in the adolescent with amenorrhea if the patient pre­ Ibuprofen Motrin, Advil (200 mg/tablet) 2–3 tablets every
sents with central nervous system symptoms or has markedly ele- 6–8 hours for
vated serum prolactin. 24–72 hours
Naproxen Aleve (220 mg/tablet), Anaprox 2 tablets at onset,
Management sodium (275 mg/tablet), Naprosyn then 1 tablet every
(250 mg/tablet) 8–12 hours
Effective management of each of these adolescent gynecologic condi-
tions is multifaceted and includes education of the patient and parent Mefenamic Ponstel (250 mg/capsule) 2 capsules at onset,
or guardian, reassurance about the ease of managing the condition, acid then 1 capsule every
and appropriate medications for those conditions requiring therapy. 6 hours or 2 capsules
Generally, for most gynecologic conditions a menstrual calendar every 8 hours
can be quite helpful for confirmation of the severity of the bleeding
and assessment of the pattern and duration of each menstrual cycle. Low-dose combination oral contraceptive is indicated for the
Digital period tracking applications are available for download onto adolescent with moderate or severe dysmenorrhea who is sexually
most smartphones to assist with monthly documentation of menses active or in the patient whose symptoms are not sufficiently relieved
and associated symptoms. by NSAIDs alone and whose own medical or family history does not
preclude the use of estrogen. Oral contraceptives decrease the pro-
Dysmenorrhea duction and release of prostaglandins and leukotrienes by inhibit-
General modalities in the management of primary dysmenorrhea ing ovulation as well as endometrial growth. Because the symptoms
include education about menstruation, proper nutrition, smoking of dysmenorrhea are prevented only after several cycles of oral con-
cessation (as appropriate), application of heat (eg, heating pad), sim- traceptive pill (OCP) use, the patient should be advised not to expect
ple exercise and/or yoga, acupuncture, and pharmacologic therapies. complete resolution of symptoms during the first month of treat-
For mild to moderate symptoms of dysmenorrhea, over-the- ment. The adolescent with a classic clinical presentation of primary
counter nonsteroidal anti-inflammatory drugs (NSAIDs), such dysmenorrhea does not require a pelvic examination before initi-
as ibuprofen, are appropriate for first-line pain management and ating oral contraceptives. Even if an adolescent is sexually active,
can also reduce blood loss. Physicians most often suggest that routine STI screening can be performed using a vaginal swab or
patients use ibuprofen initially because it is both safe and effica- urine-based nucleic acid amplification testing. Reevaluation after
cious when taken in appropriate doses and frequency. The dose at least 3 cycles is indicated to document adherence and resolution
is 400 to 600 mg every 6 to 8 hours. Ibuprofen should be taken at or improvement of menstrual symptoms.
the onset of the menstrual cycle and continued for 24 to 72 hours Thirty or 35 mcg of ethinyl estradiol-containing monophasic oral
or for the usual duration of symptoms. Any girl who is unable to contraceptives should be used for a minimum of 3 to 4 months. If
take the medication at the onset of menses secondary to vomit- symptoms do not improve, an NSAID can be added to the treatment
ing can be instructed to start the NSAID 1 to 2 days before men- regimen. Oral contraceptives are more than 90% effective in cases
ses is expected to occur. of severe dysmenorrhea, and the physician should emphasize this
For moderate to severe dysmenorrhea in the patient who is not hormonal benefit to the patient and the patient’s parent or guard-
sexually active and does not desire birth control, a faster-acting ian. Although 20 mcg of ethinyl estradiol-containing OCP formula-
NSAID, such as naproxen, may be an alternative to ibuprofen tions are available, the literature remains inconclusive concerning
(Table 61.1). The major mechanism of action of NSAIDs is the the first-line use of these for primary dysmenorrhea.
inhibition of prostaglandin synthesis. A loading dose of naproxen Other combined hormonal contraceptives, such as the trans-
is recommended at the onset of therapy. Side effects of these drugs, dermal patch and the contraceptive ring, have been studied for the
which most commonly affect the gastrointestinal tract, are nau- management of primary dysmenorrhea in adult women. The use of
sea, vomiting, and dyspepsia. These reactions can be minimized by extended OCP regimens to reduce the hormone-free interval also has
taking the medication with food or an antacid. Other adverse reac- been reported to be beneficial in reducing painful menses. Specific
tions include renal effects; skin reactions, such as erythema multi- studies in adolescents, however, are extremely limited. Long-acting
forme and urticaria; and central nervous system effects, including reversible contraception also should be considered in the adoles-
headache and dizziness. Contraindications to NSAID use include cent with primary dysmenorrhea who is unable to take combined
peptic ulcer disease, clotting disorders, and renal disease. All oral contraceptives.
NSAIDs should be administered with food and taken for 3 to 4 men- If dysmenorrhea persists despite the judicious use of NSAIDs
strual cycles before their efficacy is evaluated. and contraceptives, a search for other pelvic pathology (eg,

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 424 PART 4: ADOLESCENT HEALTH

endometriosis) is warranted, and the patient should be referred estrogens are required every 4 to 6 hours for the first 24 hours to
to a gynecologist for further evaluation and possible diagnostic stop severe acute hemorrhage. Most adolescents only require 1 or
laparoscopy. 2 doses. Intravenous estrogens should not be used in the patient with
a contraindication to estrogen (eg, deep vein thrombosis) or who is
Abnormal Uterine Bleeding not currently bleeding heavily. Otherwise, in cases of moderate to
The management of abnormal uterine bleeding depends on the severe anemia, a COCP containing 30 or 35 mcg ethinyl estradiol is
severity and frequency of the bleeding, the severity of anemia, and initiated 3 to 4 times a day; the progesterone component is neces-
the underlying etiology (Table 61.2). The goal of management is sary to stabilize the endometrium. Additionally, an antiemetic agent
4-fold: to control the bleeding, correct the anemia, replenish iron often is required 1 hour before the OCP during the first few days of
stores, and prevent further episodes of bleeding. The patient with therapy. After bleeding is controlled, the frequency of OCP admin-
mild or moderate anemia can be treated as an outpatient with weekly istration can be tapered, and the adolescent can continue a mono-
to monthly follow-up depending on how quickly the bleeding is con- phasic COCP daily, skipping the placebo pills for at least the first
trolled and the anemia resolves. Regardless of the etiology, hormone cycle, and then switching to a lower-dose combination oral contra-
treatment generally is required to stabilize the endometrium and ceptive for at least 6 cycles total. Studies have demonstrated that 20%
control future bleeding episodes, particularly if the patient presents to 25% of adolescents who require hospitalization for severe anemia
with symptomatic anemia. Monophasic combined oral contraceptive within the first year after menarche have an underlying coagulopathy
pills (COCPs) are the mainstay of treatment for abnormal uterine and therefore warrant a thorough hematologic investigation. In the
bleeding in the patient with moderate anemia and/or who desires case of excessive uterine bleeding in which oral contraceptives are
birth control. Other hormone regimens, such as progestin-only used but the patient does not desire birth control, hormonal ther-
preparations (eg, medroxyprogesterone acetate), also can be apy should not be stopped until at least 3 months after the anemia
used. This is especially important in the adolescent with a med- has resolved to ensure restoration of iron stores.
ical contraindication to estrogen or disinterest in COCP therapy. Surgical treatment, such as dilatation and curettage, is rarely
Supplemental oral iron therapy is also required for all teenage girls indicated in the adolescent patient and is reserved for individuals
with anemia. Prescribing ferrous gluconate rather than ferrous refractory to aggressive medical treatment.
sulfate may improve adherence because the former is less irritat-
ing to the stomach. Premenstrual Syndrome
Most adolescent girls with severe bleeding and symptomatic ane- The early identification of PMS or PMDD in the adolescent can be
mia require a more extensive evaluation, and such patients usually facilitated using screening questionnaires such as The Premenstrual
are hospitalized for appropriate parenteral intravenous fluid therapy Symptoms Screening Tool for Adolescents, a validated tool revised for
and possible blood transfusion. Occasionally, intravenous conjugated use in teenagers that scores the severity of premenstrual symptoms

Table 61.2. General Guidelines for the Management of Abnormal Uterine Bleeding in the Adolescent
Factor Mild Anemiaa Moderate Anemiab Severe Anemiac
Hemoglobin >11 8–11 ≤7
(g/dL)
Management Reassurance, menstrual calendar, Initially, 3–4 monophasic COCPs (30–35 mcg ethi- Hospitalization if signs of hypovolemia or severe ane-
supplemental iron twice daily, nyl estradiol and potent progestational agentd) mia; consider IV estrogen until bleeding stops; begin
COCP 1 pill daily (the latter if every 6–8 hours for 2–3 days or until bleeding 3–4 monophasic COCPs (30–35 mcg ethinyl estradiol
sexually active). stops; take with antiemetic; taper to every and potent progestational agentd) every day with anti-
Consider NSAID to help reduce 12 hours for 2–3 days, then every day after emetic and taper as with moderate anemia over next
blood loss. bleeding has stopped; skip placebos in first pill 21 days; skip placebo pills for at least 1 month or until
pack; then cycle for minimum of 3–6 months; hemoglobin has normalized; then cycle with mono-
prescribe oral iron supplementation. phasic COCPs (30–35 mcg ethinyl estradiol) for
Consider NSAID to help reduce blood loss. 6–12 months; prescribe oral iron supplementation.
Menstrual calendar. Menstrual calendar.
Follow-up 2–3 months; repeat hemoglobin. 2–3 weeks for repeat hemoglobin, then every 1–2 weeks for repeat hemoglobin, then every month.
2–3 months.
Abbreviations: COCP, combination oral contraceptive pill; IV, intravenous; NSAID, nonsteroidal anti-inflammatory drug.
a
Blood pressure and heart rate stable.
b
Blood pressure and heart rate stable, moderate flow.
c
Blood pressure stable, increased heart rate, heavy flow.
d
Potent progestin: norgestrel or levonorgestrel.

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 Chapter 61: Menstrual Disorders 425

and extent to which they interfere with work, relationships, and Vaginitis
familial responsibilities. After the condition of PMS/PMDD is iden-
See Chapters 59 and 60 for a discussion of the management of vag-
tified, the adolescent can be properly evaluated and educated about
inal discharge in adolescents.
treatment options. Although various sources have advocated many
different regimens for the management of PMS, no definitive find-
ings have been reported, and no single effective treatment has been
Prognosis
demonstrated. The overall goal of therapy is to improve the adoles- Most adolescents with common menstrual symptoms who receive
cent’s quality of life by ameliorating the debilitating symptoms asso- aggressive, appropriate care are usually symptom-free after 3 to
ciated with PMS/PMDD. 4 months of continuous therapy. Complications associated with
Treatment involves education about the menstrual cycle oral contraceptive use and NSAIDs are rare in this otherwise
and PMS, supportive self-care to reduce stress and the severity healthy patient population. Symptoms associated with an immature
of symptoms, lifestyle modifications (eg, increasing exercise), hypothalamic-pituitary-ovarian axis, such as anovulatory bleeding
dietary supplementation, and the initiation of specific medica- and abnormal uterine bleeding, may also resolve spontaneously but
tions (eg, selective serotonin reuptake inhibitors [SSRIs]) for generally respond favorably to hormonal management. The prog-
severe PMS/PMDD symptoms. For example, therapies for mild nosis for the adolescent with amenorrhea depends in part on the
to moderate symptoms include diet modification for the patient underlying etiology.
whose primary symptom is bloating, promotion of regular aero-
bic exercise, education about menstrual physiology and the rela-
CASE RESOLUTION
tionship of changing hormones to symptoms, stress management,
More information should be obtained to exclude the numerous other causes of
and cognitive-behavior therapy or group therapy. Calcium supple- abnormal uterine bleeding in the adolescent before a diagnosis of anovulatory
mentation (1,200 mg daily in divided doses) and vitamin D are the uterine bleeding can be made. Questions about breast tenderness, galactorrhea,
only evidence-based dietary modifications that have been shown weight loss, fatigue, visual changes, prolonged bleeding, and easy bruising can
to consistently improve symptoms. Other vitamin and mineral be particularly important. If the adolescent has no other symptoms, a hemoglo-
supplements as well as certain herbal preparations require more bin or hematocrit as well as a complete blood cell count and a pregnancy test
should be performed. An endocervical, vaginal, or urine specimen should be sent
definitive research before their use can be recommended. Some
for nucleic acid amplification testing for gonorrhea and chlamydia. Depending on
therapies that historically had been used extensively also have the severity of anemia and the desire for contraception, the adolescent should be
been associated with undesirable outcomes, such as the devel- placed on twice-daily iron supplementation and oral combined hormonal ther-
opment of peripheral neuropathy with pyridoxine (vitamin B6) apy for at least 3 months.
at high doses.
Although it might be assumed that ovulatory suppression with
COCPs in a conventional 21-day active/7-day placebo regimen Resource
would decrease PMS symptoms, their use has in fact been associ- Center for Young Women’s Health: https://youngwomenshealth.org
ated with incomplete suppression of ovulation and an exacerbation
of PMS symptoms during hormone withdrawal. Lower estrogen
Selected References
dosing (eg, 20 mcg ethinyl estradiol), the use of the progestin
drospirenone, and extended or continuous cycling of COCPs have Allen LM, Lam AC. Premenstrual syndrome and dysmenorrhea in adolescents.
been shown to reduce PMS symptoms in adult women with PMDD Adolesc Med State Art Rev. 2012;23(1):139–163 PMID: 22764560
versus controls. American Academy of Pediatrics Committee on Adolescence; American College of
When mood symptoms predominate and significantly impair Obstetricians and Gynecologists Committee on Adolescent Health Care. Menstruation
in girls and adolescents: using the menstrual cycle as a vital sign. Pediatrics.
function, SSRIs are considered first-line treatment in adult women;
2006;118(5):2245–2250 PMID: 17079600 https://doi.org/10.1542/peds.2006-2481
however, this is not the case for teenagers. Fluoxetine, sertraline,
American College of Obstetricians and Gynecologists. Committee opinion
and paroxetine are the only US Food and Drug Administration–
no 557. management of acute abnormal uterine bleeding in nonpregnant
approved SSRIs for use in the management of severe PMS/PMDD. reproductive-aged women. Obstet Gynecol. 2013;121(4):891–896 PMID:
Although fluoxetine is approved for use in children and adoles- 23635706 https://doi.org/10.1097/01.AOG.0000428646.67925.9a
cents, it is approved for only 2 conditions: major-depressive dis- Bordini B, Rosenfield RL. Normal pubertal development: part II: clinical aspects
order and obsessive-compulsive disorder. Therefore, the decision of puberty. Pediatr Rev. 2011;32(7):281–292 PMID: 21724902 https://doi.
to prescribe an SSRI in an adolescent with severe PMS/PMDD is org/10.1542/pir.32-7-281
at the discretion of the health professional because studies spe- Ellis MH, Beyth Y. Abnormal vaginal bleeding in adolescence as the presenting
cific to adolescents are lacking and use of an SSRI in this age symptom of a bleeding diathesis. J Pediatr Adolesc Gynecol. 1999;12(3):127–131
group requires diligent monitoring by a multidisciplinary team. PMID: 10546903 https://doi.org/10.1016/S1038-3188(99)00004-2
Anxiolytic agents, specifically alprazolam, generally are not Graham RA, Davis JA, Corrales-Medina FF. The adolescent with menor-
used in adolescents because of the possible development of drug rhagia: diagnostic approach to a suspected bleeding disorder. Pediatr Rev.
dependency. 2018;39(12):588–600 PMID: 30504251 https://doi.org/10.1542/pir.2017-0105

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Gray SH. Menstrual disorders. Pediatr Rev. 2013;34(1):6–18 PMID: 23281358 Peacock A, Alvi NS, Mushtaq T. Period problems: disorders of menstruation in
https://doi.org/10.1542/pir.34-1-6 adolescents. Arch Dis Child. 2012;97(6):554–560 PMID: 20576661 https://doi.
Gray SH, Emans SJ. Abnormal vaginal bleeding in adolescents. Pediatr Rev. org/10.1136/adc.2009.160853
2007;28(5):175–182 PMID: 17473122 https://doi.org/10.1542/pir.28-5-175 Quint EH, O’Brien RF; American Academy of Pediatrics Committee
Harel Z. Dysmenorrhea in adolescents and young adults: etiology and manage- on Adolescence; North American Society for Pediatric and Adolescent
ment. J Pediatr Adolesc Gynecol. 2006;19(6):363–371 PMID: 17174824 https:// Gynecology. Menstrual management for adolescents with disabilities.
doi.org/10.1016/j.jpag.2006.09.001 Pediatrics. 2016;138(1):e20160295 PMID: 27325636 https://doi.org/10.1542/
peds.2016-0295
James AH, Kouides PA, Abdul-Kadir R, et al. Von Willebrand disease and other
bleeding disorders in women: consensus on diagnosis and management from Ryan SA. The treatment of dysmenorrhea. Pediatr Clin North Am. 2017;64(2):
an international expert panel. Am J Obstet Gynecol. 2009;201(1):12.e1–12.e8 331–342 PMID: 28292449 https://doi.org/10.1016/j.pcl.2016.11.004
PMID: 19481722 https://doi.org/10.1016/j.ajog.2009.04.024 Talib HJ, Coupey SM. Excessive uterine bleeding. Adolesc Med State Art Rev.
Laufer M. Gynecologic pain: dysmenorrhea, acute and chronic pelvic pain, endo- 2012;23(1):53–72 PMID: 22764555
metriosis, and premenstrual syndrome. In: Emans SJ, Laufer MR, eds. Emans,
Laufer, Goldstein’s Pediatric and Adolescent Gynecology. 6th ed. Philadelphia, PA:
Lippincott Williams & Wilkins; 2012:238–271

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 CHAPTER 62

Disorders of the Breast

Monica Sifuentes, MD

CASE STUDY
A 2-year-old girl is brought to the office for bilateral Questions
breast swelling first noticed 3 weeks previously by her 1. What is premature thelarche, and how can it be dif-
mother. The swelling is nontender and does not appear ferentiated from true precocious puberty?
to be increasing in size. No history exists of galactorrhea. 2. What are the most common causes of breast hyper-
The child is otherwise healthy, takes no medications, and trophy in the infant?
is not using any estrogen-containing creams or other 3. When does pubertal breast development normally
over-the-counter products or supplements. occur in females?
On physical examination, vital signs are normal, and 4. What are the most common causes of breast masses
the child is at the 50th percentile for height and weight. in adolescent females, and how should they be
A 1.5-cm, firm, nontender mass is palpated below her managed?
left nipple. Below the right nipple, a 1-cm, nontender 5. How can physiologic pubertal gynecomastia be dif-
mass of similar consistency is present. There is no dis- ferentiated from pathologic causes of gynecomastia
charge from either nipple and no areolar widening. The in adolescent males?
abdomen is soft, with no masses palpated. The genita-
lia are those of a normal prepubescent female with no
pubic hair and vaginal mucosa that appears red and not
estrogenized.

Breast disorders occur in all pediatric age groups and can become a Benign breast hypertrophy can occur in 60% to 90% of newborns
cause for significant concern for both patients and parents or guard- and occurs in both male and female term neonates. Presentation
ians. A neonate may present to the pediatrician with bilateral breast may be unilateral or bilateral. Occasionally nipple discharge occurs,
hypertrophy and galactorrhea or mastitis. Bewildered parents or particularly in the case of well-intentioned family members who try
guardians might bring in their young prepubertal daughter because to extract the milk, inadvertently promoting the central secretion of
of what appears to be early breast development. An anxious adoles- prolactin and oxytocin via breast stimulation.
cent female may notice for the first time that her breasts are asym- Congenital anomalies of the breast include polythelia, polymas-
metric, or she may feel a lump beneath the skin. An adolescent male tia, amastia, and athelia. Polythelia, or extra accessory nipples, can
can present with unilateral or bilateral gynecomastia that makes him occur anywhere along the embryonic mammary ridge (also called
uncomfortable and causes severe psychological distress. Whatever the “milk line”) from the axilla to the groin and occurs in 2% of
the underlying cause, breast problems can be disconcerting at any the general population (Figure 62.1). Reportedly, abnormalities of
age. Primary care physicians should be equipped to differentiate the urologic and cardiovascular systems have been associated with
between normal variants of growth and pathologic conditions in polythelia. Polymastia refers to supernumerary breasts along the
newborns, infants, children, and adolescents. Significant disorders milk line and occurs less frequently than polythelia. The usual loca-
are rare, but diagnosis is important so that appropriate manage- tions for supernumerary breasts are below the breast on the chest
ment can begin. or the upper abdomen. Polythelia and polymastia may be familial
and can occur bilaterally or unilaterally. Problems associated with
Epidemiology breast development, such as a tuberous breast deformity, also can
Breast problems range from congenital anomalies and benign disor- be thought of as a congenital anomaly, although it does not manifest
ders related to hormonal stimulation to breast masses and tumors. until later in puberty when breast growth is noted to be underdevel-
Serious disorders, such as primary breast cancer, are exceedingly oped or abnormal in appearance. The breasts have the appearance
rare in children and adolescents, although inappropriate breast of a tuberous plant root, with an elevated inframammary fold, nar-
enlargement or gynecomastia as a sign of another neoplastic pro- row breast base, and “herniation” of glandular tissue through the
cess is not uncommon. areolae, which are unusually large.

427

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 428 PART 4: ADOLESCENT HEALTH

Amastia (congenital absence of glandular breast tissue) and boys. In young females, this involves breast or pubic hair develop-
athelia (absence of a nipple) are rare, but their presence often is ment, and in males it involves pubic hair development or testicu-
associated with other anomalies of the chest wall, such as pectus lar enlargement. Despite well-documented ethnic variation among
excavatum. Amastia also is seen in Poland syndrome, which includes children, 7 years is considered the lower acceptable age limit for
absence of the ipsilateral pectoral muscles, various rib deformities the onset of puberty in non-Hispanic black and Mexican American
and upper limb defects (eg, syndactyly [webbed fingers]), and radial girls.
nerve aplasia (Figure 62.2). Gynecomastia may occur in adolescent males as they progress
Premature thelarche is isolated unilateral or bilateral breast through puberty and is often called “transient pubertal gynecomastia”
development in girls between 1 and 4 years of age without other or “physiologic pubertal gynecomastia.” An estimated 60% to 70% of
signs of sexual maturation (eg, pubic hair, estrogenized vaginal adolescent males are affected, with a peak incidence between ages 13 and
mucosa, acceleration of linear growth). An estimated 60% of cases 14 years or approximately 1 year after the onset of puberty. This gener-
occur between 6 months and 2 years of age, and a diagnosis after ally corresponds to sexual maturity rating (SMR) (ie, Tanner stage) 3 to
4 years of age is uncommon. In contrast, precocious puberty is the 4 genital and pubic hair development in the young male. Like breast
appearance of any sign of secondary sexual maturation before age development in the pubertal female, transient pubertal gynecomas-
8 years in girls with a normal body mass index or age 9 years in tia may be asymmetric and painful, although concurrent or sequential
involvement of both breasts can occur. It is uncommon for pubertal
gynecomastia to occur beyond age 17 or 18 years in the adolescent male.
In the adolescent female, breast masses are not uncommon;
however, clinically significant lesions are rare. Breast cancer has an
estimated annual incidence of 0.1 in 100,000 adolescents. In most
studies of patients through age 20 years, the most common benign
breast tumor is a fibroadenoma, which has been reported in 60%
to 95% of biopsied lesions. Two-thirds of these lesions are located
in the lateral quadrants of the breast, with most in the upper outer
quadrant. The peak incidence of these lesions is in late adolescence
(17–21 years of age), and they tend to occur more commonly in black
females. Reportedly, 10% to 15% of cases are bilateral. Additionally,
25% of cases involve multiple fibroadenomas.
Fibrocystic changes are the second most common histologic
diagnosis after fibroadenomas. Other breast masses include soli-
tary cysts, abscesses, lipomas, and the phyllodes tumor (also known
as cystosarcoma phyllodes), an extremely rare, rapidly growing, pain-
less breast tumor that is nearly always benign and clinically can be
Figure 62.1. Polythelia. Supernumerary nipples along the embryonic
confused with fibroadenoma, except for its aggressive growth. If
mammary ridge (milk line).
malignant, however, cystosarcoma phyllodes can metastasize hema-
togenously to the lungs.
Malignancy is reported in less than 1% of excised lesions. Fewer
than 50 cases of primary breast cancer in children and adolescents
have been reported in the literature to date. Rhabdomyosarcoma
and fibrosarcoma are among the other rarely reported primary
tumors of the breast in adolescents. Metastatic cancer of the breast
is more common than primary breast cancer and has been reported
in children with primary hepatocellular carcinoma, leukemia,
Hodgkin and non-Hodgkin lymphoma, neuroblastoma, and rhab-
domyosarcoma. Of note is the increased lifetime risk for radiation-
induced breast cancer in girls and adolescents who undergo
mantle/chest wall irradiation during peak breast development
(10–16 years of age); such irradiation typically is administered dur-
ing treatment for Hodgkin lymphoma. The breast cancer risk for
women who are survivors of Hodgkin disease is 75 times that of the
general population. According to the literature, the cumulative risk
for breast cancer during their lifetime exceeds 40% for girls who
Figure 62.2. Amastia. Unilateral (left) complete absence of breast tissue. undergo chest irradiation for treatment of Hodgkin lymphoma.

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 Chapter 62: Disorders of the Breast 429

Normal Breast Development development. Adolescent females with a breast problem often report a
unilateral breast lump noted incidentally by the teenager. It may be ten-
In the adolescent female, the first sign of puberty is breast devel-
der, fluctuant, firm, rubbery, or nodular. The adolescent also may report
opment or thelarche. This begins with the appearance of a breast
painful breasts (mastalgia) that can be cyclic in nature. For most breast
bud beneath the areola. Under the influence of estrogen, there
masses, the overlying skin is normal, but occasionally skin changes do
is an increase in the adipose tissue along with the beginning of
occur. Rarely, an associated nipple discharge may be present.
ductal and stromal growth. Progesterone initiates alveolar budding
Because most breast masses occur in females, gynecomastia is
and lobular growth and contributes to the development of secre-
particularly anxiety provoking in young adolescent males. It usually
tory lobules and alveoli. The alveoli are later lined by milk-secreting
appears as a unilateral or bilateral 2- to 3-cm firm mass beneath the
cells under the influence of prolactin when full maturation occurs
areola, which may or may not be tender. Irritation of the skin of the
during the first pregnancy.
nipple may occur resulting from prolonged friction from clothing.
The normal progression of breast growth is divided into 5 stages
Galactorrhea rarely accompanies pubertal gynecomastia and may
or SMRs. These descriptions are used to follow normal breast devel-
be indicative of self-stimulation; illicit drug use, including cannabis,
opment, which occurs in parallel with and generally precedes pubic
opiates, benzodiazepines, and amphetamines; or exposure to other
hair development. It usually takes 2 to 4 years for the completion
medications, such as risperidone.
of breast development, although, as in all aspects of puberty, vari-
ations do occur. The practitioner should keep in mind that many
females remain in SMR 3 or 4 breast development until pregnancy. Pathophysiology
Additionally, especially between SMR 2 and 4, significant breast Neonatal breast hypertrophy seemingly is a response to mater-
asymmetry can be quite common in the adolescent without indi- nal estrogen exposure in utero. Constant stimulation can result in
cating a pathologic process. After both breasts are fully mature and persistent swelling, galactorrhea, and overt infection (ie, masti-
reach SMR 5, adequate catch-up growth usually has occurred. tis). Of note, if galactorrhea is present, it should not persist beyond
the first few weeks after birth. Generally, preterm neonates are
Clinical Presentation less responsive to maternal hormones and, therefore, breast hyper-
Neonates with breast disorders usually present in the first few weeks trophy occurs less often in this age group and its appearance may
after birth with bilateral breast enlargement that may be asymmet- be delayed for weeks.
ric (Box 62.1). They may present with associated clear or cloudy Benign premature thelarche is a variation of normal pubertal
nipple discharge. If an infection is present, the overlying skin may development with transient elevations in estrogen levels from func-
be warm and erythematous. Fever or other nonspecific symptoms, tional ovarian cysts or fluctuations in pituitary gonadotropin secre-
such as poor feeding and irritability, also may be present because tion. Often, the breast enlargement occurs without other estrogen
mastitis involves the entire breast bud; although rare, septicemia effects, such as an increase in uterine size or changes in the appear-
can occur as well. ance of the external genitalia. Typically, no linear growth or bone
In prepubertal females, benign premature thelarche presents age advancement is associated with this condition. Current research
as unilateral or bilateral nontender subareolar swelling without the is examining the potential role of leptin and its influence on sex
appearance of other secondary sexual characteristics. In contrast, steroids in the development of premature thelarche as well as puber-
girls with precocious puberty may have axillary hair, nipple and are- tal gynecomastia.
ola enlargement and thinning, and pubic hair in addition to early breast Central precocious puberty is the result of early activation of
the hypothalamic-pituitary-gonadal axis and the secretion of
gonadotropin-releasing hormone (GnRH)-dependent pituitary
gonadotropins in a pulsatile pattern. Although a search may be under-
Box 62.1. Diagnosis of Breast Disorder From
taken for an underlying central nervous system (CNS) or gonadal
Birth Through Adolescence
abnormality, most cases in females are idiopathic. In contrast, less
Neonates, Infants, Prepubescent Children, and Adolescent Males than 10% of males with precocious puberty do not have an identifi-
ww Unilateral or bilateral subareolar mass able cause, and it has been reported that approximately 50% of boys
ww Possible associated nipple discharge with precocious puberty have an identifiable intracranial process.
ww Overlying skin changes, such as erythema in neonates and infants Central nervous system tumors cause precocious puberty by imping-
Adolescent Females ing on the neuronal pathways that inhibit the GnRH pulse generator
ww Firm, rubbery, freely movable mass in childhood. Cranial irradiation, received as a part of tumor therapy,
ww Possible tenderness also can cause central sexual precocity. Pseudo-precocious puberty
ww Breast asymmetry is GnRH-independent and is caused by the extrapituitary secretion
ww Skin changes, such as shininess, venous distention, or dimpling (rare) of gonadotropins or the secretion of gonadal steroids independent
ww Possible associated nipple discharge of pulsatile GnRH stimulation. (See the article by Long in Selected
References for a general review of precocious puberty.)

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 430 PART 4: ADOLESCENT HEALTH

The cause of fibroadenomas in adolescent females is postulated For patients with suspected precocious puberty, other etiologies
to be an abnormal sensitivity to estrogen. Observations supporting must be considered in addition to exogenous hormones (Box 62.3).
this hypothesis include the presence of estrogen receptors in the Central nervous system tumors, lesions, and vascular insults are among
tumor and an increased incidence of this type of tumor during late the most common causes. Congenital tumors, such as hypothalamic
adolescence. Thus, prolonged exposure to estrogen may play a role hamartomas, are especially important to rule out because they often
in the development of fibroadenoma. Enlargement can occur dur- present before age 3 years. Other CNS tumors to consider are neurofi-
ing pregnancy or toward the end of the menstrual cycle. bromas, optic gliomas, astrocytomas, and ependymomas. Specific CNS
The definition of gynecomastia is an increase in the glandular lesions include cysts in the area of the third ventricle and congenital
and stromal tissue of the male breast. Physiologic gynecomastia is brain defects. Hydrocephalus, postinfectious encephalitis or meningitis,
thought to occur from a transient imbalance between estrogen and head trauma, and static cerebral encephalopathy also can cause sexual
androgens during puberty. Alterations in the ratio of these hormones precocity. Endocrine disorders include primary hypothyroidism, estrogen-
results in an increase in estrogen relative to testosterone. Certain producing tumors of the ovary or adrenal gland, and ovarian cysts.
medications can cause elevations in serum prolactin and lead to
gynecomastia or galactorrhea (Box 62.2). Some illicit drugs, such as Adolescents
marijuana, contain phytoestrogens that can mimic estrogen or stim- The differential diagnosis of breast masses in adolescent females
ulate estrogen receptor sites. Specific medications, such as spirono- is extensive (Box 62.4). Conditions can be distinguished from one
lactone and cimetidine, interfere with androgen receptors or induce another based on the location of the lesion; its texture, mobility, and
inhibition of enzymes necessary for steroid synthesis. size; and the speed at which it is enlarging.

Differential Diagnosis Box 62.3. Differential Diagnosis of

The differential diagnosis of breast disorders in children and adoles- Precocious Puberty
cents depends on sex and age at onset. In addition, the presence or
ww Central (true)—GnRH–dependent
absence of other secondary sexual characteristics is helpful to dif-
ww Idiopathic
ferentiate between a variation of normal pubertal development and
ww Central nervous system
a pathologic process.
—— Tumor
Infants and Children Younger Than 9 Years  Optic and hypothalamic gliomas (often associated with
In prepubertal children, the differential diagnosis of isolated early neurofibromatosis), hypothalamic hamartoma, astrocytoma,
breast development includes exposure to exogenous sources of estro- ependymoma, craniopharyngioma
gen, such as skin creams that may contain tea tree or lavender oil, —— Lesion
makeup, and medications (eg, oral contraceptives).  Congenital defects, hydrocephalus, cyst in the third ventricle
—— Insult
 Postinfectious encephalitis or meningitis, static encephalopathy
Box 62.2. Causes of Galactorrhea —— Infection
 Abscess, tuberculous granulomas of the hypothalamus
ww Mechanical stimulation of the nipple —— Head trauma
ww Medications —— Sequela of cranial radiation
—— Opiates —— Sarcoid granuloma
—— Estrogens ww Endocrine
—— Digitalis —— Hypothyroidism
—— Butyrophenones (haloperidol) —— Secondary to GnRH-independent precocious puberty
—— Phenothiazines (21-hydroxylase deficiency, Albright syndrome)
—— Risperidone —— Peripheral (pseudo-)—GnRH-independent
—— Metoclopramide —— Adrenal
—— Isoniazid  Tumor
—— Reserpine  21- or 11-hydroxylase deficiency
—— Cimetidine ww Gonadal
—— Benzodiazepines —— Tumor
—— Tricyclic antidepressants  Albright syndrome
ww Illicit drugs  Familial testotoxicosis
—— Marijuana —— Ectopic human chorionic gonadotropin–secreting tumor
—— Heroin —— Exogenous steroids
ww Hypothalamic-pituitary disorders
Abbreviation: GnRH, gonadotropin-releasing hormone.

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 Chapter 62: Disorders of the Breast 431

Box 62.4. Causes of Breast Masses in the Box 62.5. Causes of Type 2 Gynecomastia in the
Adolescent Female Adolescent Male
ww Fibroadenoma ww Idiopathic
ww Breast abscess ww Hormone-secreting tumors
ww Breast cyst —— Seminomas (account for 40% of germ cell tumors)
ww Juvenile (giant) fibroadenoma —— Leydig cell tumor
ww Cystosarcoma phyllodes (benign) —— Teratoma
ww Fat necrosis (secondary to trauma) —— Feminizing adrenal tumor
ww Lipoma —— Hepatoma
ww Hematoma —— Bronchogenic sarcoma (ectopic human chorionic gonadotropin
ww Intraductal papilloma production)
ww Adenocarcinoma ww Thyroid dysfunction (hyperthyroidism and hypothyroidism)
ww Rhabdomyosarcoma ww Renal failure and dialysis
ww Angiosarcoma ww Chronic liver disease/cirrhosis of the liver
ww Lymphoma ww Klinefelter syndrome (XXY)
ww Cystosarcoma phyllodes (malignant) ww Testicular feminization syndrome (partial androgen insensitivity
syndrome)
ww Drugs (prescription medications and substances of abuse)
—— Marijuana, amphetamines, heroin, methadone
According to some authors, gynecomastia can be classified as —— Alcohol
type 1, 2, or 3 based on physical examination findings. Type 1 is con- —— Anabolic steroids/androgens
sistent with benign pubertal hypertrophy. The differential diagnosis —— Estrogens, testosterone
for types 2 and 3 includes physiologic gynecomastia (no evidence —— Growth hormone
of an underlying disease process); organic disorders, such as hyper- —— Cimetidine, ranitidine
thyroidism, liver disease, and testicular or adrenal neoplasms; rare —— Omeprazole
genetic syndromes, such as Klinefelter syndrome; and side effects —— Digitalis
of certain prescription medications, over-the-counter supplements, —— Spironolactone
or drugs of abuse (Box 62.5). —— Phenytoin
Persistent galactorrhea can be caused by several conditions —— Tricyclic antidepressant agents
in addition to excessive stimulation of the nipple from sexual —— Anxiolytic agents: diazepam, buspirone
activity or constant friction to the area. Other etiologies include —— Risperidone
neurologic, hypothalamic, pituitary, and endocrine disorders. —— Selective serotonin reuptake inhibitors
Common causes in the adolescent female are prolactin-secreting —— Cancer chemotherapeutic agents: alkylating agents, methotrexate
tumors and hypothyroidism. The same drugs that induce —— Isoniazid
galactorrhea in females can cause gynecomastia in males (see —— Ketoconazole
Boxes 62.2 and 62.5). —— Highly active antiretroviral treatment
ww Over-the-counter herbal supplements or skin care products containing
Evaluation lavender, tea tree oil, or other oils with estrogen-like actions
ww Pseudogynecomastia (adipose tissue in male with obesity)
History
In the infant or child, the history should focus on endogenous as
well as exogenous sources of estrogen (Box 62.6). Additionally, it is
important to ascertain from the parent or guardian whether a growth Physical Examination
spurt has occurred as well as if other physical features of puberty The physical examination includes an assessment of the patient’s lin-
have appeared. With teenagers, it is important to inquire about med- ear growth, especially in cases of suspected precocious puberty. The
ications; complementary and alternative therapies, including herbal height and weight should be plotted on the growth curve and com-
remedies, supplements, and illicit drug use; and a history of sys- pared with previous measurements. Accelerations in height occur in
temic illness. All adolescent patients should be interviewed alone, sexual precocity. Excessive weight gain also should be noted; obesity
especially when discussing illicit substance use (see Chapter 63). can simulate breast enlargement in young females and gynecomas-
The adolescent male may feel particularly embarrassed and self- tia in males, and adipose tissue can be mistaken for breast develop-
conscious given the nature of his visit; thus, the physician should ment if the tissue is not palpated correctly.
be especially patient and supportive during both the interview and The extent of the breast examination depends on the age of the
the physical examination. patient. In infants and young children, the breast tissue should be

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 432 PART 4: ADOLESCENT HEALTH

and supine positions, as is done in adult women. Most adolescent

Box 62.6. What to Ask
females, however, may be uncomfortable with so extensive an exam-
Breast Disorder ination. The physician must take the time to explain the reasons for
Prepubertal Children the examination to help the patient feel less self-conscious. All phy-
ww At what age was the breast mass first noted? sicians and other health professionals should ensure that a female
ww Does it seem to be increasing in size? staff member is present to chaperone during the breast examination.
ww Is it tender or erythematous? Visual inspection should assess for SMR, appearance of the skin,
ww Is there associated discharge from the nipple? breast symmetry, and evidence of trauma. Shiny skin or superficial
ww Has the child been exposed to any estrogen-containing skin creams, venous distention on 1 breast would indicate the possible presence of
medications, or other products? an underlying large mass. A peau d’orange (ie, orange peel) appear-
ww Is the child or adolescent using any herbal supplements, such as ginseng ance of the skin or erythema and warmth should be noted as a sign
or fennel, or products that contain tea tree or lavender oil? of an infiltrative lesion or infection, respectively.
ww In the male patient, is he taking any medications known to cause Palpation of the breast mass can be accomplished with the patient
gynecomastia, such as digoxin, omeprazole, or isoniazid? in the supine position using 1 of 3 methods. Using the second, third,
ww Is the child experiencing any neurologic symptoms, such as headache, and fourth fingers of 1 hand, the examiner should gently palpate each
ataxia, or visual disturbance? breast in a pattern of concentric circles, spokes of a wheel, or vertical
ww Is there a history of head trauma, central nervous system infection, or and horizontal lines. The location of the mass should be noted; a mass
cerebral insult? beneath the areola might indicate an intraductal lesion, whereas the
ww Has the parent noted any other signs of early pubertal development upper outer quadrant is the classic location for a fibroadenoma. The
(eg, pubic hair, acne, sudden increase in height)? consistency of the lesion is important. Is it firm, rubbery, and fluc-
tuant, or irregular and lumpy? Is it freely movable or attached to the
Adolescent
chest wall? Fibroadenomas tend to be nontender, firm, discrete, freely
ww When was the breast mass first noticed, and where is it located?
movable, and rubbery. A tender, poorly defined mass is consistent
ww Does it seem to be increasing in size?
with a contusion; a hematoma is more sharply defined with associ-
ww Is the lesion tender?
ated skin ecchymoses. Fat necrosis can occur after trauma and is pain-
ww Is there a history of trauma to the breast?
less, firm, well circumscribed, and mobile. The size of the mass should
ww Is there any discharge from either nipple?
be measured and recorded. It is important to remember that tumor
ww In the female, when was the last menstrual period?
size does not generally correlate with malignant potential. Tender,
ww Is there a history of headache or visual disturbances?
diffuse, cord-like thickening, which may feel like a beanbag, may be
ww Are there any signs or symptoms of systemic illness, such as weight loss?
indicative of fibrocystic breast changes. The mean age at presenta-
ww Is there a family history of breast cancer in a first-degree relative,
tion of fibrocystic breast changes is mid-adolescence. This condition
particularly the mother? If so, what was her age at diagnosis?
may involve 1 or both breasts, is more apparent around the time of
ww Is there a history of chest wall radiation or treatment with chemothera-
menses, and may be exacerbated by the consumption of caffeinated
peutic agents?
beverages. Limiting caffeine consumption may provide symptomatic
ww In the male, is he taking any medications that can cause gynecomastia,
relief. Finally, each nipple should be gently squeezed to check for a
such as cimetidine?
discharge. If present, whether it is clear, milky, or bloody should be
ww Is the adolescent male using anabolic steroids, supplements, or
noted. A serosanguineous or sanguineous discharge is indicative of
topical oils?
an intraductal mass. Additionally, a retracted nipple is indicative of
ww Is the adolescent using any other illicit substances, such as marijuana or
involvement of the areolar area.
heroin?
In the adolescent male, the breast tissue should be gently pal-
ww Is there a family history of gynecomastia or abnormal sexual
pated in the supine position to distinguish between fat deposi-
development?
tion without glandular proliferation in the overweight patient (ie,
pseudogynecomastia, adipomastia) and true gynecomastia. Type 1
gynecomastia presents as a unilateral or bilateral freely mobile sub-
measured and the size recorded so that growth can be monitored areolar nodule, which is generally up to 3 cm in size. Enlargement
over time. Consistency of the tissue and mobility also should be eval- beyond the areolar perimeter is consistent with type 2 gynecomas-
uated. Breast growth as a result of neonatal breast hypertrophy and tia. Type 3 resembles SMR 3 breast development in girls. The breast
benign premature thelarche is nontender, firm, and freely mobile. tissue associated with types 1 and 2 also is firm and rubbery and
The nipple should be examined for a clear or cloudy discharge by may be tender to palpation, whereas type 3 has a consistency simi-
gently compressing each nipple separately. lar to female breast tissue.
In the adolescent female, the breast examination should include The examiner should palpate for axillary lymphadenopathy.
visual inspection of the breasts as well as palpation of any lesions Although most physicians associate it with breast cancer, it also
and axillary nodes. Ideally, this would be performed in the sitting may be found with infection or necrosis of a benign tumor. In the

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 Chapter 62: Disorders of the Breast 433

adolescent male, in addition to palpation of the breast tissue, it is Imaging Studies

important to evaluate liver size and texture as well as define the SMR
Left hand and wrist radiographs to determine the child’s bone
of the genitalia to determine whether pubertal development is con-
age should be obtained in children with a diagnosis of precocious
sistent with the gynecomastia. The testicles should be palpated care-
puberty. Additionally, magnetic resonance imaging of the brain
fully for size and consistency and the presence of masses, nodules, or
should be obtained in boys to evaluate for a CNS lesion or structural
asymmetry. Any evidence of atrophy (ie, decrease in testicular size)
abnormality in the hypothalamus or pituitary gland. Depending on
should be noted. Additionally, findings suggestive of hypothyroid-
the history, magnetic resonance imaging may be indicated in some
ism or hyperthyroidism, liver disease, or other stigmata suggestive
girls. Pelvic ultrasonography, although rarely necessary, can be per-
of a syndrome must be investigated.
formed to rule out the presence of an ovarian tumor or cyst in girls
A detailed neurologic examination, including examination of
with precocious puberty.
the fundi, should be performed—especially in children with sexual
Mammography is not recommended as part of the evaluation of
precocity—to confirm or detect a CNS disorder.
a breast mass in the pediatric age group. Particularly in female ado-
Laboratory Tests lescents, mammograms can be very difficult to interpret because
of the dense fibroglandular breast tissue, which reduces the over-
The laboratory workup for breast disorders is dictated by the find-
all sensitivity of the examination. Exposing developing breast tissue
ings on history and physical examination. Isolated neonatal breast
to ionizing radiation and the associated cellular changes that may
hyperplasia and premature thelarche require no laboratory stud-
result in future malignancy is also a consideration. Additionally,
ies. Although serum gonadotropin concentrations (ie, luteinizing
the risk for primary breast cancer in an otherwise healthy adoles-
hormone, follicle-stimulating hormone) and estradiol levels can be
cent girl with no risk factors is extremely low. Breast ultrasonogra-
obtained in girls with precocious puberty, definitive demonstration
phy is generally the primary imaging modality for the evaluation of
of an activated hypothalamic-pituitary axis generally requires the
a breast mass for several reasons. It accurately confirms the pres-
administration of a GnRH stimulation test by a pediatric endocrinol-
ence of the mass and helps distinguish between a cystic lesion and
ogist. In addition, a morning testosterone level should be ordered in
a solid mass. Ultrasonography is noninvasive and causes no pain
boys. Based on these results, further studies may be indicated, such
for the anxious teenager. It can also provide more accurate mea-
as a serum human chorionic gonadotropin (hCG) level.
surement of the size of the lesion and its location prior to an image-
For most breast lesions in adolescent females, no laboratory stud-
guided biopsy. Finally, specific characteristics of the lesion, such as
ies are needed. Ultrasonography-guided fine-needle aspiration of the
the shape, margins, and presence of microcalcification, can help dif-
mass may be performed in patients with an apparent discrete collec-
ferentiate benign and malignant lesions.
tion of fluid. The aspirated fluid should be sent for culture and antibi-
Testicular ultrasonography to search for a tumor is indicated in the
otic sensitivities. Sometimes, to relieve patient and parental anxiety
adolescent male if the level of serum hCG or estradiol is elevated or
or to better identify the etiology of the mass, it is necessary to per-
if a testicular mass is noted on physical examination. Other imaging
form another procedure (eg, core needle biopsy) if imaging features
studies for the evaluation of gynecomastia generally are not useful.
are atypical or the lesion has undergone rapid growth. Excisional
biopsy of the mass allows for more accurate histologic information
but is rarely indicated for most lesions. Management
In the healthy adolescent male with gynecomastia and no evi- In neonates, mastitis most often is caused by Staphylococcus aureus
dence of systemic illness, no history of medication use or illicit and group B streptococcus. Gram-negative bacilli also have been
substance abuse, and an otherwise normal physical examination reported. Parenteral antibiotics should be initiated when the diag-
(including the testicular examination), no further laboratory studies nosis is made. Incision and drainage of a breast abscess is rarely
are necessary. If pubertal gynecomastia and other systemic illnesses indicated and should only be undertaken by an experienced sur-
are ruled out, an endocrine workup is appropriate to elucidate the geon familiar with the anatomy of the breast to minimize the like-
cause of nonpubertal gynecomastia. This should begin with morning lihood of injury to the affected breast bud.
serum levels of hCG, luteinizing hormone, follicle-stimulating hor- Congenital anomalies, such as polythelia and polymastia, do not
mone, and serum testosterone and estradiol because circadian varia- require any treatment. If, however, the patient or parent/guardian
tion may affect their interpretation. Dehydroepiandrosterone sulfate, wants the tissue removed for aesthetic or psychological reasons, it
thyroid function tests, renal function studies, and liver enzymes also is recommended to do so before puberty. Reconstructive surgery
may be useful. If these laboratory studies are normal, the diagno- for amastia, as in Poland syndrome, is typically delayed until late
sis is idiopathic gynecomastia. If they are abnormal, consultation adolescence to allow full development of the unaffected breast.
with a pediatric endocrinologist or adolescent medicine special- Although no treatment is required for benign premature the-
ist should occur. larche, parents and guardians need to be reassured that the condi-
A pregnancy test and serum prolactin level should be ordered tion is self-limited. The patient should be reexamined periodically
in the adolescent female with galactorrhea, regardless of the men- to check for any further progression of puberty, such as persistent
strual history. breast growth, the appearance of pubic hair, or a growth spurt.

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 434 PART 4: ADOLESCENT HEALTH

If no underlying medical condition has been discovered, the Type 2 gynecomastia may require surgical reduction of the mam-
treatment for most children with central precocious puberty is mary gland, although some studies have shown success with med-
directed at controlling the secondary sexual development with ical therapy. The off-label use of 10 to 20 mg of tamoxifen orally
GnRH agonists/analogue therapy. When GnRH is administered on twice daily for 3 months has been shown in some cases to decrease
a routine basis or continuously, gonadotropin secretion decreases, breast tenderness and pain, followed by a decrease in breast tissue.
which delays further pubertal development. Leuprolide acetate Surgical intervention may be warranted after a period of observation
is the GnRH agonist most often prescribed in the United States. for at least 12 months in cases of nonobese males with intractable
Children should be followed every 1 to 3 months in conjunc- breast pain or tenderness, persistent breast growth, and/or signifi-
tion with a pediatric endocrinologist to monitor their progres- cant psychological distress. Surgery currently involves a combina-
sion and response to therapy. The child continues to receive the tion of ultrasonic liposuction and direct excision of the breast tissue
medication until they reach the normal age of puberty, at which beneath the nipple and areola. The adolescent male may benefit from
point the medication is discontinued and the process of puberty concurrent psychological counseling as well.
begins again. Other underlying causes for gynecomastia should be treated
Unless otherwise indicated by the type of tumor, children with a accordingly, and any drugs or medications contributing to the con-
CNS lesion as a cause of precocious puberty usually do not require dition or associated galactorrhea should be discontinued, if possible.
neurosurgical intervention. Therapy should focus on minimizing
the degree of growth acceleration and the development of second- Prognosis
ary sexual characteristics. The prognosis of a breast disorder in the child or adolescent depends
Most breast masses in adolescent females are small, well demar- on the particular lesion. Generally, most lesions, such as neonatal breast
cated, firm or rubbery, and nontender and can be managed with the hyperplasia and premature thelarche, are self-limited and resolve spon-
“wait and watch” approach. The patient should be followed every taneously. Breast development persists for 3 to 5 years in 50% of cases of
3 to 4 months, preferably allowing a few menstrual cycles to pass premature thelarche, but in 1 retrospective study, most cases regressed
between each visit. If there is no change in the lesion or just a small within 6 months to 6 years after the diagnosis. Aside from the short stat-
increase in its size, no studies or procedures are indicated because ure that may accompany idiopathic central precocious puberty, these
it is most likely a fibroadenoma. As previously noted, a core needle females also tend to have a good prognosis. Fibroadenoma in the ado-
biopsy may be requested to relieve the anxiety for the parent/guard- lescent female can recur but typically is benign and has no direct asso-
ian and the teenager that accompanies the presence of a breast lesion ciation with the development of breast cancer. Most cases of pubertal
and to confirm the diagnosis. Total excision of the tumor mass and a gynecomastia in adolescent males resolve within 1 to 3 years.
careful histologic evaluation may be warranted in some cases, espe-
cially if the mass is painful or rapidly enlarging and exceeds 3 cm;
however, excision is unnecessary in most instances. According to the
literature, if findings on physical examination, imaging, and biopsy CASE RESOLUTION
are consistent with a benign lesion, the diagnosis of a benign mass The child has a diagnosis of premature thelarche. She has no known exposure to
can be made with 99% accuracy. If this is the case, no further pro- exogenous sources of estrogen or alternative therapies that are associated with
breast growth and has isolated breast tissue development with no other second-
cedures are indicated and follow-up with the patient every 6 months ary signs of pubertal maturation. Her parents should be informed of this diag-
to 1 year is sufficient. Surgical removal of a tumor that progressively nosis and reassured that the condition is self-limited and does not indicate that
enlarges over several months (eg, giant fibroadenoma) is important. the child is starting puberty. The child should be scheduled for a follow-up visit
This type of fibroadenoma is greater than 5 cm in size at onset or in 3 to 4 months to remeasure the breast buds and reexamine the genitalia for
appears soon after menarche and accounts for 4% to 10% of fibroad- the appearance of pubic hair as well as to monitor the patient’s linear growth.
enomas of the breast. When the lesion becomes very large, an accept-
able cosmetic result is more difficult. Accordingly, surgical removal
of a giant fibroadenoma shortly after the time of diagnosis rather Selected References
than watchful waiting is warranted. It is important to note that any
De Silva NK. Breast disorders in the female adolescent. Adolesc Med State Art
adolescent patient with a palpable breast mass and a past history of Rev. 2012;23(1):34–52, x PMID: 22764554
malignant disease or family history of breast cancer should be eval-
De Silva NK, Brandt ML. Disorders of the breast in children and adolescents, part 1:
uated aggressively and referred to a pediatric surgeon or breast sur- disorders of growth and infections of the breast. J Pediatr Adolesc Gynecol.
geon directly for a diagnostic excisional biopsy. 2006;19(5):345–349 PMID: 17060019 https://doi.org/10.1016/j.jpag.2006.06.006
The primary treatment for physiologic pubertal gynecomas- De Silva NK, Brandt ML. Disorders of the breast in children and adolescents,
tia, or type 1, is reassurance for the adolescent male and his family part 2: breast masses. J Pediatr Adolesc Gynecol. 2006;19(6):415–418 PMID:
that the condition is self-limited in 75% to 90% of adolescents and 17174833 https://doi.org/10.1016/j.jpag.2006.09.002
should regress spontaneously within 1 to 2 years, although some Diamantopoulos S, Bao Y. Gynecomastia and premature thelarche: a guide for
sources report up to 3 years. In most cases, the boy should be reex- practitioners. Pediatr Rev. 2007;28(9):e57–e68 PMID: 17766590 https://doi.
amined periodically (ie, every 6 months) until resolution occurs. org/10.1542/pir.28-9-e57

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 Chapter 62: Disorders of the Breast 435

Ezer SS, Oguzkurt P, Ince E, Temiz A, Bolat FA, Hicsonmez A. Surgical treat- Lemaine V, Cayci C, Simmons PS, Petty P. Gynecomastia in adolescent
ment of the solid breast masses in female adolescents. J Pediatr Adolesc Gynecol. males. Semin Plast Surg. 2013;27(1):56–61 PMID: 24872741 https://doi.
2013;26(1):31–35 PMID: 23158756 https://doi.org/10.1016/j.jpag.2012.09.004 org/10.1055/s-0033-1347166
Frazier AL, Rosenberg SM. Preadolescent and adolescent risk factors for benign Long D. Precocious puberty. Pediatr Rev. 2015;36(7):319–321 PMID: 26133309
breast disease. J Adolesc Health. 2013;52(5 suppl):S36–S40 PMID: 23601609 https://doi.org/10.1542/pir.36-7-319
https://doi.org/10.1016/j.jadohealth.2013.01.007 Rosenfield RL, Lipton RB, Drum ML. Thelarche, pubarche, and menarche
Granada C, Omar H, Loveless MB. Update on adolescent gynecology. Adolesc attainment in children with normal and elevated body mass index. Pediatrics.
Med State Art Rev. 2013;24(1):133–154 PMID: 23705522 2009;123(1):84–88 PMID: 19117864 https://doi.org/10.1542/peds.2008-0146
Kaneda HJ, Mack J, Kasales CJ, Schetter S. Pediatric and adolescent breast Valeur NS, Rahbar H, Chapman T. Ultrasound of pediatric breast masses: what
masses: a review of pathophysiology, imaging, diagnosis, and treatment. AJR to do with lumps and bumps. Pediatr Radiol. 2015;45(11):1584–1599 PMID:
Am J Roentgenol. 2013;200(2):W204–W212 PMID: 23345385 https://doi. 26164440 https://doi.org/10.1007/s00247-015-3402-0
org/10.2214/AJR.12.9560
Kennedy RD, Boughey JC. Management of pediatric and adolescent breast
masses. Semin Plast Surg. 2013;27(1):19–22 PMID: 24872734 https://doi.
org/10.1055/s-0033-1343991

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 CHAPTER 63

Substance Use/Abuse
Monica Sifuentes, MD

CASE STUDY
A 17-year-old male is brought to your office by his father On physical examination, he appears healthy with
with a chief report of chronic cough. You have followed an occasional dry cough. He is afebrile, and his respi-
this patient and his siblings for several years and know ratory rate, heart rate, and blood pressure are normal.
the family quite well. The father appears very concerned Pertinent findings on examination include slight con-
about “this cough that just won’t go away.” The adoles- junctival injection bilaterally, nasal turbinate erythema
cent is not concerned about the cough, however, and and edema, and mild erythema of the posterior phar-
reports no associated symptoms, such as fever, sore ynx. The patient is negative for tonsillar hypertrophy. The
throat, chest pain, or sinus pain. You ask the father to remainder of the examination is within normal limits.
step out of the room for the rest of the interview and the
physical examination.
Questions
1. What are the most common manifestations of
On further questioning, the patient reports that
substance use/abuse in adolescents?
he vapes (ie, smokes electronic cigarettes) daily and
2. What are the risk factors associated with substance
has tried marijuana as well as cocaine. He denies reg-
use/abuse in adolescents?
ular use of these substances but reports exposure to
3. What other conditions must be considered when
these drugs at parties and when he spends time with
evaluating adolescents with a history of chronic
“certain friends.” The adolescent is now in the 11th
substance use/abuse?
grade, attends school regularly, and thinks school is
4. What laboratory evaluations, if any, should be
“OK.” His grades are average to above average, but he
performed for the adolescent with suspected
thinks he might fail 1 class this semester. Although he
substance use/abuse?
formerly played baseball, he stopped last year. He hopes
5. What are the specific consequences of short- and
to get a part-time job at a local fast-food restaurant
long-term use/abuse of substances such as alcohol,
this summer. Currently, he is sexually active with only
marijuana, cocaine, opiates, and hallucinogens?
females of his age and uses condoms occasionally. He
denies suicidal ideation and exposure to any firearms.

Primary care physicians are in a unique position to educate their professionals do not feel comfortable opening that avenue of con-
patients, particularly young teenagers, about alcohol and substance versation or simply do not have the time and resources to inquire
use/abuse through primary prevention and anticipatory guidance. and intervene. Time constraints, unfamiliar billing codes, and dif-
Ideally, this should begin before the teenager has first tried a cig- ficulty maintaining confidentiality for sensitive services in a busy
arette or alcoholic drink, with the physician gradually introducing office or clinic make screening for substance use challenging. As a
each topic as the preteen enters middle school and becomes accus- result, primary care physicians miss valuable opportunities to ade-
tomed to speaking to his, her, or their physician alone. Opportunities quately assess adolescents for alcohol and substance use disorders
for education include health maintenance visits, the preparticipa- and provide them with the necessary guidance to ensure their future
tion sports physical evaluation as the teenager enters high school, health, safety, and well-being.
and medical encounters for an acute injury or illness. More impor- Substance use is use of or experimentation with illicit drugs, pre-
tantly, if a primary care physician is fortunate enough to have a long- scription medications, alcohol, or tobacco. Illicit drugs include mar-
standing relationship with the teenager, the physician can identify, ijuana; cocaine; amphetamines; hallucinogens, such as lysergic acid
evaluate, and manage a substance use disorder as soon as it devel- diethylamide (LSD), mescaline, and psilocybin, which is found in
ops and assist the patient and family proactively with appropriate Psilocybe mexicana mushrooms; opiates; and phencyclidine hydro-
referrals and local resources, thereby improving the adolescent’s chloride (PCP). Substance abuse refers to the chronic use of mind-
overall outcome. altering drugs despite adverse effects. Addiction, a chronic relapsing
Ideally, all preteen and adolescent patients would be questioned disorder, is the term applied to compulsive and continued use of a
and counseled about the use of illicit substances, alcohol, and substance despite adverse consequences. Because addiction is neu-
tobacco at each health maintenance visit (see Chapters 4 and 38). rologically based, the substance may produce physical dependence
Unfortunately, this does not occur consistently because some health or symptoms of withdrawal when it is discontinued.
437

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 438 PART 4: ADOLESCENT HEALTH

36%, with nearly 20% of high school seniors reporting marijuana use
Epidemiology
1 or more times during the month preceding the survey. Daily use of
Current Trends and Prevalence Rates marijuana has been reported in 6% of high school seniors.
Adolescents in the United States currently use a wide range of sub- The use of other substances among adolescents was generally on
stances. Alcohol, tobacco, and marijuana are by far the more com- a downward trend in the late 1980s and early 1990s; however, use is
mon and most popular substances and can serve as gateway drugs once again on the rise. This phenomenon is known in the substance
to more serious illicit drug use. Several surveys tracking substance use/abuse literature as “generational forgetting,” which occurs as
use/abuse among adolescents are conducted annually in the United acknowledgment of adverse effects of specific drugs fade over the years.
States to identify the magnitude of high-risk behavior among those Reportedly, approximately 9% of high school graduates in 1997 tried
in 8th through 12th grade. The most well-known of these surveys cocaine, with approximately 4% having used it in the previous month.
are Monitoring the Future, which is administered annually to stu- These figures remained essentially unchanged until 2007, when cocaine
dents in 8th, 10th, and 12th grade by the University of Michigan use declined; currently, use of this substance is at an historical low of
for the National Institute on Drug Abuse; the Youth Risk Behavior 1% among 12th-graders. The 1991 prevalence rate for LSD usage was
Surveillance System (YRBSS) survey, conducted biannually by the 5%, and its use also remained stable over in the next 10 years until
Centers for Disease Control and Prevention (CDC) of students in 2001, when the rate increased to 8% and became more widespread
grades 9 through 12; and the National Survey on Drug Use and than cocaine use among high school students. According to the 2017
Health, a computer-assisted interview of residents 12 years and older YRBSS survey, 9% of 12th-graders nationwide tried LSD or another
conducted in the home. It is important to remember that most statis- hallucinogenic drug. Lifetime amphetamine use among 12th-graders
tics do not include the estimated 15% to 20% of students who drop was 3% in 2017, with a range of 2.3% to almost 8% across state surveys.
out of high school before their senior year. Additionally, nationwide ecstasy use was reportedly approximately 4%.
In a 2017 survey of graduating high school seniors, approximately Concurrently, the reported use of over-the-counter (OTC) non-
60% admitted to alcohol use at some time during their life. Almost prescription stimulants that contain caffeine has increased, with
30% of students reported drinking alcohol during the month pre- popular energy drinks now sold in many convenience stores and
ceding the survey. Binge drinking likely has contributed most to the supermarkets. Other substances used to “get high,” such as inhal-
overall morbidity and mortality associated with alcohol use in ado- ants (eg, aerosol spray paints, hair sprays, paint thinners, whipped
lescents and young adults. Among high school seniors in the class cream containers), are often used by younger students (ie, preteens)
of 2017, approximately 20% reported having 5 or more drinks in a and unfortunately can be found in many garages, workrooms, and
row within a couple hours on at least 1 day during the 30 days before basements. Although the rate has decreased from 1997, in 2017
the YRBSS was administered. Although tobacco use among adoles- 7% of early adolescents (ie, ninth graders) reported sniffing or
cents decreased from 1999 to 2017, data from the CDC indicate that inhaling substances to become intoxicated. Dextromethorphan also
in 2017 approximately 10% of teenagers nationwide reported cur- has become popular as an OTC product used/abused by adolescents
rent cigarette use and another 10% percent smoked at least one-half secondary to its hallucinogenic effects and easy accessibility in cough
pack of cigarettes per day. Nationwide, the current rates of smokeless syrups. Studies confirm an increasing trend in its use/abuse, partic-
tobacco use (eg, chewing tobacco, snuff, dip) and cigar or cigarillo use ularly in teenagers younger than 18 years.
are 6% and 8%, respectively. As expected, use of smokeless tobacco is The nonmedical use/abuse of prescription drugs, such as
much higher among males than females. Electronic vapor products Oxycontin, Percocet, Vicodin, Adderall, Ritalin, and Xanax, has
(ie, electronic [e-] cigarettes, e-cigars, and e-pipes; vape pipes, vaping increased more than that of most illicit drugs in the past 2 decades.
pens, hookahs), which were introduced in the US market in the mid- Many teenagers report the ease by which prescription drugs can be
dle of the first decade of the 21st century, have become the most com- obtained, resulting in continued use/abuse and future dependence
monly used tobacco product among youth in the United States, with as an adult. In 2017, nonmedical prescription drug use was reported
many adolescents and young adults later transitioning to traditional by up to 17% of teenagers 1 or more times during their life. Certain
cigarettes. In 2017 alone, greater than 40% of high school students prescription drugs, namely opioids, stimulants, sleeping pills, and
reported ever having used an electronic vapor product. E-cigarette anxiolytics, now represent the third most widely used/abused sub-
advertising aimed at teenagers and marketing strategies promot- stance in adolescents after alcohol and marijuana.
ing flavored solutions have contributed greatly to the popularity of Although not everyone considers them an illicit substance, ana-
e-cigarettes among this age group. By 2019, there were an increasing bolic steroids are used/abused by some adolescents, mostly males,
number of reports of deaths related to vaping, and a number of states to increase muscle size and strength. In 1997, approximately 3%
issued a ban on vaping product marketing, issued a ban on flavored of adolescent males admitted to using them at some time in their
vaping solutions, or withdrew vaping products from the market. life. More recently, studies indicate as many as 5.5% of high school
Marijuana is the most commonly used illicit psychoactive sub- students participating in sports use anabolic steroids (6.6% males,
stance. In 1993, 35% of high school seniors reported ever having used 3.9% females). The 2017 nationwide figure per the CDC is almost
marijuana; in 1997, this figure increased to greater than 50%. Per 3%; however, state and local surveys indicate a range of 2% to 7%
current estimates from the 2017 YRBSS, this figure is approximately for use of anabolic steroids.

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 Chapter 63: Substance Use/Abuse 439

Demographics sexual intercourse, increased risk of pregnancy) as well as academic

problems and delinquent behavior later in adolescence. Long-term
Generally, adolescent males use illicit drugs more than females do,
effects during young adulthood include difficulties with employ-
with a few exceptions. Males are more likely to use anabolic ste-
ment, criminal and aggressive behavior, and continued substance
roids, but females reportedly use amphetamines, barbiturates, tran-
use/abuse. Potential long-term health risks associated with the early
quilizers, and OTC diet pills more than their male counterparts.
initiation of alcohol, tobacco, and substance use/abuse depend on
Additionally, although annual prevalence rates for overall alcohol use
the specific exposure but include conditions such as pulmonary dis-
show little difference by sex, adolescent males have a higher rate of
ease, chronic liver disease, cardiovascular complications, and cancer.
heavy or binge drinking compared with adolescent females. Tobacco
The role of the media and technology in adolescent alcohol and
usage is essentially the same for both sexes, except for smokeless
tobacco use has been the subject of much discussion in the past
tobacco and cigars, with more boys using these products.
20 years. Previous studies confirmed that exposure to smoking on
Adolescents who do not plan to attend college are more likely
television and in movies was 1 of the key factors that prompted teen-
to use illicit substances than their college-bound counterparts, and
agers to smoke and that preteens whose parents forbad them from
these adolescents and young adults also are more likely to use illicit
viewing R-rated movies were less likely to begin smoking or drink-
drugs more frequently. No difference between the 2 groups exists,
ing. One prospective study reported that exposure to R-rated mov-
however, in the rates of ever having tried illicit substances. Binge
ies or having a television in the bedroom significantly increased the
drinking also continues to escalate among older adolescents and
risk of initiating smoking for white teenagers. Additionally, watching
young adults attending college. The specific influence of parental
more movie depictions of alcohol use is strongly predictive of drink-
education, socioeconomic status, and race or ethnicity on the use/
ing onset and binge drinking in adolescents in the United States.
abuse of illicit substances is difficult to determine because many
Advertising also contributes to the depiction of alcohol and tobacco
other factors, such as genetics and the environment, contribute to
use as normative activities. In fact, it has been reported that adver-
heavy drug use and addiction.
tising may be responsible for up to 30% of alcohol and tobacco use
among adolescents. The influence of advertising on adolescents is
Risk Factors and Behaviors now even more apparent with the current marketing of electronic
Although alcohol and tobacco are considered licit or lawful drugs, it vapor products to attract teenage consumption.
is illegal for minors to purchase and use alcohol and tobacco in the
United States. Use of these substances often begins during adoles- Clinical Presentation
cence, however, including during the preteen years. The strongest
predictor of drug use by youth is having friends who regularly use Adolescents who are consuming alcohol or are involved in drug
drugs, that is, alcohol, tobacco, or other substances (eg, marijuana). use may present to the health professional in several different ways.
Additionally, it has been shown that the more risk factors identified, Illicit substance use might be uncovered during a routine confiden-
the greater the risk of substance use/abuse in the teenager. tial interview at an annual health maintenance visit, preparticipation
Several factors are important precursors to (ie, risk factors for) sports physical evaluation, or urgent care appointment. Alternatively,
drug use during adolescence. These risk factors include association the adolescent might have physical symptoms including chronic
with drug-using peers; attitudinal factors, such as favorable attitudes cough, persistent allergies, chest pain, and fatigue. Chronic condi-
toward drug use in the family; low religiosity; poor school perfor- tions, such as asthma, may continue to worsen despite appropriate
mance or academic failure, often beginning in the late elementary therapy in the adolescent smoking tobacco or marijuana or using
years; young age of initiation of alcohol or drug use; presence of a e-cigarettes.
conduct disorder; environmental factors, such as the prevalence of Abdominal tenderness may be noted on physical examination
drug use in a given community; history of child abuse; family his- and found to be associated with gastritis, hepatitis, or pancreatitis.
tory of alcoholism or drug use; poor parenting practices; high lev- The adolescent also may have been in a recent motor vehicle crash
els of conflict within the family; minimal bonding between parents or involved in other trauma, or their parent or parents may report
and children; family disruption; and early and persistent problem that their teenager exhibits frequent mood swings, irritability, or
behaviors during childhood, such as untreated attention-deficit/ erratic sleep patterns.
hyperactivity disorder (ADHD).
It is well documented in the literature that early age of onset of Pathophysiology
alcohol and tobacco use is predictive for the use of other drugs, a Although several theories have been proposed to explain why casual
greater variety of drugs, and more potent agents. Additionally, the substance use develops into use/abuse and addiction in some adoles-
use of alcohol at an early age is associated with future alcohol-related cents and young adults and not others, the most critical factor seems
problems, such as lifetime alcohol dependence and use/abuse. The to be the presence of underlying psychopathology. Adolescents who
early initiation of alcohol also results in increased sexual risk- have untreated major depressive disorders, ADHD, or schizophrenia,
taking behavior during adolescence (ie, unprotected sexual intercourse, for example, may use mood-altering substances to manage unpleas-
exposure to multiple sexual partners, being drunk or high during ant feelings of dysphoria and low self-esteem. Initially used as a

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 440 PART 4: ADOLESCENT HEALTH

temporary measure, this method of self-medication increases the the health professional during the actual visit. Some questionnaires
likelihood of chronic substance use/abuse. address only issues concerning substance use/abuse, whereas others
It is well known that genetic influences also play a major role in are more general but also include questions about alcohol, tobacco,
adult use/abuse of alcohol; however, less evidence exists for adoles- and drugs (Figure 63.1 and Box 63.1). Controversy exists about the
cent drug use. What is known is that families and parental attitudes role of such questionnaires, primarily concerning the truthfulness
play a significant role in the development of alcohol and other drug of answers, because parents or guardians may be with teenagers as
use in teenagers. Permissive attitudes toward alcohol and drug use they are attempting to complete the form. Administering question-
by parents or guardians and parental or older sibling drug use in the naires via technology and in a designated, private space can help
setting of other environmental risk factors are predictive of increased improve honesty.
drug and alcohol use in the adolescent. More specific questions about the use of alcohol and tobacco as
well as illicit substances should be asked after general subjects have
Differential Diagnosis been discussed (Box 63.2). If adolescents seem wary of answering
these questions, it may be helpful to initially inquire about their
The differential diagnosis for symptoms and behaviors associated
friends. Questions should be phrased with the assumption that the
with substance use/abuse includes underlying psychiatric disorders.
responses will be affirmative (eg, “How many beers do your friends
Affective, antisocial, and conduct disorders as well as ADHD can be
drink in a week? And do you drink the same amount?”). It is hoped
the primary or secondary condition in adolescents who are abusing
drugs. Like adults, adolescents may use illicit drugs to self-medicate that this less-threatening approach invites more honest answers. An
associated depression, anxiety, or auditory hallucinations. The phar- assessment of the risk of suicidal behavior is also indicated.
macology and toxicity of the illicit substances most commonly used Because many physicians lack unlimited time to interview ado-
by adolescents are summarized in Table 63.1. lescents and obtain all the details in a single visit, various standard-
ized methods have been developed to efficiently screen teenagers
for substance use in the context of health supervision visits. Brief
Evaluation
screening tools that are both self- and interviewer-administered
History can be used to glean important information even in a busy prac-
The interview should be conducted in a private, quiet area to min- tice. For example, 1 screening tool uses the following 3 questions:
imize interruptions. If parents or guardians have accompanied the During the past 12 months, have you smoked marijuana? Have
adolescent, they should be politely asked to leave the room after they you drunk any alcohol? Have you used anything else to get high?
have had an opportunity to express their concerns and after issues of If an adolescent answers “no” to all 3 questions, the patient should
confidentiality are addressed in the presence of both parties. Doing still be asked if he or she have ridden in a car with a driver who
so helps avoid future uncomfortable moments when a parent or was high or had been using alcohol or drugs. Additional screen-
guardian returns and asks what was disclosed in their absence. After ing is recommended for any teenager who answers “yes” to any
parents or guardians have left the room, issues addressing confiden- of the 3 initial screening questions. Six questions, known as the
tiality and privacy should be reviewed once again with the patient. CRAFFT screening tool, are then reviewed with the adolescent to
Special circumstances, such as a disclosure of sexual or physical further identify drug and alcohol risk or problems associated with
abuse or possible suicidal ideation or homicidal intent, that dic- their use. The teenager receives 1 point for each “yes” answer; a
tate that confidentiality be broken also should be discussed with total score of 2 or more indicates a positive result and high risk for
the teenager before proceeding with the interview (see Chapter 4). a substance use disorder. It also indicates the need for additional
The interview should proceed in a casual, non-pressured, non- follow-up as well as referral to a mental health professional or thera-
judgmental fashion. Initial inquiries should address less threaten- peutic treatment program. The validity of this brief, developmentally
ing general topics, such as school, home life, and outside activities, appropriate tool for screening adolescents has been reported in the
including activities with friends. Use of the HEADSS (home, literature and is well supported by experts in the field of adolescent
employment and education, activities, drugs, sexuality [including and addiction medicine for use by primary care physicians. Another
a history of sexual abuse or assault], suicide and/or depression) screening tool, funded by the National Institute on Drug Abuse, is
assessment allows for a thorough review of the essential compo- the Screening to Brief Intervention tool (S2BI). It is used to assess
nents of the psychosocial history (see Chapter 4). Another inter- the frequency of past-year substance use for tobacco, alcohol, mar-
view tool, the SSHADESS (strengths, school, home, activities, drugs ijuana, and 5 other classes of substances (Figure 63.1). Depending
and alcohol, substance use, emotions and depression, sexuality, on the results of the S2BI tool, motivational intervention is recom-
safety) assessment, has been developed to emphasize and review mended as the next step.
positive components in an adolescent’s life in addition to any high-
risk behavior. Physical Examination
Some practitioners prefer to use questionnaires or other formal Positive findings on physical examination are rare, especially in
validated screening tools to initially obtain this background infor- adolescents who consume alcohol or other substances only occasion-
mation. A questionnaire is given to patients to fill out while they ally. In adolescents with a history of chronic substance use/abuse,
are waiting to be seen, and responses are reviewed privately with however, certain physical findings may be present.

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 Chapter 63: Substance Use/Abuse 441

Table 63.1. Pharmacology and Toxicity of Substances Commonly Used by Adolescents

Substance Pharmacology Effects Toxicity
Nicotine Potent psychoactive drug that acts Stimulation, relaxation, focused attention Nontolerant individuals: weakness,
on receptors in the CNS to produce nausea, vomiting, feeling unwell
effects
Marijuana Active ingredient (ie, delta- Low dose: euphoria, relaxation, time Acute: panic attacks, psychosis (rare)
9-tetrahydrocannabinol) is rapidly distortion, auditory and visual enhancement Chronic: short-term memory
absorbed into the bloodstream from High dose/toxic: mood fluctuations, impairment, amotivational
inhaled smoke hallucinations, paranoia, psychosis syndrome, reduced sperm counts
Alcohol Causes nerve cell membranes to Mild: disinhibition, euphoria, mild impaired Severe: confusion, stupor, coma,
expand and become more “fluid,” coordination, mild sedation respiratory depression
thereby interfering with neuronal Moderate: increased sedation, slurred speech,
conduction ataxia
Interference of neurotransmitters
Cocaine Increased release and decreased Produces a sense of well-being and Delirium, confusion, paranoia,
reuptake of biogenic amines causing heightened awareness, decreased social hypertension, tachycardia,
CNS and peripheral nervous system inhibition, and intense euphoria hyperpyrexia, mydriasis
stimulation
Local anesthesia
Vasoconstriction
Stimulants (eg, amphetamines, CNS stimulation (ie, sympathomimetic) Heightened awareness; restlessness and Hypertension, hyperthermia,
crystal methamphetamine, agitation, decreased appetite seizure, stroke, coma, arrhythmia
ecstasy) Low doses increase ability to concentrate
Hallucinogens (eg, LSD, Inhibits release of serotonin Distortions of reality—perceptual Paranoia, flashbacks, psychosis,
mescaline, mushrooms) alterations, synesthesia common (eg, depression
“hearing” smells)
PCP Dissociative anesthetic with Dissociative anesthetic Psychoses; aggressive, violent
analgesic, stimulant, depressant, Dose-dependent euphoria, dysphoria, behavior; depression; seizure,
and hallucinogenic properties perceptual distortion rhabdomyolysis
Opiates (eg, opium, heroin, Binds to opioid receptors in CNS, Sedative analgesics Respiratory depression, CNS
methadone hydrochloride) causing CNS depression Euphoria followed by sedation, somnolence depression, miosis, bradycardia,
hypotension, arrhythmia, seizure,
rhabdomyolysis
Sedatives (eg, benzodiazepines, CNS depression, binds to specific Sedation, anxiety reduction Similar to opioid intoxication
barbiturates) receptor that potentiates GABA
Anabolic steroids Bind to androgen receptors at the Euphoria; increased irritability and Psychosis
cellular level, stimulate production aggressiveness; at high doses, induction
of RNA and protein synthesis of mental changes
Inhalants CNS stimulation and excitement, Euphoria, hallucinations, psychosis Respiratory depression, arrhythmia,
progressing to depression seizure, sudden sniffing death
syndrome (ie, sudden death second-
ary to arrhythmia)
Abbreviations: CNS, central nervous system; GABA, γ-aminobutyric acid; LSD, lysergic acid diethylamide; PCP, phencyclidine hydrochloride.
Derived from Schwartz B, Alderman EM. Substances of abuse. Pediatrics in Review. 1997;18(6):204–215, and Joffe A, Blythe MJ, eds. Mental health, psychotropic medications, and substance abuse. Adolesc
Med. 2003;14:455–466.

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 442 PART 4: ADOLESCENT HEALTH

In the past year, how many times have you used

Tobacco? Alcohol? Marijuana?

No Use Once or Twice Monthly Use Weekly Use

(Mild or
(No SUD) (No SUD) (Severe SUD)
Moderate SUD)

Positive In the past year, how many times have you used prescription
Reinforcement drugs? Illegal drugs? Inhalants? Herbs or synthetic drugs?

Medical Home Motivational Intervention:

Brief Advice Assess for problems, advise to
Follow-up
quit, make a plan

Medical Home
Reduce use
Follow-up
Reduce use & Risky
& risky Behaviors &
behaviors Referral to
Treatment

Medical Home Medical Home

Care Care

Figure 63.1. The Screening to Brief Intervention tool approach to clinical screening, brief intervention, and referral
to treatment.
Abbreviation: SUD, substance use disorder.
Reprinted from S Levy, L Shrier. 2014. Boston, MA: Boston Children’s Hospital. Copyright 2014, Boston Children’s Hospital. Reprinted
under Creative Commons Attribution-Noncommercial 4.0 International License.

All vital signs should be reviewed. Tachycardia and hyper- affect should be noted. Chronic marijuana use is sometimes accom-
tension occur primarily with acute intoxication with cocaine or panied by amotivational syndrome.
stimulants (eg, amphetamines). The current weight also should Acute intoxication with some drugs (eg, cocaine) may result in
be recorded and compared with previous values, and any signif- delirium, confusion, paranoia, seizures, hypertension, tachycardia,
icant weight loss should be noted. The skin should be examined arrhythmias, mydriasis, and hyperpyrexia. Acute PCP intoxication
closely for track marks, skin abscesses, or cellulitis, especially if produces abnormal neurologic signs, tachycardia, and hypertension.
the patient admits to using drugs intravenously. The skin should Findings such as central nervous system and respiratory depression,
also be examined for evidence of self-injurious behaviors, such as miosis, and cardiovascular effects (eg, pulmonary edema, ortho-
cutting. Findings consistent with hepatitis (ie, hepatomegaly, jaun- static hypotension) are consistent with opiate overdose. Signs and
dice) may be present in these individuals. The presence of diffuse symptoms of acute intoxication generally are seen in the emergency
adenopathy, thrush, leukoplakia, seborrheic dermatitis, or paroti- department setting rather than in the primary care physician’s office
tis should raise the suspicion of HIV infection. A nonspecific mac- or clinic.
ulopapular rash also may be seen during the acute viremic phase
of an HIV infection. Upper respiratory symptoms, such as chronic Laboratory Tests
nasal congestion, long-lasting “colds” and “allergies,” and epistaxis In the clinic setting, routine drug screening as part of the initial
can occur with chronic inhalation of cocaine or another illicit sub- evaluation of substance use is not recommended and generally
stance. Signs of nasal congestion, septal perforation, and wheezing not indicated to initiate treatment. Specific laboratory studies
also may be noted on examination. Additionally, smoking crack should be performed, however, in those patients with a history
cocaine can cause chronic cough, hemoptysis, and chest pain. of known substance use/abuse and who are enrolled in a drug
Smoking marijuana over long periods can result in similar find- treatment program to monitor for abstinence; in patients who
ings. Gynecomastia can occur with use of anabolic steroids, mar- are required by court order; and in patients who exhibit acute
ijuana, amphetamines, and heroin. The adolescent female using altered mental status, intoxication, or abnormal neurologic find-
anabolic steroids may exhibit signs of virilization, such as a deep ings, such as may be seen in an emergency department setting.
voice, hirsutism, and male pattern baldness. The detailed neuro- In the office setting, these symptoms are frequently absent, and
logic evaluation is arguably the most important aspect of the exam- urine or serum studies to “check” for drug use are not particu-
ination. Any abnormalities in memory, cognitive functioning, or larly useful.

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 Chapter 63: Substance Use/Abuse 443

Box 63.1. Rapid Assessment Box 63.2. What to Ask

for Adolescent Preventive Services
Adolescent Substance Use/Abuse
1. In the past 3 months, have you taken diet pills or laxatives, made your- ww Do any of your friends drink alcohol, smoke marijuana or tobacco, or use
self vomit (throw up) after eating, or starving yourself to lose weight? any other drugs?
2. Do you eat some fruits and vegetables every day? ww What drugs, including alcohol and tobacco, are you currently using? For
3. Are you active after school or on weekends (walking, running, dancing, how long and how frequently?
swimming, biking, playing sports) for at least 1 hour, on at least 3 or ww In what environments do you use these substances?
more days each week? ww Have you ever blacked out or been arrested while under the influence of
4. When you are driving or riding in a car, truck, or van do you always drugs or alcohol?
wear a lap/seat belt? ww Has drug or alcohol use ever interfered with school, work, or other
5. Do you always wear a helmet when you do any of these activities: ride social activities?
a bike, rollerblade, or skateboard; ride a motorcycle, snowmobile or ww Has drug or alcohol use adversely affected relationships with family,
ATV; ski or snowboard? friends, or romantic partners?
6. During the past month, have you been threatened, teased, or hurt by ww Have you ever had sexual encounters while under the influence of
someone (on the internet, by text, or in person) causing you to feel sad, drugs or alcohol?
unsafe, or afraid? ww Do you ever use drugs or alcohol to feel better or to forget why you
7. Has an adult ever physically injured you (by hitting, slapping, kicking) feel sad?
or has anyone ever forced you to have sex or be involved in sexual ww Do your parent(s) or guardian(s) use alcohol, tobacco, or illicit drugs?
activities when you didn’t want to? ww Is there a f amily history of alcoholism, addiction, or mental health
8. Do you carry a weapon (gun, knife, club, other) to protect yourself from issues?
another person?
9. In the past 3 months, have you used any form of nicotine including vaping
(e-cigarettes, Juul, RUBI, Suorin, Blu, hookah, vape pens), smoking
(cigarettes, cigars, black and mild) or chewing tobacco (dip, chew, snus)? If these tests are performed in an ambulatory setting and the
10. In the past 12 months, have you driven a car while texting, drunk or patient is awake and alert, the adolescent’s consent is recommended
high, or ridden in a car with a driver who was? despite adamant requests from a parent or guardian. The results
11. In the past 3 months, have you drunk more than a few sips of alcohol should not be shared with anyone other than the patient, unless the
(beer, wine coolers, liquor, other)? patient gives permission or the test was ordered while investigating
12. In the past 3 months, have you used marijuana (weed, pot, cannabis, THC) in the cause of an acute medical condition. According to the American
any form such as vaping, smoking, edibles, drinks, pills, oil, or any other type? Academy of Pediatrics and the Society for Adolescent Health and
13. In the past 3 months, have you taken a prescription medication Medicine, obtaining a urine drug test without the consent of the ado-
(codeine, OxyContin, Norco, Vicodin, Adderall, Ritalin, Xanax, other) lescent undermines the physician-patient relationship and the devel-
without a prescription, taken more than the prescribed amount or opment of a meaningful, trusting relationship at this critical time
continued to take it after you no longer needed it? in the patient’s life. Drug testing should not be performed simply
14. Have you ever had any type of sex (vaginal, anal, or oral sex)? to allay parental anxiety or confirm suspicions about possible sub-
15. Are you physically attracted to people who are the same gender as you stance use/abuse for a parent, teacher, or athletic coach unless pre-
(girl if you are a girl/guy if you are a guy) or do you feel that you are viously authorized by the adolescent.
gay, lesbian or bisexual? If urine is obtained for drug testing, it is critical that the sample
16. If you have had sex, do you always use a condom and/or another method be collected in a controlled manner and not contaminated, diluted,
of birth control to prevent sexually transmitted infections and pregnancy? or altered in any way. The physician should also be familiar with
17. During the past month, did you often feel sad or down as though you the particular laboratory performing the drug test as well as the
had nothing to look forward to? reported sensitivity and specificity of each test. Urine testing is avail-
18. Do you have any serious problems or worries at home or at school? able to detect marijuana and its metabolites, cocaine, amphetamines,
19. In the past 12 months, have you seriously thought about killing PCP, opiates, barbiturates, and benzodiazepines. Blood levels can be
yourself, tried to kill yourself, or have you purposely cut, burned, or oth-
obtained for alcohol, marijuana, cocaine, amphetamines, barbitu-
erwise hurt yourself?
rates, and benzodiazepines. Although salivary or urinary concentra-
20. Do you have at least one adult in your life that you can talk to about
tions of cotinine and nicotine concentrations can also be performed,
any problems or worries?
these measurements are used mainly in research studies.
21. Do you destroy things, hurt yourself, or hurt other people when you are
Testing for specific inhalants is not routinely done. The delete-
angry?
rious effects of these substances on the hematologic, hepatic, and
Reprinted with permission from Possibilities for Change, ©2006 The Regents of the University of renal systems can be evaluated by performing a complete blood
Michigan, Version 8 (2020). Further reproduction or use is not permitted. For authorized use of count, liver function tests, prothrombin time and partial throm-
RAAPS visit www.possibilitiesforchange.org. boplastin time, and blood urea nitrogen and creatinine; however,

BPPCA6e_Ch063_0437-0446.indd 443 2/13/20 5:55 PM

 444 PART 4: ADOLESCENT HEALTH

results are generally nonspecific. Testing for synthetic cannabinoids, if their occasional drug use has progressed to more regular use in
dextromethorphan, and 3,4-methylenedioxymethamphetamine (ie, risky situations. Furthermore, they should receive educational coun-
ecstasy) is not typically included in drug panels. seling about the unhealthy effects of alcohol use in adolescence (eg,
the deleterious effects of alcohol on developing brain cells). Experts
Management also recommend including strength-based counseling to recognize
the positive qualities of the adolescent.
The clinical approach to the management of substance use in the
adolescent is dependent on the patient’s degree of risk-taking
Specialized Programs
behavior and drug involvement (ie, experimentation, limited use,
problematic use, abuse, or addiction/dependence); the physician’s Preteens or adolescents who are routinely using drugs, alcohol, or
relationship with the adolescent and family; the adolescent’s desire tobacco but are clearly motivated to stop often can be treated solely
to change his or her their behavior; and the family’s involvement and by their primary care physician or collaboratively with child and
awareness of the extent of the problem. A more detailed discussion is adolescent mental health specialists. Teenagers who began using
found in the 2016 policy statement, “Substance Use Screening, Brief illicit substances at an older age, have a fairly good relationship
Intervention, and Referral to Treatment,” by the American Academy with their families, have supportive relationships with friends who
of Pediatrics Committee on Substance Use and Prevention. do not use drugs, and who continue to do well in school and partic-
ipate in other outside activities are more likely to be successful than
Anticipatory Guidance teenagers who start at a younger age. The physician initially should
If preteens (ie, middle school students) or adolescents and their peers identify the problem and establish whether the adolescent desires
are not participating in any high-risk behaviors, including tobacco, to change his, her, or their behavior. After obtaining the patient’s
alcohol, or drug use (ie, the answers to all 3 CRAFFT screening ques- consent, the physician should meet with the family, develop an
tions are “no”), pediatricians should provide patients with both pos- appropriate strategy for treatment intervention, and follow the
itive reinforcement for their abstinent behavior and age-appropriate adolescent periodically in the office. Timely and consistent rein-
anticipatory guidance. This should include advice and information forcement by the primary care physician is necessary, especially
on safety issues; consequences of alcohol and tobacco use, including in the beginning of treatment. Referral to an outpatient program,
use of e-cigarettes; and exposures to peers who may be using illicit such as Alcoholics Anonymous, Alateen, or Narcotics Anonymous,
and prescription drugs. Adolescents should be praised for making also may be indicated. Appropriate community resources for the
smart choices and encouraged to continue their current behavior but teenager and family should be reviewed. Continued coordination
should be invited to return to the office if they have any questions or of services by the pediatrician is challenging but essential to ensure
concerns. This may be particularly helpful for patients whose daily adherence by the adolescent and cooperation by the family to max-
environment exposes them to high-risk situations for alcohol and imize the adolescent’s chance for a full recovery.
drug use. The pediatrician should encourage an open dialogue about
substance use/abuse between adolescents and their parents or guard- Mental Health or Treatment Programs
ians to assist teenagers in developing strategies for drug avoidance. Referral to a mental health or addiction specialist or a specialized
treatment program (eg, drug detoxification center) is indicated for
Early Intervention the adolescent who displays clear signs of dangerous behavior and
Primary care physicians should provide early intervention guidance continues to use drugs despite office treatment by the primary care
to preteens and adolescents who have begun or are engaging in occa- physician and adverse effects on the adolescent’s daily life and rela-
sional high-risk behavior and are considered to be at moderate risk tionships. Immediate intervention is also necessary for the adoles-
for substance use–associated problems. These are patients who have cent who is using intravenous drugs, combining sedative drugs,
started using alcohol or drugs and score 0 or 1 on CRAFFT screening. or consuming large quantities of alcohol. Additionally, the teen-
Such use implies only occasional or casual use of illicit substances by ager with a suspected concomitant psychiatric condition should be
patients or peers. This scenario is the most challenging because many referred immediately for psychiatric evaluation. Other criteria for
adolescents, as well as their parents or guardians, perceive occasional specialty treatment programs include a chronic history of substance
alcohol or drug use as “experimental” or a phase of “normal teenage use/abuse, a serious life-threatening event in conjunction with sub-
behavior” and therefore may trivialize any advice given by the pedi- stance use/abuse (eg, attempted suicide, motor vehicle crash), famil-
atrician. Interventional guidance involves clear advice to stop alco- ial strife, or persistent involvement with a drug-dependent crowd.
hol and other drug use and a discussion of potential risks created Primary care physicians should become familiar with local inpatient
by the adolescent’s current behavior. For example, individuals who programs and residential treatment facilities in their community and
drink alcohol or smoke marijuana at parties are at increased risk for partner with mental health and addiction specialists to provide the
involvement in a motor vehicle crash afterward as the driver or pas- patient and family with optimal services. Although the selection of
senger. Another common scenario involves alcohol intoxication and a program may be influenced by financial resources and insurance
poor judgment about sexual behavior. All teenagers should be made coverage options, it is quite important to try to select the most appro-
aware of the possible consequences of their unsafe behavior, especially priate program for the individual adolescent and family. Guidelines

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 Chapter 63: Substance Use/Abuse 445

exist to aid the physician in the selection process for public and pri- Selected References
vate facilities. These guidelines include total abstinence, appropriate
Adger H Jr, Saha S. Alcohol use disorders in adolescents. Pediatr Rev.
professionals with expertise in drug addiction, familial involvement
2013;34(3):103–114 PMID: 23457197 https://doi.org/10.1542/pir.34-3-103
in the program, family therapy, and appropriate outpatient follow-
American Academy of Pediatrics. Reaching Teens: Strength-Based, Trauma-
up. Regardless of the final course of action of the patient, it is
Sensitive, Resilience-Building Communication Strategies Rooted in Positive Youth
important for the primary care physician to remain involved with Development. Ginsburg KR, McClain ZBR, eds. Itasca, IL: American Academy
the family while making these difficult decisions and support their of Pediatrics; 2020
experience throughout the recovery process. American Academy of Pediatrics Committee on Substance Abuse. Alcohol use
by youth and adolescents: a pediatric concern. Pediatrics. 2010;125(5):1078–
Prevention Programs
1087. Reaffirmed December 2014 PMID: 20385640 https://doi.org/10.1542/
Prevention programs have been developed to assist young people from peds.2010-0438
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alcohol, tobacco, and other illicit substances. Current programs focus Substance use screening, brief intervention, and referral to treatment.
on multiple aspects of the lives of children and adolescents. Programs Pediatrics. 2016;138(1):e20161210 PMID: 27325638 https://doi.org/10.1542/
may involve individual decision-making, self-esteem, and basic edu- peds.2016-1210
cation about alcohol, tobacco, and drugs. These programs frequently Blankson KL, Thompson AM, Ahrendt DM, Patrick V. Energy drinks: what teen-
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Structured curricula also have been created for use in the schools, and D’Amico EJ, Parast L, Meredith LS, Ewing BA, Shadel WG, Stein BD. Screening
community outreach programs have been organized by groups such as in primary care: what is the best way to identify at-risk youth for substance use?
local police departments. The effectiveness of each type of program is a Pediatrics. 2016;138(6):e20161717 PMID: 27940696 https://doi.org/10.1542/
peds.2016-1717
subject of controversy, but each is aimed at preventing the initial or con-
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Med State Art Rev. 2009;20(1):22–40, vii–viii PMID: 19492689
excellent resource and guide for parents and health professionals.
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Prognosis Rev. 2011;32(4):e44–e52 PMID: 21460089 https://doi.org/10.1542/pir.32-4-e44
Gray KM, Upadhyaya HP, Deas D, Brady KT. Advances in diagnosis of adoles-
It is difficult to assess the exact outcome for the adolescent who
cent substance abuse. Adolesc Med Clin. 2006;17(2):411–425 PMID: 16814700
undergoes treatment for substance use/abuse, because definitions
Heyman RB. Screening for substance abuse in the office setting: a developmen-
of success vary. For some teenagers, success implies periods of sobri-
tal approach. Adolesc Med State Art Rev. 2009;20(1):9–21, vii PMID: 19492688
ety; for others, it means complete abstinence; and for still others, it is
Jenssen BP, Walley SC; American Academy of Pediatrics Section on Tobacco
abstinence in addition to recovery from other contributing psycho-
Control. E-cigarettes and similar devices. Pediatrics. 2019;143(2):e20183652
logical problems. Specific outcomes data indicate that abstinence PMID: 30835247 https://doi.org/10.1542/peds.2018-3652
rates are positively correlated with regular attendance in a support
Kann L, McManus T, Harris WA, et al. Youth risk behavior surveillance—United
group and parental participation in these groups. Additionally, gen- States, 2017. MMWR Surveill Summ. 2018;67(8):1–114 PMID: 29902162 https://
eral success rates range from 15% to 45%, depending whether the tool doi.org/10.15585/mmwr.ss6708a1
assesses short- or long-term outcomes. A lifetime potential exists for Knight JR, Sherritt L, Shrier LA, Harris SK, Chang G. Validity of the CRAFFT
relapse among all adolescents with a history of substance use/abuse. substance abuse screening test among adolescent clinic patients. Arch Pediatr
Adolesc Med. 2002;156(6):607–614 PMID: 12038895 https://doi.org/10.1001/
archpedi.156.6.607
Kulig JW; American Academy of Pediatrics Committee on Substance Abuse.
CASE RESOLUTION Tobacco, alcohol, and other drugs: the role of the pediatrician in prevention,
The adolescent is at high risk for continued substance use/abuse because of his identification, and management of substance abuse. Pediatrics. 2005;115(3):816–
association with friends who use drugs as well as his own ongoing tobacco use, 821. Retired July 2017 PMID: 15741395 https://doi.org/10.1542/peds.2004-2841
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ping out of baseball). The physical examination findings also are consistent with
care clinician. Adolesc Med. 2008;19:83–98
his smoking history. The physician should review these risk factors with the teen-
ager in private and acknowledge the difficulty in removing oneself from such Levy S, Schizer M; American Academy of Pediatrics Committee on Substance
an environment. The adolescent’s motivation to change his behavior should Abuse. Adolescent drug testing policies in schools. Pediatrics. 2015;135(4):
be assessed, and referrals to special intervention programs can be discussed. e1107–e1112 PMID: 25825536 https://doi.org/10.1542/peds.2015-0055
Regardless of the outcome, the physician should continue to see the teenager Levy S, Siqueira LM, Ammerman SD, et al; American Academy of Pediatrics
at an agreed-on interval to monitor his ability to quit smoking and change his Committee on Substance Abuse. Testing for drugs of abuse in children and
high-risk behavior. adolescents. Pediatrics. 2014;133(6):e1798–e1807 PMID: 24864184 https://doi.
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Levy SJ, Williams JF; American Academy of Pediatrics Committee on Substance Strasburger VC; American Academy of Pediatrics Council on Communications
Use and Prevention. Substance use screening, brief intervention, and referral and Media. Children, adolescents, substance abuse, and the media. Pediatrics.
to treatment. Pediatrics. 2016;138(1):e20161211 PMID: 27325634 https://doi. 2010;126(4):791–799. Retired July 2017 PMID: 20876181 https://doi.
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Nackers KAM, Kokotailo P, Levy SJL. Substance abuse, general principles. Pediatr Wang GS, Hoyte C. Common substances of abuse. Pediatr Rev. 2018;39(8):
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Rogers PD, Copley L. The nonmedical use of prescription drugs by adolescents.
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 CHAPTER 64

Eating Disorders
Monica Sifuentes, MD

CASE STUDY
A 16-year-old girl is brought to the office by her mother The physical examination is significant for a thin
because the mother feels that her daughter is too thin and physique, and vital signs are normal. On the growth
always appears tired. The mother reports that her daughter chart, her weight is at the 15th percentile and her height
does not eat much at dinner and generally says she is not is at the 75th percentile; her body mass index is 17 (10th
hungry. Recently, the girl bought diet pills that were adver- percentile). Her weight at a previous visit was at the 40th
tised online. The teenager claims that she has not taken percentile. The remainder of the physical examination is
the pills, so she does not understand why her mother is so unremarkable.
upset. She says she feels fine and considers herself healthy
because she has recently become a vegetarian.
Questions
1. What are the common characteristics of disordered
The girl is a 10th-grade student at a local pub-
eating in adolescents?
lic school and attends classes regularly, although her
2. What are the important historical points to include
friends are occasionally truant. She is involved in the drill
when interviewing the patient with suspected
team, swim team, and student council. She has many
eating disorder? Which teenagers are considered
friends who have “nicer” figures than she does. Neither
at risk?
she nor her friends smoke tobacco or use drugs, but they
3. How is the diagnosis of anorexia nervosa and
occasionally drink alcohol at parties. The girl is not sexu-
bulimia nervosa made?
ally active and denies a history of abuse. Her menstrual
4. What is the treatment plan for the adolescent with
periods are irregular, with the last occurring approxi-
eating disorder?
mately 3 months prior to this office visit.
5. What are the medical complications of anorexia
She currently lives with her mother, father, and 2
nervosa and bulimia nervosa?
younger siblings. Although things are “OK” at home,
6. What is the prognosis for these conditions? How
she thinks her parents are too strict and do not trust her.
can the primary care physician help improve the
They have just begun to allow her to date, but she dis-
outcome?
likes that she has a curfew.

Basic characteristics of eating disorders are summarized in Box 64.1. these conditions. The overall goal is to decrease the lifelong medi-
For more stringent criteria, refer to Diagnostic and Statistical Manual cal and psychological morbidity and mortality associated with AN
of Mental Disorders, Fifth Edition (DSM-5) criteria. An adolescent and BN to enhance the long-term health and emotional well-being
may have an atypical presentation, a history of anorexia nervosa of affected individuals.
(AN) and bulimia nervosa (BN), or an underlying affective com-
ponent that confuses the issue. The adolescent may not display a Epidemiology
blatant refusal to eat but may instead exhibit subtle characteris- Historically, eating disorders predominately affected white adoles-
tics of disordered eating, such as constant dieting, obsession with cent females in more affluent communities. Although disordered
a certain physical exercise, or irregular menstruation. Additionally, eating currently occurs in many other settings, historically, AN
preoccupation with physical appearance and weight currently and BN were rare among persons of lower socioeconomic status,
is not uncommon or necessarily pathologic in Western society. among ethnic/racial minorities, and in children younger than
The fashion industry and social media promote the idea that thin- 12 years of age. Currently, these conditions are diagnosed in individ-
ness and beauty are interrelated. Thus, the typical adolescent who uals of all ethnic, cultural, and socioeconomic backgrounds in the
longs to be accepted by peers and who is learning to develop a United States as well as in other developed countries. Additionally,
sense of independence and control is a prime target for the devel- males make up an estimated 5% to 10% of all patients with eating
opment of disordered eating. The primary care physician is in a disorders and tend to be younger, malnourished, or medically unsta-
unique position to recognize individuals at risk, appropriately screen ble when they present for treatment, which is suggestive of delayed
teenagers with specific behaviors, and provide early diagnosis of evaluation and diagnosis.
and intervention for patients with disordered eating to prevent the Although dieting behavior among adolescents and young adults
development of potentially lethal complications associated with is not uncommon, true AN has a prevalence of approximately 0.5%
447

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 448 PART 4: ADOLESCENT HEALTH

disorders. Generally, eating disorders occur with increased fre-

Box 64.1. Criteria for Anorexia Nervosa
quency in patients with a family history of eating and mood disor-
and Bulimia Nervosa
ders as well as obesity.
Anorexia Nervosa Variants of eating disorders, which do not meet full diagnos-
ww Caloric intake below caloric requirements (low weight for age, sex, tic criteria based on previous guidelines but cause significant dis-
developmental stage, physical well-being) tress and impairment in social, occupational, or other important
ww Fear of weight gain or becoming overweight/obese areas of functioning, are not uncommon in preteens and adolescents.
ww Distorted body image Exact numbers are uncertain because many of these patients remain
ww Failure to recognize dangers of low weight “under the radar” and the consequences of their eating behaviors
ww Types: are not fully recognized. Additionally, more than one-half of junior
—— Restricting type: Characterized by dieting, fasting, and/or high and high school girls have dieted at some time, many of them
excessive exercise. repeatedly. For this reason, the revised DSM-5 criteria for both AN
—— Binge-eating/purging type: Characterized by binge eating and BN are less restrictive. New diagnostic categories now include
associated with self-induced vomiting or the misuse of diuretics, the spectrum of patient behaviors: avoidant/restrictive food intake
laxatives, suppositories, or enemas. disorder, atypical AN, and binge-eating disorder.
Bulimia Nervosa
ww Binge eating Clinical Presentation
ww Eating larger than normal amounts of food The patient with eating disorder may present with general symp-
ww A sense of being out of control and using other behaviors to restrict toms related to weight loss and nutritional or volume deficiencies,
weight gain (eg, excessive exercise, fasting, vomiting, laxatives, such as fatigue or syncope. Such a patient also may exhibit mini-
diuretics, or other medications) mal weight gain according to standard growth charts or a delay in
the onset of puberty or progression of pubertal development. More
Derived from Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.
importantly, female adolescents also may present with primary or
to 1% in these individuals. Estimates have ranged from 1% to 10% secondary amenorrhea noted incidentally during an annual health
in high-risk groups, such as upper- and middle-class white females. maintenance or sports physical examination. Sometimes an under-
Less than 5% of these cases are males, with a female-to-male ratio lying psychiatric condition, such as obsessive-compulsive disorder
of 9:1, although this prevalence has been disputed to be an under- or anxiety, is the impetus for the medical referral. Occasionally, a
estimate of young males with AN. The age of onset historically was patient with undiagnosed eating disorder is hospitalized because
reported to occur during middle adolescence (age 14–16 years). It of complications related to the condition, such as electrolyte dis-
has become increasingly more common, however, for school-age turbances associated with diuretic use, hematemesis from induced
children and younger teenagers to be dissatisfied with their weight vomiting, or syncope from hypovolemia. Finally, the preteen or ado-
and concerned with body image. Studies of middle-income elemen- lescent may be referred to the primary care physician by a con-
tary school girls have reported significant body and weight dissatis- cerned parent, well-intentioned friend, informed coach, or astute
faction. Skipping meals and desserts, fasting, and vomiting all have school personnel who notice significant weight loss or restrictive
been reported specifically to lose weight. For many of these young eating patterns.
girls, the goal is not to maintain a normal weight but to be under-
weight by standard growth charts.
Pathophysiology
The prevalence of BN is approximately 1% to 4%, although some Anorexia Nervosa
studies report that as many as 8% of adolescent and college women Although several risk factors predispose an individual to the develop-
and 0.5% of men have bulimia. The age of onset of BN tends to be ment of AN, this condition, like other eating disorders, has no single
later than for AN, with symptoms beginning during late adoles- cause (Box 64.2). Longitudinal studies clearly point to a significant
cence and young adulthood (age 17–20 years). Several behavioral role for inappropriate dieting behavior in the pathogenesis of an eat-
and affective disorders have been associated with the development of ing disorder. Because not all dieters develop an eating disorder, how-
AN and BN. These include comorbid psychiatric conditions, such as ever, other contributing risk factors must be involved. Current theory
depressive, bipolar, and anxiety disorders; alcoholism and substance suggests that the etiology of AN is multifactorial and precipitated
use; other addictive behaviors (eg, laxative abuse); poor impulse by a complex interaction between genetic, environmental/cultural,
control; and obsessive-compulsive personality. Girls who are obese and psychological risk factors, along with the adolescent’s personal
or experience early puberty also are at increased risk for develop- experiences and individual personality traits.
ing an eating disorder, with symptoms starting in the context of
dieting. A history of sexual abuse may be present among patients Biologic Factors
with eating disorders. Additionally, suicidal behavior (ie, attempts) It has been postulated that the normal increase in adipose tissue with
is more likely in individuals with BN, as are reports of an increased the onset of puberty creates special challenges for some girls. An eat-
incidence of family members with substance use and dependence ing disorder may develop as an attempt to control or combat this

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 Chapter 64: Eating Disorders 449

previous problems because of their compliant, self-sacrificing, duti-

Box 64.2. Risk Factors for the Development
ful, nonassertive nature.
of an Eating Disorder
An increased association exists between AN and major depres-
ww Family history of an eating disorder or obesity sion, and many studies of women have shown that first-degree rel-
ww Affective illness or alcoholism in a first-degree relative atives of patients with AN have higher rates of depression than the
ww Specific activities/sports: ballet, gymnastics, modeling, dance, general population. Many depressive symptoms in patients with AN
figure skating, long-distance running, wrestling, lightweight rowing, improve with restoration of body weight, however. Therefore, some
pole vaulting of these clinical features of depression also may be secondary to the
ww Personality characteristics (eg, perfectionism) adolescent’s state of severe malnutrition or starvation.
ww Parental eating behavior and weight
ww Low self-esteem Familial Influences
ww History of physical or sexual abuse Researchers have noted that certain family dynamics may serve to
ww Body image dissatisfaction initiate and perpetuate AN, although it is no longer believed that
ww History of excessive dieting, frequently skipped meals, compulsive exercise family dysfunction is the main cause of disordered eating. Typically,
however, the family is overprotective and rigid, with the mother
Adapted from Rome ES, Ammerman S, Rosen DS, et al. Children and adolescents with eating often enmeshed in her daughter’s life. Conflict resolution tends to
disorders: the state of the art. Pediatrics. 2003;111(1):e98–e108. be poor, and an inability to express feelings within the family is
often evident. Diagnosis of an eating disorder often causes additional
normal pubertal weight gain with the initiation of dieting behavior. stress in the family, thus contributing to more difficulties in their
Preexisting hypothalamic dysfunction also has been implicated as a relationships. Multiple case-control studies have shown an increased
contributing factor to AN. Additionally, changes in neurotransmitter rate of AN and BN in relatives of patients with eating disorders. This
levels have been shown to occur with initial vomiting or dieting. These may be the result of inheritance patterns of personality traits as well
changes may result in specific psychiatric symptoms that may perpet- as comorbid mood and anxiety disorders. Although there is lim-
uate disordered eating. Recent data obtained with neuroimaging stud- ited research, studies suggest that there is no difference in familial
ies have shown that most of the physiological disturbances resolve with or genetic factors between males and females with eating disorders.
normalization of the patient’s body weight, however. Additionally, a
premorbid disturbance in the neurotransmitter serotonin has been Social/Environmental Pressures
speculated to be a risk factor for the development of AN and BN. The media as well as postindustrial, high-income societal standards
Serotonin controls appetite by creating a sensation of fullness or sati- are believed to play a role in setting the stage for the development of
ety. It is also known to affect sexual and social behavior, mood, and eating disorder. Individuals in affluent communities are especially at
stress responses. Although further studies are needed to confirm the risk if thinness, food, eating, and obsessive exercise become the prime
exact role of this hormone in the development of an eating disorder, focus of daily activity. In addition, young women become caught in
it has been shown that decreases in this brain neurotransmitter have what has been labeled a “slender trap,” in which thinness is equivalent
been associated with impulsivity, aggression, and depression. to attractiveness and success. Food restriction or purging is a means
Leptin, a circulating hormone produced in adipose tissue, also of attaining thinness. An inability to maintain thinness equals failure.
seems to have a significant role in mediating the neuroendocrine Role models in the media, such as fashion models and actors, also
effects of AN. Decreased concentrations of leptin are seen with serve as ideals by which young people create their physical standards.
reduced body fat stores as a result of decreased caloric intake and
energy deficits. Paradoxically, leptin levels also appear to contrib- Other Influences
ute to physical hyperactivity (eg, compulsive exercise, restlessness), Involvement in particular extracurricular activities that promote
which is often seen in patients with AN despite their inadequate leanness and endurance, such as ballet, gymnastics, figure skat-
metabolic intake. ing, cheerleading, and running, may contribute to the develop-
ment of AN in females. For male athletes, such influences include
Genetic Factors participation in sports such as wrestling, lightweight rowing, pole
A genetic predisposition to anorexia has been shown in studies of vaulting, and long-distance running, in which maintaining a low
monozygotic twins. The incidence of the disorder is increased in weight is important and dieting and/or fasting is used to achieve
sisters and other female relatives of patients with AN. that weight limit. Chronic medical conditions, such as diabetes mel-
litus or inflammatory bowel disease (IBD), also may contribute to
Personal Characteristics the development of an eating disorder.
Generally, patients with AN are described as obsessive-compulsive
personality types, perfectionists, and overachievers, particularly in Bulimia Nervosa
academics and sports. They also display low self-esteem and high Several theories have been proposed to explain the etiology of BN
anxiety levels despite their perceived successes by others. In the case but, similar to AN, no single etiology has been confirmed. Most
of girls, they are the “model daughters” who have never caused any likely, multiple factors contribute to the development of this eating

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 450 PART 4: ADOLESCENT HEALTH

disorder, and it is the complex interaction between these factors at (Box 64.3). The 2008 article titled “Interviewing the Adolescent With
a particular developmental point in an older adolescent’s life that an Eating Disorder” includes a detailed discussion of interview-
results in this condition. ing techniques to use on patients with a suspected eating disorder.
Biologic, psychological, familial, and societal influences are thought The severity of the medical and nutritional aspects of the condi-
to contribute to the development of BN in older teenagers and young tion should be determined, after which a thorough psychosocial
adults. Among other issues, adolescent and parental obesity are risk
factors for BN, as are early menarche, early sexual experiences, post-
traumatic stress disorder, and a history of childhood sexual or physical Box 64.3. What to Ask
abuse that occurs in conjunction with a comorbid psychiatric condi-
tion. More important, dieting has been documented as an important Eating Disorders Generally
risk factor in this age group. ww Have there been any changes in the adolescent’s weight? What is the
Familial dysfunction and high levels of conflict also have been asso- most and least the adolescent has ever weighed? When did these
ciated with BN. Unlike with AN, conflict might be discussed openly weights occur and for how long?
but negatively within the family, and the existence of inadequate ww How does the adolescent feel about how they look? Is there anything
expression of emotions may result in a lack of parental warmth and they would like to change? How long have they been feeling this way?
concern. As a result, the relationship between the parent and teen- ww How much does the adolescent want to weigh or think they should weigh?
ager is distant rather than enmeshed. The adolescent generally has a ww How often does the adolescent weigh themselves?
low level of self-esteem, high impulsivity, perfectionist temperament, ww How much of the day is spent thinking about food?
and body image dissatisfaction. Additionally, parents and relatives ww What is a typical day of eating like, including eating times, types of
have a high rate of affective and eating disorders as well as alcoholism. foods, beverages, amount consumed, and portion size? Do they have a
mealtime ritual?
Differential Diagnosis ww What did they eat yesterday (24-hour dietary recall)?
ww Does the adolescent have any food restrictions? Is the teenager a
It is important to differentiate AN from BN, although occasion- vegetarian? Do they count calories, fats, and carbohydrates? Binge eat?
ally this distinction may be difficult to make if a patient displays ww Does the adolescent hide or throw away food?
behaviors consistent with both conditions. Additionally, approxi- ww Do they feel guilty about eating?
mately 50% to 60% of patients with eating disorders have associated ww How do the adolescent and the adolescent’s friends manage weight
comorbid psychiatric disorders. Major affective disorders to consider control?
include depression, bipolar disorder, and obsessive-compulsive dis- ww What does the adolescent do when he, she, or they feels “fat”? Does the
order. Anxiety disorders and substance use also are commonly seen, adolescent vomit to lose weight? How often does this occur? Are there
although the latter is more strongly associated with BN. particular triggers?
Weight loss, loss of appetite, and refusal to eat can be associ- ww Has the adolescent or any of the adolescent’s friends ever used diuret-
ated with many medical conditions. Therefore, other diagnoses to ics, diet pills, coffee, enemas, or laxatives to lose weight or compensate
consider when evaluating patients for AN include IBD, malabsorp- for overeating?
tion, celiac disease, diabetes mellitus, occult malignancies, AIDS, ww Does the adolescent exercise? If so, what type and how often? Does the
Addison disease, hyperthyroidism or hypothyroidism, hypopituita- adolescent feel stressed if a workout is missed or delayed?
rism, tumors of the central nervous system, and chronic substance ww In what sports or dance activities, if any, does the adolescent participate?
use, particularly with amphetamines and cocaine. Superior mesen- ww For females, are menstrual periods regular? Last menstrual period? Age
teric artery syndrome is another important condition to consider in at menarche?
the differential diagnosis; however, it also can be a consequence of ww Does the adolescent have any other symptoms associated with compli-
an eating disorder, specifically AN. cations of eating disorders?
ww Does the adolescent have any depressive symptoms, such as sleeping
Evaluation problems or fatigue that can accompany eating disorders?
History For Patients with Bulimia Nervosa Specifically
A complete medical history, including a detailed review of systems, ww When do binges occur? With what foods?
should be obtained from all adolescents and young adults with sus- ww How much does the adolescent binge, and how often?
pected eating disorder to rule out the multiple other conditions in ww What are the precipitating factors?
the differential diagnosis of decreased appetite and weight loss. The ww What happens specifically during a typical episode?
primary care physician then should interview the patient alone and ww Does the adolescent vomit? How often?
focus on establishing the diagnosis of disordered eating by address- ww Does the adolescent use drugs or alcohol?
ing more specific issues related to changes in food preferences (eg, ww Is there a history of depression or attempted suicide? Self-injurious
vegetarian, vegan, low-fat diet), eating behaviors, dieting, calorie behavior? Sexual or physical abuse?
counting, weight history, exercise routine, and body image concerns

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 Chapter 64: Eating Disorders 451

evaluation should be conducted. Inquiries should focus on symp- should be plotted on a growth chart and the body mass index cal-
toms associated with complications of eating disorders, such as culated (weight [kg]/height [m2]). Delayed growth or short stat-
dysphagia secondary to esophagitis from recurrent vomiting, con- ure should be noted, because it can occur with severe malnutrition
stipation from fluid restriction, and muscle weakness associated with as well as other systemic conditions. Vital signs, including blood
emetine toxicity from chronic ipecac use. Because ipecac is no lon- pressure, should be recorded and compared with previous measure-
ger readily available, this adverse effect is seen less frequently than ments. Evidence of cardiovascular instability can be manifested by
in the past. Although rarely seen by the primary care physician at the tachycardia, bradycardia, or orthostatic hypotension. The patient
initial visit when the diagnosis of an eating disorder is made, recog- also may be hypothermic as a result of overall malnutrition. The gen-
nition of serious medical complications is paramount to determin- eral appearance and affect of the patient must be noted. The ado-
ing the type and urgency of further care. lescent with typical AN is often emaciated, with an obvious loss of
Interviewing an adolescent with an eating disorder can be subcutaneous tissue, and may appear apathetic or anxious or have
quite challenging; however, a thorough psychosocial assess- a flat affect. The patient with BN may have mild obesity or nor-
ment should be performed after a discussion about confiden- mal weight with a full-appearing facies secondary to parotid and
tiality has occurred with the adolescent and the parent. The submaxillary swelling, which is a complication of frequent purg-
HEADSS assessment (home, employment and education, activ- ing. In most cases, however, the teenager appears “normal” at the
ities, drugs, sexuality, suicide/depression) is useful to direct initial visit.
the psychosocial interview from general topics to more sensi- Characteristic physical findings in patients with AN are consis-
tive ones (see Chapter 4). Particular attention should be paid to tent with a “state of hibernation,” because the body adapts to starva-
the adolescent’s overall functioning at home, with friends, and tion by slowing metabolism and decreasing energy requirements to a
at school; the presence of other comorbid psychiatric disorders minimum. Hypothermia, orthostatic hypotension, bradycardia, and
(eg, depression, anxiety); and a history of suicidal ideation or lanugo (ie, downy hair) on the arms and back are seen in patients
sexual and/or physical abuse. Out-of-control behavior as a result with restrictive AN. The palms and soles may be yellow second-
of substance use also should be assessed. The use of psychologi- ary to hypercarotenemia, and pigmentation of the chest and abdo-
cal testing or questionnaires to assess cognition, anxiety, and men may be increased as a result of malnutrition. Thinning or loss
depression may be beneficial, depending on the comfort level of of pubic and scalp hair as well as dry or pale skin also may be seen.
the primary care physician with these tools. Several validated The breasts should be examined carefully for sexual maturity rating
screening tools specific for eating disorders also exist, including (ie, Tanner stage) as well as galactorrhea. The presence of galactor-
the Eating Disorders Examination Questionnaire (EDE-Q), Eating rhea, along with a history of amenorrhea, warrants further investi-
Disorder Inventory-3 (EDI-3), Eating Attitudes Test (EAT), and gation for a prolactinoma. The patient with AN may have interrupted
the Female Athlete Screening Tool (FAST). If the primary care phy- or delayed pubertal development. A cardiac murmur must be noted
sician is unfamiliar or uncomfortable with these tools, consultation and evaluated further, because one-third of patients with anorexia
with a mental health professional is warranted. have mitral valve prolapse. The abdomen should be palpated for
A detailed menstrual history also must be obtained from females tenderness or masses and may be scaphoid in appearance. Bowel
because secondary amenorrhea is frequently an early sign of AN sec- sounds are often decreased in the patient with anorexia, and stool
ondary to decreased body fat. Primary amenorrhea in the context may be palpated secondary to constipation. The presence of pubic
of pubertal delay also can occur with AN. With BN, menses may be hair (ie, genital sexual maturity rating) also should be noted. A rec-
irregular or absent. A family history of eating disorders, substance tal examination should be performed for the patient with a history
use, or psychiatric disorders should be reviewed with the teenager of bloody stool, which is a finding consistent with IBD, or evidence
and confirmed by the parent or guardian. of rectal prolapse. The extremities should be evaluated for coldness,
A dietary history should be obtained from the adolescent as mottling, or edema. They also should be palpated for tenderness
well as the parent or guardian independently and should focus on because fractures may be present resulting from loss of bone min-
any dietary restrictions or aversions. This may be difficult initially eralization. The skin, particularly the forearms, should be examined
because the patient often does not believe he, she, or they has a prob- for any evidence of self-injurious behavior (eg, cutting). Finally, a
lem with food. A 24-hour dietary recall can be an important place complete neurologic examination, including a mental status evalua-
to begin the assessment. The presence of dieting or calorie count- tion and fundoscopic examination, should be performed to exclude
ing, binge-eating and purging behaviors, amount of food consumed, a central nervous system lesion or endocrine disorder.
and the frequency and duration of these behaviors should be docu- In the patient with BN, specific physical findings, if any, often are
mented by the physician or a registered dietician. associated with dehydration and electrolyte imbalances that occur as
a result of chronic vomiting or laxative abuse. Vital signs should be
Physical Examination reviewed for tachycardia, sinus bradycardia, and orthostatic hypo-
In both AN and BN, the patient should undress, wearing only under- tension; the presence of any of these signs is indicative of hemody-
garments, for the physical examination. This prevents hiding the namic instability. The patient also may have hypothermia. The skin
true body habitus with bulky clothes. The current height and weight should be inspected on the dorsum of the hand over the knuckles

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 452 PART 4: ADOLESCENT HEALTH

for scratches, scars, or calluses from self-induced vomiting (Russell’s of treatment. The erythrocyte sedimentation rate may be low with
sign). Periorbital petechiae and subconjunctival hemorrhages also anorexia and high with IBD or other inflammatory process. Normal
may occur as a result of recurrent or severe retching. The oropharynx liver function tests aid in excluding other causes of weight loss.
should be inspected for dental caries, enamel erosion, or discoloration Normal serum amylase and lipase levels rule out other etiologies
as well as for parotid hypertrophy. Additionally, palatal scratches or for recurrent vomiting, such as pancreatitis. Serum tissue transglu-
mouth sores are evident. The abdomen should be palpated for epi- taminase and immunoglobulin A levels can be obtained if concern
gastric tenderness, fullness, or midabdominal pain. Positive findings exists for celiac disease. Other urine and serum tests (eg, thyroid
may be the result of esophagitis, gastritis, or pancreatitis. function tests) help differentiate an endocrine disorder from AN,
Any muscle weakness or cramping should be appreciated and especially in the patient with primary or secondary amenorrhea
may be indicative of an electrolyte abnormality. Edema of the (Box 64.5). Electrocardiography is used in diagnosis of QTc prolon-
extremities may be noted in the patient who abuses laxatives. gation, heart block, and arrhythmias. Additionally, electrocardiog-
raphy is indicated for the patient with electrolyte abnormalities or
a history of significant purging or weight loss.
Laboratory Tests
Laboratory abnormalities in BN reflect the type and extent of
Box 64.4 lists the laboratory studies necessary in the evaluation of purging behavior of the adolescent with this diagnosis. Box 64.4
the patient with AN and evidence of malnutrition or purging behav- summarizes the necessary laboratory studies for the patient with BN.
iors. A complete blood cell count may be helpful because leuko-
penia, anemia, and, rarely, thrombocytopenia can occur with this
disorder. Electrolytes (ie, sodium, potassium, chloride, carbon diox-
ide) and blood urea nitrogen are important, especially if the patient Box 64.5. Medical Complications of
uses diuretics, laxatives, or ipecac. Serum magnesium, calcium, and Eating Disorders
phosphorous must be monitored, especially in the refeeding phase
Associated with Purging Behavior
ww Fluid and electrolyte imbalances (from laxative abuse and vomiting)
Box 64.4. Initial Laboratory Assessment for the ww Irreversible cardiac muscle damage (from ipecac toxicity)
Patient With an Eating Disorder ww Esophagitis, dental erosion, Mallory-Weiss tearing (from chronic
vomiting)
Anorexia Nervosa
ww Renal stones (from dehydration)
ww Complete blood cell count, erythrocyte sedimentation rate
ww Amenorrhea, hypoestrogenemia, osteopenia (from decreased body mass
ww Serum electrolytes (sodium, potassium, chloride, carbon dioxide)
index)
ww Blood urea nitrogen/creatinine
ww Serum glucose Associated with Caloric-restrictive Behavior and Weight Loss
ww Serum calcium, phosphorous, magnesium, zinc ww Abnormalities on electrocardiography: QTc prolongation, low voltage,
ww Serum protein, albumin, cholesterol sinus bradycardia, sinus tachycardia, segment depression (from electro-
ww Liver function tests lyte abnormalities)
ww Endocrine laboratory tests (perform in patients with amenorrhea) ww Cardiac arrhythmias, including supraventricular beats and ventricular
—— Urine pregnancy tachycardia, with or without exercise
—— Follicle-stimulating hormone ww Mitral valve prolapse
—— Luteinizing hormone ww Pericardial effusion
—— Estradiol ww Delayed gastric emptying, slow gastrointestinal motility, constipation,
—— Thyroid function: thyroid-stimulating hormone, thyroxine 4, bloating, fullness, abnormal liver function tests (from fatty infiltration
thyroxine 3 of the liver)
—— Prolactin ww Increased blood urea nitrogen, increased risk of renal stones, total body
ww Urine pH and urinalysis depletion of sodium and potassium
ww Electrocardiography ww Refeeding syndrome (from extracellular shifts of phosphorous)
ww Leukopenia, anemia, thrombocytopenia
Bulimia Nervosa
ww Amenorrhea, hypoestrogenism, osteopenia (from decreased body mass
ww Serum electrolytes (sodium, potassium, chloride, carbon dioxide) index)
ww Serum glucose ww Growth retardation, pubertal delay
ww Serum calcium, phosphorous, magnesium, zinc ww Cognitive deficits
ww Blood urea nitrogen/creatinine ww Cortical atrophy, seizures
ww Serum amylase
ww Urine pH and urinalysis Modified from Rosen DS; American Academy of Pediatrics Committee on Adolescence. Identification and
ww Electrocardiography management of eating disorders in children and adolescents. Pediatrics. 2010;126(6):1240–1253, and
Goldstein MA, Dechant EJ, Beresin EV. Eating disorders. Pediatr Rev. 2011;32(12):508–521.

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 Chapter 64: Eating Disorders 453

Radiologic studies, such as computed tomography of the head and multidisciplinary team about the minimum acceptable weight
or magnetic resonance imaging of the brain, are indicated if the for the patient’s age, height, pubertal stage, premorbid weight, and
diagnosis of an eating disorder is uncertain or the neurologic exam- previous growth trajectory. Attaining such consensus should be done
ination is abnormal. Bone density studies, such as dual-energy x-ray in conjunction with a registered dietitian. Unlike with adults, weight
absorptiometry scanning, are not performed routinely except in ado- restoration in the adolescent also must take into account the require-
lescents with sustained amenorrhea for longer than 6 months. If ments for normal pubertal growth. An agreement that includes the
amenorrhea persists, annual dual-energy x-ray absorptiometry scan- goals of treatment, parameters for weight gain, and maintenance of
ning is recommended. An upper and lower gastrointestinal series health should be established between the adolescent and all mem-
may be warranted in the patient with esophageal symptoms or in bers of the treatment team. Refeeding/nutritional rehabilitation
whom IBD is a strong consideration. The performance of other pro- must be the initial priority, especially in the adolescent with AN,
cedures, however, should be based on the individual case and asso- as long as the patient is otherwise medically stable and does not
ciated symptoms. require inpatient psychiatric hospitalization. Details about the pro-
posed nutritional regimen, which includes a stepwise increase in
daily caloric intake, calculated amount of protein to be ingested,
Management daily fat intake, vitamin and mineral requirements, and treatment
It is the role of the primary care physician to recognize when inap- goal weights, should be developed by the dietitian and reviewed with
propriate dieting and weight loss become an obsession for an ado- the adolescent and the family in conjunction with mental health sup-
lescent and when abnormal and unhealthy behaviors develop for port and close medical follow-up. (See Rome and Strandjord, and
maintenance of obvious malnutrition. When treating the patient Garber and Kohn, in Selected References for a discussion of tradi-
with eating disorder, the physician must first establish trust; in tional and newer approaches to refeeding and refeeding syndrome.)
doing so, the patient should be reassured that the physician is Early nutritional rehabilitation and timely medical stabilization are
not attempting to remove all control by trying to make the ado- essential to correct the cognitive deficits associated with disordered
lescent “fat.” The goal is to create a therapeutic alliance between eating, especially severe restrictive AN.
the physician, adolescent, and family to restore and maintain Historically, adolescent-focused individual therapy with some
the patient’s health and emotional well-being as well as pro- family support was the cornerstone of treatment for the patient with
mote recovery and prevent acute and long-term complications. an eating disorder, particularly AN. Studies in the past 10 years,
Depending on the severity of symptoms and the comfort level of however, have shown that family-based therapy (FBT), also known
the physician to monitor early medical, nutritional, and psycho- as the Maudsley approach, is both effective and superior to individ-
logical issues, the adolescent may continue to be followed by the ual therapy for AN. Consisting of 10 to 20 family meetings over a
primary care physician in conjunction with a registered dieti- 6- to 12-month treatment course, FBT is an outpatient form of family
tian, family therapist, and mental health professional, such as a therapy that empowers parents to take charge of their adolescent’s
psychologist and/or psychiatrist. Early in the treatment course, weight restoration. Patients with disordered eating often try to hide
the primary care physician must be willing to follow the patient or minimize their illness and generally have difficulty acknowledg-
frequently—as often as once or twice a week, if necessary— ing their abnormal eating behaviors, associated patterns, and degree
depending on the medical and psychological stability of the of weight loss. Parents, too, may be in denial or may be unaware
patient. If the physician wishes to refer the patient to an experi- of the extent of their teenager’s condition. Family-based therapy
enced multidisciplinary team of specialists, it is important that emphasizes the role of the parents in taking the lead in managing
the primary care physician remain informed and involved in the their adolescent’s eating, particularly in the early stages of treatment.
care of the teenager. Therapists assist the family to problem-solve factors that may be
Numerous studies have shown that eating disorders are best perpetuating the eating disorder behaviors and thus interfere with
managed by an interdisciplinary professional team experienced improvement of the adolescent’s nutrition and weight restoration.
in providing developmentally appropriate care for children and Family-based therapy does not blame the parents for causing the
adolescents with eating disorders. This team generally consists eating disorder; rather, solutions are sought for moving forward.
of the primary care physician, an adolescent medicine special- Through a distinct 3-phase structured process, FBT initially focuses
ist, a psychologist and/or psychiatrist, a registered dietician, and on refeeding and weight restoration (phase 1); followed by gradu-
a social worker or case manager. Ideally, the team is available ally allowing the adolescent to have more responsibility for eating
to offer both inpatient and outpatient services, although most and weight gain (phase 2); and finally addressing the psychologi-
mild to moderately affected teenagers can be treated in an out- cal aspects of the eating disorder in the context of adolescent devel-
patient setting. Criteria for inpatient admission should be estab- opment and treatment termination (phase 3). It has been reported
lished by the team and reviewed with the patient and family at that weight gain in the first month of FBT is predictive of success
the onset of therapy. with this approach. The primary care physician must work cooper-
Particularly for the patient with restrictive eating disorder, con- atively with mental health colleagues to provide the necessary struc-
sensus must be reached between the adolescent, parent or guardian, tured psychological services for the patient and family. Although the

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 454 PART 4: ADOLESCENT HEALTH

process is difficult, the patient must begin to acknowledge his or her for example, may replace food restriction. Most studies report the
behaviors and accept the need for assistance before effective mental prognosis as more favorable if the patient’s condition is identified
health interventions can occur. early and treated rapidly and aggressively. Predictors of poor out-
For those who are not able to participate in FBT, individual come for AN include very low body weight at the time of initial
therapy, such as cognitive behavioral therapy, should be provided treatment, long duration of illness, a psychiatric comorbidity, a dys-
for both the patient and family. Support groups also may be ben- functional parent-child relationship, and purging behaviors. For
eficial and frequently are an important component of a day treat- BN, factors found to be predictive of poor outcome include longer
ment program (eg, day hospitalization, partial hospitalization) for duration of illness at presentation, severity of eating pathology and
the adolescent with eating disorder who requires more intensive frequency of vomiting, premorbid obesity, associated comorbid dis-
outpatient care but not an inpatient hospitalization or residential orders (eg, personality disorder, substance use), and suicidal behav-
program. Day treatment programs are generally less costly and ior. A family history of alcoholism also has been reported as a poor
may be more accessible than traditional hospital-based programs. prognostic factor for BN.
According to the American Academy of Child and Adolescent According to current literature, eating disorders have the high-
Psychiatry, psychiatric hospitalization, day programs, partial hos- est mortality of any mental illness. The mortality rate for ado-
pitalization programs, and residential programs should be consid- lescents with AN is approximately 2%. Higher numbers were
ered only when outpatient interventions have been unsuccessful previously reported when adult and adolescent data were com-
or are unavailable. (See Golden et al in Selected References for a bined. Exact figures for BN have not been determined, although
discussion of the role of the medical provider at each level of care.) the mortality rate has been quoted as being similar to that of AN.
Pharmacotherapy with agents such as selective serotonin reup- The most common cause of death in both disorders is suicide.
take inhibitors are generally not prescribed for the adolescent with Medical causes are often the result of cardiac arrhythmias from
AN except to manage comorbid conditions, such as depression and electrolyte abnormalities.
anxiety disorders. Several studies have shown, however, that these
same medications can be effective in patients with BN by decreasing
binge-eating and purging behaviors. Randomized controlled trials
support the use of fluoxetine, tricyclic antidepressants, or topiramate
CASE RESOLUTION
in the management of BN. Additional benefits are achieved when Although the adolescent may not currently meet strict criteria for the diagno-
sis of AN, her preoccupation with dieting in the context of weight loss and a BMI
medication is combined with cognitive behavioral therapy, dialec- of 17 is worrisome. Your concerns about the patient’s documented weight loss,
tical behavior therapy, or FBT. menstrual dysfunction, and current eating and dieting behaviors should be dis-
Medical complications of eating disorders are well established (see cussed openly with the teenager and her family. General laboratory tests should
Box 64.5). Inpatient treatment is required for less than 75% ideal body be performed. The adolescent should be referred to a mental health professional
weight for age, sex, and stature; continued weight loss despite inten- and registered dietician with experience in the management of eating disor-
ders for further evaluation. The emphasis should be on the teenager’s overall
sive outpatient treatment; or a history of rapid weight loss. Refusal
health and well-being. She should be followed frequently until her weight and
to eat and body fat less than 10% also are criteria for hospital admis- eating behaviors have reached the mutually agreed-on goal by all professionals
sion. Other indications include cardiovascular compromise, such as involved in her care, after which she should continue to be seen at regular inter-
bradycardia (<50 beats/minute during the day or <40 beats/minute vals by her primary care physician.
at nighttime), orthostatic hypotension (changes in blood pressure
>10 mm Hg, or pulse >20 beats/minute), or altered mental status;
evidence of persistent hypothermia (<35.6°C [<96.1°F]); suicidality
(ie, ideation, plan, or attempt) or out-of-control behavior; intracta- Selected References
ble vomiting; electrolyte disturbances or uncompensated acid-base Bachrach LK, Sills IN; American Academy of Pediatrics Section on
abnormalities (serum potassium <3.2 mEq/L or serum chloride Endocrinology. Bone densitometry in children and adolescents. Pediatrics.
<88 mEq/L); hematemesis; and significant dehydration as evidenced 2011;127(1):189–194. Reaffirmed June 2015 PMID: 21187316 https://doi.
by systolic blood pressure lower than 90 mm Hg or syncope. Cardiac org/10.1542/peds.2010-2961
arrhythmias, including prolonged QTc, also require inpatient moni- Butryn ML, Wadden TA. Treatment of overweight in children and ado-
toring. Rarely does confirmation of the diagnosis warrant an inpatient lescents: does dieting increase the risk of eating disorders? Int J Eat Disord.
2005;37(4):285–293 PMID: 15856498 https://doi.org/10.1002/eat.20098
stay. The goal of hospitalization is to correct medical complications,
document appropriate weight gain, and establish healthy and safe Carl RL, Johnson MD, Martin TJ; American Academy of Pediatrics Council on
Sports Medicine and Fitness. Promotion of healthy weight-control practices in
eating habits with the assistance of the parent or guardian.
young athletes. Pediatrics. 2017;140(3):e20171871 PMID: 28827381 https://doi.
org/10.1542/peds.2017-1871
Prognosis Fortune RS, Kaplan DW. Leg swelling a patient with anorexia nervosa. Adolesc
Overall, the outcome for the patient with an eating disorder is vari- Med State Art Rev. 2012;23(2):266–270 PMID: 23162930
able, with some patients recovering after minimal intervention and Garber AK, Kohn M. Newer approaches to acute nutritional rehabilitation for
with other patients developing more chronic problems. Binge eating, patients with anorexia nervosa. Adolesc Med. 2018;29(2):344–358

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 Chapter 64: Eating Disorders 455

Golden NH, Katzman DK, Sawyer SM, et al. Update on the medical management Rome ES, Strandjord SE. Eating disorders. Pediatr Rev. 2016;37(8):323–336
of eating disorders in adolescents. J Adolesc Health. 2015;56(4):370–375 PMID: PMID: 27482062 https://doi.org/10.1542/pir.2015-0180
25659201 https://doi.org/10.1016/j.jadohealth.2014.11.020 Rosen DS; American Academy of Pediatrics Committee on Adolescence.
Goldstein MA, Dechant EJ, Beresin EV. Eating disorders. Pediatr Rev. Identification and management of eating disorders in children and adolescents.
2011;32(12):508–521 PMID: 22135421 https://doi.org/10.1542/pir.32-12-508 Pediatrics. 2010;126(6):1240–1253. Reaffirmed November 2014 PMID: 21115584
Hogan M, Strasburger VC. Eating disorders and the media. Adolesc Med. https://doi.org/10.1542/peds.2010-2821
2018;29(2):208–227 Saldanha NE, Itriyeva K. Atypical anorexia nervosa. Adolesc Med. 2018;
Katzman DK. Medical complications in adolescents with anorexia nervosa: 29(2):279–287
a review of the literature. Int J Eat Disord. 2005;37(suppl):S52–S59 PMID: Sim LA, Lebow J, Billings M. Eating disorders in adolescents with a history
15852321 https://doi.org/10.1002/eat.20118 of obesity. Pediatrics. 2013;132(4):e1026–e1030 PMID: 24019418 https://doi.
Katzman DK, Turrini T, Grewal S. The role of the adolescent health provider and org/10.1542/peds.2012-3940
nutritionist in family-based therapy. Adolesc Med. 2018;29(2):359–374 Steinegger C, Katzman DK. Interviewing the adolescent with an eating disor-
Lock J, La Via MC; American Academy of Child and Adolescent Psychiatry der. Adolesc Med. 2008;19:18–40
Committee on Quality Issues. Practice parameter for the assessment and treat- Weiss Kelly AK, Hecht S; American Academy of Pediatrics Council on Sports
ment of children and adolescents with eating disorders. J Am Acad Child Adolesc Medicine and Fitness. The female athlete triad. Pediatrics. 2016;138(2):e20160922
Psychiatry. 2015;54(5):412–425 PMID: 25901778 https://doi.org/10.1016/j. PMID: 27432852 https://doi.org/10.1542/peds.2016-0922
jaac.2015.01.018
Phillips EL, Pratt HD. Eating disorders in college. Pediatr Clin North Am.
2005;52(1):85–96, viii PMID: 15748926 https://doi.org/10.1016/j.pcl.2004.10.003

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 CHAPTER 65

Body Modification:
Tattooing and Body Piercing
Monica Sifuentes, MD

CASE STUDY
A 16-year-old girl comes to your office for her annual On physical examination, the adolescent’s height
physical examination. Although the girl was previ- and weight are in the 50th percentile for age. Her body
ously healthy, her mother is concerned that the girl mass index is 21. Vital signs are normal. You note a small
seems irritable and unwilling recently to participate in tattoo at her right hip area. The girl’s mother is unaware
family events. The adolescent is currently in 10th grade of its presence, according to the teenager. She obtained
at a local public school, gets As and Bs in most sub- it a few months prior while visiting her sister in college.
jects, is a member of the volleyball team, and has just
begun working part-time at a movie theater. Both her
Questions
1. What is the epidemiology of body modification in
parents are employed, and the girl gets along well with
adolescents and young adults?
her 19-year-old sister, who is currently in college, and
2. What is the motivation for obtaining tattoos and
her 14-year-old brother. She has many friends in the
body piercing in this age group, and is there an asso-
neighborhood as well as at school.
ciation with high-risk behavior?
You interview the adolescent alone and learn that
3. What techniques are used to place tattoos and per-
she occasionally smokes marijuana, has tried cocaine on
form body piercing?
1 occasion, and attends parties at which many people
4. What are possible adverse consequences of body
are drinking alcohol. She has been sexually active in
modification, and what should be done to man-
the past but is not currently. She denies depression and
age them?
describes her mood as generally happy, except when she
5. How can the primary care physician assist an
is forced to spend what she believes is excessive time
adolescent in making a safe and healthy decision
with her family instead of with friends.
about body modification?

Body modification is the practice of permanently altering one’s an expression of their own individuality, or a desire to join a par-
appearance, and it has been practiced in many cultures worldwide ticular peer group, obtaining a tattoo or body piercing has become
for millennia. Such modification includes tattooing, body piercing, a widespread experience during adolescence and young adulthood
and scarification. Although much less common than tattooing and and therefore should be added to the primary care physician’s list of
body piercing, scarification uses various techniques to intention- issues to review with the teenager during the routine health main-
ally irritate the skin to produce a permanent pattern of scar tissue. tenance visit.
It is described as a more intense form of body modification and is The practice of typical body modification should be distin-
reportedly appealing to individuals seeking a more dramatic result. guished from more intense nonsuicidal self-injurious behav-
Historically, body modification, particularly tattooing, was asso- iors, such as cutting, scratching, burning, and hitting oneself. The
ciated primarily with the military and with disenfranchised individ- Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition
uals, such as criminals and gang members. Currently, however, it is (DSM-5) describes nonsuicidal self-injury disorder (NSSID) as a
considered mainstream among many individuals in US society, with mental health disorder associated with self-injury that can man-
people of all ages as well as socioeconomic and educational back- ifest in impulsive or compulsive adolescents suffering from anx-
grounds sporting tattoos and piercings. Body art is seen in most iety, depression, personality disorders, or psychotic disorders.
clinical settings serving youth and young adults as well as in mid- Adolescents engaged in self-injury expect to gain relief from their
dle schools and high schools and on college campuses. Additionally, negative emotions through physically hurting themselves and may
it is not uncommon to encounter a teenager with multiple tattoos use this behavior as a means of coping with personal emotional
and body piercings or to evaluate an adolescent for a possible com- issues. Consultation with a mental health specialist is warranted
plication of the procedure. Whether described as a rite of passage, for these challenging cases.
457

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 458 PART 4: ADOLESCENT HEALTH

Epidemiology Clinical Presentation

It was previously estimated that 13% to 25% of the general popula- Tattoos can be found on any area of the body and, depending on
tion in the United States had a tattoo. Because tattooing has grown the talent of the artist and the desire of the client, can be simple
in popularity and become a fast-growing retail business, however, and 1 color or quite elaborate and multicolored. Generally, amateur
these figures are likely a gross underestimation. Surveys conducted tattoos (ie, those performed by a nonspecialist or friend) are less
in outpatient clinics and on high school and college campuses con- intricate and are considered more risky because often they are not
firm that more than 10% to 15% of teenagers aged 12 to 18 years performed under ideal circumstances, using antiseptic techniques,
have a tattoo. In a study of more than 2,000 US high school students, or with conventional pigments and applicators. Unconventional pig-
55% expressed interest in obtaining a tattoo regardless of its perma- ments include charcoal, India ink, and mascara. Pencils, pens, sew-
nence or the students’ academic success in school. Additionally, it ing needles, and other sharp objects, including guitar strings, may
has been reported in the literature that one-half of new tattoos are be used to apply the dye for a self-administered or amateur tattoo.
obtained by women and that many are obtained during the college The most common site for piercing is high on the pinna of the ear.
years. Surveys conducted in the last 10 years reveal that approxi- Regular lobular ear piercings are not generally included in the context
mately 20% to 25% of college students have a tattoo. of body modification because they occur commonly at any age. Other
Body piercings are more common than tattoos among adoles- body sites that are pierced include the tragus of the ear, eyebrows,
cents and young adults, and some experts speculate that this may tongue, nose, nasal septum, cheek, lip, navel, and nipples. Intimate or
be because the procedure is not permanent, allowing the individ- genital piercings also can be seen in males and females. The foreskin,
ual to remove the jewelry at any time. In a study of college stu- penis, scrotum, clitoris, perineum, and labia are all common areas
dents, 45% had undergone body piercing and 22% had tattoos for intimate piercings. Although specific data by sex are not available,
with and without piercings. Other studies that include younger it is reported that more men than women obtain genital piercings.
adolescents attending an outpatient adolescent clinic report body
piercing in approximately 27% and tattoo in approximately 13%.
Technique, Application, and Safety
Most studies also confirm that females are more likely to obtain a
Standards
piercing than males; male athletes are more likely to obtain a tattoo
than their nonathlete male counterparts. Tattoos
The motivation for obtaining body art also has been studied, with The process of applying a professional or commercial tattoo is rela-
most of the literature confirming adolescents’ search for unique- tively standardized, although different tattoo parlors may have their
ness and desire to enhance their independence and self-identity. own individual practices. Generally, the client selects a design from
For some teenagers, body art is considered a form of decoration flash sheets in the shop or an individualized design by the artist,
or an opportunity to be creative; alternatively, it fulfills a desire and the tattoo is stenciled or drawn on clean-shaven skin. The skin
for peer acceptance and solidifies group membership. Attainment is cleansed again with an antiseptic solution, and a thin layer of oint-
of body art for the purpose of peer acceptance or solidifying group ment, such as petroleum jelly, is placed on the site. Most profession-
membership has been documented by studies demonstrating that als in the United States use a motorized, electric-powered tattoo gun,
friends or peers provide the strongest influence for youth pursuing which is similar to a dental drill, that holds 1 or several needles in
body art. Tattoos in particular can be a means of permanently docu- a needle bar that is dipped in ink and that punctures the skin a few
menting a relationship with an individual or group. Despite popular millimeters deep, up to several thousand times a minute. In Europe,
belief, studies do not confirm that most adolescents obtain a tattoo or small amounts of ink are applied directly to the skin and the nee-
body piercing impulsively while under the influence of illicit drugs or dle is used to push the ink into the skin. Regardless which method
alcohol. In fact, many older teenagers and young adults report taking is used, the pigment reaches the level of the dermis via the solid-
considerable time to decide whether to obtain a tattoo or piercing. bore needle or needless, while blood and serosanguineous fluid are
The extent to which parents or guardians are involved in the ado- wiped away as tattoo placement continues. When the tattoo is com-
lescent’s decision to have a tattoo placed or body piercing performed pleted or the session is finished, an antibiotic ointment is applied
is variable for many teenagers and depends on their particular and a bandage is placed over the site.
relationship, whether the adolescent is a minor, and state paren- The client is instructed to remove the dressing 24 hours later and
tal consent requirements. In some cases, parents are not consulted keep the area moist with an antibiotic ointment. Additionally, clients
before the procedure, although in many states the law requires are instructed to place an emollient or vitamin E oil over the heal-
parental consent and presence, and tattooing of minors is prohib- ing tattoo several times a day. The area is to be cleansed with a mild
ited. Current laws are not consistently enforced, however, and in soap and patted dry or blotted (not rubbed). If aftercare instruc-
many states tattooing and body piercing have different regulations. tions are followed carefully, most tattoos heal in approximately 2
Health professionals are therefore encouraged to become familiar to 3 weeks, with superficial crusting and “shedding” of epidermis
with their individual state laws related to minors obtaining tattoos as part of the natural course of tattoo placement. Sunscreen should
and piercings to counsel their patients appropriately. be worn if sun exposure cannot be avoided in the ensuing weeks.

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 Chapter 65: Body Modification: Tattooing and Body Piercing 459

Swimming, soaking in water, and direct shower jets to the area are exudate can occur with piercings of the nares or navel. Tongue or lip
discouraged for several weeks. piercings have been associated with significant swelling for several
Ideally, the application process uses inks that are poured into days after the procedure. Additionally, a yellow-white fluid secre-
single-use disposable containers and sterile needles that are disposed tion can occur that, to the unfamiliar examiner, appears to mimic
of after each client. Although the tattoo ink pigments are considered an infection. Clients with oral piercings are generally instructed
cosmetics and are subject to US Food and Drug Administration regu- to dissolve ice in their mouth immediately after piercing to help
lation, however, neither the tattooing process itself nor the use of the with pain and swelling, manage further discomfort with a nonste-
inks is regulated. Additionally, certain pigments may not be approved roidal anti-inflammatory drug, and elevate the head when sleeping.
for intradermal use and have been known to contain low concentra- Healing times vary considerably depending on the anatomic
tions of metal salts, such as lead, iron, mercury, or aluminum. site of the piercing. Generally, sites with increased vascularity and
The practice of universal precautions is required by state and exposure (eg, face, tongue) tend to heal faster than those involv-
local regulatory agencies and advocated by specific educational ing cartilage, which is poorly vascularized. Areas of the body that
groups, such as the Alliance of Professional Tattooists (APT), a non- are subject to movement also heal more slowly. For example, heal-
profit organization established in 1992 to promote standards for ing time may be 1 to 2 months for the tongue, 1 to 1.5 months for
professional and associate tattooists and develop guidelines for con- the nasal septum, 2 to 3 months for the nostril, and 2 to 4 months
sumers to evaluate the safety of individual tattooing establishments. for the tragus of the ear. High-ear piercings through the cartilage
The organization also sponsors regular educational seminars for tat- also may require 2 to 4 months for healing. Navel piercings have
too artists on the prevention of disease transmission in tattooing. the longest healing time (up to 9–12 months) because of friction
Membership in APT is voluntary and requires that the professional and moisture from clothing and often are associated with the most
tattooist pay annual dues, participate in a health and safety semi- complications.
nar, and have at least 3 years of full-time experience at a consistent As with tattooing, not all states have regulations and safety stan-
location. Other membership levels are available, with variable costs dards in place for body piercing, and if such regulations and safety
for annual dues and requirements for membership (eg, <3 years’ standards do exist, local governing bodies do not consistently enforce
experience and/or apprenticing associates). Despite APT efforts them. Universal precautions should be strictly practiced, and the
and standards, however, specific areas of concern about the tattoo adolescent should be familiar with these guidelines and know how
industry remain, including unlicensed tattoo artists and establish- to find a reputable piercer before obtaining body art.
ments, the presence of unregulated ingredients in the pigments, Because no formal training programs exist for piercers, many
inconsistent cleaning of equipment between clients, an inability learn by video or apprenticeship. Generally, practitioners in stu-
to reliably sterilize all parts of the equipment despite good efforts, dios have completed an apprenticeship and have more training
and infrequent inspections of tattoo parlors by regulatory agencies. than those in cosmetic shops, malls, or ear-piercing kiosks. They
also are more experienced in piercing sites other than the ears and
Body Piercing may be members of the Association of Professional Piercers (APP).
The process of body piercing is generally less complicated than Established in 1994, the APP is a nonprofit organization dedicated
tattooing and depends, in part, on the anatomic site to be pierced. to the education, health, and safety of body piercing for the pub-
The client chooses the jewelry and body part to be pierced, the area lic. It has developed self-regulatory policies for the industry, stan-
is cleaned with a topical antiseptic, and a large hollow needle is dards for membership in the organization, and annual conferences
brought through the skin. The jewelry is then brought through on health and safety issues. Members must have at least 1 year of
the hole following the needle, and the hole is sealed with a bead, piercing experience, documented training in blood-borne patho-
bar, or metal disc. Because the procedure is relatively quick, topical gens and cardiopulmonary resuscitation, and certification in first
anesthetics are generally not required. aid. Members also must show photographic proof of a medical-
Although earlobe piercing is a relatively straightforward proce- grade autoclave in the piercing studio and send in spore test results
dure, it is commonly performed using a piercing gun at a local mall, from the autoclave. To help document this, a detailed video of the
cosmetic shop, or kiosk. Because the stud is driven through the ear- studio is required, along with copies of all aftercare education given
lobe via the gun rather than through a hollow tube manually, the to clients. After this process is completed, the member receives a
tissue is torn or crushed rather than pierced. Additional concerns certificate to mount in the studio.
about the piercing gun include inconsistent and informal training of Current legislation addressing minors and piercing is regulated
personnel, an inability to sterilize all parts of the gun between pro- by individual states; in some states, such as California, ear piercing
cedures, and embedded earrings and ear backs. The gun cannot be performed with piercing guns is excluded from the definition of
adjusted for the thickness of other tissues, so although it is a popu- body piercing. Concerning minors, the APP requires that the par-
lar method for earring placement, this tool is not recommended for ent or legal guardian as well as the minor show proof of identifica-
sites other than the earlobe. tion before signing the consent form for body piercing. Additionally,
The immediate aftercare of piercing varies by the site pierced. nipple or genital piercings are not performed on anyone younger
For example, local skin discoloration and a nonmalodorous serous than 18 years.

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 460 PART 4: ADOLESCENT HEALTH

Evaluation Box 65.2. The Association Between

History Body Modification and High-Risk Behavior
All teenagers should be interviewed alone, after their parent or legal in Teenagersa
guardian has had an opportunity to discuss their concerns with the ww Adolescents with tattoos or body piercings were found to be more likely
health professional (see Chapter 4). A visible tattoo or body pierc- to have engaged in risk-taking behaviors than those without either type
ing allows the physician to inquire about the circumstances sur- of body modification. Risk-taking behaviors included disordered eating
rounding the body art at the beginning of the interview, in contrast behavior, gateway drug use (ie, cigarettes, alcohol, marijuana), hard
to body art noted in an inconspicuous area of the body during the drug use (ie, cocaine, crystal methamphetamine, ecstasy), sexual
physical examination. Whether parental consent was obtained before activity, and suicide. Additionally, aggressive and violent behavior was
the procedure should be addressed directly, because this issue could associated with males having tattoos and with females having body
be a basis for current as well as future familial conflict. The type of piercings. Gateway drug use was associated with younger age of tattoo-
facility in which the tattoo or piercing was obtained should be dis- ing and body piercing.b
cussed, along with whether the adolescent recalls whether univer- ww Adolescents with body modification had 3.1 times greater odds of prob-
sal precautions were followed. A general review of systems should lem substance use compared with those without body modification.c
be performed to exclude systemic conditions, such as viral hepati- ww Tattooing was significantly associated with older age, living in a single-
tis, as well as any other complications related to obtaining the tat- parent household, and lower socioeconomic status. Such adolescents
too or piercing in a nonprofessional environment. Specific questions also exhibited greater involvement in sexual intercourse, higher levels
about the area of the body that is pierced also should be reviewed of substance abuse themselves as well as among their peers, increased
(Box 65.1). For example, the teenager with a tongue piercing should violent behaviors, and school problems.d
be asked if he, she, or they has problems with mastication, swallow- ww A correlation was found between body piercing in teenagers and increased
ing, loss of taste or movement, or permanent numbness. rate of sexual intercourse, smoking, marijuana use, school truancy, running
It has been reported that amateur or self-administered tattoos are away, suicidal ideation or attempts, and peer substance use.e
associated with increased high-risk behaviors, including substance
abuse at a younger age, illicit drug use, lower academic achieve- a
Many studies have been conducted to explore the association between high-risk behavior and
ment, and an increased number of tattoos overall. Recent studies body modification in adolescents. No definitive answers exist, but this box presents published
have shown, however, that not all adolescents and young adults with findings from the early 2000s.
b
Carroll ST, Riffenburgh RH, Roberts TA, Myhre EB. Tattoos and body piercings as indicators of
multiple tattoos or body piercing(s) engage in high-risk behav-
adolescent risk-taking behaviors. Pediatrics. 2002;109(6):1021–1027.
ior (Box 65.2). Successful academic achievement and close fam- c
Brooks TL, Woods ER, Knight JR, Shrier LA. Body modification and substance use in adolescents:
ily support have been reported in tattooed and nontattooed college is there a link? J Adolesc Health. 2003;32(1):44–49.
students. Although tattoos are permanent, more than 50% of aca- d
Roberts TA, Ryan SA. Tattooing and high-risk behavior in adolescents. Pediatrics.
demically successful high school students with consistently good 2002;110(6):1058–1063.
e
Armstrong ML, Roberts AE, Owen DC, Koch JR. Contemporary college students and body piercing.
grades have reported an interest in them.
J Adolesc Health. 2004;35(1):58–61.

Box 65.1. What to Ask Whether body art is considered an expression of individuality,
Tattoos and Body Piercing rebellious behavior, or succumbing to peer pressure, the presence
ww When was the tattoo or body piercing placed? of a tattoo or body piercing on an adolescent warrants an in-depth
ww Did the teenager obtain consent from the parent or legal guardian psychosocial assessment and review of systems for possible expo-
before getting the tattoo or piercing? sure to viral infections, such as hepatitis C.
ww Is the adolescent satisfied with the tattoo or piercing?
ww Was the tattoo or body piercing placed by a professional or by a friend,
Physical Examination
acquaintance, or relative? In most adolescents with a tattoo or body piercing the routine phys-
ww If the tattoo or body piercing was obtained in a studio, where was the ical examination is generally normal, unless either a past compli-
studio located? Was it licensed? Was it clean, “like a medical facility”? cation with the tattoo or piercing occurred or a current problem
ww Did the tattooist or piercer wash his, her, or their hands before glov- exists. Poor aftercare and hygiene can prolong healing time in body
ing? Use new disposable gloves? Open all equipment in front of the piercings. Additionally, smoking can delay the healing time associ-
teenager? ated with oral piercings. If the teenager has recently undergone a
ww For tattoos, did the tattooist remove a sterile needle and tube set from a new tongue piercing, a larger barbell will be seen through the tongue.
envelope? Did the tattooist pour fresh ink in a new disposable container? Larger barbells initially are placed with tongue piercing to accom-
ww For body piercing, did the piercer use individually wrapped sterile nee- modate the swelling associated with the procedure. Later, the bar-
dles? Did the piercer use a piercing gun? bell is replaced with a shorter rod.
ww Did the teenager receive aftercare education, including written material? Infectious and noninfectious complications from tattoos and
body piercings are listed in Box 65.3. Local infection occurs in only

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 Chapter 65: Body Modification: Tattooing and Body Piercing 461

Box 65.3. Complications of Tattoos and Body Piercing: Infectious and Noninfectious
Tattooing Complications Noninfectious Noninfectious
Infectious ww Hypersensitivity to dyes or pigments Body jewelry, in general
Bacterial etiologies ww Allergic granulomas ww Artifact on radiographs
ww Local skin infections ww Malignant melanoma and basal cell High-ear piercing
—— Superficial pyoderma carcinoma at tattoo site ww Pinna deformity
—— Staphylococcus aureus ww Keloid formation Tongue piercing
—— Streptococcus pyogenes ww Swelling and burning during magnetic ww Airway obstruction
ww Systemic infections resonance imaging ww Chipped/cracked teeth
—— Deep or severe pyoderma Body Piercing Complications ww Interference with mastication/swallowing
—— Syphilis Infectious ww Permanent numbness
—— Mycobacterium tuberculosis Bacterial etiologies ww Articulation disorders
—— Mycobacterium leprae ww S aureus ww Loss of taste/movement
—— Nontuberculous mycobacteria ww Pseudomonas aeruginosa ww Oral mucosa inflammation
—— Mycobacterium chelonae ww Group A b-hemolytic streptococcus Lip piercing
—— Mycobacterium abscessus —— Cellulitis ww Injury to salivary ducts
—— Chancroid —— Septic arthritis ww Aspiration of jewelry
—— Tetanus —— Acute glomerulonephritis Navel piercing
—— Endocarditis —— Erysipelas ww Allergic dermatitis
Viral etiologies —— Endocarditis Nipple piercing
ww Human papillomavirus ww M tuberculosis ww Trauma/avulsion of nipple
ww Hepatitis B and C ww Clostridium tetani Genital piercing
ww HIV Viral etiologies ww Tissue inflammation in sexual partner
ww Hepatitis B and C ww Scarring
ww HIV ww Interruption of urinary flow in males

approximately 5% of tattoos, but infectious complications have been the henna to give the normal red-brown paste an additional black-
reported in as many as 30% of body piercings. Acute signs of infec- and-blue color. The addition of paraphenylenediamine to the henna
tion include erythema, warmth, swelling, and pain at the site, in mixture also speeds drying time and prolongs skin pigmentation.
addition to drainage in some cases. Rarely, a fluctuant, fluid-filled Keloids and hypertrophic scars can appear as a flesh-colored
mass is evident if an abscess has developed. mass at the area of the tattoo or piercing and differ in their tim-
Because some of the noninfectious complications can be related ing and resolution. A hypertrophic scar generally appears within
to the type of metal found in the jewelry, knowledge of this specific 6 weeks of the tattooing or piercing, is confined to the wound margins,
information is useful in individuals with body piercing. Only jew- and has a tendency for spontaneous regression. In contrast, keloid
elry made from surgical stainless steel, titanium, solid 14- or 18-karat formation may occur as late as 1 year after the initial wound, often
gold, or solid platinum should be used in healed piercings to avoid grows beyond the border of the wound, and persists. Keloids occur
allergic dermatitis. Certain metals, such as nickel, cobalt, and chro- primarily in black and Asian patients and can cause an itching or
mium, have been associated with the development of contact der- burning sensation that may warrant a prompt referral for removal.
matitis in sensitive individuals. Initially, during the healing phase of
piercings, however, surgical stainless steel and 14-karat gold jewelry Laboratory Tests
should be avoided because they may contain trace amounts of nickel. Generally, laboratory studies are not necessary if the adolescent is
Permanent makeup also has been reported to cause severe allergic not engaged in high-risk behavior and is certain that universal pre-
contact dermatitis that can take months to years to completely heal. cautions were followed when the tattoo or piercing was placed. In
The reported reactions included tenderness, itching, and “bumps” most cases, however, the teenager may be uncertain or may not
at the site of the permanent makeup application. Hypersensitivities remember the details. In such cases, a serum test for viral hepatitis
to dyes or pigments from a professional tattoo also can appear as an B and C should be obtained, because hepatitis C virus is found in
erythematous outline of the original work. This inflammatory reac- approximately 30% of people with tattoos, compared with 3.5% of
tion can also occur with temporary tattoos created with henna, which people without them. An antibody test for HIV should be sent for the
is approved as a hair dye but is not approved for use on the skin. Use high-risk teenager who is being screened for other sexually transmit-
of henna has been associated with severe contact dermatitis, espe- ted infections; however, the test is not necessary if the patient has a
cially if an additive containing paraphenylenediamine is mixed with tattoo or body piercing and no other indications for HIV screening.

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 462 PART 4: ADOLESCENT HEALTH

No definitive documented cases of HIV transmission from tattoo- pigments or the tattoo is modified and made into another design.
ing or body piercing have been reported to date. For instance, the name of a person can be incorporated into a new
A serum test for syphilis should be done because, unlike HIV, tattoo of an animal or object. Certain nonprofit organizations also
transmission of this and other sexually transmitted infections has offer tattoo removal to former gang members as a part of their prep-
been reported from tattooing or piercing, albeit rarely. aration for employment and educational services.
If evidence exists of abscess formation, such as may be seen with Although it seems counterintuitive, in most cases removal of jew-
perichondritis, a specimen of the purulent fluid should be obtained elry is not recommended if a piercing appears infected. The concern
and sent for culture and antimicrobial sensitivities. is that, without a wick or surgical drain, any potential space left at
the site after the jewelry is removed could result in the development
of an abscess. Instead, the adolescent should be instructed to leave
Management the jewelry in place to allow drainage of the wound, use warm com-
Although tattoo removal or modification is not ordinarily requested presses, and clean the area with an antimicrobial soap and water. The
by most adolescents, it is available for the patient who no longer wants use of topical antibiotic ointments is controversial because they can
the tattoo or is unhappy with its current appearance. Recent studies be occlusive and contribute to delayed healing. Certain individuals
have examined the motivation for tattoo removal after a duration of with specific medical conditions are at increased risk for infections
at least 10 years. Reasons for removal are varied and include embar- after body piercing, including patients with diabetes mellitus, sys-
rassment, a need to disassociate from the past, improved self-esteem, temic lupus erythematosus, and conditions requiring chronic corti-
being tired of the tattoo, and negative social remarks about the tattoo. costeroid use. In such cases, if infection occurs it may be necessary
Professional employment or job advancement was not consistently to remove the jewelry early in the course of the infection and initi-
cited as a common reason for removal, although some surveys attri- ate appropriate antibiotic coverage.
bute tattoos in certain visible locations as career limiting. Oral antibiotic coverage against skin staphylococcal and strepto-
Historically, tattoo removal was quite difficult, and only approxi- coccal species should be administered if the tattoo or piercing appears
mately 70% of tattoos could be completely cleared because of impu- superficially infected and other measures have not been effective.
rities in tattoo pigments, different ink densities and depths, and More aggressive treatment is required if the piercing site involves the
the presence of certain metals in the dyes. Selective photothermol- cartilage, such as with high-ear piercings, or if the patient is immu-
ysis, which is a newer technology that uses a selective type of laser nocompromised. Auricular infections can occur even after the use of
to target specific color pigments, is quite effective in removing sev- strict antiseptic techniques and may appear a few weeks after the ini-
eral ink colors. Using a quality-switched laser system, the wave- tial piercing. The cartilaginous helical area of the ear is particularly
length of the laser is set to match the specific absorption pattern prone to infection because it is poorly vascularized and is slow to heal.
of the different color pigments in the tattoo and a pulse is deliv- Additional antimicrobial coverage against Pseudomonas aeruginosa
ered over nanoseconds with extremely rapid heating. Fragmentation is essential in these cases, along with diligent follow-up to monitor
of the tattoo pigment occurs and, upon releasing it into the skin, the initial response to oral antibiotic therapy. Currently, oral fluo-
an acute inflammatory process follows along with phagocytosis of roquinolones offer good antipseudomonal and antistaphylococcal
fragmented pigment particles. Subsequent laser treatments can be coverage and penetrate cartilage well. Inpatient hospitalization for
performed 4 weeks later, although longer intervals between treat- intravenous antimicrobial therapy and subsequent drainage of the
ments may reduce the risk of permanent changes to the pigment of site may be necessary for moderate or unresponsive infections. Early
the skin. No laser method is completely successful in removing all recognition of perichondritis and appropriate management of it are
evidence of the tattoo, especially intricate and colorful ones, and in essential to prevent the development of a persistent infection and to
most cases, immediate lightening of the skin occurs with subsequent minimize the risk of a permanent auricular deformity. Additionally,
hypopigmentation. Hyperpigmentation, allergic reactions, and scar- timely consultation with a plastic surgeon or otolaryngologist early in
ring resulting from thermal burn injury may also occur after laser the course of the suspected infection is recommended for early inci-
treatment. Although these complications are usually transient, they sion and drainage of a perichondral abscess, appropriate wound care,
can be permanent, and the patient must be made aware of the risks and possible reconstruction of any disfigurement. Additional infor-
associated with each process. Tattoo removal can be costly. Whereas mation about body piercing complications and their management
the average cost for tattoo placement may be $50 to $100 per hour in adolescents and young adults can be found in the 2017 American
of service, removal may cost several thousands of dollars depending Academy of Pediatrics Committee on Adolescence article on tattoo-
on the size, complexity, and number of colors in the tattoo. Other, ing, piercing, and scarification in that population.
less popular techniques for tattoo removal include dermabrasion,
which is less desirable because of concerns for infection and its vari-
able effectiveness; salabrasion (ie, use of a salt solution to abrade the Role of the Primary Care Physician
skin); scarification; and surgical excision, with or without the use of Education is essential for the adolescent who has not yet obtained
tissue expanders and grafting. Camouflaging also can be performed; a tattoo or piercing or who already has one and is contemplating
with this technique, either a new pattern is made using skin-toned the placement of another. The physician should inquire where the

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 Chapter 65: Body Modification: Tattooing and Body Piercing 463

adolescent plans to have the procedure performed and educate the Armstrong ML, Roberts AE, Owen DC, Koch JR. Contemporary college students
patient on what key questions to ask and how to find a reputable and body piercing. J Adolesc Health. 2004;35(1):58–61 PMID: 15193575 https://
studio. Additional information, such as the APT (www.safe-tattoos. doi.org/10.1016/S1054-139X(03)00338-0

com) and APP (www.safepiercing.org) websites, should be shared Association of Professional Piercers. https://www.safepiercing.org. Accessed
with the patient, and written materials that contain safety guide- April 5, 2019

lines should be provided to the adolescent at the visit. Teenagers Beers MS, Meires J, Loriz L. Body piercing: coming to a patient near you.
are often reluctant to ask their health professionals for information Nurse Pract. 2007;32(2):55–60 PMID: 17264796 https://doi.org/10.1097/
00006205-200702000-00011
about obtaining tattoos and piercings and almost never contact a
health professional if they believe they may have a complication Braverman PK. Body art: piercing, tattooing, and scarification. Adolesc Med Clin.
2006;17(3):505–519 PMID: 17030277
associated with tattooing or piercing. Instead, they tend to ask their
peers or contact the establishment at which the tattoo or piercing Breuner CC, Levine DA; American Academy of Pediatrics Committee on
Adolescence. Adolescent and young adult tattooing, piercing, and scarification.
was initially obtained. Primary care physicians should be a nonjudg-
Pediatrics. 2017;140(4):e20171962 PMID: 28924063 https://doi.org/10.1542/
mental resource during health maintenance visits to ensure the ado- peds.2017-1962
lescent’s continued health and safety. Additionally, the appearance
Brooks TL, Woods ER, Knight JR, Shrier LA. Body modification and substance
of an uncommon medical condition, such as unexplained hepatitis, use in adolescents: is there a link? J Adolesc Health. 2003;32(1):44–49 PMID:
endocarditis, or toxic shock, in the adolescent patient or young adult 12507800 https://doi.org/10.1016/S1054-139X(02)00446-9
warrants careful consideration for a possible complication related to Carroll ST, Riffenburgh RH, Roberts TA, Myhre EB. Tattoos and body pierc-
tattooing or body piercing. ings as indicators of adolescent risk-taking behaviors. Pediatrics. 2002;109(6):
1021–1027 PMID: 12042538 https://doi.org/10.1542/peds.109.6.1021
Prognosis Desai NA, Smith ML. Body art in adolescents: paint, piercings, and perils. Adolesc
Most teenagers and young adults experience no adverse effects from Med State Art Rev. 2011;22(1):97–118, viii–ix PMID: 21815446
body modification. Complications are uncommon with the place- Glassy CM, Glassy MS, Aldasouqi S. Tattooing: medical uses and problems.
ment of a professional tattoo but can occur with body piercing and Cleve Clin J Med. 2012;79(11):761–770 PMID: 23125325 https://doi.org/10.3949/
ccjm.79a.12016
usually are amenable to medical management. Risk-taking behav-
ior that may occur in conjunction with body modification can carry Juhas E, English JC III. Tattoo-associated complications. J Pediatr Adolesc
Gynecol. 2013;26(2):125–129 PMID: 23287600 https://doi.org/10.1016/j.
long-term sequelae, but the association between defiant behavior
jpag.2012.08.005
and having a tattoo has changed over time. Although body mod-
Kluger N. Acute complications of tattooing presenting in the ED. Am J Emerg
ification has become more widespread and generally is acceptable
Med. 2012;30(9):2055–2063 PMID: 22944541 https://doi.org/10.1016/j.
based on public opinion, any decision to pursue body modification ajem.2012.06.014
should be made in the context of the adolescent’s long-term and
Laumann AE, Derick AJ. Tattoos and body piercings in the United States: a
professional career goals, because studies have documented nega- national data set. J Am Acad Dermatol. 2006;55(3):413–421 PMID: 16908345
tive repercussions in some areas of employment. https://doi.org/10.1016/j.jaad.2006.03.026
Meltzer DI. Complications of body piercing. Am Fam Physician. 2005;
72(10):2029–2034 PMID: 16342832
CASE RESOLUTION Messahel A, Musgrove B. Infective complications of tattooing and skin piercing.
Because the presence of 1 tattoo may be associated with a likelihood to obtain J Infect Public Health. 2009;2(1):7–13 PMID: 20701856 https://doi.org/10.1016/j.
another tattoo, the primary care physician should review safety guidelines for jiph.2009.01.006
obtaining a tattoo and body piercing with the adolescent and offer the teenager National Conference of State Legislatures. Tattooing and body piercing: state
educational material or refer her to select websites to reinforce the discussion.
laws, statutes, and regulations. NCSL.org website. http://www.ncsl.org/research/
The immunization status of the teenager also should be assessed, with particu-
health/tattooing-and-body-piercing.aspx. Updated March 13, 2019. Accessed
lar attention to tetanus and hepatitis A and B. Additionally, in private discussion
July 19, 2019
with the teenager, the physician should reiterate any concern about the adoles-
cent’s current high-risk behavior and its possible consequences. It also may be Roberts TA, Ryan SA. Tattooing and high-risk behavior in adolescents.
worthwhile to discuss with the teenager the pros and cons of telling her parents Pediatrics. 2002;110(6):1058–1063 PMID: 12456900 https://doi.org/10.1542/
about the tattoo before they find out inadvertently. peds.110.6.1058
Stewart GM, Thorp A, Brown L. Perichondritis—a complication of high ear
piercing. Pediatr Emerg Care. 2006;22(12):804–806 PMID: 17198212 https://
doi.org/10.1097/01.pec.0000248687.96433.63
Selected References
Straetemans M, Katz LM, Belson M. Adverse reactions after permanent-makeup
Alliance of Professional Tattooists. http://www.safe-tattoos.com. Accessed July 19, procedures [correspondence]. N Engl J Med. 2007;356(26):2753 PMID: 17596617
2019 https://doi.org/10.1056/NEJMc063122
Armstrong ML, Caliendo C, Roberts AE. Genital piercings: what is known and Tohme RA, Holmberg SD. Transmission of hepatitis C virus infection through
what people with genital piercings tell us. Urol Nurs. 2006;26(3):173–179 PMID: tattooing and piercing: a critical review. Clin Infect Dis. 2012;54(8):1167–1178
16800324 PMID: 22291098 https://doi.org/10.1093/cid/cir991

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 CHAPTER 66

Depression and Suicide

in Adolescents
Monica Sifuentes, MD, and Robin Steinberg-Epstein, MD

CASE STUDY
A 15-year-old girl is brought to your office by her mother Questionnaire. The physical examination is entirely nor-
with the chief report of easy fatigability. The mother mal, although the girl’s affect appears somewhat flat.
is concerned because her daughter is always tired,
although several other physicians have told her that the
Questions
1. What is the significance of nonspecific symptoms,
girl is healthy. The adolescent, who states no complaints
such as fatigue, during adolescence?
or concerns, appears quite shy. She is currently in the
2. What factors contribute to depression in the
10th grade, likes school, receives average grades, and
adolescent?
speaks English and Spanish. The mother, a single par-
3. What are the classic signs and symptoms of depres-
ent, moved to the United States from El Salvador approx-
sion in the adolescent?
imately 2 years ago with her 2 daughters. Currently,
4. What are some important points to cover in the
they are living with relatives in a two-bedroom apart-
history when interviewing the adolescent with
ment. The mother is employed as a housekeeper, and
suspected depression?
the patient and her sister help their mother clean homes
5. What is the purpose of the depression/suicide
on weekends. During the week they make dinner for the
screening tool (eg, Patient Health Questionnaire-9)?
rest of the family as a means of contributing to the rent.
How should the results be interpreted and used?
When you speak to the girl alone, she acknowledges she
6. How is the risk of suicide assessed in the adoles-
has a few friends at school and adamantly denies any
cent patient?
drug, alcohol, or tobacco use. She has never been sex-
7. How should suicidal behavior (ie, suicide attempts)
ually active and reports no history of sexual or phys-
be managed in the adolescent?
ical abuse. She scores 11 on the 9-item Patient Health

The number of people in the United States with mental health family physicians) write most of the antidepressant prescriptions
concerns, including depression and suicidality, far surpasses the in the United States.
number of mental health specialists. For this reason, the American Depression and suicidality are common in the pediatric and
Academy of Pediatrics recommends that primary care physicians adolescent population. Thus, it is important to remain cognizant
take an active role in the identification and early management of of their clinical presentation and to diligently screen and probe for
uncomplicated mental health concerns in children and adoles- their presence.
cents. Furthermore, the importance of primary care physicians Depression is among the multiple risk factors that predispose
in this arena is emphasized by research findings. Patients who adolescents to suicide. Not all teenagers who attempt suicide
ultimately die by suicide visit primary care physicians more than are depressed, however; conversely, not all depressed adoles-
twice as often as mental health clinicians in the months leading up cents attempt suicide. This distinction is important to keep in
to their death. A review of studies analyzing this clinical scenario mind when evaluating any adolescent for depression or suicidal
estimated 45% of those who died by suicide saw their primary care behavior. Early identification of risk factors in the susceptible
physician in the month before their death, whereas only 20% saw adolescent along with early intervention for those with depres-
a mental health professional in the preceding month. Women and sive symptoms will, it is hoped, benefit the teenager at risk
older patients are more likely to have sought care in the month for suicide and allow the primary care pediatrician to provide
before their suicide compared with men and younger patients. first-line intervention for the adolescent patient experiencing
Those who practice general medicine (ie, internists, pediatricians, emotional distress.

465

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 466 PART 4: ADOLESCENT HEALTH

Epidemiology with suicide plans reported attempting suicide, and 2.4% of the
individuals who attempted suicide required medical attention. The
Depression
prevalence of developing a suicide plan was higher among gay, les-
The exact prevalence of depression in adolescents is difficult to deter- bian, and bisexual students (38.0%) and “not sure” youth (25.6%)
mine because depression is often underreported. It is considered 1 than among heterosexual students (10.4%). Rates also differ by race
of the main psychiatric conditions affecting children and adoles- and ethnicity, with black and Asian teenagers having lower suicide
cents, however, along with anxiety. Depressive symptoms have been rates than white teenagers, and black females having the lowest
reported in as many as 50% of girls and 40% of boys in the 14- to suicide rate of all adolescents. American Indian/Alaska Native males
15-year age group. The overall prevalence of depression as an ill- have the highest suicide rate among this age group.
ness is approximately 5%; mild depression is reported in 13% to In discussing adolescent depression and suicide, it is impor-
28% of teenagers, moderate depression in 7%, and severe depres- tant to clarify the meaning of specific terms. Suicidal ideation is
sion in 1.3%. Depression occurs more commonly in adolescents than thoughts of engaging in suicide-related behavior. Suicidal intent is
in prepubertal children and is more frequent in females than males having the aim or resolve to follow through with a plan. Suicidal
after puberty. behaviors are behaviors related to suicide, including preparatory
Several risk factors contribute to the development of depressive acts, suicide attempts, and death. Suicide attempt is a nonfatal, self-
disorders in adolescents (Box 66.1). Certain psychiatric conditions directed, potentially injurious behavior with any intent to die as the
also are associated with depression, including generalized anxiety result of the behavior. A suicide attempt may or may not result in
disorders, eating disorders, substance abuse, conduct disorders, and injury. Suicide is death caused by self-directed injurious behavior
borderline personality disorders. with any intent to die as the result of the behavior.
Several risk factors associated with adolescent suicide have been
Suicide and Suicidal Behavior
identified (Box 66.2). Suicide is rarely associated with depression but
Suicide is the second-leading cause of death in the United States in is most often associated with a recent, abrupt crisis (eg, breakup of
individuals 10 to 24 years of age; only motor vehicle crashes result in
more deaths in young people. In 1960, the annual suicide rate in this
age group was 5.2 per 100,000. The suicide rate has continued to rise Box 66.2. Risk Factors Associated With Suicide
over the past 50 years. According to the Centers for Disease Control in Adolescents
and Prevention, the suicide rate in 2017 was 11.8 per 100,000, with
6,241 completed suicides in 15- to 24-year-olds. It has been stated ww History of a previous suicide attempt (most important)
that for every suicide that is completed successfully, 50 to 100 sui- ww Male sex
cides are attempted. More than 75% of teenagers who committed ww History of adoption
suicide had not been on medication and were not under treatment ww Lesbian, gay, bisexual, or questioning sexual orientation
for depression or suicidal concerns. ww Transgender identification
According to the 2017 Youth Risk Behavior Survey of the Centers ww History of physical and/or sexual abuse or exposure to violence
for Disease Control and Prevention, 17.2% of all students in grades ww Family history of psychiatric disorders, especially depression, substance
9 to 12 nationwide had seriously considered attempting suicide dur- abuse, and suicidal behavior
ing the previous 12 months. Approximately 14% of students nation- ww Personal mental health problems
wide had made specific suicide plans, more than 50% of students —— Sleep disturbances
—— Psychological characteristics, such as aggression, impulsivity, and
hopelessness or severe anger
Box 66.1. Risk Factors Associated With Depressive —— Preexisting psychiatric condition (eg, depressive/bipolar disorder,
Disorders in Adolescents conduct disorder, posttraumatic stress disorder)
—— Alcohol and illicit substance abuse or dependence
ww Family history of psychiatric illness (eg, parent with an affective —— Pathologic internet use
condition, another family member with a bipolar or recurrent unipo- ww Social and environmental issues
lar disorder) —— Family disruption or stressful life event, including violence, divorce,
ww Age at onset of depression in the affected parent; the earlier the age of or death of a loved one
onset, the greater the likelihood of depression in any children —— Impaired parent-child relationship
ww Exposure to an unexpected suicide attempt or completion in the school —— Living outside the home (eg, homeless, corrections facility, group
or community home)
ww History of environmental trauma (eg, sexual or physical abuse, loss of a —— Bullying
loved one) —— Exposure to an unexpected suicide attempt or completion in the
ww Chronic illness school or community
ww Certain medications (eg, propranolol, phenobarbital, prednisone) —— Availability of firearms in the home

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 Chapter 66: Depression and Suicide in Adolescents 467

a romantic relationship, accusation, failure). Although adolescent commit suicide have a family member or close relative who has
females are more likely to attempt suicide than males (22% and committed suicide. Similarly, a family history of major depression
12%, respectively), males are more likely to succeed (male-to-female is a significant risk factor for depression in children and adolescents.
ratio, 4:1). This fact may result from the lethality of the methods, Studies suggest the incomplete penetrance of a dominant gene as
such as firearms or hanging, that males usually choose. Although a possible etiology for this finding. Regardless of the exact mecha-
females are more likely to ingest pills, the role of firearms in suicide nism, genetic influences can increase the adolescent’s vulnerability
attempts or completion in females is increasing. The availability of for depression. Specific environmental events can occur in an adoles-
firearms and alcohol, which varies from state to state, greatly con- cent’s life that may precipitate a depressive episode, such as the loss
tributes to the occurrence of suicide. Up to 45% of individuals who of a loved one or parental divorce. Other events, such as physical or
have committed suicide show some evidence of intoxication at the sexual abuse, also can trigger depression in a susceptible teenager.
time of death. Although most suicide attempts are impulsive, studies
have shown that adolescents often have communicated their suicidal Differential Diagnosis
intent or ideation to someone before the attempt. Approximately The differential diagnosis of depression includes any condition
50% of adolescents who attempt suicide have sought medical care that may alter an individual’s cognition or affect. For example, if a
within the preceding month and 25% within the preceding week. In disease alters one’s nutritional status and leads to malnourishment,
contrast, only one-third have previously received mental health care. this may alter affect and energy, which may resemble depression
(Box 66.3). Examples of such diseases include cancer, tuberculosis,
Clinical Presentation and eating disorders (eg, anorexia nervosa). Endocrine disorders,
The depressed or suicidal adolescent may visit a physician for a such as hypothyroidism, hyperthyroidism, and Addison disease,
variety of clinical reasons, but rarely do they seek professional assis- can mimic depression. Central nervous system (CNS) pathology,
tance for feeling “depressed.” Some adolescents have a difficult time although rare, includes tumors, infections, postconcussion syn-
accurately understanding and communicating their emotions. A dromes, and cerebrovascular accidents. Concomitant systemic ill-
depressed teenager often presents as irritable, argumentative, or nesses, such as systemic lupus erythematosus, diabetes mellitus, and
angry rather than sad. Teenagers may exhibit diminished interest AIDS, can have CNS manifestations that may be mistaken for an
or pleasure in activities or relationships and changes in cognitive isolated episode of depression. Although these diseases can occur,
functioning (eg, concentration), sleep, appetite, or energy, which their prevalence pales in comparison to the prevalence and signifi-
results in impairments in multiple activities of daily living. They cant contribution of substance and alcohol abuse. It is also impor-
also may present with seemingly nonemergent complaints and a flat tant to recognize that many chronic conditions are stressful and
affect or with multiple somatic concerns and an anxious appearance. can place patients at risk for comorbid depression. Other mental
Additionally, the teenager may have frequent visits to the primary
care physician’s office for acute conditions that on first glance seem
unrelated but later indicate possible substance abuse or a mood dis- Box 66.3. Diagnosis of Depression in Adolescents
order. Some adolescents are accompanied by a family member or SIGE CAPS Mnemonic
friend, which initially may make the teenager reticent to discuss psy- S: Sleep changes
chosocial issues with the physician. According to the Diagnostic and I: Interests—decreased interest in school or activities
Statistical Manual of Mental Disorders, Fifth Edition, Text Revision G: Guilt, helpless, hopeless
(DSM-5), major depressive disorder (MDD) is diagnosed when at E: Energy (decreased), fatigue
least 5 of 9 listed symptoms or signs occur for a duration of at least C: Concentration decreased
2 weeks. At least 1 symptom must be sadness or loss of interest A: Appetite (increased or decreased)
for most of every day, and a significant change in function must P: Psychomotor agitation and retardation
exist. Changes can manifest as poor academic performance; school S: Suicidal ideation
attendance issues, including truancy and disruptive behaviors; and
difficulties with peer and familial relationships (Box 66.1). Criteria for Major Depressive Disorder
ww Depressed or irritable mood most of the day, nearly every day
ww Decreased interest in most daily activities, including school
Pathophysiology
ww Significant weight changes
The exact neurobiologic etiology of depression remains elusive. It is ww Sleep problems (insomnia or hypersomnia)
believed to involve impaired serotonin and norepinephrine trans- ww Psychomotor agitation or retardation
mission in critical areas of the brain, most notably the frontal lobes. ww Low energy or fatigue
Like other complex psychiatric conditions, the etiology of depression ww Feelings of worthlessness or guilt
seems to be multifactorial, with a strong genetic and psychosocial/ ww Diminished ability to concentrate or think
environmental basis. The genetic basis of depression is suggested ww Preoccupation with death or suicide
by statistics that indicate, for instance, that 25% of children who

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 468 PART 4: ADOLESCENT HEALTH

health conditions, such as early-onset bipolar disorder, can present

Box 66.4. What to Ask
initially with depressive symptoms. Approximately 20% to 40% of
children with MDD eventually develop bipolar disorder. In contrast, History of Depression in an Adolescent
longitudinal studies have found that very few individuals diagnosed ww Does the adolescent occasionally feel sad or “blue” and not under-
with bipolar disorder as children meet criteria for the condition as stand why?
adults. Another disorder to consider in children with depressive ww Does the adolescent have unexplained crying spells?
symptoms is disruptive mood dysregulation disorder, which was ww Does the adolescent feel “mad,”“bored,” or “grouchy”?
introduced in 2013 to identify those children with irritability and ww Does the adolescent seem inappropriately jovial?
persistent, prolonged tantrums. Additionally, children with autism ww Have any recent losses occurred in the adolescent’s life that may explain
spectrum disorder often develop comorbid depression during ado- his, her, or their feelings?
lescence as a manifestation of their isolation and poor coping skills. ww Is the adolescent having trouble with concentration or memory?
Other DSM-5 psychiatric diagnoses to consider include adjustment ww Does the adolescent have trouble falling asleep at night or have early
disorders, uncomplicated bereavement, separation anxiety, and morning awaking?
dysthymia, which is more chronic and sometimes less severe than ww Has the adolescent lost weight recently or shown any disinterest in food?
major depression. Finally, side effects of prescribed and over-the- ww Does the adolescent have any self-harm (eg, cutting) behaviors?
counter medications may produce clinical symptoms consistent ww Does the adolescent have feelings of hopelessness and have any desire
with depression and therefore should be considered in the differ- to cause self-harm? Has the adolescent made any previous suicide
ential diagnosis. attempts?
ww Do the parents or siblings have a history of drug or alcohol use?
Evaluation ww Is there a family history of affective disorders? What has been their
response to treatment?
History
ww Is there a history of family violence?
The assessment of an adolescent for depressive symptoms is an
important part of any routine encounter. At all visits a standardized
screening tool should be given to the patient to complete before
seeing the medical professional. These are short, quick assessments A full psychiatric assessment also should be completed to obtain
that provide an additional means by which a teenager can commu- information about a possible comorbid condition, including features
nicate depression or suicidality to the health professional before of psychosis, anxiety disorders, disruptive behaviors, and mania. It is
the start of the in-person encounter. Two such validated question- important to note, however, that severe depression may be accompa-
naires are the Patient Health Questionnaire-9 (PHQ-9), which is nied by symptoms of psychosis. Information about a family history
a self-report survey with 9 questions based on DSM-5, and the of psychiatric illness or substance abuse, including alcohol abuse,
Columbia-Suicide Severity Rating Scale. The PHQ-9 is composed also should be reviewed with the teenager as well as the parent(s)
of 9 questions with a score that ranges from 0 to 27. A score of 5 or guardian(s).
to 9 corresponds with mild depression, 10 to 14 with moderate To identify youth at risk for suicide, health professionals must
depression, 15 to 19 with moderately severe depression, and 20 inquire about specific areas of adolescents’ lives. Questions should
and above with severe depression. A positive result on either the be posed in an open and direct manner, such as, “Sometimes when
PHQ-9 or the Columbia-Suicide Severity Rating Scale warrants a people are very sad or upset they think of hurting themselves. Have
more comprehensive and complete conversation, especially if the you ever felt this way?”, not, “You don’t want to hurt yourself, right?”
screening tool for suicidality raises concern based on the validated The health professional also should probe for a history of or ongo-
scoring criteria. ing sexual or physical abuse, assault, violence, or neglect as well as
A diagnosis of depression may be difficult to make after just current interpersonal conflicts, such as fights, punishments, break-
1 interview. At the initial visit, along with use of a depression/ ups, or traumas. An early history of sexual abuse has a strong
suicide screening tool, a complete psychosocial or HEADSS assess- association with the manifestation of suicidal ideation by age 15 years.
ment (home, employment and education, activities, drugs, sexuality Feelings of hopelessness, agitation, and impulsivity should be iden-
[including a history of sexual abuse or assault], suicide/depression) tified, along with frequent thoughts of suicide or death. It has been
should be performed on all adolescents (see Chapter 4). The reported that having frequent thoughts of suicide is the best pre-
parent(s) or guardian(s) should be included during the first part of dictor of suicide attempts. Use of any illicit substance also raises the
the interview to review their observations and concerns about their risk for suicide attempts. The adolescent who is suspected of being at
adolescent’s behavior and mood, after which the teenager should be risk for suicide must be questioned directly about suicidal ideation,
interviewed alone and a thorough history of recent feelings, behav- specifically whether the teenager has a plan in mind and access to
iors, and attitudes should be obtained (Box 66.4). Mood changes firearms, medications, or other means of suicide. Previous suicide
should be noted; a labile affect can be a symptom of ongoing depres- attempts also must be reviewed with the teenager, because 35% to
sion. The physician should keep in mind that malnourishment can 45% of adolescents who complete suicide have a positive history of
result in a depressed or seemingly flat affect. a previous attempt. Nonlethal suicide gestures or other methods

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 Chapter 66: Depression and Suicide in Adolescents 469

of self-inflicted harm should never be taken lightly or minimized. in differentiating various psychiatric conditions (eg, bipolar disor-
Initial questions should be nonspecific and become more specific as der, schizophrenia).
the interview proceeds, especially if answers to previous questions
are positive (Box 66.5). Laboratory Tests
Promises to maintain confidentiality with the depressed adoles- Although no routine laboratory studies are regularly recom-
cent who is considered at risk for suicide are discouraged because mended, several laboratory tests warrant consideration in evaluating
parental or guardian involvement is strongly advised. Precipitating for physiologic contributions to depression. Such tests include a
and motivating factors for any previous suicide attempts should be thyroid panel, fasting blood glucose level, complete blood count,
determined before a treatment plan is developed. More important, electrolyte test, and urine or serum toxicology screening. Laboratory
the lethality of previous attempts must be evaluated. studies are also important screening measures prior to pharmaco-
therapy; thus, in addition to the aforementioned tests, the phy-
Physical Examination sician also should consider a blood urea nitrogen and creatinine
A thorough physical examination and review of systems should be test, liver panel, electrocardiography, and, in females, a pregnancy
completed to rule out a chronic medical condition, such as hypo- test. Psychometric testing may help rule out a concomitant learning
thyroidism, inflammatory bowel disease, lupus, or anemia, or an disability or attention-deficit/hyperactivity disorder.
organic etiology for nonspecific symptoms. For the patient with a
history of sexual abuse or assault or of sexual activity, a genital exam- Imaging Studies
ination should be performed to evaluate for sexually transmitted Radiologic imaging, such as computed tomography of the head or
infections, taking great care to avoid further trauma to the patient. In magnetic resonance imaging of the brain, is indicated if either the
most cases, the physical examination may be of little yield in the ado- history or the physical examination is suggestive of a CNS process.
lescent with a true affective disorder; however, careful examination
may reveal findings such as cut marks, track marks, skin picking, Management
or loss of tooth enamel, any of which may be helpful in diagnosing a
comorbid condition, such as substance abuse or an eating disorder.
Depression
A careful detailed neurologic examination, including a mental sta- Management of depression typically consists of psychotherapy, phar-
tus examination evaluating eye contact, rate of speech, spontaneity macotherapy, or a combination of both. The National Institute of
in conversation, thought content, affect, and processing, is essential Mental Health supports psychotherapeutic intervention for mild
depression. The types of therapy shown to be most effective for ado-
lescents with depression include cognitive behavioral therapy and
interpersonal therapy. For more severe depression or depression with
Box 66.5. What to Ask suicidal ideation, the current first-line medical treatment in the pri-
History of Risk for Suicide in the Adolescent mary care setting involves the use of selective serotonin reuptake
ww Is the adolescent on any prescribed medications (eg, isotretinoin)? inhibitors (SSRIs). Pharmacotherapy may be used with the afore-
ww Is the adolescent experiencing any psychiatric difficulties, social mentioned psychotherapies. Not all therapists are trained in the
maladjustments, or family or environmental challenges (eg, recent delivery of these therapeutic methods, however, and in many com-
parental divorce or separation, school expulsion)? munities these interventions are not readily available. When psycho-
ww Does the adolescent have a history of symptoms of depression, conduct therapy is not available or is not effective, SSRIs may be necessary.
problems, or psychosis? If depressive symptoms are associated with a specific adjustment
ww How is the adolescent progressing in school? disorder, such as divorce, a recent move, or death, and if family,
ww Does the adolescent have a history of substance abuse? peers, or school factors are affected, supportive counseling is indi-
ww Does the adolescent have any legal problems? cated. The duration and depth of counseling depends, in part, on
ww Does the adolescent suffer any social isolation or have interpersonal the comfort level of the primary care physician performing this task
conflicts with family or friends? and how receptive the adolescent and family are to this intervention.
ww Has the adolescent suffered any personal losses recently? Identification of the specific problem, exploration of the teenager’s
ww Has a suicide recently occurred in the school or community? response to the problem, and development of a reasonable solution
ww Are there any family problems, such as abuse or neglect? with the adolescent and parent(s) or guardian(s) may be helpful to
ww Has the adolescent ever thought that life was not worth living? improve adherence to psychotherapy. Regardless of the existence
ww Does the adolescent ever feel hopeless? of a clear trigger for a depressive episode, medication may still be
ww Has the adolescent ever thought of causing self-harm? necessary when the symptoms impair daily functioning.
ww Does the adolescent have a previous history of suicide attempts? If family difficulties or dysfunction is the major issue, the family,
ww Does the adolescent currently have a plan for suicide? adolescent, and physician or counselor should meet to assess the
ww Does the adolescent have access to firearms, medications, or other magnitude of the problem and the motivation required to address
means of suicide? it. The physician should use this opportunity to educate the adoles-
cent and the family about the signs and symptoms of depression and

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 470 PART 4: ADOLESCENT HEALTH

the significant effect of depression on school functioning, family worsening of symptoms, suicidal behavior, or unusual changes in
and peer relationships, and social interactions. Cognitive behavioral behavior. Families should be educated on the importance of close
therapy is a specific type of therapy that has been found to be par- follow-up and immediate, open communication with the physician
ticularly helpful in adolescents with depression. The need for indi- should these symptoms occur. Initially, the adolescent should be seen
vidual cognitive-behavioral or family therapy should be discussed or the family contacted frequently during the first 6 weeks of such
as well as the effectiveness of psychiatric medication in the appro- treatment. Improvements in vegetative functions, such as sleeping
priate setting. Attention to parental mental health and understand- and eating, often occur within the first 3 weeks. Family observations
ing the strategies necessary to manage the adolescent’s irritability are initially more telling than self-observation. Often, the last feature
and isolation also are extremely important. Psychological referral to improve is the patient’s self-report of mood elevation. An adequate
should be initiated if the patient requires more prolonged or inten- trial of SSRIs is reported to be at least 4 to 6 weeks. Frequent medica-
sive psychotherapeutic treatment, the severity of depression seems tion adjustments are not advised, and abruptly stopping SSRIs is not
to worsen, suicidal behavior becomes an issue and additional men- recommended because of the possibility for a withdrawal syndrome.
tal health consultation is necessary, or a comorbid psychiatric con- Stopping medication after several weeks should include a slow taper.
dition is suspected.
Immediate psychiatric consultation and referral are indicated Suicide and Suicidal Behavior
if an adolescent has severe depressive features that interfere with Adolescents who are considered at risk for suicide must be asked
daily functioning or if the patient is experiencing suicidal intent, directly at every visit if they are suicidal and if they have a plan
homicidal intent, or psychosis. Ensuring the adolescent’s safety is (Box 66.5). Past suicide attempts are the most robust predictor of a
the priority. A mental health referral is also appropriate if support- future suicide attempt. Inquiry should include probes for thoughts of
ive counseling by the primary care physician has been ineffective or death, suicidal ideation, plan for suicide, means available, and intent.
in cases of recurrent or chronic depression. The teenager should be interviewed alone in an empathic and open-
Psychiatric intervention generally includes pharmacotherapy ended fashion. The parent or guardian also should be interviewed
in conjunction with psychotherapy, because most cases of depres- separately. Positive responses to this inquiry determine whether
sion include psychological, social, and environmental components. the adolescent will be treated on an inpatient or outpatient basis.
Although much attention has been given to safety concerns about The health professional should assess for protective factors as well
the use of antidepressant medications among children and adoles- (Box 66.6).
cents, SSRIs are considered first-line medications for the manage- A suicide risk assessment and triage resource should be used,
ment of moderate to severe depression in teenagers. Two SSRIs, such as the SAFE-T suicide risk assessment (Table 66.1). In most
fluoxetine hydrochloride (eg, Prozac, Sarafem) and escitalopram cases, the adolescent with no previous suicide attempts, who exhib-
oxalate (eg, Lexapro), have been approved by the US Food and Drug its ambivalence about suicidal thoughts, with no real intent to die,
Administration (FDA) for managing depression in adolescents, but and with a good family support system may be treated as an outpa-
several other antidepressants are commonly prescribed in an off- tient. A safety plan must be devised, however. The patient is asked
label manner. Side effects of this class of medication tend to be dose to agree to contact the clinician, parent, or another responsible adult
related, and most subside with time (1–2 weeks) or with dose reduc- if the patient feels a suicidal urge or experiences suicidal intent. The
tion. Common adverse effects include headache, abdominal pain, precipitants for possible suicidal behavior must be reviewed, and
diarrhea, sleep changes, and jitteriness or agitation. Serious behav- alternative methods for coping should be rehearsed with the teen-
ioral symptoms, such as aggression, hostility, and impulsivity, must ager. Additionally, all potential means of suicide, particularly fire-
be reviewed with a psychiatrist. arms and toxic medications, must be removed from the home or
In 2004, the FDA added a black box warning for antidepres- place of residence. It is not enough to “secure” firearms; they must
sant medications stating that on rare occasions children and ado-
lescents treated with these drugs have an increased likelihood for
displaying suicidal behavior; however, no increase in the risk of com- Box 66.6. Protective Factors
pleted suicides was noted in a meta-analysis conducted by the FDA. ww Intact reality testing
Subsequently, physicians wrote fewer prescriptions for antidepres- ww Children in home
sants, which resulted in an increased rate of completed suicides. ww Spiritual beliefs and/or practices
It now is generally accepted that although a small risk of suicidal ww Moral beliefs
behavior may exist, as long as patients are appropriately monitored ww Social stigma
the benefits of prescribing antidepressants outweigh the risks. This ww Future-oriented thought
caution should be presented in context with the risks of untreated ww Presence of positive social relationships
depression and discussed openly with the parent or guardian and ww Fear of death and/or suicide
the adolescent. Before starting the medication, informed consent ww Problem-solving skills
must be obtained from the parent or guardian and assent must be ww Goals and/or aspirations
attained from the teenager. Patients should be observed closely for

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 Chapter 66: Depression and Suicide in Adolescents 471

Table 66.1. Suicide Threat Assessment

Risk Level Risk/Protective Factor Suicidality Possible Interventions
High Psychiatric disorders with severe symptoms, Potentially lethal suicide attempt Admission generally indicated unless a
or acute precipitating event; protective factors or persistent ideation with strong significant change reduces risk. Suicide
not relevant intent or suicide rehearsal precautions
Moderate Multiple risk factors, few protective factors Suicidal ideation with plan, but no Admission may be necessary depending
intent or behavior on risk factors. Develop crisis plan. Give
emergency/crisis numbers
Low Modifiable risk factors, strong protective Thoughts of death, no plan, intent Outpatient referral, symptom reduction. Give
factors or behavior emergency/crisis numbers
Reprinted with permission from Substance Abuse and Mental Health Services Administration. Suicide Assessment Five-step Evaluation and Triage (SAFE-T). Rockville, MD: Substance Abuse and Mental
Health Services Administration; 2009. www.integration.samhsa.gov/images/res/SAFE_T.pdf

be removed. Increased supervision must be implemented by par- another depressive episode within 5 years. Additionally, youth with a
ents or guardian as well as peers. Random room checks must be depressive disorder have a 4-fold risk for experiencing the same dis-
agreed on as well as an open-door policy until the therapist clears the order as an adult. Prepubertal-onset depression is associated with an
patient for more privacy. The family also must agree to find a thera- approximately 30% risk for future bipolar disorder or mania.
pist and begin treatment immediately. An emergency plan must be The risk of repeated suicidal behavior seems to be greatest within
developed, including “permission” to call 911. Resources should be the first 3 months after the initial attempt. Reported reattempt rates
provided. A crisis prevention card might be created that includes are 6% to 15% in the first 1 to 3 years after the initial attempt.
items such as identification of common triggers, an outline of cop-
ing skills, identification of a support system complete with telephone
numbers, and the therapist’s telephone number as well as a suicide CASE RESOLUTION
hotline telephone number. Referral to a therapist is recommended The girl’s symptoms may be indicative of depression, because she has a flat affect
as soon as possible. Preferably, this should be arranged while the and seems to be somewhat isolated (ie, insufficient time for friends, recent move
adolescent is in the office, and the patient should be given a defi- to the United States). After much inquiry, she seems to be at low risk for suicide;
nite time and date for the appointment. Ideally, the therapist should however, her PHQ-9 score of 11 indicates that she is at moderate risk of depres-
sion. The physician should continue to inquire about symptoms of depression and
meet with the family before the first appointment with the adoles- ask her directly about suicidal behaviors, then arrange for cognitive behavioral
cent alone. Detoxification from drugs or alcohol, if necessary, also therapy and close follow up. If depression is confirmed and does not improve
should be addressed with the family and teenager. with therapy, medication may be indicated. If the girl becomes suicidal, she and
Because all suicidal threats, gestures, or ideations by adolescents her family should be referred to an emergency department for an emergent men-
must be taken seriously, emergent psychiatric referral is required for tal health evaluation and possible intervention, including hospital admission.
most of these individuals. The adolescent deemed to be at serious
risk for suicide should be treated as an inpatient and admitted to a
pediatric or adolescent unit for 72 hours of observation. The purpose Resources
of this brief hospitalization is 3-fold: to stabilize the patient medi- Columbia-Suicide Severity Rating Scale
cally, if necessary; to observe and evaluate patient-family dynamics; http://cssrs.columbia.edu
and to impress on the patient and family that the attempt has been
Military OneSource
recognized and taken seriously. Intervention requires the involve-
www.militaryonesource.mil
ment of mental health professionals and social services. Referral to
the emergency department for any patient considered to be of mod- National Suicide Prevention Lifeline
erate to severe risk or anyone who concerns the health professional is 800-273-TALK (8255)
crucial. It is not the role of the primary care physician to clear some-
Safety Planning Intervention
one of the need for emergent psychiatric intervention.
www.suicidesafetyplan.com

Prognosis Substance Abuse and Mental Health Services Administration

(SAMHSA)
The risk of recurrence of major depression in adolescents who have
www.samhsa.gov
recovered is substantial. One study reports that 5% of patients relapse
within 6 months of recovery, 12% within 1 year, and an estimated 33% Garrett Lee Smith State/Tribal Youth Suicide Prevention
within 4 years. Higher rates of recurrence have been reported, how- Program and Early Intervention Grant Program
ever, with approximately 70% of teenagers with MDD experiencing www.samhsa.gov/grants/grant-announcements/sm-19-006

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 472 PART 4: ADOLESCENT HEALTH

Suicide Prevention Resource Center Hammad TA, Laughren T, Racoosin J. Suicidality in pediatric patients treated
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16520440 https://doi.org/10.1001/archpsyc.63.3.332
American Indian and Alaska Native suicide prevention programs Kann L, McManus T, Harris WA, et al. Youth risk behavior surveillance—United
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doi.org/10.15585/mmwr.ss6708a1
CALM: Counseling on Access to Lethal Means
www.sprc.org/resources-programs/calm-counseling-access-lethal- Leslie LK, Newman TB, Chesney PJ, Perrin JM. The Food and Drug
Administration’s deliberations on antidepressant use in pediatric patients.
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Pediatrics. 2005;116(1):195–204 PMID: 15995053 https://doi.org/10.1542/
Safety Planning Guide: A Quick Guide for Clinicians peds.2005-0074
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20Guide%20for%20Clinicians.pdf Depression Study (TADS): long-term effectiveness and safety outcomes.
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Zero Suicide in Health and Behavioral Health Care org/10.1001/archpsyc.64.10.1132
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 PART 5

Acute and Emergent

Problems
67. Fever and Bacteremia.......................................................475
68. Emerging Infectious Diseases..........................................483
69. Febrile Seizures.................................................................495
70. Respiratory Distress..........................................................501
71. Stridor and Croup.............................................................507
72. Sudden Unexpected Infant Death and Brief
Resolved Unexplained Events..........................................513
73. Syncope..............................................................................521
74. Shock.................................................................................527
75. Approach to the Traumatized Child................................537
76. Abdominal Trauma...........................................................543
77. Acute Abdomen (Appendicitis).......................................549
78. Head Trauma.....................................................................555
79. Increased Intracranial Pressure.......................................563
80. Management of Dehydration in Children:
Fluid and Electrolyte Therapy..........................................571
81. Acute Kidney Injury.........................................................583
82. Ingestions: Diagnosis and Management.........................591
83. Disaster Preparedness......................................................599

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BPPCA6e_Ch067_0473-0482.indd 474 2/14/20 10:43 AM
 CHAPTER 67

Fever and Bacteremia

Eric R. Schmitt, MD, MPH, FACEP, FAAP

CASE STUDY
An 8-month-old girl is brought to the emergency depart- Questions
ment with a 2-day history of fever and increased fussi- 1. What are the serious bacterial infections in febrile
ness. She is irritable but consolable by her parents. Her newborns and infants?
parents believe that her immunizations are current, but 2. What has been the effect of conjugated vaccines
they do not have the immunization record with them. against Haemophilus influenzae and Streptococcus
On examination, she has a rectal temperature of 39.5°C pneumoniae on the incidence of bacteremia and
(103.1°F). The rest of the physical examination is within meningitis in febrile newborns and infants?
normal limits, and no source for the fever is apparent. 3. What are the challenges in differentiating between
serious and benign febrile illnesses in young children?
4. What diagnostic studies are recommended in the
evaluation of febrile newborns, infants, and children?
5. When are empiric antibiotics indicated, and when
should febrile newborns and infants be hospitalized?

Fever is among the most common chief complaints among pediatric SBI among febrile newborns and infants younger than 90 days with
patients seeking medical attention in physician offices, urgent care a temperature of 38.0°C (100.4°F) or higher is approximately 10%;
centers, and emergency departments and accounts for up to 30% the rate approaches 20% in newborns younger than 28 days. Urinary
of these visits. Most such patients have a benign, self-limited viral tract infections are by far the most common source of SBI, with a
illness. Some patients, however, have a serious bacterial infection smaller percentage having pneumonia, bacteremia, or meningitis.
(SBI), such as meningitis, urinary tract infection (UTI) and/or Contemporary studies have demonstrated that febrile newborns and
pyelonephritis, pneumonia, bacteremia, septic arthritis, osteomyelitis, young infants with diagnosed viral infections have lower rates of SBI
cellulitis, or deep tissue infection. Bacteremia is a bacterial infec- than those without viral infections (4% and 12%, respectively). In
tion within the bloodstream; it is considered occult in the absence these studies, none of the febrile newborns and young infants with
of an apparent source of infection after a thorough physical exami- viral infections confirmed on diagnostic testing had meningitis, but
nation in an otherwise healthy-appearing child. some did have UTIs and, rarely, bacteremia.
Febrile infants and children age 3 to 36 months are at a higher risk
Epidemiology for bacteremia than older children but less so than newborns and
Historically, management decisions about febrile children have been young infants. Although the physical examination is more reliable
largely dictated by age. Patients are typically divided into the fol- in this age group than in newborns and younger infants, in many
lowing age-defined categories: newborns and infants younger than patients the examination is normal without any localizing source of
90 days, infants and young children 3 to 36 months of age, and chil- infection. These individuals may, in turn, have occult bacteremia.
dren age 3 years and older. Febrile newborns and young infants Vaccine development and widespread immunization programs have
(ie, younger than 90 days) have higher rates of SBI than older chil- dramatically changed the epidemiology and clinical course of bac-
dren and often represent a diagnostic challenge. They have relatively teremia in this age group within the United States over the past sev-
immature immune systems, which renders them particularly sus- eral decades. Before the introduction of the Haemophilus influenzae
ceptible to bacterial infections and have not yet received most of type b (Hib) and pneumococcal vaccines, the prevalence rates of bac-
their immunizations. They often have limited responses to bacterial teremia were approximately 3%. During this time, Haemophilus
infections and exhibit relatively nonspecific signs and symptoms. In was considered the most significant organism causing bacteremia
addition, newborns and young infants have different bacterial patho- because of its invasiveness and ability to cause localized infection,
gens that can cause these serious infections, including Escherichia particularly meningitis. In the mid-1980s, the Hib vaccine was intro-
coli; Streptococcus agalactiae (group B streptococci); less commonly, duced, which has nearly eliminated this particularly invasive organ-
Streptococcus pneumoniae; and, rarely, Listeria monocytogenes and ism. In the post-Hib but prepneumococcal conjugate vaccine era, the
other gram-negative organisms (Box 67.1). The overall prevalence of rates of occult bacteremia ranged from 1.6% to 1.9% in children with a

475

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 476 PART 5: ACUTE AND EMERGENT PROBLEMS

be given at 2, 4, 6, and 12 to 15 months of age. A pneumococcal poly-

Box 67.1. Organisms Implicated
saccharide vaccine (PPSV23) was approved in late 2014 that provides
in Serious Bacterial Infection/Occult
even broader protection. The addition of PPSV23 is recommended
Bacteremia in Children
for children older than 2 years with certain high-risk chronic medi-
Newborns and Infants Age 3 Months and Younger cal conditions after they have received PCV13. Continued surveillance
ww Escherichia coli of invasive disease is necessary to ascertain what effect the changes
ww Group B streptococci in epidemiology of these newer vaccines may have on the diagnosis
ww Streptococcus pneumoniae and management of bacteremia in young febrile children. Preliminary
ww Listeria monocytogenes data have shown a 42% decrease overall in the incidence of invasive
ww Salmonella species (infants >1 month) pneumococcal infection in 2011 after the implementation of routine
ww Haemophilus influenzae type b (Hiba; infants >1 month) PCV13 immunization and a 53% decrease in the incidence in children
Children Age 3–36 Months younger than 24 months compared with years 2007 through 2009.
ww S pneumoniae
ww Neisseria meningitidis
Clinical Presentation
ww Salmonella species In many children, fever is the only symptom or manifestation of dis-
ww Staphylococcus aureus ease and no other signs or symptoms may be apparent. These chil-
ww Hiba dren may look well and behave normally. Young children, especially
newborns and infants younger than 3 months, are likely to have fewer
a
Hib disease has been nearly eliminated with the routine use of Hib conjugate vaccines. and more subtle behavioral signs with bacterial infections. Physicians
must, therefore, maintain a high index of suspicion for the presence
temperature of 39.0°C (102.2°F) or higher and no obvious source of an SBI, even in the absence of localizing signs (Box 67.2). Occult
of infection. More than 90% of cases of occult bacteremia in this bacteremia, by definition, has no abnormal physical manifestations
age group were caused by S pneumoniae, with the remainder being aside from fever.
caused by Salmonella, Neisseria meningitides, Staphylococcus aureus,
and a few other rare organisms. Pneumococcus is not as virulent a Pathophysiology
microorganism as some other bacteria, and many cases of occult Fever is an elevation in the thermoregulatory set point of the body.
pneumococcal bacteremia resolved spontaneously without any The thermoregulatory center is located in the preoptic region of
intervention. Left untreated, a small percentage (3%–5%) went on the anterior hypothalamus, and an elevation in the hypothalamic
to develop pneumococcal meningitis, which has the most serious set point above the normal body temperature initiates the physio-
complication and fatality rate. In 2000, the heptavalent pneumo- logic changes that result in fever. Exogenous pyrogens (eg, bacteria,
coccal conjugate vaccine was licensed by the US Food and Drug viruses, antigen-antibody complexes) stimulate host inflamma-
Administration for use within the United States. This vaccine pro- tory cells (eg, macrophages, polymorphonuclear cells) to produce
vided coverage against the 7 main serotypes of S pneumoniae, which endogenous pyrogens. Interleukin-1 is currently regarded as the
were responsible for approximately 80% of the cases of invasive prototypical endogenous pyrogen. Endogenous pyrogens cause the
pneumococcal disease in the United States and Canada at the time. hypothalamic endothelium to increase intermediary substances,
Following the introduction of this vaccine, the rate of invasive pneu- such as prostaglandins and neurotransmitters, which then act on
mococcal disease (including bacteremia) and carriage for the sero- the preoptic neurons of the anterior hypothalamus to produce an
types covered by the vaccine dropped considerably. Additionally, elevated set point. The body uses physiologic mechanisms (eg,
there was evidence suggestive of herd immunity, in that a decline in peripheral vasoconstriction, shivering) and behavioral actions
invasive pneumococcal disease was noted among older individuals
who had not received the vaccine. Consequently, a selective pressure
has increased the prevalence of invasive pneumococcal disease caused Box 67.2. Diagnosis of Serious Bacterial
by strains not covered by the heptavalent vaccine, although the overall Illness in Children
magnitude of this effect appears to be relatively small. Additionally, ww Lethargy, irritability, or change in mental status
other bacteria, such as E coli, Salmonella, and S aureus, appear to have ww Tachycardia disproportionate to the degree of temperature elevation
increased in relative frequency as a source of bacteremia. In 2010, a ww Tachypnea or labored respirations
new 13-valent pneumococcal conjugate vaccine (PCV13) was licensed ww Bulging or depressed anterior fontanel
and expanded coverage to include 6 different serotypes that have ww Nuchal rigidity
emerged with increasing frequency as a cause of invasive pneumo- ww Petechiae
coccal disease following the introduction of the heptavalent vaccine. ww Localized erythema, tenderness, or swelling
The American Academy of Pediatrics and the Advisory Committee ww Abdominal or flank tenderness
on Immunization Practices currently recommends the pneumococ- ww Fever
cal conjugate vaccine (ie, PCV13) for all infants in a 4-dose regimen to

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 Chapter 67: Fever and Bacteremia 477

(eg, bundling up, drinking hot tea) to increase body temperature The differential diagnosis of children with an acute febrile
to reach and maintain this higher set point, thus producing fever illness is primarily infectious (Box 67.4), including benign and
(Figure 67.1). generally self-limited illnesses (eg, upper respiratory infections)
This contrasts with hyperthermia, in which the thermoregula- and less common but more serious illnesses (eg, meningitis, osteo-
tory set point of the body is normal. Because of abnormal physiologic myelitis). Occasionally, a child with a fever without a source has a
processes, heat gain exceeds heat loss, and the body temperature
rises despite efforts to return to the control set point.
Box 67.3. Risk Factors for Occult Bacteremia

Differential Diagnosis ww Age 36 months or younger

ww Temperature ≥39.5°C (≥103.1°F)
In most cases, the duration of fever in children is short, and signs
ww White blood cell count ≥15,000 cells/mm3 or ≤5,000 cells/mm3
and symptoms are localized. Fever without a source involves an
ww Total band cells ≥1,500 cells/mm3
acute episode of fever that lasts 1 week or less in children in whom
ww Erythrocyte sedimentation rate ≥30 mm/hour
history, physical examination, and laboratory tests do not reveal
ww Underlying chronic disease (eg, malignancy, immunodeficiency, sickle
a source. Most affected children are eventually diagnosed with
cell disease, malnutrition)
an acute, generally benign, infectious illness. Occult bacteremia
ww Clinical appearance (eg, irritability, lethargy, toxic appearance)
remains a major concern in young children, primarily in infants
younger than 3 months. High-risk factors for occult bacteremia
are presented in Box 67.3. Fever of unknown origin is fever of
at least 8 days’ duration in infants or children in whom routine Box 67.4. Common Infectious Causes of an Acute
history, physical examination, and laboratory assessment do not Episode of Fever in Children
reveal a source.
Upper Respiratory Tract
ww Upper respiratory infection (ie, common cold)
ww Otitis media
ww Sinusitis
Exogenous Pyrogens
(eg, bacteria, viruses, antigen-antibody complexes) Pulmonary
ww Bronchiolitis
ww Pneumonia
Host Inflammatory Cells
(eg, macrophages, neutrophils, Kupffer cells, Oral Cavity
splenic and alveolar macrophages) ww Gingivostomatitis
ww Pharyngitis
ww Dental abscess
Endogenous Pyrogens Produced
(interleukin–1, interleukin–6, tumor necrosis factor, Gastrointestinal Tract
and other pyrogenic cytokines)
ww Acute gastroenteritis (bacterial or viral)
ww Appendicitis
Synthesis of PGE2 in Hypothalamus Genitourinary Tract
ww Urinary tract infection
ww Pyelonephritis
Thermoregulatory Center
(preoptic neurons of anterior hypothalamus) Musculoskeletal
ww Septic arthritis
ww Osteomyelitis
Elevation of Thermostat “Set Point”
Central Nervous System
ww Meningitis
Physiologic and Behavioral Changes ww Encephalitis
Miscellaneous (Including Noninfectious Causes)
ww Bacteremia
Fever
ww Immunization reaction
ww Viral exanthems (eg, chickenpox, measles)
ww Neoplasia
Figure 67.1. Pathophysiology of fever production. Antipyretic agents work by ww Collagen vascular disease
blocking the synthesis of prostaglandin E2 (PGE2).

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 478 PART 5: ACUTE AND EMERGENT PROBLEMS

noninfectious illness, such as collagen vascular disease or neopla-

Box 67.6. What to Ask
sia. By comparison, the differential diagnosis of children with fever
of unknown origin is quite broad and includes infectious and non- Fever and Bacteremia
infectious disorders (Box 67.5). ww How long has the child had fever? How high has the temperature been?
ww Does the child have any other symptoms, such as rash, vomiting, diar-
Evaluation rhea, abdominal pain, or dysuria; cough, rhinorrhea, or other respiratory
History symptoms; lethargy, irritability, or change in mental status?
ww Is anyone sick at home?
The medical history provides a great deal of valuable informa-
ww Has the child’s activity level changed (eg, more sleepy than usual), or is
tion in the evaluation of children with fever (Box 67.6). The his-
the child more irritable than usual?
tory should focus on the duration and severity of the fever as
ww What immunizations has the child received?
ww Is the child taking any medications?
ww Do any pets live in the house?
Box 67.5. Common Causes of Fever of Unknown ww Has there been any history of recent travel, especially outside the
Origin in Children country?
ww Has the child ever been hospitalized for an infectious illness?
Infectious Diseases ww Does the child have any medical problems, especially asthma, sickle cell
Bacterial anemia, congenital heart disease, or immunodeficiency?
ww Localized infection: mastoiditis, sinusitis, pneumonia, osteomyelitis,
pyelonephritis, abscess (eg, abdominal, pelvic)
ww Systemic: tuberculosis, brucellosis, salmonellosis, leptospirosis,
well as the presence of associated symptoms that may localize
tularemia
an infection to a specific organ system. Additionally, caregivers
Viral should be specifically asked about infectious risk factors, such as
ww Hepatitis viruses potential sick contacts, immunization status, and chronic med-
ww Cytomegalovirus ical conditions.
ww Epstein-Barr virus (infectious mononucleosis)
ww HIV Physical Examination
Rectal temperature should be obtained in all newborns, infants, and
Fungal
young children. Temperatures obtained by other routes (eg, axillary,
ww Disseminated coccidioidomycosis
oral, cutaneous) are less reliable. An elevation in rectal tempera-
ww Disseminated histoplasmosis
ture in a newborn or an infant should not be attributed to over-
Miscellaneous bundling. Other vital signs may also provide important diagnostic
ww Malaria clues. Tachycardia disproportionate to the degree of temperature ele-
ww Rocky Mountain spotted fever vation may be suggestive of dehydration or sepsis. Tachypnea may
ww Syphilis be the only sign of a respiratory infection, and it also can occur in
ww Lyme disease response to metabolic acidosis (eg, secondary to sepsis or shock).
Neoplasia These changes may suggest an occult focus of infection. Response of
ww Leukemia the temperature and other vital signs to antipyretic agents should be
ww Lymphoma noted; the physician must remain cautious, however, because a clin-
ww Hodgkin disease ical improvement in response to antipyretic agents can occur even
ww Neuroblastoma in the setting of an SBI. Newborns and young infants with recorded
fevers at home but who are afebrile on physician evaluation should
Collagen Vascular Diseases be treated as if they have a fever. The report of a tactile fever with-
ww Juvenile idiopathic arthritis out a recorded temperature is sensitive, but not specific, and there-
ww Systemic lupus erythematosus fore overestimates the true likelihood of a fever. In newborns and
ww Rheumatic fever young infants in whom the risk of SBI is relatively high, however,
Miscellaneous the report of a tactile temperature should be taken seriously,
ww Inflammatory bowel diseases and these patients should be managed as though the fever were
ww Kawasaki disease documented.
ww Thyroiditis Observation of the overall hydration status and activity of the
ww Drugs patient is extremely important. An attempt should be made to
ww Factitious fever determine whether the patient is behaving and responding in an
age-appropriate fashion. Physicians should look for eye contact,

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 Chapter 67: Fever and Bacteremia 479

spontaneous motor movements, negative responses to adverse stim- should not be based on the screening peripheral WBC count in this
uli, and positive responses to pleasant stimuli. age group. Additionally, the standard urinalysis has a sensitivity of
All febrile children should undergo a complete physical exam- only approximately 85% in this age group and should not be used
ination. This is important even when the history may suggest to determine the need for urine culture.
involvement of only 1 organ system. For example, in young chil- Rapid viral diagnostic techniques that can reliably identify sev-
dren vomiting and fever may be signs of a viral illness, but they also eral of the more common viral pathogens (eg, respiratory syncytial
may signal a more serious infection such as a UTI or meningitis. virus, influenza, adenovirus, parainfluenza) are becoming increas-
The underlying condition may go undiagnosed unless a thorough ingly available. The presence of a positive viral test result, how-
examination and appropriate diagnostic evaluation are performed. ever, does not automatically preclude further diagnostic testing in
The anterior fontanel should be palpated. It may be normal, bulg- this age group. Studies have identified a small but significant num-
ing as a result of CNS infection, or depressed secondary to dehy- ber of patients with UTIs who also happen to have a positive viral
dration. The ears should be examined carefully and pneumatic test result, and for this reason, urine culture should still be rou-
otoscopy performed to evaluate for otitis media as the source of tinely obtained. The risk of bacteremia and meningitis is signifi-
fever, especially in children younger than 3 years. The occurrence cantly decreased, however, and in well-appearing patients older than
of otitis media should not preclude further workup for invasive bac- 1 month with a positive viral test result further testing may not be
terial disease in children who do not appear well (see Chapter 87). indicated or cost effective.
The oropharynx also should be examined. Dry mucous membranes If empiric antibiotics are to be administered, a lumbar puncture
may indicate dehydration. Enlarged, inflamed, or exudative tonsils must be performed so as not to obscure the possibility of partially
may signal the presence of a viral infection or group A streptococcal treated meningitis should pleocytosis be discovered on a subsequent
infection in older children. Respiratory symptoms, such as retrac- cerebrospinal fluid specimen. Stool analysis and culture should be
tions, nasal flaring, grunting, stridor, rales, rhonchi, and wheezing, reserved for febrile newborns and young infants with diarrhea.
may all be clues to respiratory tract infections. Enanthems on the Routine diagnostic radiographic studies (eg, chest) are not neces-
buccal mucosa or exanthems on the skin are often signs of viral sary and should be reserved for infants with respiratory symptoms
infections. The presence of petechiae in association with fever is or examination findings (eg, tachypnea, hypoxia, rales, wheezes,
usually benign; in rare cases, however, it may indicate a serious increased work of breathing).
underlying infection, such as meningococcemia. The capillary refill
time, quality of peripheral pulses, and the general temperature of the Infants 3 to 36 Months of Age
extremities can be used to assess perfusion. Localized areas of ten- The diagnostic approach to older infants and young children has
derness, erythema, swelling, induration, or fluctuation may point to changed following the introduction of pneumococcal conjugate vac-
cellulitis, septic arthritis, osteomyelitis, or the presence of an abscess. cines. Before widespread vaccine use, the standard of care involved an
Nuchal rigidity can be an important clue to the presence of menin- aggressive diagnostic approach looking for occult bacteremia in febrile
gitis. This clinical finding is rarely present in children younger than children 3 to 24 months of age with temperature higher than 39.0°C
15 to 18 months, and physicians must rely on other clinical factors (102.2°F) and in febrile children 2 to 3 years of age with temperature
and maintain an index of suspicion for meningitis in febrile chil- higher than 39.5°C (103.1°F) without any apparent source of infection.
dren of this age. Newborns and infants with meningitis may display This historical approach included a screening CBC, blood cultures, and
paradoxical irritability, which is when crying is made worse by hold- empirically treating infants and children with an elevated WBC count
ing and trying to console the child. greater than 15,000 cells/mm3 or an absolute neutrophil count (ANC)
greater than 10,000 cells/mm3 because they were at higher risk for
Laboratory Tests occult bacteremia. The ANC was generally considered to be the best
Newborns and Infants 90 Days or Younger predictor of risk for occult bacteremia. More recently, other acute phase
The physical examination alone cannot reliably identify an SBI in reactants, such as erythrocyte sedimentation rate, C-reactive protein,
newborns and infants 90 days or younger. For all patients with a and procalcitonin, have been studied. These levels are all commonly
temperature of 38.0°C (100.4°F) or higher, a thorough evaluation elevated in serious infections but have inadequate sensitivity and spec-
for a bacterial source of infection is therefore required. This evalu- ificity and have not been shown to be reliably better predictors for
ation includes a complete blood cell count (CBC) with differential, occult bacteremia than the peripheral WBC count or ANC. At this time
urinalysis with microscopic evaluation, and blood and urine cul- other laboratory tests, including antigen testing, serum cytokine mea-
tures. Cerebrospinal fluid studies and cultures should be performed surements, and polymerase chain reaction (quantifying the patient’s
on all newborns and infants younger than 29 days and strongly con- molecular response to infection), are not particularly useful because
sidered for those aged between 29 to 90 days. Peripheral white blood of their limited availability, relatively high false-positive rates, or cost.
cell (WBC) counts, although possibly helpful in older infants and However, there is significant interest and ongoing study of these tech-
children, do not reliably predict UTIs, bacteremia, or meningitis nologies and it is probable that they will be part of patient care in the
in febrile newborns and young infants. Decisions about whether future, but currently there is no single test that has reliably identified
to send blood and urine cultures or to perform a lumbar puncture all young febrile children with occult bacteremia.

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 480 PART 5: ACUTE AND EMERGENT PROBLEMS

With the declining prevalence of invasive pneumococcal disease Management

postlicensure of the heptavalent conjugate vaccine and the further
Management of children with fever includes controlling the fever
decrease with PCV13, the need for routine screening, culturing, and
and managing the underlying process causing the fever. No evidence
selective antibiotic use for occult bacteremia has been challenged.
exists that fever itself is harmful. To the contrary, animal studies have
The peripheral WBC count has become a less useful screening tool in
suggested that fever may have some survival advantage. Despite the
an era of such low rates of invasive disease. Additionally, the rates of
possible beneficial effects of fever, febrile children may feel uncom-
contaminated blood cultures are now much higher than actual cases
fortable, and fever should be reduced to relieve the associated dis-
of bacteremia, which often results in unnecessary additional testing,
comfort and malaise. Antipyretic agents, such as acetaminophen and
hospitalization, antibiotic administration, and family stress. Infants
ibuprofen, can be used. The use of both of these medications concur-
and children who have received at least 2 doses of the PCV13 vaccine
rently can result in dosing errors; therefore, in most cases, families
can be safely managed without blood tests, because this is no longer a
should be advised to use only a single medication. Aspirin should not
cost-effective strategy. Individuals who are at increased risk because
be used for fever control in children because of the association with
they are unimmunized, whose vaccine status is uncertain, or who
Reye syndrome and viral illnesses. Sponging or bathing with tepid
have received only 1 dose of the vaccine may need screening evalua-
water and unbundling children aid in fever reduction. Ice water or
tion with CBC, blood, and urine cultures and expectant antibiotics if
alcohol baths should be avoided to prevent inadvertent hypothermia.
they are found to have an elevated WBC count or ANC. The increase
All toxic-appearing febrile newborns, infants, and children,
in herd immunity and change in epidemiology of bacteremia follow-
regardless of age, require hospitalization and administration of
ing the widespread use of the pneumococcal vaccine may warrant
broad-spectrum antibiotics. Hospitalization and the initiation of
reconsideration of this in the future following long-term surveillance
empiric intravenous (IV) antibiotics are recommended for all febrile
of invasive disease.
newborns 28 days or younger pending culture results. Ampicillin
Infants with temperature higher than 39.0°C (102.2°F) warrant
and gentamicin are the most commonly used initial antibiot-
urine testing, especially in girls younger than 2 years, uncircumcised
ics within this age group; however, use of these antibiotics must
boys younger than 12 months, and circumcised boys younger than
be considered carefully in the context of resistance patterns and
6 months. Chest radiography should be considered in infants with
local practice within a geographic region. With the onset of stan-
significant respiratory symptoms or auscultatory findings sugges-
dard group-B streptococcus surveillance during pregnancy and the
tive of pneumonia. In infants with temperatures higher than 39.5°C
use of intrapartum antibiotics, more gram-negative organisms and
(103.1°F) and WBC counts greater than 20,000 cells/mm3, chest
increased gentamicin resistance have been reported, necessitating
radiographs should also be obtained to detect occult pneumonia,
the use of a third-generation cephalosporin, such as cefotaxime,
which is reported in up to 25% of these patients, even in the absence of
for initial empiric coverage. Ceftriaxone is generally avoided in this
significant respiratory symptoms or auscultatory findings.
age group, particularly among jaundiced newborns, because of its
Children Older Than 3 Years of Age ability to displace bilirubin from albumin, thereby increasing the
The laboratory evaluation of children older than 3 years is individu- risk of kernicterus. The addition of empiric vancomycin and/or
alized and influenced by the history and physical examination; most acyclovir should be considered for patients at risk for resistant gram-
patients do not need any testing. Healthy children in this age group positive organisms or congenital Herpes simplex infection.
are not at high risk for occult bacteremia. The physical examination Well-appearing febrile infants aged 29 to 90 days may be treated
of children this age is more reliable, and they are better able to com- on an outpatient basis with antibiotics (ceftriaxone 50 mg/kg intra-
municate their symptoms than younger children. Routine screening venously or intramuscularly) or close observation alone, provided
tests are not generally indicated in healthy individuals and should they meet established low risk for SBI criteria (Box 67.7). For this
be reserved for those who appear toxic or have an underlying dis- approach to be safe, however, parents or guardians must be reliable
ease that puts them at increased risk for bacterial infections (eg, and have means of communication and transportation in the event
sickle cell disease, cancer, immunodeficiency, nephrotic syndrome). of a positive culture result so that they can be notified to return the
infant for reevaluation and possible admission. All febrile infants
Imaging Studies aged 29 to 90 days need very close follow-up, typically within
As noted previously, chest radiography is the most common imaging 24 hours. Most pathogens are isolated from cultures within the first
study done in the routine evaluation of a newborn, infant, or child 24 hours, and hospitalization with IV antibiotics is generally war-
with fever and symptoms consistent with lower respiratory infection ranted for any young infant with a positive culture result consistent
or with fever and no apparent source. Other imaging studies, such with a pathogen. The addition of vancomycin must be considered if
as bone scanning, gallium scanning, magnetic resonance imaging, resistant pneumococcus is a possibility.
and computed tomography, are indicated if infection is suspected, The management of well-appearing febrile infants and chil-
such as an occult abscess or osteomyelitis, and if positive imaging dren between 3 and 36 months of age is dependent on the identi-
findings will change patient management. Brain computed tomog- fication of a focal infection on physical examination or diagnostic
raphy is not needed prior to lumbar puncture in young children studies. Antibiotics should be administered if a bacterial infection
without focal neurologic deficits. is identified. Well-appearing infants who are tolerating oral fluids

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 Chapter 67: Fever and Bacteremia 481

do not undergo antimicrobial therapy, the risk of persistent bacte-

Box 67.7. Low-Risk Criteria for Serious Bacterial
remia is approximately 20% and the risk of meningitis is approxi-
Infection in Young Febrile Infants
mately 5% to 10%. These risks vary depending on which organism
1. Well-appearing is isolated from the blood. In general, the risk of developing seri-
2. No focal bacterial infection apparent on examination ous sequelae is greater with bacteremia caused by H influenzae than
3. Previously healthy term infant with unremarkable neonatal course S pneumoniae (25% and 5%, respectively). Currently, concern about
4. WBC count between 5,000 and 15,000 cells/mm3 SBI related to either of these organisms is significantly diminished
5. Absolute band cell count <1,500 cells/mm3 or a band-to-neutrophil ratio with the introduction of the conjugate vaccines.
of ≤0.2
6. Normal cerebrospinal fluid examination with <8 WBCs per high-power
field and negative Gram stain
7. Normal urinalysis with <10 WBCs per high-power field
CASE RESOLUTION
The infant is irritable and has a high fever of unknown source. Her vaccine sta-
8. Stool studies with <5 WBCs per high-power field (if diarrhea present)
tus is uncertain. No source of her infection is revealed on physical examination.
9. Normal chest radiograph (if respiratory signs/symptoms present) Because her fever is 39.5°C (103.1°F), she is irritable, and her immune status
Abbreviation: WBC, white blood cell. is uncertain, a complete laboratory assessment, including a lumbar puncture,
should be performed. Management should be determined after all laboratory
data are available. If laboratory assessment reveals a focus of infection, such
as a UTI, she should be managed with antibiotics. If laboratory assessment
well and have no significant respiratory distress or hypoxia can be
does not reveal a source for the fever and her WBC count is greater than
managed on an outpatient basis, even if they have a UTI or pneu- 15,000 cells/mm3 or her ANC is greater than 10,000 cells/mm3, she can be
monia. Clinicians may administer a dose of ceftriaxone for the administered an intramuscular injection of ceftriaxone as expectant manage-
first 24 hours and transition to an oral antibiotic thereafter. Continued ment for occult bacteremia and undergo reevaluation in 24 hours or undergo
close follow-up is necessary, usually within 24 to 48 hours. Infants who treatment without antibiotics.
have received 1 dose or less of the pneumococcal vaccine can receive a
dose of ceftriaxone pending culture results if the WBC count is greater
than 15,000 cells/mm3 or the ANC is greater than 10,000 cells/mm3.
Healthy and well-appearing febrile children older than 3 years
Selected References
can be treated on an outpatient basis if they are well hydrated and American College of Emergency Physicians Clinical Policies Committee,
have no respiratory distress or hypoxia. Children with underlying Clinical Policies Subcommittee on Pediatric Fever. Clinical policy for children
medical conditions or who are at risk for bacterial infections may younger than three years presenting to the emergency department with fever.
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require treatment in the hospital with IV antibiotics regardless of
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age, depending on their individual circumstances.
Baraff LJ. Management of infants and young children with fever without
Children with occult pneumococcal bacteremia who are afebrile
source. Pediatr Ann. 2008;37(10):673–679 PMID: 18972849 https://doi.org/
and well-appearing at follow-up likely can continue to be treated on an
10.3928/00904481-20081001-01
outpatient basis. Additional dosing of ceftriaxone may be necessary;
Biondi E, Evans R, Mischler M, et al. Epidemiology of bacteremia in febrile
transition to an oral antibiotic can be considered if sensitivity testing
infants in the United States. Pediatrics. 2013;132(6):990–996 PMID: 24218461
has been performed. Repeat blood cultures likely are not necessary if https://doi.org/10.1542/peds.2013-1759
the child is afebrile, because the likelihood of persistent bacteremia
Byington CL, Enriquez FR, Hoff C, et al. Serious bacterial infections in
is low. Those who are younger than 90 days, are still febrile at follow- febrile infants 1 to 90 days old with and without viral infections. Pediatrics.
up, or have clinically worsened warrant hospitalization and IV anti- 2004;113(6):1662–1666 PMID: 15173488 https://doi.org/10.1542/
biotics. Additionally, a lumbar puncture should be considered if not peds.113.6.1662
previously performed in a child with a positive blood culture. All indi- Centers for Disease Control and Prevention. Direct and indirect effects of rou-
viduals with occult N meningitides bacteremia warrant hospitalization tine vaccination of children with 7-valent pneumococcal conjugate vaccine on
and IV antibiotics. A lumbar puncture also should be strongly con- incidence of invasive pneumococcal disease—United States, 1998-2003. MMWR
sidered if not previously performed because of the substantial risk for Morb Mortal Wkly Rep. 2005;54(36):893–897 PMID: 16163262
meningitis with this particular microorganism. In general, most other Grijalva CG, Poehling KA, Nuorti JP, et al. National impact of universal child-
cases of occult bacteremia warrant hospitalization and IV antibiotics. hood immunization with pneumococcal conjugate vaccine on outpatient medical
Consultation with an infectious disease specialist may be indicated to care visits in the United States. Pediatrics. 2006;118(3):865–873 PMID: 16950975
https://doi.org/10.1542/peds.2006-0492
determine appropriate antibiotic selection and duration of therapy.
Huppler AR, Eickhoff JC, Wald ER. Performance of low-risk criteria in the
evaluation of young infants with fever: review of the literature. Pediatrics.
Prognosis 2010;125(2):228–233 PMID: 20083517 https://doi.org/10.1542/peds.2009-1070
Occult bacteremia may resolve without therapy and have no Joffe MD, Alpern ER. Occult pneumococcal bacteremia: a review. Pediatr
sequelae, but it may persist or produce localized infections, such Emerg Care. 2010;26(6):448–454 PMID: 20531134 https://doi.org/10.1097/
as meningitis or septic arthritis. In children with bacteremia who PEC.0b013e3181e15e36

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Kaplan SL, Barson WJ, Lin PL, et al. Early trends for invasive pneumococcal Newman TB, Bernzweig JA, Takayama JI, Finch SA, Wasserman RC, Pantell RH.
infections in children after the introduction of the 13-valent pneumococcal con- Urine testing and urinary tract infections in febrile infants seen in office set-
jugate vaccine. Pediatr Infect Dis J. 2013;32(3):203–207 PMID: 23558320 https:// tings: the Pediatric Research in Office Settings’ Febrile Infant Study. Arch Pediatr
doi.org/10.1097/INF.0b013e318275614b Adolesc Med. 2002;156(1):44–54 PMID: 11772190 https://doi.org/10.1001/
Krief WI, Levine DA, Platt SL, et al; Multicenter RSV-SBI Study Group of archpedi.156.1.44
the Pediatric Emergency Medicine Collaborative Research Committee of the Nigrovic LE, Kuppermann N, Malley R; Bacterial Meningitis Study Group of
American Academy of Pediatrics. Influenza virus infection and the risk of seri- the Pediatric Emergency Medicine Collaborative Research Committee of the
ous bacterial infections in young febrile infants. Pediatrics. 2009;124(1):30–39 American Academy of Pediatrics. Children with bacterial meningitis present-
PMID: 19564280 https://doi.org/10.1542/peds.2008-2915 ing to the emergency department during the pneumococcal conjugate vaccine
Lee GM, Fleisher GR, Harper MB. Management of febrile children in the age of era. Acad Emerg Med. 2008;15(6):522–528 PMID: 18616437 https://doi.org/
the conjugate pneumococcal vaccine: a cost-effectiveness analysis. Pediatrics. 10.1111/j.1553-2712.2008.00117.x
2001;108(4):835–844 PMID: 11581433 https://doi.org/10.1542/peds. Pantell RH, Roberts KB, Greenhow TL, Pantell MS. Advances in the diagnosis
108.4.835 and management of febrile infants. Adv Pediatr. 2018;65(1):173–208 PMID:
Levine DA, Platt SL, Dayan PS, et al; Multicenter RSV-SBI Study Group of the 30053923 https://doi.org/10.1016/j.yapd.2018.04.012
Pediatric Emergency Medicine Collaborative Research Committee of the American Rudinsky SL, Carstairs KL, Reardon JM, Simon LV, Riffenburgh RH, Tanen DA.
Academy of Pediatrics. Risk of serious bacterial infection in young febrile infants Serious bacterial infections in febrile infants in the post-pneumococcal conju-
with respiratory syncytial virus infections. Pediatrics. 2004;113(6):1728–1734 gate vaccine era. Acad Emerg Med. 2009;16(7):585–590 PMID: 19538500 https://
PMID: 15173498 https://doi.org/10.1542/peds.113.6.1728 doi.org/10.1111/j.1553-2712.2009.00444.x
Manzano S, Bailey B, Gervaix A, Cousineau J, Delvin E, Girodias JB. Wilkinson M, Bulloch B, Smith M. Prevalence of occult bacteremia in children
Markers for bacterial infection in children with fever without source. Arch aged 3 to 36 months presenting to the emergency department with fever in the
Dis Child. 2011;96(5):440–446 PMID: 21278424 https://doi.org/10.1136/ postpneumococcal conjugate vaccine era. Acad Emerg Med. 2009;16(3):220–225
adc.2010.203760 PMID: 19133844 https://doi.org/10.1111/j.1553-2712.2008.00328.x

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 CHAPTER 68

Emerging Infectious Diseases

Christian B. Ramers, MD, MPH, AAHIVS, and Thomas R. Hawn, MD, PhD

CASE STUDY
A previously healthy 8-year-old boy is brought to studies sent from the office reveal microscopic hematuria,
his pediatrician’s office in late August with 2 days of leukopenia (white blood cell count 2,800 cells/mm3), and
fever, fatigue, headache, myalgias, nausea, and gingival thrombocytopenia (platelet count 85,000 platelets/mm3).
bleeding. On the morning of the visit his mother
noted a rash on his legs. He lives with his family in the
Questions
1. What is an emerging or reemerging infection?
Northeastern United States but recently returned from
2. What pathogens are associated with emerging
a 1-week vacation in Key West, Florida. He engaged in
infections?
extensive outdoor activities, including snorkeling, hiking,
3. What are some common or emerging infectious
and several evening boat trips, and he sustained multi-
diseases that may cause the clinical syndrome in
ple mosquito bites during the trip. He received all rou-
the case scenario?
tine childhood immunizations, denies any allergies, and
4. How does recent travel influence the differential
takes no medications. No other family members are ill.
diagnosis?
On physical examination, his temperature is 38.7°C
5. What resources can a primary care physician access
(101.7°F) and he is generally ill-appearing. He has pho-
to help in making a diagnosis?
tophobia and mild meningismus, and a petechial rash
is noted on his trunk and lower extremities. Laboratory

Emerging and reemerging infectious diseases are defined as those primary care physician to access local diagnostic and public health
for which the incidence in human populations has increased in the support.
past 2 decades or threatens to do so in the near future. They may
represent the resurgence of an ancient human scourge, a novel zoo- Contributing Factors
nosis that has broadened its host range, a common pathogen that The spectrum of infectious diseases has always changed and evolved
has acquired a new antimicrobial resistance profile, or more rarely along with societal and environmental changes. Literature supports
a previously unidentified or unknown microorganism. A startling the supposition that throughout human history, several general
diversity of organisms has met these criteria, including viral, bac- driving forces influence the emergence or reemergence of certain
terial, fungal, and parasitic pathogens. Likewise, a variety of factors infectious diseases. The most important factors are human migra-
affect the emergence or reemergence of these pathogens, including tion, environmental and ecological changes, changing patterns of
range and susceptibility of human hosts, evolution and antigenic human host susceptibility and immunity and, more recently, the
shift of the pathogen, and ecological and environmental changes, use and overuse of antimicrobial agents. Table 68.1 shows some
such as vector amplification or breakdown of public health mea- of the mechanisms identified in recent emerging infectious dis-
sures. Although a select few of these emerging pathogens represent eases and provides illustrative examples from the United States and
malicious propagation or bioterrorism, most appear spontaneously abroad. In reality, many simultaneous contributing factors often are
at ambulatory or emergency health facilities and thus are relevant at play, and diseases may emerge or retreat within human popula-
to the practicing primary care physician. It has only been through tions without clear drivers. Common themes that result in recogniz-
astute clinical observation, targeted outbreak investigation, and a able emergence events typically couple a vulnerable host population
coordinated public health response that many emerging infectious with a pathogen to which that population lacks immunity or prior
diseases have been identified. exposure.
In this chapter we review the factors involved in the emergence Pediatric populations are particularly susceptible to emerging
and reemergence of infectious diseases of public health significance, and reemerging diseases in several of these categories. The recent
discuss several specific examples that are likely to be most relevant Zika virus epidemic has also shed light on the particular risks to the
to pediatric practice, summarize regional and global outbreaks of fetus in the setting of a newly emerging or reemerging infection.
emerging infectious diseases, and provide practical steps for the After immunity from acquired maternal antibody wanes, children

483

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 484 PART 5: ACUTE AND EMERGENT PROBLEMS

Table 68.1. Factors Contributing to Infectious Disease Emergence/Reemergence

Contributing Factor Examples Illustrative Pathogens
Societal change Economic impoverishment Cholera, malaria, salmonella
Population growth or migration
Globalization of food distribution
Urban decay
Advances in health care New medical devices Aspergillosis, cytomegalovirus, methicillin-resistant Staphylococcus aureus
Organ transplantation
Drugs causing immunosuppression
Use of antimicrobial agents
Human behavior Worldwide travel HIV/AIDS, hepatitis C virus, histoplasmosis
Injection drug use
Sexual activity
Outdoor recreation activities
Environmental changes Deforestation/reforestation Cryptococcus gattii, dengue, Burkholderia pseudomallei
Flood/drought
Global warming
Public health infrastructure and control Reduction of prevention programs Mycobacterium tuberculosis (multidrug-resistant and extensively drug-
Inadequate surveillance resistant), measles, mumps, pertussis
Waning immunization rates
Microbial adaptation and change Antigenic drift/shift H1N1, H5N1, and H7N9 influenza; chloroquine-resistant malaria;
Changes in virulence factors vancomycin-resistant enterococci
Development of drug resistance
Derived from Morens DM, Folkers GK, Fauci AS. The challenge of emerging and re-emerging infectious diseases. Nature. 2004;430(6996):242–249.

develop adaptive immunity on their own and may experience up to Special Situations
12 upper respiratory and/or diarrheal diseases per year. In some cases
Increasingly, primary care physicians are required to carefully con-
an unexplained increase in pediatric mortality from a typical clin-
sider the risks of emerging or reemerging infectious diseases. Some
ical syndrome, such as upper respiratory or flu-like illness, may be
of the unique clinical settings in which less common or emerging
the harbinger of an emerging pathogen. Large institutional settings,
infectious diseases warrant consideration include expanded inter-
such as child care centers and schools, place children at high risk for
national travel, immigration and international adoption, immune
exposure to infectious agents via close contact, and respiratory and
suppression and immunomodulation, and the unvaccinated or
droplet spread. Similarly, differing hygiene practices, such as hand-
undervaccinated child.
washing, cough etiquette, sharing of fomites, and fecal/urinary incon-
tinence, place infants and children at particular risk of exposure.
Antimicrobial agents are commonly prescribed in the primary Expanded International Travel
care setting for otitis media, pharyngitis, and respiratory infection, With the increasing accessibility of long-distance international
and overuse of antibiotics in this setting has been associated with travel, children are more frequently being included in tourist trips
an increased risk of colonization and infection with drug-resistant or, in the case of immigrant families, visits to friends or relatives in
organisms. In many cases, what was once a first-line therapy for a their home country. Children are less likely to seek pretravel advice
particular clinical syndrome must be reconsidered because of the and consequently are less likely to adhere to recommended travel
emergence of altered antibiotic susceptibility patterns. Advances in guidelines. A report from GeoSentinel, a large group of worldwide
medical care has resulted in growth in the number of vulnerable travel clinics, found that only 32% of children visiting friends and
hosts through increased survival of preterm infants, cancer chemo- relatives in developing countries received recommended travel vac-
therapy, organ transplantation, and the use of immunosuppressive cines or prophylactic medications even though they were more likely
or immunomodulatory agents. Additionally, because of parental to pre­sent with illness and require hospital admission after travel.
belief systems, personal choice, and lack of access, immunization Primary care physicians evaluating returning travelers must consider
rates in certain regions remain suboptimal, placing children at risk detailed travel history, prophylaxis or protective measures taken
of acquiring vaccine-preventable disease. (if any), risk profile of the region visited, and incubation period of

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 Chapter 68: Emerging Infectious Diseases 485

the suspected pathogen. Although by far the most common travel- rates. Although the most widely cited study linking autism to mea-
related illnesses are self-limited diarrheal disease, emerging infec- sles, mumps, rubella (MMR) vaccination was retracted by the
tions, such as Ebola, Zika, dengue, chikungunya, and H5N1, H7N9, Lancet in February 2010, several recent parental surveys indicate
or H1N1 influenza, must be considered along with other infectious persistent beliefs about a suspected vaccine–autism link. In 2004,
diseases, such as malaria and tuberculosis. the Institute of Medicine Immunization Safety Review Committee
published a comprehensive report that found no convincing
Immigration and International Adoption evidence of a causal link between the MMR vaccine or any
Increasing rates of international adoption or recent immigration thimerosol-containing vaccine and autism. In a survey of 1,552
may result in evaluation by primary care physicians of children parents conducted in 2009, however, 25% agreed with the state-
with unknown or unavailable birth, early childhood, or immuni- ment, “Some vaccines cause autism in healthy children.” Decreasing
zation histories (see Chapter 37 and Chapter 39). Vaccine sched- vaccination rates have resulted in increased risk for outbreaks of
ules vary by country of origin, including some vaccines that are no reemerging infectious diseases. Measles was officially declared
longer given in the United States (eg, bacille Calmette-Guérin, live “eliminated” (defined as the absence of endemic measles trans-
oral polio virus vaccine). Considerable variation exists in reliabil- mission for >12 months) in the United States in the year 2000.
ity of medical reporting in these situations, with some countries During the first 8 months of 2019, however, more than 1,200
achieving or exceeding developed world standards but most pro- cases of measles were reported in more than 30 states; 75% of the
viding reports of dubious quality. Many physicians who special- cases occurred in New York State, where individuals had not been
ize in “adoption clinics” or work in settings with large immigrant vaccinated. This is the greatest number of cases reported since
populations obtain serologic evidence of prior immunization 1992. This compares with a median 60 cases reported annually
(eg, measles, mumps, varicella, polio, diphtheria, tetanus). Physicians every year from 2001 through 2011. Similar to resurgent measles
must also be aware of infections with clinically silent latent phases outbreaks, a 2010 to 2011 pertussis epidemic in California became
(eg, viral hepatitis, latent tuberculosis, intestinal helminth infections, the largest since 1955, affecting more than 9,000 individuals and
HIV). Several emerging or reemerging infectious diseases in the causing 10 infant deaths. Thus, it is crucial for physicians to include
United States may be endemic in the countries of origin of adopted vaccination status and exposure history when evaluating children
or recently arrived immigrant children. with an infectious syndrome. Increasingly, the differential diagno-
sis and diagnostic workup must include emerging and reemerg-
Immune Suppression ing diseases, some of which may be unfamiliar to physicians from
and Immunomodulation their training or clinical experience.
Therapeutic advances in pediatric oncology, organ transplanta-
tion, rheumatology, and care of chronic congenital conditions have Select Emerging Pathogens
resulted in an increasing population of children with immunosup-
pression. Although typically under the care of specialists, these chil- Major emerging and reemerging infectious diseases of the past
dren may have a medical home in a primary care facility and thus 20 years are shown in Figure 68.1 and Table 68.2. The table is not
can present with an opportunistic or emerging infectious disease meant to be an exhaustive list, but rather a sampling of emerging
to their primary care physician. The spectrum of risk for infectious pathogens most likely to present to a primary care physician.
diseases varies considerably depending on the type of immunosup-
Viruses
pression. For example, tumor necrosis factor-α inhibitors, which are
commonly used in the management of juvenile idiopathic arthri- Zika
tis, convey a particularly high risk of fungal and mycobacterial Zika is a flavivirus that was initially isolated from a monkey in the
infection. Neutropenia from cytotoxic chemotherapy is associated Zika forest in Uganda in 1947. The geographic distribution was pre-
with an increased risk of bloodstream bacterial infection among viously thought to be limited to Africa with mild clinical manifesta-
others. Similarly, lymphopenia related to solid organ transplanta- tions. From 2007 to 2014, however, Zika caused outbreaks in several
tion portends a particular vulnerability to viral infections, ranging of the Pacific Islands, after which a major epidemic emerged in Brazil
from widespread community respiratory viruses to reactivation of in 2015 with rapid spread throughout the Americas, with outbreaks
common agents, such as varicella-zoster virus. Emerging infectious in the United States in 2016. Currently, Zika is present in more than
diseases, such as new coronaviruses (Middle East respiratory syn- 80 countries in Africa, Asia, and the Americas. Aedes aegypti as
drome coronavirus [MERS-CoV], severe acute respiratory syndrome well as other aedes species are the primary vectors with a predilec-
[SARS]), human metapneumovirus, or H1N1 influenza, may have tion for urban environments, similar to dengue, chikungunya, and
particularly severe clinical manifestations in children with immu- yellow fever. Non-vector routes of transmission include blood
nosuppression compared with the general population. transfusions and sexual contact.
Major features of the reemergence of Zika include both the
Unvaccinated or Undervaccinated Child expanded geographic distribution and discovery of its cause of severe
Despite the ongoing efforts of public health authorities, some fetal neurologic infections. Zika is neurotropic and targets neural
regions have noted a worrisome downward trend in immunization progenitor cells in the developing brain. The resurgence of Zika in

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 486 PART 5: ACUTE AND EMERGENT PROBLEMS

Ebola virus disease

Antimicrobial- West nile virus MERS-CoV
Cryptosporidiosis
resistant threats Diphtheria Akhmeta virus
- CRE Powassan E. coli Drug-resistant
Enterovirus D68
- MRSA virus O104:H4 malaria Rift valley fever
- C. difficile Heartland Typhoid fever
- N. gonorrhoeae
SFTSV bunyavirus
H3N2v influenza
Cyclosporiasis Hepatitis C
E. coli O157:H7
E. coli O157:H7
Lyme vCJD H10N8
Measles
disease influenza
Human
monkeypox H7N9
Listeriosis influenza
Bourbon virus H5N1
Lassa
2009 H1N1 fever influenza
influenza HIV SARS
Adenovirus 14 Nipah
Anthrax virus
bioterrorism Hendra virus
Chikungunya
Enterovirus 71
Hantavirus Dengue
pulmonary Human monkeypox
syndrome Zika virus Ebola virus disease

Yellow fever Marburg MDR/XDR Zika virus

hemorrhagic tuberculosis Plague
Cholera
fever
Human African
typanosomiasis

Newly emerging Re-emerging/resurging “Deliberately emerging” September 2017

Figure 68.1. Global examples of recently emerging and reemerging infectious diseases.
Abbreviations: C. difficile, Clostridium difficile; CRE, carbapenem-resistant Enterobacteriaceae; E. coli, Escherichia coli; MDR, multi-drug resistant; MERS-CoV, Middle East respiratory
syndrome coronavirus; N. gonorrhoeae, Neisseria gonorrhoeae; SARS, severe acute respiratory syndrome; SFTSV, severe fever with thrombocytopenia syndrome virus; vCJD, variant
Creutzfeldt-Jakob disease; XDR, extensively drug-resistant.
Reprinted from National Institute of Allergy & Infectious Diseases. Global Examples of Emerging and Re-Emerging Infectious Diseases. Bethesda, MD: National Institute of Allergy &
Infectious Diseases; 2017. https://www.niaid.nih.gov/news-events/three-decades-responding-infectious-disease-outbreaks

the Americas has included clinically devastating congenital central pregnancy. Travel-related precautions pertaining to sexual trans-
nervous system (CNS) malformations, including microcephaly, ven- mission extend to the post-travel time period because Zika can
triculomegaly, cerebral calcifications, and ocular abnormalities. In persist in bodily fluids (eg, RNA is detected for approximately
adults, approximately 50% of infected individuals have no symp- 2 weeks in plasma, 6 weeks in urine, and up to 6 months in
toms, with the remaining developing a rash, fever, conjunctivitis, semen). Currently, the CDC recommends that men wait at least
and arthralgias. Uncommon manifestations include Guillain-Barré 3 months before engaging in unprotected sex if they are plan-
syndrome. The cause of the apparent shift to more prominent CNS ning to conceive with their partner and may have had a Zika virus
clinical manifestations of the recent epidemics is not known. Genetic exposure. Previously, the waiting period was 6 months, but the
data indicate that Zika acquired a single amino acid mutation in a recommendation was updated based on data indicating that the
surface protein that causes increased neurovirulence, viral repli- longest period from symptom onset to potential sexual transmis-
cation, and rates of microcephaly in cellular and animal models. sion was 32 to 41 days. Evaluation of pregnant women for possi-
This mutation appeared in approximately 2013 and has been sta- ble Zika infection includes exposure risk assessment, symptom
bly transmitted during the epidemic. Although this genetic change assessment, and diagnostic testing options that are tiered based
may explain the new clinical manifestations, it remains possible that on time from exposure and stage of pregnancy. Potential diagnos-
neurologic involvement was not previously apparent because of a tic tests include nucleic acid tests in serum and urine, immuno-
lower disease incidence. globulin (Ig) M serology, and a plaque reduction neutralization
Several issues are important for clinical management of test. For pregnant women diagnosed with acute Zika virus infec-
Zika, including transmission prevention, evaluation and treat- tion, further diagnostic testing in the form of ultrasonography
ment of pregnant women, and treatment of infected neonates. and amniocentesis can be performed to assess for fetal infection
Prevention of vector-borne transmission includes mosquito and complications. Currently, no specific treatment or vaccine is
precautions as well as avoiding or postponing travel during available for Zika virus.

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 Chapter 68: Emerging Infectious Diseases 487

Table 68.2. A Sampling of Recent Emerging Infectious Diseases

Pathogen Clinical Syndrome Diagnosis Management
Zika virus Adult: asymptomatic or viral Exposure/symptom assessment, IgM, PCR Supportive
exanthem with arthralgias
Fetal: severe CNS malformations
Ebola virus Hemorrhagic fever Clinical case definition Supportive
Confirm on serology, PCR testing Experimental therapies: conva-
lescent serum, monoclonal
antibodies, experimental antiviral
agents, preventive vaccine
Measles virus Measles Clinical Supportive
Pneumonia Confirm with IgM Consider antibiotics for bacterial
Postinfectious encephalitis superinfections
Mumps virus Parotitis Serology Supportive
Orchitis Culture NSAIDs
Encephalopathy PCR testing
Dengue virus Unspecified febrile illness Serology Supportive
Dengue fever Aggressive fluid management
Dengue hemorrhagic fever
Influenza virus (H1N1 [2009], H5N1 Fever, respiratory symptoms PCR or antigen testing Oseltamivir phosphate, zanamivir,
[2007], H7N9 [2013]) amantadine hydrochloride,
rimantadine hydrochloride
Coronavirus (ie, SARS, MERS-CoV) Fever, respiratory symptoms PCR testing Supportive
Chikungunya virus Febrile syndrome with arthralgias, Clinical Supportive, NSAIDs
rash, conjunctivitis Confirm with serology, viral culture, or PCR
testing
WNV Asymptomatic Serology Supportive (ribavirin and
West Nile fever WNV antigen or PCR testing interferon-α-2b are experimental)
West Nile encephalitis (flaccid IgM in CSF
ascending paralysis)
MRSA Skin and soft tissue infections, Culture and susceptibility Antibiotics
bacteremia, pneumonia
Resistant gram-negative bacteria Pneumonia, UTI, bacteremia, sepsis Culture and susceptibility Antibiotics
Resistant Streptococcus pneumoniae Pneumonia, meningitis, otitis media Culture and susceptibility Antibiotics
Ehrlichiosis/anaplasmosis Fever, headache, myalgia, rash PCR testing or peripheral blood smear Doxycycline
Cryptococcus gattii Pneumonia, meningitis Culture or cryptococcal antigen testing Amphotericin, 5-FC, fluconazole
Abbreviations: 5-FC, 5-fluorocytosine; CSF, cerebrospinal fluid; CNS, central nervous system; Ig, immunoglobulin; MERS-CoV, Middle East respiratory syndrome coronavirus; MRSA, methicillin-resistant
Staphylococcus aureus; NSAIDs, nonsteroidal anti-inflammatory drugs; PCR, polymerase chain reaction; SARS, severe acute respiratory syndrome; UTI, urinary tract infection; WNV, West Nile virus.

Ebola a case fatality rate of approximately 50% and has caused several
First described near the Ebola river in Zaire (now the Democratic moderate-sized outbreaks in the border region of Sudan, Uganda, and
Republic of Congo [DRC]) in 1976, Ebola virus is a member of the the DRC. Zaire ebolavirus, the most lethal species, has caused most
genus Filoviridae of hemorrhagic fever viruses. Although until of the sporadic outbreaks throughout sub-Saharan Africa, includ-
recently only seen in sporadic and remote outbreaks in sub-Saharan ing the largest outbreak ever recorded in 2014 to 2016 that engulfed
African villages, Ebola’s reputation has far outpaced its reach because several West African countries, with approximately 28,600 cases and
of a case fatality rate of 88% in early descriptions of the first out- more than 11,000 deaths. Bundibugyo ebolavirus is a third species
breaks. Four species are known to cause disease in humans, with discovered in 2007 that has caused 2 well-documented outbreaks in
variable geographic footprints and virulence. Sudan ebolavirus has the DRC and along the border of the DRC and Uganda. Finally, Taï

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 488 PART 5: ACUTE AND EMERGENT PROBLEMS

Forest ebolavirus has been identified in a single case in Côte d’Ivoire ethical discussions, experimental treatments were mobilized dur-
in West Africa. At the time of publication, an ongoing outbreak of the ing the 2014 to 2016 West Africa outbreak, including transfusions
Zaire ebolavirus is occurring in the North Kivu and Ituri provinces using convalescent serum of survivors, monoclonal antibody combi-
of DRC, with at least 129 confirmed or probable cases and 89 deaths. nations (ie, ZMapp, ZMab, MIL77), antiviral drugs thought to have
Although the reservoir of Ebola is unknown, scientists suspect inhibitory activity against Ebola (ie, TKM-Ebola, favipiravir, brin-
a fruit bat or non-human primate may serve as the natural host, cidofovir, amiodarone), and agents purported to counteract capil-
with uncommon “spillover events” occurring after direct human- lary leak (ie, FX06, melanocortin). No agents have been approved
to-animal contact. Human-to-human transmission can occur after by the US Food and Drug Administration for use in individuals with
1 of these events via direct contact (through broken skin or mucus Ebola virus disease, and each of these cases underwent considerable
membranes) with infected blood or body fluids, including urine, ethical scrutiny and evaluation.
saliva, sweat, feces, vomit, human milk, and semen. Importantly, Prevention of Ebola virus disease has relied mainly on early diag-
human-to-human transmission does not occur in the absence of nosis as well as strict isolation and infection control procedures. An
symptoms. No evidence exists indicating that mosquitos or other experimental vaccine was developed and preliminarily tested in 2015
insects can transmit Ebola, and secondary foodborne transmission in Guinea during the large outbreak in that country. In a small trial,
is not thought to occur except from direct consumption of the meat the vaccine appeared to be highly protective against Ebola virus dis-
of an infected primate. ease. The National Institutes of Health is conducting an ongoing
After an incubation period of approximately 8 to 10 days (range, open label, pre-exposure clinical trial in adults at potential occu-
2–21 days), Ebola causes an array of nonspecific systemic symp- pational risk.
toms, such as fever, nausea, vomiting, diarrhea, weakness, severe Measles
headaches, myalgias, and abdominal pain. The diagnosis should be
Measles, which is caused by a virus from the Paramyxoviridae fam-
suspected in cases with both a combination of suspected symptoms
ily, began to decline as a major threat in the United States after a safe
and a possible exposure to Ebola virus within the previous 21 days.
and effective vaccine was developed in 1963. A significant resurgence
Isolation of “patients under investigation” and strict contact pre-
occurred between 1989 and 1991, however, largely because of a pool of
cautions are necessary to contain outbreaks and prevent spread to
vulnerable, unvaccinated preschool-age children. Nearly 55,000 cases
health care personnel. Specialized molecular testing for viremia is
and 130 deaths occurred in the United States, prompting a renewed
available in public health laboratories and is typically positive within
effort at prevention through vaccination. A second dose of vaccine
3 days of the onset of symptoms.
for school-age children was also recommended after this outbreak.
The pathophysiology of Ebola virus disease (formerly Ebola
Another resurgence of new cases occurred after 2004, but with new
hemorrhagic fever) involves massive fluid, electrolyte, and protein
epidemiologic features; 90% of the cases were either directly imported
wasting as well as capillary leak and hemorrhage with resultant
from travelers or immigrants, or were associated with importation
blood loss, dehydration, oliguria, circulatory collapse, and respira-
from outside the United States. Large outbreaks in the United States
tory failure. In a well-studied case series of 27 patients evacuated
have been reported in 2008, 2011, 2013, 2014, and 2019.
from the West Africa outbreak to the United States or Europe, peak
Measles is a highly contagious pathogen passed via respiratory
plasma viral RNA levels occurred at a median of 7 days and was
droplets. Secondary transmission is thought to be greater than 90%
cleared a median of 17.5 days after onset of symptoms. With max-
among susceptible household contacts. Approximately 10 days after
imal supportive care as well as experimental therapies in 85% of
exposure, clinical illness is characterized by a distinctive febrile pro-
patients, the case fatality rate in this cohort was 18.5%, which was
drome (ie, conjunctivitis, coryza, cough), followed by Koplik spots
lower than previously reported.
(blue-gray enanthem on buccal mucosa) and, ultimately, the classic
Postmortem human-to-human transmission of Ebola has
maculopapular erythematous eruption. Diagnosis is usually ascer-
occurred as well, particularly related to burial rituals such as crema-
tained based on clinical evidence alone given the distinct clinical
tion, cleansing of bodies, and postmortem autopsy evaluations. The
presentation; however, for confirmatory testing, the immunoglob-
CDC has developed guidelines for safe handling of human remains
ulin (Ig) M serology is nearly 100% sensitive if performed after the
focusing on the use of personal protective equipment as well as proper
onset of rash. Respiratory droplet isolation should occur until 4 days
disposal of medical equipment and safe interment of the body. In sur-
following appearance of the rash in immunocompetent patients and
vivors who recover from the infection, ocular complications and lin-
until the clinical illness resolves in those who are immunocompro-
gering arthralgias have been described. Persistence of virus has also
mised. Treatment is largely supportive; however, respiratory and
been observed in immune-privileged sites, such as aqueous humor,
neurologic complications can occur in 6% and 0.1% of patients,
cerebrospinal fluid (CSF), and semen; however, the transmission
respectively. Further control of this reemerging infectious disease
dynamics of convalescing patients are poorly understood.
will likely depend on renewed attention to domestic vaccination
The foundation of managing Ebola virus disease is supportive
efforts and the roll-out of vaccination worldwide.
care, principally intravenous hydration and electrolyte replacement,
oxygen and mechanical ventilation as necessary, renal replacement Mumps
therapy, blood pressure and blood product support, and antibiotic Although the clinical syndrome of the mumps virus is distinct from
treatment for any suspected secondary infections. After intensive that of measles, the 2 members of the Paramyxoviridae family share

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 Chapter 68: Emerging Infectious Diseases 489

a similar history of initial control and recent reemergence. A live, are diverse and may include hepatitis, myocarditis, pericarditis, and
attenuated mumps vaccine was licensed in 1967 and incorporated encephalopathy. Leukopenia and thrombocytopenia are common
into the Advisory Committee on Immunization Practices recom- laboratory findings, and in severe cases, a coagulopathy and bleeding
mended schedule by 1977. Due to high vaccination rates, mumps manifestations seem to be the most dangerous sequelae. Diagnosis
had declined by more than 99% by 2005. However, there have been can be made with a compatible clinical history and confirmed on
2 major resurgences in the United States. In 2005 to 2006 a total of serologic testing. No direct-acting anti­virals exist, nor is a vaccine
6,584 cases were reported in a multistate outbreak in the Midwestern available. Care is generally supportive.
United States. Although numerically most of these cases occurred
among college students who had been previously vaccinated, attack Influenza Viruses
rates were considerably higher in unvaccinated individuals. Equally In 2009, a novel strain of influenza A known as H1N1 caused the first
large outbreaks involving more than 6,000 cases were reported in global influenza pandemic since 1968, with an estimated 59 million
2016 and 2017. During the first 8 months of 2019, there were more illnesses and 12,000 deaths in the United States alone. Although early
than 2,360 reported cases in 47 states. in the year it seemed as though “bird flu” or H5N1 influenza would
The clinical presentation of mumps typically involves fever, mal- be the greatest concern to public health, it was a different strain
aise, and parotitis. Complications are rare, but in some studies up of swine origin that resulted in a global pandemic. In April 2013, a
to 10% of patients had aseptic meningitis, of which hearing loss is different strain of bird flu known as H7N9 emerged in China with
an important sequela. Up to 37% of adolescent and adult males can a disturbing 28% case fatality rate. Like the related H5N1 strain,
present with orchitis, which may result in sterility. Diagnosis is typi- however, human-to-human transmission was not observed, and
cally made based on a compatible clinical syndrome with confirma- outbreaks have been limited to clusters of individuals with very high
tion by isolation or polymerase chain reaction (PCR)-based detection levels of exposure to poultry.
of the virus from saliva, CSF, urine, or semen. Immunoglobulin M Influenza viruses have a segmented genome and thus are able
serology is also a useful confirmatory method. to adapt and evolve quite rapidly to evade slower adaptive immune
Management is generally supportive, with analgesics used for responses. Through antigenic drift, small changes occur in cell sur-
the pain of parotitis and/or orchitis. For severe cases, intravenous face genes through time, resulting in subtle structural changes to
Ig has been used to mitigate immune-mediated postinfectious com- the cell surface proteins neuraminidase and hemagglutinin and
plications, and interferon-α-2b has been used to alleviate orchitis. decreased recognition by the immune system. In antigenic shift,
The previously discussed outbreaks have been the focus of genome segments from diverse strains recombine in a single new
considerable scrutiny as indicators of vaccine effectiveness and virus particle, resulting in abrupt and substantial changes in anti-
community vaccination rates. Based on extensive analyses, MMR genic variation. Typically, shifts are more likely to cause pandem-
vaccine is still considered to be 80% to 90% effective after 2 doses. ics because of the increased number of nonimmune hosts in the
However, a significant portion of the population remains vulner- population.
able to occasional outbreaks. No change was made to immuniza- Although the clinical manifestations of H1N1 influenza seemed
tion schedules or interim recommendations after these outbreaks. to be similar to those of prior influenza outbreaks, this strain resulted
in more severe cases and higher mortality in previously healthy
Dengue young people than in typical influenza epidemics. Testing for H1N1
Dengue fever virus is a member of the Flaviviridae family and is most commonly involves antigen-based PCR methods with variable
known to occur in 4 serotypes. It is transmitted via a vector, usually sensitivities and specificities. Management of severe cases consists
A aegypti, and is present in more than 100 countries throughout the either of oral oseltamivir phosphate or inhaled zanamivir. Although
Americas, Asia, and Africa. Although historically dengue fever virus oseltamivir phosphate resistance outside the United States has been
was confined to tropical and subtropical regions roughly overlap- reported, it remains the drug of choice.
ping with malarial zones, its range is expanding. In the United States, The more recently recognized H7N9 strain seems to be more
no cases of locally acquired dengue were reported between 1946 virulent than H1N1, with a high proportion of patients presenting
and 1980. Since 1980, sporadic cases have been reported along the with severe pulmonary manifestations. In the preliminary reports
United States–Mexico border, but in 2009 to 2010 a small outbreak of the first 111 patients in China, 77% were admitted to an inten-
of locally acquired dengue occurred in Key West, Florida. During sive care unit and 28% died. Because of the ability of influenza virus
the first 8 months of 2019, 408 cases were reported in the United to change rapidly with genetic drift and shift, health officials are on
States, with 6 additional cases in US territories. The worldwide inci- alert for any increase in H7N9 activity in the fall influenza season.
dence of dengue has increased at least 4-fold in the past 3 decades The 2018 to 2019 influenza season was moderately severe, and the
for unclear reasons. 21-week season was longer than seasons from the prior 10 years.
Clinical manifestations occur over a wide spectrum, from asymp- The 2 major strains were H1N1 and H3N2.
tomatic seroconversion to severe, even fatal disease. Headache and
petechial rash are common. Classically the disease is thought to occur Chikungunya
in 3 forms: undifferentiated febrile illness, dengue fever, and den- A vector-borne disease transmitted primary by Aedes species
gue hemorrhagic fever. In reality, however, clinical manifestations mosquitoes, chikungunya was first described in Tanzania in 1953. It

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 490 PART 5: ACUTE AND EMERGENT PROBLEMS

often occurs in epidemic outbreaks rather than steady endemic pat- Bacteria: Drug-Resistant
terns and is most commonly seen in tropical Africa and Asia. More
Community-Acquired Methicillin-Resistant
recently, outbreak ranges have expanded slightly, occurring in Italy
Staphylococcus Aureus
and Madagascar. Chikungunya virus disease became a nationally
notifiable condition in 2015. A total of 156 chikungunya virus dis- Methicillin-resistant S aureus (MRSA) strains were recognized
ease cases with illness onset in 2017 have been reported from 28 US shortly after the introduction of methicillin in the 1960s and
states. All reported cases occurred in travelers returning from affected have been a substantive problem in health care settings for sev-
areas. No locally transmitted cases have been reported in the United eral decades. Health care-associated MRSA (HA-MRSA) has
States. The clinical hallmark of chikungunya fever is the presence of well-established risk factors, including exposure in the health
intense arthralgias and occasionally frank arthritis after a febrile ill- care setting (eg, hospital, nursing facility) and the presence of
ness with rash and conjunctivitis. Whereas the clinical illness of mal- comorbid medical conditions (eg, malignancy, chronic liver or
aise and fever may last days to weeks, the joint symptoms may last lung disease, indwelling catheters). In the 1990s, a new strain of
months to years. Other than the possibility of persistent and nagging community-acquired MRSA (CA-MRSA) appeared that was not
arthralgias, severity is typically mild, and fatality is rare. Management associated with these traditional risk factors, because often it
of chikungunya is generally supportive, because no specific antiviral was found in otherwise healthy individuals with no health care–
agents are available. related exposure. Furthermore, CA-MRSA carries the mecA resis-
tance gene on a type IV or V cassette chromosomes in contrast
Coronaviruses: Severe Acute Respiratory to HA-MRSA, which carries type I through III cassette chromo-
Syndrome and Middle East Respiratory Syndrome somes. Community-acquired MRSA is also more likely to con-
From 2002 to 2004, an epidemic of severe pneumonia resulting tain the Panton-Valentine leukocidin genes, which may encode
from a previously unrecognized coronavirus (ie, SARS-CoV) caused virulence factors that influence clinical symptoms. These geno-
considerable international concern because of its highly infectious typic differences have facilitated epidemiologic studies that sug-
nature and high mortality rate. Epidemiologic studies resulted in gest that CA-MRSA is a distinct MRSA strain that has increased in
identification of palm civets as the main reservoir of transmission frequency throughout the United States and is a bona fide emerg-
to humans from contact in the marketplace. Further studies sug- ing pathogen. In addition to genotypic differences, CA-MRSA is
gested that horseshoe bats were the likely natural reservoir. The epi- less likely than HA-MRSA to have a multidrug–resistant suscep-
demic, which originated in China, eventually spread to 29 countries tibility pattern. Treatment of CA-MRSA follows similar principles
with an overall mortality rate of 9.6%, which included numerous to HA-MRSA with the exception that more antibiotic choices are
health workers. Clinical features included a mean incubation period generally available. For an uncomplicated cutaneous abscess, inci-
of 4.6 days, with a presentation of severe pneumonia with a high rate sion and drainage without antibiotics is often sufficient. For deeper
of respiratory failure. Additional clinical manifestations included or more severe infections, empiric treatment with trimethoprim-
watery diarrhea and hepatitis. Common laboratory features included sulfamethoxazole, clindamycin, a tetracycline (doxycycline
lymphopenia, neutropenia, and disseminated intravascular coagula- or minocycline), or linezolid are empiric options while awaiting
tion. The cornerstone of management was supportive care. Although antibiotic susceptibilities. Although linezolid is an effective drug, it
many individuals received ribavirin, no proven role for it or any is far more expensive than the other choices. Tetracyclines should
antiviral agent existed during the outbreak. Despite the impressive not be used in children younger than 8 years. For impetigo and
nature of this epidemic, it subsided rapidly and no evidence exists other minor infections, topical mupirocin can be used.
of ongoing SARS-CoV transmission. This epidemic highlighted an
agent with high transmissibility, morbidity, and mortality but with Resistant Gram-Negative Bacteria
only a transient global impact. and Streptococcus Pneumoniae
In 2012, a second emerging coronavirus was identified as the Similar to CA-MRSA, other resistant bacteria have established signifi­
cause of a severe acute respiratory infection in a patient in Saudi cant niches. For example, S pneumoniae was historically uniformly
Arabia. From 2012 to 2013, 130 cases were reported, all of which sensitive to penicillin. Currently, penicillin- and ceftriaxone-resistant
involved direct or indirect travel or residence in 4 countries: Saudi strains of S pneumoniae are now common and circulating in the
Arabia, Qatar, Jordan, and the United Arab Emirates. The reservoir community. Similarly, several gram-negative bacteria, such as
has not been conclusively established yet, although a zoonotic ori- Escherichia coli and Klebsiella pneumoniae, are highly resistant
gin has been suggested resulting from the identification of related because of a variety of plasmid and chromosomally encoded mech-
coronaviruses in bats and camels. As of 2017, approximately 2,000 anisms, such as b-lactamases, cephalosporinases, carbapenemases,
cases have been confirmed in countries in the Arabian peninsula. porins, and efflux pumps. These strains are most common in the
The clinical features include an incubation period of 5.2 days with nosocomial setting, although community circulation of these strains
symptoms that range from none or mild to severe disease, includ- has also occurred. Although the emergence of these strains is not
ing death in 45% of reported cases. A large proportion of patients as extensive or as clearly delineated as CA-MRSA, each of these
(96%) have underlying comorbidities, and 80% required ventila- strains has similarly “emerged” to a prevalence level in the popula-
tory support. tion that substantially affects human health.

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 Chapter 68: Emerging Infectious Diseases 491

Ehrlichiosis and Anaplasmosis region (eg, Vancouver Island) in a clonal manner. The clinical pre-
Ehrlichia chaffeensis, the etiologic agent of human monocytic sentation of C gattii is similar to C neoformans, although C gattii
ehrlichiosis (HME), and Anaplasma phagocytophilum, the etiologic may be associated with an increased frequency of cryptococcoma
agent of human granulocytic anaplasmosis (HGA [formerly human in the lungs and CNS. Treatment principles are the same for both
granulocytic ehrlichiosis]), are examples of infections that were iden- species and include initial management with amphotericin B and
tified after the development of new diagnostic tests. Both infections 5-fluorocytosine for meningitis followed by consolidation ther-
were initially recognized as infections of the veterinary world until apy with fluconazole. For uncomplicated pulmonary disease, flu-
the application of molecular methods to humans with undiagnosed conazole is the cornerstone of treatment. Cryptococcus species,
febrile illnesses. These infections likely have caused human disease including gattii, infect children as well, and treatment principles
for a long time, although the incidence may have increased with the are similar to those for adults.
recent resurgence of populations of some animal reservoirs, such as
the white-tailed deer. In the early 1990s, E chaffeensis and A phago- Summary
cytophilum were identified as human pathogens that are transmit- Contrary to myopic claims that public health would conquer infec-
ted by ticks. Ehrlichia chaffeensis is transmitted by several ticks (ie, tious diseases in the 20th century, new pathogens have contin-
Amblyomma americanum, Dermacentor variabilis, Ixodes pacifi- ued to emerge and old ones have reemerged time and time again,
cus) and is found in the Southeastern and South Central United making for a challenging future of disease identification and con-
States as well as California. Anaplasma phagocytophilum is trans- trol. Transcontinental air travel has made even the most remote areas
mitted by Ixodes scapularis and is found in the northern United of the world reachable within 24 hours, bringing the distant popu-
States. Both agents cause a febrile illness with headache, myalgia, lations much closer and exponentially increasing the potential for
and malaise that is often accompanied by thrombocytopenia, leu- disease transmission and outbreak propagation. The tools used by
kopenia, and transaminitis. Rash, which occurs in 90% of subjects public health include surveillance and response; however, most of
with Rocky Mountain spotted fever (caused by Rickettsia rickettsii), the major epidemics identified in the past 20 years began with astute
is less often found with HME (31%) and rarely with HGA. Diagnosis clinical observation at the primary care level. Thus, it is essential for
of these infections can be made by PCR testing and less commonly primary care physicians and others caring for children to remain vig-
with direct microscopy because the latter methods are insensitive ilant to the constant and unpredictable nature of emerging infectious
(<10% for HME and 25%–75% for HGA). Because of the potential diseases. With astute primary care physicians, attentive scrutiny of
severity of the illness, however, if clinical suspicion is high empiric new outbreaks, and collaboration with regional and national pub-
treatment should be initiated while awaiting the diagnostic workup. lic health laboratories and officials, it is hoped that the medical field
Doxycycline is the drug of choice for management of HGA and HME. will keep pace with emerging and reemerging pathogens.
Because of a lack of reliable alternative drugs, doxycycline is recom-
mended for children younger than 8 years as well.

Fungi: Cryptococcus Gattii CASE RESOLUTION

The patient was hospitalized and underwent an extensive diagnostic workup for
Cryptococcus gattii (formerly Cryptococcus neoformans var gattii) infectious causes of fever and rash. A lumbar puncture revealed mild lymphocytic
and C neoformans are yeast that cause pneumonia and CNS infec- pleocytosis. The patient received 2 days of empiric antibiotic therapy, which was
tions in immunocompetent and immunocompromised hosts. discontinued when cultures were negative for 48 hours. The pediatrician notified
Although C neoformans is present in most regions of the world, the local health department, which facilitated laboratory testing performed by the
C gattii has a restricted geographic distribution and previously state public health laboratory, and the CDC.
Serologic testing at the state health department was positive for IgM anti-
had been identified in tropical and subtropical countries such as bodies against dengue virus. This was confirmed on samples sent to the CDC;
Australia, New Zealand, and Papua New Guinea. In the early 2000s, additionally, based on reverse-transcriptase PCR testing, CSF sent to the CDC was
C gattii was identified as a cause of meningoencephalitis for the found to be positive for dengue virus serotype 1. The patient recovered unevent-
first time on Vancouver Island in British Columbia. From 1999 to fully in the following 2 weeks, but a public health investigation was launched
2007, 218 cases were reported, with a case fatality rate of 8.7%. that eventually resulted in the identification of 27 total cases of dengue fever
acquired in Key West. Subsequently, an adult serosurvey was conducted indicat-
Subsequent studies identified its presence in the Pacific Northwest,
ing recent exposure to dengue in 5.4% of the adults studied.
including the states of Washington and Oregon. Similar to many This outbreak, which occurred in 2009 to 2010, represented the first reported
emerging pathogens, the increased number of cases may be the cases of dengue fever acquired in Florida since 1934. Although dengue is the
result of improved diagnostics and surveillance as opposed to the most common virus transmitted by mosquitoes in the world, no cases had been
actual emergence of a new infection to a region. Some molecu- acquired in the continental United States between 1946 and 1980 and, subse-
lar evidence suggests that the strain on Vancouver Island is novel, quently, only sporadic cases were known along the United States (specifically,
Texas)–Mexico border. Reported dengue cases have increased 4-fold in Latin
however. Clonal analysis suggests that it arose from an unusual America since 1980, and incidence has risen steadily among returning travelers
type of sexual mating that generated a hypervirulent strain. This from the United States. Dengue represents a truly reemerging infectious disease,
mechanism of emergence suggests that a species endemic to an and primary care physicians should be aware of its rising incidence.
original location (eg, tropics) can emerge in a new geographic

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 492 PART 5: ACUTE AND EMERGENT PROBLEMS

Feikin DR, Lezotte DC, Hamman RF, Salmon DA, Chen RT, Hoffman RE.
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 CHAPTER 69

Febrile Seizures
Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD

CASE STUDY
A 12-month-old girl is brought to the emergency depart- abnormality other than her unresponsive mental status
ment by paramedics because she is having a seizure. She is an inflamed and bulging right tympanic membrane.
is unresponsive and hypertonic, with arched trunk and The girl’s parents tell you that she has had a mildly
extended arms and legs that are jerking rhythmically. stuffy nose for 2 days but has been afebrile and has
Her eyes are open, but her gaze is directed upward. She seemed to be her usual self. While she was playing she
has bubbles of saliva around her lips as well as circum- became irritable, and her parents put her in her crib for
oral cyanosis. Her vital signs are a respiratory rate of her nap. Thirty minutes later they heard grunting noises,
60 breaths/minute, heart rate of 125 beats/minute, found her in the midst of a seizure, and called the para-
blood pressure of 130/78 mm Hg, and temperature medics. This is the girl’s first seizure. Her father recalls
of 41.0°C (105.8°F). An assessment of her respiratory that his mother once told him that he had several “fever
status shows that she is moving air in all lung fields, and seizures” as an infant.
no evidence exists of upper airway obstruction.
The paramedics inform you that the girl has been
Questions
1. What are the characteristics of simple febrile
convulsing with varying intensity of tone and move-
seizures versus complex febrile seizures?
ments but has remained unresponsive for approxi-
2. What is the appropriate evaluation of the child with
mately 6 minutes. Glucometer testing reveals a normal
febrile seizure, whether it is the first or a recurrence?
serum glucose level. Blood samples for other tests are
3. What is the recurrence risk for febrile seizure and
sent to the laboratory, and urine is collected. An intra-
the risk of developing unprovoked seizures after a
venous (IV) line is started, and the girl is given loraze-
febrile seizure?
pam by IV push. Within 2 minutes the movements cease,
4. What are the management options for the child
and her respirations become slow and even. No signs of
with febrile seizure?
trauma are evident on physical examination. Her only

Febrile seizures are easily recognized, dramatic, generalized con- younger than 1 year, drops to 25% between 1 and 3 years of age, and
vulsions. A febrile seizure is defined by the presence of a fever or falls to 12% after age 3 years. Seizures are associated with a higher
an acute inflammatory illness (often sudden) from a source out- maximum temperature and may occur with the rise in temperature
side the nervous system; patient age of approximately 5 years or and often so suddenly that the febrile illness is not recognized by
younger; absence of chronic brain pathology, including devel- the family prior to the seizure. Frequently, the febrile illness eventu-
opmental delay; absence of metabolic or structural abnormal- ally is diagnosed as an upper respiratory tract or influenza infection
ities of the brain; and absence of previous nonfebrile seizures. or follows immunization. Human herpesvirus 6 infections may be
Frequently, familial predisposition to similar seizures or a history associated with one-third of first-time febrile seizures and a some-
of similar events in other family members is present. Despite the what higher rate of complex febrile seizures. Febrile seizures often
relatively uniform presentation of the seizure, other factors, such as occur in children with a first-degree relative who experienced the
genetic abnormalities in channels, neurotransmitter receptors, or problem at the same age.
hippocampal damage may influence prognosis, and individual clin-
ical variables and social factors may influence management. Clinical Presentation
A simple febrile seizure is characterized by a single episode of gener-
Epidemiology alized, symmetric, tonic posturing and clonic movements of a few
Febrile seizures occur in children between 6 months and approx- minutes’ duration that occurs suddenly in the child whose develop-
imately 5 years of age, but they are more common in children mental progress is generally normal. Fever or an acute inflammatory
younger than 3 years. Some studies indicate that as many as illness is present, although it may not have been recognized before
5% of all children in the United States experience at least 1 febrile the seizure, and its source is outside the nervous system. A short
seizure, and the prevalence is higher in the Asian population (eg, time after the seizure (typically after 1–2 hours of postictal sleep-
6%–9% in Japan). The recurrence rate is 30% to 50% in children iness), the child returns to a normal neurologic state (Box 69.1).

495

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 496 PART 5: ACUTE AND EMERGENT PROBLEMS

fever-induced change in the status of a dynamic membrane receptor

Box 69.1. Diagnosis of Febrile Seizure
system, producing reduced numbers of available GABAA receptors.
in the Pediatric Patient
In other families with GEFS+, it has been found that mutations
ww Sudden unresponsiveness, tonic posturing, and generalized rhythmic in the SCN1A (sodium voltage-gated channel alpha subunit 1) gene
jerking have occurred. Mutations in this same gene may occur in sequences
ww Fever or acute inflammatory illness source outside nervous system that encode for the critical “pore” region of the subunit protein that
ww Age 6 months to approximately 5 years participates in ion selectivity and results in a more severe pheno-
ww Normal neurodevelopmental history type with early prolonged resistant seizures and later, intellectual
and developmental disability and Dravet syndrome. A less severe
GEFS+ phenotype is associated with a less functionally critical posi-
The recently described generalized epilepsy with febrile seizures tion of the missense mutation in the SCN1A gene. A rat model of
plus (GEFS+) is characterized by the association of generalized hyperthermic seizures has a comparable mutation and may provide
febrile seizures after age 6 years and afebrile generalized convul- additional clues related to pathophysiology.
sions, a positive family history of epilepsy with variable phenotypes,
and a benign prognosis in most cases. The same families may have Differential Diagnosis
simple febrile seizures, febrile seizures after age 6 years, febrile
When a young child presents with a fever and a seizure, the pos-
seizures and absences, atonic or myoclonic seizures, and myoclonic-
sibility that the seizure is symptomatic of meningitis, encephalitis,
astatic epilepsy or Dravet syndrome.
or brain abscess must be considered, although other clinical signs
are nearly always present. Signs of meningeal irritation may not be
Pathophysiology reliable in infants younger than 12 months, however, and these and
The susceptibility of young children to febrile seizures may be related other signs of the illness may be obscured in the postictal period.
to an increased incidence of sudden high fevers in this age group, a If a history of lethargy or persistent vomiting exists; the seizure is
developmental genetic factor that may lower the seizure threshold, focal in onset or prolonged or multiple seizures occur; or the post-
or both. Animal models indicate that the immature brain has a lower ictal depression is prolonged, an examination of the cerebrospinal
seizure threshold than the adult brain, and seizures in the immature fluid (CSF) should be done. In addition, if the patient is deficient
brain are more likely to occur by a mechanism different from that in in Haemophilus influenzae type b or Streptococcus pneumoniae
the adult. Febrile seizure has a mild association with prenatal nic- immunizations or the patient has been pretreated with antibiot-
otine exposure, iron insufficiency, and atopic conditions. A sudden ics, which could mask meningeal signs or symptoms, a CSF exam-
increase in temperature to a sufficiently high level can provoke sei- ination should be considered. It is rare that a febrile seizure would
zure regardless of age. Cultured neurons subjected to hyperthermia cause CSF pleocytosis (>10–20 white blood cells/mm3). The fever
show epileptiform activity. Seizures occur more frequently with fever also could be provocative or coincidental to a seizure of different
in many seizure-prone patients who have seizures of different etiolo- etiology, such as trauma, toxic ingestion, metabolic derangement,
gies, including genetic epilepsies. It may be that circulating pyrogens degenerative or neurocutaneous disorder, or stroke.
or interleukins interact with a brain cellular circuitry mechanism A useful concept for the physician caring for a child with a
or susceptible ion channels, causing hypersynchronous depolariza- febrile seizure has been the differentiation of simple febrile sei-
tion and seizure. The commonly noted multigenerational familial zure from complex febrile seizure (Table 69.1). A question with
history is suggestive of a dominantly expressed genetic transmis- prognostic implications is whether a child in the appropriate age
sion with reduced penetrance or a polygenic model. Different fam- range has had a true febrile seizure or a seizure with fever, which
ilies have different loci linkages. may be an early fever-provoked episode of a nonfebrile seizure
The precise definitions of the genetic markers and mechanisms of disorder. The factors that define a complex febrile seizure also pre-
generalized seizures in all cases are not yet fully understood. Recent dict an increased likelihood of later unprovoked nonfebrile sei-
work suggests possible mechanisms. Mutations in γ-aminobutyric zures. A complex febrile seizure may last 15 minutes or longer.
acid (GABA)A receptor genes have been found to be associated with Most febrile seizures are shorter than 90 seconds, although a sig-
febrile seizures and GEFS+ pedigrees. The GABAA receptors transmit nificant number present in status epilepticus. Recurrent febrile
inhibitory signals and are composed of combinations of 5 subunits. seizures are considered complex if more than 1 febrile seizure
The γ2 subunit is critical for receptor trafficking, clustering, and syn- occurs during the same infectious illness or during the first
aptic maintenance, and mutations in this subunit have been asso- 24-hour period following the initial seizure. Febrile seizures are
ciated with febrile seizure families. It has also been found that the also complex if there is a history of a focal or partial onset or the
trafficking of mutant receptors with γ2 subunit mutations was more presence of postictal focal neurologic signs. An abnormal neu-
highly temperature dependent than mutations of other subunits and rodevelopmental history prior to the febrile seizure or abnormal
impaired membrane insertion or accelerated receptor endocytosis neurologic examination or brain imaging study before or after
when brief increases of 37°C to 40°C (98.6°F to 104°F) occurred. In the seizure also is indicative of an increased likelihood of later
families with these mutations, febrile seizures may be caused by a unprovoked seizures.

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 Chapter 69: Febrile Seizures 497

Table 69.1. Simple Versus Complex patient remains ill-appearing after the postictal period, cultures
Febrile Seizures should be obtained and metabolic and toxicologic blood and urine
studies sent. A lumbar puncture for CSF examination for meningitis
Feature Simple Complex
or encephalitis should be done unless signs exist of increased intra-
Onset of clonic movements Generalized Focal cranial pressure or a lateralized neurologic examination, in which
Length <15 minutes (usually ≥15 minutes case antibiotics should be given and an imaging study obtained prior
<90 seconds) to the lumbar puncture.
Number of seizures per 1 Recurrent If the seizure is a simple febrile seizure, without history or signs
24-hour febrile illness of dehydration, blood tests are of low yield. Likewise, electroen-
Neurodevelopmental Normal Abnormal cephalography has limited usefulness. The record is often abnor-
history mal in a nonspecific way and not helpful in predicting future simple
febrile seizures or epilepsy. If the patient does not fully recover after
Parent or sibling history of Often positive Often negative
the postictal period, electroencephalography may be useful to help
febrile seizure
define the nature of the encephalopathy.
Genetic testing for SCN1A mutations may be considered for the
child with GEFS+.
Evaluation
History Imaging Studies
Computed tomography and magnetic resonance imaging have a low
After the seizure has been controlled and the child has been stabi-
yield of abnormal results in children with simple febrile seizures.
lized, a more detailed history relating to the circumstances of the
However, for the child with a persistently abnormal neurologic exam-
seizure should be obtained, including the child’s state leading up to
ination or signs of increased intracranial pressure, or with an abnor-
the seizure; prenatal, birth, and developmental histories; and fam-
mal neurodevelopmental history or a focal or partial onset to seizure,
ily seizure history (Box 69.2).
an imaging study should be performed to detect a structural lesion
Physical Examination that may be acute and the source of the present seizure and that may
The child should be examined thoroughly after stabilization, serve as a nidus for future seizures.
noting the possibility that the fever may be coincidental and signs
from an unrelated cause inciting the seizure could be present. The
Management
physician should look for bruising, fracture, retinal hemorrhage, and If the child is still convulsing on presentation and has been for
other signs of trauma. The presence of dysmorphic features, enlarged at least 5 minutes, the condition should be managed as for status
organs, or bony changes should be noted. The skin should be exam- epilepticus (see Chapter 131). The airway must be secured, blood
ined for abnormal, pigmented, or textured spots. Lateralized signs drawn and sent to the laboratory for testing, an intravenous line
of tone or strength should be assessed. An appropriate examina- started, and lorazepam administered in the appropriate dose to
tion to determine the etiology of the fever should also be performed stop the seizure.
(see Chapter 67). Meningismus, bulging fontanelle, and prolonged If the child is not in status epilepticus, treatment decisions are
postictal drowsiness should prompt consideration of meningitis or made based on a more long-term outlook (Box 69.3). Whether
encephalitis. or not to recommend anticonvulsant prophylaxis for the child
who has experienced febrile seizures is controversial. Factors
Laboratory Tests that must be considered include the benign, age-limited nature
If the seizure is prolonged, focal, or multiple; if a history exists of of the condition; the morbidity of the anticonvulsant treatment;
lethargy, stupor, or persistent vomiting before the seizure; or if the the chance of recurrence of febrile or nonfebrile seizures; the risk

Box 69.2. What to Ask Box 69.3. Treatment Options for the Pediatric
Patient With Febrile Seizure
Febrile Seizure in the Pediatric Patient
ww What were the child’s symptoms for the few days before the seizure? ww Cooling measures during febrile illness (ie, antipyretic agents or bathing
ww Where was the child, and what was the child doing immediately before in tepid water)
the seizure? ww Family reassurance and education
ww Were there any pregnancy-related or perinatal complications? ww Diazepam, 0.3 mg/kg orally or 5–10 mg rectal gel (eg, Diastat) every
ww Has the child’s development been normal or similar to that of 8 hours (during febrile illness only)
siblings? ww Phenobarbital, 3–5 mg/kg orally daily, for prophylaxis
ww Have any other family members had seizures of any kind, including ww Valproic acid (divalproex sodium), 30 mg/kg orally divided twice daily,
during infancy? for prophylaxis

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 498 PART 5: ACUTE AND EMERGENT PROBLEMS

of overmedication during an acute recurrence; and the family’s febrile seizure recurrence. The risk of febrile seizure recurrence
reaction and social disruption caused by the seizures. Given the is most dependent on age: 50% of infants younger than 1 year
combination of these factors, particularly the weight of the first 2, at the time of their first seizure will have a recurrence, but only
most physicians do not recommend antiepileptic drug prophy- 20% of children older than 3 years will have a recurrence. Other
laxis for febrile seizures. factors that have a lesser influence on the recurrence risk include
Daily dosing of phenobarbital or valproic acid is an effective form family history of febrile seizures but not epilepsy, temperature at
of anticonvulsant prophylaxis. The most commonly used regimen is the initial seizure, time since the previous seizure, and history of
daily phenobarbital; however, the potential side effects of this agent previous recurrences.
include attention-deficit/hyperactivity disorder and depressed cog- Animal models suggest that in the immature brain, seizures—
nition and learning. Valproic acid can produce thrombocytopenia even status epilepticus episodes—are less often associated with
and may have the potential of provoking acute liver dysfunction in a neuronal death; however, seizures, particularly hyperthermic sei-
patient younger than 2 years who is taking other medications. Fever zures, can modify brain development by altering cortical motor
control measures should be instituted to make the patient more com- maps and the expression of ion channels and inhibitory receptor
fortable, but these have not been found to be effective as preven- subunits that regulate neuronal excitability. Additionally, epilep-
tion for seizures. Intermittent anticonvulsant therapy with diazepam togenic mechanisms may be more robust in the immature brain
or clobazam has the advantage of reducing (but not always elimi- than in the adult brain. However, children with febrile convul-
nating) the side effects of hyperactivity, lethargy, ataxia, and seda- sions have shown no difference in later academic progress or
tion; however, this regimen is reliant on recognizing the fever before behavior compared with control children.
the seizure and mandates greater vigilance for compliance during Evidence suggests that complex febrile seizures are associated
each fever. Additionally, the evidence of this approach for reduc- with temporal lobe epilepsy in some cases. A history of prolonged
ing recurrence risk has been shown to be inconsistent at different complex febrile seizures is reported in 30% of patients with mesial
ages. Because prolonged febrile seizures tend to recur as repeated temporal sclerosis (MTS) who underwent surgery for intractable
prolonged seizures, such patients may benefit from the availability temporal lobe epilepsy; this is in contrast to only 6% of patients
of rectal diazepam or intranasal midazolam to administer at home with complex febrile seizures who did not have MTS. It is pos-
as an abortive drug to stop the seizure earlier in its course while sible that MTS is the consequence of prolonged febrile seizure
paramedics are being called. when the hippocampus is developmentally vulnerable. Mesial tem-
Recommendations for prophylactic anticonvulsant treatment poral sclerosis is frequently unilateral and focal complex febrile
are often individualized. Anticonvulsants usually are not recom- seizures originate in the temporal lobe in some children; additional
mended unless the child has presented in status epilepticus, has evidence is suggestive of causality. An alternative explanation is
experienced marked respiratory compromise (perhaps needing ven- that some preexisting hippocampal malformation, genetic predis-
tilatory support) during the seizure, or has had complex febrile sei- position, or subsequent damage may be present in the patient with
zures. No definitive evidence exists that anticonvulsant prophylaxis hippocampal sclerosis and prolonged febrile seizures and that the
for simple febrile seizures prevents the development of unprovoked complex febrile seizure is a collateral phenomenon. Ten percent of
seizures. The child who has had frequently recurring seizures that children who present with febrile status epilepticus have evidence
are extremely disruptive for the family and deleterious to parent- of hippocampal malrotation.
child interactions despite educational efforts by medical caregivers A small percentage of infants with febrile seizures may develop
may also be a candidate for prophylactic anticonvulsant treatment, Dravet syndrome, a phenotype influencing neurocognition as well
including the use of oral or rectal diazepam (eg, Diastat) every as later medication-resistant epilepsy, if they carry a particular
8 hours during the febrile illness. channel subunit gene mutation. Additionally, a quite rare con-
sequence of prolonged focal febrile seizures is hemiconvulsion-
Prognosis hemiplegia-epilepsy syndrome, which is characterized by
unilateral swelling and later cerebral hemiatrophy of the epileptic
Febrile seizures are a common age-limited problem. The progno-
hemisphere.
sis for children with simple febrile seizures is generally good and
not associated with permanent neurologic deficits; the incidence
of seizure episodes later in life is 3 to 6 times higher than in the
general population at the same age but is still low (2% to 3%).
Patients with complex febrile seizures have a higher likelihood
CASE RESOLUTION
of developing a nonfebrile seizure disorder (ie, epilepsy) com- The girl had a somewhat prolonged simple febrile seizure, a diagnosis that
was supported by a positive family history for febrile seizures. Her family is
pared to patients with simple febrile seizures; however, this risk educated about treatment options. They are comfortable with a decision to
is only 6% if 2 of the first 3 factors listed in Table 69.1 are pres- use only rectal diazepam for a subsequent febrile seizure lasting longer than
ent or 17% if the patients have neurodevelopmental abnormality. 5 minutes.
Overall, one-third of children with febrile seizures experience a

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 Chapter 69: Febrile Seizures 499

Selected References Kanai K, Hirose S, Oguni H, et al. Effect of localization of missense mutations in
SCN1A on epilepsy phenotype severity. Neurology. 2004;63(2):329–334 PMID:
American Academy of Pediatrics Subcommittee on Febrile Seizures. 15277629 https://doi.org/10.1212/01.WNL.0000129829.31179.5B
Neurodiagnostic evaluation of the child with a simple febrile seizure. Pediatrics. Kang JQ, Shen W, Macdonald RL. Why does fever trigger febrile seizures?
2011;127(2):389–394 PMID: 21285335 https://doi.org/10.1542/peds.2010-3318 GABAA receptor 2 subunit mutations associated with idiopathic gen-
Auvin S, Bellavoine V, Merdariu D, et al. Hemiconvulsion-hemiplegia-epilepsy eralized epilepsies have temperature-dependent trafficking deficiencies.
syndrome: current understandings. Eur J Paediatr Neurol. 2012;16(5):413–421 J Neurosci. 2006;26(9):2590–2597 PMID: 16510738 https://doi.org/10.1523/
PMID: 22341151 https://doi.org/10.1016/j.ejpn.2012.01.007 JNEUROSCI.4243-05.2006
Baram TZ, Shinnar S, eds. Febrile Seizures. San Diego, CA: Academic Press; 2002 Korff CM, Nordli DR Jr. Epilepsy syndromes in infancy. Pediatr Neurol.
Cendes F. Febrile seizures and mesial temporal sclerosis. Curr Opin Neurol. 2006;34(4):253–263 PMID: 16638498 https://doi.org/10.1016/j.
2004;17(2):161–164 PMID: 15021243 https://doi.org/10.1097/00019052- pediatrneurol.2005.08.005
200404000-00013 Mewasingh L. Febrile seizures. Clin Evid. 2006;(15):415–422 PMID: 16973016
Jensen FE. Pediatric epilepsy models. Epilepsy Res. 2006;68(1):28–31 PMID: Mewasingh LD. Febrile seizures. BMJ Clin Evid. 2010;11:324 PMID: 21406130
16377142 https://doi.org/10.1016/j.eplepsyres.2005.09.013 Nelson KB, Ellenberg JH. Febrile Seizures. New York, NY: Raven Press; 1981

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 CHAPTER 70

Respiratory Distress
David B. Burbulys, MD

CASE STUDY
A 6-month-old boy has been coughing and breath- Questions
ing fast for the past day. This morning he refused feed- 1. What are the causes of respiratory distress in infants
ing and has been irritable. On examination, the infant and children?
is fussy. He has an oxygen saturation of 92%, a respira- 2. What are the signs and symptoms of respiratory
tory rate of 60 breaths per minute, a pulse of 140 beats distress in infants and children?
per minute, and a normal blood pressure and temper- 3. What are the signs and symptoms of impending
ature. Additionally, he has nasal flaring, intercostal and respiratory failure in infants and children?
supraclavicular retractions, and occasional grunting. 4. What are the critical interventions for infants and
children in respiratory distress?

Respiratory distress and respiratory failure may cause significant pathology. Similarly, hypoxia that does not improve with sup-
morbidity and mortality in infants and children. The signs and plemental oxygen may be suggestive of a primary cardiac lesion.
symptoms of respiratory compromise may be subtle, particularly Signs of poor oxygenation include alterations in mental status, head
in small infants and early on. Decompensation may occur rapidly bobbing, and change in skin color. Pallor, mottling, and cyanosis
if ventilation or oxygenation is inadequate but may be prevented by are often late signs of respiratory failure and shock. The child with
prompt recognition and management. Respiratory distress is defined severe hypoxemia may initially appear dusky or pale. If the child is
as increased work of breathing, and it usually precedes respiratory anemic, cyanosis may not be evident even in the presence of low
failure. Respiratory failure occurs when ventilation or oxygenation oxygen saturation (Box 70.2).
is insufficient to meet the metabolic demands of the tissues (ie, oxy-
genation of the blood is inadequate or carbon dioxide is not elimi- Pathophysiology
nated). Respiratory failure may be caused by diseases of the airway, The adequacy of respiration depends on the ability to move an ade-
inadequate gas exchange in the lungs, or poor respiratory effort quate volume of gas in and out of the airways as well as effective
(Box 70.1). Respiratory failure may result in cardiopulmonary arrest gas exchange of carbon dioxide and oxygen. Infants and children
if not corrected promptly. generally breathe with minimal effort. In very young children, the
diaphragm and abdominal musculature are primarily used for ven-
Epidemiology tilation, and the tidal volume is approximately 6 to 8 mL/kg. If the
Primary care physicians frequently care for children in respira- tidal volume is decreased because of obstruction, children compen-
tory distress in offices and emergency departments. Respiratory sate by increasing the respiratory rate, thus attempting to maintain
distress remains the most common reason for hospital admission. adequate minute ventilation (minute ventilation = rate × tidal vol-
Such admissions usually involve young infants with acute infec- ume). If the minute ventilation remains insufficient for adequate
tions, such as bronchiolitis or croup. Reactive airways disease (eg, gas exchange or the child can no longer sustain the increased work
asthma) is a common reason for respiratory distress-related admis- of breathing, respiratory failure ensues. Respiratory failure may
sion in older children. then result in acidosis, myocardial dysfunction, and shock and may
progress to complete cardiopulmonary arrest.
Clinical Presentation Infants and children are more prone than adults to respiratory
Increases in respiratory rate and work of breathing are the most distress because of the differences between their respiratory sys-
common signs of respiratory disease. Tachycardia is often present; tems (Box 70.3).
the presence of bradycardia, however, may be an ominous sign of
impending cardiopulmonary failure and arrest. Effortless tachypnea Differential Diagnosis
(ie, Kussmaul breathing) may be a sign of respiratory compensa- The differential diagnosis of children with respiratory distress
tion for metabolic acidosis rather than an indication of pulmonary can include abnormalities with the pulmonary, cardiovascular,

501

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 502 PART 5: ACUTE AND EMERGENT PROBLEMS

nervous, or metabolic systems. It is also important to make an ini-

Box 70.1. Common Causes of Respiratory
tial differentiation between upper and lower airway disease based
Distress in Infants and Children
on the presence or absence of stridor, rhonchi, rales, or wheezes
Upper Airway on examination. Many common causes are listed in Box 70.1.
ww Aspirated foreign body
ww Croup Evaluation
ww Epiglottitis History
ww Anaphylaxis
A brief history should be obtained while concomitant physical exam-
ww Airway anomalies or immaturity
ination proceeds and initial treatment is begun (Box 70.4).
Lower Airway
ww Reactive airways disease (eg, asthma) Physical Examination
ww Bronchiolitis Before a complete assessment can proceed, critical interventions
ww Pneumonia that may change a child’s clinical status should be undertaken. The
ww Pulmonary edema child should be placed in a position of comfort if possible, and
Metabolic oxygen should be applied. Ventilation and oxygenation should be
ww Acidosis assessed. Nasal and/or oropharyngeal suctioning should be done
ww Anemia if necessary.
Cardiac
ww Congenital heart disease Box 70.3. Comparison of Respiratory
ww Congestive heart failure Systems in Children and Adults
ww Dysrhythmia
ww The head in children is proportionally larger and has less muscular
ww Pericarditis or tamponade
support.
Neurologic ww The tongue in children is larger in relation to the mouth, is poorly
ww Central controlled, and can cause airway obstruction.
ww Peripheral ww The airway diameter is smaller in children and collapses easily.
ww Neuromuscular Reductions in size resulting from secretions or inflammation cause
Traumatic greater resistance to air flow (resistance is proportional to 1/radius4).
ww Chest wall injury ww The larynx is higher and more anterior in children and the epiglottis is
ww Pneumothorax, hemothorax, or pulmonary contusion floppy, which makes visualization of the vocal cords more difficult.
ww Submersion injury ww The narrowest part of the airway in children is at the cricoid ring, unlike
ww Smoke inhalation in adults, in whom the narrowest point is at the vocal cords.
ww Toxin exposure or ingestion ww The trachea in children is short. In newborns it is 4 cm long; in
18-month-old infants, 7 cm long; and in adults, 12 cm long.
ww The major muscle of respiration in children is the diaphragm. Any
Box 70.2. Respiratory Distress interference with diaphragmatic motion in young children impedes
respiratory function. Intercostal muscles are immature in children
ww Increased respiratory rate and fatigue easily.
ww Poor feeding ww Children have less pulmonary reserve and higher metabolic demands.
ww Inability to speak in sentences ww Normal respiratory rates are higher in children and vary by age.
ww Changes in tidal volume or minute ventilation
ww Nasal flaring
ww Presence of retractions: intercostal, substernal, diaphragmatic,
Box 70.4. What to Ask
or supraclavicular
ww Changes in inspiratory-expiratory ratio Respiratory Distress
ww Production of sounds with respiration (eg, grunting, gurgling, stridor, ww What was the onset of this problem like, and how has it progressed?
rhonchi, rales, wheezes) ww For how long has it been occurring, and has a similar problem ever
ww Diaphoresis occurred before?
ww Decreased or absent breath sounds ww Did the problem begin while the child was eating or playing?
ww Presence of pale or cyanotic skin ww Has the child had any recent infections or fever?
ww Presence of central cyanosis ww Are any members of the household ill?
ww Alterations in mental status ww Is the child taking any medications?

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 Chapter 70: Respiratory Distress 503

Respiratory and heart rates should be determined for a period muscles of respiration are used, breathing is abnormally noisy, or
of at least 30 seconds. In the infant, abdominal excursions should nasal flaring is seen. The normal work of breathing consumes 2% to
be counted; in the older child, chest excursions should be counted. 3% of total oxygen consumption. The increased work of breathing
Respiratory rates in children are higher than in adults; infants may in the child with severe respiratory distress can potentially increase
breathe 40 times per minute, 1-year-olds 25 times per minute, and total oxygen consumption to 50%. Increased work of breathing can
10-year-olds 18 times per minute (Table 70.1). These rates vary with also be manifested by feeding difficulties and diaphoresis in infants
age and changes of activity, emotion, and illness. Abnormal respi- and young children.
ratory rates are defined as being faster than normal (ie, tachypnea), Additionally, the physician should observe the inspiratory-
slower than normal (ie, bradypnea), or absent (ie, apnea). The neo- expiratory ratio while assessing the work of breathing. The ratio is
nate may exhibit periodic breathing, with periods of regular respi- approximately 1:1 in most patients. Prolonged expirations are most
rations alternating with irregular breathing. This is a normal variant often noted with reactive airways disease.
for age. True apnea (ie, cessation of respiration) is accompanied by Oxygen saturation should be measured by pulse oximetry in
change in skin color or muscle tone and may be accompanied by every child with respiratory symptoms. Levels below 93% while
bradycardia or altered level of consciousness. awake are indicative of significant hypoxemia.
The depth of respiration should be noted. Whether breaths are
Laboratory Tests
deep, gasping, or shallow should be determined. Rapid, shallow res-
pirations may not provide enough inspiratory time for adequate gas Although the physical examination is the most important tool for
exchange. The heart rate may also reflect respiratory compromise. assessing children in respiratory distress, laboratory tests such as
Breath sounds should first be listened to in the axillae and then at the respiratory viral and bacterial respiratory panels using polymerase
bases and apices. The absence of breath sounds may be an ominous chain reactions and other methodologies, complete and differen-
sign. Children’s breath sounds are usually well transmitted across the tial blood cell counts, and blood cultures may help in the diagnosis
thorax because of the thin chest wall. It is common to hear upper of infection. Point-of-care respiratory syncytial virus or influenza
airway noises when auscultating the lungs. testing may also be beneficial during the peak seasons. It is impor-
Abnormal sounds are caused by turbulent air passing through a tant to note that meningitis, sepsis, and metabolic derangement may
narrowed airway. Resistance to flow through a hollow tube increases present with effortless tachypnea not associated with increased work
to the fourth power. Thus, the smaller the airway, the greater the of breathing. Arterial or venous blood gases may be beneficial in this
resistance to flow generated by even small changes in the radius (as situation; however, generally these should be reserved for patients
with edema, secretions, or foreign bodies). The nature of the sounds in impending respiratory failure.
produced depends on the location of the narrowing in the airway. Peak expiratory flow or forced expiratory volume in 1-second
Gurgling, snoring, and stridor arise from the upper airway; rales, determinations can be helpful in assessing the compliant older child
rhonchi, and wheezing arise from the lower airway. If no abnormal with reactive airways disease. Oxygen saturation measurements can
sounds are evident and breath sounds are absent or decreased, the also be helpful in these children, because reduced levels (<100%)
upper or lower airways may be totally obstructed. Grunting is caused often correlate with the severity of disease.
by turbulent air contacting a partially closed glottis. The child who
Imaging Studies
grunts is generating partial obstruction of the upper airway and pos-
itive end-expiratory pressure to increase oxygenation. Chest radiography can aid in assessing the child in respiratory
The physician should also observe the effort the child expends distress, but such imaging should not be routinely obtained in
in breathing. Increased work of breathing occurs when intercostal, patients with known reactive airways disease unless the child has a
subcostal, or supraclavicular retractions are present, the accessory fever or is in status asthmaticus. Anteroposterior and lateral radio-
graphs of the neck may also be beneficial in the patient with stridor.
A patient with significant respiratory distress should not be moved
Table 70.1. Vital Signs by Age
from a monitored setting to the radiology suite; rather, portable
Systolic Blood radiographs should be obtained, if necessary.
Respiration Pressure
Age (breaths/minute) Pulse (beats/minute) (mm Hg) Management
Newborn 30–60 100–160 50–70
All infants and children in respiratory distress should be managed
1–6 weeks 30–60 100–160 70–95 emergently. As stated previously, in such situations assessment and
6 months 25–40 90–120 80–100 intervention often occur simultaneously. All children in respiratory
1 year 20–40 90–120 80–100 distress should be reassessed frequently. Initially, the highest pos-
3 years 20–30 80–120 80–110 sible oxygen concentration should be delivered. Children who are
able to maintain their own airway should never be forced to use an
6 years 12–25 70–110 80–110
airway adjunct, because this may cause increased anxiety and dis-
10 years 12–20 60–90 90–120 tress. The patient with clear airways can be maintained with simple

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 504 PART 5: ACUTE AND EMERGENT PROBLEMS

interventions, such as oxygen blown by the face or given by mask or

nasal prongs. Many patients with more severe croup, reactive airways
disease, bronchiolitis, or pneumonia may benefit from noninvasive
respiratory support, such as with continuous positive airway pres-
sure or a heated humidified high-flow nasal cannula. More advanced
airway management, such as bag-valve-mask ventilation or endo-
tracheal intubation, may be necessary for the child who required
assisted ventilation, airway protection, or hyperventilation.

Position
The child in respiratory distress who is alert and breathing sponta-
neously should be allowed to choose a position of comfort. Small
infants who are incapable of positioning themselves are best placed
upright with care taken not to flex or extend the neck. Children and
their caregivers should be kept together to reduce anxiety. Figure 70.1. A non-rebreathing mask, which can deliver a high concentration
The proper position for the unconscious child is the “sniffing posi- of oxygen to a patient in respiratory distress.
tion,” with the neck slightly flexed and the head extended to open the
airway. This can be facilitated by placing a towel under the occiput of
the head or shoulders. If simple positioning does not relieve an obstruc-
tion, the airway should be opened using the chin lift or jaw thrust. If
spinal trauma is a possibility, only the jaw thrust should be used. If
this is unsuccessful, airway adjuncts, such as nasopharyngeal or
oropharyngeal airways, can be placed to help prevent the soft tissue of
the oropharynx from collapsing against the posterior pharyngeal wall.

Monitoring
All infants and children in respiratory distress should be carefully mon-
itored. Pulse oximetry is helpful in determining the degree of oxygen
saturation, and cardiac and respiratory monitoring provides constant
readings of respirations and heart rate. Continuous end-tidal capnogra-
phy may be beneficial in monitoring the patient with impending respi-
ratory failure or the patient who requires ventilatory support. Frequent
assessments of the patient are critical to ensure a good outcome.
Figure 70.2. Use of a face tent.
Oxygen Administration
Oxygen should be delivered by the best method tolerated by the of allowing access to the face and mouth. A pocket mask is a small
child. The 2 advantages of nasal prongs are that they are noninva- device that can be readily used in the office setting (Figure 70.3).
sive and allow maintenance of a constant gas flow even when talking A Venturi mask is rarely used in children but has the advantage of
and eating. The concentration of oxygen delivered is limited, how- precisely titrating the oxygen concentration to be delivered from
ever, and irritation and drying of the mucous membranes may result. 24% to 60%.
Oxygen masks deliver a higher concentration of humidified oxy- Noninvasive respiratory support, such as continuous positive
gen than nasal prongs. Disadvantages include obstruction of the airway pressure or heated humidified high-flow nasal cannula, is
child’s visual field, the potential for carbon dioxide retention, and frequently used in patients with more significant disease or impend-
anxiety because the face is covered. Various types of mask are avail- ing respiratory failure with excellent results. It decreases the work
able. The simple mask can deliver 30% to 60% oxygen concentra- of breathing, increases oxygenation, and frequently serves as a
tion at flow rates of 6 to 10 L/min. Room air is drawn into the mask bridge treatment while other agents are taking effect (eg, β agonists,
through the exhalation ports in the side of the mask. A non-rebreathing steroids, antibiotics).
mask has valves that allow only oxygen (85%–95%) to flow from the The child with respiratory failure requires assisted ventilation
reservoir bag to the patient on inhalation and additional valves on with a bag-valve-mask device or endotracheal intubation. A mask
the exhalation ports of the mask that prevent entrapment of room of the proper size should be used. The upper edge of the mask
air (Figure 70.1). The face tent is a soft plastic bucket shaped to the should fit snugly over the bridge of the nose without touching the
chin that is well tolerated by children (Figure 70.2). The face tent eyes. The lower edge should rest directly on or just above the man-
allows up to 40% oxygen to be delivered, and it has the advantage dible. In the unconscious child, an oropharyngeal airway should

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 Chapter 70: Respiratory Distress 505

be inserted to prevent the tongue from obstructing the upper air-

way (Figure 70.4A). An appropriately sized oropharyngeal airway CASE RESOLUTION
should reach from the patient’s earlobe to the corner of the mouth. The fussy infant has obvious signs of respiratory distress, including tachypnea,
If the patient has an intact gag reflex, a nasopharyngeal airway may tachycardia, grunting, nasal flaring, and retractions. The differential diag-
be inserted to achieve the same goal (Figure 70.4B). The nasopha- nosis includes foreign body, infection (eg, croup, bronchiolitis), and reactive
ryngeal airway is measured from the child’s earlobe to the tip of airways disease. The infant is placed in a position of comfort seated on his
mother’s lap and provided blow-by oxygen and a treatment with albuterol. A
the nostril. Endotracheal intubation is indicated in the child who
portable chest radiograph is obtained that shows mild hyperinflation but no
requires control of the airway, needs airway protection, or requires infiltrate. His pulse oximetry reveals a level of 94%. A respiratory viral panel is
hyperventilation. obtained and the results reveal the presence of respiratory syncytial virus. The
infant falls asleep, and his respiratory and heart rates return to normal. He is
Prognosis diagnosed with bronchiolitis and discharged home, and his mother is advised
to return should he experience any further respiratory distress.
Respiratory failure and resulting cardiopulmonary arrest are pre-
ventable in most infants and children if the condition is carefully
assessed and appropriate critical interventions are implemented.
Careful attention to ventilation and oxygenation usually results in
a good outcome. Selected References
American Academy of Pediatrics. APLS: The Pediatric Emergency Medicine
Resource. AAP.org website. www.aap.org/en-us/continuing-medical-education/
life-support/APLS-The-Pediatric-Emergency-Medicine-Resource/Pages/APLS-
The-Pediatric-Emergency-Medicine-Resource.aspx. Accessed June 26, 2019
American Heart Association. PEARS. Cpr.heart.org website. https://cpr.heart.
org/AHAECC/CPRAndECC/Training/HealthcareProfessional/Pediatric/UCM_
476633_PEARS.jsp. Accessed June 26, 2019
American Heart Association. Pediatric Advanced Life Support (PALS). Cpr.
heart.org website. https://cpr.heart.org/AHAECC/CPRAndECC/Training/
HealthcareProfessional/Pediatric/UCM_476258_PALS.jsp. Accessed June 26,
2019
Choi J, Lee GL. Common pediatric respiratory emergencies. Emerg Med Clin
North Am. 2012;30(2):529–563, x PMID: 22487117 https://doi.org/10.1016/j.
emc.2011.10.009
Combret Y, Prieur G, Le Roux P, Medrinal C. Non-invasive ventilation improves
respiratory distress in children with acute viral bronchiolitis: a systematic
review. Minerva Anestesiol. 2017;83(6):624–637 PMID: 28192893 https://doi.
org/10.23736/S0375-9393.17.11708-6
Figure 70.3. A pocket mask with a one-way valve and side oxygen port that Fuchs S. Initial assessment and stabilization of children with respiratory or
can be used for assisted ventilation in the office setting. circulatory compromise. In Torrey SB, ed. Waltham, MA: UpToDate; 2017. www.
uptodate.com/contents/initial-assessment-and-stabilization-of-children-with-
respiratory-or-circulatory-compromise Accessed September 3, 2019
Kou M, Hwang V, Ramkellawan N. Bronchiolitis: from practice guideline to clin-
ical practice. Emerg Med Clin North Am. 2018;36(2):275–286 PMID: 29622322
A https://doi.org/10.1016/j.emc.2017.12.006
Slain KN, Shein SL, Rotta AT. The use of high-flow nasal cannula in the pedi-
atric emergency department. J Pediatr (Rio J). 2017;93(suppl 1):36–45 PMID:
28818509 https://doi.org/10.1016/j.jped.2017.06.006
Weiner DL. Acute respiratory distress in children: emergency evaluation and
initial stabilization. In Fleisher GR, ed. Waltham, MA: UpToDate; 2016. www.
uptodate.com/contents/acute-respiratory-distress-in-children-emergency-
evaluation-and-initial-stabilization. Accessed September 3, 2019
B
Weiner DL. Causes of acute respiratory distress children. In Fleisher GR, ed.
Waltham, MA: UpToDate; 2018. www.uptodate.com/contents/causes-of-acute-
respiratory-distress-in-children. Accessed September 3, 2019
Yuknis ML, Weinstein E, Maxey H, et al. Frequency of pediatric emergencies
in ambulatory practices. Pediatrics. 2018;142(2):e20173082 PMID: 30030368
Figure 70.4. A, Oropharyngeal airways. B, Nasopharyngeal airways. https://doi.org/10.1542/peds.2017-3082

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BPPCA6e_Ch070_0501-0506.indd 506 2/13/20 5:53 PM
 CHAPTER 71

Stridor and Croup

David B. Burbulys, MD

CASE STUDY
A 2-year-old boy has been breathing noisily for 1 day. For of 101.2°F (38.4°C). He has intercostal retractions, his
the past 3 days he has had a “cold,” with a runny nose, breath sounds are slightly decreased bilaterally, and
fever (temperature up to 100.4°F [38°C]), and slight his skin is pale. The remainder of the examination is
cough. The cough has gradually worsened and now has normal.
a barking quality.
On examination, the child is sitting up and has a
Questions
1. What is stridor?
respiratory rate of 48 breaths per minute with marked
2. What are the common causes of stridor?
inspiratory stridor and an occasional barking cough. His
3. What is the pathophysiology of viral croup?
other vital signs include an oxygen saturation of 95%,
4. How are children with croup managed?
heart rate of 100 beats per minute, and temperature

Noisy breathing is a common symptom that often accompanies Clinical Presentation

respiratory infections in children. The presence of stridor, a high-
pitched crowing sound, often concerns children’s caregivers. Some
Viral Croup
parents and guardians try home remedies to alleviate the symp- Viral croup commonly begins with 2 to 3 days of viral upper respi-
toms, whereas many others immediately seek help in the office ratory symptoms (eg, rhinorrhea, sore throat, cough) and a gen-
or emergency department setting. Croup is an inflammation of erally low-grade fever. Higher-grade fevers may occur depending
the larynx, trachea, and upper bronchioles (ie, laryngotracheo- on the causative agent. At day 3 or 4, the characteristic signs of a
bronchitis) that causes noisy breathing and stridor. It is among hoarse voice, barking cough, and inspiratory stridor manifest. This
the most common causes of a seal-like barking cough and stri- frequently occurs suddenly and often at night. Symptoms worsen
dor in children. in the next 1 to 2 days and then resolve over the next several days.

Spasmodic Croup
Epidemiology Spasmodic croup typically occurs in children 2 to 5 years of age
Croup most commonly affects children between 6 months and 3 years and often presents suddenly, commonly at night, without the pre-
of age, generally in the fall or early winter. Children younger than vious complaint of upper respiratory symptoms. As in viral croup,
1 year account for 26% of cases. Infants are frequently more severely hoarseness, barking cough, and stridor occur; however, these symp-
affected than older children. The condition is more common in toms generally are less severe in spasmodic croup. The condition fre-
boys than girls; two-thirds of all hospitalized children with croup quently resolves completely when affected children are exposed to
are boys. cool or humified air. It may be recurrent. Some children may have
Stridor, which may be indicative of croup, may also be a sign of the prodrome of a viral upper respiratory syndrome. Fever is uncom-
epiglottitis. The incidence of epiglottitis in children has dramati- mon. The etiology is unknown but is likely a reaction to a viral infec-
cally decreased since 1988 following the development and wide- tion or an allergic phenomenon. A family history of recurrent stridor
spread use of the vaccine against Haemophilus influenzae type b. in children with spasmodic croup may exist.
Many young children may be incompletely immunized, and other
bacteria exist that may cause epiglottitis; thus, epiglottitis should Bacterial Tracheitis
still be considered in toxic-appearing children with rapid onset The classic presentation of bacterial tracheitis is of a school-age child
of symptoms of upper airway obstruction. Before Haemophilus presenting with a prodrome of an upper respiratory infection. After a
influenzae type b immunization, the ratio of cases of epiglottitis few days, this is followed by the abrupt progression to toxic appear-
to croup was 1:100. Currently, epiglottitis in children is exceed- ance. A high fever, productive cough, inspiratory stridor, and tach­
ingly rare. ypnea with moderate to severe respiratory distress commonly occur.

507

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 508 PART 5: ACUTE AND EMERGENT PROBLEMS

This generally occurs as the airway becomes secondarily infected with

Box 71.1. Causes of Stridor
staphylococcal or streptococcal species. White blood cell counts are
frequently significantly elevated. Abrupt decompensation may occur Congenital Anomalies
as pseudomembranes loosen and obstruct the airway. ww Choanal atresia
ww Laryngeal web
Epiglottitis ww Laryngocele
Whereas acute bacterial epiglottitis was classically seen in young ww Laryngomalacia
children, currently it is more common in adolescents and young ww Macroglossia (Beckwith syndrome)
adults. Historically, it presented with the abrupt onset of toxic ww Subglottic stenosis
appearance, high fever, sore throat, odynophagia, and muffled ww Tracheal web or cyst
voice. Rapid progression followed with severe respiratory distress, ww Tracheomalacia
drooling, stridor, tripod positioning, and frequent airway obstruc- ww Vascular ring
tion. Modern presentations are commonly less severe or progres-
Inflammatory/Infectious Lesions
sive because of the change in pathogens. Sore throat, odynophagia,
ww Abscess (ie, retropharyngeal, peritonsillar, parapharyngeal)
muffled voice, and significant tenderness to palpitation of the lar-
ww Angioedema
ynx frequently occur, but currently, toxic appearance and progres-
ww Bronchitis
sion to airway obstruction are uncommon.
ww Diphtheria
Foreign Body ww Epiglottitis
ww Infectious mononucleosis
Laryngotracheal foreign body aspiration may occur as a witnessed
ww Severe tonsillitis
event. Choking, coughing, gasping, and stridor frequently occur,
ww Tracheitis
and the patient may present with these symptoms or they may
ww Viral croup
have resolved by the time the patient is seen in the doctor’s office.
Presenting symptoms of foreign bodies in the lower larynx include Trauma
dysphagia, gagging, and throat discomfort. Presenting symptoms ww Direct trauma to the upper airway
of foreign bodies in the trachea include coughing, wheezing, and, ww Postintubation subglottic stenosis
with time, pneumonia. Neurogenic
ww Laryngeal paralysis
Pathophysiology ww Poor pharyngeal muscle tone
Stridor Caustic or Thermal Injury
Stridor is generally caused by partial obstruction of the airway ww Hot gas or liquid
between the nose and larger bronchi. Obstruction at the level of ww Lye or caustic ingestion
the nose or pharynx may produce snoring or gurgling sounds. Foreign Body
Turbulent airflow in the laryngeal area or upper trachea causes the ww Neoplasm
high-pitched crowing sound characteristic of stridor. Edema and
inflammation at the vocal cords and subglottic areas result in inspi-
ratory stridor, whereas obstruction below the cricoid cartilage may below the level of obstruction, which causes narrowing of the air-
cause inspiratory and expiratory stridor. Some of the more common way and turbulence of the airflow. This occurs more often in chil-
causes of stridor are listed in Box 71.1. dren than adults because the tracheal rings are not well formed.
The sounds produced at various levels of obstruction can give Additionally, the smaller size of the airway in children makes resis-
the primary care physician clues about the etiology of the problem. tance to airflow greater when obstruction is present.
The most common cause of stridor that begins shortly after birth Resistance increases exponentially, so the smaller the airway the
is tracheomalacia, a condition secondary to the immaturity of the greater the resistance to flow. Alterations in the diameter of the air-
cartilage of the trachea. Laryngomalacia, which is caused by floppy way are most often caused by edema and inflammation. Stridor may
supraglottic structures, resolves after several months. also occur from a variety of conditions, including congenital anoma-
The upper airway of infants and children is more susceptible to lies, infection, allergic and anaphylactic reactions, cysts, tumors, and
obstruction as the result of anatomic differences between children trauma. Even small localized areas of airway narrowing in infants
and adults. The tongues of children are relatively large, and the epi- and children can cause respiratory distress.
glottis is floppy and shaped somewhat like the Greek letter omega
(Ω). The angle between the epiglottis and glottis is more acute in Croup
children, which makes direct visualization of the airway more diffi- Croup is most often caused by an infection with parainfluenza virus
cult than in adults. Cartilaginous structures are less rigid in infants. (type 1 or 2). Several other causes include respiratory syncytial virus,
During inspiration, negative intraluminal pressure is generated influenza virus A or B, adenovirus, rhinovirus, coxsackievirus,

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 Chapter 71: Stridor and Croup 509

measles, and herpes simplex virus infections. Metapneumovirus (see Chapter 70). The stridorous sounds produced are usually
and novel coronavirus, which more commonly cause bronchiolitis, inspiratory but may be inspiratory and expiratory if the disease pro-
have also been implicated. Simultaneous infections with more than gresses to the lower airway. The presence of stridor at rest or with
1 virus are common. Particularly severe disease may be associated sleep should be assessed as well as the severity of retractions with
with influenza A, respiratory syncytial virus, or adenovirus infec- breathing. Breath sounds may be decreased bilaterally, and severe
tion. Infection occurs via respiratory droplets spread from other tachypnea, with respiratory rates from 40 to 80 breaths per minute,
infected individuals. may occur. Peripheral or central cyanosis and alterations in mental
The virus first attacks the nasopharynx and subsequently spreads status associated with severe disease should be noted. Assessment
to the larynx and upper trachea. The infection causes inflammation of croup severity may be helpful to direct initial therapy in mild,
and edema of the airway that often involves the vocal cords and sub- moderate, or severe cases as well as monitor response to treatment
glottic areas, producing the typical barking cough, hoarseness, and and predict disposition (Table 71.2).
inspiratory stridor. Uncommonly, in severe cases, the lower airways
also may be involved, resulting in impaired alveolar ventilation and Laboratory Tests
wheezing. In some children, secondary bacterial superinfection may Investigations, such as a complete blood cell count and cultures,
rarely occur with bacterial tracheitis or extension of infection to are rarely helpful unless the physician is concerned about sec-
the lower airway producing pneumonia. Airways of infants are small ondary bacterial infection. The white blood cell count may be
and particularly susceptible to obstruction because of the narrow normal or mildly elevated, and the differential count may show
subglottic region and laxity of the cartilaginous structures. a predominance of polymorphonuclear cells. Polymerase chain
reaction viral and bacterial respiratory panels may be helpful in
Differential Diagnosis more severely ill or admitted patients.
The differential diagnosis of stridor is presented in Table 71.1. Imaging Studies
Radiographs of the soft tissues of the upper airway are some-
Evaluation times helpful. In children with croup, the classic steeple sign of
History the subglottic area where the airway narrows like a church stee-
A complete history should be obtained (Box 71.2). ple or pencil tip is demonstrated on the frontal view. A lateral
neck radiograph may reveal ballooning of the hypopharynx, a nor-
Physical Examination mal epiglottis, and a normal retropharyngeal space (Figure 71.1).
It is important to assess the degree of respiratory distress and place Radiography is not recommended with a classic presentation of
children in a position of comfort; monitor heart rate, ventilation, and croup. Thickening of the epiglottis, which appears thumbprint-
oxygenation; deliver oxygen; and suction the nasopharynx if necessary shaped, and obliteration of the vallecular space may be seen on

Table 71.1. Differential Diagnosis of Stridor

Viral Croup Spasmodic Croup Bacterial Tracheitis Epiglottitis Foreign Body
Factor (Age 3–36 Months) (Age 3–36 Months) (Age 1–10 Years) (Age 1–8 Years) (All Ages)
Prodrome Upper respiratory None; sudden onset at Upper respiratory infection Usually none; rapid onset None; sudden onset
infection symptoms; night symptoms for days, followed over several hours
onset over 2–5 days by rapid-onset fever and
respiratory distress
Fever Low grade None Low grade initially, then Usually temperature above None
higher 102.2°F (39°C)
Cough Barking Barking Dry initially, then barking None or dry May or may not be present
Respiratory Present Present Absent or mild initially, Present and severe Usually present
distress then severe
White blood cell Normal or slightly Normal Normal initially, then Elevated Normal
count elevated elevated
Blood culture No growth No growth Growth uncommon Haemophilus influenzae No growth
Radiograph Subglottic narrow- Subglottic narrowing may Subglottic narrowing; Swollen epiglottis Air trapping; may demon-
ing; steeple sign or may not be present steeple sign and ragged strate a foreign body
trachea

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 510 PART 5: ACUTE AND EMERGENT PROBLEMS

head and neck specialist. If a specific cause of stridor is identi-

Box 71.2. What to Ask
fied, such as a foreign body, appropriate management should be
Stridor and Croup instituted.
ww Did the child have an antecedent respiratory infection? Children with croup should be treated with gentleness and
ww Does the child have a fever? If so, how high is the temperature? should not be upset. They should remain with their caregivers and
ww Was the onset of stridor abrupt? be allowed to assume a position of comfort. Agitation and crying
ww Is there stridor at rest/sleep or only with agitation? increase respiratory distress and oxygen demand. Procedures should
ww Has the child had stridor previously? be kept to a minimum. The heart rate, respiratory status, and oxygen
ww Does the child have any ill contacts? saturation should be continuously monitored while the patient is
ww Does the child have any associated symptoms, such as vomiting, being treated and observed. Airway adjuncts that increase agitation
diarrhea, or rash? should not be used.
ww Is the child feeding normally? Historically, cool mist has been provided by blow-by or mask.
ww Is the child drooling? Humidified oxygen or air, which is thought to decrease the vis-
cosity of secretions and reduce airway edema, had long been the
mainstay of therapy for croup. Several randomized, controlled
lateral neck radiographs in patients with epiglottitis. Widening of trials have questioned this practice, however, demonstrating no
the retropharyngeal space dorsal to the tracheal air column may benefit of cool humidified mist therapy. Consensus statements
be seen with retropharyngeal abscess. Children with respiratory now reflect this finding, and most physicians have abandoned
compromise should be evaluated in the emergency department this treatment practice. Symptoms of spasmodic croup usually
with portable X-ray equipment. resolve within 6 hours of the onset without treatment; however,
Direct visualization or imaging studies (eg, barium swallow, cool, humidified mist is still often used as the sole therapy for
computed tomography of the neck or thorax) are helpful in this disorder.
diagnosing congenital anomalies (eg, vascular ring) in atypical or Currently, treatment of croup with corticosteroids is the main-
recurrent cases. stay of therapy, and its use is less controversial than the use of cool
mist. Several studies have demonstrated that dexamethasone given
Management as a single dose of 0.6 mg/kg is effective in shortening the course
Most children with croup and stridor can be managed as outpa- and severity of mild, moderate, and severe croup if given in the first
tients. Caregivers should be given careful instructions so that they 3 days. Several smaller randomized trials have also suggested
understand the course of the illness, know what to expect, and that one-half the dose of dexamethasone may be equally effec-
realize when emergency care is needed. Children with a prolonged tive, with fewer adverse effects in patients with mild or moderate
or recurrent history of stridor may require consultation with a croup. Dexamethasone should be given orally but may be given

Table 71.2. Clinical Assessment of Croup Severity

Sign or Symptom Normal Mild Moderate Severe
Air entry Normal Mild decrease Moderate decrease Marked decrease
Color Normal Normal Cyanotic if agitated Cyanotic at rest
Level of consciousness Normal Restless when disturbed Restless when undisturbed Lethargic
Retraction None Mild Moderate Severe
Stridor None Only with agitation Moderate at rest Severe at rest
Disease Categories
Degree Management
Mild Dexamethasone 0.3–0.6 mg/kg orally
Outpatient observation
Mild to moderate Dexamethasone 0.3–0.6 mg/kg orally; consider racemic epinephrine
If child improves after observation, is >6 months of age, and has a reliable family, outpatient observation
Moderate Dexamethasone 0.6 mg/kg orally or intramuscularly; racemic epinephrine and hospital admission
Severe Racemic epinephrine, dexamethasone
0.6 mg/kg orally or intramuscularly, oxygen, and admission to intensive care unit

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 Chapter 71: Stridor and Croup 511

return or worsening of stridor. The anti-inflammatory effects of

dexamethasone take several hours for onset, and racemic epineph-
rine may have significant bridging effects in reducing symptoms
during this period.
Hospitalization is required for less than 10% of patients present-
ing to the emergency department. It should be considered for chil-
dren with an oxygen requirement, who are in moderate to severe
respiratory distress, with significant stridor at rest, who are unable
to eat, or whose parents are unable to cope with the tasks required
to manage the child at home. It is always indicated for children with
a bacterial infection, such as epiglottitis or tracheitis.
Studies are ongoing, but early results suggest that continuous pos-
itive airway pressure and heated humidified high-flow nasal cannula
noninvasive ventilation significantly improve croup symptoms,
decrease the work of breathing, and reduce the rates of respiratory
failure and need for invasive ventilation in many patients. Use of either
therapy use should be considered in most hospitalized patients.
With early steroid administration, racemic epinephrine use,
and noninvasive ventilatory support only approximately 1% of
infants and children with croup require controlled ventilation
with endotracheal intubation. Indications for intubation include
severe respiratory distress, altered mental status, hypoxia, and
hypercapnia. A major complication of endotracheal intubation in
children with croup is subglottic stenosis, which occurs because
the endotracheal tube may traumatize the inflamed airway, result-
Figure 71.1. Lateral neck radiograph of viral croup. Note the ballooning of the ing in permanent damage. Patients should be extubated as soon
hypopharynx and narrowing in the subglottic area. as possible.
Courtesy of Dr J. S. Seidel, Division of General and Emergency Pediatrics, Harbor-UCLA Direct laryngoscopy may be necessary in some children in whom
Medical Center, West Carson, California. the etiology of stridor is not clear. This procedure should be done by
a physician who is experienced in managing the airway.

as an intramuscular or intravenous injection for children who are Prognosis

unable to take oral medication or have persistent emesis. Use of Stridor is a serious sign indicative of upper airway obstruction and
the tablet preparation ground up in applesauce is often recom- potential respiratory compromise. Although it has many causes, the
mended, because the liquid preparation does not taste good and prognosis typically is determined by the rapidity of diagnosis and
is not well tolerated. Recent studies using nebulized budesonide institution of appropriate therapeutic measures, particularly stabi-
also demonstrate the same efficacy in reducing respiratory distress lizing the patient and ensuring patency of the airway. Certain condi-
and decreasing croup severity but at higher cost. Continuous or tions, such as croup, often resolve spontaneously within a few days,
long-term therapy with steroids does not seem to alter the clini- although some children require hospitalization and, rarely, assisted
cal course of the disease or the period of hospitalization and is not ventilation. Other conditions, such as foreign bodies or tumors,
recommended. Treatment with antibiotics is not indicated unless necessitate aggressive intervention to prevent death resulting from
infection with bacteria is evident. airway obstruction.
Use of racemic epinephrine has also become less controversial.
Several older studies have shown that there was no difference in the
long-term outcome between children who were treated with epi-
nephrine and those who received a placebo. More recent and robust CASE RESOLUTION
studies have clearly demonstrated clinical benefit of the drug when The 2-year-old with the antecedent infection and stridor has the classic signs
given via nebulizer. This effect lasts a few hours, and repeat doses of mild to moderate croup. Initially, adequate ventilation, oxygenation, and
circulation should be ensured. Following this, other diagnostic studies and
of the drug may be needed until the anti-inflammatory properties
specific therapy can be considered, such as nasal suctioning, dexamethasone,
of the administered steroids show clinical effect. Thus, if racemic and racemic epinephrine. A period of posttreatment observation is warranted. If
epinephrine is used on patients who are going to be discharged, his condition improves, close outpatient management may be considered.
it is prudent to observe them for 3 to 4 hours after treatment for

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 512 PART 5: ACUTE AND EMERGENT PROBLEMS

Selected References Quintero DR, Fakhoury K. Assessment of stridor in children. In: Redding G,
ed. Waltham, MA: UpToDate; 2018. https://www.uptodate.com/contents/
Bjornson C, Russell K, Vandermeer B, Klassen TP, Johnson DW. assessment-of-stridor-in-children. Updated July 9, 2018. Accessed May 6, 2019
Nebulized epinephrine for croup in children. Cochrane Database Syst Rev. Woods CR. Croup: approach to management. In: Kaplan SL, Messner AH, eds.
2013;10(10):CD006619 PMID: 24114291 10.1002/14651858.CD006619.pub3 Waltham, MA: UpToDate; 2019. https://www.uptodate.com/contents/croup-
Choi J, Lee GL. Common pediatric respiratory emergencies. Emerg Med Clin approach-to-management. Updated January 2, 2019. Accessed May 6, 2019
North Am. 2012;30(2):529–563, x PMID: 22487117 https://doi.org/10.1016/j. Woods CR. Croup: clinical features, evaluation, and diagnosis. In: Redding G,
emc.2011.10.009 Messner AH, Kaplan SL, eds. Waltham, MA: UpToDate; 2018. https://www.
Everard ML. Acute bronchiolitis and croup. Pediatr Clin North Am. 2009;56(1): uptodate.com/contents/croup-clinical-features-evaluation-and-diagnosis. Updated
119–133, x–xi PMID: 19135584 https://doi.org/10.1016/j.pcl.2008.10.007 June 15, 2018. Accessed May 6, 2019
Gates A, Gates M, Vandermeer B, et al. Glucocorticoids for croup in chil- Woods CR. Croup: pharmacologic and supportive interventions. In: Kaplan SL,
dren. Cochrane Database Syst Rev. 2018;8(8):CD001955 PMID: 30133690 Messner AH, eds. Waltham, MA: UpToDate; 2019. https://www.uptodate.
10.1002/14651858.CD001955.pub4 com/contents/croup-pharmacologic-and-supportive-interventions. Updated
Loftis LL. Emergency evaluation of acute upper airway obstruction in children. January 2, 2019. Accessed May 6, 2019
In: Teach SJ, Randolph AG, eds. Waltham, MA: UpToDate; 2017. https://www. Woods CR. Epiglottitis (supraglottitis): clinical features and diagnosis.
uptodate.com/contents/emergency-evaluation-of-acute-upper-airway-obstruction- In: Edwards MS, Isaacson GC, Fleischer GR, eds. Waltham, MA: UpToDate; 2018.
in-children. Updated September 19, 2017. Accessed May 6, 2019 https://www.uptodate.com/contents/epiglottitis-supraglottitis-clinical-features-
Ortiz-Alvarez O. Acute management of croup in the emergency department [in and-diagnosis. Updated September 19, 2018. Accessed May 6, 2019
English, French]. Paediatr Child Health. 2017;22(3):166–173 PMID: 29532807
https://doi.org/10.1093/pch/pxx019

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 CHAPTER 72

Sudden Unexpected Infant

Death and Brief Resolved
Unexplained Events
Sarah M. Gustafson, MD, FAAP, and Lynne M. Smith, MD, FAAP

CASE STUDY
A 4-month-old boy is brought to the emergency depart- 2. What is the relationship between sudden infant
ment by paramedics after being found blue and not death syndrome and sudden unexpected infant
breathing by his mother. He had previously been well death?
except for a mild upper respiratory infection. His mother 3. What should parents be advised to help prevent
fed him at 2:00 am and found him blue and lifeless lying sudden unexpected infant death?
next to her in bed at 6:00 am. Although the mother 4. What is the appropriate evaluation of the infant who
smoked cigarettes during pregnancy, the pregnancy and presents with a brief resolved unexplained event?
delivery were otherwise normal. The infant received the 5. Why are sudden unexpected infant death and brief
appropriate immunizations at 2 months of age. resolved unexplained events not related?
6. What services are available to families whose infant
Questions has died from sudden unexpected infant death?
1. What factors are associated with sudden unex-
pected infant death?

Sudden unexpected infant death (SUID) refers to all sudden, unex- Sudden Unexpected Infant Death
pected death in infants younger than 1 year. Sudden infant death
Epidemiology and Risk Factors
syndrome (SIDS), a subcategory of SUID, is a diagnosis of exclusion
following the death of a previously healthy infant younger than Sudden unexpected infant death accounts for 3,000 to 4,000 infant
1 year in which no contributing factors are identified (including the deaths per year in the United States, with an overall incidence of
absence of an unsafe sleep environment) after obtaining a compre- 0.93 per 1,000 live births. These figures were dramatically higher
hensive medical history of the infant and family and performing before the Safe to Sleep campaign (originally the Back to Sleep
a thorough postmortem examination and death scene investiga- campaign) promoted by the American Academy of Pediatrics
tion. In the United States, SUID is the most common cause of death (AAP) to place babies in the supine position for sleep. Before the
in children younger than 1 year (excluding the neonatal period); institution of this campaign, the annual death rate from what was
in most cases, a contributory factor is present, such as an unsafe then termed SIDS was approximately 5,000 to 8,000, with an inci-
sleep environment. dence of approximately 1.4 per 1,000 live births.
A brief resolved unexplained event (BRUE) is a sudden, brief, Sudden unexpected infant death more commonly affects boys
and resolved event that occurs in an infant younger than 1 year than girls and occurs more often in the winter months. The peak
of age and that involves at least 1 of the following findings: cyano- incidence of SUID occurs at 2 to 3 months of age, with 90% of deaths
sis or pallor; decreased, absent, or irregular breathing; change in occurring before age 6 months.
tone; or decreased responsiveness. What is now known as BRUE The frequency of SUID differs in different populations in the
was formerly termed an “apparent life-threatening event” (ALTE). United States and other countries. Although the incidence of SUID
For historical reasons, BRUE and SUID are discussed in this chapter is decreasing among all groups, the rates in black and American
because ALTE and SIDS were once thought to be related. A BRUE is Indian/Alaska Native children is 2 to 3 times the national average.
not a risk factor for SUID, however. One factor contributing to the higher rate of SUID is the increased

513

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 514 PART 5: ACUTE AND EMERGENT PROBLEMS

incidence of nonsupine sleeping in black infants. In 2001, the prev- had a negative view of bedsharing, parents were less likely to bed-
alence of prone positioning was 11% for white infants and 15% to share. If the pediatrician had a neutral view, parents were more likely
21% for black infants. to bedshare. Because approximately 20% of SUID cases in the United
States occur while in the care of someone other than the parent,
Clinical Presentation secondary caregivers and staff at child care centers also should be
Patients with SUID present in cardiopulmonary arrest, with a history educated about the critical need for babies to sleep on their backs
of previous good health or antecedent upper respiratory infection. and in a sleep space free of blankets, pillows, and other objects that
They often present in the early morning hours, having succumbed may obstruct an infant’s airway.
during sleep. The physician cannot determine the cause of death of In addition to exposure to the environmental factors that increase
the deceased infant; that is the role of the coroner. an infant’s risk for SUID, most theories suggest the existence of an
underlying vulnerability in those who experience SUID. The brain-
Pathophysiology and Risk Factors stems of infants who died of SUID have significantly lower concentra-
Numerous epidemiologic, maternal, and infant factors have been tions of serotonin and tryptophan hydroxylase, a biosynthetic enzyme
associated with SUID, including preterm birth and intrauterine of serotonin; higher serotonergic neuron counts; decreased serotonin
growth restriction (Box 72.1). Mothers of children with SUID are 1A receptor binding; and reduced serotonin transporter binding in
frequently young and unmarried, smoke cigarettes, and have had the medulla. Furthermore, several studies have shown a significant
fewer than recommended doctor visits during the prenatal and increase in monoamine oxidase A (MAO-A) gene polymorphisms that
postpartum periods. Parental alcohol use is also a risk factor for could cause overexpression of MAO-A. These findings suggest that
SUID. In 1 study SUID rates were 33% higher on New Year’s Day abnormalities in serotonin synthesis, release, and clearance impair
than any other day, which suggests that parents under the influ- the infant’s ability to appropriately regulate arousal and respiratory
ence of alcohol are less able to monitor their infants safely. Despite drive in response to potential life-threatening challenges during sleep.
initial reports and significant research efforts, no data have estab- Numerous other associations with SUID have been reported,
lished a causal relationship of BRUEs, apnea, immunizations, or including altered polymorphisms of proinflammatory cytokines,
repeated episodes of cyanosis with SUID. abnormalities in other neurotransmitters, small mandibular size, dis-
Although bedsharing was once promoted to enhance breastfeed- orders of fatty acid oxidation, and cardiac channelopathies, including
ing, accidental suffocation and SUID are associated with this prac- long QT syndrome. The AAP does not currently recommend univer-
tice. The importance of a safe sleep environment is underscored by sal electrocardiography (ECG) screening at birth to identify poten-
a study published in 2000 in which the authors investigated 119 tial SUID patients, however, although ECG has been recommended
SUID cases over a 4-year period following the initiation of the Back for infants with abnormal hearing screening because of the associa-
to Sleep campaign. In only 8.4% of these SUID cases was the infant tion of hearing deficits with long QT syndrome. Additionally, pulse
found in a nonprone position, alone in their bed and without any oximetry in the newborn period may help identify infants with occult
potential obstructions of the external airway by bedding. congenital heart disease.
Pediatricians play a critical role in counselling parents about The association between SUID and fatal child abuse has also
safe sleep practices. One study reported that 11% of new mothers received attention, although infanticide is estimated to be the cause
reported “usually” bedsharing, yet only 36% of parents had a conver- in less than 5% of suspected SUID cases. The evaluation of the home
sation about bedsharing with their pediatrician. If their pediatrician environment of infants who have died from SUID, referred to as death
scene investigations, may reveal factors that contributed to the death
of some of these infants. Unsafe sleeping environments (eg, sofas)
Box 72.1. Factors Associated With Sudden and parental drug paraphernalia may identify such factors. A com-
Unexplained Infant Death plete postmortem examination may reveal prior or recent trauma.
ww Sleeping in prone or side-lying position An autopsy should include an assessment for long bone fractures as
ww Soft bedding well as intracranial hemorrhage, although these findings may account
ww Overheating only for the existence of prior trauma rather than for the infant’s
ww Bedsharing death. In some municipalities, child fatality boards review each case
ww Socioeconomic disadvantage of reputed SUID to assess whether an etiology can be determined.
ww Maternal smoking Infants who are targets of medical child abuse may present with SUID
ww Preterm birth or BRUE. Such infants are suffocated by the parents until they become
ww Male sex apneic or die. Because distinguishing between SUID and intentional
ww Maternal youth suffocation is quite difficult pathologically, the AAP Committee on
ww Low birth weight Child Abuse and Neglect has cited factors that should heighten the
ww Poor prenatal care physician’s suspicion for possible child abuse (Box 72.2). The use of
ww Family previously reported to child protective services in-hospital covert video surveillance has facilitated the recognition
of apnea secondary to medical child abuse.

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 Chapter 72: Sudden Unexpected Infant Death and Brief Resolved Unexplained Events 515

Box 72.2. Circumstances in Which the Physician Box 72.3. American Academy of Pediatrics
Should Be Alert to the Possibility of Child Abuse Recommendations to Reduce the Risk
of Sudden Unexpected Infant Death
ww Previous recurrent cyanosis, apnea, or brief resolved unexplained event
while in the care of the same person ww Back to sleep for every sleep.
ww Previous unexpected or unexplained death of 1 or more siblings ww Use a firm sleep surface.
ww Simultaneous or near-simultaneous deaths of twins ww Breastfeeding is recommended.
ww Death of other infants while cared for by the same unrelated person ww Room-sharing with the infant on a separate sleep surface is
ww Blood on the infant’s nose or mouth prior to cardiopulmonary recommended.
resuscitation ww Keep soft objects and loose bedding away from the infant’s sleep area.
ww Infant older than 6 months ww Consider offering a pacifier at naptime and bedtime.
ww Avoid smoke exposure during pregnancy and after birth.
ww Avoid alcohol and illicit drug use during pregnancy and after birth.
ww Avoid overheating.
Management ww Pregnant women should seek and obtain regular prenatal care.
In most jurisdictions, cases of SUID must be reported to the cor- ww Infants should be immunized in accordance with American Academy
oner’s office. The AAP recommends a prompt death scene investi- of Pediatrics and Centers for Disease Control and Prevention
gation; appropriate use of available medical specialists by medical recommendations.
examiners and coroners, including pediatricians; and a postmortem ww Do not use home cardiorespiratory monitors as a strategy to reduce the
examination within 24 hours of death, including radiologic skeletal risk of SIDS.
surveys and toxicology and metabolic screening. A complete review ww Health care providers, staff in newborn nurseries and neonatal intensive
of the medical records of the patient is essential. A timely informa- care units, and child care providers should endorse and model the SIDS
tion session with parents is recommended when the results of the risk-reduction recommendations from birth.
investigation determine SUID or another cause of death. ww Media and manufacturers should follow safe sleep guidelines in their
Physicians must provide care to families whose infant has messaging and advertising.
succumbed to SUID. Parents should be guided through issues ww Continue the “Safe to Sleep” campaign, focusing on ways to reduce the
such as planning the funeral and ending lactation when appro- risk of all sleep-related infant deaths, including SIDS, suffocation, and
priate. For ongoing support, parents should be referred to groups other unintentional deaths. Pediatricians and other primary care provid-
and agencies to help them cope with the unexpected loss of their ers should actively participate in this campaign.
child. Information about these organizations can be obtained from ww Avoid the use of commercial devices that are inconsistent with safe sleep
First Candle (1-800-221-7437; www.firstcandle.org). recommendations.
ww Supervised, awake tummy time is recommended to facilitate develop-
Prevention ment and to minimize development of positional plagiocephaly.
Prevention of SUID has become a focus of public health mea- ww Continue research and surveillance on the risk factors, causes, and
sures, including promotion of smoking cessation and access to pathophysiologic mechanisms of SIDS and other sleep-related infant
prenatal care. Parents must be instructed to avoid soft bedding for deaths, with the ultimate goal of eliminating these deaths entirely.
their infant, bedsharing, placing their infant on a sofa for sleep, ww There is no evidence to recommend swaddling as a strategy to reduce
and overheating, and they should be instructed to place their the risk of SIDS.
infant in the supine position in a crib. See Box 72.3 for additional
Abbreviation: SIDS, sudden infant death syndrome.
recommendations. Adapted with permission from American Academy of Pediatrics Task Force on Sudden Infant Death
Pacifier use has been associated with a decreased incidence of Syndrome. SIDS and other sleep-related infant deaths: updated 2016 recommendations for a safe
SUID. The AAP recommends that caregivers consider offering a pac- infant sleeping environment. Pediatrics. 2016;138(5):e20162938.
ifier at naptime and bedtime through age 12 months. The pacifier
should not be reinserted after the infant falls asleep or coated in any The AAP recommends that parents sleep in a separate but prox-
sweet solution. The pacifier should be cleaned often and replaced imate sleeping environment from their infant (ie, room-sharing but
regularly. For infants fed mother’s milk, pacifier introduction should not bedsharing). Dressing babies in light clothing during sleep and
be delayed until breastfeeding is well established. maintaining the room temperature at a comfortable level for adults
The AAP recommends only the supine sleep position, because is recommended to avoid overheating.
side sleeping increases the risk of SUID 2-fold relative to back sleep- During the first weeks of hospitalization, preterm newborns are
ing. A firm crib mattress covered by a fitted sheet is the recom- often placed in a nonsupine position because of respiratory compli-
mended sleep surface for infants. Soft bedding, such as water beds cations and gastroesophageal reflux. The newborns become accus-
and couches, or objects in the sleep environment, such as stuffed tomed to this position, and the parents learn from the modeling
toys, pillows, quilts, and comforters, are not safe. of the hospital staff to place them in this unsafe position. The AAP

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 516 PART 5: ACUTE AND EMERGENT PROBLEMS

Task Force on Sudden Infant Death Syndrome encourages neonatol-

Box 72.4. What to Ask
ogists and nurses to begin placing preterm newborns on their backs
“significantly before the infant’s anticipated discharge.” Brief Resolved Unexplained Event
One unintended consequence of supine sleep is the increased ww What were the events leading up to the episode?
incidence of positional plagiocephaly. To minimize the risk for head ww Was the infant awake and eating? (Consider gastroesophageal reflux.)
deformities and foster appropriate motor development, parents and ww Was the infant awake, and did the eyes roll back or the body stiffen or
caregivers are encouraged to vary the head position at sleep times, jerk? (Consider a seizure.)
avoid swing and bouncy seats that increase pressure on the back of ww How serious was the event? Did breathing resume spontaneously, or was
the head, and encourage adult-observed tummy time during waking it necessary to initiate cardiopulmonary resuscitation?
hours starting from birth. The pediatrician can suggest tummy time ww Have similar events occurred in the past?
at daytime diaper changes, even if for only 1 or 2 minutes. ww Is the infant well? Has the infant been ill recently?
ww Has the infant had respiratory symptoms, such as wheezing or cough?
Brief Resolved Unexplained Events ww Was the infant given an over-the-counter cough and cold product?
Epidemiology ww Do siblings have a history of sudden unexpected infant death or brief
resolved unexplained event that would suggest the presence of a
Because BRUE is a relatively new term, studies are needed to
familial disorder or child abuse?
gather epidemiologic data. The rates for ALTEs, the term previously
ww Were there any problems with the pregnancy or delivery?
used for many BRUEs, varied between 0.6 and 2.46 per 1,000 live
births. The rates of BRUE likely are lower because unlike with an
ALTE, after a cause for a BRUE is established the event is no longer
classified as a BRUE; instead, it is then classified by the condition Box 72.5. Brief Resolved Unexplained Events:
that precipitated the event. Alternative Diagnoses to Consider
ww Gastroesophageal reflux or overfeeding
Clinical Presentation
ww Lower respiratory tract infection
The infant who has experienced a BRUE at home appears well with- ww Sepsis
out any need for medical intervention but has a highly variable his- ww Pertussis
tory in terms of whether resuscitation was required, length of the ww Respiratory syncytial virus
episode, and associated symptoms. An infant who is ill-appearing on ww Infantile botulism
presentation by definition does not have a resolved event and requires ww Seizure
immediate intervention and further workup. Patients with BRUE ww Incorrect medication dose or overdose
often present between the hours of 8:00 am and 8:00 pm—a time ww Inborn errors of metabolism
of day different from the typical early morning hours for an SUID. ww Child abuse
Differential Diagnosis ww Intracranial hemorrhage
ww Airway anomaly
The major challenge for physicians is to evaluate infants who have
ww Aspiration
experienced a BRUE and determine if any underlying condition
ww Breath-holding spell
caused the episode (Box 72.4). In most cases, no etiology for a BRUE
ww Cardiac arrhythmia/anomaly
is established, and the episode represents an isolated event. If an
etiology is found, by definition the event is no longer unexplained,
and the discharge diagnosis should reflect the etiology. cough is consistent with a respiratory infection, such as respira-
tory syncytial virus or pertussis. After an etiology for the BRUE is
Evaluation
determined (eg, respiratory syncytial virus), the diagnosis of BRUE
History should be changed to reflect the etiology.
The history may provide the clue to the etiology of BRUE (Box 72.4).
Risk Stratification
Physical Examination Before publication of the current guidelines, it was common to admit
A complete physical examination should be conducted. If the infant all infants with BRUE for observation, workup, and monitoring.
is floppy, has poor color, or has required mouth-to-mouth resusci- The 2016 AAP guidelines identify a low-risk subset of patients who
tation or vigorous stimulation, the event is not considered a BRUE require a much more limited evaluation without laboratory test-
because the symptoms are not resolved. The physician should ing (Box 72.6).
check for the presence of bruising, retinal hemorrhage, dysmorphic
features, growth impairment, and abnormal neurologic or devel- Management
opmental findings, which may suggest an alternative etiology The management of BRUE depends on whether the BRUE is classi-
(Box 72.5). The presence of tachypnea, retractions, wheezing, or fied as low- or high-risk. Guidelines for management of BRUE are

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 Chapter 72: Sudden Unexpected Infant Death and Brief Resolved Unexplained Events 517

not recommend home apnea monitors for the prevention of SUID.

Box 72.6. Patients at Low Risk for Brief Resolved
Home apnea monitoring may be considered for the preterm new-
Unexplained Event
born or infant at risk for repeated episodes of apnea of prematurity
ww Age >60 days on discharge from the hospital. Discontinuation of such monitor-
ww Born ≥32 weeks’ gestational age and corrected gestational age ing at approximately 43 weeks’ gestational age should be considered.
≥45 weeks If questions about home monitoring arise, the family of an infant
ww No cardiopulmonary resuscitation by a trained medical provider who experienced BRUE should be informed that a home apnea mon-
ww Event duration <1 minute itor has not been shown to prevent SUID and is not indicated for
ww First event that purpose. Parents and caregivers can be informed that a BRUE
ww No concerns on history and physical examination (eg, no family history is not a risk factor for SUID. In a prospective study, patients who
of sudden cardiac death) experienced what was then termed ALTE did not experience SUID.
The Collaborative Home Infant Monitoring Evaluation study insti-
tuted home monitoring for more than 1,000 healthy term infants
and found that 43% of term infants experienced apnea or bradycar-
detailed in this section, and a treatment algorithm is provided in dia episodes, which indicates that these cardiorespiratory events are
Figure 72.1. common in all infants and are unlikely to be precursors to SUID.
Low-Risk Brief Resolved Unexplained Event Furthermore, SUID usually occurs during infant sleep, whereas an
The patient may be observed briefly (ie, for 1–4 hours) with serial estimated 50% of BRUEs occur during wakefulness.
examinations with or without pulse oximetry. Pertussis testing may High-Risk Brief Resolved Unexplained Event
be considered, especially in the setting of exposure or unvaccinated If the patient does not meet all the criteria for low-risk BRUE, the
caregivers. If a diagnosis of pertussis is likely, the patient typically is patient is considered high risk. For these infants, if any localizing
observed and started on empiric antibiotics pending results. signs or symptoms exist, the workup should be directed toward
Although arrhythmia is rare, an ECG may be indicated because that diagnosis. The infant who was not fully resuscitated in the field
it is a noninvasive test with high negative predictive value. Syncope should be resuscitated, stabilized, and admitted. Any identifiable
is associated with future sudden cardiac death in patients with long conditions, such as sepsis, seizures, and gastroesophageal reflux,
QT syndrome. Other channelopathies, Wolff-Parkinson-White syn- should be appropriately managed.
drome, and cardiomyopathy or myocarditis also can be detected
on ECG. Prognosis
In general, the low-risk infant who experienced a BRUE and does Further research is necessary to determine the prognosis for the
not have an identifiable precipitating condition may be discharged child who has experienced a BRUE. Parents and caregivers can be
from the emergency department after a brief observation of 1 to 4 assured that most BRUEs are isolated events and the risk of a chronic
hours. Prior to discharge from the hospital, the parent or caregiver of condition (eg, seizure disorder) is extremely rare.
the infant who experienced a BRUE should receive resources for car-
diopulmonary resuscitation training, be educated about BRUE, and
be counseled to arrange close follow-up care. The previous guidelines
for ALTEs advised eliminating tobacco smoke exposure, which is not
CASE RESOLUTION
explicitly addressed in the BRUE guidelines. However, given the over- The infant succumbed to SUID. Despite resuscitative efforts by the paramedics, he
could not be revived. The mother was advised of the diagnosis of suspected SUID
all health risks of secondhand smoke exposure, the physician should and referred to appropriate agencies and support groups. The coroner was notified
use every opportunity to counsel caregivers on smoking cessation. of the case. The mother was advised that a coroner investigator would visit her to
Historically, home apnea monitoring was prescribed for the new- learn more about the circumstances surrounding the sudden death of her infant.
born at risk for apnea. Based on a plethora of studies, the AAP does

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 518 PART 5: ACUTE AND EMERGENT PROBLEMS

BRUE Diagnosis

Patient presents for intial medical assessment after a brief,

resolved event that was observed by caregiver
in a child <1 year of age Use event
characteristics,
Patient has additional rather than the
symptoms or abnormal term “ALTE,” to
Patient is
vital signs (eg, cough, describe the
well-appearing
respiratory difficulties, event
or fever)

Clinician characterizes the event as a sudden, brief and

now resolved episode of one or more of the following:
Event
• cyanosis or pallor Not a BRUE
criteria
• absent, decreased, or irregular breathing
absent
• marked change in tone (hyper- or hypotonia)
• altered responsiveness Explanation for
event identified
Event criteria present (eg, GER, feeding difficulties,
or airway abnormality)

Perform appropriate history and PE*

Out of guideline
No explanation for event identified scope; manage
accordingly
Diagnosis of Brief Resolved Unexplained Event is made

BRUE RIsk Classification

Concerns identified from history or PE

No concerns identified (eg, FH of sudden cardiac death or
from history and PE* subtle, non-diagnostic social, feeding
or respiratory problems)

Apply risk stratification

• Age >60 days
• Born 32 weeks gestation and corrected gestational age 45 weeks Higher Risk
• No CPR by trained medical provider No
Patient
• Event lasted <1 minute
• First event
Yes

Lower Risk Patient

Management Recommendations for Lower Risk Patients **

Should Should Not

• Educate caregivers about BRUEs and engage • Obtain WBC count, blood culture, or CSF analysis or
in shared decision–making to guide evaluation, culture, serum sodium, potassium, chloride, blood
disposition, and follow-up urea nitrogen, creatinine, calcium, ammonia, blood
• Offer resources for CPR training to caregiver gases, urine organic acids, plasma amino acids or
acylcarnitines, chest radiograph, echocardiogram,
EEG, studies for GER, or laboratory evaluation
for anemia
• Initiate home cardio-respiratory monitoring
• Prescribe acid suppression therapy or anti-epileptic
medications
May Need Not
• Obtain pertussis testing and 12-lead ECG • Obtain viral respiratory test, urinalysis, blood
• Briefly monitor patients with continuous pulse glucose, serum bicarbonate, serum lactic acid,
oximetry and serial observations or neuroimaging
• Admit the patient to the hospital solely for
cardiorespiratory monitoring

Figure 72.1. Diagnosis, risk classification, and recommended management of a BRUE.

Abbreviations: ALTE, apparent life-threatening event; BRUE, brief resolved unexplained event; CPR, cardiopulmonary resuscitation; CSF, cerebrospinal fluid; ECG, electrocardiography;
EEG, electroencephalogram; FH, family history; GER, gastroesophageal reflux; PE, physical examination; WBC, white blood cell.
* Refer to Tables 3 and 4 in www.pediatrics.org/cgi/doi/10.1542/peds.2016-0591 for the determination of an appropriate and negative history and PE.
** Refer to Figure 2 in www.pediatrics.org/cgi/doi/10.1542/peds.2016-0591 for the American Academy of Pediatrics method for rating of evidence and recommendations.
Reprinted with permission from Tieder JS, Bonkowsky JL, Etzel RA, et al; American Academy of Pediatrics Subcommittee on Apparent Life Threatening Events. Brief resolved unexplained
events (formerly apparent life-threatening events) and evaluation of lower-risk infants: executive summary. Pediatrics. 2016;137(5):e20160591.

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 Chapter 72: Sudden Unexpected Infant Death and Brief Resolved Unexplained Events 519

Selected References Kemp JS, Unger B, Wilkins D, et al. Unsafe sleep practices and an analysis of bed-
sharing among infants dying suddenly and unexpectedly: results of a four-year,
American Academy of Pediatrics Task Force on Sudden Infant Death Syndrome; population-based, death-scene investigation study of sudden infant death syn-
Moon RY. SIDS and other sleep-related infant deaths: expansion of recommen- drome and related deaths. Pediatrics. 2000;106(3):e41 PMID: 10969125 https://
dations for a safe infant sleeping environment. Pediatrics. 2011;128(5):1030– doi.org/10.1542/peds.106.3.e41
1039. Reaffirmed October 2014 PMID: 22007004 https://doi.org/10.1542/ Kinney HC, Thach BT. The sudden infant death syndrome. N Engl J Med.
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Berkowitz CD. Sudden infant death syndrome, sudden unexpected infant death, Lahr MB, Rosenberg KD, Lapidus JA. Bedsharing and maternal smoking in a
and apparent life-threatening events. Adv Pediatr. 2012;59(1):183–208 PMID: population-based survey of new mothers. Pediatrics. 2005;116(4):e530–e542
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Blair PS, Sidebotham P, Berry PJ, Evans M, Fleming PJ. Major epidemiolog- Mathews AA, Joyner BL, Oden RP, Alamo I, Moon RY. Comparison of infant
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study in the UK. Lancet. 2006;367(9507):314–319 PMID: 16443038 https://doi. sleep-related infant death. J Immigr Minor Health. 2015;17(3):834–842 PMID:
org/10.1016/S0140-6736(06)67968-3 24705738 https://doi.org/10.1007/s10903-014-0016-9
Bonkowsky JL, Guenther E, Filloux FM, Srivastava R. Death, child abuse, and Neary MT, Breckenridge RA. Hypoxia at the heart of sudden infant death
adverse neurological outcome of infants after an apparent life-threatening event. syndrome? Pediatr Res. 2013;74(4):375–379 PMID: 23863852 https://
Pediatrics. 2008;122(1):125–131 PMID: 18595995 https://doi.org/10.1542/ doi.org/10.1038/pr.2013.122
peds.2007-3376
Phillips DP, Brewer KM, Wadensweiler P. Alcohol as a risk factor for sudden
Centers for Disease Control and Prevention. Sudden unexpected infant death infant death syndrome (SIDS). Addiction. 2011;106(3):516–525 PMID: 21059188
and sudden infant death syndrome: data and statistics. trends in sudden unex- https://doi.org/10.1111/j.1360-0443.2010.03199.x
pected infant death by cause, 1990-2017. CDC.gov website. https://www.cdc.
Putnam-Hornstein E, Schneiderman JU, Cleves MA, Magruder J, Krous HF. A
gov/sids/data.htm#cause. Accessed June 18, 2019
prospective study of sudden unexpected infant death after reported maltreat-
Colson ER, Willinger M, Rybin D, et al. Trends and factors associated with ment. J Pediatr. 2014;164(1):142–148 PMID: 24139442 https://doi.org/10.1016/j.
infant bed sharing, 1993-2010: the National Infant Sleep Position Study. JAMA jpeds.2013.08.073
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Shapiro-Mendoza CK, Parks SE, Brustrom J, et al. Variations in cause-
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Courts C, Grabmüller M, Madea B. Monoamine oxidase A gene polymor- 2017;140(1):e20170087 PMID: 28759406 https://doi.org/10.1542/peds.
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2013;163(1):89–93 PMID: 23391042 https://doi.org/10.1016/j.jpeds.2012.12.072
Tieder JS, Bonkowsky JL, Etzel RA, et al; American Academy of Pediatrics
Duncan JR, Paterson DS, Hoffman JM, et al. Brainstem serotonergic deficiency Subcommittee on Apparent Life Threatening Events. Brief resolved unex-
in sudden infant death syndrome. JAMA. 2010;303(5):430–437 PMID: 20124538 plained events (formerly apparent life-threatening events) and evaluation of
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Esani N, Hodgman JE, Ehsani N, Hoppenbrouwers T. Apparent life-threatening doi.org/10.1542/peds.2016-0590
events and sudden infant death syndrome: comparison of risk factors. J Trachtenberg FL, Haas EA, Kinney HC, Stanley C, Krous HF. Risk factor changes
Pediatr. 2008;152(3):365–370 PMID: 18280841 https://doi.org/10.1016/j. for sudden infant death syndrome after initiation of Back-to-Sleep campaign.
jpeds.2007.07.054 Pediatrics. 2012;129(4):630–638 PMID: 22451703 https://doi.org/10.1542/
Franco P, Kato I, Richardson HL, Yang JS, Montemitro E, Horne RS. Arousal from peds.2011-1419
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BPPCA6e_Ch072_0513-0520.indd 520 2/13/20 6:11 PM
 CHAPTER 73

Syncope
David Atkinson, MD, and Michael Nguyen, DO

CASE STUDY
A 16-year-old girl presents to your office with the chief She has only gone to the emergency department 1 time
report of fainting at marching band practice on the day previously, when she was 2 years old. At that time, she
prior. She has been in marching band for the past 2 years passed out for 30 seconds after crying. She was diag-
and states that nothing like this has occurred before. nosed with a breath-holding spell and has not had any
She is concerned about fainting again. She tells you that other issues since. There is no family history of sudden
she “wasn’t able to eat or drink” on the day she fainted death and seizures. When questioned alone, she denies
because she was too busy studying for finals. use of any illicit drugs or any sexual activity. Her mother
She reports that practice was fairly routine up until asks if it is okay for her to continue to participate in phys-
her fainting episode. Prior to the episode, she was stand- ical activities. She has recently read about sudden death
ing in the field, listening to her teacher give instructions in high school athletes.
for a new routine. The last thing she remembers after The girl’s physical examination is unremarkable,
standing awhile was feeling lightheaded and sweaty. and all vital signs are within normal limits for age.
The next thing she can recall is lying on the ground with Electrocardiography shows normal sinus rhythm with
her classmates and teacher around her. She denies any normal voltages and intervals for her age.
chest pain, shortness of breath, or palpitations prior to
the episode. Her teacher told her she was unconscious
Questions
1. What are the causes of syncope?
for approximately 10 to 15 seconds without any shak-
2. What workup is recommended to evaluate for
ing of extremities. She was immediately back to base-
syncope?
line after she woke up. She denies incontinence. She says
3. When should patients who experience syncope be
that when she stands up too quickly she sometimes feels
referred to a subspecialist?
lightheaded for a few seconds, but she had never fainted
4. Which pediatric subspecialists assist in the evalua-
before yesterday.
tion of a patient with syncope?
When asked, she denies any past significant med-
5. Which patients presenting with syncope are at
ical history. Her mother states that she is very healthy.
greatest risk for sudden death?

Syncope, or fainting, is a transient loss of consciousness and tone; it mechanisms. Syncopal events are very common in the pediatric pop-
is a common clinical problem in pediatric patients, particularly dur- ulation; up to 50% of college undergraduates have reported experi-
ing puberty and adolescence. The most common causes of syncope in encing syncope or near syncope, and it accounts for approximately 1%
pediatric patients are benign neurocardiogenic events; however, in rare of all pediatric emergency department visits. Females are more
instances syncope is a harbinger of sudden death from arrhythmia, commonly affected than males, and the mean age at presentation
obstruction of aortic outflow, or other serious cardiovascular events. is 10 to 12 years. Syncope is uncommon in children younger than
The 3 general categories of syncope are neurocardiogenic (also 5 years. Many cases of syncope quickly resolve and medical atten-
called vasovagal syncope), cardiac syncope, and noncardiac syncope tion is not sought; thus, the true incidence of syncope is almost
(Box 73.1). The workup for syncope can easily become expensive certainly underestimated.
and time-consuming, and it may provide little information beyond
that gleaned by the initial history and physical examination. It is the Clinical Presentation
role of the pediatrician to appropriately direct the evaluation for syn- The clinical presentation of syncope varies with the etiology.
cope so that a cost-effective evaluation may occur without missing Vasovagal syncope often is associated with a prodrome of symp-
the patient who may be at risk for a sudden death event. toms, including lightheadedness, visual disturbances, nausea, and
diaphoresis. The patient has usually been standing for a long period
Epidemiology or has suddenly moved from the supine or sitting position to stand-
Syncope is a temporary, transient loss of consciousness and muscle ing. Other forms of neurally mediated syncope include hair-
tone that usually is associated with rapid recovery. It is the result of grooming syncope, which occurs mostly in girls while combing,
decreased cerebral blood flow that can occur through many different brushing, or blow-drying their hair. Micturition syncope, although
521

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 522 PART 5: ACUTE AND EMERGENT PROBLEMS

Syncope related to seizures generally has a longer recovery time

Box 73.1. Causes of Syncope
associated with the postictal phase; witnesses may describe the patient
Neurocardiogenic (ie, Vasovagal Syncope) as being dazed or “having a blank look on their face” before fainting.
Cardiac These episodes may occur whether the patient is recumbent or upright.
ww Tachyarrhythmias
—— Supraventricular tachycardia Pathophysiology
—— Ventricular tachycardia Autonomic
ww Bradyarrhythmias
Autonomic causes of syncope are the most common etiology,
—— Second- or third-degree heart block
accounting for up to 80% of cases of syncope that come to medi-
—— Sinus node dysfunction
cal attention. They are also referred to as neurally mediated reflex-
ww Left or right ventricular outflow tract obstruction
ive syncope, vasovagal syncope, neurocardiogenic syncope, and, in
—— Hypertrophic cardiomyopathy
toddlers, pallid breath-holding spells. They have in common dis-
—— Aortic stenosis
turbances in autonomic control of heart rate and blood pressure in
ww Idiopathic pulmonary hypertension
response to postural changes, bodily functions, pain, fear, or other
ww Coronary artery disease
strong emotional events. Vasovagal syncopal events usually occur
—— Acquired coronary artery disease
when the patient is upright, resulting in decreased venous return,
 Kawasaki disease
decreased arterial blood pressure, and decreased left ventricular
—— Congenital coronary anomaly
volume. The resultant reflex stimulation of vagal fibers results in
 Intramural coronary artery
bradycardia, vasodilation, and worsening hypotension (ie, Bezold-
 Anomalous origin of a coronary artery
Jarisch reflex). The 3 clinical types of neurally mediated syncope are
ww Primary cardiac dysfunction
vasodepressor, which starts and is primarily marked by profound
—— Dilated cardiomyopathy
hypotension; cardioinhibitory, which is marked by severe bradycar-
—— Noncompaction cardiomyopathy
dia or even brief asystole; and mixed response, which is a mixture
ww Secondary cardiac dysfunction
of both vasodepressor and cardioinhibitory types.
—— Viral or idiopathic myocarditis
—— Restrictive cardiomyopathy
Cardiac
Noncardiac Cardiac causes of syncope are more likely than noncardiac causes to be
ww Orthostatic hypotension associated with sudden death than non-cardiac causes, so it is impor-
ww Neurologic (eg, seizures, atypical migraine, dysautonomia) tant to identify and treat these abnormalities. Cardiac mechanisms
ww Breath-holding spells of syncope are mainly related to obstructive lesions or arrhythmias.
ww Psychogenic (eg, hysteria, hyperventilation) Obstructive lesions cause decreased ventricular outflow, resulting in
ww Self-induced (eg, hyperventilation, “the choking game”) decreased cerebral perfusion; arrhythmia may result in decreased
ww Metabolic abnormality (eg, hypoglycemia, anemia) ejection volume, also causing cerebral hypoperfusion and syncope.
Left-sided obstructive lesions, including aortic stenosis and
hypertrophic cardiomyopathy, are the most likely cause of obstruc-
most common in the elderly, may occur in individuals of any age. tive syncope. Rarely, syncope is caused by pulmonary stenosis or
Younger patients with this type syncope tend to be male; predis- severe primary pulmonary hypertension, mitral stenosis, atrial myx-
posing factors may include reduced food intake, fatigue, alcohol oma, or cardiac tamponade. Cardiac syncope, unlike routine vasova-
ingestion, and recent respiratory infection. Micturition syncope gal syncope, often occurs during exertion, because of the inability of
often occurs at night when voiding after awakening from sleep (ie, the heart to increase cardiac output to meet the demands placed on
while standing immediately after being recumbent). Recurrences it by increased physical activity. Increased diastolic pressure caused
of micturition syncope are rare in young patients. Breath-holding by an obstructive lesion may also decrease myocardial perfusion,
spells in toddlers brought on by anger, pain, fear, or frustration resulting in cardiac ischemia, dyskinesis, or ventricular arrhyth-
may be associated with syncope; this is an infantile form of car- mias, thereby further decreasing ventricular output.
dioinhibitory neurally mediated syncope. Infants who experience Anomalous origin of the coronary arteries is not an obstructive
syncopal breath-holding spells are more likely to grow up and have lesion; however, it too may cause syncope with exercise. The left cor-
neurally mediated syncope (see Chapter 52). onary artery may arise from the pulmonary artery, delivering deox-
Syncope of cardiac etiology often lacks the prodrome of vaso- ygenated blood to the left coronary system; alternatively, the left
vagal syncope. The main cardiac causes of syncope are arrhyth- coronary artery may arise from the right coronary cusp and course
mia and left ventricular outflow obstruction. Patients may report between the aorta and pulmonary arteries. When the patient is in a
palpitations, chest pain, or chest tightness. Cardiac syncope com- high cardiac output state, such as during exercise, the left coronary
monly occurs during physical activity and may be accompanied by artery may be compressed between the great arteries, resulting in
complete loss of body tone. ischemia or arrhythmia.

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 Chapter 73: Syncope 523

Primary arrhythmias causing syncope are a rare but important of the corrected QT interval. The prolongation of the repolarization
cause of syncope. Typically, chest radiography, echocardiography, period of the heart puts patients with long QT syndrome at risk for
and other imaging modalities are normal, with no evidence of struc- torsades de pointes, a malignant form of ventricular tachycardia. The
tural heart disease or pulmonary edema. Supraventricular tachycar- genetic forms of long QT syndrome result from mutations in genes
dia may cause syncope or near syncope. In most pediatric patients, that code for ion transport channels or related proteins. Jervell and
the tachycardia is propagated through a concealed pathway, and the Lange-Nielsen syndrome is an autosomal-recessive form of long QT
resting electrocardiogram (ECG) is normal if the tachycardia is not syndrome that is associated with congenital deafness. Autosomal-
occurring while the ECG is being obtained. Supraventricular tachy- dominant long QT syndrome that is not associated with congenital
cardia may also be associated with Wolff-Parkinson-White syn- deafness has been referred to as Romano-Ward syndrome. Although
drome, which itself is characterized by a short P-R interval followed the clinical diagnosis of long QT syndrome is based on a QTc that
by an abnormally wide QRS complex with an initial delta wave. is prolonged for the patient’s age, an estimated 20% of patients with
Ventricular tachycardia is rare in children with no underlying struc- a gene mutation associated with long QT syndrome have a
tural heart disease, but it may be brought on by infection (especially normal resting ECG; thus, a critical part of the evaluation of the
myocarditis or pericarditis), cardiomyopathies, drugs (eg, cocaine, syncopal patient is obtaining a family history of long QT syn-
amphetamines), drug interactions (eg, non-sedating antihistamines drome, sudden death or near sudden death, seizures, or a history of
taken with erythromycin or ketoconazole), and long QT syndrome. torsade de pointes. Long QT syndrome also may be brought on by
Patients with long QT syndrome have prolonged cardiac repolar- electrolyte imbalance, increased intracranial pressure, or medications
ization, which usually manifests on the resting ECG as a prolongation (Table 73.1).

Table 73.1. Drugs Known to Increase the Risk for Ventricular Arrhythmia in
Patients With Long QT Syndromea
Drug Class Clinical Use
Amiodarone hydrochloride Antiarrhythmic Abnormal heart rhythm
Arsenic trioxide Anticancer Leukemia
Bepridil hydrochloride Antianginal Heart pain
Chloroquine Antimalarial Malaria infection
Chlorpromazine Antipsychotic/antiemetic Schizophrenia/nausea
Cisapride GI stimulant Heartburn
Clarithromycin Antibiotic Bacterial infection
Disopyramide Antiarrhythmic Abnormal heart rhythm
Dofetilide Antiarrhythmic Abnormal heart rhythm
Droperidol Sedative/antiemetic Anesthesia adjunct/nausea
Erythromycin Antibiotic/GI stimulant Bacterial infection/Increase GI motility
Halofantrine hydrochloride Antimalarial Malaria infection
Haloperidol Antipsychotic Schizophrenia, agitation
Ibutilide fumarate Antiarrhythmic Abnormal heart rhythm
Levomethadyl acetate Opiate agonist Pain control, narcotic dependence
Mesoridazine Antipsychotic Schizophrenia
Methadone hydrochloride Opiate agonist Pain control, narcotic dependence
Pentamidine isethionate Anti-infective Pneumocystis pneumonia
Pimozide Antipsychotic Tourette syndrome tics
Procainamide hydrochloride Antiarrhythmic Abnormal heart rhythm
Quinidine Antiarrhythmic Abnormal heart rhythm
Sotalol hydrochloride Antiarrhythmic Abnormal heart rhythm
Sparfloxacin Antibiotic Bacterial infection
Thioridazine Antipsychotic Schizophrenia
Abbreviation: GI, gastrointestinal.
a
For a complete list of drugs to avoid in patients with long QT syndrome, visit www.crediblemeds.org.

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 524 PART 5: ACUTE AND EMERGENT PROBLEMS

Noncardiac Box 73.2. What to Ask

Noncardiac causes of syncope include neurologic etiologies (eg,
seizures, migraines), metabolic disturbances (eg, hypoglycemia), Syncope
hyperventilation (panic attacks or self-induced), and hysteria. Low ww What was the patient doing when the episode occurred?
iron stores have been associated with neurally mediated syncope in ww What was the position of the patient?
both children and adolescents and may provide a partial explana- ww Did the patient have any symptoms before the syncopal event?
tion for the higher incidence of neurocardiogenic syncopal events in ww Did the patient have any chest pain or palpitations during the event?
adolescent girls than boys. Seizures may be difficult to distinguish ww How long did it take for the patient to recover from the syncopal event?
from vasovagal events, because both can have tonic-clonic move- ww Were any residual symptoms present after the syncopal event?
ments. Unlike vasovagal or cardiac syncope, seizures result in uncon- ww Has the patient recently been ill, dehydrated, or fatigued?
sciousness secondary to neurologic dysfunction. Syncope associated ww Does the patient have a history of underlying cardiac disease?
with hypoglycemia is caused by the insufficient delivery of substrate ww Is the patient taking any type of medication (prescribed, over-the-
(glucose) to the brain. Cerebral vasoconstriction secondary to arte- counter, or illicit)?
rial hypocapnia produces syncope with hyperventilation that may ww Does the patient have a history of breath-holding or pallid spells?
be secondary to panic attacks or may be self-induced. The “choking ww Is there a family history of sudden death, seizures, deafness, or cardiac
game” or “fainting game” refers to intentional cutting off of oxygen abnormalities?
to the brain, with the goal of inducing a “high” or euphoric feeling,
usually resulting in syncope and occasionally in serious injury or
death. Hysterical syncope lacks a true prodrome, and patients usu- pubertal females, a pregnancy test should be obtained as well as a
ally suffer no injury when they fall. This form of syncope is thought hemoglobin level. Iron levels may be low in patients with neurally
to be related to Munchausen syndrome. mediated syncope.

Differential Diagnosis Imaging Studies

Every patient who is being evaluated for syncope requires an ECG.
The differential diagnosis of syncope is presented in Box 73.1. Events
The ECG may reveal Wolff-Parkinson-White syndrome or a pro-
that precede and follow the syncopal event are key in determining
longed QT interval, neither of which can be diagnosed on history
the etiology of syncope.
and physical examination alone. These studies also can be useful in
identifying underlying structural abnormalities, such as right ven-
Evaluation tricular hypertrophy secondary to pulmonary hypertension, left ven-
History tricular hypertrophy secondary to hypertrophied cardiomyopathy,
A thorough history should be obtained from the family and patient, or Q waves associated with anomalous origin of the coronary arter-
focusing on the patient’s symptoms, the situation surrounding the ies. Patients with findings suggestive of cardiac disease, that is, with
event, and the family history (Box 73.2). A primary goal of the his- a positive history, physical findings, or an abnormal ECG, require
tory is to identify any underlying cardiac problems, because these referral to a pediatric cardiologist for further evaluation. The car-
patients are at the greatest risk for sudden death. diac workup of syncope may include a Holter monitor or an event
recorder. The Holter monitor is worn for 24 hours and continuously
Physical Examination records the heart rhythm. Because this is a limited window of obser-
The patient with syncope must undergo a thorough physical exam- vation, the Holter monitor captures arrhythmia less than 20% of the
ination. Physicians should assess the blood pressure to screen for time, even in patients with known arrhythmias. Event recorders are
hypotension and hypovolemia. The cardiac examination should worn for longer periods, often up to several weeks. They can record
include palpation of the chest for the point of maximal intensity, and retain the cardiac rhythm before, during, and after an event.
thrills and lifts, and auscultation to assess the intensity of the heart When the patient activates the monitor, the recording is saved for
sounds and detect the presence of murmurs or other adventitial download to the cardiologist. If the monitor is not activated, the
sounds. Upper- and lower-extremity pulses should be palpated event recorder will not save the information but will record over it.
for their presence and quality. The remainder of the examination Although the event recorder has a higher probability than a Holter
should focus on identifying any abnormal neurologic findings. monitor of capturing an arrhythmia, the tracings are generally lower
quality. Small, digital ambulatory cardiac monitors are a relatively
Laboratory Tests new innovation. They are waterproof, may be worn for up to 14 days,
The history and physical examination should guide which labo- and may provide benefits of both the Holter monitor and the tradi-
ratory tests are necessary. Generally, few tests are needed. Serum tional event recorder. Exercise stress testing is often used to evaluate
glucose level shortly after the syncopal event may be abnormally the patient’s response to exertion and potentially replicate symptoms
low and reveal hypoglycemia. Fasting glucose or glucose toler- in a monitored setting. Electrophysiology studies may be ordered if
ance tests are usually normal, and such testing is not indicated. In an arrhythmia is uncovered during the evaluation.

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 Chapter 73: Syncope 525

The workup for patients without evidence of cardiac disease, that is only through identification and treatment of these structural or
is, with a negative family history for cardiac disease or sudden death rhythm abnormalities that sudden death may be prevented.
and no exertional symptoms such as chest pain, should focus on the
noncardiac or autonomic causes of syncope. Patients with prolonged
recovery time or persistent neurologic symptoms following an event CASE RESOLUTION
should be referred to a neurologist and may require electroenceph- The adolescent girl describes symptoms consistent with vasovagal syncope. Her
alography to rule out seizure disorders. family history, physical examination, and ECG are not suggestive of underlying
A tilt test may be used to aid in the diagnosis of vasovagal syn- cardiac disease. The patient and her family should be informed that certain fac-
cope, although in most cases the history is sufficient. For this test, tors, such as dehydration, fatigue, and hunger, can precipitate syncope. Behavioral
the patient is placed on a table that is then tilted to simulate stand- changes, such as eating breakfast and drinking plenty of water, should be imple-
mented to prevent or limit recurrence of syncope. The patient should be encour-
ing in an upright position, a condition that is commonly associ- aged to carry a water bottle in school, and if necessary a physician note should be
ated with vasovagal syncope. Great variability exists in the tilt test, sent to the school to allow her to do so. Management with medications is not
however, with a false-positive rate of up to 20%. Factors that influ- indicated at this time.
ence the test results include the time of day, whether the patient has
fasted, hydration status, and whether the test was augmented with
isoproterenol. Few false-negative tilt test results have been reported;
however, because of the high number of false-positive results the
Selected References
tilt test should be reserved for refractory, recurrent, or unexplained Evans WN, Acherman R, Kip K, Restrepo H. Hair-grooming syncope in children.
syncope only. Clin Pediatr (Phila). 2009;48(8):834–836 PMID: 19571334 https://
doi.org/10.1177/0009922809339204
Fischer JWJ, Cho CS. Pediatric syncope: cases from the emergency department.
Management Emerg Med Clin North Am. 2010;28(3):501–516 PMID: 20709241 https://
Management of syncope depends on its cause. Recurrent vasovagal doi.org/10.1016/j.emc.2010.03.009
syncope may be treated simply with increased fluid intake, includ- Goble MM, Benitez C, Baumgardner M, Fenske K. ED management of pediatric
ing carrying a water bottle in school, and increased salt intake. For syncope: searching for a rationale. Am J Emerg Med. 2008;26(1):66–70 PMID:
patients who are not responding to conservative measures and 18082784 https://doi.org/10.1016/j.ajem.2007.06.012
who are experiencing recurrent vasovagal syncope, it is reasonable Grubb BP. Neurocardiogenic syncope. N Engl J Med. 2005;352(10):1004–1010
to prescribe midodrine hydrochloride. Fludrocortisone acetate, a PMID: 15758011 https://doi.org/10.1056/NEJMcp042601
mineralocorticoid, is a common medical intervention for vasovagal Jarjour IT, Jarjour LK. Low iron storage in children and adolescents with neu-
syncope, although the efficacy is not well established. Other treat- rally mediated syncope. J Pediatr. 2008;153(1):40–44 PMID: 18571533 https://
ments include vagolytic drugs (eg, disopyramide), or centrally acting doi.org/10.1016/j.jpeds.2008.01.034
drugs (eg, imipramine, fluoxetine). These medications have varied Khositseth A, Martinez MW, Driscoll DJ, Ackerman MJ. Syncope in children and
benefits, and results between small controlled trials are not consis- adolescents and the congenital long QT syndrome. Am J Cardiol. 2003;92(6):
tent. The use of beta blockers is not beneficial and may result in a 746–749 PMID: 12972126 https://doi.org/10.1016/S0002-9149(03)00846-4

higher recurrence rate. Kumpf M, Sieverding L, Gass M, Kaulitz R, Ziemer G, Hofbeck M.

Patients with neurologic syncope secondary to seizures should Anomalous origin of left coronary artery in young athletes with syncope.
BMJ. 2006;332(7550):1139–1141 PMID: 16690672 https://doi.org/10.1136/
be treated with anticonvulsants. Patients with hyperventilation, hys-
bmj.332.7550.1139
teria, or hypoglycemia are most amenable to behavioral interven-
Massin MM, Malekzadeh-Milani S, Benatar A. Cardiac syncope in pediatric
tions. Patients with syncope secondary to a cardiac etiology should
patients. Clin Cardiol. 2007;30(2):81–85 PMID: 17326062 https://
be referred to a cardiologist for further evaluation and correction doi.org/10.1002/clc.28
of the underlying problem. Syncopal episodes of a cardiac etiology
Petko C, Bradley DJ, Tristani-Firouzi M, et al. Congenital long QT syndrome in
may require more aggressive management, such as surgery or car- children identified by family screening. Am J Cardiol. 2008;101(12):1756–1758
diac pacing, because they are more likely to result in sudden death. PMID: 18549854 https://doi.org/10.1016/j.amjcard.2008.02.068
Piccirillo G, Naso C, Moisè A, et al. Heart rate and blood pressure variability
Prognosis in subjects with vasovagal syncope. Clin Sci (Lond). 2004;107(1):55–61 PMID:
14982493 https://doi.org/10.1042/CS20030327
The prognosis for autonomic and noncardiac syncope is good. Often,
Ramowski SK, Nystrom RJ, Rosenberg KD, Gilchrist J, Chaumeton NR. Health
patients with vasovagal syncope experience only a single event, and
risks of Oregon eighth-grade participants in the “choking game”: results from
those with recurrent events can be treated with increased fluid intake a population-based survey. Pediatrics. 2012;129(5):846–851 PMID: 22508913
or with medication. One subgroup to be aware of is children with https://doi.org/10.1542/peds.2011-2482
pallid breath-holding spells; although these spells resolve by 5 years Salim MA, Di Sessa TG. Effectiveness of fludrocortisone and salt in preventing
of age in most children, up to 17% of patients may continue to have syncope recurrence in children: a double-blind, placebo-controlled, random-
syncope in adulthood. Patients who have syncope with an underly- ized trial. J Am Coll Cardiol. 2005;45(4):484–488 PMID: 15708690 https://
ing cardiac etiology are at the greatest risk for sudden death, and it doi.org/10.1016/j.jacc.2004.11.033

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 526 PART 5: ACUTE AND EMERGENT PROBLEMS

Shen WK, Sheldon RS, Benditt DG, et al. 2017 ACC/AHA/HRS Guideline for Disease in the Young, and Stroke; Quality of Care and Outcomes Research
the evaluation and management of patients with syncope: executive summary: Interdisciplinary Working Group; American College of Cardiology Foundation;
a report of the American College of Cardiology/American Heart Association Heart Rhythm Society; American Autonomic Society. AHA/ACCF Scientific
Task Force on Clinical Practice Guidelines, and the Heart Rhythm Society. statement on the evaluation of syncope. Circulation. 2006;113(2):316–327 PMID:
Circulation. 2017;136(5):e25-e59 PMID: 28280232 https://doi.org/10.1161/ 16418451 https://doi.org/10.1161/CIRCULATIONAHA.105.170274
CIR.0000000000000498 Sun BC, Emond JA, Camargo CA Jr. Inconsistent electrocardiographic test-
Stewart JM. Postural tachycardia syndrome and reflex syncope: similarities ing for syncope in United States emergency departments. Am J Cardiol.
and differences. J Pediatr. 2009;154(4):481–485 PMID: 19324216 https:// 2004;93(10):1306–1308 PMID: 15135712 https://doi.org/10.1016/j.
doi.org/10.1016/j.jpeds.2009.01.004 amjcard.2004.02.021
Strickberger SA, Benson DW, Biaggioni I, et al; American Heart Association
Councils on Clinical Cardiology, Cardiovascular Nursing, Cardiovascular

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 CHAPTER 74

Shock
Kelly D. Young, MD, MS, FAAP

CASE STUDY
A 7-month-old boy is brought in by his parents with a Questions
history of vomiting and diarrhea for 2 days. He also has 1. What is shock, and what clinical signs can help in
had a low-grade fever and, according to his parents, has the recognition and assessment of shock?
become progressively more listless. Vital signs show a 2. What are the stages of shock?
heart rate of 200 beats per minute, respiratory rate of 30 3. What are the different types of shock, and what are
breaths per minute, and blood pressure of 72/35 mm Hg. the possible causes of each type?
The infant is lethargic, and his skin is mottled. Capillary 4. What are the management priorities in treating
refill time is 3 seconds. His anterior fontanelle is sunken, shock?
and his mucous membranes are dry. The abdomen is flat
and nontender, and hyperactive bowel sounds are heard.

Shock is defined as a state of circulatory dysfunction resulting in progresses it becomes uncompensated, resulting in impairment of
insufficient delivery of oxygen and other metabolic substrates to the vital organ perfusion. Signs of uncompensated shock include hypo-
tissues. Shock is not a disease but rather an abnormal physiologic tension; altered mental status (eg, irritability, lethargy, decreased
state that may result from many disease processes. Early recogni- interactivity); weak, thready, or absent pulses (although pulses
tion and prompt management of shock are critical to avoid perma- may be bounding in “warm” septic shock); and severely mot-
nent end-organ damage or death. tled or cyanotic skin (Box 74.1). In the Nevada epidemiologic
study, young children commonly presented with poor extremity
Epidemiology
The most common type of shock in children worldwide is hypovole-
mic shock, and the most common causes are dehydration resulting Box 74.1. Diagnosis of the Stages of Shock
from gastrointestinal infections that cause vomiting and diarrhea, Compensated
and hemorrhage resulting from traumatic injury. Epidemiology ww Tachycardia
may differ in tertiary care health care systems in developed coun- ww Normal blood pressure
tries, however. In a case series of 147 pediatric patients with shock ww Normal or bounding pulses
(excluding trauma patients) from Children’s Hospital of Nevada ww Normal or cool, clammy skin
at UMC, septic shock was the most common etiology, with 57% ww Pale or mottled skin color
of patients presenting with that type. Of the remaining patients, ww Alert, anxious mental state
24% had hypovolemic shock, 14% had distributive shock, and ww Mildly delayed capillary refill time
5% had cardiogenic shock. Shock may occur in any age group, but ww Decreased urine output
it is more difficult to recognize the early stages in young children Uncompensated
because early clinical signs of shock in children are subjective and ww Tachycardia or bradycardia
may be attributed to other causes. By the time young children have ww Hypotension
developed more typical signs, such as a thready pulse and hypoten- ww Weak, thready, or absent pulses
sion, they are in the late stages of shock. ww Cool, clammy skin
ww Severely mottled or cyanotic skin color
Clinical Presentation ww Altered mental state, lethargic
Early signs of shock include tachycardia; cool, clammy, pale, or mot- ww Delayed capillary refill time
tled skin; and delayed capillary refill time. The patient may have a ww Decreased or absent urine output
history of decreased urine output. In this early compensated stage, Irreversible
perfusion to vital organs, such as the brain and heart, is main- ww Multiple organ failure and death
tained by compensatory physiologic processes. As the shock state
527

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 528 PART 5: ACUTE AND EMERGENT PROBLEMS

perfusion and poor pulses, whereas adolescents presented with

hypotension. Irreversible shock occurs when multiple organs fail
and death occurs.
B

Muscle fiber performance

Pathophysiology
Shock occurs when oxygen delivery to tissues is impaired.
A
Adequate oxygen delivery depends on sufficient blood oxygen
content and adequate circulatory blood flow (Figure 74.1). The
oxygen content in blood depends primarily on the concentration
of hemoglobin and the amount of oxygen bound to hemoglobin.
In children, oxygen consumption by end organs depends most on
oxygen delivery, whereas in adults it depends on oxygen extrac-
tion by the tissues.
Blood flow, or cardiac output, is determined by heart rate and Muscle fiber length
stroke volume. Stroke volume depends on preload, contractility, and
afterload. Preload refers to the amount of blood entering the heart
from the systemic vasculature. Increasing preload, for example,
Figure 74.2. Starling curve of cardiac output. As muscle fiber length increases
via administration of intravenous (IV) fluid boluses, will increase (A), performance increases. After muscle fiber reaches its optimal length
cardiac output until a point of optimal heart muscle fiber length is (B), performance declines.
reached. The Starling curve demonstrates that increased stretch-
ing of a muscle fiber results in improved performance of that mus-
cle fiber (ie, improved stroke volume and cardiac output); however, redistribute blood to essential organs, such as the heart and brain).
after the point of optimal stretching is reached, performance declines The vasoconstriction in children may make hypotension a late sign
(Figure 74.2). of shock. Irreversible septic shock in adults is often caused by vaso-
Contractility, or inotropy, is the intrinsic ability of the heart to motor collapse, whereas in children cardiac failure plays a larger
contract and pump blood to the body. Afterload refers to the systemic role in this type of shock.
vascular resistance impeding ejection of blood from the ventricles.
Optimal cardiac output depends on sufficient preload, unimpaired Differential Diagnosis
cardiac contractility, and the ability of the heart to overcome any The several types of shock differ based on the underlying patho-
afterload. During states of decreased cardiac output resulting in physiology (Table 74.1). Hypovolemic shock is the most common
decreased tissue perfusion, adults compensate primarily by decreas- type occurring in children and usually results from dehydration
ing systemic vascular resistance, increasing cardiac contractility, or traumatic hemorrhage. Hypovolemia results in inadequate
and increasing heart rate, whereas children compensate primarily by preload, which leads to impaired cardiac output and impaired
increasing their heart rate and by vasoconstriction (to preferentially perfusion. Other causes of hypovolemic shock include dehydra-
tion caused by osmotic diuresis in diabetic ketoacidosis and third
spacing of fluids (ie, shifting from intravascular to extravascular
Preload sites) from peritonitis and burns. Nontraumatic hemorrhage may
Contractility occur from entities such as epistaxis, gastrointestinal bleeding, and
Afterload vessel fistula formation.
Stroke Distributive shock is a relative hypovolemia; vasodilation results
volume
in inadequate circulating blood volume relative to the vasodila-
tion (ie, the “tank” has been made larger by vasodilation, result-
Cardiac Blood
output flow ing in insufficient fluid to fill the tank). Causes include anaphylaxis
and sepsis, which result in the release of vasoactive mediators that
Heart
Oxygen
cause vasodilation. Spinal cord injury, which can result in neu-
rate rogenic shock, may result in loss of sympathetic nerve-mediated
delivery to
tissues vascular tone and subsequent vasodilation. Certain ingestions, such
as iron, barbiturates, and tricyclic antidepressants, can cause vaso-
Hemoglobin concentration Blood dilation and distributive shock.
oxygen
Oxygen bound to hemoglobin content
Cardiogenic shock is an uncommon but important cause of shock
in children. Congestive heart failure caused by a congenital heart
Figure 74.1. Pathophysiology of shock. Factors affecting oxygen delivery to lesion, myocarditis, or cardiomyopathy results in impaired cardiac
tissue. contractility and decreased cardiac output. Tachydysrhythmias, such

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 Chapter 74: Shock 529

Table 74.1. Types of Shock professional must consider whether the child’s history is consistent
with risk for shock and whether the physical examination as a whole
Type of Shock Physiologic Mechanism Common Causes
supports the diagnosis.
Hypovolemic Inadequate preload Dehydration
Traumatic hemorrhage
Evaluation
Nontraumatic hemorrhage
Early recognition and prompt treatment of shock is the goal. A rapid,
Diabetic ketoacidosis
focused history and physical examination should be performed to
Peritonitis identify patients in shock, and early therapy should be instituted
Burns before taking the time to perform a more complete evaluation.
Distributive Relative hypovolemia Sepsis Recognition of shock depends on history and physical examina-
resulting from vasodilation Anaphylaxis tion alone; therapy should never be withheld while awaiting results
Neurogenic of diagnostic tests.
Toxin-mediated History
Cardiogenic Decreased contractility Congestive heart failure from A history of vomiting with or without diarrhea, decreased oral
congenital lesions intake, and decreased urine output, especially in infants, should
Myocarditis alert the physician to possible hypovolemic shock. Children pre-
Tachydysrhythmias senting with major trauma should be evaluated for hemorrhagic
Obstructive Impaired cardiac output to Pulmonary embolus shock. A history of fever, lethargy, or irritability, and sometimes a
systemic circulation Pericardial tamponade rash, may point toward septic shock. Patients with asplenia, sickle
cell disease, or indwelling catheters and those who are immuno-
Tension pneumothorax
compromised (eg, young infants or children on chemotherapy) are
Ductal-dependent cardiac at increased risk for sepsis. Children in cardiogenic shock may have
lesions a history of a murmur, poor feeding, sweating with feeds, cyano-
Dissociative Abnormal hemoglobin— Carbon monoxide poisoning sis, tachypnea, or dyspnea, and the older child may have a history
inadequate oxygen bound Methemoglobinemia of palpitations.

Physical Examination
as supraventricular tachycardia, may also result in cardiogenic shock A brief physical examination to identify shock focuses on
because they do not allow sufficient time for the ventricles to fill with mental status, vital signs, pulses, and skin signs. Impaired level of
blood, resulting in decreased stroke volume. consciousness, such as lethargy or lack of recognition of parents,
Rare causes of shock in pediatric patients include obstructive and occurs later in shock. Earlier in the process, children are anxious,
dissociative types. In obstructive shock, cardiac output to the sys- fussy, or irritable. Tachycardia occurs early in shock but must be
temic circulation is obstructed as the result of pulmonary embolus, interpreted in the context of other signs of shock, because tachycar-
cardiac tamponade, or tension pneumothorax. Closure of the duc- dia also may result from fever, pain, or fear of the examination pro-
tus arteriosus in a neonate with a ductal-dependent congenital heart cess. Bradycardia is a late, ominous sign in shock and often results
lesion is another cause of insufficient cardiac output and obstructive from hypoxemia. Hypotension is also a late sign in pediatric shock.
shock. In dissociative shock, abnormal hemoglobin (eg, methemo- It is important to remember that normal values for heart rate and
globin), or carboxyhemoglobin caused by carbon monoxide poison- blood pressure vary by age. The lower limit of acceptable systolic
ing results in decreased oxygen bound to hemoglobin and decreased blood pressure in a neonate from birth to 1 month is 60 mm Hg
oxygen delivered to tissues. and in an infant from 1 month to 1 year is 70 mm Hg. For a child
Septic shock combines elements of distributive, hypovolemic, and 1 year or older, the lower limit can be estimated using the formula
cardiogenic shock. Vasoactive mediators cause decreased systemic 70 + (2 × age in years) mm Hg; the lower limit is 90 mm Hg for
vascular resistance and relative hypovolemia. Third spacing of fluid children 10 years or older. Systolic blood pressures lower than these
results in a true intravascular hypovolemia as well. Additionally, guidelines represent hypotension and late uncompensated shock.
mediators of sepsis cause impaired cardiac function. Heart rate and blood pressure values requiring immediate atten-
Because shock is a physiologic state resulting from a variety of tion are shown in Table 74.2.
etiologies and because it is recognized through clinical findings, it Presence and quality of pulses should be checked. Weak, thready,
is important to interpret individual findings in the context of the or absent peripheral pulses are indicative of shock. However, in
patient as a whole. Heart rate may be elevated for many reasons, warm septic shock, pulses may be bounding. Skin color, moisture,
including fear, anxiety, and fever. Capillary refill may appear delayed and temperature give valuable clues to diagnosis. Children in shock
in the extremities of a child who is cold. Blood pressure may appear may have pale, cyanotic, or mottled skin. Early in shock, however,
artificially low when too large a cuff is used to measure it. The health skin color may be normal. Some infants may also have mottled skin

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 530 PART 5: ACUTE AND EMERGENT PROBLEMS

Table 74.2. Critically Abnormal Heart Rate Approximately 20% of children with septic shock present with
and Blood Pressure the classic adult form of warm shock, including increased car-
diac output, hypotension, decreased systemic vascular resistance,
Age Bradycardia Tachycardia Hypotension
warm non-mottled skin, bounding pulses, and flash capillary refill.
Neonate 0–28 days <100 bpm >180 bpm <60 mm Hg Because children compensate for shock with vasoconstriction, they
Infant 1–12 months <90 bpm >160 bpm <70 mm Hg are more likely than adults to present with cold septic shock, includ-
Child 1–10 years <60 bpm >140 bpm <70 + (2 × age) mm Hg ing decreased cardiac output; increased systemic vascular resistance;
Child >10 years <60 bpm >120 bpm <90 mm Hg normal blood pressure to hypotension; cool, clammy, or mottled
skin; thready pulses; and delayed capillary refill. The remaining 20%
Abbreviation: bpm, beats per minute.
of children with septic shock present with both decreased cardiac
output and decreased systemic vascular resistance. Petechiae or pur-
pura are suggestive of meningococcemia as the etiology of septic
normally. As with tachycardia, isolated signs must be correlated with shock. A sunburn-like rash may occur in patients with toxic shock
the bigger clinical picture to diagnose shock. Decreased perfusion syndrome caused by streptococcus or staphylococcus.
in shock results in cool and clammy skin. This is often best initially
appreciated in the hands and feet. Laboratory Tests
Capillary refill is tested by compressing the capillary bed of a fin- The suspected cause of shock dictates which laboratory tests are
gertip, palm, or dorsal foot with gentle pressure until it blanches. On performed. In hypovolemic shock secondary to dehydration, a chem-
release, color should return in 2 seconds or less; a capillary refill time istry panel should be obtained for electrolyte abnormalities and
of 3 seconds or more is abnormal and indicative of shock. Children acidosis. Serial hematocrit determinations and a type and
in warm septic shock may display “flash” (ie, shortened) capillary crossmatch are important studies in traumatic and nontraumatic
refill time. Capillary refill should be tested with the extremity ele- hemorrhage, whether known or suspected. In septic shock, a com-
vated above the heart so that arterial, not venous, perfusion is tested. plete blood cell count and blood cultures should be obtained, as
Additionally, cool ambient temperatures can falsely delay capillary well as cultures of other potential sources of infection (eg, urine,
refill times. cerebrospinal fluid, wound, indwelling venous access line). Results
In hypovolemic shock caused by dehydration, the patient should of coagulation studies, including panels to evaluate for dissemi-
be assessed for signs of dehydration, such as dry mucous mem- nated intravascular coagulopathy, and results of electrolyte studies,
branes, lack of tears, sunken eyes, sunken anterior fontanelle in including calcium and magnesium levels, are frequently abnor-
infants, and poor skin turgor. Often, the degree of dehydration can mal in sepsis. Hypoglycemia is a common finding in any type
be estimated clinically (see Chapter 80). Patients with hemorrhage, of shock, and a rapid bedside glucose determination should be per-
whether traumatic or nontraumatic, must be examined thoroughly formed for all critically ill pediatric patients. Arterial blood gases
to locate the source of hemorrhage. can demonstrate adequacy of oxygenation and degree of acido-
Children with congestive heart failure and cardiogenic shock sis and are necessary to diagnose elevated carboxyhemoglobin
may demonstrate dyspnea on exertion, tachypnea, orthop- and methemoglobin levels. Initial lactate levels, particularly in
nea, rales, hepatomegaly, gallop rhythm, and a heart murmur; patients with septic shock and in trauma patients, may be corre-
these physical examination signs may be difficult to appreciate lated with overall prognosis and can be followed serially to chart
in a tachycardic, fussy, ill child. Jugular venous distention and progress. Procalcitonin is another increasingly popular biomarker
peripheral edema are appreciated less often in children com- followed in suspected sepsis. Troponins may be useful in deter-
pared with adults. Other signs may include hepatomegaly and/ mining severity of disease and following patients with cardiogenic
or cardiomegaly on chest radiography as well as a differential shock. D-dimer assay is useful in patients with suspected pulmo-
in pulses, blood pressure, or pulse oximetry between upper and nary embolism.
lower extremities.
Ductal-dependent cardiogenic shock should be suspected in the Other Studies
newborn who presents with shock and/or severe cyanosis unre- Chest radiography, electrocardiography, and echocardiography
sponsive to oxygen therapy in the first few weeks after birth. Cardiac may be obtained for patients with cardiogenic or ductal-dependent
tamponade is suspected in the patient with muffled or decreased obstructive shock to further elucidate the specific etiology. Workup
heart tones, paradoxical pulse (ie, decrease in systolic blood pres- of stabilized trauma patients may include bedside ultrasonography,
sure >10 mm Hg during inspiration), and distended neck veins. radiography, or computed tomography. Imaging studies contrib-
Tension pneumothorax is suspected in patients with deviated tra- ute to the diagnoses of cardiac tamponade, tension pneumotho-
chea (ie, away from the affected side), decreased breath sounds and rax, and pulmonary embolism. Invasive monitoring with arterial
hyperresonance to percussion on the affected side, and distended lines for systemic arterial blood pressure and central venous lines
neck veins. Pulmonary embolism is rare in pediatric patients, and for central venous pressure or pulmonary artery wedge pressure
the signs are subtle. It is mainly suspected in the presence of pre- may be helpful in the ongoing management of shock, particularly
disposing factors. fluid-resistant shock.

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 Chapter 74: Shock 531

Management A well-designed and executed trial performed in Africa, the

Fluid Expansion as Supportive Therapy (FEAST) trial, has called
The first management priority in the treatment of any critically ill
into question aggressive fluid resuscitation in patients with septic
child is attention to airway patency and ventilation. For the patient
shock. Although patients randomized to rapid fluid therapy showed
with significant respiratory compromise, bag-and-mask ventilation
earlier improvement in circulatory parameters, their mortality was
followed by endotracheal intubation is performed. Usually, however,
consistently higher. It has been suggested that the results of this trial
patients in compensated shock do not require initial advanced air-
may not be generalizable to patients in developed countries because
way management. Instead, the immediate priorities are adminis-
of differences in malaria infection, nutritional status, and other fac-
tration of oxygen and initiation of cardiorespiratory monitoring.
tors. The authors suggest that aggressive fluid therapy is particu-
Oxygen by mask or nasal cannula, preferably heated and humidified,
larly associated with increased mortality in highly acidotic patients,
should be administered immediately. Elective rather than emergent
especially in developing countries in which inotropic support and
endotracheal intubation should be considered to reduce metabolic
mechanical ventilation may not be available. Currently, expert guide-
demands caused by increased work of breathing. When intubating
lines still recommend early aggressive fluid therapy for septic shock
and sedating patients in shock, sedative agents with fewer hemody-
in developed countries in which inotropes and mechanical ventila-
namic effects, such as ketamine or fentanyl, are preferred over those
tion are available.
that may contribute to hypotension, such as other opiates, benzo-
The mnemonic SHOCKED (sound the alarm, help hypovole-
diazepines, and propofol. Etomidate, however, although its effects
mia, optimize oxygenation, constrict and contract, keep in mind
are hemodynamically neutral, is not recommended in septic shock
underlying causes, electrolytes and glucose normalized, decrease
because of its cortisol suppressive effect. Mechanical ventilation set-
metabolic demand) may be used to recall overall management
tings should emphasize a lung-protective approach.
(Box 74.2). Further management in addition to fluids is depen-
Almost concurrently, the next priority is achieving intravascu-
dent on the specific etiology. Patients in septic shock should receive
lar access and, in most cases, administering fluids. Peripheral IV
empiric broad-spectrum antibiotic coverage within the first hour
access should be attempted. If this is unsuccessful after 3 attempts
after presentation. Patients with toxic shock syndrome should
or 90 seconds, an intraosseous line may be placed or IV access may
receive antibiotics, including clindamycin. A surgeon must assist
be obtained by placement of a central venous catheter or by cut-
in identifying the source of hemorrhage and controlling the bleed-
down technique. Ultrasound-guided vascular access is another
ing in patients with traumatic hemorrhage and may be required for
option. Intraosseous lines have been demonstrated to provide much
more rapid vascular access compared with central line placement.
Umbilical venous lines can sometimes be placed in neonates within
the first 1 to 2 weeks after birth. More than 1 intravascular line is Box 74.2. SHOCKED Mnemonic
usually needed for managing patients in shock. for Management of Shock
Decreased preload and hypovolemia (actual or relative) are pres-
ww Sound the alarm: Obtain help from consultants, intensivists, and
ent in the most common causes of pediatric shock. Cardiogenic shock
ancillary personnel. Move the patient to a monitored room and place on
is the only form of shock that may not benefit from increasing pre-
a cardiorespiratory monitor, automated blood pressure measurement,
load via a fluid bolus. For other forms of shock, an initial fluid bolus
and pulse oximetry.
of 20 mL/kg isotonic crystalloid fluid (maximum 1 L), such as nor-
ww Help hypovolemia: Get intravascular access and start 20 mL/kg
mal saline or lactated Ringer solution, should be rapidly infused over
crystalloid fluid bolus. Reassess after each bolus and continue giving
5 to 10 minutes; this may require manually pushing the fluid using
boluses unless hepatomegaly or rales develop. Patients may require
a large syringe or a pressure bag. Fluid boluses can be hand pushed
≥80 mL/kg of fluids.
into an intraosseous line. Colloid fluid (eg, albumin) theoretically has
ww Optimize oxygenation: Give supplemental oxygen regardless of pulse
the advantage of remaining intravascular a longer period of time than
oximetry values. Consider elective intubation and artificial ventilation to
normal saline, but this has not been proved to result in a measurable
reduce metabolic demands as indicated. Transfusion may be required if
benefit. Crystalloid fluid is recommended for initial boluses because
hemoglobin is low (<8–10 g/dL).
it is less expensive and more readily available than colloid fluid. If a
ww Constrict and contract: Use inotropic and vasoconstrictive agents
patient with traumatic hemorrhage remains hemodynamically unstable
as necessary for fluid-refractory shock.
after 2 crystalloid fluid boluses, packed red blood cells at 10 mL/kg
ww Keep in mind underlying causes: Give or apply therapies specific to
may be required. The patient should be assessed for improvement in
the underlying cause.
mentation, vital signs, peripheral pulses, and skin signs after each
ww Electrolytes and glucose normalized: Measure and normalize
fluid bolus. Repeat fluid boluses of 20 mL/kg (1 L) to a total of 80 mL/kg
electrolytes (especially calcium) and glucose. Control hyperglycemia,
(4 L) or more may be necessary to restore intravascular volume.
with a target of ≤180 mg/dL.
Patients should be reassessed after each bolus before ordering another
ww Decrease metabolic demand: Manage hyperthermia and pain to
bolus. Development of hepatomegaly or rales may indicate fluid over-
reduce metabolic demand on the patient. Consider elective intubation.
load and the need to begin other therapies, such as vasoactive infu-
Keep patient nil per os (ie, nothing by mouth).
sions. This is particularly true for cardiogenic and septic shock.

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 532 PART 5: ACUTE AND EMERGENT PROBLEMS

management of nontraumatic hemorrhage as well depending on the improvements in cardiac contractility and cardiac output without
specific etiology. Blood transfusions may be required. Spinal cord compromising renal perfusion or worsening hypotension. Dopamine
injury is treated with supportive care in consultation with a neu- and dobutamine may be less effective in infants younger than
rosurgeon. Anaphylactic shock is treated with IV epinephrine, IV 12 months than in older children. This is another reason that some
diphenhydramine, antihistamine H2 receptor blockers, glucocorti- institutions recommend epinephrine as the first-line inotropic agent.
coids, and nebulized albuterol. Pericardial tamponade is relieved by Patients in cardiogenic shock or cold septic shock may also ben-
pericardiocentesis, tension pneumothorax by needle or tube thora- efit from afterload reduction using systemic vasodilators, such as
costomy, and pulmonary embolus with supportive care and throm- nitroprusside (0.5–5 mcg/kg/min). If these are used, blood pres-
bolytic agents. Carbon monoxide poisoning is managed with 100% sure should be continuously monitored, typically in the setting of an
oxygen and, if severe, hyperbaric oxygen therapy. Patients with met- intensive care unit. Cold septic shock refractory to epinephrine may
hemoglobinemia appear cyanotic even while receiving 100% oxygen also be treated with type 3 phosphodiesterase inhibitors (eg, inamri-
and may be treated with methylene blue. Supraventricular tachy- none 1–20 mcg/kg/min, milrinone 0.25–1.0 mcg/kg/min), which
cardia should be managed with adenosine if the patient is hemody- exert inotropic and vasodilator actions (ie, inodilators). Typically, a
namically stable and with synchronized cardioversion if the patient pediatric intensive care specialist should be involved in the care of
is unstable. Ductal-dependent obstructive shock should be treated the patient, and central venous pressure monitoring should be begun
with prostaglandin E1 (PGE1) infusion. before vasodilator agents are started.
Patients with cardiogenic shock require inotropic agents to Neonates with ductal-dependent lesions present with a sudden
increase cardiac contractility and improve tissue perfusion. Patients onset of shock and cyanosis, typically in the first 2 weeks after birth.
in the later stages of other forms of shock (eg, hypovolemic, distrib- Common lesions include hypoplastic left heart syndrome, aortic
utive, septic) may also suffer cardiac dysfunction. In such patients, coarctation, and tricuspid atresia. Prostaglandin E1 (0.1 mcg/kg/
only after adequate fluid resuscitation has been performed and signs min, titrated to effect), which acts to keep the ductus arteriosus
of shock or hypotension persist (ie, fluid-refractory shock) should open, should be immediately infused if a ductal-dependent lesion is
inotropic agents be started. Central venous pressure monitoring may suspected as the cause of shock. Apnea may result from PGE1 ther-
be necessary to determine whether fluid resuscitation is adequate. apy, and attention to airway management is of critical importance.
Patients with septic shock may require vasoactive agents to reduce Historically, it was common to mix vasoactive infusions using
or increase systemic vascular resistance. the “rule of 6 and 0.6.” That is, for dopamine, dobutamine, and
Epinephrine or dopamine is often the first-line inotropic agent, nitroprusside, mix 6 mcg/kg of drug with enough dextrose 5% in
with recent literature and expert opinion favoring epinephrine. water to produce a final volume of 100 mL. Infusion at 1 mL per
Guidelines recommend beginning inotropic agents when indi- hour provides a dose of 1 mcg/kg per minute. For epinephrine,
cated in a peripheral line until a central line is available; that is, norepinephrine, and PGE1, mix 0.6 mcg/kg of drug with enough
do not delay. At low doses (2–5 mcg/kg/min), dopamine improves dextrose 5% in water to produce a final volume of 100 mL.
renal blood flow and enhances urine output. At midrange doses Infusion of 1 mL per hour provides a dose of 0.1 mcg/kg per min-
(5–10 mcg/kg/min), dopamine exerts primarily a b-adrenergic effect, ute. Calculations such as these are prone to error, however, and
improving contractility and increasing heart rate. At higher doses computerized order forms with automatic error alerts or “smart”
(10–20 mcg/kg/min), the a-adrenergic effects of dopamine cause pumps that automatically calculate doses based on the patient’s
peripheral vasoconstriction to improve hypotension. Recent data input weight are better choices.
show increased mortality and increased dysrhythmias in patients Treatment of patients in shock must include attention to condi-
receiving dopamine as first-line inotrope; as a result, epinephrine tions that increase metabolic demand. Acidosis should be assessed,
has become the preferred first-line therapy. Epinephrine has pre- and medical therapy and ventilator management should be done
dominantly b-adrenergic effects at lower doses (0.05–0.1 mcg/kg/ with the intent to improve acid-base status. Temperature should be
min) and a-adrenergic effects at higher doses (≤1.0 mcg/kg/min). kept neutral, with antipyretic agents and cooling measures used as
Because epinephrine is a strong inotrope, it is recommended for needed. Electrolyte abnormalities, particularly hypocalcemia and
cold septic shock. Norepinephrine (0.01–1.0 mcg/kg/min) has pre- hypoglycemia, must be assessed and corrected. If a hypothyroid
dominantly a-adrenergic vasoconstrictive effects and therefore is state is suspected, thyroid hormone replacement therapy is impor-
preferred for warm septic shock with low systemic vascular resis- tant. Blood products may be required for patients with septic shock
tance and for distributive shock states (eg, anaphylaxis, neurogenic and disseminated intravascular coagulation. Packed red blood cells,
shock, certain toxin-induced shock states). Dobutamine (1–20 10 mL/kg at a time, should be administered to maintain hemoglo-
mcg/kg/min) may be the most useful drug for cardiogenic shock bin of at least 10 g/dL for unstable, hypoxemic, or hemorrhaging
because it is selective for b-adrenergic effects, thereby increas- patients, and 7 to 9 g/dL for stable patients. Fresh frozen plasma
ing cardiac contractility. In the setting of hypotension, however, may be administered to correct abnormalities in prothrombin and
dobutamine-mediated peripheral vasodilation may be detrimental. partial thromboplastin times but should not be pushed because of
Dobutamine is typically used in a range of 10 to 20 mcg/kg per min- its propensity to cause further hypotension. Cryoprecipitate should
ute. Combinations of inotropic agents may be beneficial to maximize be reserved for documented hypofibrinogenemic states.

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 Chapter 74: Shock 533

An expert panel from the American College of Critical Care to ventricular assist devices or extracorporeal membrane oxygen-
Medicine created clinical practice parameters for the treatment ation. Recombinant activated protein C (ie, drotrecogin alfa) was
of pediatric septic shock (Figure 74.3). Recommended therapy is recommended for some septic adult patients but not for pediatric
divided between therapies for the first hour (ie, “golden hour”) and patients, and the commercial product Xigris is no longer available.
therapies beyond the first hour (often with critical care specialists
involved). In the first 15 minutes after recognition of septic shock, Prevention
practitioners should attend to the airway and establish intravascu- Improving outcomes is focused primarily on early recognition and
lar access, begin fluid boluses in 20 mL/kg increments, and diag- early appropriate therapy of shock. One study showed significantly
nose and correct any hypoglycemia and hypocalcemia. The goal of reduced morbidity and mortality in shock patients transferred to a
therapy is normalization of heart rate, blood pressure, and capil- tertiary pediatric medical center if community hospital physicians
lary refill (≤2 seconds), no difference between peripheral and cen- recognized shock and used pediatric advanced life support (PALS)–
tral pulses and warm extremities, urine output greater than 1 mL/kg recommended interventions early. Appropriate PALS-recommended
per hour, normal mental status, cardiac index between 3.3 and therapy was defined as more than 20 mL/kg of fluids (except in
6.0 L/min/m2, and superior vena cava (SVC) oxygen saturation those with cardiac conditions) and use of inotropes in patients in
(O2 sat) 70% or higher. Patients who are responsive to fluid may fluid-refractory shock. Unfortunately, although 37% of the patients
be observed in the pediatric intensive care unit. Epinephrine infu- transferred during the study period were in shock, as defined by
sion should be started for those who remain hypotensive after fluid prolonged capillary refill time or hypotension, only 7% were iden-
resuscitation. Norepinephrine infusion is recommended for the less tified as in shock during the referral process. Early recognition of
common warm septic shock state. The guidelines emphasize that compensated shock is a key preventive measure to reduce mortal-
inotropic therapy should not be withheld because there is no cen- ity. In the same study, only 36% of those in shock received appropri-
tral line; it can be administered peripherally if no other options exist. ate PALS-recommended therapy before transfer. Community health
Two studies of patients transferred into a tertiary pediatric medi- professionals must concentrate on obtaining vascular access (with
cal center showed significantly reduced mortality and morbidity for
an intraosseous needle, if necessary), giving fluid boluses early, and
patients cared for by community practitioners who followed these
starting inotropes (through a peripheral IV line, if necessary) for the
guidelines for the first hour of care.
management of fluid-refractory shock within the first hour. Sepsis
Corticosteroids are controversial in the management of sepsis.
recognition bundles including a trigger tool based on vital signs,
Guidelines suggest administering “stress doses” of hydrocortisone
triage physical examination, and patient risk factors (the trigger
2 mg/kg or 50 mg/m2 body surface area beyond the first hour of
tool is often incorporated into electronic medical record systems)
therapy for catecholamine-resistant septic shock in cases of sus-
with rapid clinician assessment within 15 minutes for those that are
pected adrenal insufficiency. Adrenal insufficiency may be suspected
trigger positive, are recommended to improve recognition. Early
in patients with a history of a central nervous system abnormality
therapy, which often occurs before the patient reaches a tertiary care
or pituitary abnormality, a known adrenal gland disorder, recent center, is another important preventive measure. Rapid response
surgery, history of chronic steroid therapy (eg, for asthma, inflam- teams are increasingly being used in hospitals to institute appro-
matory bowel disease, a rheumatologic condition), and in purpura priate medical therapy for inpatients with concerning symptoms or
fulminans. “Shock doses” of hydrocortisone (50 mg/kg) may be vital signs. At a minimum, community pediatricians should have the
administered in the setting of catecholamine-resistant fulminant ability to administer oxygen and obtain intravascular or intraosse-
septic shock and dopamine-resistant purpura fulminans. It is sug- ous access and administer rapid fluid boluses in their offices. Early
gested that a baseline cortisol level be drawn before administering broad-spectrum antibiotic therapy within 60 minutes of recogni-
corticosteroids. tion of possible sepsis (along with blood culture, but only if it does
After the first hour of therapy, a vasodilator (eg, nitroprusside) not delay antibiotic administration) is another important element
or type 3 phosphodiesterase inhibitor (ie, inamrinone, milrinone) of recommended resuscitation bundles.
along with further volume loading may be helpful in catecholamine-
resistant cold septic shock with normal blood pressure and SVC O2
sat less than 70%. In cases of cold septic shock with low blood pres- Prognosis
sure and SVC O2 sat less than 70%, continued titration of epinephrine Children in shock are critically ill and at risk for progression to mul-
and volume, addition of norepinephrine, and consideration of other tiorgan failure and death. Prognosis depends on how early shock is
vasoactive drugs is recommended. For warm septic shock, contin- recognized and treated and on the underlying etiology. Pediatric
ued titration of norepinephrine and volume is recommended, with septic shock carries a 2% mortality rate in previously healthy chil-
the possible addition of vasopressin (0.0003–0.0008 U/kg/min), terli- dren but an 8% mortality rate in children with chronic illness. These
pressin, or angiotensin, and consideration of other vasoactive drugs. rates are significantly improved from 60% in the 1980s and 97% in
Vasopressin and terlipressin have not been well studied in children the 1960s and are also lower than the adult septic shock mortality
but show promise in adults. For persistent catecholamine-resistant rate. Mortality rates are even lower with prompt recognition and
shock (particularly septic or cardiogenic), consideration may be given adequate treatment.

BPPCA6e_Ch074_0527-0536.indd 533 2/13/20 6:10 PM

 534 PART 5: ACUTE AND EMERGENT PROBLEMS

0 min Recognize decreased mental status and perfusion.

Begin high flow O2 and establish IO/IV access according to PALS.

5 min

If no hepatomegaly or rales/crackles then push 20 mL/kg

isotonic saline boluses and reassess after each bolus up to
60 mL/kg until improved perfusion. Stop for rales, crackles
or hepatomegaly. Correct hypoglycemia and hypocalcemia.
Begin antibiotics.
15 min Fluid refractory shock?

Begin peripheral IV/IO inotrope infusion, preferably Epinephrine 0.05–0.3 µg/kg/min

Use Atropine/Ketamine IV/IO/IM if needed for Central Vein or Airway Access

Titrate Epinephrine 0.05–0.3 µg/kg/min for Cold Shock.

(Titrate Central Dopamine 5–9 µg/kg/min if Epinephrine not available)
Titrate central Norepinephrine from 0.05 µg/kg/min and upward to reverse Warm Shock.
(Titrate Cental Dopamine ≥ 10 µg/kg/min if Norepinephrine not available)
60 min
Catecholamine resistant shock?

If at risk for Absolute Adrenal Insufficiency consider Hydrocortisone.

Use doppler US, PICCO, FATD or PAC to Direct Fluid, Inotrope, Vasopressor, Vasodilators.
Goal is normal MAP-CVP, ScvO2 >70%* and CI 3.3–6.0 L/min/m2.

Normal Blood Pressure Low Blood Pressure Low Blood Pressure

Cold Shock Cold Shock Warm Shock
ScvO2 <70%*/Hgb >10g/dL ScvO2 <70%*/Hgb >10g/dL ScvO2 <70%*
on Epinephrine? on Epinephrine? on Norepinephrine?

Begin Milrinone infusion. Add Norepinephrine to If euvolemic, add Vasopressin,

Add Nitroso-vasodilator if CI Epinephrine to attain normal Terlipressin, or Anigotensin.
<3.3 L/min/m2 with High SVRI diastolic blood pressure. if But, if CI decreases below 3.3
and/or poor skin perfusion. CI <3.3 L/min/m2 and L/min/m2 add Epinephrine,
Consider Levosimendan if Dobutamine, Enoximone, Dobutamine, Enoximone,
unsuccessful. Levosimendan, or Milrinone. Levosimendan.

Persistent catecholamine resistant shock Refractory shock

Evaluate pericardial effusion or

ECMO
Pneumothorax, maintain IAP <12 mmHg

Figure 74.3. American College of Critical Care Medicine algorithm for time-sensitive, goal-directed stepwise management of hemodynamic support in infants
and children. Proceed to next step if shock persists. 1) First hour goals: Restore and maintain heart rate thresholds, capillary refill within 2 seconds, and normal
blood pressure in the first hour/emergency department. 2) Subsequent intensive care unit goals: If shock is not reversed, proceed to restore and maintain
normal perfusion pressure (MAP – CVP) for age, ScvO2 > 70% (* except congenital heart patients with mixing lesions), and cardiac index of 3.3 to 6.0 L/min/m2
in the pediatric intensive care unit.
Abbreviations: CI, cardiac index; CVP, central venous pressure; ECMO, extracorporeal membrane oxygenation; FATD, femoral arterial thermodilution method; Hgb, hemoglobin;
IAP, intra-abdominal pressure; ICU, intensive care unit; IM, intramuscular; IO, intraosseous; IV, intravenous; MAP, mean arterial pressure; PAC, premature atrial contractions; PALS,
pediatric advanced life support; PICCO, Pulse index Continuous Cardiac Output; ScvO2, central venous oxygen saturation; SVC, superior vena cava; SVRI, systemic vascular resistance
index; US, ultrasonography.
Reprinted with permission from Davis AL, Carcillo JA, Aneja RK, et al. American College of Critical Care Medicine clinical practice parameters for hemodynamic support of pediatric and
neonatal septic shock. Crit Care Med. 2017;45(6):1061–1093.

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 Chapter 74: Shock 535

Crit Care Med. 2013;41(2):580–637 PMID: 23353941 https://doi.org/10.1097/

CASE RESOLUTION CCM.0b013e31827e83af

The boy is in barely compensated (ie, not hypotensive) hypovolemic shock Fisher JD, Nelson DG, Beyersdorf H, Satkowiak LJ. Clinical spectrum of shock in
resulting from diarrhea, vomiting, and dehydration. He should receive oxygen the pediatric emergency department. Pediatr Emerg Care. 2010;26(9):622–625
and cardiorespiratory monitoring, and IV access should be rapidly established. PMID: 20805778 https://doi.org/10.1097/PEC.0b013e3181ef04b9
Isotonic fluid boluses of 20 mL/kg should be given, with reassessment performed Han YY, Carcillo JA, Dragotta MA, et al. Early reversal of pediatric-neonatal
between each bolus. As much as 80 mL/kg may be needed before improvements septic shock by community physicians is associated with improved outcome.
in mentation, vital signs, pulses, and skin signs are evident. Pediatrics. 2003;112(4):793–799 PMID: 14523168 https://doi.org/10.1542/
peds.112.4.793
Louden DT, Rutman LE. Inotropic therapy for sepsis. Pediatr Emerg
Selected References Care. 2018;34(2):132–135 PMID: 29384994 https://doi.org/10.1097/
PEC.0000000000001399
Aneja RK, Carcillo JA. Differences between adult and pediatric septic shock.
Minerva Anestesiol. 2011;77(10):986–992 PMID: 21952599 Maitland K, George EC, Evans JA, et al; FEAST trial group. Exploring mechanisms
of excess mortality with early fluid resuscitation: insights from the FEAST trial.
Carcillo JA, Kuch BA, Han YY, et al. Mortality and functional morbidity after
BMC Med. 2013;11:68 PMID: 23496872 https://doi.org/10.1186/1741-7015-11-68
use of PALS/APLS by community physicians. Pediatrics. 2009;124(2):500–508
PMID: 19651576 https://doi.org/10.1542/peds.2008-1967 Mendelson J. Emergency department management of pediatric shock. Emerg
Med Clin North Am. 2018;36(2):427–440 PMID: 29622332 https://doi.
Davis AL, Carcillo JA, Aneja RK, et al. American College of Critical Care Medicine
org/10.1016/j.emc.2017.12.010
clinical practice parameters for hemodynamic support of pediatric and neona-
tal septic shock. Crit Care Med. 2017;45(6):1061–1093 PMID: 28509730 https:// Mtaweh H, Trakas EV, Su E, Carcillo JA, Aneja RK. Advances in monitoring
doi.org/10.1097/CCM.0000000000002425 and management of shock. Pediatr Clin North Am. 2013;60(3):641–654 PMID:
23639660 https://doi.org/10.1016/j.pcl.2013.02.013
Dellinger RP, Levy MM, Rhodes A, et al; Surviving Sepsis Campaign Guidelines
Committee including the Pediatric Subgroup. Surviving sepsis campaign: inter- Subramaniam S, Rutman M. Cardiogenic shock. Pediatr Rev. 2015;36(5):
national guidelines for management of severe sepsis and septic shock: 2012. 225–226 PMID: 25934914 https://doi.org/10.1542/pir.36-5-225

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BPPCA6e_Ch074_0527-0536.indd 536 2/13/20 6:10 PM
 CHAPTER 75

Approach to the
Traumatized Child
David B. Burbulys, MD

CASE STUDY
A 6-year-old boy is brought to the emergency depart- Questions
ment after being struck by an automobile while crossing 1. What are the most common mechanisms of injury
the street. He was found unconscious at the scene. Initial responsible for trauma in children?
evaluation shows that he has an altered level of con- 2. What are some of the physiologic differences
sciousness, shallow respirations, ecchymosis across the between adults and children that make children
upper abdomen, and a deformed, swollen left thigh. The more susceptible to certain types of injury?
pediatric emergency physician is called in to discuss an 3. Which areas of the body are most likely to be injured
initial assessment and management plan for the injured in a typical automobile versus pedestrian collision?
child with the trauma surgeon. 4. What are the components of a primary survey in
pediatric trauma patients?
5. What radiologic and laboratory studies should be
performed in children with multiple injuries?

Trauma is often referred to as the neglected disease of modern soci-

Box 75.1. Characteristics of Children That Result
ety. Childhood trauma, in particular, is poorly understood and stud-
in Increased Susceptibility to Injury
ied. Death from trauma is higher in pediatric patients than in adult
patients. Mechanisms of injury may be similar in adults and chil- Anatomic
dren, but children have particular anatomic differences and physi- ww Smaller body size allows for the greater distribution of force with
ologic responses to injury. Health professionals should realize that trauma, so multisystem injury is common.
children have unique anatomic and physiologic features compared ww A prominent occiput, exaggerated head-to-body ratio, weak neck
with adults (Box 75.1). Evaluation and management of traumatized muscles, and higher center of gravity predispose younger children to
children requires specialized knowledge, training, and equipment. head injury.
Recognition of such facts, coupled with expertise in the performance ww Cranial bones are thinner, and the brain is less myelinated, resulting
of emergency procedures, has improved the outcomes of children in more serious head injury.
who sustain major injuries. ww Skeletal and ligamentous structures have increased flexibility, which
results in greater transmission of force to internal organs.
Epidemiology ww Less protective muscle and subcutaneous tissue over internal organs
Traumatic injuries are the most common cause of death in children expose them to injury.
aged 1 to 21 years (and in adults aged 21–44 years) in the United ww Growth plates are not yet fused, which results in Salter-Harris-type
States. In many of these age groups, murder and suicide are the sec- fractures and possible bone growth abnormalities with healing.
ond and third leading cause of death, respectively. Approximately 7 Physiologic
to 8 million pediatric injuries result in 250,000 to 500,000 hospital- ww High body surface area-to-weight ratio predisposes children to
izations and 15,000 to 25,000 deaths each year. Medical costs asso- hypothermia, which may complicate shock and worsen acidosis and
ciated with these injuries exceed $50 billion annually. Infants are coagulopathy if it is not corrected.
most likely to die from suffocation, young children from drown- ww Hypoxia and respiratory failure are more likely in children.
ing, school-age children from motor vehicle collisions or after ww Hemorrhagic shock is initially well tolerated by increasing heart rate and
being struck by an automobile, and adolescents from motor vehicle peripheral vascular resistance without significant changes in systolic
crashes. Boys are twice as likely as girls to be injured and die. The blood pressure.
magnitude of pediatric trauma becomes even more evident when
537

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 538 PART 5: ACUTE AND EMERGENT PROBLEMS

morbidity is considered. Between 50,000 and 100,000 children per In adults, blood pressure tends to decline after a less significant
year become permanently disabled as a result of their injuries. Such blood loss, resulting in earlier recognition of the extent of blood
disabilities have an enormous effect on society; they result in finan- loss (Figure 75.2). Subtle changes in heart rate, blood pressure, pulse
cial and emotional losses for families and years of lost productivity pressure, and capillary refill may indicate impending cardiovascu-
for the injured individuals themselves. lar collapse in children who have sustained traumatic injury and
Blunt trauma, which is more common than penetrating injury should not be overlooked. Indicators of end-organ perfusion, such
in children, represents approximately 87% of all childhood injuries. as lactic acid levels or calculated base deficit, may also be helpful
Head injuries, followed by thoracoabdominal injuries, are the lead- and predictive.
ing causes of death in this group. In adolescents and young adults,
however, penetrating injury (ie, homicide, suicide) accounts for a
higher percentage of total trauma, especially among minority pop-
ulations in urban areas. Causes of nonpenetrating trauma are motor Spleen or
chest struck
vehicle crashes (>40%), falls (25%–30%), drowning (10%–15%), by fender
and burns (5%–10%). Included in the remainder are bicycle-related
and automobile versus pedestrian injuries. These numbers vary
significantly by locale and age. In some centers, a high percentage
of trauma deaths are related to child abuse.

Clinical Presentation
Left femur
Children who sustain severe trauma present with multiorgan sys- struck by
tem injury manifested by shock, respiratory failure, or altered mental bumper
status, either alone or in combination. Those with mild to moderate
injury may present in this way or may simply present with localized
signs and symptoms in the injured area.
Lands on right side of head
Pathophysiology
Figure 75.1. The Waddell triad, that is, femur, abdominal, and contralateral
It is important to identify patterns of injury to develop strategies for
head injuries, should be expected to result from automobile versus pedestrian
injury prevention as well as anticipate injuries during treatment. One
collisions in the United States. For example, a child crossing the street is struck
common pattern is the Waddell triad, that is, the triad of injuries that on the left side of the body by an automobile traveling on the right side of the
results from an automobile versus pedestrian collision (Figure 75.1). road. The left femur is likely to be injured by the bumper, and the abdomen
Multisystem injury is the rule rather than the exception in chil- or chest strikes the grille as the child is lifted into the air and lands on the
dren. Internal injury must always be suspected when the mecha- opposite side of the head, sustaining blunt head trauma. The Waddell triad
nism of injury warrants such injury, even in the absence of apparent illustrates the necessity of having a high degree of suspicion for predictable
evidence suggestive of external trauma. Because children are ana- injuries based on a well-known mechanism.
tomically and physiologically different from adults, they are more
susceptible to diverse types of injury (see Box 75.1). The most strik-
ing physiologic differences between adults and children concern
140 Vascular
responses to acute blood loss. Children have a tremendous capac-
120 resistance
ity to maintain systolic blood pressure despite 25% to 30% acute
Percent of Control

blood loss. 100

Hypovolemic shock secondary to acute blood loss is the most 80
common cause of shock in pediatric trauma patients. Hemorrhagic 60
shock is a clinical state in which cardiac output is unable to meet 40
the metabolic demands of tissues for oxygen and nutrients; it is not 20 Cardiac Blood
defined by any absolute blood pressure value. output pressure
0
Acute blood loss stimulates peripheral and central receptors and 0 25 50 75
results in increased production of catecholamines and corticoste- Percent of Blood Volume Deficit
roids. The body responds by increasing peripheral vascular resis-
tance, stroke volume, and heart rate. Children have the capacity to Figure 75.2. Cardiovascular response to hypovolemia in children. Blood
dramatically increase heart rate and peripheral vascular resistance, pressure does not begin to decline until the volume deficit is more than
and they often may exhibit normal blood pressure in the presence 25% because of the compensatory increase in vascular resistance. Cardiac
of hypovolemic shock. By the time their blood pressure falls, they output drops earlier and is manifested clinically as delayed capillary refill;
commonly have lost 20% to 25% of their circulating blood volume. cool, clammy skin; and tachycardia.

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 Chapter 75: Approach to the Traumatized Child 539

Another obstacle to the recognition of shock in children is the subspecialists, emergency nurses, respiratory therapists, social work-
lack of knowledge on the part of many health professionals of age- ers, and radiology technicians.
appropriate vital signs, particularly blood pressure. Table 75.1 gives Several approaches to the assessment of trauma patients have
the normal blood pressure ranges for children of different ages. been developed by professional organizations. The Advanced Trauma
Three stages of shock correspond to the progression of volume Life Support (ATLS) course of the American College of Surgeons and
loss. In the first stage, compensated shock, mechanisms for preserv- the International Trauma Life Support (ITLS) course (formerly Basic
ing blood pressure remain effective. Decreased capillary refill, dimin- Trauma Life Support, which was initially funded by the American
ished pulses, cool extremities, and tachypnea may be apparent, but College of Emergency Physicians) are 2 such approaches. The ATLS
blood pressure is normal (although accompanied by tachycardia). and ITLS methods stress the importance of a primary evaluation, or
Unrecognized, untreated compensated shock rapidly progresses to primary survey, to identify and manage immediate life-threatening
uncompensated shock. Examination reveals decreased level of con- injuries followed by a more detailed regional examination, or sec-
sciousness, pallor, reduced urine output, and lower blood pressure ondary survey, after stabilization, to identify and manage all other
with weak, thready pulses and marked tachycardia. With inadequate injuries. Additionally, both protocols adhere to the principles of
therapy, uncompensated shock becomes irreversible shock, result- serial examination and reassessment after each intervention. The
ing in irreparable organ damage and often unpreventable death. (See primary survey and initial resuscitation efforts must occur simul-
Chapter 74 for a more extensive discussion.) taneously and within the first several minutes of the evaluation. The
Shock has several causes, and it is important to emphasize that secondary survey is meant to enhance the primary survey. Vital signs
in trauma patients, it should always be initially attributed to hemor- should be reassessed frequently during the primary and secondary
rhage. Shock resulting from obstructive cardiac output causes, such survey until the trauma team feels the patient has been adequately
as tension pneumothorax or cardiac tamponade, is much less com- stabilized. The physician should understand the rationale for the
mon. Shock resulting from spinal cord injury is exceedingly rare. trauma examination and its parts. This topic is beyond the scope of
Shock should never be attributed solely to head trauma. The path- this chapter, but articles that explain the rationale for trauma exam-
ways resulting in decreased blood pressure in patients with head ination and provide detailed descriptions of evaluation and man-
trauma are present only at the terminal stages. Therefore, the possi- agement techniques are listed in the Selected References section.
bility of blood loss from internal organs should be pursued promptly
and aggressively. The most common site of hemorrhage resulting in Physical Examination
preventable mortality is intra-abdominal. Other sources of hemor- The primary survey begins with an assessment of level of conscious-
rhage are external, thoracic, pelvic, and retroperitoneal. ness, patency of the airway (Box 75.2), and quality of breathing
(Box 75.3). When evaluating injured patients, physicians should
Evaluation and Management always assume that the cervical spine has been injured and should
use in-line immobilization to secure it. Basic airway maneuvers for
Because of the high potential for serious morbidity and mortality in
positioning should be performed, the safest of which is the jaw thrust
trauma patients, evaluation and management are performed simul-
to avoid moving the cervical spine (Figure 75.3). The oral cavity
taneously. This care is best managed using an organized, multidis-
should be examined for foreign bodies, blood, or secretions. The
ciplinary team approach, with preestablished criteria for activation
most common form of airway obstruction in children is a posteri-
of the trauma team. History of the event provides important infor-
orly displaced tongue, which is relieved by good airway position-
mation when implementing these criteria. For example, the entire
ing. Advanced airway maneuvers (ie, bag-valve-mask ventilation,
team responds for all pedestrians struck by an automobile. The types
endotracheal tube intubation) are performed during the primary
of subspecialists that make up a trauma team are decided by indi-
survey if the child has apnea, significant respiratory distress, severe
vidual institutions and commonly include pediatric emergency and
head trauma, or an airway that cannot be maintained with basic
critical care specialists, anesthesiologists, trauma surgeons, surgical

Box 75.2. Airway Assessment and Treatment

Table 75.1. Normal Vital Signs for Children by Age
Respiration Blood Pressure Assessment
Age (breaths/min) Pulse (beats/min) (Systolic) ww Airway patency and ability to protect it.
ww Level of consciousness.
Newborn 30–60 100–160 50–70
ww Stridor.
1–6 weeks 30–60 100–160 70–95
Treatment
6 months 25–40 90–120 80–100 ww Spinal immobilization.
1 year 20–40 90–120 80–100 ww Jaw thrust and suctioning.
3 years 20–30 80–120 80–110 ww 100% oxygen by non-rebreathing mask.
6 years 12–25 70–110 80–110 ww Intubate for Glasgow Coma Scale score <9, or absence of an intact
gag reflex.
10 years 12–20 60–90 90–120

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 540 PART 5: ACUTE AND EMERGENT PROBLEMS

Box 75.3. Breathing Assessment and Treatment Box 75.4. Circulation Assessment and Treatment
Assessment Assessment
ww Respiratory rate and depth. ww Identify obvious bleeding sites.
ww Chest wall compliance, symmetry, and movement. ww Peripheral pulses and capillary refill.
ww Tracheal deviation. ww Heart rate.
Treatment ww Level of consciousness.
ww 100% oxygen by non-rebreathing mask. Treatment
ww Intubate for respiratory failure or severe flail chest. ww Compress obvious bleeding sites.
ww Compress obvious bleeding sites. ww 100% oxygen by non-rebreathing mask.
ww Seal open pneumothorax with occlusive dressing. ww 2 large-bore intravenous lines.
ww Needle decompression for tension pneumothorax. ww Fluid resuscitation with 20 mL/kg normal saline.
ww Place chest tube for pneumothorax or hemothorax. ww Administer packed red blood cells at 10 mL/kg if after 60 mL/kg normal
saline and patient still in shock.

techniques. All trauma patients are initially given supplemental A brief neurologic assessment to assess patient disability is also per-
oxygen by non-rebreathing mask at a concentration of 100%. The formed during the primary survey. One rapid assessment technique is
adequacy of ventilation is assessed by a general evaluation of the the AVPU system (alert; responds to verbal stimuli; responds to pain-
respiratory rate, depth, chest movement and symmetry, and tra- ful stimuli; unresponsive). Subsequently, a Pediatric Glasgow Coma
cheal deviation. Scale or Children’s Coma Scale score should be calculated (Table 75.2).
After a patent airway and adequate ventilation have been estab- After life-threatening conditions are stabilized, more information
lished, circulatory status is assessed. All pediatric trauma patients can be collected. The secondary survey involves a thorough head-
require placement of the largest bore intravenous catheter obtainable to-toe examination of the child, fully exposed, to identify additional
for that patient; these should be placed, if possible, in each antecubital injuries, while taking great care to maintain normothermia. It also
fossa. Peripheral vascular access is attempted 3 times or for 90 seconds, includes a SAMPLE (symptoms, allergies, medications, past history/
whichever comes first. If peripheral attempts are unsuccessful, intraos- hospitalizations/surgeries, last meal, events preceding trauma) his-
seous infusion or central venous access should be used. A bolus of tory. A detailed history of events preceding trauma should ensure
20 mL/kg of an isotonic fluid (ie, normal saline or lactated Ringer solution) that injuries are consistent with the causal mechanism. Health pro-
should be given. This may be repeated, if necessary, to manage hypovo- fessionals should be prepared to consider abuse when specific diag-
lemic shock. After 60 mL/kg, administration of 10 mL/kg of packed red noses do not correlate with the history given by the caregiver or the
blood cells should be considered if the patient is still in shock. The like- developmental ability of the child. Measurement of vital signs should
lihood of surgical exploration is high. Acutely exsanguinating wounds occur as previously described and use of other devices, such as Foley
are managed using direct pressure or tourniquet (Box 75.4). catheters and nasogastric tubes, should be considered at this time.
Each time an intervention is performed, repeat reassessments that
incorporate the elements of the primary survey are made.

Laboratory Tests
Most institutions have a standardized trauma panel that is initiated
for all patients, consisting of complete blood cell count with differen-
tial; assessment of electrolyte blood urea nitrogen, creatinine, glucose,
and lactate levels; blood gas analysis with pH and base deficit; blood
tests for amylase and lipase; liver function tests; assessment of pro-
thrombin time and partial thromboplastin time; urinalysis; and blood
typing and cross matching. Additionally, drug and alcohol screening
may provide important information, particularly in the child with
altered mental status. Female pediatric patients of potential child-
bearing age should undergo a point-of-care pregnancy test as well.

Imaging Studies
Sophisticated imaging techniques, such as ultrasonography and
computed tomography, are a usual part of the evaluation of the seri-
Figure 75.3. Correct method for positioning the head with chin lift or jaw thrust. ously injured pediatric trauma patient. The choice of test depends

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 Chapter 75: Approach to the Traumatized Child 541

Table 75.2. Pediatric Glasgow Coma Scale

>1 Year < 1 Year Score
Eye Opening Spontaneously Spontaneously 4
To verbal command To shout 3
To pain To pain 2
No response No response 1
Motor Response Obeys Obeys 6
Localized pain Localized pain 5
Flexion-withdrawal Flexion-withdrawal 4
Flexion-abnormal (decorticate rigidity) Flexion-abnormal (decorticate rigidity) 3
Extension (decerebrate rigidity) Extension (decerebrate rigidity) 2
No response No response 1
>5 Years 2-5 Years 0-23 Months
Verbal Response Orientated Appropriate words/phrases Smiles/coos appropriately 5
Disorientated/confused Inappropriate words Cries and is consolable 4
Inappropriate words Persistent cries and screams Persistent inappropriate crying and/or screaming 3
Incomprehensible sounds Grunts Grunts, agitated, and restless 2
No response No response No response 1
Total Pediatric Glasgow Coma Score (3-15)
Reprinted with permission from Singh AP. Glasgow Coma Scale and Pediatric Glasgow Coma Scale. https://boneandspine.com/pediatric-glasgow-coma-scale.

on the experience of the trauma team and individual characteris- interventions are centered around reducing or preventing burns,
tics of the patient. drowning, falls, gunshot injuries, and poisoning as well as improv-
Point-of-care ultrasonography has become a routine part of the ing playground, road traffic, and sports safety.
secondary survey in adult trauma patients. The extended focused
assessment with sonography in trauma (e-FAST) examination seeks Prognosis
to detect intraperitoneal fluid (ie, hemoperitoneum), pericardial Survival rates are highest for seriously injured children who are
fluid (ie, hemopericardium), intrathoracic fluid (ie, hemothorax), brought to the operating room for treatment within 1 hour of injury.
and pneumothorax. The results are near instantaneous, and the
Definitive care for trauma takes place in the operating room, and ini-
examination may be done without moving the patient from the tial stabilization takes place in the emergency department. Absolute
trauma bay. In critically unstable patients the e-FAST has great util- indications for surgery include hemodynamic instability despite
ity in directing the initial resuscitative efforts. The utility of e-FAST aggressive resuscitation, transfusion of more than 50% of the total
is less clear in the pediatric patient, but its use is supported, espe- blood volume, pneumoperitoneum, intraperitoneal bladder rupture,
cially in the hemodynamically unstable patient. severe renovascular injury, gunshot wounds to the abdomen, evis-
When major trauma is suspected, computed tomography of the ceration, and peritonitis. Other injuries, such as solid organ injuries,
brain, cervical spine, chest, abdomen, and pelvis should be strongly often in contradistinction to similar injuries in adult patients, are fre-
considered after initial stabilization procedures are completed. This quently treated more conservatively in the intensive care unit after
prevents missing injuries in children who may be unconscious or complete consultation with all involved practitioners.
who need lifesaving procedures during resuscitation, which obscure An organized, preestablished, multidisciplinary approach to care
an area of injury from examination. Additional radiographs of the is essential. Studies have shown that the single most important ele-
extremities, for example, may be indicated when other areas of injury ment for any hospital treating injured children is the commitment
are detected on secondary survey. Children are often initially dis- on the part of the institution and its surgeons. Regional pediatric
tracted from 1 injury because of the presence of a more painful injury. trauma centers have increased resources for managing severely
injured patients that include long-term care and rehabilitation. Other
Prevention nondesignated hospitals may do an excellent job in the initial stabi-
Mortality rates have changed little in the past several years even as lization phase of care. Indications for transfer to a specialty center
trauma systems have matured and become widespread. Prevention include inability to provide definitive surgical intervention, inabil-
strategies have become more important in reducing traumatic ity to provide an appropriate intensive care environment, presence
injury, but resources remain limited. Current pediatric prevention of multisystem injuries or injuries requiring extensive orthopedic

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 542 PART 5: ACUTE AND EMERGENT PROBLEMS

or plastic surgery procedures, and major burns. Health profession- Avarello JT, Cantor RM. Pediatric major trauma: an approach to evaluation
als who treat children should not only become adept in the recog- and management. Emerg Med Clin North Am. 2007;25(3):803–836, x PMID:
nition and initial stabilization of injuries but should also serve as 17826219 https://doi.org/10.1016/j.emc.2007.06.013

advocates for injury prevention and coordinated prehospital care Brazelton T, Gosain A. Classification of trauma in children. In Wiley JF,
services in the community. ed. Waltham, MA: UpToDate; 2018. https://www.uptodate.com/contents/
classification-of-trauma-in-children. Accessed September 3, 2019
International Trauma Life Support. International Trauma Life Support website.
https://www.itrauma.org. Accessed June 27, 2019
CASE RESOLUTION
Kenefake ME, Swarm M, Walthall J. Nuances in pediatric trauma. Emerg Med
The 6-year-old boy sustained multiple trauma from an automobile versus pedes-
Clin North Am. 2013;31(3):627–652 PMID: 23915597 https://doi.org/10.1016/j.
trian collision. He presents with altered level of consciousness; respiratory failure
emc.2013.04.004
(ie, shallow respirations); possible internal organ injury, which has the poten-
tial to result in shock; and probable fracture of the left femur, which may also Lee LK, Fleisher GR. Approach to the initially stable child with blunt or pene-
contribute to the development of shock secondary to hemorrhage. These inju- trating injury. In Bachur RG, ed. Waltham, MA: UpToDate; 2017. https://www.
ries are identified based on a primary and secondary survey. Proper management uptodate.com/contents/approach-to-the-initially-stable-child-with-blunt-or-
includes stabilization of the cervical spine, airway management, aggressive early penetrating-injury. Accessed September 3, 2019
shock treatment with fluid replacement, and a vigilant search for additional Lee LK, Fleisher GR. Trauma management: approach to the unstable child. In
injuries. Continued reassessment is also an integral part of emergency depart- Bachur RG, ed. Waltham, MA: UpToDate; 2018. https://www.uptodate.com/
ment stabilization. Because of the presence of multisystem injuries, after initial contents/trauma-management-approach-to-the-unstable-child. Accessed
stabilization the patient is transferred to a regional pediatric trauma center for
September 3, 2019
extended care.
Leeson K, Leeson B. Pediatric ultrasound: applications in the emergency depart-
ment. Emerg Med Clin North Am. 2013;31(3):809–829 PMID: 23915605 https://
doi.org/10.1016/j.emc.2013.05.005
Selected References Overly FL, Wills H, Valente JH. ‘Not just little adults’—a pediatric trauma primer.
R I Med J (2013). 2014;97(1):27–30 PMID: 24400309
American Academy of Pediatrics. APLS: The Pediatric Emergency Medicine
Resource. AAP.org website. https://www.aap.org/en-us/continuing-medical- Scaife ER, Rollins MD, Barnhart DC, et al. The role of focused abdomi-
education/life-support/APLS-The-Pediatric-Emergency-Medicine-Resource/Pages/ nal sonography for trauma (FAST) in pediatric trauma evaluation. J Pediatr
APLS-The-Pediatric-Emergency-Medicine-Resource.aspx. Accessed June 27, 2019 Surg. 2013;48(6):1377–1383 PMID: 23845633 https://doi.org/10.1016/j.
jpedsurg.2013.03.038
American College of Surgeons. Advanced Trauma Life Support. FACS.org web-
site. https://www.facs.org/quality-programs/trauma/atls. Accessed June 27, 2019 Tiyyagura G, Beucher M, Bechtel K. Nonaccidental injury in pediatric patients:
detection, evaluation, and treatment. Pediatr Emerg Med Pract. 2017;14(7):
American Heart Association. Pediatric Advanced Life Support (PALS). CPR.
1–32 PMID: 28665574
heart.org website. https://cpr.heart.org/AHAECC/CPRAndECC/Training/
HealthcareProfessional/Pediatric/UCM_476258_PALS.jsp. Accessed June 27, 2019

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 CHAPTER 76

Abdominal Trauma
David B. Burbulys, MD

CASE STUDY
An 8-year-old boy who was riding downhill on a bicycle Questions
crashed into a tree and was transported to the local 1. What are the most common mechanisms of
trauma center by emergency medical services. On arrival intra-abdominal injury in children?
he was brought to the pediatric emergency department, 2. What are the diagnostic studies used to evaluate
where the paramedics report that the bike handlebars abdominal trauma?
struck the child’s abdomen. The boy reports dizziness 3. What is a simple rule for establishing the lower limit
and vomits several times. Initial vital signs show a of normal blood pressure in children when assessing
heart rate of 135 beats per minute, blood pressure of a child for shock?
105/60 mm Hg, oxygen saturation of 98% on room air, 4. What are the basic components of the treatment of
and a respiratory rate of 24 breaths per minute. The shock that occur after abdominal trauma?
abdomen is flat but tender to palpation in the mid-
epigastric region and left upper quadrant.

Abdominal trauma is the leading preventable cause of fatal injury Clinical Presentation
in trauma patients. Death results when the extent and nature of
Pain, tenderness, ecchymoses, and peritoneal signs (ie, voluntary or
abdominal injuries are neither appreciated nor appropriately man-
involuntary guarding, rebound tenderness) are among the more reli-
aged, fluid replacement is inadequate, and airway maintenance and
able signs of pathology, whereas abdominal distention and absence
surgical intervention are not implemented soon enough. Primary
of bowel sounds are less consistent markers of injury. (See Box 76.1
abdominal trauma is the third leading cause of traumatic death,
for signs and symptoms suggestive of abdominal trauma.) It is par-
after head and thoracic injury. Clinicians should be knowledgeable
ticularly important to note that no sign is completely reliable and
about mechanisms of injury that result in abdominal trauma, early
that acute hemorrhage into the abdomen does not result in peri-
manifestations of shock, and methods of aggressive treatment of
toneal irritation initially. A high index of suspicion must be main-
hemorrhagic shock.
tained in situations in which it is warranted based on the severity of
the mechanism of injury, despite minimal initial physical findings.
Epidemiology Unexplained hypotension or shock mandates further investigation
Twenty-five percent of children who sustain multisystem trauma with ultrasonography or computed tomography (CT) to assess for
have significant abdominal injury, and 9% die from abdominal- intra-abdominal hemorrhage.
associated trauma. The risk of death is higher with simultaneous
head and abdominal injury than with the occurrence of either
injury alone. Blunt-force mechanisms are responsible for nearly Box 76.1. Signs and Symptoms Suggestive
85% of abdominal injuries, with the remainder resulting from pen- of Abdominal Trauma
etrating injuries. Examples of blunt-force mechanisms, presented ww Pain
in order of frequency from most to least frequent, include motor ww Tenderness
vehicle crashes, which also are the most lethal; pedestrian versus ww Distention
automobile collisions; falls; bicycle injuries; sports injuries; and ww Peritoneal signs (eg, absent or diminished bowel sounds, rebound
direct blows from abuse and assault. Injuries to the spleen and tenderness, guarding)
liver predominate, followed by injuries to the kidney, bowel, and ww Ecchymoses
pancreas. In patients with multiple injuries, the incidence of ww Tire tracks
trauma involving pelvic bones and organs (eg, bladder, ureter, ww Seat belt marks
iliac vessels) is also high. A straddle injury (eg, a fall that occurs ww Urine, stool, or nasogastric aspirate positive for blood
when climbing over a fence) can also result in abdominal and ww Unexplained hypotension or other signs of hypovolemic shock
pelvic trauma.

543

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 544 PART 5: ACUTE AND EMERGENT PROBLEMS

Pathophysiology Evaluation
Blunt trauma largely involves injury to solid, not hollow, intra- Determining which organ or organs may be injured as the result of
abdominal organs (ie, spleen and liver rather than small bowel) for a abdominal trauma is difficult. Up to 50% of significant injuries are
variety of reasons. First, the rib cage is flexible in children. As a result, missed on initial physical examination. Children are often uncooper-
rib fractures are less likely to occur, thereby reducing the potential ative or unable to assist with the evaluation. Physicians tend to focus
for penetration of hollow abdominal organs by broken ribs. Second, on injuries to the extremities, pelvis, face, or chest that are painful
children have less well-developed abdominal musculature and less and distracting to children and more clinically obvious to the exam-
adipose tissue than adults and larger organs relative to overall body iner. Initial clinical impressions may be incorrect, causing delayed
size. Thus, in children blunt force is more easily and more diffusely diagnosis or unnecessary surgical exploration.
transmitted to the solid organs. Third, because the diaphragm is
oriented more horizontally in children than in adults, the liver and History
spleen lie more anteriorly and caudally within the abdomen. The history should focus on the mechanism of injury and the phys-
It is important to emphasize that abdominal injury may result iologic response of the child, especially in the pre-hospital setting
in excessive blood loss. The pathophysiology of hemorrhagic shock (eg, initial hypotension, tachycardia, cyanosis; Box 76.2). A poor
is discussed in detail in Chapters 74 and 75. The liver and spleen history concerning the circumstances of the injury may contribute
are highly vascularized organs that bleed profusely when lacer- to a delayed diagnosis.
ated. Even the accumulation of a subcapsular hematoma with-
out rupture may cause a profound drop in hematocrit. Because Physical Examination
intra-abdominal organs are not directly visible when a patient As stated previously, an abnormal physical examination may not
is examined, signs and symptoms of injury are not always obvi- always be indicative of pathology. Clinicians should avoid relying
ous. Therefore, hemorrhagic shock should always be suspected on physical examination alone as a predictor of abdominal injury.
in patients with abdominal trauma. Likewise, large volumes of Studies have demonstrated that patients with and without proven
blood can accumulate in the pelvis and retroperitoneum, and injuries often showed no significant differences with respect to phys-
because of their proximity to the abdomen, they should always ical findings. In particular, children with abusive abdominal trauma
be considered as a reservoir for hemorrhage in abdominal as well often have no cutaneous evidence of bruising, especially immedi-
as pelvic trauma. ately after the injury is inflicted (see Chapter 142). Thus, definitive
evaluation of the abdomen is mandated for patients with signifi-
Differential Diagnosis cant mechanism of injury. Such evaluation often includes point of
Physicians should be familiar with the most common patterns of care ultrasonography, rapid CT, formal ultrasonography, diagnostic
abdominal injury and should consider the possibility of specific peritoneal lavage, laparoscopy, or laparotomy.
injuries. Any solid abdominal organ can be injured by any mecha- Vital signs should be monitored and trends followed. In
nism, whether blunt or penetrating. The spleen is the most common children, the range for normal heart rate, respiratory rate, and
intra-abdominal organ injured by a blunt force. Hepatic injuries are blood pressure is age dependent. A simple rule for calculating the
the most common fatal abdominal injuries, although they are less lower limit of normal systolic blood pressure is 70 + (2 3 age in
frequent than splenic injuries. The right lobe of the liver is injured years). Physicians should always remember that a drop in blood
more frequently than the left lobe. pressure is a very late sign in the development of shock in children
Injuries to hollow viscera, such as the stomach and intestines, (see Chapters 74 and 75).
which represent only approximately 5% to 15% of injuries from Serial abdominal examinations increase the likelihood of detect-
blunt forces, are difficult to diagnose and often present late only ing a previously missed condition. Inspection of the abdomen to
after peritonitis manifests. Three mechanisms result in injury of evaluate for ecchymoses, distention, tire tracks, penetrations, or par-
hollow structures: “crush” between the anterior wall of the abdo- adoxical motion should occur first. Auscultation for bowel sounds
men and the vertebral column; deceleration, which causes shear- follows this inspection, and palpation should be done last. Palpation
ing of the bowel from its mesenteric attachments; and “burst,” should be done in all 4 quadrants to elicit tenderness, rebound, and
which occurs when an air- or fluid-filled loop of bowel is closed at
both ends at the time of impact. Peritonitis may manifest within
6 to 48 hours secondary to fecal spillage or devascularization as Box 76.2. What to Ask
the result of any of these mechanisms. Occasionally, a diagnosis of Abdominal Trauma
hollow viscera injury is made incidentally or may be delayed more ww How was the child injured?
than 48 hours, which reinforces the necessity of observation and ww How long ago did the injury occur?
serial examinations. Duodenal and pancreatic injuries are examples ww What parts of the body were injured?
of potentially delayed diagnoses that can have grave consequences. ww Did the child receive any treatment before coming to the hospital, and
Leakage of bile and enzymes may activate autolysis of the pancreas what was the response?
and result in sepsis syndrome.

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 Chapter 76: Abdominal Trauma 545

guarding. If a hepatic or splenic injury is initially suspected, palpa- Management

tion should be minimized to avoid further hemorrhaging.
Management of abdominal trauma in children occurs simultane-
Laboratory Tests ously with evaluation. The stabilization of children with abdomi-
Laboratory evaluation should be guided by the history and physi- nal trauma, especially in the context of multiple trauma, requires
cal examination. A urinalysis is helpful to evaluate for hematuria a multidisciplinary team approach that includes surgeons, pedia-
and associated genitourinary injuries. Elevated serum transami- tricians, and emergency physicians. A discussion on approach to
nases, amylase, lipase, and alkaline phosphatase may be indicative trauma management can be found in Chapter 75.
of injury; however, normal values do not exclude pathology. A com- Hypovolemic shock, if present, is the primary complication of
prehensive trauma panel, which usually includes a complete blood abdominal trauma on which to focus, because the leading unrec-
cell count and differential, serum electrolytes, blood urea nitrogen, ognized cause of death in affected children is profound blood loss.
creatinine, glucose, prothrombin time, partial thromboplastin time, Airway problems and breathing difficulties should be addressed
blood gas analysis with pH and base deficit, lactic acid, and blood initially, followed by vascular access and fluid replacement. No
type and crossmatch as well, should be performed for all patients more than 3 attempts at peripheral vascular access should be made
with serious or multiple injuries (see Chapter 75). Additionally, drug within 90 seconds before proceeding to more invasive procedures.
and alcohol screens may provide important information, particularly The intraosseous route should be used for the next vascular access
in children with altered mental status. Female patients of childbear- attempt. The flat, medial portion of the proximal tibia is most com-
ing age also should undergo a point-of-care pregnancy test. monly used for the procedure, which is performed with an intraos-
seous, bone marrow, or spinal needle (Figure 76.1). Fluids and
Imaging Studies medications delivered into the marrow cavity flow into the venous
Multiple imaging modalities are available to assess the pediatric circulation. Intraosseous cannulation is rapid and simple and may be
trauma patient with suspected abdominal injuries. The time to per- lifesaving. It is limited by low flow rates (approximately 30 mL/min),
form an imaging study is after the patient is responding appropriately which can be augmented by using pressure on the intravenous bag or
to resuscitation (ie, fluid therapy). Unstable patients require surgical by pushing fluid by hand through a syringe. Complications associated
exploration for definitive treatment of abdominal or pelvic injury. with intraosseous line placement are rare. All physicians who care
Computed tomography remains the standard of care for imag- for pediatric trauma patients should be familiar with this technique.
ing the injured abdomen of a pediatric trauma patient. Computed A variety of central venous access sites, such as femoral, sub-
tomography has greater than 97% accuracy in identifying abdominal clavian, and internal jugular, may be used in older children. The
or retroperitoneal injury, is noninvasive, and, most notably, provides Seldinger technique, with insertion of a large-bore catheter over a
detailed specific information about injuries. The images are also rou- guidewire, is often used. Intravenous access above the diaphragm
tinely extended to include the pelvis, as necessary. Disadvantages is preferred in patients with blunt or penetrating abdominal trauma
of CT may include the distance from the trauma suite, which may who have the potential for disruption of the vena cava or other
not be ideal for unstable patients; the length of time required to per- large veins.
form the procedure; the need for an intravenous contrast, which has
inherent risks, such as allergic reactions and renal toxicity; and radi-
ation exposure (see Chapter 17).
Tibial tuberosity
The use of point-of-care and formal ultrasonography has become
quite popular, with encouraging results for the identification of
abdominal injury, although its use in pediatric trauma patients
remains somewhat controversial. Ultrasonography can be used to
rapidly and noninvasively document the presence of intraperitoneal
and pelvic fluid (ie, blood). Although ultrasonography is nearly as
effective as CT for documenting the presence of injury, it does not Anterior
provide as much specific information about the nature of the injury border
and does not image the retroperitoneal space. Ultrasonography has
a few additional advantages. It is relatively inexpensive; does not
require contrast or radiation exposure; can be performed in minutes
in the trauma room, thus minimizing the risk to an unstable patient;
and can be used many times for serial assessments.
Although for several decades diagnostic peritoneal lavage was a 90° to
medial
dependable method of detecting intra-abdominal hemorrhage, its surface
use has been largely replaced by ultrasonography, and it has limited
use in the pediatric population because currently, many children
with blunt trauma to the abdomen are often treated nonsurgically. Figure 76.1. Intraosseous cannulation technique.

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 546 PART 5: ACUTE AND EMERGENT PROBLEMS

Fluid replacement begins with a 20 to 40 mL/kg bolus of crystal- number of injuries to the spleen and liver are managed with obser-
loid solution (warmed normal saline or lactated Ringer solution). vation in the pediatric intensive care unit. Surgical exploration and
If no improvement in circulation occurs, additional 10 to 20 mL/ repair are performed only if patients become hemodynamically
kg boluses may be given, and type-specific packed red blood cells unstable. Reduction in anesthesia-related mortality, postsplenec-
should be considered. In most scenarios, type-specific blood is given tomy sepsis, and other postoperative complications have resulted
after 60 mL/kg of crystalloid has failed to improve circulatory param- from this shift in practice style. Without surgical intervention, how-
eters. Frequent hematocrits or hemoglobins should be determined ever, a severe injury such as complete splenic rupture has a 90% to
to monitor ongoing blood loss. Vital signs should be repeated fre- 100% mortality rate.
quently. Serial examinations, which detect the signs and symptoms
of shock (Box 76.3), are the most important gauge of hemodynamic
recovery and stability. All children require close hospital observa- CASE RESOLUTION
tion, preferably in a pediatric trauma center and pediatric inten- The boy sustained isolated abdominal trauma. Initial presenting signs and
sive care unit. symptoms are concerning for internal organ injury, specifically splenic hema-
Orthostatic fall in blood pressure and supine hypotension (late toma, pancreatic injury, internal hemorrhage, and compensated shock (eg,
sign) must be aggressively managed. If hemodynamic stabilization tachycardia, tachypnea). The child is managed with standard initial resuscita-
is not achieved after appropriate vascular access and fluid resusci- tion, including fluid repletion. Because serial hemodynamic measurement and
hematocrits are stable, he undergoes an abdominal CT scan, which demonstrates
tation, the trauma surgeon will most likely perform an exploratory
a splenic hematoma. A pediatric surgeon is consulted and recommends obser-
laparotomy. If the child has been stabilized with initial airway vation with continued monitoring in the pediatric intensive care unit.
and circulatory support, diagnostic procedures (eg, CT) can be
performed as part of the emergency department evaluation. Once
identified, specific organ injury can be managed.
Other specific management concerns for pediatric patients with Selected References
abdominal injury are early decompression of the stomach with a American Academy of Pediatrics; American College of Emergency Physicians.
nasogastric or orogastric tube to prevent respiratory compromise APLS: The pediatric emergency medicine resource. https://www.aap.org/en-us/
and urinary catheter insertion to decompress the bladder. Before continuing-medical-education/life-support/APLS-The-Pediatric-Emergency-
inserting a urinary catheter, the trauma team should evaluate for Medicine-Resource/Pages/APLS-The-Pediatric-Emergency-Medicine-Resource.
possible urethral trauma and check for the presence of blood in the aspx. Accessed May 7, 2019
urine, which may indicate other genitourinary trauma. American College of Emergency Physicians. International Trauma Life Support
Nonsurgical management of minor to moderate liver or spleen website. https://www.itrauma.org. Accessed May 7, 2019
injuries is common in children. A watchful waiting approach is often American College of Surgeons. Advanced trauma life support. https://www.facs.
used after hemodynamic stability has been achieved. More severe org/quality-programs/trauma/atls. Accessed May 7, 2019
injuries, including bowel rupture, require surgical intervention. American Heart Association. Pediatric advanced life support (PALS). https://cpr.
For injuries not requiring truly emergent surgical intervention, heart.org/AHAECC/CPRAndECC/Training/HealthcareProfessional/Pediatric/
interventional radiology-guided arterial embolization has become UCM_476258_PALS.jsp. Accessed May 7, 2019
a useful tool for significant liver, spleen, and kidney injuries, as well Boleken ME, Cevik M, Yagiz B, Ter M, Dorterler ME, Aksoy TR. The charac-
as pelvic fractures. teristics and outcomes of penetrating thoracic and abdominal trauma among
children. Pediatr Surg Int. 2013;29(8):795–800 PMID: 23811959 https://doi.org/
10.1007/s00383-013-3339-z
Prognosis
Gaines BA. Intra-abdominal solid organ injury in children: diagnosis and treat-
Morbidity and mortality related to abdominal trauma depend on the ment. J Trauma. 2009;67(2 suppl):S135–S139 PMID: 19667846 https://doi.
specific organ injury and style of management. Up to 40% of patients org/10.1097/TA.0b013e3181adc17a
with major liver injuries die. However, several less severe liver inju- Goodwin SJ, Flanagan SG, McDonald K. Imaging of chest and abdominal trauma
ries can be managed without surgery. Currently, an increasing in children. Curr Pediatr Rev. 2015;11(4):251–261 PMID: 26219741 https://doi.
org/10.2174/1573396311666150729121123
Guzzo H, Middlesworth W. Hollow viscus blunt abdominal trauma in chil-
Box 76.3. Signs and Symptoms of dren. In: Torrey SB, ed. Waltham, MA: UpToDate; 2017. https://www.uptodate.
Hemorrhagic Shock in Children com/contents/hollow-viscus-blunt-abdominal-trauma-in-children. Accessed
September 1, 2018
ww Anxiety, irritability, decreased responsiveness Hom J. The risk of intra-abdominal injuries in pediatric patients with sta-
ww Cool and mottled skin, pallor ble blunt abdominal trauma and negative abdominal computed tomogra-
ww Delayed capillary refill (>2 seconds) phy. Acad Emerg Med. 2010;17(5):469–475 PMID: 20536798 https://doi.
ww Respiratory distress org/10.1111/j.1553-2712.2010.00737.x
ww Tachycardia Leeson K, Leeson B. Pediatric ultrasound: applications in the emergency depart-
ww Thirst ment. Emerg Med Clin North Am. 2013;31(3):809–829 PMID: 23915605 https://
doi.org/10.1016/j.emc.2013.05.005

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Notrica DM. Pediatric blunt abdominal trauma: current management. Curr Opin Schonfeld D, Lee LK. Blunt abdominal trauma in children. Curr Opin
Crit Care. 2015;21(6):531–537 PMID: 26418761 https://doi.org/10.1097/ Pediatr. 2012;24(3):314–318 PMID: 22450250 https://doi.org/10.1097/
MCC.0000000000000249 MOP.0b013e328352de97
Saladino RA, Conti K. Pediatric blunt abdominal trauma: initial evaluation and Sivit CJ. Abdominal trauma imaging: imaging choices and appropriateness.
stabilization. In: Bachur RG, Woodward GA, eds. Waltham, MA: UpToDate; 2018. Pediatr Radiol. 2009;39(suppl 2):S158–S160 PMID: 19308377 https://doi.
https://www.uptodate.com/contents/pediatric-blunt-abdominal-trauma-initial- org/10.1007/s00247-008-1127-z
evaluation-and-stabilization. Accessed September 1, 2018 Wesson DE. Liver, spleen, and pancreas injury in children with blunt abdom-
Scaife ER, Rollins MD, Barnhart DC, et al. The role of focused abdomi- inal trauma. In: Torrey SB, ed. Waltham, MA: UpToDate; 2017. https://www.
nal sonography for trauma (FAST) in pediatric trauma evaluation. J Pediatr uptodate.com/contents/liver-spleen-and-pancreas-injury-in-children-with-
Surg. 2013;48(6):1377–1383 PMID: 23845633 https://doi.org/10.1016/j. blunt-abdominal-trauma. Accessed September 1, 2018
jpedsurg.2013.03.038

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BPPCA6e_Ch076_0543-0548.indd 548 2/13/20 6:10 PM
 CHAPTER 77

Acute Abdomen (Appendicitis)

Roxanne L. Massoumi, MD, and Steven L. Lee, MD, MBA, FACS, FAAP

CASE STUDY
A 10-year-old girl presents with abdominal pain of Questions
24 hours’ duration. The pain began in the periumbilical 1. What is the differential diagnosis for patients with
area and now is located in the right lower quadrant. She acute abdominal pain?
had 1 bout of emesis but no diarrhea. She has no fever or 2. What is the appropriate workup for children with
chills. She also has some pain with voiding. On physical suspected appendicitis?
examination, she has a low-grade fever and tachycardia. 3. What is the current management for children with
She is lying still in bed. Her abdomen is nondistended, appendicitis?
but she has tenderness to palpation in the right lower 4. What is the expected postoperative course and
quadrant. She also has rebound tenderness and guard- possible complications following appendectomy?
ing in this area.

Appendicitis is among the most common surgical emergencies in to the periumbilical region, and distention of hindgut structures
children. Pediatricians play a key role in the diagnosis and man- localizes to the suprapubic region. Midgut structures are supplied by
agement of patients with abdominal pain and must be able to the superior mesenteric artery (ie, duodenum to transverse colon)
distinguish appendicitis from other causes of abdominal pain. When and hindgut structures by the inferior mesenteric artery (ie, trans-
a patient presents with the classic signs and symptoms of appendi- verse colon to rectum). Unlike visceral pain, somatic pain is well
citis, the diagnosis is simple. Unfortunately, approximately 50% of localized. Irritation of the parietal peritoneum results in sharp pain
patients present with atypical signs and symptoms of appendicitis, and localized tenderness on examination. Anything that causes
making the diagnosis difficult. Until recently, nearly every aspect the irritated peritoneum to move or stretch worsens the pain and
of the diagnosis and management of children with appendicitis has tenderness.
been controversial. Thus, it is important for primary care physicians, The classic example of these 2 types of pain occurs with appen-
emergency department physicians, and surgeons to communicate dicitis. The basic pathophysiology of appendicitis is obstruction of
effectively to provide the highest level of care and cost efficiency. the lumen of the appendix followed by infection. Obstruction may
be caused by fecal material (ie, appendicolith, fecalith), lymphoid
Epidemiology hyperplasia, foreign body, tumor, or parasites. Following obstruc-
tion, the appendix becomes distended from accumulation of mucus
More than 70,000 children are affected by appendicitis each year
and proliferation of bacteria. This distention results in a vague
in the United States. The lifetime risk of appendicitis is 9% in boys
periumbilical pain. As intraluminal pressure increases, lymphatic
and 7% in girls.
and venous drainage are impaired, resulting in edema of the appen-
dicular wall. As the overlying parietal peritoneum becomes progres-
Pathophysiology
sively more irritated, the pain localizes to the right lower quadrant
The 2 main causes of abdominal pain are distention of the (RLQ). This stage is known as acute appendicitis. Further increase
viscera, causing visceral pain, and irritation of the peritoneum, caus- in pressure limits arterial inflow and ultimately results in tissue
ing somatic pain. Distention of any hollow organ in the abdomen necrosis and perforation. Although the natural history of untreated
causes crampy and intermittent abdominal pain. Examples include appendicitis is usually perforation and abscess, not all patients
distention of the biliary tree, small or large intestine, urinary struc- progress to perforation.
tures (ie, bladder, ureters), or gynecologic structures (ie, uterus,
fallopian tubes). This visceral pain is poorly localized and tends
to be reported in the midline. Distention of any foregut structure Clinical Presentation
localizes to the epigastric region. Foregut structures derive their Abdominal pain is the most common symptom of and is present in
blood supply from the celiac trunk and include the stomach, duo- nearly every patient with appendicitis. The classic presentation of
denum, and biliary tree. Distention of midgut structures localizes a child with appendicitis includes a history of initial periumbilical

549

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 550 PART 5: ACUTE AND EMERGENT PROBLEMS

pain migrating to the RLQ. The pain is gradual in onset and pro-
Box 77.1. What to Ask
gressively worsens. Anorexia, nausea, and vomiting typically are
associated with appendicitis. In most cases, these associated symp- Abdominal Pain
toms manifest after the onset of abdominal pain. Intermittent, ww When did the pain start?
crampy pain that manifests after the onset of vomiting or diarrhea is ww Can you describe the nature of your pain?
less commonly associated with appendicitis. The inflamed appendix ww Is your pain constant or intermittent?
irritates the overlying peritoneum by direct contact, which results in ww Where is your pain?
focal peritonitis and localized RLQ pain. The symptoms vary based ww What makes your pain worse? Better?
on the location of the appendix, however. When the appendix is ww Do you have any fever or chills?
retrocecal, a dull ache is often described. When the tip of the ww Do you have any nausea, vomiting, or diarrhea?
appendix is located in the pelvis, atypical pain is described. A ww When was your last bowel movement?
child may report dysuria and urinary frequency resulting from the ww Do you have any pain with urinating?
inflamed appendix irritating the bladder. Diarrhea or tenesmus may ww Are you hungry?
occur if the appendix is adjacent to the rectum. Fever, tachycardia, ww When was the last time you ate? Drank?
and leukocytosis occur as a consequence of systemic inflammatory ww Have you had any ill contacts?
mediators released by ischemic tissues, white blood cells, and bacte- ww Have you had any upper respiratory symptoms?
ria. Higher fevers are associated with perforated appendicitis.

Differential Diagnosis movement worsens the pain. The most common finding is
Acute appendicitis can mimic nearly any intra-abdominal pro- focal tenderness in the RLQ. Applying pressure to a stethoscope
cess and should be high on the differential in all children who while listening to the abdomen is a subtle means of palpating
report abdominal pain. Other causes of RLQ pain that are often the abdomen in the frightened child in whom it is difficult to
indistinguishable from acute appendicitis include mesenteric obtain an accurate examination. Because of the level of discom-
adenitis, viral gastroenteritis, regional bacterial enteritis, tubo- fort, it may be difficult to elicit rebound tenderness and pal-
ovarian pathologic processes, inflammatory bowel disease, Meckel pate for a mass. Asking the child to walk or jump is an easier
diverticulum, cecal diverticulitis, and constipation. Other causes and more accurate method of determining the degree of perito-
of lower abdominal pain include urinary tract infection, kidney neal irritation. Narcotic analgesics improve patient comfort but
stone, uterine pathologic process, bowel obstruction, and malig- do not alter the inflammatory process; thus, tenderness persists.
nancy (eg, lymphoma). Vague abdominal pain can be caused by Localized tenderness is dependent on peritoneal irritation; thus,
right lower lobe pneumonia, sigmoid diverticulitis, pancreatitis, obesity, a retrocecal appendix, or walling off of the appendix by
hepatitis, and cholecystitis. the omentum, mesentery, or small bowel may make the diagno-
sis of appendicitis more challenging.
Evaluation Laboratory Tests
History Laboratory studies often show a mild leukocytosis. A markedly
A careful history is required to distinguish acute appendicitis from elevated leukocyte count is suggestive of perforation or another diag-
other causes of abdominal pain (Box 77.1). In most patients with nosis. A “shift to the left” in the complete blood count and differ-
acute appendicitis, pain is often the first symptom. Associated symp- ential may be a better diagnostic indicator for appendicitis. Other
toms, such as nausea, vomiting, and diarrhea, present after the onset inflammatory markers, including C-reactive protein, procalcito-
of pain. It is important to distinguish intermittent crampy pain nin, and lactic acid, have also been investigated but have not been
from constant and progressively worsening pain. If the patient has routinely used in the workup of patients with suspected appen-
nausea, vomiting, or diarrhea followed by intermittent crampy pain, dicitis. A urinalysis should also be obtained and usually is free of
the diagnosis of gastroenteritis is more likely than appendicitis. A bacteria; however, a few or moderate number of red or white
patient may develop a low-grade fever within 24 hours of the pain. blood cells may be found because the inflammatory process of the
Higher fevers manifest later and occur more frequently with perfo- appendix may cause localized irritation of the bladder or ureter.
rated appendicitis. For the patient in whom fever is the first sign or
symptom, appendicitis is less likely. Imaging Studies
Appropriate use of diagnostic imaging can minimize negative appen-
Physical Examination
dectomy and perforation rates.
A thorough physical examination is necessary to rule out
other causes of abdominal pain. Upper respiratory infections Plain Radiography
may result in mesenteric adenitis, causing abdominal pain. In general, plain radiographs of the abdomen and chest may be more
The patient with acute appendicitis usually lies still, because useful to evaluate for other disease processes when the suspicion

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 Chapter 77: Acute Abdomen (Appendicitis) 551

for appendicitis is low. Specific to appendicitis, plain radiography CT include an enlarged appendix (>6 mm), appendicular wall thick-
can show fecaliths in 10% to 20% of patients. Other helpful find- ening (>1 mm), periappendicular fat stranding, and appendicu-
ings include lumbar scoliosis and obliteration of the psoas shadow. lar wall enhancement (Figure 77.2). Computed tomography has a
sensitivity and specificity of approximately 95%. Several concerns
Ultrasonography exist with CT, however. Significant delay in obtaining the study may
Ultrasonography is an efficient bedside study that is noninvasive, occur if oral contrast is administered; the younger child may require
requires no contrast, and emits no radiation. It should be the first sedation to complete the study; and growing concern exists for the
study used in the workup of the patient with suspected appendici- increased radiation exposure from CT (see Chapter 17). Developing
tis. Common ultrasonography findings consistent with appendicitis tissues have increased sensitivity to the effects of radiation, as evi-
include a fluid-filled, noncompressible tubular structure; a diameter denced by an increased risk of radiation-induced malignancy in
greater than 6 mm; appendicolith; and periappendicular or perice- patients exposed at a younger age.
cal fluid (Figure 77.1). With an experienced technician and in the
Magnetic Resonance Imaging
ideal situation, ultrasonography has sensitivity greater than 85% and
specificity greater than 90%. Results are also influenced by patient Magnetic resonance (MR) imaging has a high diagnostic accuracy
factors, such as bowel gas pattern, obesity, and guarding or move- similar to CT and can be used to diagnose acute appendicitis in lieu
ment. When a normal appendix is identified, ultrasonography is a of CT. Findings on MR imaging that are consistent with acute appen-
reliable study to rule out appendicitis. Only 10% to 50% of children dicitis include an enlarged (>6 mm), curved, and thickened blind-
with a normal appendix can be identified, however. Furthermore, ended and fluid-filled tubular structure that is markedly enhanced
when the appendix is not identified a risk for appendicitis remains on contrast-enhanced T1-weighted imaging (Figure 77.3). Magnetic
despite otherwise normal ultrasonography findings. resonance imaging has several advantages compared with CT, such
as lower radiation exposure, which is particularly beneficial in the
Computed Tomography pediatric population. Additionally, MR imaging affords better visu-
When appendicitis cannot be excluded or confirmed on ultraso- alization of an acutely inflamed and/or abnormally located appen-
nography, additional imaging with computed tomography (CT) or dix than ultrasonography. However, MR imaging is much costlier
observation is warranted. Findings consistent with appendicitis on than ultrasonography and CT, patients must remain still for longer
periods of time, it is not well suited for patients with claustropho-
bia, and it is not readily available at many institutions.

Observation
When appendicitis cannot be excluded or confirmed based on the
history, physical examination, laboratory studies, and ultrasonog-
raphy, additional imaging (ie, CT or MR imaging) or observation
is indicated. Given the radiation risks associated with CT and the
limited availability of MR imaging, admission to the hospital for
intravenous (IV) fluids and serial examinations is a safe alterna-
tive. Food and liquids should be withheld, and a repeat complete
A blood cell count with manual differential should be obtained the
next morning. In most instances, patients who do not have appen-
dicitis improve and can safely be allowed to eat and discharged
home. In children with appendicitis the pain will increase, and
IV antibiotics should be administered while arrangements for
appendectomy are made.

Management
The management of appendicitis begins with IV fluids and broad-
spectrum IV antibiotics. Single- or double-agent therapy has been
shown to be as effective as and more cost-efficient than triple-agent
antibiotics. Management after initiating antimicrobial therapy is
B based on whether the patient is likely to have nonperforated or
perforated appendicitis. This distinction is not always clear, even
Figure 77.1. Sonograms consistent with acute appendicitis. A, Transverse if preoperative imaging studies have been obtained. For the patient
view. B, Longitudinal view. In both images, the arrows are used to measure presenting with symptoms that have been present for less than
the diameter of the appendix. 24 hours, the risk of perforated appendicitis is low. These patients

BPPCA6e_Ch077_0549-0554.indd 551 2/13/20 6:09 PM

 552 PART 5: ACUTE AND EMERGENT PROBLEMS

A B

C D

Figure 77.2. Computed tomography images consistent with appendicitis. A, an axial view showing an appendicolith
(arrow). B, a coronal view showing an appendicolith (arrow), periappendiceal inflammatory changes (dashed arrow),
and an inflamed appendix (arrowhead). C, a coronal view showing an inflamed appendix (arrow) and an inflamed
lymph node (arrowhead). D, a coronal view showing an inflamed appendix (arrow).

should be adequately resuscitated with IV fluids and antibiotics and

scheduled for appendectomy at a later time. Antibiotic therapy typi-
cally halts or even reverses disease progression. Thus, the likelihood
of nonperforated appendicitis progressing to perforated appendici-
tis is highly unlikely after appropriate antibiotics have been started.
For this reason, appendectomy may be considered a semielective
procedure rather than an urgent or emergent procedure as it has
historically been considered. Duration of symptoms longer than
24 hours is associated with increased risk for perforation. In most
instances, the patient with symptoms lasting fewer than 5 to 7 days
is treated as described previously.
The patient with symptoms lasting more than 7 days likely has
perforated appendicitis with or without an abscess. In the patient
with prolonged duration of symptoms, appendectomy can be more
Figure 77.3. Axial magnetic resonance image used to diagnose acute technically challenging and associated with an increased rate of post-
appendicitis (arrow). operative complications.

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 Chapter 77: Acute Abdomen (Appendicitis) 553

Nonsurgical management has been done in an attempt to reduce infection occurs in less than 1% of patients with nonperforated
these risks. Nonsurgical management includes administration of appendicitis and up to 16% of patients with perforated appendici-
IV antibiotics and drainage of intra-abdominal abscess (if present) tis. Rates of postoperative abscess are less than 1% for nonperforated
using interventional radiologic techniques. Initial nonsurgical man- appendicitis and less than 15% for perforated appendicitis. Mortality
agement is successful in approximately 85% of patients. Historically, related to appendicitis is rare.
after a patient was clinically stable the individual was discharged
and elective interval appendectomy was typically performed 6 to
8 weeks later. Recent studies, however, indicate that an inter- CASE RESOLUTION
val appendectomy may not be indicated for most patients unless The patient was found to have an elevated white blood cell count with a shift to
symptoms recur. If nonsurgical management is unsuccessful, appen- the left and a small number of white blood cells in the urinalysis. Ultrasonography
dectomy is performed. findings were consistent with acute appendicitis. She was administered IV fluids
and antibiotics and underwent laparoscopic appendectomy. The postoperative
Charles McBurney, MD, first described open appendectomy
course was unremarkable, and the patient was discharged the next day. She did
through a traditional RLQ incision and muscle-splitting technique well in follow-up and was cleared for full activity 2 weeks postoperatively.
in 1893. Laparoscopic appendectomy was introduced more than
30 years ago and has largely replaced open appendectomy. Compared
with the open technique, laparoscopic appendectomy is associated
with lower wound infection rates, shorter hospital stay, fewer post- Selected References
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Although earlier studies associated laparoscopic appendectomy with children with perforated appendicitis. Arch Surg. 2011;146(6):660–665 PMID:
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ference in these areas. In fact, some studies have shown shorter sur- terns in the treatment of perforated appendicitis in children. J Am Coll
gical time and lower abscess rates with laparoscopic appendectomy. Surg. 2003;196(2):212–221 PMID: 12595049 https://doi.org/10.1016/
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Primary nonsurgical management of acute appendicitis, without
the intention of eventual appendectomy, recently has been gaining Duke E, Kalb B, Arif-Tiwari H, et al. A systematic review and meta-analysis of
diagnostic performance of MRI for evaluation of acute appendicitis. AJR Am
in popularity and appears to be a clinically effective, cost efficient,
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and safe approach. Currently, primary nonsurgical management is AJR.15.14544
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ated with a high initial failure rate of nonsurgical management, and inconclusive results in ultrasound for appendicitis in children and teenagers. Can
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tial success rate of nonsurgical management is 90% to 95%, with carj.2010.03.009
a recurrence rate of approximately 20% at 1-year follow-up. Thus, Kaminski A, Liu IL, Applebaum H, Lee SL, Haigh PI. Routine interval appen-
the overall success rate at 1 year is 75%. Patients who return with a dectomy is not justified after initial nonoperative treatment of acute appendici-
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dectomy, antibiotics are administered for a maximum of 24 hours Surgical Association Outcomes and Clinical Trials Committee. Antibiotics
and appendicitis in the pediatric population: an American Pediatric Surgical
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tive dose of antibiotics has been shown to decrease the risk of wound
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discharged on oral antibiotics to complete a 7-day course. Martin AE, Vollman D, Adler B, Caniano DA. CT scans may not reduce the neg-
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Minneci PC, Sulkowski JP, Nacion KM, et al. Feasibility of a nonoperative
Overall complication rates are less than 3% for nonperforated appen- management strategy for uncomplicated acute appendicitis in children. J Am
dicitis and 16% to 18% for perforated appendicitis. The common Coll Surg. 2014;219(2):272–279 PMID: 24951281 https://doi.org/10.1016/j.
complications after appendectomy are infection related. Wound jamcollsurg.2014.02.031

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BPPCA6e_Ch077_0549-0554.indd 554 2/13/20 6:09 PM
 CHAPTER 78

Head Trauma
Joseph Ravera, MD

CASE STUDY
A 2-year-old girl is playing on a window ledge unsu- Questions
pervised. She pushes the screen out and falls onto the 1. What are the priorities in the initial stabilization and
concrete sidewalk below, striking her head. A neighbor management of pediatric head trauma?
reports that she is unconscious for 10 minutes. When 2. What is the difference between primary and second-
paramedics arrive, the girl is awake but lethargic. She ary brain injury?
is transported to the emergency department. Her vital 3. What are the common signs and symptoms
signs are normal. A scalp hematoma is present, and a manifested by children with head trauma?
depressed area of cranial bone is palpated. 4. What are the various modalities available for
management of increased intracranial pressure?
5. What are the scoring systems used in the evaluation
of mental status in children with head trauma?

Although most childhood head injuries are minor and can be man- or signs and symptoms related to the injury. These include external
aged on an outpatient basis, it is important for physicians to become bruising or lacerations, alterations in the level of consciousness, and
adept at recognizing and managing concussions and more severe neurologic findings, including seizure. Vital signs may be altered;
forms of head injury. Health professionals can also help reduce in particular, deep or irregular respirations, hypertension, or bra-
mortality from head trauma by actively promoting injury preven- dycardia may be apparent (Box 78.1). These changes are indicative
tion to patients and communities. of elevated intracranial pressure (ICP).

Epidemiology Pathophysiology
Head trauma is among the most common pediatric injuries and the Children have significant anatomic differences from adults that
leading cause of morbidity and mortality among pediatric trauma predispose them to head trauma and certain types of intracranial
patients. Pediatric head trauma accounts for more than 500,000
emergency department (ED) visits, 95,000 hospital admissions,
7,000 deaths, and 29,000 permanent disabilities per year in the Box 78.1. Diagnosis of Head Traumaa
United States. Hospital care costs exceed $1 billion annually. In pedi-
atric patients with multiple injuries, 70% of deaths that occur within ww Loss of consciousness
48 hours of hospitalization are the result of trauma to the head. Rates ww Somnolence
of intracranial injuries in children with only minor head trauma are ww Pallor
low, however, with the largest numbers occurring in young chil- ww Emesis/nausea/anorexia
dren and infants, with a prevalence of 3% to 6%. Only 0.4% to 1% of ww Irritability
children require surgical intervention after minor closed head injury. ww Lethargy
Falls account for most cases of pediatric head trauma. Other ww Seizure
major causes include motor vehicle crashes, vehicle versus pedes- ww Ataxia
trian collisions, bicycle crashes, sports-related injuries, and recre- ww Weakness
ational activities. Nonaccidental trauma (ie, child abuse) must be ww Pain
recognized as another important cause of head injury in children, ww Paresthesias
particularly among those younger than 2 years. ww Amnesia
ww Headache
ww Visual changes
Clinical Presentation
ww Confusion/altered mental status
The child who has sustained head trauma may present with a his-
tory of an antecedent event (eg, fall, collision with another child) a
All symptoms need not be present for a diagnosis of head trauma.

555

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 556 PART 5: ACUTE AND EMERGENT PROBLEMS

injury (Figure 78.1). They have a higher center of gravity, an increased only through education and safety, such as advocating for wearing
head to body ratio, and weaker neck muscles compared with adults. helmets in appropriate situations.
Additionally, children have thinner cranial bones and less myelinated
brain tissue, which predisposes them to intraparenchymal injuries.
Types of Head Injury
Whereas adults are more likely to have focal intracranial hematomas,
children are more likely to develop diffuse cerebral edema. Cerebral Even minor head trauma in a child can result in skull fracture or
edema can disrupt cerebral blood flow, resulting in ischemic injury. intracranial injuries. Most skull fractures are simple and linear. Other
Normally, blood flow to the brain is maintained at a constant fracture types are comminuted, diastatic, basilar, and depressed.
rate by the process of autoregulation. With severe brain injury, A comminuted fracture is one involving multiple skull fragments.
autoregulation is disrupted and blood flow to the brain is deter- A diastatic fracture is one with a wide separation at the fracture site.
mined by cerebral perfusion pressure (CPP), which is a measure Basilar fractures occur at the base of the skull and often have char-
of the mean arterial pressure (MAP) less ICP (CPP = MAP – ICP). acteristic findings on physical examination (ie, bilateral periorbital
Cerebral blood flow is therefore compromised when the MAP ecchymosis [ie, raccoon eyes], hemotympanum, postauricular ecchy-
is too low (ie, hypotension) or the ICP is too high (ie, cerebral mosis [ie, Battle sign]). In a depressed fracture, fragments of the skull
edema). Several of the management strategies in children with are displaced inward, potentially damaging intracranial structures.
severe brain injuries focus on maintaining MAP and reducing ICP; Head trauma may result in concussion, mild traumatic brain
however, control of CPP after head injury can be quite difficult. injury, or intracranial hemorrhage. A concussion is defined as a
Children have a greater capacity for recovery than adults; this is trauma-induced impairment of neurologic function. This may occur
especially true for infants and very young children, whose open with or without a loss of consciousness (LOC). Neurologic examina-
sutures and fontanels permit expansion of the skull in response tion is usually normal, but the patient may experience somatic symp-
to edema and blood. toms (eg, headache), physical signs (eg, LOC, amnesia), behavioral
In head trauma, primary and secondary brain injury can occur. changes, cognitive impairment, or sleep disturbances. Some of these
Primary injury is the structural damage that occurs to the cranium minor and subtle neurologic sequelae can last for months after the
and its contents at the time of injury. Secondary injury is damage to injury (ie, postconcussion syndrome). Most resolve within a rela-
the brain tissue after the initial event. Such damage may result from tively short period, typically 7 to 10 days; however, with more severe
hypoxia, hypoperfusion, hypercapnia, hyperthermia, and altered trauma the symptoms can last longer.
glucose or sodium metabolism. The main treatment strategies for A cerebral contusion is a bruise of the brain tissue and typically
patients who have sustained head trauma focus on the prevention occurs with a more severe injury, such as a high-speed motor vehi-
of secondary brain injury. Primary brain injury can be prevented cle crash. A contrecoup contusion may be sustained when the brain
strikes the skull on direct impact, bruising 1 portion of the brain, with
resulting injury to the opposite side of the brain on rapid deceleration.
Suture site 1 Galea Clinical manifestations depend on the location of the contusion but
Pericranium
often include altered mental status, excessive sleepiness, confusion,
2 3 4 and agitation. Small intraparenchymal hemorrhages and swelling of
Skull
the surrounding tissues are often seen on computed tomography (CT).
5 Fracture site
An epidural hematoma is a collection of blood that accumulates
6 between the skull bone and the tough outer covering of the brain
Dura
Arachnoid (ie, dura mater). These are often the result of tears in the middle
Subarachnoid meningeal artery caused by skull fractures. Classically, patients have
7 space initial LOC followed by a lucid interval and then rapid deteriora-
Pia tion secondary to brain compression. On CT, an epidural hematoma
Brain appears as a large collection of blood with convex borders next to the
skull (Figure 78.2A). Surgical evacuation is required in most cases.
The subdural hematoma accumulates between the dura and
the underlying brain tissue. These are associated with skull frac-
tures and contusions. On CT, they appear to have a crescent-shaped
8
border (Figure 78.2B). Large subdural hematomas usually require
surgical evacuation. In infants and young children, subdural hema-
tomas are often the result of nonaccidental trauma.
Figure 78.1. Functional anatomy of the brain and surrounding structures with
Diffuse axonal injury (DAI) involves extensive damage to the
sites of pathology. 1, Caput succedaneum. 2, Subgaleal hematoma.
axonal white matter of the brain that results from shearing forces
3, Cephalhematoma. 4, Porencephalic or arachnoid cyst. 5, Epidural hematoma.
6, Subdural hematoma. 7, Cerebral contusion. 8, Cerebral laceration. that typically occur with rapid acceleration or deceleration of
Reprinted with permission from Tecklenburg FW, Wright MS. Minor head trauma in the pediatric the brain (Figure 78.2C). The child with DAI may have normal or
patient. Pediatr Emerg Care. 1991;7(1):40–47, with permission from Wolters Kluwer Health. nonspecific findings on CT.

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 Chapter 78: Head Trauma 557

A B C

Figure 78.2. A, Epidural hematoma (asterisk). Note convex borders and midline shift. B, Subdural hematoma (arrows). Note the crescent shape. C, Diffuse axonal
injury. Note the ground-glass appearance and tightly compressed ventricles.
Reprinted with permission from Harris JH Jr, Harris WH, Norelline RA. The Radiology of Emergency Medicine. 3rd ed. Baltimore, MD: Williams & Wilkins; 1993:15, 16, 17.

Evaluation Secondary survey actions include palpation and inspection of

the scalp for soft tissue swelling, step offs (ie, indentation of the
History
skull), lacerations, and fullness of the fontanel. Facial bones should
History is obtained during the secondary survey or reassessment be tested for stability and deformities. Other clues to possible head
phase of evaluation. Prehospital health professionals or witnesses to trauma include the presence of a septal hematoma, draining blood
the injury should be asked about details of the event and the child’s or fluid from the nose or ears, dental injury, and malocclusion of the
status following the event (Box 78.2). mandible. The tympanic membranes should be visualized for the
presence of hemotympanum or cerebrospinal fluid otorrhea, which,
Physical Examination
along with postauricular ecchymosis (ie, Battle sign), periorbital
Careful attention to the vital signs of the child with head injury ecchymosis (ie, raccoon eyes), or cranial nerve palsies, is sugges-
is important. The presence of hypertension, bradycardia, and an tive of a basilar skull fracture. If possible, funduscopic examination
irregular breathing pattern (ie, Cushing triad) is suggestive of a should be performed to look for the presence of papilledema asso-
significant intracranial injury with associated increased ICP. ciated with increased ICP or retinal hemorrhages, which are indic-
ative of nonaccidental trauma.
A comprehensive neurologic examination is the most important
aspect of the secondary survey. This examination should include a
Box 78.2. What to Ask mental status assessment, cranial nerve evaluation, and assessment
Head Trauma of the presence and quality of deep tendon reflexes, muscle tone,
ww What was the mechanism of injury (eg, motor vehicle crash, ejection muscle strength, sensation, and cerebellar function.
from motor vehicle, fall, assault)? When describing mental status, imprecise terms such as “altered,”
ww If a fall, what was the height of the fall? “lethargic,” and “obtunded” should be avoided. Several scoring sys-
ww What was the type of impact surface? tems are available for assessing the mental status of children who
ww What was the shape of the object(s) striking the head? have sustained head trauma. Many are useful predictors of intracra-
ww What was the child’s immediate status after injury? nial injury and are also useful in assessing level of consciousness in
ww What changes in status occurred before arrival at the hospital? pediatric patients. The most universally accepted and widely used of
ww Did the child lose consciousness? If so, for how long? these scales is the Glasgow Coma Scale (GCS). It is used routinely in
ww Did the child vomit? children older than 5 years but can be modified for younger children.
ww Did the child have a seizure? If so, when did it occur in relation to the Like many other systems, the GCS measures responses to a variety of
injury, and does the child have an underlying seizure disorder? stimuli in 3 areas—eye opening, verbal, and motor. Scores should be
tabulated when the child first presents to establish a baseline, after

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 558 PART 5: ACUTE AND EMERGENT PROBLEMS

which the scores can be used for reassessment on a regular basis until quite sensitive for the detection of acute hemorrhage and skull frac-
the patient has stabilized or returned to normal mental status. Use ture. It can also provide additional information on the severity of
of these scores helps promote consistent and accurate communica- injury, indicating increased ICP, cerebral edema, or pending herni-
tion among health professionals. Table 78.1 shows how to calculate ation. Among the findings on CT that indicate severe brain injury
the GCS and modified GCS. In some circumstances the calculation are the shift of midline structures, effacement of the sulci, ventric-
of a precise GCS can be cumbersome, especially in time-critical ular enlargement or compression, and loss of normal gray/white
situations. Several rapid scoring systems have been developed and matter differentiation.
are currently under active study. One system, the AVPU, describes An emergent head CT is warranted for any child with altered
the type of stimulus required to provoke response in a patient as mental status, a GCS below 14, penetrating trauma, or focal neuro-
either alert, verbal, painful, or unresponsive. Recent literature has logic deficit. The question of which children with minor head trauma
shown that either an alert or a verbal response strongly correlates should undergo CT was evaluated in a study of 17,000 children from
with a GCS above 8. the Pediatric Emergency Care Applied Research Network (PECARN)
database. In this study, a decision rule was retrospectively derived
Laboratory Tests and then prospectively validated as a method to identify children
A complete blood cell count and serum electrolyte panel should be at very low risk for intracranial injury. These criteria can be found
performed for all pediatric patients with significant head trauma. in Box 78.3. If a child is otherwise healthy and meets these criteria,
Bedside glucose monitoring should be performed in any child with the risk of intracranial injury is extremely low and the child can be
a head injury with an altered level of consciousness. Toxicology eval- safely discharged from the ED or clinic with anticipatory guidance
uation may be indicated in the adolescent who appears to be intox- and return precautions. Neither CT nor a period of observation is
icated or has an altered level of consciousness. The infant or child required. It should be noted that this decision rule was validated as
with an intracranial hemorrhage should undergo screening coag- “rule out” only and meant to identify the child at very low risk. If
ulation studies (ie, prothrombin time, activated partial thrombo- a child does not meet all the criteria, it does not mean a CT scan
plastin time) as well as a type and screen test or crossmatch, in case is required.
surgery is required. With the speed and widespread availability of CT machines,
radiographs of the skull have relatively little role in the acute eval-
Imaging Studies uation of pediatric patients with head trauma. Currently, CT can
In cases of acute pediatric blunt or penetrating trauma, a noncon- be performed very quickly, often with little or no need for seda-
trast CT of the head is currently the diagnostic study of choice. It is tion. Although plain radiographs are sensitive for the detection of

Table 78.1. Pediatric Glasgow Coma Scale

>1 Year < 1 Year Score
Eye Opening Spontaneously Spontaneously 4
To verbal command To shout 3
To pain To pain 2
No response No response 1
Motor Response Obeys Obeys 6
Localized pain Localized pain 5
Flexion-withdrawal Flexion-withdrawal 4
Flexion-abnormal (decorticate rigidity) Flexion-abnormal (decorticate rigidity) 3
Extension (decerebrate rigidity) Extension (decerebrate rigidity) 2
No response No response 1
>5 Years 2-5 Years 0-23 Months
Verbal Response Orientated Appropriate words/phrases Smiles/coos appropriately 5
Disorientated/confused Inappropriate words Cries and is consolable 4
Inappropriate words Persistent cries and screams Persistent inappropriate crying and/or screaming 3
Incomprehensible sounds Grunts Grunts, agitated, and restless 2
No response No response No response 1
Total Pediatric Glasgow Coma Score (3-15)
Reprinted with permission from Singh AP. Glasgow Coma Scale and Pediatric Glasgow Coma Scale. https://boneandspine.com/pediatric-glasgow-coma-scale.

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 Chapter 78: Head Trauma 559

Box 78.3. Low-risk Criteria for Pediatric Management

Intracranial Injury Assessment and management occur simultaneously in the child with
acute injury. A primary survey is initially performed with attention
Age <2 Years
to circulation, airway, and breathing as well as cervical spine immo-
ww Normal mental status
bilization. Prompt neurosurgical consultation should be obtained for
ww No scalp hematoma except frontal
all children with significant head trauma to assess the need for sur-
ww No loss of consciousness, or loss of consciousness for <5 seconds
gical management and ICP monitoring. The primary management
ww Non-severe injury mechanism
goal of significant acute head trauma is the prevention of secondary
ww No palpable skull fracture
brain injury by maximizing oxygenation and ventilation, support-
ww Acting normally according to parents
ing circulation to maximize cerebral perfusion, decreasing elevated
Age ≥2 Years ICP, and decreasing cerebral metabolic demands.
ww Normal mental status Individuals with significant intracranial injuries often require
ww No loss of consciousness intubation for airway protection. Those individuals with a GCS
ww No vomiting score of 8 or lower should undergo rapid sequence intubation to
ww Non-severe injury mechanism control the airway. These individuals should be premedicated with a
ww No signs of basilar skull fracture sedative. Historically, etomidate was the sedative of choice because
ww No severe headache it is cardiovascular neutral with minimal effect on blood pressure
and because most other sedatives can cause hypotension, thereby
decreasing MAP and reducing CPP. One area of controversy involves
skull fractures, they do not provide any information about associ- the use of ketamine for sedation prior to intubation. Ketamine is
ated intracranial injuries. Additionally, several studies have dem- well known to raise the MAP, which has a positive effect on CPP;
onstrated that intracranial injuries occur in the absence of a skull however, it was historically thought to increase ICP, thus making it
fracture, particularly among young pediatric patients. Plain radiog- less ideal. Recent literature, mostly in adults, has called into ques-
raphy is obtained only as part of the skeletal survey in cases of sus- tion the effect of ketamine on ICP. Currently, no clinical data exist to
pected nonaccidental trauma. recommend or not recommend ketamine as an indication agent for
Magnetic resonance imaging has little role in the treatment of intubation in a severely injured pediatric patient with head trauma.
the patient with acute injury resulting from head trauma. Computed Historically, lidocaine was thought to potentially blunt the tran-
tomography is often more sensitive in detecting acute intracranial sient increase in ICP often associated with direct laryngoscopy and
hemorrhages and is sufficient to guide most immediate patient care orotracheal intubation. Given no difference in outcomes and ques-
issues. Magnetic resonance imaging may be superior to CT for iden- tionable benefit recent recommendations based on adult literature
tifying DAI and subtle brain injuries. Generally, magnetic resonance no longer recommend routine administration of lidocaine before
imaging takes longer to complete and often requires sedation, and rapid sequence intubation in patients with head trauma. Although
the scanners frequently are located away from the ED in an area in there are no published trials involving children, short-acting opi-
which the child cannot be monitored appropriately. oids such as fentanyl (1 mcg/kg) may be considered prior to induc-
One important consideration with any trauma victim is the tion to blunt response to direct laryngoscopy and rapid sequence
possibility of a cervical spine injury. Although cervical spine inju- intubation. When intubation is indicated, steps should be taken
ries are rare in children, the physician must maintain a high index to minimize desaturations, because several studies have demon-
of suspicion when presented with a child who has suffered signif- strated worse outcomes with hypoxia in children with severe trau-
icant head trauma, particularly from a high-energy mechanism matic brain injury.
such as an unrestrained motor vehicle crash. Careful evaluation Historically, moderate hyperventilation was advocated to reduce
and imaging of the cervical spine is required for all children with ICP. Hyperventilation reduces partial pressure of carbon dioxide,
an altered level of consciousness; significant, painful, distract- arterial, resulting in cerebral vasoconstriction and decreased cere-
ing injuries; an inability to communicate; focal neurologic def- bral blood flow, thereby reducing ICP. This has been shown to have
icits; or localized pain, swelling, or ecchymosis of the cervical the unwanted effect of disrupting cerebral metabolism and possibly
spine. Younger children (<8 years) have a larger occiput-body exacerbating ischemic injury. Currently, normal ventilation is rec-
ratio than older children and adults and consequently a higher ommended, with the goal of maintaining partial pressure of carbon
fulcrum; thus, most cervical spine injuries in this age group tend dioxide, arterial, no lower than 34 mm Hg. Moderate hyperventila-
to be at higher levels (ie, C1, C2, C3). Furthermore, as the result tion may have a role in the transient management of serious or acute
of increased flexibility and lack of ossification, children are at risk life-threatening elevations in ICP (ie, acute herniation) as a tempo-
for ligamentous or direct cord injury in the absence of a bony rizing measure until more definitive care (eg, neurosurgical decom-
injury. The physician should maintain a high index of suspicion pression) can be performed.
with severely injured children, even if no bony injury is evident Circulation and MAP should be aggressively supported with
on radiographs or CT. fluids to prevent hypoperfusion to the brain. Hypovolemia or

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 560 PART 5: ACUTE AND EMERGENT PROBLEMS

hypotension should never be assumed to result from head trauma As previously discussed, the child with no high-risk criteria as
alone. The child should be examined carefully for evidence of addi- outlined by the PECARN guidelines can be safely discharged with-
tional injury. Central venous pressure monitoring may be useful in out a period of observation or neuroimaging. However, the child with
addressing volume status; however, recent literature has suggested minor head injury (GCS score, 14–15) who does not strictly meet
that point-of-care bedside ultrasonography of the size of the infe- the PECARN criteria warrants either a period of observation or CT
rior vena cava and its respiratory variation may provide a reliable evaluation. Signs of a basilar skull fracture and altered mental sta-
estimate of the patient’s intravascular volume status. Vasoactive tus are considered high-risk features, and the affected patient should
medications may be necessary to maintain MAP in patients with undergo prompt CT of the head. Mechanism of injury, a scalp hema-
euvolemia. Morbidity has been shown to significantly increase with toma in children younger than 2 years, and severe headache and vom-
subsequent episodes of hypotension. iting in children age 2 years and older are less specific for intracranial
Elevations in ICP may impede cerebral blood flow and exacer- bleeding or skull fracture. Therefore, in these patients a period of
bate ischemic injury. Administration of hypertonic solutions is indi- observation, including a cautious challenge orally of food or liquid, is
cated in the management of increased ICP, particularly with signs a reasonable strategy to avoid the radiation risk of CT. An algorithm
of herniation. In the most recent guidelines, hypertonic (3%) saline of this approach is shown in Figure 78.3. The optimal time of observa-
is the first-line agent (as opposed to mannitol). If hypertonic saline tion is unknown; however, in most EDs a 6-hour observation period
is not readily available or the patient does not have the appropri- is considered appropriate. If during the observation period the child
ate access for its administration, however, mannitol can be used as deteriorates clinically or does not return to baseline, CT of the head
a temporizing measure. It is important to note that the use of man- should be performed. The physician should be wary of discharging a
nitol and other diuretics is contraindicated in patients with border- child whose condition has not improved to baseline after minor injury
line blood pressures because these agents can cause hypotension, or who has persistent emesis, even if CT is normal.
which can in turn decrease the MAP, thereby worsening cerebral More moderate head injuries (initial GCS score, 9–12) necessi-
perfusion. Additionally, elevation of the head of the bed to 30° tate a longer period of evaluation, likely in a monitored setting along
to promote venous drainage is also used to reduce ICP. Paralytic with neurosurgical consultation. Severe head injuries (GCS score
agents, sedatives, and analgesics may be necessary to prevent < 8) require aggressive stabilization in the ED with the measures
agitation, which also results in increased ICP and increases cere- described previously and admission to a pediatric intensive care unit.
bral metabolic demands. Painful procedures (eg, suctioning) should The child who has sustained a concussion warrants close obser-
be preceded by administration of adequate premedication with vation and reevaluation before resuming sports activities, because
sedatives and analgesics. The use of intraventricular pressure mounting evidence exists that multiple, sequential concussions
catheters is often necessary to allow for close monitoring of ICP. can have long-term debilitating effects. The American Academy of
Additionally, these can be used to drain cerebrospinal fluid to help Neurology has guidelines for evaluation and return-to-play parame-
decrease elevated ICP. ters. These recommendations include an evaluation by a health pro-
Hyperthermia and seizure activity should be managed aggres- fessional familiar with concussion and the use of sideline assessment
sively, because they increase cerebral metabolic demands. tools to rapidly evaluate the athlete for removal from play. Before
Hyperthermia should be managed with antipyretic agents and returning to play, the athlete should have complete physical and cog-
active cooling measures. Conflicting evidence exists on controlled nitive rest until the athlete is symptom-free. Additionally, the neuro-
hypothermia in children with severe brain injuries. The most logic examination and imaging (if performed) should be normal. Some
recent meta-analysis did not show a benefit for therapeutic hypo- programs use neuropsychiatric testing to evaluate for subtle deficits in
thermia in children; however, this remains an area of active study. the evaluation of return to play. Generally, the emergency physician
Anticonvulsant prophylaxis should also be considered in the child should stress to the athlete being evaluated the serious nature of a con-
with severe brain injury, especially within the first 7 days. It is impor- cussion and not clear the athlete to play from the ED; rather, the patient
tant to remember that the ability to detect clinical seizure activity is should be referred through the appropriate protocols of the team.
lost if the child is paralyzed.
Serum electrolyte levels should be followed closely, and any alter- Prognosis
ations should be minimized. The patient with head injury should be Age is the most important prognostic factor in outcome. Younger
monitored closely for the development of diabetes insipidus or syn- children tend to do better than older children. It remains difficult to
drome of inappropriate antidiuretic hormone. predict the outcome of any individual patient. Scalp lacerations, most
Often, the child with a large epidural and subdural hematoma skull fractures, and concussion are low-risk injuries. Intracranial
requires surgical evacuation. The individual with a depressed hemorrhage, specific skull fractures, head injury secondary to non-
skull fracture often requires surgery to lift the depressed frag- accidental trauma, and trauma accompanied by diffuse cerebral
ment away from the underlying brain. The child with significant edema are high-risk injuries. If untreated, severe head injury may
penetrating head trauma warrants antibiotic and antiepileptic pro- result in death from herniation.
phylaxis and may need angiography to assess for vascular injury. In Other complications from severe head trauma are posttrau-
all cases, tetanus status should be updated if necessary. matic seizures, requiring lifelong treatment with anticonvulsants;

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 Chapter 78: Head Trauma 561

A
GCS14 or other signs of
altered mental status,† Yes
CT recommended
or palpable skull fracture 13.9% of population
4.4% risk of ciTBI
No

Occipital or parietal or temporal scalp

haematoma, or history of LOC 5 s, Yes Observation versus CT on the basis
or severe mechanism of injury,‡ 32.9% of population of other clinical factors including:
or not acting normally per parent 0.9% risk of ciTBI • Physician experience
• Multiple versus isolated§ findings
• Worsening symptoms or signs
53.2% of population after emergency department
No 0.02% risk of ciTBI observation
• Age 3 months
• Parental preference
CT not recommended¶

B
GCS14 or other signs
of altered mental status,† Yes
CT recommended
or signs of basilar skull fracture 14.0% of population
4.3% risk of ciTBI
No

History of LOC, or histroy of Yes Observation versus CT on the basis

vomiting, or severe mechanism of other clinical factors including:
of injury,‡ or severe headache 28.8% of population
0.8% risk of ciTBI • Physician experience
• Multiple versus isolated§ findings
• Worsening symptoms or signs
after emergency department
57.2% of population
No observation
0.05% risk of ciTBI
• Parental preference

CT not recommended¶

Figure 78.3. Suggested algorithm for computed tomography in children younger than 2 years (A) and for those age 2 years and older (B) with GCS scores
of 14 to 15 after head trauma.*
Abbreviations: ciTBI, clinically-important traumatic brain injury; CT, computed tomography; GCS, Glasgow Coma Scale score; LOC, loss of consciousness; s, seconds.
*
Data are from the combined derivation and validation populations.
†
Other signs of altered mental status: agitation, somnolence, repetitive questioning, or slow response to verbal communication.
‡
Severe mechanism of injury: motor vehicle crash with patient ejection, death of another passenger, or rollover; pedestrian or bicyclist without helmet struck by a high-impact object; falls of more than
0.9 m (3 ft) (or more than 1.5 m [5 ft] for panel B); or head struck by a high-impact object.
§
Patients with certain isolated findings (ie, with no other findings suggestive of traumatic brain injury), such as isolated LOC, isolated headache, isolated vomiting, and certain types of isolated scalp
hematomas in infants older than 3 months, have a risk of ciTBI substantially lower than 1%.
¶
Risk of ciTBI exceedingly low, generally lower than risk of CT-induced malignancies. Therefore, CT scans are not indicated for most patients in this group.
Reprinted with permission from Kuppermann N, Holmes JF, Dayan PS, et al; Pediatric Emergency Care Applied Research Network (PECARN). Identification of children at very low risk of clinically-important
brain injuries after head trauma: a prospective cohort study. Lancet. 2009;374(9696):1160–1170.

hydrocephalus, necessitating placement of a ventriculoperitoneal permanent. The child with postconcussion syndrome may warrant
shunt catheter; and persistent vegetative or severely impaired men- formal neurobehavioral testing.
tal state. Penetrating head injuries can result in infections (eg, men-
ingitis, abscess) and vascular injuries (eg, aneurysm, arteriovenous Prevention
malformations). Sequelae such as postconcussion syndrome may Despite advancing medical knowledge and excellent critical care
result from less severe head trauma. Some of the characteristics of available to children with head trauma, little can be done to reduce
this syndrome include dizziness, headache, irritability, memory def- the severity of primary brain injury after it has occurred. Therefore,
icits, impaired behavior, and impaired cognitive development. These pediatric health professionals should make every attempt to educate
may persist for months after the head injury and sometimes are patients and families about prevention strategies. Some of the most

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 562 PART 5: ACUTE AND EMERGENT PROBLEMS

successful prevention strategies involve the required use of restraint after traumatic brain injury in children. N Engl J Med. 2008;358(23):2447–2456
devices (eg, seat belts) and proper safety gear (eg, bicycle helmets). PMID: 18525042 https://doi.org/10.1056/NEJMoa0706930
Anticipatory guidance and home safety recommendations provided Kirkwood MW, Yeates KO, Wilson PE. Pediatric sport-related concussion: a
to parents and caregivers are also worthwhile. Finally, communities review of the clinical management of an oft-neglected population. Pediatrics.
can contribute to injury prevention by providing playground resur- 2006;117(4):1359–1371 PMID: 16585334 https://doi.org/10.1542/peds.2005-0994

facing, reducing the height of playground equipment, and chang- Kochanek PM, Carney N, Adelson PD, et al; American Academy of Pediatrics
ing traffic laws. It is only through a combination of these prevention Section on Neurological Surgery; American Association of Neurological Surgeons/
Congress of Neurological Surgeons; Child Neurology Society; European Society
strategies that morbidity and mortality of pediatric head trauma will
of Pediatric and Neonatal Intensive Care; Neurocritical Care Society; Pediatric
be meaningfully reduced. Neurocritical Care Research Group; Society of Critical Care Medicine; Paediatric
Intensive Care Society UK; Society for Neuroscience in Anesthesiology and Critical
Care; World Federation of Pediatric Intensive and Critical Care Societies. Guidelines
CASE RESOLUTION for the acute medical management of severe traumatic brain injury in infants,
The young child has a significant mechanism of injury, brief LOC, and a depressed, children, and adolescents—second edition. Pediatr Crit Care Med. 2012;13(suppl 1):
altered mental status. Initial physical findings prompt suspicion of a depressed S1–S82 PMID: 22217782 https://doi.org/10.1097/PCC.0b013e31823f435c
skull fracture and overlying soft tissue injury. Appropriate diagnostic tools after
Koestler J, Keshavarz R. Penetrating head injury in children: a case report and
evaluation of circulation, airway, and breathing are cranial CT followed by admis-
review of the literature. J Emerg Med. 2001;21(2):145–150 PMID: 11489404
sion for observation, monitoring, and serial neurologic examination. Surgical
https://doi.org/10.1016/S0736-4679(01)00363-8
repair of the skull fracture may be necessary.
Kramer N, Lebowitz D, Walsh M, Ganti L. Rapid sequence intubation in trau-
matic brain-injured adults. Cureus. 2018;10(4):e2530 PMID: 29946498 https://
doi.org/10.7759/cureus.2530
Selected References
Kuppermann N, Holmes JF, Dayan PS, et al; Pediatric Emergency Care Applied
Atabaki SM, Stiell IG, Bazarian JJ, et al. A clinical decision rule for cranial Research Network (PECARN). Identification of children at very low risk of
computed tomography in minor pediatric head trauma. Arch Pediatr Adolesc Med. clinically-important brain injuries after head trauma: a prospective cohort
2008;162(5):439–445 PMID: 18458190 https://doi.org/10.1001/archpedi.162.5.439 study. Lancet. 2009;374(9696):1160–1170 PMID: 19758692 https://doi.
Brown RL, Brunn MA, Garcia VF. Cervical spine injuries in children: a review of org/10.1016/S0140-6736(09)61558-0
103 patients treated consecutively at a level 1 pediatric trauma center. J Pediatr Surg. McCrory P, Meeuwisse W, Johnston K, et al. Consensus statement on concussion
2001;36(8):1107–1114 PMID: 11479837 https://doi.org/10.1053/jpsu.2001.25665 in sport: the 3rd International Conference on Concussion in Sport held in Zurich,
Bruce DA. Head trauma. In: Fleisher GR, Ludwig S, Henretig FM, eds. Textbook November 2008. J Athl Train. 2009;44(4):434–448 PMID: 19593427 https://doi.
of Pediatric Emergency Medicine. 5th ed. Baltimore, MD: Lippincott Williams org/10.4085/1062-6050-44.4.434
& Wilkins; 2005 Osmond MH, Klassen TP, Wells GA, et al; Pediatric Emergency Research
Crompton EM, Lubomirova I, Cotlarciuc I, Han TS, Sharma SD, Sharma P. Canada (PERC) Head Injury Study Group. CATCH: a clinical decision rule
Meta-analysis of therapeutic hypothermia for traumatic brain injury in adult and for the use of computed tomography in children with minor head injury.
pediatric patients. Crit Care Med. 2017;45(4):575–583 PMID: 27941370 https:// CMAJ. 2010;182(4):341–348 PMID: 20142371 https://doi.org/10.1503/cmaj.
doi.org/10.1097/CCM.0000000000002205 091421

Dunning J, Daly JP, Lomas JP, Lecky F, Batchelor J, Mackway-Jones K; Children’s Palchak MJ, Holmes JF, Vance CW, et al. A decision rule for identifying children at
Head Injury Algorithm for The Prediction of Important Clinical Events Study low risk for brain injuries after blunt head trauma. Ann Emerg Med. 2003;42(4):
Group. Derivation of the children’s head injury algorithm for the prediction of 492–506 PMID: 14520320 https://doi.org/10.1067/S0196-0644(03)00425-6
important clinical events decision rule for head injury in children. Arch Dis Child. Palchak MJ, Holmes JF, Vance CW, et al. Does an isolated history of loss of con-
2006;91(11):885–891 PMID: 17056862 https://doi.org/10.1136/adc.2005.083980 sciousness or amnesia predict brain injuries in children after blunt head trauma?
Giza CC, Kutcher JS, Ashwal S, et al. Summary of evidence-based guideline Pediatrics. 2004;113(6):e507–e513 PMID: 15173529 https://doi.org/10.1542/
update: evaluation and management of concussion in sports: report of the peds.113.6.e507
Guideline Development Subcommittee of the American Academy of Neurology. Schutzman SA, Barnes P, Duhaime AC, et al. Evaluation and management
Neurology. 2013;80(24):2250–2257 PMID: 23508730 https://doi.org/10.1212/ of children younger than two years old with apparently minor head trauma:
WNL.0b013e31828d57dd proposed guidelines. Pediatrics. 2001;107(5):983–993 PMID: 11331675 https://
Halstead ME, Walter KD; American Academy of Pediatrics Council on Sports doi.org/10.1542/peds.107.5.983
Medicine and Fitness. Sport-related concussion in children and adolescents. Schutzman SA, Greenes DS. Pediatric minor head trauma. Ann Emerg Med.
Pediatrics. 2010;126(3):597–615. Revised December 2018 PMID: 20805152 2001;37(1):65–74 PMID: 11145776 https://doi.org/10.1067/mem.2001.109440
https://doi.org/10.1542/peds.2010-2005 Sun BC, Hoffman JR, Mower WR. Evaluation of a modified prediction instru-
Hoffmann F, Schmalhofer M, Lehner M, Zimatschek S, Grote V, Reiter K. ment to identify significant pediatric intracranial injury after blunt head
Comparison of the AVPU scale and the Pediatric GCS in prehospital setting. trauma. Ann Emerg Med. 2007;49(3):325–332.e1 PMID: 17210207 https://doi.
Prehosp Emerg Care. 2016;20(4):493–498 PMID: 26954262 https://doi.org/10.3 org/10.1016/j.annemergmed.2006.08.032
109/10903127.2016.1139216 Swaminathan A, Levy P, Legome E. Evaluation and management of moderate to
Huh JW, Raghupathi R. New concepts in treatment of pediatric traumatic brain severe pediatric head trauma. J Emerg Med. 2009;37(1):63–68 PMID: 19303237
injury. Anesthesiol Clin. 2009;27(2):213–240 PMID: 19703674 https://doi. https://doi.org/10.1016/j.jemermed.2009.02.003
org/10.1016/j.anclin.2009.05.006 Zeiler FA, Teitelbaum J, West M, Gillman LM. The ketamine effect on ICP in
Hutchison JS, Ward RE, Lacroix J, et al; Hypothermia Pediatric Head Injury Trial traumatic brain injury. Neurocrit Care. 2014;21(1):163–173 PMID: 24515638
Investigators and the Canadian Critical Care Trials Group. Hypothermia therapy https://doi.org/10.1007/s12028-013-9950-y

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 CHAPTER 79

Increased Intracranial Pressure

Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD

CASE STUDY
A 7-year-old boy has a 2-week history of recurrent gaze is somewhat limited. Dysconjugate left gaze is
vomiting. No fever, abdominal pain, or diarrhea has apparent, with slight failure of left eye abduction. The
accompanied the vomiting; the vomiting has no par- left eye does not blink as much as the right eye. Fundal
ticular relationship to meals; and the boy’s appetite examination discloses elevated discs with indistinct mar-
has decreased only slightly. The vomiting has gradually gins. No upper extremity weakness is evident. The right
increased in frequency and is occurring every night. The foot is slightly weaker than the left, ankle tone is bilat-
day before this visit there were 4 episodes. The boy’s par- erally increased, and 3 to 4 beats of clonus on the right
ents have noticed that their son is generally less active; and bilateral positive Babinski reflexes are present. Some
he spends more time playing on the floor of his room tremor occurs in both arms with finger-to-nose testing.
and does not want to ride his bicycle or play with neigh- The boy walks with shuffling, small steps; his gait has a
borhood friends. Some unsteadiness in the boy’s gait has slight lurching character; and he veers to the right.
manifested in the past few days. His parents attribute
this to weakness from the vomiting.
Questions
1. What clinical situations are associated with increased
The child’s vital signs are normal except for a blood
intracranial pressure?
pressure of 130/80 mm Hg. Although the boy is some-
2. What is the pathophysiological process leading to
what pale and uncomfortable, he does not appear to be
increased intracranial pressure?
in acute distress. His abdominal examination is unre-
3. What studies are used to evaluate the child with
markable. His speech is grammatically correct but sparse
increased intracranial pressure?
and hesitant, and he seems inattentive. On lateral and
4. What measures are used to treat the child with
upward gaze the boy has coarse nystagmus, and upward
increased intracranial pressure?

The signs and symptoms of increased intracranial pressure (ICP) of trauma mortality and morbidity in children, most often as the
often signal a serious, potentially brain damaging intracranial result of brain-damaging increases in ICP.
process that may require surgical or intensive care intervention Brain tumors are the most common solid neoplasms in children
depending on the underlying cause. Recognizing signs and symp- and frequently result in subacutely increased ICP by direct mass
toms early often results in a determination of the underlying cause, effect or blockage of cerebrospinal fluid (CSF) flow. Diagnosis may
after which management can resolve the secondarily increased ICP sometimes be suggested by neurocutaneous signs or other evidence
problem. Increased ICP can also be a critical care issue by itself even of tumor suppressor gene mutation. Ischemic brain damage result-
if the specific etiologic diagnosis is indeterminate. A growing num- ing from a difficult delivery at birth, a near drowning incident, or
ber of effective medical and surgical treatments for increased ICP a major intracranial arterial or venous vessel thrombosis is also a
are available. It is critical to have an understanding about when to significant etiologic contributor to increased ICP. Other causes of
initiate them because they can be lifesaving. brain swelling, such as lead intoxication and liver failure in Reye
syndrome, have become less common etiologies.
Epidemiology Idiopathic intracranial hypertension (IIHP, also called benign
A wide variety of clinical situations, including both acute and sub- intracranial hypertension or pseudotumor cerebri), which has an
acute processes that occur at all ages, in both sexes, and among all overall prevalence of 1 to 2 per 100,000 but is 20 times higher
ethnic groups, are commonly responsible for increased ICP. Only a in adolescent girls with obesity, may ultimately be found as the
few can be mentioned herein. Traumatic brain injury (TBI) is a lead- cause for the increased ICP. It often occurs spontaneously but
ing cause of increased ICP. Fifty-two percent of infants with TBI may may also follow the use of high doses of vitamin A, growth hor-
be victims of nonaccidental trauma; older children may be stricken mone, or tetracycline, or it may occur after withdrawal of steroid
pedestrians or bicycle riders, occupants of crashed motor vehicles, therapy. Similar physiology is seen with thrombosis of a venous
or victims of falls or sports injuries; and children of any age may suf- sinus caused by a clotting diathesis or complicated otitis media
fer gunshot wounding. Traumatic brain injury is the leading source or mastoiditis.

563

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 564 PART 5: ACUTE AND EMERGENT PROBLEMS

Clinical Presentation factors are overwhelmed. Irreversible damage to brain tissue occurs
primarily as a result of pressure of the other components overtak-
The child with increased ICP may present with a history of recurrent
ing the arterial blood pressure and not allowing adequate tissue
vomiting, lethargy, and new headaches of increasing frequency or
perfusion. In younger children, nonfused sutures allow more com-
severity (ie, crescendo headaches) or that awaken the child from sleep.
pliance if volume increases are relatively slow, but this factor is less
The physician must be acutely aware of the clinical situation. A prior
true for acute volume increases. Additionally, pressure gradients
history of trauma, ischemia, meningitis, hypertension, or vasculi-
exist across compartments or sites of CSF flow obstruction, or even
tis; presence of a CSF shunt; or a concomitant history of intoxication
around lesions within brain parenchyma, which results in focal find-
or metabolic aberration (ie, carbon monoxide, hyperammonemia,
ings in addition to those caused by global ICP or perfusion changes.
or diabetic ketoacidosis) may also be suspicious for increased ICP
Changes in any of the 3 components comprising the intracra-
in the child with compatible examination findings. Neonates with
nial volume may result in increased ICP in several ways. First, the
intraventricular hemorrhage or myelomeningocele or other major
brain parenchyma component may be directly increased by mass
central nervous system malformations are prone to hydrocephalus.
lesions, such as neoplasms, abscesses, or hemorrhages. Vasogenic
Children with cyanotic congenital heart disease are prone to cere-
edema may increase the brain parenchyma volume because of vas-
bral abscesses, and children with sickle cell disease can present with
cular leakage due to cytokines. Brain edema may also result from
stroke or hemorrhage, resulting in increased ICP. In endemic areas
cytotoxic damage, cell death, and necrosis, producing increased
of the world, cerebral malaria and intraventricular cysticercosis are
interstitial oncotic pressure from released proteins and ions, and cel-
frequent causes of increased ICP. The pertinent physical findings
lular inflammatory and repair processes. The immediate cause may
may include elevated optic disc, failure of upward gaze, hyperto-
be mediated by cellular insults, including hypoxemia; intermediary
nicity of the extremities, and either depressed alertness or inatten-
metabolic toxins, including neuronal excitotoxins; and depletion of
tion or severely altered mental status. More localized findings on
energy substrates that are consequential to major vessel occlusion,
neurologic examination may also point to a lesion indicative of
contusional trauma or traumatic diffuse axonal injury, anoxia from
a space-occupying intracranial mass, which could contribute to
cardiac arrest, hypertensive encephalopathy, encephalitic infection,
increased ICP (Box 79.1).
or external metabolic poisoning. Edema with head trauma is known
Pathophysiology to be worse in children than in adults and may be a combination of
vasogenic and cytotoxic edema and may be related to neurogenic
The problem of increased ICP can be understood in terms of the inflammatory release of substance P and calcitonin gene-related
Monro-Kellie doctrine, which applies to the rigid cranial com- peptide at the molecular level close to vessels.
partment and pressure-volume relationships of the contents. This Second, the pressure of the CSF volume component (ventricles
doctrine is conceptually useful even though not always quantitatively or subarachnoid spaces) may increase in the setting of hydroceph-
predictive because of the variable compliance of the child’s skull and alus. Hydrocephalus can result in 2 ways: from a discrepancy in the
dural membranes, particularly in the first 2 years after birth before rate of formation of CSF relative to absorption and from an obstruc-
most of the cranial sutures are fused. The skull and dura mater form tion between the point of formation in the lateral ventricles and the
a relatively rigid compartment; any increase in 1 of the 3 intracra- sites of absorption at the arachnoid granulations. An obstruction can
nial volume components—brain parenchyma, CSF, and blood— occur with a congenital malformation; a parenchymal or intraven-
must occur at the expense of 1 or both of the other 2. Decreased tricular mass, such as a cyst or neoplasm; CSF inflammatory cells
volume results in increased pressure in an inverse relationship; from meningitis, ventriculitis, or hemorrhage; subarachnoid protein
the rise becomes much steeper, however, when initial compliance or debris; displaced brain parenchyma from mass effect; or over-
growth of dural tissue. The small passageways connecting the ven-
tricular system, the foramen of Monro, and the aqueduct of Sylvius;
Box 79.1. Diagnosis of Increased the exits of the ventricular system, the foramen of Magendie, and the
Intracranial Pressure foramen of Luschka; and the cisterns surrounding the brain stem are
ww Loss of appetite, nausea, vomiting, headache, or lethargy particularly vulnerable points of obstruction. Another type of brain
ww Inattention, decreased ability to arouse edema, interstitial edema, is characterized by periventricular tran-
ww Full fontanelle, increased head circumference sudation of CSF into the adjacent white matter and generally occurs
ww Papilledema, upward gaze paresis in the patient with acute or subacute hydrocephalus.
ww Increased tone, positive Babinski reflex Third, ICP may rise because the intravascular volume compo-
ww Focal signs and history compatible with an intracranial mass nent may increase. One process that leads to this increase is venous
ww Mass lesion, cerebral edema, occluded major vessel, or enlarged ventri- outflow obstruction, such as with a dural sinus thrombosis. Many
cles on an imaging study patients initially diagnosed as having IIHP are subsequently found
ww Elevated cerebrospinal fluid pressure in the lumbar intrathecal or intra- to have a diagnosis of transverse sinus stenosis or thrombosis. Other
cranial space as measured using a manometer processes that raise jugular venous pressure may also increase ICP.
Additionally, the intracranial arterial vascular volume is affected

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 Chapter 79: Increased Intracranial Pressure 565

by partial pressure of carbon dioxide. It not only increases with reliable, when it is present with other suggestive clinical circum-
hypercapnia and inadequate ventilation but also decreases stances, the rise in systemic pressure can be a useful clinical sign of
with hypocapnia, which occurs with compensatory central neuro- increased ICP. Normally, changes in arterial cerebrovascular resis-
genic hyperventilation or iatrogenic reduction of ICP by mechani- tance meet changes in perfusion pressure to maintain constant
cal hyperventilation. cerebral blood flow, a process called autoregulation. This process is
Because the physiology is dynamic, it has proven useful to quan- frequently compromised after head trauma or asphyxia, however,
titate ICP for management purposes. Intracranial pressure is often and is shifted with chronic hypertension.
measured as centimeters of water (cm H2O), whereas blood pres- Acute or subacute changes in pressure within an intracranial
sure is noted as millimeters of mercury (mm Hg). Normal ICP levels compartment may produce a pressure gradient across compartments
are somewhat lower in the neonatal and infantile period, at approx- that may precipitate brain herniation syndrome (Figure 79.1). An
imately 6 cm H2O (5 mm Hg), but in adolescents, pressures above ominous heralding sign of transtentorial herniation of the uncus
25 cm H2O (18 mm Hg) are abnormal and may produce symptoms. of the temporal lobe is loss of the pupillary light reflex caused by
Although it is possible to have normal cognitive function at an ICP entrapment of cranial nerve III. This herniation often results in irre-
of 52 cm H2O (40 mm Hg), this assumes an adequate perfusion versible brain stem damage as well as infarcts and additional sec-
pressure. Perfusion pressure is the mean arterial pressure (MAP) ondary edema, which can end with brain death. Focally increased
less the ICP. The ICP becomes clinically significant when the per- posterior fossa pressure may result in a pressure cone downward
fusion pressure is compromised, which may occur when the ICP is through the foramen magnum, compressing medullary centers,
78 cm H2O (60 mm Hg) below the MAP, which might translate to an sequentially extinguishing cranial nerve functions, producing
ICP as low as 20 mm Hg if the MAP is 80 mm Hg. It can become decerebrate posturing, and finally causing apnea and brain death.
dangerous when the ICP is only 52 cm H2O (40 mm Hg) below the A marginally compensated system could be decompensated by an
MAP, which translates to an ICP of 40 mm Hg if the MAP is 80 mm Hg. ill-advised lumbar puncture when the spinal compartment pressure
Decreased perfusion produces swollen, damaged tissue, which is acutely decreased, thereby increasing the pressure gradient across
increases the brain parenchymal compartment volume and further the foramen magnum and producing herniation.
exacerbates the pressure-volume problem in a cascading fashion.
Total loss of brain perfusion occurs when the rise in ICP overtakes
and becomes equal to the MAP. Differential Diagnosis
As ICP increases, brain perfusion pressure may be maintained Complicated migraine, seizures, and metabolic derangements are
transiently by a spontaneous increase in MAP, a response referred common problems that sometimes have a clinical presentation sim-
to as the Cushing response (ie, hypertension along with bradycardia ilar to increased ICP because they may present with headache and
and bradypnea). Although the relationship may not be universally altered mental status. A characteristic prodrome or the “pounding”

2
4

Figure 79.1. Left, Illustration of a normal brain. Right, Illustration of the anatomy of several potential herniation syndromes caused by intracranial compartment
pressure gradients related to a mass in a cerebral hemisphere. 1, Transfalcine herniation. 2, Uncal herniation. 3, Contralateral tentorial-midbrain damage.
4, Central herniation and foramen magnum pressure cone. These syndromes often result in further brain ischemia and additional increases in intracranial
pressure.

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 566 PART 5: ACUTE AND EMERGENT PROBLEMS

nature of the pain may help separate migraine from increased ICP. may awaken the patient from sleep or be worsened by cough, mictu-
At the initial headache presentation or when only a short headache rition, defecation, or other Valsalva-like maneuvers. A “thunderclap”
history is present, the complicated migraine diagnosis may be one headache may be indicative of an intracranial hemorrhage. Rapid
of exclusion. If the child displays focal neurologic signs with some progression of symptoms generally motivates concern.
of the general symptoms of increased ICP, an imaging study to rule
out a space-occupying lesion and confirm the safety of a lumbar Physical Examination
puncture as well as a subsequent measurement of normal pressure Vital signs and head circumference should be noted. Neck stiff-
by lumbar puncture manometry may be necessary to support the ness is an important sign of either meningeal irritation or cervical
diagnosis of complicated migraine rather than increased ICP. trauma. A careful neurologic examination is warranted when-
In the child who is only partially responsive, the task of distin- ever increased ICP is suspected. Particular attention should be
guishing a seizing or postictal state from a condition that may be paid to the components of the mental status and level of respon-
producing increased ICP is sometimes difficult. Findings sugges- siveness and alertness of patients. The presence of papilledema
tive of a seizure include rhythmic, clonic movements or sudden and the cranial nerve functions, including visual fields, should
myoclonic jerks; rapid or variable changes of tone or posturing that be assessed. Papilledema may not appear for a few days after ICP
are different from the decerebrate posturing that may accompany is increased. Retinal hemorrhages in an infant may be suspicious
a process producing increased ICP; abrupt, fluctuating changes of for nonaccidental trauma. Cranial nerve VI is particularly suscep-
autonomic function (eg, heart rate, blood pressure, pupillary size); tible to increased ICP, and an abnormality may be falsely localiz-
saliva production without swallowing; and a history of prior sei- ing. Impaired upward gaze and lid retraction may be present. The
zures. Sometimes, however, only direct electroencephalography patient may tilt the head to compensate for dysconjugate gaze.
(EEG) monitoring with ICP monitoring can distinguish ongoing Specific muscle tone and strength as well as gait characteristics
electrographic “subclinical” seizure activity from increased ICP as and ataxia should be evaluated in the child who cannot cooper-
the cause of the change in level of responsiveness. ate. In the comatose child, posturing responses to stimulation and
In some instances, diffuse brain dysfunction from a toxic or the breathing pattern should be noted to help ascertain brain stem
metabolic etiology mimics increased ICP. Such toxic or metabolic localization of a lesion. In a comatose child, findings should be
causes include medication toxicity, electrolyte or blood chemistry reassessed at frequent intervals until stable to follow a potentially
imbalance, and systemic infection. With toxic or metabolic dis- rapidly progressing devastating process, such as impending ten-
orders, inattention is often accompanied by an acute confusional torial or brain stem herniation, which would necessitate immedi-
state with disorientation, incoherence, and sometimes agitation. ate surgical intervention.
In contrast, with subacutely increased ICP, inattention frequently A useful quick-assessment instrument for initial, rapid evalu-
is accompanied by slowness of thought, perseveration, decreased ation and subsequent monitoring is the pediatric Glasgow Coma
mental activity, and impaired gait. Scale (Table 79.1). These scales are a useful shorthand description
for emergent triage purposes and perhaps for acute ventriculos-
Evaluation tomy decisions in trauma cases but are not sufficient for all clinical
decisions related to patients with increased ICP.
History
The newborn or infant may display a unique collection of signs—
A thorough neurologic history should be obtained (Box 79.2). enlarged head circumference; bulging, raised fontanelle; frontal bone
Headache, nausea and vomiting, drowsiness, personality change, bossing with prominent venous distention; irritability, setting sun
declining school performance, and changes in visual acuity and sign (ie, inability to elevate the eyes and lid retraction resulting from
obscurations are important historical factors. The headache history midbrain tectal pressure); hypertonicity; and hyperreflexia—that
may be one of crescendoing in frequency or intensity, consistent may be secondary to increased ICP resulting from hydrocephalus.
localization, and only a few days’ or weeks’ duration. The headache Papilledema is generally not present, perhaps related to greater com-
pliance of the newborn and infant skull.
Chronic hydrocephalus as a cause of increased ICP may result
in optic atrophy, depressed hypothalamic functions, spastic lower
Box 79.2. What to Ask
limbs, incontinence, and learning problems.
Increased Intracranial Pressure
ww How long has the child been vomiting, and when does the vomiting
Laboratory Tests
occur? If mental status changes are suggestive of a toxic or metabolic aberra-
ww Do headaches awaken the child? tion, appropriate laboratory screening should be performed, includ-
ww Does the child have weakness or change in gait? ing complete blood count, glucose level, electrolyte level, toxicology
ww Does the child have a recent history of trauma? screening, liver function tests, arterial blood gasses, and kidney
ww Has there been a progressive decline in activity level or loss of develop- function tests. If signs of meningeal irritation or infection are also
mental skills? present without lateralized signs of altered tone or strength, evidenc-
ing an intercompartmental pressure gradient, a CSF examination

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 Chapter 79: Increased Intracranial Pressure 567

should be done. Otherwise, if the patient is at risk for herniation Management

after lumbar puncture, a preceding brain imaging study is neces-
If IIHP is diagnosed, the pressure can be monitored with lumbar
sary. If the imaging study suggests that performing a lumbar tap
punctures until it is stable. Visual fields can be monitored using
carries a risk, CSF analysis and pressure measurements can be done
optical coherence tomography to assess the degree of retinal dam-
based on ventriculostomy instead. For the patient with clinical signs
age. Monitoring should occur at regular intervals until full recovery.
of increased ICP and no focal clinical or imaging signs of a mass, a
Increased ICP may respond to diuretic agents, such as acetazolamide
lumbar puncture with opening pressure measurement is also nec-
at high dose (20 mg/kg/day) or furosemide, as well as the removal
essary for the diagnosis of IIHP.
of CSF during the lumbar puncture as a temporizing measure. A
weight loss program is quite important if obesity is present. The
Imaging Studies primary danger in this condition is eventual blindness caused by
Computed tomography (CT) should be performed whenever a child’s pressure at the optic nerve head. If medical therapy is not success-
signs and symptoms indicate the possibility of increased ICP and ful, lumbar or ventricular peritoneal shunt placement or optic nerve
should be performed even in the setting of symptoms without signs sheath fenestration can be done surgically. The risk of severe vision
in a child younger than 6 years. Patients with severe head trauma loss is 6% to 14%.
frequently have a particularly dynamic pathophysiology, and The child with encephalopathy whose ICP is markedly ele-
an additional early CT scan after a few hours may be indicated to vated or likely to rise rapidly requires treatment in an intensive
assess for increasing hemorrhage, mass effect, or ventricular size care unit. When diagnostic imaging studies reveal an etiology for
along with ICP monitoring. Magnetic resonance imaging may the increased ICP, such as a rapidly enlarging epidural hematoma,
be necessary to detect ischemia early. Intravenous (IV) contrast immediate neurosurgical craniotomy may be necessary. Other
should be given if a source of disruption in the blood-brain barrier focal space-occupying lesions noted on imaging studies may not
(eg, infection, inflammation, neoplasia) is suspected. Magnetic res- require immediate craniotomy, depending on size and position
onance venography can be obtained if a possibility exists of a venous of the lesion as well as the distortion of normal brain tissue and
sinus thrombosis. Computed tomography angiography or traditional potential for imminent loss of perfusion; neoplastic lesions may
intraluminal angiography with the option of intervention to prevent require diagnostic biopsy or excisional biopsy within a few days
rebleeding may be the best studies in the patient with intracranial if surgically accessible. Mineralocorticoids (eg, dexamethasone
hemorrhage of unknown source. 0.25–0.5 mg/kg every 6 hours) are useful in situations in which the

Table 79.1. Pediatric Glasgow Coma Scale

>1 Year < 1 Year Score
Eye Opening Spontaneously Spontaneously 4
To verbal command To shout 3
To pain To pain 2
No response No response 1
Motor Response Obeys Obeys 6
Localized pain Localized pain 5
Flexion-withdrawal Flexion-withdrawal 4
Flexion-abnormal (decorticate rigidity) Flexion-abnormal (decorticate rigidity) 3
Extension (decerebrate rigidity) Extension (decerebrate rigidity) 2
No response No response 1
>5 Years 2-5 Years 0-23 Months
Verbal Response Orientated Appropriate words/phrases Smiles/coos appropriately 5
Disorientated/confused Inappropriate words Cries and is consolable 4
Inappropriate words Persistent cries and screams Persistent inappropriate crying and/or 3
screaming
Incomprehensible sounds Grunts Grunts, agitated, and restless 2
No response No response No response 1
Total Pediatric Glasgow Coma Score (3-15)
Reprinted with permission from Singh AP. Glasgow Coma Scale and Pediatric Glasgow Coma Scale. https://boneandspine.com/pediatric-glasgow-coma-scale/.

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 568 PART 5: ACUTE AND EMERGENT PROBLEMS

pressure is produced by a component of vasogenic edema, such as and decreasing the inspiratory phase of the ventilator and avoiding
that surrounding neoplasms. Measures should also be used to help high positive pressure and end-expiratory pressure. If acute reduc-
prevent a stress ulcer. Hypotonic IV fluids should be avoided, and tion of pressure is necessary, hyperventilation to reduce the intra-
serum and urine osmolality should be monitored for the syndrome cranial arterial blood volume is quite effective; on a chronic basis,
of inappropriate antidiuretic hormone secretion. Hypoglycemia however, partial pressure of carbon dioxide should be kept at 32 to
and hyperglycemia should be avoided as well. If the patient is in 38 mm Hg to avoid decreasing brain cell perfusion. Indomethacin
acute danger for herniation resulting from a pressure gradient pro- is also a cerebral vasoconstrictor and carries the same risk to ade-
duced by CSF flow blockage, a temporary ventriculostomy may be quate perfusion. Elevating the head of the bed to approximately
indicated to relieve the CSF pressure. If an infectious process is sus- 30° and avoiding flexion or turning of the neck to prevent jugular
pected, including focal lesions, abscess, cerebritis, or encephalitis, kinking are effective in reducing ICP. Pain, fever, shivering, and sei-
antibiotics or antiviral agents are indicated. After directed specific zures must be managed aggressively. Because the goal is to ensure
treatment of the underlying lesion, the increased ICP may resolve perfusion while reducing ICP, maintaining and even elevating sys-
spontaneously. If hydrocephalus resulting from obstruction of CSF temic MAP by appropriate use of fluid therapy and pressor agents
flow persists after initial therapy is completed, ventriculoperito- are key therapeutic measures.
neal shunting of CSF may be necessary. Endoscopic third ventric- Diuretic agents, such as mannitol 0.25 to 1 g/kg bolus, which is
ulostomy avoids the long-term complications of obstruction and a form of osmotherapy, may also be useful through reducing brain
infection associated with ventriculoperitoneal shunt hardware but volume by removing water, changing the rheologic characteristics of
is less often successful in relieving the pressure in younger chil- blood, and producing reflex vasoconstriction. Caution is advised, how-
dren than older ones. ever. Mannitol used as a chronic infusion can eventually cross the
In the patient with no mass or space-occupying lesion requir- blood-brain barrier and draw more fluid into the brain. It is most effec-
ing surgical removal, interventional therapies are directed toward tive in patients in whom the blood-brain barrier is intact. Hypertonic
maintaining perfusion of recovering brain tissue. In the patient saline (3%) 2 to 6 mL/kg bolus followed by 0.1 to 1.0 mL/kg per hour
with head injury, a Glasgow Coma Scale score of 8 or less can be as a continuous infusion may be an effective alternative. Serum osmo-
used as a guideline for ICP monitoring. Intracranial pressure can larity greater than 320 mOsm/kg can result in renal failure. Generally,
be monitored on an ongoing basis with commonly used neurosur- such effects can be avoided by giving diuretic agents at intervals as a
gically placed devices, including the fiberoptic microtransducer and bolus and titrating up to the ICP-reducing dose. In some instances
intraventricular catheter or ventriculostomy. The fiberoptic micro- these agents are used to counter ICP plateau waves or increased pres-
transducer can measure pressure in brain parenchyma as well as in sure associated with endotracheal suctioning or other procedures.
fluid-filled spaces. A distinct advantage of the intraventricular cath- In the child with severe refractory increased ICP, especially if
eter is in allowing for therapeutic CSF drainage to relieve pressure, secondary to an acute focal process, a barbiturate (eg, pentobar-
although it may be difficult to place if the ventricles are small or bital) or the benzodiazepine midazolam can be given as a contin-
shifted; additionally, this device carries a slight risk of hemorrhage uous IV infusion with appropriate monitoring of brain electrical
or infection, increasing to a plateau at day 4 of 1% to 2% per day. activity, serum levels, and systemic and brain perfusion pressures
Intracranial hypertension frequently peaks at 1 to 4 days after severe (Box 79.3). These agents may serve to reduce brain metabolism
trauma. Intracranial pressure monitoring with devices and therapy without significantly impairing vascular autoregulation; however,
based on the aforementioned measurements, however, has not been potential risks include reducing cardiac output and inducing associ-
helpful in most patients with severe ischemic damage, infection, or ated infections, particularly pneumonia. Xenon CT measures multi-
poisoning. This finding may be because of the widespread nature of ple areas of local blood flow and may be a useful bedside technique
the insult and brain involvement so that little normally responding to help specify targeted therapies. Decompressive craniotomy or
tissue remains in which perfusion could be maintained. craniectomy in early severe trauma in small series of patients has
The child with a decreased or fluctuating level of responsiveness been associated with good outcomes in up to 50%. Hypothermia has
may require EEG monitoring of cerebral electrical activity. Seizures not been useful in the management of cardiac arrest in the pediatric
may occur even in the presence of increased ICP. Anticonvulsant patient. Numerous “neuroprotective” agents continue to be studied
therapy is indicated if evidence of clinical or electrographic seizures as means of slowing metabolism and the resulting excitotoxic gluta-
is present. Additionally, the EEG may be used to monitor barbiturate- matergic damage and thereby reduce cytotoxic edema and spread of
or benzodiazepine-induced coma, which is used in the setting of the volume of irreversibly damaged brain tissue into the surround-
severely increased ICP. ing penumbra of damaged but not dead brain.
Respiratory physiology and ventilation are important for the
child with increased ICP, because hypoxia and hypercapnia can Prognosis
contribute to vasodilation and increased pressure. Rapid sequence The overall mortality rate for children brought to an emergency
intubation and avoidance of ketamine and succinylcholine help room with TBI is 4% to 5%. Most of these children die from
minimize elevations of ICP. Transmission of elevated intratho- increased ICP. Disability occurs in many of the survivors, but
racic pressure to intracranial vessels can be avoided by sedation the extent of disability may not be known for months to years

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 Chapter 79: Increased Intracranial Pressure 569

Box 79.3. A Simplified Intensive Care

Interventions Protocol for the Child CASE RESOLUTION
With Increased Intracranial Pressure The boy has focal signs and symptoms referable to the posterior fossa brain stem
and cerebellum as well as symptoms of increased ICP. Emergent CT shows sub-
ww Attend to airway, breathing, and circulation; intubation for airway acute hydrocephalus resulting from obstruction of CSF flow produced by a large
control for GCS score <8. mass in the cerebellum and brain stem on the left side. He begins taking dexa-
methasone and experiences some relief of increased ICP symptoms. Two days
ww Keep head of bed elevated to 30°.
later a ventriculostomy is placed and the mass, which is found to be a medul-
ww Neurosurgical consultation to consider ICP monitor for the patient with loblastoma, is surgically removed. Because the ICP eventually subsides over the
GCS score <8. next few days and decreasing ventriculomegaly is evident on CT, a ventriculo-
ww Maintain normal or elevated systemic blood pressure, normal tempera- peritoneal shunt is not required and the ventricular drain is removed. The boy’s
ture, and normal serum glucose level. recovery is otherwise uneventful, and he begins the staging evaluation for
medulloblastoma radiotherapy and chemotherapy.
ww Continuous general and local anesthesia, sedation, muscle relaxants,
and anticonvulsant agents.
ww Control hyperventilation to Paco2 at 32–38 mm Hg and provide
supplemental oxygen and PEEP as necessary to maintain Selected References
Pao2 > 90 mm Hg.
Adelson PD, Wisniewski SR, Beca J, et al; Paediatric Traumatic Brain Injury
ww Dexamethasone 0.5 mg/kg (maximum of 10 mg) every 6 hours if
Consortium. Comparison of hypothermia and normothermia after severe trau-
evidence exists of vasogenic edema on a brain imaging study.
matic brain injury in children (Cool Kids): a phase 3, randomised controlled trial.
ww If an ICP monitor is present, target cerebral perfusion pressure at Lancet Neurol. 2013;12(6):546–553 PMID: 23664370 https://doi.org/10.1016/
40–50 mm Hg (age ≥5 years) and 50–60 mm Hg (age 6–17 years). S1474-4422(13)70077-2
ww If ICP progressively rises, pressure waves >20 mm Hg last longerthan Ball AK, Clarke CE. Idiopathic intracranial hypertension. Lancet
5 minutes, or any pressure in the first 24 hours is >30 mm Hg, give Neurol. 2006;5(5):433–442 PMID: 16632314 https://doi.org/10.1016/
3% saline 5 mL/kg. Alternatively, give mannitol 250–1,000 mg/kg IV. This S1474-4422(06)70442-2
may be repeated to maintain serum osmolality at 300–320 mOsm. Christian CW, Block R; American Academy of Pediatrics Committee on Child
ww If 3% saline or mannitol needs repeating in <6 hours or osmolality is Abuse and Neglect. Abusive head trauma in infants and children. Pediatrics.
>320 mOsm: 2009;123(5):1409–1411. Reaffirmed April 2017 PMID: 19403508 https://doi.
Give pentobarbital 5 mg/kg IV, then 2 mg/kg/hour IV monitoring to org/10.1542/peds.2009-0408
blood level of 25–35 mg/mL, burst-suppression pattern with 10 sec- Del Bigio MR. Cellular damage and prevention in childhood hydroceph-
onds between bursts on EEG, and cardiac index >2.7 L/minute/m2. alus. Brain Pathol. 2004;14(3):317–324 PMID: 15446588 https://doi.org/
Or 10.1111/j.1750-3639.2004.tb00071.x
Give midazolam by titrating the dose upward starting at 0.1 mg/ Garton HJ, Piatt JH Jr. Hydrocephalus. Pediatr Clin North Am. 2004;51(2):
kg/hour IV to the same EEG criteria and limited by the same cardiac 305–325 PMID: 15062673 https://doi.org/10.1016/j.pcl.2003.12.002
index criteria. Huff K. Central nervous system failure. In: Osborn L, DeWitt T, First L, Zenel J,
ww Particularly in trauma cases in which the cerebral lesion is primarily eds. Pediatrics. Philadelphia, PA: Mosby; 2005;264–270 https://doi.org/10.1016/
B978-0-323-01199-0.50036-0
unihemispheric and pressure is rapidly increasing, hemicraniectomy
should be considered. Kochanek PM, Carney N, Adelson PD, et al. Guidelines for the acute medical man-
agement of severe traumatic brain injury in infants, children, and adolescents—
Abbreviations: EEG, electroencephalography; GCS, Glasgow Coma Scale; ICP, intracranial pressure; second edition. Pediatr Crit Care Med. 2012;13(suppl 1):S1–S82 PMID: 22217782
IV, intravenous; Paco2, partial pressure of carbon dioxide; Pao2, partial pressure of oxygen; https://doi.org/10.1097/PCC.0b013e31823f435c
PEEP, positive end-expiratory pressure. Kotagal S. Increased intracranial pressure. In: Swaiman KF, Ashwal S, Ferriero
DM, Schor NF, eds. Pediatric Neurology Principles and Practice. 4th ed.
Philadelphia, PA: Mosby; 2006
afterward because recovery can be quite slow. The 10-year sur- Marcoux KK. Management of increased intracranial pressure in the critically ill
vival rate for all children with brain tumor is 70%. In children child with an acute neurological injury. AACN Clin Issues. 2005;16(2):212–231
with encephalopathy with increased ICP with systemic hypoten- PMID: 15876889 https://doi.org/10.1097/00044067-200504000-00012
sion, hyperglycemia, or disseminated intravascular coagulation,
the prognosis for death or disability is worse regardless of cause.
Some types of lesion, such as ischemia, can generate increased
ICP that may advance too rapidly for therapeutic measures to take
effect or are refractory to medical or surgical treatment, resulting
in irreversible brain tissue damage or death. However, children
can recover from increased ICP if brain perfusion pressure is ade-
quately maintained and the underlying brain lesion generating the
increased pressure can be resolved.

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BPPCA6e_Ch079_0563-0570.indd 570 2/13/20 6:09 PM
 CHAPTER 80

Management of Dehydration
in Children: Fluid and
Electrolyte Therapy
Gangadarshni Chandramohan, MD, MSc, FASN, FAAP

CASE STUDY
A 2-year-old boy presents to your office after 2 days of electrolyte, blood urea nitrogen, and creatinine levels
vomiting and diarrhea. His siblings were both ill a few and initiate intravenous rehydration by administering
days previously with similar symptoms. At a well-child 2 boluses each of 240 mL normal saline (0.9% sodium
visit 2 weeks previously, his weight was 12 kg (26.5 lb). chloride solution).
Today his weight is 10.8 kg (23.8 lb). He has a pulse of
130 beats per minute, respiratory rate of 28 breaths per
Questions
1. How is the magnitude of dehydration in a child
minute, and blood pressure of 85/55 mm Hg. He is alert
assessed?
and responsive but appears tired. He has dry mucous
2. What are the different types of dehydration?
membranes, no tears with crying, and slightly sunken-
3. How is the type and amount of fluid required by the
appearing eyeballs. His capillary refill is 2 seconds. He
dehydrated child determined?
urinated a small amount approximately 6 hours before
4. How is renal status assessed in the dehydrated child?
this office visit. Despite his mother’s best efforts in your
5. What is the role of electrolyte and acid-base labora-
office, the patient has vomited all the oral rehydration
tory tests in the evaluation of the dehydrated child?
therapy given to him. You draw blood for analyzing

Dehydration resulting from gastrointestinal (GI) and other dis- physicians forgo sodium calculations in hospitalized children
orders, especially diarrhea, is among the most common medical and rely solely on isotonic NS (0.9% NaCl solution) for manage-
problems encountered in children younger than 5 years. During ment. Although this chapter incorporates these suggestions where
the past 50 years or more, the usual therapy for children who are appropriate, it describes the traditional approach to maintenance
hospitalized with dehydration has been to administer intrave- and deficit therapy because an understanding of the pathophysi-
nous (IV) fluids starting with 1 or 2 boluses of normal saline (NS; ology of dehydration helps in the treatment not only of the dehy-
0.9% sodium chloride [NaCl] solution at 20 mL/kg). This is fol- drated child but also of children with other types of fluid and
lowed by the administration of a sodium (Na+) solution of vari- electrolyte disorders.
able concentration (usually 0.45% NaCl) mixed with 5% dextrose
over the next 24 to 48 hours until the child is able to take oral flu- Epidemiology
ids. The exact amount of fluid and electrolytes is calculated using Over the past 30 years, hospital admissions and mortality resulting
complicated formulas to provide maintenance fluids and correc- from diarrhea and dehydration have decreased worldwide; never-
tion of remaining deficit (ie, deficit therapy). The calculation of theless, diarrhea remains 1 of the leading medical problems in chil-
maintenance therapy was first recommended in the 1950s, but dren younger than 5 years. According to the Centers for Disease
more recently it has been suggested that dehydration management Control and Prevention, more than 200,000 hospitalizations and
should focus on rapid restoration of extracellular fluid (ECF def- 300 deaths of children occur each year in the United States result-
icit) followed by oral rehydration therapy (ORT), and traditional ing from diarrhea. Additionally, diarrhea is responsible for 2 to
calculations of fluid deficits should be abandoned. Alternatively, 3 million outpatient visits each year and contributes to 10% of all
pediatric nephrologists and intensivists have recommended that hospital admissions.

571

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 572 PART 5: ACUTE AND EMERGENT PROBLEMS

Maintenance Fluid and Electrolyte (as in the example in Box 80.1, in which the child receives noth-
Requirements ing orally in preparation for surgery). Electrolyte quantities usu-
The body has a maintenance fluid requirement to replace daily ally are expressed as milliequivalent (mEq) or millimole (mmol)
normal losses that occur through the kidney, intestines, skin, and amount per 100 mL of fluid required. Traditionally, the recom-
respiratory tract. Of the various methods used to determine fluid mended sodium requirement for a healthy child is 3 mEq/100 mL
needs, the most common is the caloric method, also called the fluid required (approximately 0.2% NaCl or 0.25 NS), and the potas-
Holliday-Segar method, which is based on the linear relationship sium (K+) requirement is 2 to 2.5 mEq/100 mL of fluid (see Box 80.1,
between metabolic rate and fluid needs. For every calorie expended in part B, for sample calculation and IV order). Potassium should be
metabolism, a child requires approximately 1 mL of water. Metabolic administered only after ensuring adequate renal function. These
rate in children is a function of body surface area. Infants, with their estimations of sodium and potassium requirements are meant to
higher relative surface areas per unit of body weight, have higher replace normal daily losses and would not be adequate in the set-
metabolic rates and, therefore, higher fluid requirements per unit ting of increased electrolyte losses that can occur in a number of
weight compared with older children and adults. As the child grows, pathologic conditions (eg, diarrhea). Additionally, increased atten-
the relative surface area decreases, as do the metabolic rate and fluid tion has recently been given to the risk of hyponatremia and related
requirement per unit weight. Using this relationship, maintenance complications in hospitalized ill children.
fluid needs can be calculated for the healthy child using the method Relatively healthy, well-nourished children receiving IV fluids for
outlined in Table 80.1. These calculations of fluid needs are often a brief period (ie, 1–2 days) during hospitalization do not routinely
used to determine the amount of IV fluids provided to a hospitalized require supplementation with other electrolytes, such as calcium
child or to calculate the approximate amount of fluid a healthy child and magnesium. However, it is important to realize that standard
requires orally to maintain hydration. These calculations may not be IV fluids containing 5% dextrose, sodium chloride, and potassium
appropriate for children who are critically ill, however, some of whom chloride provide only minimal caloric needs and do not adequately
require fluid restriction and others of whom may have increased fluid support weight gain or provide other necessary nutrients. The child
needs. Moreover, the caloric method makes no allowance for extra who requires prolonged IV therapy because of inadequate GI tract
fluid needed for weight gain, growth, activity, or pathophysiologi- function should receive total parenteral nutrition to better meet the
cal states that increase fluid needs (eg, fever). The fluid requirement child’s caloric and nutritional needs.
derived from this method is valid to determine the daily fluid need
for an essentially healthy child. Thriving infants normally drink more Box 80.1. Example of Fluid Calculationsa
fluid than indicated by this method. On average, a growing infant may
take 150 to 200 mL/kg per day of milk (human milk or infant for- Part A
mula) as desired to support the average weight gain of 30 g (1.1 oz) Case: A boy weighing 22 kg is given nothing orally in preparation for an
per day usually observed in the first few months after birth. elective abdominal surgery. The following calculation is used to deter-
Replacement of normal daily losses of electrolytes is considered mine the appropriate amount of IV fluid per hour to administer as he awaits
when a child is not able to take adequate nutritional intake orally surgery.
ww For first 10 kg: 100 mL/kg/day × 10 kg = 1,000 mL
ww For next 10 kg (to get to 20 kg): 50 mL/kg/day × 10 kg = 500 mL
ww For next 2 kg (to get to 22 kg): 20 mL/kg/day × 2 kg = 40 mL
Table 80.1. Caloric (ie, Holliday-Segar)
1,000 mL + 500 mL + 40 mL = 1,540 mL/24 hour
Method of Determining Maintenance Fluid
IV rate per hour = 64.2 mL/hour (with a healthy child, round off to 65 mL/hour
Requirements in Healthy Children
for ease of administration)
Weight Maintenance Fluid Requirement for 24 Hours
Part B
<10 kg 100 mL/kg/daya
Question: How much Na+ and K+ should this patient receive in his IV fluids?
or Answer:
4 mL/kg/hour Based on physiologic losses: 3 mEq Na+/100 mL (1 dL) of fluid = 3 mEq ×
11–20 kg 50 mL/kg/day for each kg >10 kg + 1,000 mL (fluid 15.4 dL = 46.2 mEq Na+/day in 1,540 mL of water or 30 mEq NaCl/L
requirement for first 10 kg) 2.0 mEq K+/100 mL (1 dL) of fluid = 2.0 × 15.4 = 30.8 mEq K+/day in
or 1,540 mL of water or 20 mEq KCl/L
40 + 2 mL/kg/hour for each kg between 11 and 20 kg Therefore, IV order for this patient is as follows:
D5 0.2% NaCl (or 0.25 NS) with 20 mEq KCl/L to run at 65 mL/hour
>20 kg 20 mL/kg/day for each kg >20 kg + 1,500 mL (fluid
requirement for first 20 kg) Abbreviation: D5 = 5% dextrose water; IV, intravenous; K+, potassium; KCl = potassium chloride;
or Na+, sodium; NaCl, sodium chloride; NS, normal saline.
a
Even though in this example the calculations for sodium concentration in the IV fluid is
60 + 1 mL/kg/hour for each kg >20 kg physiologic, recent American Academy of Pediatrics guidelines recommend use of NS with
a
Excluding neonates and preterm infants. 5% dextrose preoperatively to prevent potential postoperative hyponatremia.

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 Chapter 80: Management of Dehydration in Children: Fluid and Electrolyte Therapy 573

Alterations in Fluid Needs in Illness brain stem herniation, permanent brain damage, or death. Recently,
Several conditions can influence fluid requirements. Conditions some pediatric nephrologists and intensivists have recommended
that increase a patient’s metabolic rate (eg, fever) will also increase forgoing sodium calculations in hospitalized very sick children and
a patient’s fluid requirement. A child’s metabolic rate is increased instead relying solely on isotonic fluids. Others have cautioned that
12% for every 1°C temperature elevation above normal. Most oth- physicians must make certain that the new recommendations to use
erwise healthy children with free access to fluids will increase their isotonic fluids do not result in excessive congestive heart failure or
own intake to account for increased needs when febrile. Other less hypernatremia before abandoning previous practices. Regardless of
common hypermetabolic states, such as thyrotoxicosis or salicylate the approach used, close attention to the type and quantity of fluids
poisoning, may have an even more dramatic effect, perhaps increas- provided, quantity of body fluid output, weight change, and serial
ing metabolic rate by 25% to 50% over maintenance. In these cases electrolyte assessments are important in the management of all sick
and for children who are dependent on others to provide their flu- children, and fluid and electrolytes must be individualized to each
ids, the physician must be aware of the magnitude of increased need patient to prevent serious complications.
and provide supplemental fluids to avoid dehydration.
Other conditions may decrease a child’s fluid requirement. In Pathophysiology
hypometabolic states, such as hypothyroidism, metabolic rate and Dehydration is among the most common pathophysiological alter-
fluid needs are decreased by 10% to 25%. Fluid requirements are ations in fluid balance encountered in pediatrics. Although strictly
decreased by 10% to 25% in high environmental humidity unless speaking, dehydration means deficit of water only, most children
the ambient temperature is also high and results in visible sweat- with dehydration have lost water and electrolytes. Dehydration can
ing. In these situations, a healthy child with normal renal function result from diminished intake, excessive losses through the GI tract
given extra fluid beyond what is needed can, within limits, effectively (eg, diarrhea, vomiting), excessive losses from the kidney or skin
excrete any excessive intake. The child with renal failure, however, (eg, polyuria resulting from osmotic diuresis in uncontrolled dia-
poses a special challenge for the physician in the management of fluid betes), or a combination of these factors.
and electrolytes. When a child cannot adequately excrete excessive Children are at increased risk for episodes of dehydration for
fluid intake, this fluid can accumulate and result in complications many reasons. Infants and young children have 2 to 4 times the
such as congestive heart failure and pulmonary edema. Without func- body surface area per unit body weight compared with adults and
tioning kidneys, only insensible fluid losses need replacing. Insensible as a result have relatively higher fluid needs. It is therefore much
losses occur primarily through the skin and respiratory tract; they easier for children to become dehydrated in the setting of decreased
account for approximately 40% of maintenance fluid needs. However, intake or increased losses that often accompany common childhood
fluid needs for patients with renal failure usually are estimated to be illnesses. For example, acute gastroenteritis, which is common in
30% of the maintenance requirement, with additional fluids provided young children, often results in anorexia, recurrent vomiting, and
if necessary. Limiting fluids avoids the accumulation of excessive frequent or large-volume stools, with proportionately more severe
fluids that may require dialysis for removal. fluid loss than in older children and adults. Additionally, infants and
Fluid requirements may also be decreased under circumstances young children are dependent beings who are unable to increase
in which arginine vasopressin (AVP; also called antidiuretic hor- their own fluid intake in response to thirst and must rely on others
mone) is increased. In addition to hypovolemia or hypertonicity to provide their fluid needs. If these fluid needs are not met or are
(ie, hyperosmolality), AVP release is also stimulated by pain, nau- underestimated, a child can easily become dehydrated.
sea, surgery (ie, in the postoperative period), central nervous system Dehydration is classified as isotonic, hypotonic, or hypertonic.
(CNS) infections (eg, meningitis, encephalitis), severe pneumonia or These terms often are used interchangeably with isonatremic,
respirator use, and certain medications, including thiazide diuretics, hyponatremic, and hypernatremic, respectively. The latter terms
chemotherapeutic agents, and selective serotonin reuptake inhibi- reflect the sodium content of the ECF that largely determines
tors. Arginine vasopressin release in the absence of hypovolemia or serum osmolality in the otherwise healthy dehydrated child. Acute
hypertonicity results in hyponatremia and is referred to as syndrome isotonic or isonatremic dehydration (serum Na+ 135–145 mEq/L),
of inappropriate antidiuretic hormone secretion. In patients with this which is the most common type of dehydration, involves net loss
syndrome, fluid restriction as well as administration of fluids with of isotonic fluid containing sodium and potassium (Figure 80.1,
a higher sodium concentration may be indicated. top). In diarrhea-related dehydration, sodium, the primary ECF
The most appropriate sodium concentration of IV fluids for the cation, is not only lost from the body but also shifts into the intra-
hospitalized child admitted to a pediatric intensive care unit or in cellular fluid (ICF) compartment to balance the loss of potassium,
the postoperative patient is controversial. Over the past 25 years, because potassium losses from cells generally are not accompanied
most such children have been maintained on a solution contain- by intracellular anionic losses in acute dehydration. The sodium
ing 5% dextrose water in half NS (D5 0.5 NS) or lower sodium con- that has shifted into the ICF compartment will return to the ECF
centrations (D5 0.25 NS). Studies suggest that because the kidney compartment during rehydration as potassium is being replen-
retains free water in response to excessive AVP in these children, ished, by the action of sodium/potassium adenosinetriphospha-
they are at risk for hyponatremia, hyponatremic encephalopathy, tase (ATPase). No net loss of fluid from the ICF occurs in this

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 574 PART 5: ACUTE AND EMERGENT PROBLEMS

dehydration because it can result in cerebral edema and eventu-

Isotonic/isonatremic dehydration
ally, brain herniation.
ECF Hypertonic or hypernatremic dehydration (serum Na+ >145
ICF
mEq/L) occurs when net loss of water exceeds that of solute loss
+
Na (Figure 80.1, bottom). It usually is seen in clinical conditions in
K+ which rapid loss of hypotonic fluid in stool, vomit, or urine occurs,
H2O accompanied by failure of adequate water intake because of anorexia
or vomiting. Fever or hyperventilation, if present, may intensify the
disproportionate loss of water. Occasionally, hypertonic dehydration
Hypotonic dehydration
may be caused by excessive solute intake. Urinary excretion of excess
ECF solute obligates loss of large volumes of water, resulting in dehydra-
ICF
+
tion. The history may reveal that the child was accidentally fed a
Na
high sodium solution because of incorrect mixing of oral rehydration
K+
packets or concentrated formula. In hypertonic dehydration, shift of
H2O
fluid from the ICF to the ECF occurs to attain osmotic balance. As
such, the ECF volume is somewhat spared at the expense of the ICF,
and signs of dehydration may be delayed. However, fluid loss from
Hypertonic dehydration
the ICF results in intracellular dehydration, the most serious effect
ECF
ICF
of which can occur in the brain. If hypernatremia occurs rapidly, not
+ only does a decrease in brain size occur, but a fall in cerebrospinal
Na
fluid pressure occurs as well resulting from diffusion of water from
K+
cerebrospinal fluid to the blood. As the brain shrinks, the bridging
H2O H2O
veins within the skull may stretch and even tear, resulting in intra-
cranial hemorrhage or other complications. If the hypertonic state
manifests more slowly, brain cell size may initially shrink minimally
Figure 80.1. Pathophysiology of various types of dehydration. Top, Isotonic/
but will gradually return to normal size even with continued hyper-
isonatremic dehydration. Middle, Hypotonic dehydration. Bottom, Hypertonic
natremia. Preservation of the brain cell volume despite hypernatre-
dehydration. Abbreviations: ECF, extracellular fluid; H2O, water; ICF, intracellular
fluid; K+, potassium; Na+, sodium.
mia is thought to be caused by the generation of idiogenic osmoles
(eg, myoinositol, trimethylamines, taurine, and other amino acids)
that prevent water loss and attract water back into the cell and thus
process; the total water deficit in dehydration comes primarily maintain cell volume. Rehydration of the patient with hypernatre-
from the ECF, although some investigators have suggested, in the mia must occur slowly and cautiously to avoid brain cell swelling
absence of valid data, that two-thirds of the losses come from ECF (Figure 80.2). In the child with hypertonic dehydration, on clini-
and one-third from ICF. cal examination the skin may sometimes feel “doughy” because of
A variety of mechanisms exist by which hyponatremia (serum intracellular dehydration. This finding, however, is inconsistent even
Na+ <135 mEq/L) occurs in association with dehydration. In hypo- when evaluation is done by an experienced pediatrician; thus, clini-
tonic or hyponatremic dehydration, the ECF volume is compro- cal examination of the skin should not substitute for serum sodium
mised to a greater degree than in isotonic dehydration because measurements in the diagnosis of hypernatremia.
of osmotic shifts of ECF into the cells, resulting in more severe
signs of dehydration (Figure 80.1, middle). Hypotonic dehydra-
Evaluation
tion typically occurs in children with gastroenteritis in the set-
ting of excessive sodium losses in stool and oral fluids replacement History
with a reduced amount of sodium (ie, water, low-sodium beverages In addition to signs and symptoms of the current illness, the history
[eg, juice, tea]). Furthermore, the kidneys often retain free water should focus on the cause of dehydration. The parent or guardian
(ie, excrete a concentrated urine) despite hyponatremia, because should be questioned about the type and amount of oral intake; the
AVP is stimulated by the decreased effective circulating volume in duration, quality, and frequency of vomiting or diarrhea; whether
such settings. Intravascular volume depletion seems to be a potent blood is present in the stool; the presence or absence of fever; fre-
stimulus for AVP release, overriding the AVP suppressive effect of quency of urination; and whether a recent pre-illness weight is
hypotonicity/hyponatremia. The result is that serum sodium levels known for the child (Box 80.2). Changes of mental status reported
are further decreased because of dilution. Hypotonic dehydration by a parent or guardian is of particular concern because this can
also may occur as the result of excessive loss of sodium (relative to occur as the result of significant electrolyte (eg, sodium) distur-
body water) in stool (eg, cholera) or in the urine (eg, adrenogenital bance, marked dehydration, or other serious infection or illness.
syndrome, cerebral salt wasting, pseudohypoaldosteronism, other The most accurate means of assessing the degree of dehydration is
salt-wasting renal disorders). This is the most dangerous form of to compare current weight with a recent pre-illness weight. In acute

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 Chapter 80: Management of Dehydration in Children: Fluid and Electrolyte Therapy 575

320
mOsm

Brain shrinkage

Gradual correction
n
io
at
dr
hy
de
d
pi
Ra

290 320 290

mOsm mOsm mOsm
Slow dehydration Slow correction

Normal brain volume Normal brain volume Normal brain volume

Rapid correction

n
tio
ec
rr
co
al
du
ra
G

290
mOsm

Brain edema

Figure 80.2. Illustration of the steps in managing hypertonic dehydration showing the effects on brain volume of rapid versus slow development of
hypernatremia and the results of rapid versus slow correction of hypernatremia.

dehydration, weight loss is primarily the result of fluid loss. The the physical examination, it is important to assess for the following
difference between pre-illness and current weight can be used to factors: whether the oral mucosal membranes appear tacky or dry,
determine the degree of the fluid deficit. whether tears are present or absent, if tenting of the skin is present
(ie, tenting remains after the skin is pinched between 2 fingers),
Physical Examination and perfusion status of the extremities. In the process, it is impor-
An important goal of the physical examination of the dehydrated tant that the physician recognize whether shock is present, because
child is to assess the degree of dehydration. In the process, vital this is a life-threatening condition requiring emergent treatment
signs, including blood pressure and a current weight, should be (see Chapter 74).
obtained. Specific attention should be paid to the general appear- If comparison to an accurate recent pre-illness weight is not possi-
ance of the child and, in particular, whether the child is ill-appearing, ble, the physician must rely on vital signs as well as clinical signs and
listless, or less reactive. In addition to the usual components of symptoms to assess the degree of dehydration (Table 80.2). In infants

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 576 PART 5: ACUTE AND EMERGENT PROBLEMS

without other signs of respiratory distress) suggestive of acidosis.

Box 80.2. What to Ask
Generally, the more signs that are present, the greater the severity
Dehydration of dehydration; however, a combination of prolonged capillary refill,
ww Has the child been vomiting and/or having diarrhea? absent tears dry mucosal membranes, and general ill appearance
ww How many stools has the child had, and how large was each stool? may be more diagnostic than tenting and hyperpnea combined in
ww How many times did the child vomit, and how much vomitus occurred identifying the child with more than mild to moderate dehydration.
each time?
ww Does the child have fever? Laboratory Tests
ww What type fluid has the child been drinking? Laboratory tests typically are not indicated in the child who pre­
ww Has the child been urinating? How many wet diapers did the child have sents with mild dehydration. The dehydrated child who is treated with
in a day? IV fluids after a failed attempt at oral rehydration either at home or
ww How much did the child weigh at the last visit to the physician? in the emergency department should undergo initial assessment of
ww Has your child’s behavior or level of alertness changed from normal? serum electrolyte, blood urea nitrogen, and creatinine levels. Initial
and serial measurements of these values also should be performed
during rehydration in the child with shock, severe dehydration, or
and young children (ie, younger than 5 years), the estimated fluid decreased urine output who does not improve after initial restoration
loss for mild dehydration is less than or equal to 5% deficit of body of intravascular volume; with a history and clinical findings incon-
water; moderate dehydration, 6% to 9%; and severe dehydration, sistent with straightforward isotonic dehydration; or who is found
10% to 15%. The corresponding numbers for estimated fluid loss in to have dysnatremia (ie, serum sodium outside the normal range of
children 5 years and older are 3%, 6%, and 9%, respectively. A variety 135–145 mEq/L, whether too low or too high). Dehydration in asso-
of clinical signs have been proposed to evaluate the degree of ciation with dysnatremia can have serious complications, and treat-
dehydration—some more valid and reliable than others. A systematic ment requires special considerations. Hemolytic uremic syndrome,
review found that assessment of capillary refill was the most useful although uncommon, should be considered in any child with gas-
single sign in detecting dehydration of 5% or more. Capillary refill troenteritis, particularly with a history of grossly bloody stool, who
is assessed by placing brief pressure on the distal palmar aspect of a also has decreased urine output.
fingertip and assessing the amount of time for the blanched area to Very ill children may require an arterial blood gas measurement
refill; normal is considered less than or equal to 2 seconds. Two other to more accurately assess their acid-base status; in others, assessing
single signs found to be important in predicting dehydration of 3% serum electrolyte levels is sufficient. The usual acid-base derange-
to 5% or more were abnormal skin turgor (ie, tenting) and respira- ment in the moderately dehydrated child is a non-anion gap acido-
tory disturbance, in particular hyperpnea (ie, deep, rapid breathing sis with decreased serum bicarbonate and hyperchloremia resulting

Table 80.2. Clinical Assessment of Magnitude of Dehydration

Clinical Sign Mild Dehydrationa Moderate Dehydration Severe Dehydrationb
Loss of body weight
<5 years ≤5% 6–9% ≥10%
≥5 years 3% 6% 9%
Skin turgor Normal to slightly reduced Decreased Markedly decreased (ie, tenting)
Skin color and temperature Pale or normala Ashen, cool Mottled, cool
Dry mucous membranes ± a
+ ++
Absent tears ± + ++
Sunken eyeballs ± + ++
Increased pulse ± c
+ c
++ (may be thready)
Blood pressure Normal Normal Reduced (ie, in late shock)
Postural decrease ±
Urine output Normal or reducedc Oliguriac Oliguria, anuria
Capillary refill time Slightly prolonged c
Prolonged c
Prolonged
Abbreviations: ++, certain to occur; +, likely to occur; ±, may occur.
a
In mild dehydration, may be only a history of fluid loss in the form of diarrhea or vomiting without any of the signs of dehydration listed in this table.
b
Often, such patients present with hypovolemic shock, need more intense treatment, and may require additional volume expanders (eg, colloids, blood products).
c
Usually corrects with restoration of intravascular volume.

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 Chapter 80: Management of Dehydration in Children: Fluid and Electrolyte Therapy 577

from bicarbonate losses in the stool. Additionally, the severely 30 minutes and the child admitted to the hospital for further care-
dehydrated child also may exhibit anion gap acidosis resulting from ful evaluation, including assessment for other causes of shock (eg,
lactic acid or ketone accumulation in the peripheral tissues second- sepsis). Rapid restoration of ECF volume with up to 4 boluses of
ary to the decreased perfusion that accompanies hypovolemia. The NS, if necessary, in the first 4 hours is currently recommended. The
exception is in infants with pyloric stenosis, who typically develop physician must take care not to give excessive fluids to a child with
a hypokalemic, hypochloremic metabolic alkalosis. cardiac compromise, because doing so could precipitate congestive
heart failure. Administration of excess fluids also results in decreased
Imaging Studies AVP/antidiuretic hormone levels and the chances of hyponatremia in
Imaging studies, such as chest radiography, abdominal ultrasonog- subsequent therapy even if 0.45% NaCl (0.5 NS) solutions are used
raphy, and computed tomography, are indicated based on the sus- rather than NS to correct the remaining deficit and maintenance
pected etiology of the dehydration. therapy. The validity of rapid restoration of ECF volume with up
to 4 boluses of NS, however, has not been substantiated compared
with the past standard rehydration therapy in which typically only
Management
1 or 2 boluses were used. All ORT fluids generally use hypotonic
Fluid management of the dehydrated child involves consideration containing sodium concentrations of 45 to 75 mEq/L.
of 3 components: normal maintenance, deficit replacement, and the During the second phase of rehydration, the remaining fluid and
ongoing losses of fluid and electrolytes incurred during the present electrolyte deficits are replaced based on the magnitude of these
illness. Most commonly, ongoing losses result from continued vom- losses. These replacements are in addition to the daily maintenance
iting and diarrhea. Losses from diarrhea can be estimated at 10 mL/ requirements as well as any ongoing losses, as discussed previously,
kg per stool and for vomiting at 5 mL/kg per episode. Other forms but must take into consideration the NS boluses administered during
of ongoing losses that occasionally must be considered and replaced the initial phase, which may have already restored a substantial por-
include those associated with burns, gastric secretion suctioned via tion of the total fluid deficit. Each 20 mL/kg fluid bolus corrects 2%
nasogastric tube, hyperventilation, or prolonged fever. The estima- dehydration. Thus, in the child with moderate dehydration use of 3
tion of the child’s fluid and electrolyte needs and losses are almost boluses of 20 mL/kg of NS would correct 6% dehydration, with the
always an approximation and require close follow-up, reassessment, result that the child may no longer have any remaining fluid deficit.
and readjustment throughout treatment. At the very least, monitor- Various protocols exist to restore fluid and electrolyte deficits, and
ing during treatment for dehydration requires regular assessment of approaches to treatment of dehydration vary by institution. Many
vital signs, body weight, intake, and output. of the differences in rehydration strategies lie in the composition of
Fluid given to the dehydrated child may be provided enterally or treatment fluid and the rate at which it is administered. Some phy-
parenterally. Whenever possible, oral replacement therapy using oral sicians prefer to administer one-half of the total fluid needs over the
rehydration solution (ORS) is preferred for the child with mild dehy- first 8 hours and the remainder over the next 16 hours, whereas other
dration and for most children with moderate dehydration. Parenteral physicians prefer to replace the fluid at the same rate over the entire
fluid therapy should be used in the child with more severe dehy- rehydration period. The latter method is presented in the case reso-
dration, in the setting of failure of oral therapy (eg, resulting from lution at the end of the chapter. Usually the fluid deficit is replaced
intractable vomiting or lethargy) despite an adequate trial, in the within 24 hours, although noteworthy exceptions exist. The man-
child in shock or impending shock, or in the child with a suspected agement of dehydration associated with dysnatremia (ie, abnormally
anatomic defect, such as pyloric stenosis or ileus. low or high serum Na+) should entail slower return (12 mEq serum
Na+ change per 24 hours or 0.5 mEq change per hour) to a normal
Parenteral Fluid Therapy range and may require 48 to 72 hours for correction.
The parenteral management of moderate or severe dehydration can Sodium replacement in the child with dehydration depends on
be divided into 2 phases: an initial phase (first 1–2 hours) and the the type of dehydration. In the management of isotonic dehydra-
main phase of rehydration. The aim of the initial phase is to restore tion, some physicians elect to replace the entire fluid deficit with
intravascular volume, thus improving perfusion and renal function NS, whereas others use a saline solution containing 110 mEq Na+/L,
and reversing tissue hypoxia, metabolic acidosis, and increased AVP. and still others use 0.5 NS (77 mEq Na+/L). We recommend NS
Regardless of the type of dehydration (ie, isotonic, hypertonic, hypo- (154 mEq Na+/L) to replace the fluid deficit (see Case Resolution
tonic), NS (0.9% NaCl) at 20 mL/kg per hour generally provides for example). This amount of sodium is somewhat more than
the most rapid and effective means of expanding the intravascu- the actual loss of sodium to the environment, which is closer to
lar volume at acute presentation. If shock is present or imminent, 110 mEq Na+/L, because during isotonic dehydration some sodium
treatment is more aggressive (see Chapter 74). The child should in lost from the ECF is shifted intracellularly to balance potassium
rapid succession receive 2 to 4 boluses of 20 mL/kg of NS given over losses and thus returns to the ECF during rehydration. To calcu-
20 to 30 minutes each. After each bolus, the child should be reas- late the ongoing losses, although the content of excreted body fluids
sessed, and if signs and symptoms of intravascular depletion per- can be analyzed for electrolyte content for more exact replacement,
sist, the next IV bolus of 20 mL/kg of NS should be given over 20 to the diarrheal stools are commonly replaced with 0.5 NS at 10 mL/kg

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 578 PART 5: ACUTE AND EMERGENT PROBLEMS

per stool. (This amount should be adequate in sodium content for (eg, a child who is seizing) or in the setting of serum Na level of less
most patients because diarrhea secondary to rotavirus contains than 120 mEq/L. Hypertonic saline is administered as 3.0 mL/kg
approximately 30–40 mEq Na+/L and enterotoxigenic Escherichia of 3% saline given by IV over 15 to 30 minutes or until seizures
coli, 50–60 mEq/L; however, sodium stool losses in cholera are stop. This volume of 3% saline raises the serum sodium approxi-
90–120 mEq/L.) mately 2.5 mEq/L. Based on a volume of 3.0 mL/kg of 3% saline,
the child should receive volume sufficient to bring up the serum Na
Management of Electrolyte level to above 120 mEq/L, which is considered to be the safe level, at
Disturbances which improvement in serious signs and symptoms are anticipated.
Hypernatremia and Hyponatremia Recalculation is done 4 hours after the initial 3% NaCl infusion to
In hypernatremic/hypertonic dehydration, the patient is considered determine the need for another infusion, if the level is still low or if
to have a relative free water deficit but usually has lost not only body there is no improvement in CNS-related symptoms. After a level of
water but also some sodium. The amount of free water required to greater than 120 mEq/L is achieved or the patient is asymptomatic,
restore serum sodium to normal (eg, 145 mEq/L is desired serum any remaining deficit is corrected more slowly to avoid exceeding an
Na+) is calculated as follows: increase of 12 mEq/L per 24 hours. A too rapid correction of serum
sodium, particularly in the setting of long-standing hyponatremia,
[patient’s weight in kg] × [actual serum Na+ − 145] × 4 mL/kg can potentially cause central pontine demyelination, manifested by
For serum sodium greater than 170 mEq/L, 3 mL/kg of free disorientation and eventual coma.
water is estimated to decrease the sodium to the desired level, As stated previously, hyponatremia in the hospitalized child can
in which case the 4 mL/kg shown in the equation is changed to result from factors other than sodium loss. Arginine vasopressin
3 mL/kg. The quantity of free water provided by this equation release in response to hypovolemia, hypertonicity, or other stimuli
is only part of the patient’s total needs. The remainder of the followed by free water retention can result in dilutional hyponatre-
patient’s fluid needs include isotonic losses that occurred during mia (ie, water intoxication). Hyponatremia in infants given exces-
the dehydration process, ongoing losses, and maintenance fluids sively diluted baby formula results from inadequate sodium intake
as well. Hypertonic dehydration is corrected slowly to avoid cere- and free water retention. Encephalopathy, brain stem herniation,
bral edema, which can result in brain stem herniation and death. and death occurring in hospitalized children with hyponatremia
In hypernatremia, the various equations used for phase 2 of ther- have been reported. The adverse effects of hyponatremia on the CNS
apy often calculate the sodium concentration of the final solution are accentuated in the setting of hypoxemia. With rehydration and
considering the amount of free water to achieve isotonicity. We rec- sodium administration, kidneys excrete relatively more dilute urine
ommend initially providing 0.9% NaCl in 5% dextrose (a higher that can sometimes result in rapid and unpredictable increases in
content of sodium than calculated by various equations) to ensure a serum sodium levels, necessitating close monitoring of serum elec-
slow rate of serum sodium decline and later decreasing the sodium trolyte levels. Consultation with a pediatric nephrologist or pedi-
concentration to 0.45% NaCl if the serum Na level remains high atric intensivist experienced in managing alterations in fluid and
24 to 48 hours after this treatment is begun. Serial monitoring of electrolyte balance is helpful.
electrolytes at least every 6 hours and as necessary is important to
ensure that the sodium level is decreasing at the expected slow rate Potassium Replacement
and is not decreasing so quickly as to result in life-threatening CNS Potassium deficits are more difficult to determine, and no spe-
complications. cific method exists for calculating the exact amount of potas-
Management of hyponatremia/hypotonic dehydration also poses sium required by a dehydrated child. Additionally, as the acidosis
challenges. In addition to isotonic losses, additional sodium loss may that commonly accompanies moderate and severe dehydration
have occurred. The amount of additional sodium (in mEq) to cor- corrects, potassium shifts intracellularly. What initially seems
rect the serum sodium into a normal range (desired Na+ level [eg, to be a normal serum potassium may fall into the hypokalemic
135 mEq/L]) historically has been calculated using the following equa- zone, potentially resulting in adverse effects on neuromuscular
tion (where 0.6 represents the body space affected by Na+ changes): and cardiac function. Frequent reassessment of serum potassium
and adjustment of potassium content of the IV fluids may be
[patient’s weight in kg] × [135 − actual Na+ level] × 0.6
necessary. Generally, after adequate urine output has been estab-
This amount of sodium represents an additional need beyond a lished, potassium may be added to the IV fluids to provide 3 to
patient’s isotonic losses, ongoing losses, and maintenance require- 4 mEq/kg per 24 hours. Usually, this need can be met by add-
ments. Although precise calculations of sodium requirement to ing potassium chloride 20 mEq/L to the IV fluids. The child with
manage hypotonic dehydration may be desirable, in most patients decreased urine output or another indicator of renal impairment
with hyponatremia treatment with NS (0.9% NaCl) in 5% dex- should not receive potassium until normal urine output has been
trose is adequate for gradual correction of the hyponatremia. The restored. Hyperkalemia, which is a serious and life-threatening
use of hypertonic saline (3% containing 513 mEq Na+/L) is gen- condition, may occur if a child is unable to excrete excess potas-
erally reserved for the child with symptomatic hyponatremia sium via the kidney because of renal impairment.

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 Chapter 80: Management of Dehydration in Children: Fluid and Electrolyte Therapy 579

Oral Rehydration Rice-based oral electrolyte solutions contain rice syrup solids as
Oral rehydration therapy refers to specially prepared, balanced their source of carbohydrates. Electrolyte solutions with rice syrup
preparations of carbohydrates and electrolytes meant for oral solids may reduce stool output as well as replete fluid volume. It is
consumption. Clinical trials have repeatedly shown ORT to be as not necessary to change to ORT in a breastfed child who is toler-
efficacious as IV therapy in the treatment of the child with mild ating human milk; these children can continue to receive human
or moderate dehydration. Additional advantages of ORT over IV milk for rehydration, although they may require shorter, more fre-
therapy are that it costs less, is noninvasive, and requires little quent feedings.
technology. Oral rehydration therapy has been credited with the The composition of various ORSs is presented in Table 80.3. The
dramatic reduction in death associated with diarrhea in the devel- cost of commercially available ORS may be prohibitive for some
oping world. In 2002, the World Health Organization and United families. Given the simplicity of the ORS packet in the developing
Nations Children’s Fund announced a new ORS with reduced world and commercially available ORS in the developed world, these
osmolarity (proportionally lower Na+ and glucose concentration) remain the first choice. Some solutions, such as fruit juices, ORSs,
based on several clinical studies demonstrating less vomiting, or chicken broth, do not contain the proper balance of sodium and
lower stool output, and reduced need for IV fluids relative to the carbohydrate to effectively rehydrate a dehydrated patient; however,
prior formulation. these can be used at home for mild cases of diarrhea if the patient
In the United States, Pedialyte and generic equivalents are the still tolerates oral fluids.
most widely commercially available products. Flavored solutions The amount of ORT fluid necessary for rehydration can be cal-
and freezer pop preparations of these solutions are available and culated in much the same fashion as for determining the parenteral
often are preferred by older children over the unflavored variety. fluid requirement for a dehydrated child (Table 80.4). However,

Table 80.3. Composition of Various Oral Rehydration Solutions

Solution Carbohydrate (gm/L) Sodium (mEq/L) Potassium (mEq/L) Base (mmol/L) Osmolarity (mOsm/kg H2O)
Appropriate for Rehydration
Pedialyte 25 45 20 30 250
Enfalyte 30 50 25 33 160
World Health Organization–UNICEF 13.5 70 20 30 245
oral rehydration solution (2002)
Not Appropriate for Rehydration
Cola a
110 2 0 13 750
Apple juice a
120 3 32 0 730
Chicken brotha 0 250 8 0 500
Sports beverages 40 20 3 3 330
Abbreviation: UNICEF, United Nations Children’s Fund.
a
These fluids should be avoided because they have very high osmolality that can worsen diarrhea.

Table 80.4 Guidelines for Administration of Oral Solutions to Replace Deficit Over 4 Hours
Mild Dehydration (3%–5%) Moderate Dehydration (6%–9%)
Weight (kg) Total Volume Over 4 Hours Volume per Administration Total Volume Over 4 Hours Volume per Administration
5 150–250 mL 5 mL every 5–8 min 300–450 mL 6–9 mL every 5 min
10 300–500 mL 6–10 mL every 5 min 600–900 mL 12–18 mL every 5 min
15 450–750 mL 10–15 mL every 5 min 900–1,350 mL 18–28 mL every 5 min
20 600–1,000 mL 12–20 mL every 5 min 1,200–1,800 mL 25–37 mL every 5 min
25 750–1,250 mL 15–25 mL every 5 min 1,500–2,250 mL 30–45 mL every 5 min
30 900–1,500 mL 18–30 mL every 5 min 1,800–2,700 mL 37–55 mL every 5 min
40 1,200–2,000 mL 25–40 mL every 5 min 2,400–3,600 mL 50–75 mL every 5 min
Reprinted with permission from Powers KS. Dehydration: isonatremic, hyponatremic, and hypernatremic recognition and management. Pediatr Rev. 2015;36(7):274–285.

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 580 PART 5: ACUTE AND EMERGENT PROBLEMS

Table 80.5. Treatment With Oral Rehydration Therapy

Dehydration ORT (Given Over
Degree 3–4 hours) Replacement of Losses Dietary Therapy
Mild (5%) (≥13 years = 3%) 50 mL/kg of ORS 10 mL/kg for each diarrheal stool Return to formula or milk as soon as vomiting
5 mL/kg for each vomitus resolves. Children who eat solid food can con-
tinue their regular diet.
Moderate (10%) (≥13 years = 6%) 100 mL/kg of ORS 10 mL/kg for each diarrheal stool Return to formula or milk as soon as vomiting
5 mL/kg for each vomitus resolves. Children who eat solid food can con-
tinue their regular diet.
Abbreviations: ORS, oral rehydration solution; ORT, oral rehydration therapy.

most children with mild dehydration can be rehydrated relatively

quickly and, in response to thirst, are likely to request the amount CASE RESOLUTION
of fluid they need. Therefore, a simplified method of determin- The child has moderate dehydration. Based on clinical assessment and weight
ing ORT fluid requirements has been developed (Table 80.5). This change since the boy’s last clinic visit, he is approximately 10% dehydrated. He
quantity of fluid is given over a period of 3 to 4 hours. In the does not show evidence of shock. His laboratory studies show a sodium level
child with ongoing losses, these losses should be replaced as well. of 140 mEq/L, potassium of 3.7 mEq/L, chloride of 112 mEq/L, bicarbonate of
13 mEq/L, blood urea nitrogen of 13 mg/dL, and creatinine of 0.4 mg/dL. His
Typically, it is not necessary to calculate the quantity of electro-
renal status is likely to be adequate because he is urinating, and blood urea nitro-
lytes that should be provided because these solutions are designed gen and creatinine are normal for the patient’s age. The child’s serum sodium is
to adequately replace electrolytes in the otherwise healthy child 140 mEq/L, which is in the isotonic range. The serum potassium is 3.7 mEq/L,
who is dehydrated. which is within normal range; however, this level may not accurately reflect this
The parent or guardian should receive guidance about the patient’s total body potassium status. The level may decrease substantially as he
is rehydrated and acidosis is corrected, indicating total body potassium depletion.
volume (converted into common household measures [eg, 5 mL =
His serum bicarbonate is 13 mEq/L, and his anion gap is 15 [140 – (112 + 13)],
1 teaspoon and 15 mL = 1 tablespoon of water or ORS]) as well which is mildly increased and likely related to ketosis or mild lactic acidosis.
as the frequency and duration of ORT to be given at home. Small The calculation of this child’s fluid and electrolyte needs is as follows, keep-
volumes of 5 to 15 mL administered with a syringe or teaspoon ing in mind that his pre-illness weight was 12 kg:
every 2 to 5 minutes are much more likely to be retained by the Maintenance
child who vomits larger volumes. Although this technique is Fluid requirement: 1,000 mL for first 10 kg + 100 mL for next 2 kg = 1,100 mL.
labor intensive, it can be done by the parent or guardian and can Deficit
deliver 150 to 300 mL/hour. As dehydration is corrected, vomit- Fluid replacement: 10% of the child’s weight has been lost during this episode
ing often decreases and the child subsequently can tolerate larger of dehydration = 1,200 mL deficit.
volumes. With ORT, the frequency and amount of passing stool Ongoing Losses
often increases during the initial period of treatment. The parent Additional Fluid: Estimate this child’s ongoing losses at 10 mL/kg for each stool.
or guardian should be made aware that the primary purpose of He had 1 loose stool while in the office, so 120 mL of additional fluid is added.
ORT is to rehydrate the child, not to stop diarrhea, and that diar- Sodium: The sodium content of diarrhea is variable; however, it is usually
replaced with 0.9% NS.
rhea will gradually decrease spontaneously. “Gut rest” (limiting
Total fluid needs: 1,100 mL (maintenance) + 1,200 mL (deficit) + 120 mL
oral intake) is not appropriate in most cases, and early refeeding (ongoing losses) = 2,420 mL/24 hours.
with a return to the usual formula or milk and solids, if appro-
Electrolyte Needs
priate, should be prioritized.
Sodium: 0.9% NS is used based on current recommendation.
Potassium: Estimate the child’s maintenance and replacement needs to be
20 mEq K+/1,000 mL fluid provided; this estimate can be modified based on
Prognosis follow-up laboratory values, if necessary.
The child with mild or moderate dehydration resulting from a self- Treatment
limited childhood illness is likely to recover completely when given In the initial phase of therapy, provide NS 40 mL/kg per hour for approximately
timely and appropriate rehydration therapy. It is more difficult to 2 hours. During this period, the patient’s heart rate normalizes and he urinates.
predict the prognosis of the child with severe dehydration or sig- The initial parenteral phase provides 480 mL fluid as NS (0.9% NaCl). This
amount of fluid is subtracted from the patient’s total fluid needs. The remaining
nificant aberration in electrolyte balance. If managed appropriately,
amount to be provided is 1,940 mL fluid. It is not necessary to prepare a special
most such children also completely recover; however, despite closely IV solution; 5% dextrose in 0.9% NS with 20 mEq/L KCl to run at 80 mL per hour
monitored care, some children may experience permanent sequelae is appropriate. As his GI symptoms improve, IV therapy is discontinued and ORT is
or poor outcomes. instituted. The patient tolerates the ORT well and is discharged home.

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 Chapter 80: Management of Dehydration in Children: Fluid and Electrolyte Therapy 581

Selected References Holliday MA, Ray PE, Friedman AL. Fluid therapy for children: facts, fashions
and questions. Arch Dis Child. 2007;92(6):546–550 PMID: 17175577 https://doi.
Denno D. Global child health. Pediatr Rev. 2011;32(2):e25–e38 PMID: 21285299 org/10.1136/adc.2006.106377
https://doi.org/10.1542/pir.32-2-e25 Holliday MA, Segar WE. The maintenance need for water in parenteral fluid
Feld LG, Neuspiel DR, Foster BA, et al; American Academy of Pediatrics therapy. Pediatrics. 1957;19(5):823–832 PMID: 13431307
Subcommittee on Fluid and Electrolyte Therapy. Clinical practice guideline: Moritz ML, Ayus JC. New aspects in the pathogenesis, prevention, and treatment
maintenance intravenous fluids in children. Pediatrics. 2018;142(6):e20183083 of hyponatremic encephalopathy in children. Pediatr Nephrol. 2010;25(7):1225–
PMID: 30478247 https://doi.org/10.1542/peds.2018-3083 1238 PMID: 19894066 https://doi.org/10.1007/s00467-009-1323-6
Fischer TK, Viboud C, Parashar U, et al. Hospitalizations and deaths from Powers KS. Dehydration: isonatremic, hyponatremic, and hypernatremic rec-
diarrhea and rotavirus among children <5 years of age in the United States, ognition and management. Pediatr Rev. 2015;36(7):274–285 PMID: 26133303
1993-2003. J Infect Dis. 2007;195(8):1117–1125 PMID: 17357047 https://doi. https://doi.org/10.1542/pir.36-7-274
org/10.1086/512863
Rose BD, Post TW. Clinical Physiology of Acid-Base and Electrolyte Disorders.
Freedman SB, Parkin PC, Willan AR, Schuh S. Rapid versus standard intrave- 5th ed. New York, NY: McGraw Hill; 2001
nous rehydration in pediatric gastroenteritis: pragmatic blinded randomised
Vega RM, Avner JR. A prospective study of the usefulness of clinical and
clinical trial. BMJ. 2011;343:d6976
laboratory parameters for predicting percentage of dehydration in chil-
Friedman AL. Pediatric hydration therapy: historical review and a new dren. Pediatr Emerg Care. 1997;13(3):179–182 PMID: 9220501 https://doi.
approach. Kidney Int. 2005;67(1):380–388 PMID: 15610273 https://doi. org/10.1097/00006565-199706000-00001
org/10.1111/j.1523-1755.2005.00092.x
Winters RW, ed. The Body Fluids in Pediatrics. Boston, MA: Little, Brown and
Holliday M. The evolution of therapy for dehydration: should deficit therapy still Co; 1973
be taught? Pediatrics. 1996;98:171–177 PMID: 8692613

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 CHAPTER 81

Acute Kidney Injury

Gangadarshni Chandramohan, MD, MSc, FASN, FAAP

CASE STUDY
A 10-month-old girl has a 2-day history of fever, vomit- an appropriately sized cuff. Capillary refill is 2 to 3 seconds.
ing, and watery diarrhea. The child has previously been The skin appears dry, but no rash is present. Head and
healthy. Her diet has consisted of infant formula forti- neck, chest, heart, and abdominal examinations are nor-
fied with iron, baby food, and some table food. Since the mal. Pending the results of her blood studies, an intra-
onset of her illness, she has not been drinking or eating venous fluid bolus of 180 mL normal saline (20 mL/kg)
well, and she has thrown up most of what she has eaten. over 20 to 30 minutes is administered. This is followed
Her mother has tried to give her oral electrolyte solution by 2 more boluses of 180 mL normal saline each. The
and apple juice on several occasions but has had limited girl is catheterized to obtain urine and determine the
success. The child has had 8 to 10 watery stools without urine flow rate over the next several hours. A urinalysis
blood or mucus each day. Her temperature has varied is performed.
between 37.0°C (98.6°F) and 38.8°C (101.8°F); the mother
has given her daughter acetaminophen, which she has
Questions
1. What are the 3 stages of acute kidney injury?
vomited up. The girl’s 4-year-old brother and her parents
2. What is the etiology of acute kidney injury?
are doing well and have no vomiting or diarrhea.
3. How would the physician assess a patient with acute
The physical examination reveals a severely dehy-
kidney injury?
drated (estimated amount 15%), listless infant. Her
4. How would the physician manage a child with acute
weight is 9.4 kg (20.7 lb), her height is 74 cm (29.1 in),
kidney injury?
her temperature is 38.4°C (101.1°F), her heart rate is
5. What are the indications for renal replacement
168 beats per minute, her respiratory rate is 30 breaths
therapy?
per minute, and her blood pressure is 72/40 mm Hg with

Acute kidney injury (AKI) is encountered in outpatient and inpatient output may remain normal or even increase. Decline in urine out-
settings and is associated with a high rate of morbidity and mortal- put is an essential component of risk assessment when determining
ity depending on the primary cause of the insult. Acute kidney injury severity of AKI in the pediatric patient because of the known asso-
is defined as a sudden decrease in kidney function, signified by the ciation between the duration of oliguria and increased mortality.
accumulation of nitrogenous waste products (ie, blood urea nitrogen The pRIFLE criteria are not applicable to newborns during the
[BUN] and various other metabolic waste products) and impaired first few days after birth, however, because they may exhibit phys-
balance of fluid and electrolytes. With a better understanding of the iological oliguria during the first 24 hours after birth and their
pathophysiology of acute deterioration in renal function, the term serum creatinine level initially reflects maternal creatinine values.
AKI delineates the process of renal injury and encompasses the full Therefore, the pRIFLE criteria were further modified for neonates
spectrum of renal dysfunction, from early, mild renal injury with only based on findings from the Assessment of Worldwide Acute Kidney
a small elevation in serum creatinine level, to severe kidney injury Injury Epidemiology in Neonates (AWAKEN) study, which set the
requiring renal replacement therapy (ie, dialysis) as a continuum. baseline creatinine level as the lowest level based on gestational age
To better delineate the progression of AKI, in 2004 the Acute and set the serum creatinine threshold for stage 3 AKI at greater
Kidney Injury Network Acute Dialysis Quality Initiative workgroup than or equal to 2.5 mg/dL rather than greater than 4 mg/dL.
set forth the RIFLE criteria, which is based on serum creatinine level The pRIFLE classification is intended to emphasize the reversible
and urine output. The acronym RIFLE defines 3 stages of progres- nature of the renal insult, which often is present in critically ill chil-
sively increasing severity of renal injury (risk, injury, and failure) dren admitted to pediatric or neonatal intensive care units (ICUs). It
followed by 2 outcomes variables (loss and end-stage renal disease). is anticipated that this precise and universal definition of AKI likely
These criteria, proposed in 2004 and validated in 2012 by the Kidney will enable physicians to rapidly recognize at-risk individuals and
Disease: Improving Global Outcomes (KDIEGO) AKI workgroup, intervene promptly to improve immediate and long-term outcomes.
modified RIFLE for the pediatric population (pRIFLE) and its clinical The major limitation to the use of this classification is that it is not
use has been shown to improve outcomes in children (Figure 81.1). validated in children who present with AKI in an outpatient setting,
This classification is based in part on declining urine output; however, whose etiology and outcomes often are dissimilar to those of inpa-
some children experience nonoliguric renal failure, in which urine tient, acutely ill children.
583

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 584 PART 5: ACUTE AND EMERGENT PROBLEMS

may be the first indication of AKI. However, decreased urine out-

Risk
• eCrCl decrease by 25%
put, edema, hematuria, and/or hypertension may be the early sign
R
• Urine output <0.5 mL/kg/hour for 8 hours or signs specifically related to declining renal function (Box 81.1).
Injury
Additionally, hyperkalemia with cardiac arrhythmia, hyperventi-
• eCrCl decrease by 50%
• Urine output <0.05 mL/kg/hour for 16 hours
lation resulting from acidosis, and nausea and vomiting resulting
I
from uremia may occur with progression of renal failure.
Failure
• eCrCl decrease by 75% or eCrCl <35 mL/min/1.73 m2
• Urine output <0.3 mL/kg/hour for 24 hours or anuria for 12 hours Etiology and Pathophysiology
F
Because of the recent epidemiologic shift from renal to nonrenal causes
Loss
• Persistent failure for >4 weeks as common etiologic factors, for every child the primary care physi-
L cian as well as the neonatal and pediatric intensivist should perform a
End-stage renal disease risk assessment using the pRIFLE criteria and take the necessary steps
• Persistent failure for >3 months
E to prevent AKI before the kidneys sustain serious injury. Frequently,
the cause is multifactorial, and ischemic-hypoxic injury and nephro-
toxic insults are important contributors; the pathophysiology of hypoxic
Figure 81.1. Pediatric risk for renal dysfunction, injury to the kidney, failure of ischemic injury and nephrotoxic insults is described herein.
kidney function, loss of kidney function, and end-stage renal disease (pRIFLE)
The causes of AKI are usually grouped into 3 categories:
criteria to assess the stage of renal injury.
prerenal, postrenal, and intrinsic renal disorders (Box 81.2 and
Abbreviations: eCrCl, estimated creatinine clearance.
Figure 81.2). Although prerenal failure and acute tubular necro-
Derived from Bellomo R, Ronco C, Kellum JA , Mehta RL, Palevsky P; ADQI Workgroup. Acute renal
failure–definition, outcome measures, animal models, fluid therapy and information technology sis (ie, intrinsic renal failure) may in fact be on opposite ends of a
needs: the Second International Consensus Conference of the Acute Dialysis Quality Initiative continuum, rather than separate entities, this classification system
(ADQI) Group. Crit Care. 2004;8(4):R204–R212 PMID: 15312219 https://doi.org/10.1186/cc2872. still aids in the conceptualization of the underlying problem and
formulation of the initial treatment plan. Correspondingly, with-
out timely intervention postrenal failure has the potential to cause
Epidemiology renal damage and ultimately can result in intrinsic renal failure.
Acute kidney injury occurs in children with a wide variety of Prerenal Disorders
medical or surgical conditions and especially in children who are
Prerenal disorders are the most common cause of AKI in pediat-
critically ill. The exact incidence of AKI in children is unknown
ric patients and result in a decrease in total or effective circulating
because it not plausible to capture all cases of AKI, particularly in
blood volume. An absolute decrease in circulating volume can be
children who present to outpatient settings. The prevalence of AKI
caused by blood loss from acute hemorrhage secondary to trauma
varies depending on geographic and demographic factors, which are
or fluid loss and dehydration secondary to gastroenteritis. Heart fail-
responsible for the etiology of AKI. In developed countries, AKI has
ure or redistribution of body fluids (ie, third spacing) can result in
been increasing over the past few decades because of the increasing
decreased effective circulating volume. In each of these situations,
number of cardiopulmonary bypass surgeries performed to manage
the resulting decrease in the glomerular filtration rate (GFR) can be
congenital heart disease, an increase in the number of solid organ
readily reversed by improving renal perfusion in its early stages. If
and bone marrow transplantations, and the use of newly discovered
hypoperfusion is prolonged, however, ischemic damage to the kid-
nephrotoxic agents to manage a variety of disorders. Acute kidney
ney occurs and intrinsic renal failure occurs.
injury resulting from primary renal disease appears to be on the
decline compared with AKI caused by other systemic illnesses or Postrenal Disorders
their treatments. Acute kidney injury is often observed in patients
In infancy and early childhood, urinary obstruction caused by
in the ICU with sepsis or major trauma with severe bleeding or in
posterior or anterior urethral valves or other congenital lesions
the postoperative period after major heart surgery. According to
involving the urinary tract can result in AKI. In the older child, kidney
Assessment of Worldwide Acute Kidney Injury, Renal Angina and
Epidemiology (AWARE) data published in 2016, of 4,683 critically
ill children, 26.9% developed AKI and 11.6% had severe AKI (stage 2 Box 81.1. Diagnosis of Acute Kidney Injury
and 3). Approximate 5% to 10% of patients in neonatal ICUs develop
ww Decreased urine output
AKI, and most commonly as the result of perinatal hypoxia and
ww Hypertension
postnatal hypotension.
ww Hematuria
ww Edema
Clinical Presentation
ww Elevated serum creatinine level
Most children with AKI initially present with clinical findings of ww Blood urea nitrogen-creatinine ratio <20
the primary condition that ultimately results in the renal prob- ww Elevated fractional excretion of sodium
lem. In the critically ill child, a small increase in serum creatinine

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 Chapter 81: Acute Kidney Injury 585

Box 81.2. Etiology of Acute Kidney

Injury in Children
Glomerular
diseases
Prerenal Disorders Congenital Rhabdo-
ww Decreased plasma volume genitourinary myolysis
anomalies
ww Dehydration
ww Hemorrhage
ww Third spacing of plasma volume in the setting of burns, sepsis, bowel Shock/
reduced Exogenous
obstruction extracellular toxins
ww Other causes of renal hypoperfusion volume Acute kidney
injury
ww Shock
ww Hypoxia
Reduced
ww Congestive heart failure effective Drug
ww Hepatorenal syndrome circulatory toxicity
volume
ww Bilateral renal artery stenosis
Impaired
ww Cardiac surgery renal
Sepsis/SIRS
Postrenal Disorders microcir-
culation
ww Bilateral ureteropelvic or ureterovesical junction obstruction
ww Posterior urethral valves
ww Trauma to urethra Figure 81.2. Common causes of acute kidney injury in children.
ww Urethral stricture Abbreviation: SIRS, systemic inflammatory response syndrome.

ww Neurogenic bladder
ww Obstruction caused by kidney stone at the bladder neck or stone obstruc-
cells, detachment of epithelial cells from basement membrane, and
tion of both urinary tracts
cast formation from cellular debris and protein.
Intrinsic Renal Disorders The child with prolonged shock that manifests postcardiac
ww Vascular: renal artery or vein thrombosis, disseminated intravascular surgery or that is caused by sepsis, trauma (ie, hemorrhage), or
coagulation. dehydration often develops ATN if effective circulating volume is
ww Glomerular: hemolytic uremic syndrome, severe (ie, rapidly progressive) not reestablished. This is the most frequent type of intrinsic AKI
glomerulonephritis from any etiology. observed in children. Nonsteroidal anti-inflammatory drugs are
ww Interstitial: interstitial nephritis resulting from allergic reaction to drugs increasingly being recognized as a cause of AKI in children, espe-
(eg, nonsteroidal anti-inflammatory drugs, oxacillin, methicillin), sepsis. cially when used in patients with volume depletion. In the newborn
ww Tubular (acute tubular necrosis): sepsis, postcardiac surgery, ischemia or young infant, AKI may be superimposed on existing chronic con-
resulting from prolonged hypoperfusion; all causes listed in prerenal cat- genital renal disease. In neonates, the prevalence of AKI ranges from
egory, if sufficiently prolonged, may lead to acute tubular necrosis. 8% to 24% and is higher in neonates with severe asphyxia than in
ww Nephrotoxins: aminoglycoside antibiotics, indomethacin, radiocontrast those with moderate asphyxia.
agents, ethylene glycol, methanol, heavy metals. The pathogenesis of ATN in humans is controversial, and no
ww Pigments: myoglobinuria, hemoglobinuria. single mechanism completely explains the sequence of events that
ww Uric acid: hyperuricemia, tumor lysis syndrome. results in ATN. Ischemic and toxic ATN result from a complex inter-
ww Congenital renal anomalies (especially in newborns and young infants). play of hemodynamic, vascular, and tubulointerstitial changes,
ww Bilateral cystic dysplastic kidneys, reflux nephropathy, polycystic kid- including decreased blood flow to glomerular and tubular capil-
neys, oligomeganephronia. laries, resulting in reduced GFR; injury to cortical and medullary
tubules with their cellular debris, resulting in tubular obstruction;
and “back leak” of solute and water from the lumen to the inter-
stones, pelvic trauma, or complications following pelvic surgery are stitium, with further reduction in GFR. Increased production of
possible causes of postrenal failure. endothelin and reduced production of nitrous oxide in the micro-
vascular smooth muscle cells result in increased vasoconstriction
Intrinsic Disorders and reduced perfusion, thereby perpetuating the renal injury. Renal
Intrinsic renal failure occurs because of injury to the vascular, tubular cells respond to the injury in many different ways, including
glomerular, interstitial, or tubular components of the kidney no or minimal damage, sublethal injury, apoptosis, and necrosis. In
(see Box 81.2). Intrinsic AKI can result from infection, ischemia, the tubules, at the cellular level decreased oxygen delivery results
sepsis, or toxins. Acute kidney injury resulting from renal tubular in decreased production of adenosine triphosphate, which causes
lesions is called acute tubular necrosis (ATN). Histologic changes that damage to cell membranes and cell cytoskeletons. Cell damage
characterize ATN include loss of brush border microvilli in tubular alters cell polarity, thereby promoting entry of increased amounts

BPPCA6e_Ch081_0583-0590.indd 585 2/13/20 6:28 PM

 586 PART 5: ACUTE AND EMERGENT PROBLEMS

of calcium into cells and increased intracellular free-radical forma-

Box 81.3. What to Ask
tion. This in turn results in altered cell function, cell swelling, and
apoptosis and cell death. Acute Kidney Injury
Certain intrinsic proteins also influence the onset of AKI, however. ww How frequently is the child passing urine?
Low serum levels of bone morphogenetic protein 7, which is an anti- ww Is the amount of daily urine decreased, increased, or unchanged?
fibrotic, anti-inflammatory, and antiapoptotic factor that belongs to ww Does the child have hematuria or dysuria?
the transforming growth factor-b superfamily of ligands, is thought to ww Does the child have nausea or vomiting?
play a role in the pathogenesis of postcardiac surgery–associated AKI. ww Has the child had any previous urinary problems?
Acute kidney injury occurs more commonly in very low-birth-weight ww For the older child: Does the child have a history of enuresis or nocturia?
neonates carrying the heat shock protein 72 (1267) GG genetic ww Is the child’s physical development normal or delayed?
variation, which is associated with low inducibility of heat shock pro- ww Does the child have a history consistent with a primary condition that
tein 72, which itself plays an important role in ische­mic renal injury. may have resulted in the acute renal failure/acute kidney injury?
This suggests that some neonates are more susceptible to ischemic
injury than others. It is hoped that with better understanding of the
pathophysiology of AKI it will be possible to develop innovative, kidney injury, differentiate among multiple etiologies, and predict
improved means of preventing, diagnosing, and managing the disease. its severity. Many promising urinary biomarkers exist, includ-
ing urinary or plasma neutrophil gelatinase-associated lipocalin,
Differential Diagnosis kidney injury molecule-1, interleukin-18, liver-type fatty acid
The diagnosis of AKI is established by the demonstration of a sudden binding protein, markers of cell-cycle arrest (eg, tissue inhibitor
increase in serum creatinine or BUN level. Decreased urine output of metalloproteinases-2), and insulin-like growth factor-binding
is another helpful diagnostic factor. Identification of the clinical dis- protein 7, which have been identified to correlate with AKI to
order that resulted in AKI is sometimes obvious, but at other times variable degrees. Both neutrophil gelatinase-associated lipocalin
extensive evaluation may be necessary to discover the etiology of and tissue inhibitor of metalloproteinases-1 have been shown to
the primary disorder. It is also necessary to determine whether the correlate significantly with AKI in children in ICUs with various
child has chronic kidney disease or is experiencing superimposition types of predisposing conditions. Recently, consideration has been
of AKI on a preexisting renal condition (see Figure 81.2). given to the potential for assessing multiple biomarkers to deter-
mine changes in kidneys during the early phase of AKI. Studies ana-
Evaluation lyzing the value of this tool are ongoing. In parallel, ongoing studies
are assessing the value of screening programs, such as Nephrotoxic
Acute kidney injury in children is among the few conditions for Injury Negated by Just-in-Time Action (NINJA) and renal angina
which laboratory and radiologic tests are often more helpful diag- index, as a simplified alternative to already existing biomarkers. It
nostically than the history and physical examination. is anticipated that in future this information will help physicians
History recognize at-risk individuals and intervene early to prevent pro-
gressive and permanent renal injuries.
The possibility for AKI should be anticipated in every child who is
Box 81.4 shows a list of laboratory tests recommended in all
critically ill. A history of decreasing urine output, hematuria, dysuria,
children with established or suspected AKI. If the child is not
nausea, and vomiting should be sought in all patients. Prenatal and
voiding frequently, temporary catheterization of the bladder is advis-
birth history may help identify the cause of AKI, such as oligomeg­
able (4–6 hours) to obtain urine for analysis. Residual volume should
anephronia in the child who was small for gestational age at birth
also be assessed, and the urinary flow rate (especially the response to
or another complication that would have resulted in AKI. The pres-
initial fluid therapy) and presence of an outflow obstruction should
ence of previous genitourinary disorders, delayed growth, and ane-
mia may point to preexisting kidney conditions (Box 81.3). Family
history of renal disorders can also aid in the differential diagnosis.
Box 81.4. Laboratory Tests Recommended
Physical Examination for Children with Established or Suspected
An evaluation of physical growth, hypotension or hypertension, Acute Kidney Injury
arrhythmia, dehydration, and edema should be made. Examination ww Serum sodium, potassium, and bicarbonate levels
of the flank area for renal enlargement or tenderness and of the blad- ww Blood urea nitrogen level
der for distention is necessary to help determine the etiology of AKI. ww Creatinine, uric acid, calcium, and phosphorus levels
ww Glucose level
Laboratory Tests ww Total protein and albumin serum concentration
Many biomarkers have been investigated for the early diagnosis ww Urinalysis and urine culture (if indicated)
of AKI in adults and children. The hope is that biomarkers found ww Spot urinary sodium and creatinine concentration and osmolality
in the serum or urine will allow physicians to better detect early

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 Chapter 81: Acute Kidney Injury 587

be determined. All children with AKI and evidence of hyperkalemia and should be avoided. Failure to respond to fluid and diuretic
should undergo electrocardiography (ECG). therapy is suggestive of intrinsic AKI.
Diagnosis of AKI can easily be established by laboratory tests
and determination of urinary output over a specific time. Oliguria Imaging Studies
is defined as urine output less than 400 mL/m2 per day or less than Renal ultrasonography is the most useful test for differentiating
1 mL/kg per hour in infants 1 year and younger, less than 0.75 mL/kg postrenal from other forms of AKI. Renal ultrasonography can detect
per hour in the child age 2 to 6 years, and less than 0.5 mL/kg per the presence or absence of kidneys, enlarged kidneys, dilated pyelo-
hour in children older than 6 years. Urinalysis, urine-specific gravity calyceal system, distended bladder, and other congenital anomalies.
or osmolality, urine-plasma creatinine ratio, urinary sodium concen- Other investigative tests, such as voiding cystourethrography, renal
tration, and fractional excretion of sodium help differentiate prerenal scanning, angiography, computed tomography, magnetic resonance
from intrinsic AKI (Table 81.1). Although a BUN-creatinine ratio of imaging, and renal biopsy, may occasionally be necessary but gener-
greater than 20:1 is suggestive of prerenal azotemia in adults, this is ally are not indicated in the child with AKI during the initial workup.
not necessarily true in infants and young children because they often If a glomerular cause is suspected based on laboratory findings or
normally have a BUN-creatinine ratio equal to or greater than 20. radiologic evaluation, a biopsy is an appropriate next step. Chest
Tubular epithelial cells and brown-pigmented casts are common radiography may also be helpful in detecting cardiac enlargement
in patients with ATN. Evidence of hematuria or proteinuria signi- or pulmonary edema caused by fluid overload.
fies glomerular disease, especially glomerulonephritis. The presence
of blood on urine dipstick but absence of red blood cells (RBCs) on
Management
sediment examination is suggestive of hemoglobinuria (eg, hemo-
lytic uremic syndrome) or myoglobinuria (eg, rhabdomyolysis) as Prevention is better than cure, and because of exponential advance-
the basis of ATN. ment in the area of biomarkers to predict AKI in the acutely
Although urinary indices are helpful in differentiating prerenal ill child, preventive measures are already in place to overcome
from intrinsic AKI, a simple clinical method can be used to distin- manifestation of AKI by early intervention. The most common
guish between them. A therapeutic trial of volume expansion with etiology among all children who develop AKI is prerenal azotemia.
20 mL/kg of normal saline is administered intravenously over 30 to This condition is corrected by reestablishing adequate circu-
60 minutes after first excluding the possibility of congestive heart lating volume. Prevention is particularly important because
failure or urinary obstruction. If oliguria persists at the end of no currently available treatment can induce rapid recovery of
1 hour, furosemide (2 mg/kg) can be administered. If urinary output renal function in humans after the condition has progressed to
does not increase after furosemide administration, repeat admin- intrinsic AKI. Although low-dose dopamine and furosemide
istration of high-dose furosemide has few benefits and can cause often are used in the initial management of AKI, many studies
toxicity, especially hearing loss, particularly in the preterm newborn, have shown that these medications do not enhance recovery of
renal function. Fenoldopam mesylate, a dopamine receptor ago-
nist, has been used in critically ill, hemodynamically unstable
Table 81.1. Diagnostic Indices patients with AKI to improve renal perfusion, with some benefit
in Acute Kidney Injurya in select patients. Certain drugs, such as adenosine triphosphate-
Test Prerenal Disorder Intrinsic Renal Disorder magnesium chloride, thyroxine, atrial natriuretic peptide, and
insulinlike growth factors, have been used in experimental
Urinalysis Normal, occasional Renal epithelial cells;
animal models and some human trials without much success.
granular casts pigment casts
The goal of therapeutic management of intrinsic AKI is mainte-
Urine osmolality >600 <400 nance of normal body homeostasis while awaiting spontaneous
(mOsm/kg H2O) improvement, because proximal tubules can undergo repair and
Urine specific >1.020 <1.015 regeneration after damage.
gravity After dehydration is corrected, if urine output is still not estab-
Urine sodium <15 >40 lished daily fluid intake should be limited to replacement of insen-
(mEq/L) sible water loss (approximately 30%–40% of daily recommended
U-P creatinine >40 <20 fluid intake for age), any urinary losses, and fluid losses from non-
renal sources (eg, nasogastric drainage). Hyperhydration should be
Fractional excretion <1% >2%
avoided in the patient with AKI because of its association with a
of sodium (FENa)b
high mortality rate and increased morbidity from edema, conges-
Abbreviation: U-P creatinine, urine-creatinine (mg/dL) to plasma creatinine (mg/dL). tive heart failure, hypertension, hyponatremia, encephalopathy, and
a
Values in patients with nonoliguric acute kidney injury often overlap and fall between prerenal
seizures. Recent studies have shown that fluid overload in patients
and renal values. Additionally, values in newborns differ from those in children older than 1 year.
b
FENa = (UNa/UCr) × (PCr/PNa) × 100. UNa, urinary concentration of sodium (mEq/L); FENa, in an ICU setting who did not previously have AKI can induce AKI,
fractional excretion of sodium; UCr, urinary concentration of creatinine (mg/dL); PCr, plasma and in patients with AKI fluid overload can contribute to increased
concentration of creatinine (mg/dL); PNa, plasma concentration of sodium (mEq/L). morbidity and mortality.

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 588 PART 5: ACUTE AND EMERGENT PROBLEMS

Patients with complete anuria require no sodium intake. Sodium hyperkalemia or acidosis, volume overload with pulmonary edema
losses, however, should be replaced daily in patients with any uri- or congestive heart failure unresponsive to diuretic treatment, pro-
nary output. Preferably, the amount of sodium required is deter- gressive uremia with BUN level greater than 100 mg/dL, or creati-
mined by measuring daily urinary sodium losses, which can vary nine clearance less than 15 mL/min/1.73 m2. In preterm and term
by individual patient. neonates, peritoneal dialysis is usually preferred over hemodialysis
In the patient with suspected AKI, potassium intake from to avoid the major hemodynamic instability that often occurs with
all sources should be restricted. Severe hyperkalemia can often hemodialysis. In the critically ill child with overwhelming sepsis or
be avoided early in the course of the disease with strict adher- multisystem organ dysfunction, however, early continuous venove-
ence to potassium restriction. The level of serum potassium as nous hemodiafiltration is indicated for more gradual fluid removal,
well as changes on ECG should be closely monitored. The patient thereby avoiding significant fluctuations in the fluid balance and
with mild hyperkalemia may be treated with ion exchange resin; optimizing nutritional support.
sodium polystyrene sulfonate (eg, Kayexalate, Resonium A) may Acute renal injury often can be prevented by anticipating its
be given orally every 4 to 6 hours or by retention enema every possible occurrence in the child with a high-risk condition, such
1 to 2 hours. Sodium polystyrene sulfonate should be mixed in as dehydration, trauma, sepsis, and shock, or after cardiac surgery.
water; mixtures containing polysorbate should be avoided because Prompt recognition of prerenal failure and aggressive management
they may cause bowel perforation. Moderate hyperkalemia can be of it with volume expansion may prevent the manifestation of intrin-
managed with insulin and glucose infusions or b agonists, which sic AKI. Nephrotoxic agents, such as gentamicin, should be avoided
will drive the potassium intracellularly. In the patient with changes in the high-risk patient if possible. When these drugs are used, they
on ECG suggestive of hyperkalemia, such as tall T waves or should be monitored meticulously, with frequent measurement of
widened QRS complexes, calcium gluconate should be given to sta- blood levels.
bilize the myocardium. If serum potassium continues to rise or evi-
dence exists of cardiac instability despite conservative treatment,
dialysis should be initiated to reduce the total burden on body Prognosis
potassium. Hypocalcemia and hyperphosphatemia are common The in-hospital and long-term complications in the child with AKI
in patients with AKI, and no treatment is required to address can be associated with poor cardiovascular and renal prognosis. Three
small alterations in levels of calcium and phosphorus. For serum scenarios exist in which a child with AKI can develop chronic kidney
phosphate greater than 8 mg/dL, a phosphate binder (eg, calcium disease. First, the initial episode of AKI may cause permanent dam-
carbonate, calcium acetate) may be used if the child can take nothing age to the kidneys, resulting in end-stage renal disease. Second, recov-
by mouth. If serum calcium is less than 8 mg/dL, intravenous (IV) ery from the initial episode may be incomplete, resulting in relatively
or oral calcium should be administered to prevent tetany. If oral low renal function compared with baseline function, and consequently,
calcium supplements are given, the child will require 1,25 (OH) chronic kidney disease. Third, the child who regains near-normal or nor-
vitamin D (calcitriol by mouth or IV calcitriol) to enhance gastro- mal renal function continues to be at increased risk for developing kid-
intestinal absorption of calcium. ney failure years later compared with the child who did not have AKI.
Mild metabolic acidosis is common in AKI and requires no treat- The duration of oliguria in AKI may be short (1–2 days) or long
ment. If blood pH is less than 7.2 or serum bicarbonate is less than (a few weeks). Typically, recovery is first indicated by an increase in
12 mEq/L, sodium bicarbonate can be initiated and continued until urinary output. Blood urea nitrogen and creatinine levels may rise
renal function improves. during the first few days of diuresis before beginning to return to
Adequate nutrition is important in the patient with AKI because normal. During diuresis, large quantities of sodium and potassium
it prevents excessive tissue breakdown. If renal failure is expected may be lost in the urine. Serum electrolyte levels should be closely
to be short in duration (3–4 days), most calories may be provided monitored, and adequate replacements should be made to prevent
as carbohydrates. If AKI is expected to last longer, adequate calo- hyponatremia and hypokalemia.
ries in the form of carbohydrates along with daily protein intake of In the child with AKI, outcomes mainly depend on the primary
1 g/kg should be provided. condition, severity of damage to other organs, and physician exper-
Anemia should be identified and corrected if hemoglobin is tise in managing AKI. Nonoliguric AKI is consistently associated
less than 10 g/dL to improve oxygen and nutrient delivery to the with a shorter clinical course and better prognosis than oliguric AKI.
tubules to facilitate regeneration of cells and establish their func- Most children with ATN recover completely. However, children with
tion. This can be achieved by maintaining an optimal hemoglobin more severe kidney involvement (eg, cortical necrosis) may have
level by transfusing packed RBCs and initiating subcutaneous residual renal impairment or chronic renal failure, and children who
or IV administration of epoetin alfa (eg, Procrit, Epogen) if renal are critically ill have a 60% mortality rate. Studies on older chil-
failure is prolonged. dren have also shown that AKI results in chronic kidney disease in
Many children with AKI can be managed by the conservative a higher percentage of children than was previously appreciated.
measures described previously. If renal failure lasts more than Early recognition of potential risk factors and prompt interven-
a few days or if complications arise, however, dialysis should be tion will reduce long-term sequelae of AKI, particularly the devel-
planned. The usual indications for dialysis include uncontrollable opment of end-stage renal disease in the long term.

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 Chapter 81: Acute Kidney Injury 589

Askenazi DJ, Ambalavanan N, Goldstein SL. Acute kidney injury in crit-

ically ill newborns: what do we know? what do we need to learn? Pediatr
CASE RESOLUTION Nephrol. 2009;24(2):265–274 PMID: 19082634 https://doi.org/10.1007/
A series of diagnostic studies is performed. The laboratory results are hemoglobin, s00467-008-1060-2
13.8 g/dL; hematocrit, 41%; white blood cell count, 12,400/mcL; neutrophils,
58%; band forms, 6%; lymphocytes, 32%; monocytes, 3%; and eosinophils, 1%. Basu RK, Devarajan P, Wong H, Wheeler DS. An update and review of acute
The platelet count is 277,500 platelets/mcL. Serum sodium is 136 mEq/L; potas- kidney injury in pediatrics. Pediatr Crit Care Med. 2011;12(3):339–347 PMID:
sium, 5.1 mEq/L; chloride, 110 mEq/L; bicarbonate, 10 mEq/L; BUN, 84 mg/dL; 21057358 https://doi.org/10.1097/PCC.0b013e3181fe2e0b
creatinine, 2.8 mg/dL; and glucose, 68 mg/dL. The urinalysis reveals a specific Ciccia E, Devarajan P. Pediatric acute kidney injury: prevalence, impact and man-
gravity of 1.015; trace protein, blood, white blood cell, and nitrite are all nega- agement challenges. Int J Nephrol Renovasc Dis. 2017;10:77–84 PMID: 28435306
tive; and the sediment has many epithelial cells, 1 to 2 RBCs, and many granular https://doi.org/10.2147/IJNRD.S103785
and pigmented casts. Spot urinary sodium is 65 mEq/L, creatinine is 39 mg/dL,
Du Y, Zappitelli M, Mian A, et al. Urinary biomarkers to detect acute kidney
and fractional excretion of sodium is 3.4%.
injury in the pediatric emergency center. Pediatr Nephrol. 2011;26(2):267–274
These results, particularly the increased fractional excretion of sodium, are
PMID: 20978799 https://doi.org/10.1007/s00467-010-1673-0
most consistent with a diagnosis of intrinsic AKI. The history points to prerenal
failure initially, after which prolonged hypovolemia contributed to ischemia of Fortenberry JD, Paden ML, Goldstein SL. Acute kidney injury in children: an
the kidneys, resulting in intrinsic renal failure. According to the pRIFLE criteria, update on diagnosis and treatment. Pediatr Clin North Am. 2013;60(3):669–688
the laboratory results indicate that the patient is likely in stage 3 failure. Recovery PMID: 23639662 https://doi.org/10.1016/j.pcl.2013.02.006
may take a few days to a few weeks. The patient is admitted to the pediatric Goldstein SL, Jaber BL, Faubel S, Chawla LS; Acute Kidney Injury Advisory
step-down unit, where fluids are adjusted according to her urine output and Group of American Society of Nephrology. AKI transition of care: a potential
electrolytes are monitored frequently. opportunity to detect and prevent CKD. Clin J Am Soc Nephrol. 2013;8(3):
476–483 PMID: 23471414 https://doi.org/10.2215/CJN.12101112
Khwaja A. KDIGO clinical practice guidelines for acute kidney injury. Nephron
Clin Pract. 2012;120:c179–c184 https://doi.org/10.1159/000339789
Selected References
Ringer SA. Acute renal failure in the neonate. NeoReviews. 2010;11(5):
Al-Ismaili Z, Palijan A, Zappitelli M. Biomarkers of acute kidney injury in e243–e251 https://doi.org/10.1542/neo.11-5-e243
children: discovery, evaluation, and clinical application. Pediatr Nephrol. Schneider J, Khemani R, Grushkin C, Bart R. Serum creatinine as stratified in the
2011;26(1):29–40 PMID: 20623143 https://doi.org/10.1007/s00467-010-1576-0 RIFLE score for acute kidney injury is associated with mortality and length of
Andreoli SP. Acute kidney injury in children. Pediatr Nephrol. 2009;24(2):253–263 stay for children in the pediatric intensive care unit. Crit Care Med. 2010;38(3):
PMID: 19083019 https://doi.org/10.1007/s00467-008-1074-9 933–939 PMID: 20124891 https://doi.org/10.1097/CCM.0b013e3181cd12e1

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 CHAPTER 82

Ingestions: Diagnosis
and Management
Kelly D. Young, MD, MS, FAAP

CASE STUDY
A 2-year-old girl is found by her mother with an open Questions
bottle of pills and pill fragments in her hands and 1. What history questions should be asked to help
mouth. She is rushed to the emergency department. identify the substance ingested?
She is sleepy but able to be aroused. The vital signs are 2. What physical examination findings can offer clues
temperature of 37.1°C (98.8°F), heart rate of 120 beats to the substance ingested and the seriousness of the
per minute, respiratory rate of 12 breaths per minute, ingestion?
and blood pressure of 85/42 mm Hg. The pupils are 2 mm 3. What other diagnostic tests might be helpful in
and reactive. Skin color, temperature, and moisture are treating ingestion patients?
normal. She has no other medical problems. 4. What are the management priorities?

Ingestions are a common problem presenting to pediatric prac- battery ingestions. A registry that included cases on which a med-
titioners. Three scenarios frequently encountered are accidental ical toxicologist was consulted (presumably for serious exposures)
ingestions by preschool-age children, intentional suicide attempts at 31 participating centers reported on the most common agents
by adolescents, and recreational drug use. This chapter discusses involved for infants and toddlers age 2 years and younger: 16%
the general approach to the child who has ingested a potentially cardiac drugs, 15% psychotropic drugs, 9% recreational drugs and con-
poisonous substance. Ingestions of specific substances are beyond trolled narcotics, 9% analgesics, 7% cleaning products, 5% scorpion
the scope of this chapter, as is toxicity occurring by dermal, oph- stings, and 4% toxic alcohols.
thalmologic, and inhalational routes. The general approach to Fatalities are uncommon overall and are more likely to occur
the history, physical examination, laboratory tests and diagnostic with intentional ingestion by older children. Poison control cen-
studies, and management, especially decontamination, is useful for ter data from 2016 revealed 31 pediatric fatalities (age 0–12 years)
all ingestions, however. and 42 adolescent fatalities (age 13–19 years). Children accounted
for 2% of total toxicologic fatalities for the year, whereas adoles-
Epidemiology cents accounted for 3% and adults for the remainder. Young chil-
Most calls made to poison control centers involve pediatric patients. dren tend to ingest nontoxic substances or small quantities of toxic
Poison control center data from 2016 show that pediatric patients substances. Review of trends over the past few years indicates
younger than 20 years accounted for 60% of exposures and young a reduction in overall calls but an increase in calls about serious
children aged 0 to 5 years accounted for 46%. Among younger exposures. Fatality rates have remained stable.
children boys were more commonly exposed, whereas girls pre- The frequency of exposures to analgesics (narcotics), cardiac
dominated in adolescence. For children aged 0 to 12 years 3.5% of drugs, and psychotropic drugs in pediatric patients is linked to an
exposures are intentional, whereas for adolescents aged 13 to 19 years overall rise in adult prescription drug use. Cough and cold medi-
27% are intentional, and for adults 69% are intentional. cations are an increasingly recognized source of toxicity in young
The most common substances ingested overall are analgesics, children, and the US Food and Drug Administration recommends
including acetaminophen, nonsteroidal anti-inflammatory drugs, against their use in children younger than 6 years. Another new
and narcotics; household cleaning substances; and cosmetics/ source of serious toxic exposures in children is laundry and dish-
personal care products. The most common fatal ingestion in children washer detergent capsules, which can have an appearance similar
is analgesics (often narcotics that are not their own prescription). to candy. A rise in inadvertent pediatric marijuana exposures has
Other common pediatric fatal poisonings are stimulants and street been reported in states with legalized marijuana, with edible sources
drugs, carbon monoxide poisoning, antidepressants, and disc playing a sizeable role.

591

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 592 PART 5: ACUTE AND EMERGENT PROBLEMS

Recreational drug use is another source of serious exposures in

Box 82.1. Symptoms of Toxic Ingestion
adolescents. Narcotics, cocaine, amphetamines, and ecstasy remain
popular, and newer forms of recreational drugs include energy ww Bradycardia or tachycardia
drinks with or without alcohol, synthetic cathinones (“bath salts”), ww Hypothermia or hyperthermia
synthetic cannabinoids (“K2,” “Spice”), dextromethorphan, inhal- ww Respiratory depression or hyperpnea
ants (especially computer cleaners), and the hallucinogenic herb ww Hypotension or hypertension
salvia, which is legal in many states and also has opioid effects. ww Mydriasis or miosis
Contamination of street drugs with high-potency opiates, such as ww Altered mental status or abnormal behavior
carfentanil, is contributing to the rise in opioid fatalities. ww Seizure
ww Cardiac dysrhythmia
Clinical Presentation ww Metabolic derangement
ww Nausea, vomiting, diarrhea
The clinical presentation following an ingestion varies considerably
depending on the substance ingested. Some patients may not present
with a clear history of a toxic ingestion. The physician must maintain
a high index of suspicion for poisoning as the cause of symptoms
such as altered behavior, depressed level of consciousness, cardiac
Evaluation
dysrhythmia, vomiting, seizure, and autonomic changes. A detailed history of what and how much the patient ingested is key
to the evaluation. Physical examination should focus on identifying
Pathophysiology symptoms and serious complications. The patient should be mon-
itored and reassessed frequently. Laboratory and other diagnostic
The pathophysiologic profile depends on the substance. Some toxins act
studies can be tailored to the specific ingestion.
on a particular organ system (eg, acetaminophen on the liver, ethanol
on the central nervous system), whereas others act diffusely at the cellu- History
lar level (eg, cyanide). Generally, drugs are absorbed, distributed within
The most important questions address the ingestion (Box 82.2),
the body, metabolized, and excreted. Drug levels obtained prior to com-
such as, What did the patient ingest? What is the maximum possi-
pletion of absorption and distribution may not reflect the peak level.
ble amount that was ingested? When did the ingestion occur? What
Interventions focus on preventing absorption, sometimes on preventing
symptoms are occurring? History of previous medical conditions
metabolism into a more toxic by-product, and on enhancing excretion.
is also important to identify increased susceptibility to a particu-
Toxic effects may be delayed or prolonged when an extended-release
lar toxin (eg, seizure disorder with ingestion of a substance that
form of a drug has been ingested, with drugs likely to form concretions
causes seizures); assess for factors that may have precipitated the
(eg, iron, aspirin, theophylline), or when toxicity results from an active
ingestion, such as depression; and assess for medications that may
metabolite (eg, toxic alcohols, acetaminophen, acetonitrile, dapsone).
interact with or exacerbate the toxic effects of the ingested substance
Pharmacogenetics are increasingly recognized as having an important
(eg, acetaminophen ingestion in a patient taking a cytochrome P-450
role in individual responses to medications and toxins. Genetic varia-
inhibitor).
tions in enzymatic activity in drug metabolism may result in toxicity
Parents and caregivers should be encouraged to bring in the con-
through excessively rapid metabolism of a drug to its active metabo-
tainer and any remains of the substance ingested. The physician
lite, or through slow metabolism of a drug to its inactive metabolite.
should attempt to obtain the exact formulation because generic and
For example, a cytochrome P-450 CYP2D6 genotype has been linked
brand name drugs may differ. The physician must also be aware of
to rapid metabolism of codeine to active morphine, resulting in
possible combination products. Investigative methods such as calling
toxicity and even fatality. The US Food and Drug Administration has
added a boxed warning on codeine and a contraindication for its use
after tonsillectomy or adenoidectomy in children. Box 82.2. What to Ask
Toxic Ingestion
Differential Diagnosis
ww What did the child take? (If unknown, what is available to the child?)
The differential diagnosis of toxic ingestions is broad. For the patient ww How much did the child take? (If unknown, what is the maximum
with a history of ingestion, the differential diagnosis is narrowed to amount possible?)
substances available to the child. If no history of ingestion is given, ww When did the child take it? (If unknown, how long was the child unat-
the physician should include ingestion in the differential diagnosis tended or unobserved?)
when evaluating symptoms and signs such as altered mental sta- ww What symptoms have occurred, and when did the symptoms begin rela-
tus, altered behavior, metabolic derangement, cardiac dysrhythmia, tive to the time of ingestion?
hypotension and shock states, seizure, respiratory distress or apnea, ww What other medical conditions does the child have?
cyanosis, vomiting, and diarrhea (Box 82.1). In fact, almost any ww What medications does the child take regularly?
symptom complex may result from a toxic ingestion.

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 Chapter 82: Ingestions: Diagnosis and Management 593

or sending a family member to the home to identify the product, co-ingestion of another undisclosed substance must be considered.
calling the pharmacy on a prescription label, or identifying a pill Particular attention should be paid to all 4 vital signs (ie, temper-
by comparing its picture and imprint to those in a pharmaceuticals ature, respiratory rate, heart rate, blood pressure); pupillary size
reference may be necessary. Internet search engines may be used to and reaction; breathing (eg, Kussmaul respiration that occurs with
search the imprint on a pill or identify foreign medications. acidosis); mental status; distinctive breath odors; presence or
Caregivers should be questioned about all available drugs or absence of bowel sounds; and skin color, temperature, and mois-
other toxic substances in the household. Sometimes caregivers must ture. The patient’s weight should be measured, because toxicity is
be encouraged to mention all substances in the household, even often estimated based on milligrams of drug ingested per kilogram
those they do not think the child could possibly have obtained. The of body weight. Because symptoms may develop or worsen if peak
physician must also ask about medications used by recent visitors levels of the toxic substance have not been reached at the time of ini-
(eg, grandparents) and the possibility of an exposure at a recently tial evaluation, continual reassessment and cardiorespiratory mon-
visited household or location. It is important not to overlook herbal itoring are imperative. If a symptomatic patient is noted to have a
preparations, vitamins, alternative medications, household prod- typical toxidrome, therapy may be initiated based on the toxidrome
ucts (including cleaning and personal care products), gardening without confirmation of the exact substance ingested. Some common
products, chemicals used in hobbies or work, and alcohol or illicit toxidromes and their treatments are listed in Table 82.1.
drugs belonging to an adult. Caregivers may initially overlook these
as they concentrate only on recalling “medications.” It may be help- Laboratory Tests
ful to interview siblings or friends of an adolescent suspected of Qualitative drug screening (reporting only the presence or absence
recreational drug use. The physician must maintain a high index of the drug) of urine or blood often is done when poisoning is part
of suspicion for unreported co-ingestants, especially in adolescent of a broader differential diagnosis for symptoms such as altered
suicide attempts. mental status or acute behavioral changes. Such drug screening is
Although often difficult, it is important to attempt to deter- rarely helpful in the patient with acute poisoning because typically
mine the quantity of drug that was available to the patient and how results are not rapidly reported, testing can be done for only a lim-
much is currently missing. It may be necessary to count pills or mea- ited number of substances, and false-positive and -negative results
sure liquid to make the determination. For estimating liquids, the may occur. Given the frequency of narcotic ingestions, it is important
approximate volume of a swallow is 0.3 mL/kg. The physician should to note that synthetic opioids (eg, fentanyl, methadone, oxycodone,
always assume the “worst case” (ie, the patient took all of the drug hydrocodone) are not detected by typical hospital immunoassay
that is missing). History about the amount ingested may be inaccu- “tox screens.” Laboratory tests and diagnostic studies to consider
rate, especially when elicited from the adolescent with intentional for the patient with known or suspected toxic ingestion are listed
ingestion. in Box 82.3.
The physician should attempt to determine approximately what Quantitative drug levels for specific drugs can be helpful to esti-
time the ingestion occurred. Symptoms are usually expected within mate severity of expected symptoms or to rule out ingestion of that
a defined time range. Recommended observation periods before dis- drug, however. Examples include acetaminophen, salicylate, eth-
charge take into account expected symptoms based on the length anol, methanol, ethylene glycol, iron, theophylline, lithium, anti-
of time since the ingestion. Timing may also be important in deter- convulsants, and levels of carboxyhemoglobin or methemoglobin
mining what substance was most likely ingested. For example, inges- by blood gas analysis. With the exception of acetaminophen and
tion of mushrooms that cause a self-limited illness usually results in ethanol, such levels should be measured only when suggested by
gastrointestinal (GI) upset within 4 to 6 hours, whereas Amanita the history or physical examination. Many toxicology experts feel
mushrooms that may ultimately result in hepatic failure typically that because acetaminophen overdose produces few acute symp-
present with GI upset in 6 to 12 hours. toms, may lead to fulminant hepatic failure, and is readily treatable
The physician should ask about current symptoms and when they with an antidote, and because acetaminophen is a frequent ingredi-
started relative to the time of the ingestion. In a patient without a ent in combination products, acetaminophen level should be deter-
definite history of ingestion, certain toxidromes (ie, recognizable mined for all patients with a history of ingestion. In adolescents and
combinations of symptoms suggestive of a certain class of medica- adults, ethanol is a common co-ingestant, and ethanol levels are rou-
tions or toxins) may be suggestive of a specific substance or class tinely measured. Routine salicylate levels are likely to be low yield in
of substances. Whether the patient is symptomatic and what symp- the absence of suspicion based on history or physical examination,
toms are present may guide the workup for an unknown ingestion, although some physicians do obtain them as well.
determine whether hospitalization is necessary, or dictate therapy. Serum chemistries and osmolarity may offer clues about what
was ingested when the substance is unknown. The anion gap is
Physical Examination calculated as [Na] − ([Cl] + [HCO3]) and is normally 8 to 12 mEq/L.
If the substance ingested is known, the physical examination should An elevated anion gap indicates the presence of metabolic acidosis
be focused on identifying expected symptoms of toxicity. A general and occurs in ingestions and conditions identified by the MUDPILES
physical examination should always be performed, however, because mnemonic: methanol, uremia, diabetic ketoacidosis, paraldehyde

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 594 PART 5: ACUTE AND EMERGENT PROBLEMS

Table 82.1. Toxidromes

Toxidrome Toxins/Drugs Symptoms Treatment
Narcotic or opiate Oxycodone, hydrocodone, methadone, Nausea/vomiting, respiratory depression, Naloxone, respiratory support
fentanyl, heroin, codeine, morphine miosis, altered mental status, coma
Cholinergic Organophosphate and carbamate Diarrhea, urination, miosis, bronchorrhea and Atropine, pralidoxime (ie, 2-PAM)
(parasympathomimetic) pesticides bronchospasm, emesis, lacrimation, lethargy,
salivation (ie, DUMBELLS)
Anticholinergic Antihistamines, jimson weed, Flushing (“red as a beet”), dry skin and Supportive care
antipsychotic agents, some antide- mucous membranes (“dry as a bone”),
pressants, Parkinson medications hyperthermia (“hot as a hare”), delirium
(“mad as a hatter”), mydriasis (“blind as a
bat”), tachycardia, urinary retention, ileus/
decreased bowel sounds
Sympathomimetic Cocaine, amphetamines, ephedrine Mydriasis, anxiety, tachycardia, hypertension, Quiet environment, benzodiazepines
hyperthermia, diaphoresis
Sympatholytic Clonidine, beta blocker (eg, Bradycardia, hypotension, miosis, possibly Supportive care, fluids and
propranolol) lethargy pressors if necessary for hypoten-
sion, atropine for symptomatic
bradycardia
Tricyclic antidepressant Imipramine, amitriptylinea Seizure, tachycardia, prolonged QRS com- Sodium bicarbonate
plex, altered level of consciousness, cardiac
dysrhythmia
Salicylate Aspirin, methyl salicylate, oil of Hyperventilation, nausea/vomiting, tinnitus, Alkalinization with sodium
wintergreen hyperthermia, metabolic acidosis bicarbonate (ie, NaHCO3; confirm
salicylate level)
Serotonin syndrome Selective serotonin reuptake Altered mental status, neuromuscular rigidity, Supportive care
inhibitorsa tremors, or hyperreflexia; autonomic instability:
hyperthermia, mydriasis, tachycardia,
hypertension or hypotension
a
Not an exhaustive list.

and phenformin, iron and isoniazid, lactic acidosis, ethylene

Box 82.3. Laboratory Tests and Diagnostic glycol and ethanol, and salicylates and solvents (eg, toluene).
Studies to Consider for Toxic Ingestion The osmolar gap is the difference between the measured serum
ww Specific drug levels as indicated osmolarity and the calculated osmolarity (given by the formula
ww Qualitative urine or blood toxicology screening 2[Na] + [glucose]/18 + [BUN]/2.8). The normal osmolar gap is less
ww Acetaminophen level; consider ethanol and salicylate levels than 10 mOsm. An elevated osmolar gap occurs with inges-
ww Serum chemistries (ie, calculate serum anion gap) tion of alcohols such as ethanol, methanol, ethylene glycol, and
ww Serum osmolarity (ie, calculate osmolar gap) isopropanol.
ww Liver function panel and renal function tests Depending on the level of suspicion for acidosis, hypoxemia,
ww Rapid bedside glucose test or abnormal hemoglobins (ie, carboxyhemoglobin and methe-
ww Urine pregnancy test moglobin), arterial blood gas analysis may be indicated. Because
ww Pulse oximetry hypoglycemia may be noted with some ingestions and is easily
ww Electrocardiography treated, a rapid bedside glucose test should be done on all patients.
ww Arterial blood gas with carbon monoxide and methemoglobin levels Urinalysis and creatinine phosphokinase level tests may be per-
ww Urinalysis formed to evaluate for signs of rhabdomyolysis if the patient is
ww Creatinine phosphokinase level deemed at risk. All females of childbearing age should undergo
ww Chest radiography a urine pregnancy test. Assessment of liver and renal function
ww Abdominal radiography for radiopaque tablets is often important because many substances are metabolized by
these routes.

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 Chapter 82: Ingestions: Diagnosis and Management 595

Diagnostic Studies when available and indicated, and meticulous supportive care, often
Pulse oximetry and cardiorespiratory monitoring should be instituted in an intensive care unit.
for all serious ingestions. Electrocardiography may be indicated if Decontamination
cardiac toxicity is expected. Other studies are tailored to the specific
Decontamination techniques are used to prevent or minimize
ingestion, such as endoscopy after ingestion of caustic acids or alkalis.
absorption of the toxic substance and to enhance its elimination.
Imaging Studies They are a critical part of the treatment of the acutely poisoned
patient and should be used whenever a significant ingestion is
Specific imaging studies may be indicated for certain ingestions,
suspected (Table 82.2).
such as chest radiography in the case of hydrocarbon ingestion to
Historically, ipecac syrup was commonly recommended for home
look for signs of aspiration. An abdominal radiograph may be help-
use to induce vomiting in the event of an accidental ingestion. The
ful in identifying ingestion of radiopaque substances and monitor-
American Academy of Pediatrics released a policy statement in 2003
ing the effectiveness of GI decontamination procedures for removing
stating that ipecac syrup should no longer be kept in homes and is
such substances. The mnemonic CHIPS can be used for remem-
not recommended because studies showed that its use resulted in
bering which medications are radiopaque: chloral hydrate, heavy
no difference in outcomes. Gastric lavage, in which a large nasogas-
metals, iron, phenothiazines, and slow-release (ie, enteric-coated)
tric tube is placed and the stomach is washed with normal saline
medications. In practice, abdominal radiography is primarily used
theoretically removes toxic substance from the stomach, thereby pre-
in iron ingestion and suspected body-packing with illicit drugs.
venting absorption. At best (ie, immediate performance after inges-
tion), however, less than one-third of gastric contents are removed
Management by this method. Additionally, this technique may interfere with the
Management strategies are specific to the substance ingested. The use of activated charcoal, which usually is a more effective therapy.
regional poison control center should be consulted for advice on Gastric lavage is technically difficult to perform in young children
treatment and length of time to observe the asymptomatic patient. because of the need to pass a large-bore tube. Gastric lavage also
A single telephone number, 1-800-222-1222, automatically routes has a high rate of complications, such as aspiration and esophageal
the caller to 1 of the appropriate 55 regional poison control centers trauma. Gastric lavage is not recommended for routine use. The
in the United States. Generally, the approach to management American Academy of Pediatrics released a policy statement in 2003
includes attention to the basics of resuscitation (circulation, stating that ipecac syrup should no longer be kept in homes. Ipecac
airway, breathing), decontamination methods, specific antidotes syrup was administered in 0.01% of pediatric ingestions in 2011.

Table 82.2. Summary of Gastric Decontamination Techniques

Technique Dose Contraindications
Gastric lavage 15 mL/kg aliquots normal saline to maximum of 400 mL until lavage ALOC with unprotected airway
fluid is clear (may be several liters) Caustics: acids and alkalis
Hydrocarbons
Expected ALOC
>1 hour since ingestion
Activated charcoal 1–2 g/kg or ALOC with unprotected airway
<6 years: 25–50 g Absent bowel sounds or bowel obstruction
≥6 years: 50–100 g Substance not bound by charcoal
Cathartics Magnesium citrate 4 mL/kg Not recommended for routine use
70% sorbitol 1 g/kg Repeated doses can cause dehydration or
electrolyte imbalances
Whole-bowel irrigation Toddler and preschool age: polyethylene glycol solution 500 mL/hour Bowel obstruction, ileus, perforation, or hemorrhage
6–12 years: 1,000 mL/hour ALOC with unprotected airway
Adolescent and adult: 1.5–2 L/hour
Continue until rectal effluent is clear
Lipid emulsion 20% 1.5 mL/kg initial bolus intravenous, then 0.25 mL/kg/minute for For lipophilic drug overdose with severe cardiotoxicity
30–60 minutes
Abbreviation: ALOC, altered level of consciousness.

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 596 PART 5: ACUTE AND EMERGENT PROBLEMS

Activated charcoal is the mainstay in decontamination therapy Hemodialysis may be used for serious ingestions of ethylene gly-
of ingestion. Charcoal binds toxins, and because it is not absorbed col, methanol, phenobarbital, lithium, salicylate, or theophylline.
in the GI tract, the charcoal-toxin complex passes through and Charcoal hemoperfusion, in which blood passes through a charcoal
is eliminated. Its efficacy decreases with increasing time since cartridge rather than a dialysis machine, is used rarely for severe
ingestion, and ideally it should be started within 1 hour of the theophylline poisoning. Urinary alkalinization (by administration
ingestion. The optimal dose of charcoal is 10 times the amount of of sodium bicarbonate) can increase elimination of weak acids by
substance ingested. Because the exact amount of toxin ingested is keeping the drug in its ionic state, thus preventing reabsorption in
often unknown, activated charcoal is usually dosed at 1 to 2 g/kg the renal tubule. It is used mainly for significant salicylate, pheno-
(teenagers and adults, 25–100 g). The amount of charcoal given is barbital, and isoniazid poisonings.
limited only by what the child can tolerate. Only a few substances Lipid emulsion (eg, Intralipid) is becoming recognized as a
are not absorbed by activated charcoal, and the mnemonic PHAILS potential treatment for lipophilic drug overdoses and has been used
can be used to remember them: pesticides; hydrocarbons and heavy successfully for managing severe cardiotoxicity from bupivacaine
metals; acids, alkalis, and alcohols; iron; lithium; and solvents. hydrochloride, haloperidol, and verapamil hydrochloride overdoses.
The main complication of charcoal administration is aspiration It is unclear by what method this agent works. Although ideal dos-
pneumonitis, which mainly occurs in patients with altered level ing and indications have not been established, 1 suggested treatment
of consciousness and an unprotected airway. If charcoal is not vol- protocol to consider is 1.5 mL/kg of 20% lipid emulsion initial bolus,
untarily taken by the child, it may be administered via nasogas- followed by 0.25 mL/kg per minute for 30 to 60 minutes. Boluses may
tric tube. Endotracheal intubation (preferably with a cuffed tube) be repeated in the setting of severe cardiotoxicity and dysrhythmias.
to protect the airway first may be necessary in the patient with
altered mental status, because charcoal aspiration can result in Supportive Care
severe chemical pneumonitis. It is imperative that nasogastric tube Attention to the basics of resuscitation (circulation, airway, breathing)
placement in the GI tract (as opposed to the respiratory tract) be is always the first step in management. Hypoglycemia must be assessed
confirmed before charcoal administration. and managed as soon as possible. Dextrose 0.5 to 1 g/kg intravenously
Cathartics (most commonly sorbitol) have been used to decrease is administered for hypoglycemia; glucagon may be used if dextrose
transit time and improve elimination of the toxin through the GI cannot be given. Seizure generally is treatable with benzodiazepines.
tract and to counteract activated charcoal-induced constipation. Glucose and electrolyte levels should be normalized. For the patient
The cathartic is often mixed with the activated charcoal and may with seizure caused by isoniazid ingestion, pyridoxine is indicated.
serve to improve the palatability of the charcoal. A significant benefit Shock requires aggressive fluid resuscitation. Fluid-resistant shock
from cathartic use has never been demonstrated, however, and a risk may require vasopressors, most commonly dopamine, epinephrine,
of dehydration and electrolyte disturbances exists, particularly in or norepinephrine. Resistant shock in the setting of beta blocker
young children. The American Academy of Clinical Toxicology rec- ingestion may respond to glucagon, and in the setting of a calcium
ommends against use of cathartics. Under no circumstances should channel blocker ingestion to insulin plus glucose. Dysrhythmias
repeat doses of cathartics be administered. generally should be managed by following pediatric advanced life
Multiple-dose charcoal, which has been called “GI dialysis,” may support (PALS) protocols, although specific ingestions may respond
remove drugs from the bloodstream by promoting diffusion back to specific therapies. Sodium bicarbonate is the first-line treatment
into the GI tract and subsequent binding to charcoal. Activated char- for dysrhythmias associated with ingestions of antihistamines, class 1
coal at the same dose previously used is repeated approximately antiarrhythmic drugs (ie, lidocaine, quinidine, procainamide hydro-
every 4 hours. Cathartics should not be mixed with the charcoal for chloride), cocaine, and tricyclic antidepressants. Beta blocker inges-
repeat doses. Multiple-dose charcoal is useful for a small number of tions may respond to atropine and glucagon, whereas ingestions of
drugs, such as phenobarbital, theophylline, carbamazepine, dapsone, calcium channel blockers are managed with calcium. Procainamide
and quinine. It should be used only if a potentially life-threatening hydrochloride, which is found on PALS algorithms, should be avoided
amount has been ingested. It should not be used for drugs that can for patients with dysrhythmia resulting from overdoses of antihista-
cause an ileus (eg, tricyclic antidepressants). mines, quinidine and other class 1 antiarrhythmic drugs, digoxin,
Whole-bowel irrigation involves infusion of a solution usually quinine, and tricyclic antidepressants. Amiodarone hydrochloride,
used for cleansing of the bowel prior to GI surgery (eg, polyethylene another agent found on PALS algorithms, should also be avoided in
glycol). It is especially useful for slow-release medications, for tab- the management of antihistamine ingestions. Electrolyte imbalances
lets that dissolve slowly and may cause concretions (eg, iron), and in should be assessed and corrected. Suicidal ideation should be assessed,
ingestions for which charcoal is not likely to be effective (eg, heavy often in conjunction with a mental health professional.
metals). A nasogastric tube is used to infuse the solution at a rate
of 500 mL/hour in young children and 1 to 2 L/hour in older chil- Antidotes
dren and adolescents. Clear rectal effluent is the end point; a bed- Antidotes or medications that counteract the pathophysiologic
pan may be necessary. Whole-bowel irrigation should not be used in mechanisms of the toxin are available for only a few ingestions
the setting of bowel obstruction, ileus, perforation, or hemorrhage (Table 82.3). Important antidotes are N-acetylcysteine for acetamin-
or altered mental status with an unprotected airway. ophen, naloxone hydrochloride for narcotics, oxygen for carbon

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 Chapter 82: Ingestions: Diagnosis and Management 597

Table 82.3. Select Antidotes for Specific Ingestions soda bottles, cups). Medications should not be referred to as “candy”
to entice youngsters to take them. Family members and visitors should
Toxin Antidote
be asked to store medications out of the child’s reach and to dispose of
Acetaminophen N-acetylcysteine leftover medications and used transdermal patches safely. Used trans-
Anticoagulants Vitamin K dermal patches may still contain up to 75% of the medication dose.
(warfarin-like) Additionally, chewing on patches, as toddlers may do, releases the
Anticholinergic Physostigmine medication much faster. Parents and caregivers should have the uni-
Benzodiazepine Flumazenil versal telephone number for the poison control center and telephone
numbers for local emergency departments readily available. Carbon
Beta blocker Glucagon
monoxide detectors should be placed near children’s bedrooms.
Calcium channel blocker Calcium, insulin + glucose Activated charcoal for home use is controversial and not currently
Carbamate pesticide Atropine recommended, although it is available without prescription from many
Carbon monoxide Oxygen pharmacies. Parents should not give activated charcoal without first
Cyanide Cyanide antidote kit speaking to poison control center staff or medical personnel.
Digoxin Digoxin immune Fab (ovine; Digibind) Prognosis
Ethylene glycol Fomepizole, ethanol Prognosis depends on the toxicity of the substance ingested. For a
Iron Deferoxamine few substances, a small amount can be fatal (Box 82.4), whereas for
Isoniazid Pyridoxine others, even large ingestions are generally benign. Prognosis is gen-
Lead Dimercaprol (ie, BAL), ethylenediaminetet- erally excellent; fatalities in children are rare. Prognosis is worse for
raacetic acid, DMSA intentional ingestions, often because patients delay or do not reveal
that they attempted overdose.
Mercury BAL, DMSA
Methanol Fomepizole, ethanol
Box 82.4. Substances Potentially Fatal in
Methemoglobinemia Methylene blue
1 to 2 Pills or Teaspoons
Narcotics Naloxone hydrochloride
Organophosphate pesticide Atropine, pralidoxime (ie, 2-PAM) ww Camphor (found in Vicks VapoRub, Campho-Phenique, Tiger Balm)
ww Imidazoline decongestants (found in over-the-counter nasal drops and
Rattlesnake bite Crotalidae polyvalent immune Fab (ovine;
eyedrops)
CroFab)
ww Acetonitrile nail glue remover
Sulfonylurea oral Dextrose, octreotide ww Clonidine hydrochloride (also available in transdermal patches)
hypoglycemic ww Opiates (also available in transdermal patches)
Tricyclic antidepressant Sodium bicarbonate ww Methyl salicylate (oil of wintergreen, Bengay)
Abbreviations: BAL, British antilewisite; DMSA, dimercaptosuccinic acid. ww Calcium channel blockers
ww Toxic alcohols (ie, methanol, ethylene glycol, ethanol, isopropanol)
monoxide poisoning, sodium bicarbonate for tricyclic antidepres- ww Tricyclic antidepressants
sant cardiotoxicity, digoxin immune fab (eg, Digibind, DigiFab) ww Diphenoxylate and atropine (eg, Lomotil)
for digoxin, and deferoxamine for iron. Antidotes are not without ww Sulfonylurea oral hypoglycemics
adverse effects themselves and should be given only in cases of symp- ww Chloroquine and hydroxychloroquine sulfate antimalarial agents
tomatic or, as in the case of acetaminophen, potentially symptom- ww Hydrofluoric acid
atic ingestions of significant amounts. The poison control center staff ww Selenious acid (gun bluing solution)
can be quite helpful in guiding physicians in the use of antidotes. ww Buffered saline solution
ww Benzocaine-induced methemoglobinemia
Anticipatory Guidance and Prevention
Most ingestions are nontoxic and require only observation for a few
hours. These episodes do, however, provide an excellent opportunity to
discuss poisoning prevention with parents and caregivers. Possible tox- CASE RESOLUTION
ins, including prescription and over-the-counter medications, clean- Because the respiratory rate of this 2-year-old is slow and the child exhibits symp-
ing and household products, cosmetics and nail care products, toxic toms of miosis and altered level of consciousness narcotic ingestion is suspected,
plants, gardening and hobby chemicals, and kitchen items (eg, alco- and naloxone is administered. The child becomes more alert, and respiratory rate
increases to 24 breaths per minute. The father is instructed to retrieve the bottle,
hol) should be kept out of reach of children. Visitors to the house- and the substance is found to be a prescription narcotic analgesic left in the house
hold should also be cautioned to keep medications out of reach of by a recent visitor. The child is given activated charcoal, observed overnight in the
children. Substances should never be stored in unmarked contain- hospital, and discharged on the following day without sequelae.
ers, particularly in containers that typically hold beverages (eg, old

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 598 PART 5: ACUTE AND EMERGENT PROBLEMS

Selected References National Poison Data System (NPDS): 34th annual report. Clin Toxicol (Phila).
2017;55(10):1072–1252 PMID: 29185815 https://doi.org/10.1080/15563650.2
American Association of Poison Control Centers. https://aapcc.org. Accessed 017.1388087
June 25, 2019 Henry K, Harris CR. Deadly ingestions. Pediatr Clin North Am. 2006;53(2):293–315
Bailey B. To decontaminate or not to decontaminate? the balance between PMID: 16574527 https://doi.org/10.1016/j.pcl.2005.09.007
potential risks and foreseeable benefits. Clinical Pediatric Emergency Medicine. Hines EQ. Pediatric poisonings: the risk of over-the-counter pharmaceu-
2008;9(1):17–23 https://doi.org/10.1016/j.cpem.2007.11.001 ticals. Pediatr Ann. 2017;46(12):e454–e458 PMID: 29227521 https://doi.
Baker KA, Austin EB, Wang GS. Antidotes: familiar friends and new approaches for org/10.3928/19382359-20171120-02
the treatment of select pediatric toxicological exposures. Clinical Pediatric Emergency Lee VR, Connolly M, Calello DP. Pediatric poisoning by ingestion: developmental
Medicine. 2017;18(3):218–226 https://doi.org/10.1016/j.cpem.2017.07.007 overview and synopsis of national trends. Pediatr Ann. 2017;46(12):e443–e448
Dart RC, Goldfrank LR, Erstad BL, et al. Expert consensus guidelines for stocking PMID: 29227519 https://doi.org/10.3928/19382359-20171121-01
of antidotes in hospitals that provide emergency care. Ann Emerg Med. 2018;71(3): Lowry JA, Burns M, Calello DP. Pediatric pharmaceutical ingestions. Pediatr
314–325.e1 PMID: 28669553 https://doi.org/10.1016/j.annemergmed.2017.05.021 Ann. 2017;46(12):e459–e465 PMID: 29227522 https://doi.org/10.3928/
Drugs.co. Pill identification. http://www.drugs.co/pill_identification.html. 19382359-20171122-01
Accessed June 25, 2019 Smith HS. Opioid metabolism. Mayo Clin Proc. 2009;84(7):613–624 PMID:
Ferreirós N, Dresen S, Hermanns-Clausen M, et al. Fatal and severe codeine 19567715 https://doi.org/10.1016/S0025-6196(11)60750-7
intoxication in 3-year-old twins—interpretation of drug and metabolite con- Toce MS, Burns MM. The poisoned pediatric patient. Pediatr Rev.
centrations. Int J Legal Med. 2009;123(5):387–394 PMID: 19350261 https://doi. 2017;38(5):207–220 PMID: 28461612 https://doi.org/10.1542/pir.2016-0130
org/10.1007/s00414-009-0340-0
U.S. Food and Drug Administration. Safety review update of codeine use in children;
Finkelstein Y, Hutson JR, Wax PM, Brent J; Toxicology Investigators Consortium new boxed warning and contraindication on use after tonsillectomy and/or ade-
(ToxIC) Case Registry. Toxico-surveillance of infant and toddler poisonings in noidectomy. FDA.gov website. https://www.fda.gov/media/85072/download.
the United States. J Med Toxicol. 2012;8(3):263–266 PMID: 22528591 https:// Accessed June 25, 2019
doi.org/10.1007/s13181-012-0227-1
Weinberg G. LipidRescue resuscitation. http://lipidrescue.org. Accessed June
Gummin DD, Mowry JB, Spyker DA, Brooks DE, Fraser MO, Banner W. 25, 2019
2016 annual report of the American Association of Poison Control Centers’

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 CHAPTER 83

Disaster Preparedness
Ireal Johnson Fusco, MD, FAAP, and Katherine E. Remick, MD, FACEP, FAEMS, FAAP

CASE STUDY
A family comes in for a well-child visit with their Questions
7-year-old son and 9-month-old daughter, the latter 1. What are the 4 phases of disaster preparedness with
of whom has complex congenital heart disease. The which the pediatrician should be familiar?
mother is concerned after a recent tornado in the next 2. What should be included in disaster preparedness
town resulted in prolonged power outages. She is won- kits? How should medications for all family
dering what the family might do in this situation. The members be included?
daughter needs daily breathing treatments and often 3. When should a family consider getting a backup
requires oxygen at nighttime. She is on multiple med- generator?
ications and a special formula. All her specialty doc- 4. What is the role of the local hospital and emergency
tors are at the children’s hospital, which is more than medical services for the family with a child or children
an hour from their house. She is also concerned because with special health care and critical medical needs?
her husband has a seizure disorder that requires med- 5. What should the pediatrician recommend to the
ication. She asks whether the family should stay family about children’s immunization records and
together in a disaster or separate to get her daughter to important medical history?
the children’s hospital. 6. How does the physician assess for the effect of
traumatic events on children and their families?

Disaster preparedness has become an increasingly relevant topic include catastrophic events, such as multiple-vehicle collisions, mass
for children and their families. Although natural disasters, war, shootings, and hazardous materials spills. Patients must be quickly
and pandemic infections have always threatened human popu- triaged, treated, and transported. Typically, local government facil-
lations, increasing population density, global warming, interna- itates the initial response in accordance with emergency prepared-
tional trade, and terrorist threats have heightened our awareness ness policies and procedures. This may include coordinating efforts
of disasters and the need for preparedness. State and federal sys- with surrounding communities as per regional preparedness plans,
tems are an essential component of disaster preparedness, but along with additional assistance as necessary from state govern-
significant delays in the delivery of resources can occur. Because of a ment. A public health emergency is declared only when an event
growing need to address the availability of resources and inherently exceeds the ability of local, regional, and state resources to provide
delayed response times of the state and national systems, disas- routine care as the result of any incident that poses substantial risk
ter preparedness is important for families and local communities. for human fatalities or long-term disabilities. Preparedness experts
Recent major disasters, whether human-induced events, such as suggest evaluating public health emergencies from what is called
the 2013 bombing during the Boston Marathon, or natural events, an all-hazards approach, which focuses on the key elements neces-
such as Hurricane Harvey, which devastated Houston, Texas, in sary to ensure the provision of routine care during any type of disas-
2017, demonstrate the vulnerability of communities and the need ter or mass casualty incident. Specific scenarios that occur rarely,
for extensive local preparation. In the first hours to days after a such as chemical or radiation exposure, require access to special-
disaster, community response is vital to well-being, and the com- ized resources that may be impractical to stockpile or are limited in
munity needs to be prepared to play a greater role than was histor- availability. From a practical standpoint, the all-hazards approach
ically anticipated. As frontline health care providers and advocates is more effective.
for children, pediatricians have a particular responsibility to main- Multiple factors make children more vulnerable than adults dur-
tain a baseline understanding of chemical, biological, and radioac- ing a disaster. In the United States, children make up approximately
tive exposures as well as emerging pandemic infections to provide 25% of the population. Younger children, especially, are reliant on
guidance to families in the event of such disasters. others for food, shelter, and, importantly, psychological support.
In addition to large-scale disasters, incidents in which local Their unique nutritional needs put them at risk for malnourish-
emergency medical services (EMS) are overwhelmed by the num- ment if specific dietary requirements are not available. Additionally,
ber and severity of casualties, termed mass casualty incidents, also children often have less mature immune systems. In a setting of
have the potential to overwhelm community resources. These may physical stress and unreliable sanitation, children are at increased
599

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 600 PART 5: ACUTE AND EMERGENT PROBLEMS

risk for infection. Moreover, decreased fluid reserves make chil- centers in developing disaster preparedness plans. All child care
dren more susceptible to blood loss or dehydration from agents facilities should have a plan in place that addresses all-hazards safety,
that cause diarrhea and vomiting. In the setting of a blast or fall, medical needs, evacuation and transportation, and reunification
children are at increased risk for traumatic brain injury because with families. The idea of family-centered care that seeks to keep
of their large head-to-body ratio. Their more pliable skeleton also family units together even as care is needed for individual mem-
increases the likelihood for internal organ injury. Furthermore, bers is important for the immediate physical health and long-term
children are at increased risk for exposure from chemical, biolog- mental health of children during and after disasters.
ical, and radiation disasters because of their unique physiology.
Infants and children have higher minute ventilation, resulting in
increased inhalation of aerosolized agents. Their smaller height The Role of the Pediatrician
increases their exposure to high-vapor density agents, which are With Families
in higher concentrations closer to the ground. Additionally, the The pediatrician serves as an important resource for disaster pre-
skin of infants and children is more permeable because of lesser paredness planning for families. The pediatrician should consider
keratinization compared with adults, and infants and children have assessing a family’s level of readiness for a disaster and then tailor
a larger surface area-to-body mass ratio. As a result, exposed chil- anticipatory guidance accordingly. Families must stay informed and
dren receive a higher dose of transdermally absorbed toxins than realize that everyone is susceptible to some type of disaster. The pedi-
adults exposed under identical circumstances. This larger sur- atrician can also ensure that families understand the importance
face area-to-body mass ratio also complicates treatment, because of preparation and the special needs of children during a disaster.
children are at increased risk for hypothermia during the decontam- The US Federal Emergency Management Agency (FEMA) offers a
ination process. Finally, the psychological effect of being separated free smartphone application (www.fema.gov/smartphone-app) that
from family and experiencing other disaster-related trauma can be includes specific information on various types of natural disasters,
devastating in the short- and long-term. how to build a disaster kit, resources for victims of disasters, and a
The 4 phases that the pediatrician should understand when it disaster reporting feature. Families should prepare an emergency
comes to disaster preparedness and the importance of advocating kit that provides up to 3 days of basic necessities, including food,
for children at each of these steps are planning, rescue, recovery, water, and clothing. Families of newborns and infants must include
and mitigation. Planning includes training and education as well formula and diapers as well as any daily medications for all family
as identifying specific local risks. For example, some communi- members. Copies of immunization and general medical information
ties might need to anticipate hurricanes and flooding, whereas are useful as well as pictures of family members in case the family
others are more concerned about earthquakes or blizzards. This unit is separated. Parents must be prepared to handle nonemergent
is a key area in which the pediatrician can intervene and both problems, because formal medical care may be limited to the seri-
work with families to develop disaster plans and interact with the ously ill and injured during a disaster. If a family needs acute medical
local disaster response community to improve the capacity to care care, it may be necessary to treat children in adult facilities; alter-
for children. Rescue refers to the actions taken during a disaster, and natively, for the family unit to remain together it may be necessary
this is typically what receives the most attention in the media and for adults within the family to undergo treatment in pediatric facil-
by the public. Recovery is the process that begins immediately after ities. The more information families can provide about any medical
the disaster occurs—often simultaneously with the rescue phase— conditions requiring attention, the easier it will be to receive appro-
in which the community works toward returning to normal routines. priate care in a disaster.
This is also the phase during which mental health problems begin to Families of children with special needs are especially vulnera-
emerge. Mitigation is an important and often overlooked phase in ble after a disaster because access to routine medical care may not
which individuals and the community learn from the response to the be available. Experience in Japan during the 2011 earthquake and
disaster to prevent future occurrences or improve on the response subsequent tsunami showed increased mortality among children
to decrease the effect of future disasters. with special needs and increased hospitalizations for children who
Unfortunately, many disaster response teams lack pediatric train- were technology dependent. Not only should families have a suffi-
ing, protocols, and equipment. Recently, various public health and cient supply of medication, they should have a surplus of necessary
disaster organizations have lobbied for states to mandate disaster medical equipment and nutritional supplements. Common supplies,
preparedness regulations for children. Specifically, many states lack such as a feeding tube or catheters, may be in short supply or
basic emergency preparedness regulations for schools and child care unavailable during a disaster. Families with a child on a ventilator
facilities. The developmental vulnerabilities of infants, toddlers, and or one who is oxygen dependent should notify local utilities to flag
young children make them physically less able to escape a disaster their address for priority status during power outages. They should
scene and cognitively less able to recognize the need to flee and fol- consider the benefits of backup battery units and a backup genera-
low directions from authorities. Children with special health care tor at their home. These families would also benefit from notifying
needs, whether because of physical or cognitive disabilities, require their local EMS agency and hospital of their child’s medical needs,
specific attention. The pediatrician can assist schools and child care because some EMS systems keep a registry of children with special

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 Chapter 83: Disaster Preparedness 601

needs. Some communities have developed systems in which posters Depending on availability of human milk, newborns and infants may
are disseminated for placement in a window of the home specifying require formula and a sterile water supply. The food needs of young
if any occupant may require special services from EMS in the event children differ from those of adults. Stockpiling of medications for
of a disaster or terrorist attack. Additionally, the American Academy biological, chemical, or radiation disasters must take into consider-
of Pediatrics (AAP) and American College of Emergency Physicians ation dosing differences for children compared with adults. It is nec-
offer an emergency information form that can be completed with essary to make available suspensions of medications in addition to pill
the pediatrician and should be part of the emergency prepared- forms. Furthermore, many recommended antidotes and treatments
ness kit. The emergency information form contains information are not approved for use in the pediatric population, and policies on
on diagnoses, procedures, medications, common presenting prob- the risks and benefits of their use in disasters should be established.
lems, and suggested medical management (see Online Resources). Emergency medical services systems are charged with the ini-
The family of a child with special needs can also contact the National tial and rapid triage of all victims. Various well-known triage algo-
Organization on Disability (www.nod.org) or Family Voices (www. rithms are available, including sort, assess, lifesaving interventions,
familyvoices.org) for more detailed information on preparing for treatment/transport (SALT) and simple triage and rapid treatment
a disaster. (START). Common to all is the rapid sorting of patients based on
During the recovery phase after a disaster, children and adoles- ability to ambulate followed by assessment of respiratory status,
cents may develop chronic medical problems as a result of injuries circulation/perfusion, and motor skills. Although multiple triage tools
sustained during the event. Beyond physical injuries, all disasters exist, the physiologic parameters and mental status assessments
have a psychological effect on children. The experiences and effects used in adult-based algorithms may not be suitable for children
of disaster are unique to each patient, and the pediatrician must of all ages. Triage systems must take into account physiological
individualize treatment accordingly. Multiple studies of various differences of children as well as their psychological response to
types of disasters demonstrate the increase in mental health symp- strangers. For example, young children may not be able to com-
toms among children and adolescents exposed to a disaster. This municate their complaint, and because their vital signs are nor-
is true even if a family is not directly affected by the disaster but mally different from those of adults, medical personnel accustomed
is exposed to the event within the community, on television, or to working with adults may misinterpret physical findings and
through the internet and social media. A child may present with overtriage children. JumpSTART is a widely recognized pediatric-
somatic symptoms, such as headaches and abdominal pain, or may specific disaster triage tool that parallels START but is customized
not want to participate in his or her normal activities. Long-term to address a child’s developmental ability and age-appropriate vital
effects include depression, anxiety, aggression, and substance abuse. signs. However, it fails to capture children who are dependent on
Age-appropriate discussions should be encouraged along with vali- technology or those with special health care needs. Whether or not
dation of the child’s concerns while assuring the safety of the individ- a triage tool is readily available, clinical decision making can be
ual child. Posttraumatic stress disorder should be considered in the relied on to help sort and triage victims in a disaster.
differential diagnosis of the patient with persistent symptoms that Ideally, children should remain with their caregiver as part of
do not respond to family support. Families and health profession- family-centered disaster care. If this is not possible, it is necessary
als can obtain further information through the AAP (www.aap.org) for a child advocate to be with the child at all times, although the
and the Substance Abuse and Mental Health Services Administration nature of disaster response may make this challenging if not planned
(www.samhsa.gov). in advance. Additionally, incorporation of child life specialists and
techniques for distraction during medical procedures should be
encouraged. Children may not respond well to new environments
The Role of the Pediatrician in the and disaster protocols. The simple process of decontamination can
Community be devastating to a young child without the presence of a parent or
Many state and regional disaster preparedness plans are tailored other familiar caregiver. A child may have concerns about being
for an adult population and may not consider the special needs of sprayed with water or may refuse to disrobe in front of strangers,
children. The pediatrician can participate in the development of a which may affect the success of decontamination for children and
community-wide disaster preparedness plan (eg, identifying emer- adolescents. It is assumed that adults will comply with protocols,
gency meeting locations) as well as surveillance to identify potential but such compliance is less predictable in a pediatric population. A
disasters as part of the planning phase of disaster response. From an child may be afraid of strangers or may simply wander off before tri-
operational standpoint, it is more effective to have 1 plan that can age is complete. Nonmedical personnel or bystanders may be called
take into consideration the needs of multiple vulnerable populations on to assist with supervising ambulatory children.
rather than a separate disaster preparedness plan for each population. Facility-based issues must also be addressed in regional disaster
The pediatrician should serve as a consultant about local preparation preparedness plans. These include providing for increased staffing
and provide guidance about the unique medical, nutritional, and psy- in adult facilities caring for children as well as the need for stockpil-
chological needs of children. For example, increased staffing needs ing of pediatric supplies at those facilities. Similarly, parents may be
should be anticipated when caring for younger children and infants. triaged with their children, so pediatric facilities should be prepared

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 602 PART 5: ACUTE AND EMERGENT PROBLEMS

to manage adult victims as well. Additionally, facilities need to plan symptoms with an associated high fatality rate. Fever and dyspnea
for children arriving without a caregiver and establish an identifi- associated with a widened mediastinum are common and may pro­
cation system that allows children to be reunited with their fami- gress to shock. Ciprofloxacin and doxycycline are recommended for
lies. This was a significant problem for children displaced during prophylaxis and treatment among adults. Despite the risks to bone
Hurricane Katrina. Strategies to address this include using digital and cartilage that generally restrict its use to healthy children, cip-
cameras to photograph children on arrival with their original cloth- rofloxacin is approved by the US Food and Drug Administration
ing as a means of facilitating family reunification. (FDA) for use in children with inhalational anthrax exposure.
Doxycycline should generally be avoided in children younger than
8 years, although it may be considered on a case-by-case basis. The
The Role of the Pediatrician in physician must consider consulting with experts to assist in assess-
Disaster Surveillance and Management ing the risks and benefits associated with using these medications.
Pediatricians function as key public health workers. Their knowl- Among the viruses, variola, more commonly known as smallpox,
edge and diligence aids in local and regional surveillance for poten- is an agent of concern. After its global eradication in 1980, chil-
tial chemical, biological, and radiation disasters. Families may seek dren were no longer immunized, leaving all children and most
care from their pediatrician rather than an emergency department adults susceptible to the virus. Similar to varicella (ie, chickenpox),
for early symptoms during and after a disaster. Although it is beyond it presents with vesicles with umbilicated centers but is associated
the scope of this chapter to provide details about signs and symp- with a higher mortality rate of 3% to 30% among nonimmunized
toms after every type of disaster, important concepts in identify- individuals. Exposure to the potent botulinal toxin results in cra-
ing and treating patients with exposures to chemical and biological nial nerve disturbances, descending paralysis, and respiratory dis-
agents as well as radiation are highlighted herein. tress. Ricin, which is derived from the castor bean, is another potent
Chemical exposures usually result in immediate symptoms and toxin. Inhalation results in fever, cough, and pulmonary edema,
require special protection for emergency personnel as well as decon- often resulting in death within days. Ingestion presents with severe
tamination for the victims. These exposures can occur from terror- vomiting and diarrhea, resulting in hypovolemic shock. For a com-
ism as well as (more commonly) industrial accidents. Insecticides, plete list of biological agents, presenting symptoms, and potential
herbicides, and nerve gases are organophosphates that inhibit the treatment or prophylaxis, physicians should consult the Centers for
enzyme acetylcholinesterase. This results in the accumulation of Disease Control and Prevention.
acetylcholine and excessive cholinergic stimulation at muscarinic Radiation exposure may occur as a result of damage to a facility
and nicotinic receptors. Symptoms include the muscarinic SLUDGE containing nuclear material, detonation of a nuclear weapon, or dis-
toxidrome (increased salivation, lacrimation, urination, diaphore- persal of nuclear material by a radioactive dispersal device. Ionizing
sis, gastric distress, and emesis) as well as the MTWHF nicotinic radiation presents the greatest health risk because of its high-
toxidrome (mydriasis, tachycardia, weakness, hypertension, and frequency energy. It causes chromosomal breaks in cells that can cause
fasciculation). Vesicant exposure, such as mustard gas and lewisite, long-term damage and increased risk of cancer. The 5 types of ion-
causes irreversible damage to mucous membranes, skin, and the izing radiation with specific characteristics, behaviors, and toxicities
respiratory system soon after exposure. Cyanide is another common are alpha particles, beta particles, gamma rays, x-rays, and neutrons.
chemical agent, known for its bitter almond taste. Cyanide inhibits Alpha particles have limited ability to penetrate but when inhaled
cellular metabolism and causes rapid hypotension, coma, seizures, or ingested can cause internal damage. Beta particles are most com-
and death. Agents other than nerve agents usually do not result monly found in a medical setting, and they have greater penetration
in severe mortality but rather incapacitate the victim. Many other than alpha particles. Beta particles can cause skin damage as well as
chemical agents from industrial accidents can cause a variety of skin damage when ingested. Gamma rays and x-rays are part of the elec-
and pulmonary symptoms. tromagnetic spectrum. Gamma rays are high energy and cause sig-
Biological agents include bacteria, viruses, and preformed tox- nificant damage. This type radiation would be seen after a nuclear
ins. These agents may be easy to disperse and can affect large pop- detonation or from radioactive materials. Much less common are
ulations. Unlike in chemical exposures, the onset of symptoms is neutrons, which induce radioactivity. Exposure to radiation is clas-
delayed by hours to days, and symptoms are more difficult to dis- sified as external, internal, whole body, and partial body. The effects
tinguish from common ailments. Secondary transmission of the of radiation can directly damage the target tissue, or the effects can
infection is also of concern with some agents. Management of bio- be indirect, caused by the creation of free radicals. Tissue sensitivity
logical disaster requires detailed surveillance and containment of is based on the cellular rate of division and level of differentiation.
exposed populations. The most sensitive to least is as follows: lymphoid, gastrointestinal,
Although there are too many biological agents to discuss in any reproductive, dermal, bone marrow, nervous system. The severity
detail in this brief chapter, a few of particular relevance to disas- of exposure is also dependent on the dose of radiation, type of radi-
ter planning are mentioned here. Anthrax, from Bacillus anthracis, ation, and age of the victim.
is a gram-positive sporulating rod. When used as a bioterrorism Radiation exposure is quantified by the amount of energy
agent in its inhaled form, victims present with severe influenza-like absorbed (ie, rad [radiation absorbed dose]) and the relative

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 Chapter 83: Disaster Preparedness 603

biological effectiveness of doses (RBE) based on the type of ioniz- volunteer do so under the auspices of an official disaster agency
ing radiation. The rem is the product of the rad and RBE. Under the or recognized relief organization to ensure the greatest protec-
International System of Units, the rad and rem are being replaced tion from liability.
by the gray (1 Gy = 100 rad) and sievert (1 Sv = 100 rem). Typically,
doses for common radiation exposures are given in millisieverts Conclusion
(1 mSv = 0.001 Sv). Radiation exposure from common radiographic The pediatrician has a vital role in predisaster, disaster, and postdi-
procedures can range from 0.1 mSv for a chest radiograph to 2 to saster management on the local, regional, state, and national level,
20 mSv for a computed tomography scan. not only as a medical service professional but also as an advocate for
Symptoms associated with radiation exposure depend on the the special needs of children and their families. The essential com-
total exposure. Nausea and vomiting can present with exposures ponents of disaster management are to provide for all basic human
of 0.75 to 1 Gy and lymphoid and bone marrow suppression with requirements, reduce an individual’s vulnerability to disasters, and,
exposures of 1 to 6 Gy. The mean lethal dose, the radiation dose for after a disaster has occurred, reduce the exposure risk. The pedia-
which one-half of the population is expected to die within 60 days, trician can educate and assist families in preparing for disasters.
is 4 Gy. Long-term effects of radiation include increased incidence Additionally, the pediatrician can guide communities in their disaster
of cancer and psychological distress. Evacuation is the ideal inter- preparedness planning to accommodate the particular vulnerabil-
vention to decrease exposure, but this may not be feasible in a timely ities of children. As with other health professionals, pediatricians
fashion in highly populated areas. can also contribute to the essential medical and public health work-
Seeking shelter can greatly decrease the level of exposure, with force during a disaster. Pediatricians can access the most current
large cement structures providing the best protection. The use of guidelines and recommendations through multiple professional and
potassium iodide is effective in exposures to radioactive iodine, governmental resources. It is imperative for the physician to have
which is associated with nuclear power facilities. It can be dispensed easy access to telephone numbers and websites specific to pediatric
in a pill and in suspension form. Dosage is based on level of radiation disaster preparedness and response for the relevant local, state, and
exposure and patient age, and physicians should consult the FDA federal agencies.
(www.fda.gov) or the US Nuclear Regulatory Commission (www.
nrc.gov) to determine the appropriate dosage of potassium iodide
depending on the level of radiation exposure. For individuals seek-
ing medical care, containment and decontamination are essential. CASE RESOLUTION
Removal of clothing and washing the skin with warm water is quite The family is relieved to discuss the importance of preparing for a disaster.
effective. Supportive medical care is essential in managing patients They now have an idea of what is involved in disaster preparation and feel less
with radiation exposure. Radiation results in significant immune vulnerable. They plan to create and store an emergency kit with a 3-day supply of
suppression, neutropenia, and lymphocytopenia, which last for food, water, and medications as well as a first aid kit. Additionally, they will refer
weeks and need close monitoring. The physician should be aggres- to the FEMA application for further recommendations. Together with their pedia-
trician, they complete an emergency information form for the kit. In the event of
sive in managing infections and consider treatments to increase
a disaster, they plan to stay together. The mother also shares her plan to call their
bone marrow regeneration. Expert consultation in radiation sick- local utility company to identify their house as a priority during a power failure
ness would be prudent. and indicates she will consider purchasing a backup generator. Before leaving the
In addition to caring for patients, pediatricians need to take into office, the mother shares that her son has been sleeping less since the tornado and
consideration the well-being of their own family as well as that of does not want to go to school because he is afraid of being away from the family.
office staff. During a disaster, office staff may not be able to get to The pediatrician encourages the family to discuss the boy’s fears while ensuring
his safety. Having the son participate in making the emergency kit and creating a
work. For those able to report to work, extra supplies of food and family plan may help. A follow-up visit is scheduled to reassess his symptoms and
water must be available in case staff cannot return to their homes. An decide if further intervention is needed.
office disaster plan should be implemented with emergency contacts
and preparation for the staff ’s basic needs. Basic medical supplies
should be available to care for patients during a disaster. Depending
on the type and severity of the disaster, access to the office facility Online Resources
may be prohibited. Plans for backing up patient medical records
American Academy of Pediatrics
should be implemented as well as for alternative sites in which med- www.aap.org/disasters
ical services can be delivered. Children and disasters: disaster preparedness to meet children’s needs.
Physicians need to review their medical liability policies American College of Emergency Physicians
addressing the provision of care in a disaster situation. Most www.acep.org/disaster
policies only provide coverage for care that is provided in the EMS and disaster preparedness.
office setting. Good Samaritan laws vary in each state about what Centers for Disease Control and Prevention
level of protection is provided to the health professional. The AAP https://emergency.cdc.gov/children
recommends that during a disaster situation, pediatricians who Caring for children in a disaster.

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 604 PART 5: ACUTE AND EMERGENT PROBLEMS

Family Voices Baker LR, Cormier LA. Disaster preparedness and families of children with spe-
http://familyvoices.org/wp-content/uploads/2010/10/Disasters_Emergencies- cial needs: a geographic comparison. J Community Health. 2013;38(1):106–112
tip-sheet-final-5.23.18.pdf PMID: 22821052 https://doi.org/10.1007/s10900-012-9587-3
Disasters and emergencies: keeping children and youth safe. Cicero MX, Baum CR. Pediatric disaster preparedness: best planning for the
National Safety Council worst-case scenario. Pediatr Emerg Care. 2008;24(7):478–481 PMID: 18633312
www.nsc.org/safety_home/emergencypreparedness/Pages/Emergency https://doi.org/10.1097/PEC.0b013e31817e2f2d
Preparedness.aspx Gausche-Hill M. Pediatric disaster preparedness: are we really prepared?
Emergency preparedness: are you ready for a disaster? J Trauma. 2009;67(2 suppl):S73–S76 PMID: 19667856 https://doi.org/10.1097/
US Department of Health and Human Services Assistant Secretary for TA.0b013e3181af2fff
Preparedness and Response Hagan JF Jr; American Academy of Pediatrics Committee on Psychosocial
www.phe.gov/Preparedness/planning/abc/Pages/webinar-resources- Aspects of Child and Family Health; Task Force on Terrorism. Psychosocial
130620.aspx implications of disaster or terrorism on children: a guide for the pediatrician.
Pediatric preparedness for healthcare coalitions. Pediatrics. 2005;116(3):787–795. Reaffirmed November 2014 PMID: 16140724
https://doi.org/10.1542/peds.2005-1498
Selected References Markenson D, Reynolds S; American Academy of Pediatrics Committee on
American Academy of Pediatrics. Pediatric Terrorism and Disaster Preparedness: Pediatric Emergency Medicine; Task Force on Terrorism. The pediatrician and
A Resource for Pediatricians. Foltin GL, Schonfeld DJ, Shannon MW, eds. disaster preparedness. Pediatrics. 2006;117(2):e340–e362. Reaffirmed June 2009
Rockville, MD: Agency for Healthcare Research and Quality; 2006. AHRQ PMID: 16452341 https://doi.org/10.1542/peds.2005-2752
Publication No. 06(07)-0056. https://archive.ahrq.gov/research/pedprep. Nakayama T, Tanaka S, Uematsu M, et al. Effect of a blackout in pediatric
Accessed August 1, 2019 patients with home medical devices during the 2011 eastern Japan earthquake.
American Academy of Pediatrics Disaster Preparedness Advisory Council and Brain Dev. 2014;36(2):143–147 PMID: 23452913 https://doi.org/10.1016/
Committee on Pediatric Emergency Medicine. Ensuring the health of children j.braindev.2013.02.001
in disasters. Pediatrics. 2015;136(5):e1407–1417 PMID: 26482663 https://doi. Olympia RP, Rivera R, Heverley S, Anyanwu U, Gregorits M. Natural
org/10.1542/peds.2015-3112 disasters and mass-casualty events affecting children and families: a
American Academy of Pediatrics Committee on Pediatric Emergency description of emergency preparedness and the role of the primary care phy-
Medicine and Council on Clinical Information Technology; American College sician. Clin Pediatr (Phila). 2010;49(7):686–698 PMID: 20356922 https://doi.
of Emergency Physicians Pediatric Emergency Medicine Committee. Policy org/10.1177/0009922810364657
statement—emergency information forms and emergency preparedness for chil- Sakashita K, Matthews WJ, Yamamoto LG. Disaster preparedness for tech-
dren with special health care needs. Pediatrics. 2010;125(4):829–837. Reaffirmed nology and electricity-dependent children and youth with special health care
October 2014 PMID: 20351008 https://doi.org/10.1542/peds.2010-0186 needs. Clin Pediatr (Phila). 2013;52(6):549–556 PMID: 23539684 https://doi.
American Academy of Pediatrics Disaster Preparedness Advisory Council, org/10.1177/0009922813482762
Committee on Pediatric Emergency Medicine. Ensuring the health of children Tanaka S. Issues in the support and disaster preparedness of severely disabled
in disasters. Pediatrics. 2015;136(5):e1407–e1417 PMID: 26482663 https://doi. children in affected areas. Brain Dev. 2013;35(3):209–213 PMID: 23312950
org/10.1542/peds.2015-3112 https://doi.org/10.1016/j.braindev.2012.09.008

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 PART 6

Head, Neck, and

Respiratory System
84. Approach to the Child With Dysmorphism....................607
85. Craniofacial Anomalies....................................................613
86. Common Oral Lesions......................................................621
87. Otitis Media.......................................................................627
88. Hearing Impairments.......................................................635
89. Sore Throat........................................................................645
90. Nosebleeds.........................................................................655
91. Strabismus.........................................................................661
92. Infections of the Eye.........................................................667
93. Excessive Tearing..............................................................673
94. Neck Masses......................................................................677
95. Allergic Disease.................................................................687
96. Wheezing and Asthma.....................................................699
97. Cough................................................................................713

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 CHAPTER 84

Approach to the Child

With Dysmorphism
Henry J. Lin, MD, and Moin Vera, MD, PhD

CASE STUDY
A 13-year-old boy presents to the office for the first time On physical examination, the boy is at greater than
for an evaluation after moving to the area. His parents the 90th percentile for height and weight. He exhibits
note that he has unexplained intellectual disability and mild prognathism with large ears. His fingers are hyper-
has had problems with hyperactivity in school. The preg- extensible. A complete physical examination reveals
nancy was uncomplicated and the mother, who was a that his testicles appear large (6 cm), and his sexual
32-year-old gravida 1, para 1 at the time of the child’s maturity rating (ie, Tanner stage) is 3. The rest of the
birth, denies alcohol or drug use or exposure to any examination is normal.
teratogens during pregnancy. Delivery was by cesarean
section secondary to cephalopelvic disproportion, but
Questions
1. What history is important to elicit in evaluating a
the Apgar score was 8 at 1 minute and 9 at 5 minutes.
child with dysmorphic features?
As a newborn the patient was noted to have macro-
2. What are the possible causes of errors in
cephaly and to be large for gestational age. He did well
morphogenesis?
in the newborn period and had no feeding problems.
3. What clues on physical examination can aid in
Subsequently, he had no significant medical illnesses,
establishing a specific diagnosis?
including no seizures, but at 1 year of age he was noted
4. What laboratory tests can confirm a diagnosis?
to be developmentally delayed. This delay continued,
5. When is it appropriate to obtain a genetics consulta-
and he has been in special education classes throughout
tion or refer a patient for genetic counseling?
his schooling. Family history is negative for any relatives
6. What are the benefits of establishing a specific
with disabilities.
diagnosis?

Evaluation for structural anomalies is an essential part of all intellectual disability; necessitate significant surgical procedures;
pediatric examinations. Visible errors in morphogenesis are a source are disfiguring; or interfere with physical performance.”
of potentially useful information in the evaluation of a patient with
abnormal symptoms, such as seizures. Additionally, major malfor- Epidemiology
mations frequently require treatment, and the presence of 1 anom- Structural anomalies are common in the general population. Most
aly suggests that others may also exist. are minor. In the first comprehensive analysis of minor structural
The study of congenital defects was termed dysmorphology by anomalies, Marden in 1964 reported that 7% to 14% of newborns
David Smith, MD, in 1966. The anomalies fall into 2 categories: have at least 1 minor anomaly on surface examination. Other studies
minor and major. Minor malformations are those of “no medical or indicate that up to 40% of newborns have 1 anomaly. The presence of
cosmetic consequence to the patient.” An example is a supernumer- 3 or more minor malformations has predictive value in identifying
ary nipple that appears as a hyperpigmented papule along the nipple a major malformation. Among newborns, 0.8% have 2 minor mal-
line. Identification of minor malformations is important, because formations, and 11% of these patients have a major malformation.
they may indicate the presence of a more generalized pattern of mal- Three or more minor malformations occur in 0.5% of newborns,
formation. Major malformations are those that have “an adverse and 90% of these patients have a major malformation. Data from
effect on either the function or social acceptability of the individual.” the National Collaborative Perinatal Project revealed that 44.8% of
Cleft lip and palate are major malformations that have functional these anomalies were craniofacial manifestations and 45.3% were
as well as cosmetic relevance to the patient’s health. Severe congen- skin abnormalities. Autopsies of expired fetuses show an increased
ital malformations as defined by the Centers for Disease Control incidence of minor and major malformations. Males are affected with
and Prevention are “defects that cause death, hospitalization or minor malformations more often than females. Frequencies of minor

607

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 608 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

and major malformations vary along racial lines, depending on the

Box 84.1. Pathophysiology of Structural Defects
specific malformations. For example, postaxial polydactyly occurs
in 16 of 10,000 births among whites and in 140 of 10,000 births Malformation
among blacks. Hemangiomas occur in 350 of 10,000 births among ww Chromosomal abnormality
whites, whereas the frequency among blacks is 100 in 10,000 births. ww Single gene disorder
Three percent of all pregnancies produce a child with a signifi- Deformation
cant genetic disease or birth defect. These malformations account Disruption
for a great proportion of morbidity and mortality in the pediatric ww Vascular compromise
population. Of all pediatric hospital admissions, 33% to 50% involve ww Viral infection
a child with a disease with a genetic component. ww Mechanical (eg, amniotic bands)
ww Teratogens (eg, alcohol, drugs, irradiation)
Clinical Presentation
Dysplasia
With the advent of various prenatal tests and diagnostic modal- ww Metabolic or structural protein disorder
ities, detection of anomalies may occur during pregnancy.
Abnormalities on routine prenatal screening raise the possibil-
ity of finding an anomaly in the newborn. For example, an ele- of that structure. An example of a malformation is an endocardial
vated maternal a-fetoprotein may be indicative of a neural tube cushion defect in a patient with Down syndrome. Malformation
defect. Amniocentesis or chorionic villus biopsy may reveal syndromes frequently are caused by chromosomal abnormalities,
abnormal chromosomes associated with malformations. Standard single-gene disorders involving genes that program for structure, or
prenatal ultrasonography examinations can demonstrate struc- environmental teratogens. Teratogens interfere with organ develop-
tural defects in utero, which can be further delineated on spe- ment during a critical period in embryogenesis, resulting in organ
cific fetal ultrasonography examinations. More recently, maternal dysgenesis. Alcohol, certain drugs, x-ray irradiation, viral infections,
plasma cell-free DNA has been used for noninvasive prenatal and other environmental exposures can all be teratogens. More than
testing for trisomy syndromes. 30 drugs have been proven to be teratogenic.
Most structural anomalies develop during the first trimester Deformation results from exertion of mechanical pressure
of gestation and, if not detected prenatally, are noted in the deliv- by external forces on the developing fetus. This mechanism
ery room or newborn nursery (see Chapter 25). Many major con- involves no intrinsic defect of the fetus. Deformation associated
genital defects are obvious on a thorough physical examination. with intrauterine forces can be caused by uterine constraint,
Other major defects, such as a tracheoesophageal fistula, are not an abnormally shaped uterus, or multiparous pregnancy. For
evident on clinical examination but become readily apparent as example, flexible talipes equinovarus (ie, clubfoot) is a defor-
the neonate adapts to extrauterine life and experiences difficul- mation caused by uterine constraint. Postnatal deformations can
ties with feeding. Minor anomalies may be overlooked on initial also occur, such as plagiocephaly caused by an infant sleeping
newborn examination. If developmental disability manifests by on his or her back.
age 6 months in an infant who exhibited minor anomalies as a With disruption, an agent outside the fetus causes cell death,
newborn, however, the physician should thoroughly evaluate the resulting in a permanent defect in fetal development. Disruptive
infant for minor anomalies that may aid in identifying the cause events result in tissue destruction. Examples of disruptive events
of the condition. are tissue ischemia secondary to vascular compromise, viral
Sometimes dysmorphic features are not present at birth but infection at a critical gestational age (eg, Zika virus infection),
become apparent as the child grows and develops. Such features and mechanical interference with normal development. An
may be associated with dysplasias, that is, defects in cellular metab- amniotic band is an example of a mechanical disruptive agent;
olism that manifest after birth (eg, skeletal dysplasia that becomes an amniotic band can cause amputation of a developing limb by
more apparent as the bones grow). restricting blood flow to it.
Dysplasias, which often are caused by a single-gene defect,
Pathophysiology are structural abnormalities that occur as the result of abnormal
In terms of pathophysiology, structural defects can be separated cellular metabolism and/or organization. Dysplasia can be appar-
into 4 different types of errors in morphogenesis: malformation, ent in utero (and therefore at birth [eg, skeletal dysplasia]) or later.
deformation, disruption, and dysplasia (Box 84.1). All these types Mucopolysaccharidosis type I causes dysplasias that manifest
of errors can result in a sequence of abnormalities. postnatally secondary to absence of the lysosomal hydrolase,
Previously, the term “malformation” was used descriptively to a-L-iduronidase. Glycosaminoglycans accumulate in parenchymal
denote an anomaly; however, it is also used to denote a specific and mesenchymal tissues after birth. Affected children develop
pathogenic mechanism. A malformation is a permanent defect in coarse facies, an enlarged tongue, misshapen bones, and hepato-
a structure caused by an intrinsic abnormality in the development splenomegaly, among other features.

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 Chapter 84: Approach to the Child With Dysmorphism 609

Differential Diagnosis A syndrome is a constellation of anomalies that are pathoge-

netically related. Chromosomal abnormalities (eg, trisomy 21),
In generating an appropriate differential diagnosis for a child
single-gene disorders, and teratogens can produce syndromes.
with dysmorphism, it is essential to identify all minor and major
Alcohol is the most common teratogen to which fetuses may be
malformations. The differential diagnosis varies depending on the
exposed. Alcohol exposure in utero causes growth failure, intellec-
specific findings. A thorough patient history and physical exami-
tual disability, microcephaly, a short nose, and small distal phalanges
nation are necessary to aid in establishing the list of conditions to
(see Chapter 147). Children with intellectual disability and structural
consider. For the patient with multiple anomalies, identifying the
anomalies are at increased risk for having a syndrome and should
most specific and rarest anomaly can direct the health professional
undergo genetic or genomic testing.
to a narrower list of possible diagnoses. For example, nail hypoplasia,
which occurs in fetal hydantoin syndrome, is much rarer than
congenital cardiac disease, which occurs in multiple syndromes.
Evaluation
The health professional should be familiar with the prominent fea- History
tures of the more common syndromes. Reference texts, such as A complete history is essential (Box 84.2). The history begins pre-
Smith’s Recognizable Patterns of Human Malformation, are help- natally with information from the mother on duration of pregnancy,
ful in establishing differential diagnosis lists and in finalizing diag- possible teratogen exposure, fetal activity, diagnostic test results,
noses. In addition, several helpful websites are available, such as and any complications of pregnancy. The delivery history should
OMIM (Online Mendelian Inheritance in Man), PubMed, Google include information on the type of delivery, newborn presentation,
and Google Scholar, and others that were specifically developed for and size at birth, including growth percentiles. Neonatal adaptation
the delineation of syndromes and genetic disorders. and feeding patterns are important parameters to assess. The sub-
In evaluating a neonate or an infant with congenital anoma- sequent medical history also should be obtained. The identification
lies, the physician should attempt to separate the findings into 1 of of associated intellectual disability is an essential part of the history.
5 categories: an isolated defect, a developmental field defect, a birth A thorough family history is equally important, including a ped-
defect association, a sequence pattern, or a dysmorphic syndrome. igree. Specifically, the family should be questioned about paren-
The first step is to determine whether the anomaly is isolated. If so, tal ages, information pertaining to possible consanguinity, and any
does it represent a failure in development in 1 location, such as a history of fetal loss or early infant deaths. Family histories of birth
cleft lip? Most isolated anomalies are believed to have a multifacto- defects or disabilities should be documented.
rial inheritance, representing the interaction between multiple genes
and unknown external influences. Typically, the risk of recurrence Physical Examination
for isolated anomalies in future pregnancies is 2% to 5%. The physical examination should be extremely thorough, and all
A developmental field defect is a pattern of anomalies that morphologic findings should be noted. As previously mentioned, the
occurs in structures that are in close physical proximity during craniofacial area and skin are common sites for anomalies, but all
embryologic development. The defects may involve a limited organ systems should be extensively evaluated. Even minor anom-
region and may be the result of a single disruptive event (eg, vas- alies, such as a supernumerary nipple or clinodactyly, may have
cular compromise); thus, the risk for recurrence in subsequent
pregnancies is low. One example of such a defect is hemifacial
microsomia (ie, oculoauriculovertebral defect), which is believed Box 84.2. What to Ask
to be caused by disruption of vascular flow to the first and Child With Dysmorphism
second branchial arches. The disruption results in hypoplasia ww How long was the pregnancy?
of the malar, maxillary, and mandibular region on 1 side with ww Did the mother take any medications, smoke cigarettes, or use alcohol or
associated microtia and vertebral defects. any illicit drug?
Birth defect associations are those in which a combination of ww What was the fetal activity?
anomalies occurs together frequently but the pattern does not fit ww Were there any complications of pregnancy?
a known field defect or syndrome. Etiologies of association defects ww What was the type of delivery and presentation of the baby?
may be unknown, and risk for recurrence in subsequent pregnancies ww What was the newborn’s size at birth?
may be low. One of the more common associations is the VACTERL ww How did the newborn feed?
association (vertebral anomalies, anal atresia, cardiac defects, ww What is the subsequent medical and developmental history?
tracheoesophageal fistula, renal defects, and limb defects). ww Are the parents related?
A sequence pattern of anomalies occurs when 1 malformation ww What are the ages of the parents?
results in multiple dysmorphic features. For example, oligohydram- ww Is there a history of fetal or infant deaths?
nios sequence with Potter facies results from renal agenesis. The ww Is there a history of birth defects in the family?
renal malformation causes oligohydramnios, which in turn causes ww Do any family members have disabilities?
fetal joint contractures, pulmonary hypoplasia, and a flattened face.

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 610 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

significance. Objective measurements should be obtained when Academy of Neurology recommends routine neuroimaging with
possible. Normal growth curves are available for evaluating mea- magnetic resonance imaging.
surements of the face (and other body parts), such as inner canthal
distances, palpebral fissure lengths, and ear lengths. Unusual hair Management
whorl patterns and dermatoglyphics (eg, appearance of the palmar Children who present with dysmorphic features should be evaluated
creases) should be noted. Physical data, including height, weight, to determine a specific diagnosis, if possible. Obtaining a diagnosis
and head circumference, should be plotted and the growth percen- is of vital importance for patient care and parental counseling.
tiles checked. A complete ophthalmologic evaluation may be indi- Knowing the diagnosis can direct testing for associated abnormal-
cated to detect abnormalities such as cataracts or cherry-red spots. ities. Treatment options may be available, and a prognosis can be
established. Defining the developmental prognosis for children is
Laboratory Tests essential for school planning. If intellectual disability is not asso-
In assessing children who appear dysmorphic, the physical examina- ciated with the diagnosis, such as with cleft lip and palate, the pri-
tion is the most important part of the evaluation. Findings on phys- mary care physician should reassure the parent or parents of that.
ical examination help guide selection of laboratory tests. Additional Parents also need to know the risk for recurrence for future children.
studies may then confirm a suspected diagnosis. Similarly, when pre- Occasionally, further testing of parents may be needed to accurately
natal screening detects an abnormality, specific prenatal tests may determine recurrence risks. For example, the risk for a chromosomal
be used to assess the fetus for associated disorders. abnormality in a subsequent child is increased if 1 of the parents is
Cytogenetic testing has been a major tool for evaluating a carrier of a balanced chromosome translocation.
children suspected of having a chromosome disorder, and it also has Health supervision strategies have been established for specific
been used for children with malformations and intellectual disabil- disorders. For instance, published guidelines recommend hear-
ity. Chromosomal microarrays have largely replaced standard chro- ing, ophthalmologic, and thyroid screening, among other tests, for
mosomal analysis, because microarrays are better for detecting copy patients with Down syndrome. The field of genetic diseases advances
number variants, such as chromosome deletions or duplications. rapidly, and it is often difficult for the primary care physician to
The older methods (eg, G-banded karyotyping, fluorescence in situ stay informed. A geneticist can provide guidance on up-to-date
hybridization) are still used in certain situations, however, such as in recommendations.
distinguishing between trisomy 21 versus Down syndrome involv- Parents may wish to be referred to support groups for specific
ing a translocation. conditions. Such groups can be invaluable in helping parents under-
Whole-exome sequencing and gene sequencing panels have stand their child’s condition and adjust to the disorder. They can also
become important components of dysmorphology evaluations and advise parents about community and educational resources and help
are often performed as first-line testing. Whole-exome sequenc- parents advocate for their child’s unique needs.
ing is a powerful tool for uncovering single-gene disorders and has Referral to a genetic counselor can provide parents with infor-
also been used to detect Turner syndrome, which is a chromosomal mation about prenatal testing, recurrence risk, and alternatives
disorder (45,X). Whole-genome sequencing is starting to be used for addressing recurrence risks. Counseling is extremely useful in
clinically and offers greater detection ability compared with whole- helping parents understand the mode of inheritance.
exome sequencing, because sequences obtained are not limited to
coding regions. Consultation with a medical geneticist is helpful in
selecting the most appropriate tests for specific situations. Also, the Prognosis
possibility of detecting findings of unknown clinical significance needs As with management, defining an accurate prognosis for each patient
to be addressed, before whole-exome or -genome sequencing is used. depends on recognition of the specific condition. Some conditions,
Some syndromes can be detected by metabolic testing. For exam- such as trisomy 13, are lethal, whereas other conditions allow for a
ple, Smith-Lemli-Opitz syndrome is a malformation syndrome normal life span.
caused by a disorder in cholesterol metabolism. Plasma amino acids, Malformations are permanent defects that generally have a recur-
urine organic acids, and a plasma acylcarnitine profile are examples rence risk. They may be correctable with surgery or treatment, but
of metabolic tests that are widely available. Many of the analytes on frequently there will be residual disability.
these tests are also included in state newborn screening protocols. Deformations usually resolve with treatment and have no recur-
rence risk, except in cases in which the deformation is secondary to
Imaging Studies a uterine abnormality (eg, bicornuate uterus).
Imaging studies are extremely useful in deriving information on A disruption may be treated with surgery or therapy to improve
internal malformations. Radiographs, including skeletal surveys, function; however, as with malformations, residual disability fre-
may detect skeletal anomalies. Echocardiography, renal ultraso- quently remains. For disruption resulting from tissue ischemia or
nography, computed tomography, and magnetic resonance imag- a mechanical agent, no recurrence risk is expected. For disruption
ing studies all can be used when appropriate. With the findings of resulting from a teratogen, however, the disruption may recur with
anomalies and global developmental delay in a patient, the American exposure to the same teratogen.

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 Chapter 84: Approach to the Child With Dysmorphism 611

Dysplasias tend to persist or worsen with time, unless a spe- and Genomics. Genet Med. 2017;19(2):249–255 PMID: 27854360 https://doi.
cific treatment is available. Generally, a risk for recurrence exists. org/10.1038/gim.2016.190
Specific treatments are available for only a limited number of Mefford HC, Batshaw ML, Hoffman EP. Genomics, intellectual disability, and
diseases, although progress is being made, such as in the area of autism. N Engl J Med. 2012;366(8):733–743 PMID: 22356326 https://doi.
enzyme replacement therapy. org/10.1056/NEJMra1114194
Meng L, Pammi M, Saronwala A, et al. Use of exome sequencing for infants in
intensive care units: ascertainment of severe single-gene disorders and effect on
medical management. JAMA Pediatr. 2017;171(12):e173438 PMID: 28973083
CASE RESOLUTION
https://doi.org/10.1001/jamapediatrics.2017.3438
The child has features that are suggestive of a dysmorphic syndrome. The most
specific finding on examination is macro-orchidism. This finding is associated Mestek-Boukhibar L, Clement E, Jones WD, et al. Rapid Paediatric Sequencing
with fragile X syndrome. The patient is referred to a genetic specialist for diag- (RaPS): comprehensive real-life workflow for rapid diagnosis of critically ill
nosis and counseling. Specific DNA-based molecular analysis is performed and is children. J Med Genet. 2018;55(11):721–728 PMID: 30049826 https://doi.
positive for a fragile site on the X chromosome at Xq27.3. org/10.1136/jmedgenet-2018-105396
The parents are counseled that this condition has an X-linked inheritance Moeschler JB, Shevell M; American Academy of Pediatrics Committee on
mode. The child will have a normal life span but may need early intervention Genetics. Clinical genetic evaluation of the child with mental retardation or
services as well as a special education program later in his schooling. He may developmental delays. Pediatrics. 2006;117(6):2304–2316 PMID: 16740881
not be capable of independent living as an adult. The primary care physician https://doi.org/10.1542/peds.2006-1006
will be notified of the diagnosis and coordinate further services. The parents are
Murdock DR, Donovan FX, Chandrasekharappa SC, et al. Whole-exome sequenc-
encouraged to attend a parents’ support group and consult with experts to learn
how their child’s full potential may be realized. ing for diagnosis of Turner syndrome: toward next-generation sequencing and
newborn screening. J Clin Endocrinol Metab. 2017;102(5):1529–1537 PMID:
28324009 https://doi.org/10.1210/jc.2016-3414
Platt FM. Emptying the stores: lysosomal diseases and therapeutic strategies. Nat
Selected References
Rev Drug Discov. 2018;17(2):133–150 PMID: 29147032 https://doi.org/10.1038/
Brent RL. The role of the pediatrician in preventing congenital malformations. nrd.2017.214
Pediatr Rev. 2011;32(10):411–422 PMID: 21965708 https://doi.org/10.1542/ Richer J, Laberge AM. Secondary findings from next-generation sequencing:
pir.32-10-411 what does actionable in childhood really mean? Genet Med. 2019;21(1):124–
Graham JM Jr, Sanchez-Lara PA. Smith’s Recognizable Patterns of Human 132 PMID: 29875419 https://doi.org/10.1038/s41436-018-0034-4
Deformation. 4th ed. Philadelphia, PA: Elsevier; 2016 Southard AE, Edelmann LJ, Gelb BD. Role of copy number variants in struc-
Harris S, Reed D, Vora NL. Screening for fetal chromosomal and subchromo- tural birth defects. Pediatrics. 2012;129(4):755–763 PMID: 22430448 https://
somal disorders. Semin Fetal Neonatal Med. 2018;23(2):85–93 PMID: 29128491 doi.org/10.1542/peds.2011-2337
https://doi.org/10.1016/j.siny.2017.10.006 Toriello HV. Role of the dysmorphologic evaluation in the child with develop-
Jones KL, Jones MC, Casanelles MD. Smith’s Recognizable Patterns of Human mental delay. Pediatr Clin North Am. 2008;55(5):1085–1098, xi PMID: 18929053
Malformation. 7th ed. Philadelphia, PA: Elsevier Saunders; 2013 https://doi.org/10.1016/j.pcl.2008.07.009
Kalia SS, Adelman K, Bale SJ, et al. Recommendations for reporting of second- Walker WO Jr, Johnson CP. Mental retardation: overview and diagnosis.
ary findings in clinical exome and genome sequencing, 2016 update (ACMG Pediatr Rev. 2006;27(6):204–212 PMID: 16740804 https://doi.org/10.1542/
SF v2.0): a policy statement of the American College of Medical Genetics pir.27-6-204

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 CHAPTER 85

Craniofacial Anomalies
Carol D. Berkowitz, MD, FAAP

CASE STUDY
A boy weighing 3,500 g (7.7 lb) is born by normal spon- hospital. Except for the cleft, the physical examination
taneous vaginal delivery to a 28-year-old gravida 3, para is normal.
3 mother after an uncomplicated term gestation. Apgar
scores are 9 and 10. On physical examination, the new-
Questions
1. What craniofacial anomalies are common in infants
born is well but has an incomplete, left-sided unilateral
and children?
cleft of the lip and palate.
2. What are feeding considerations in the newborn
No other family member has such a deformity, but
with cleft lip or palate?
the mother and father are distantly related. The mother
3. What is the appropriate timing of surgery for the
had prenatal care. During the pregnancy she had no ill-
more common craniofacial anomalies?
nesses, took vitamins but no other medications, and did
4. What are the major medical problems that children
not smoke, drink alcohol, or use illicit drugs.
with craniofacial anomalies, particularly clefts of the
The mother is planning to feed the newborn with
lip or palate, experience?
formula and wonders if she should do anything spe-
5. What is positional plagiocephaly? How is its preva-
cial. She is also wondering if her son’s lip deformity
lence related to supine sleeping?
can be repaired before she takes him home from the

A neonate may be born with a readily apparent craniofacial anom- clefts in subsequent offspring. Generally, the risk for recurrence of
aly, such as cleft lip, cleft palate, or microtia, or anomaly may emerge clefts is 4% to 7% for cleft lip with or without cleft palate and 3%
as an infant ages. The latter includes conditions that may be genet- for isolated cleft palate.
ically based but do not manifest until later, such as facial asym- Clefts may occur as isolated findings or as part of syndromes or
metry (ie, hemifacial microsomia) and premature closure of 1 or sequences. In Van der Woude syndrome, clefts of the lip or palate are
more sutures. Alternatively, these anomalies may be environmen- associated with lip pits. This condition is inherited in an autosomal-
tally influenced, such as positional or deformational plagioceph- dominant manner and is the most common cause of syndromic cleft
aly. Deformational plagiocephaly is defined as a condition in which lip. Currently, more than 500 Mendelian syndromes are associated with
an infant’s head and sometimes face are misshapen as a result of clefts, with approximately 30% of newborns with clefts having other
prenatal and, in recent years, postnatal external molding, which congenital anomalies associated with specific syndromes. Pierre Robin
occur on the infant’s malleable cranium. sequence includes micrognathia and glossoptosis (ie, retrodeviated
tongue) and a distinct U-shaped cleft. One theory relates the sequence
Epidemiology to failure of the fetal neck to extend normally, resulting in compression
The overall prevalence of cleft lip with or without cleft palate is 1 in of the mandible on the chest, thereby restricting its growth and caus-
1,000 and that of isolated cleft palate is 1 in 2,500 live births. Cleft lip ing malposition of the tongue, thus preventing closure of the palate.
with or without cleft palate is the second most common birth defect True craniosynostosis occurs in approximately 1 in 2,000 to
in the United States, after Down syndrome, with nearly 7,000 infants 3,000 live births, and this prevalence is the same in all ethnic groups.
with clefts born annually. Racial, ethnic, and geographic variation Sex variation exists among the different types of craniosynostosis.
exists in the prevalence of clefts. For example, the prevalence of Deformational plagiocephaly is reported in 25% to 45% of infants.
clefts in parts of the Philippines is 1 in 200. Similarly, cleft lip with The term plagiocephaly comes from the Greek plagio, meaning
or without cleft palate is most common among Asians and Native oblique, twisted, or slanted, and kephale for head. Firstborn and
Americans (1 in 500) and least common among blacks (1 in 22,500). male sex increase the risk of deformational plagiocephaly at birth.
The sex distribution varies with the type of cleft. Isolated clefts of the Most cases of deformational plagiocephaly resolve over time with-
palate occur twice as frequently in girls, but clefts of the lip with or out specific medical intervention.
without clefts of the palate appear twice as often in boys. Microtia is less common and occurs in 1 in 6,000 to 8,000 live
The type of cleft, the sex of the child, and whether a parent or births. Other ear malformations, such as auricular dystopia (ie, ear
sibling(s) is similarly affected influence the risk for recurrence of located on the check) or total atresia of the external area, are less

613

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 614 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

common and can be associated with other syndromes. Other ear

anomalies involve protuberant ears (sometimes referred to as “out-
standing ears”) or pinna with folds or flattened components (eg,
antihelix).

Clinical Presentation
Most craniofacial anomalies are readily apparent (Box 85.1). Some
anomalies, such as cleft lips or microtia, are noted immediately in the
delivery room. Other anomalies, such as craniosynostosis, develop A
over time. Because the onset of craniosynostosis may be gradual,
the parent(s)/guardian(s) may not recognize the condition, which
usually appears as asymmetry of the face or skull. Deformational
plagiocephaly also evolves over time and is more often noted by the
physician rather than the parent or guardian.
The child with craniofacial anomaly may also have medical
problems that occur secondary to the deformity. The newborn or
infant with cleft palate may present with failure to thrive because
of difficulty feeding. The older infant or the child may experience
recurrent otitis media, speech impairment, or psychosocial stress. B
Nasal regurgitation of liquids may occur in the child with obvious
palatal cleft or more subtle deformity, such as submucosal cleft of
the soft palate.

Pathophysiology
Clefts of the lip and palate (Figure 85.1) are believed to develop
as a result of an interruption in the merging of the middle and
lateral portions of the face during the sixth to seventh week of
gestation. The palate normally closes with an anterior to posterior
progression. Any interference with this progression (eg, tumor or
C
encephalocele in the roof of the mouth) leads to a cleft. A vascu-
lar disruption may also result in ischemia in the involved areas.
Figure 85.1. Cleft lips. A, Unilateral, complete cleft lip. B, Unilateral,
Although the etiology of clefts is not fully determined, it is felt
incomplete cleft lip. C, Bilateral, complete cleft lip.
to be multifactorial. Multiple genetic risk loci have been associ-
ated with nonsyndromic cleft lip with or without cleft palate. The
interferon regulatory factor 6 (IRF6) gene is consistently associated The presence of a cleft palate affects normal oropharyngeal func-
with nonsyndromic cleft lip and palate. As with other clinical condi- tioning, including sucking and speech. A child may exhibit hyper-
tions, genetic predisposition interacts with environmental factors to nasal speech caused by the escape of air through the nose and have
increase the risk of the emergence of a disorder. A newborn with the articulation problems. Recurrent otitis media seems to be related
A2 form of the transforming growth factor-α (TGFA) gene is 8 times to dysfunction of the eustachian tube.
more likely to have a facial cleft if the mother smokes. Other environ- Facial asymmetry may be the result of hemifacial microsomia
mental teratogens associated with clefts include hydantoin, alcohol, either in isolation or as part of a syndrome. Facial asymmetry that is
warfarin, trimethadione, thalidomide, aminopterin, and topiramate. only noted with crying is referred to as “asymmetric crying facies.”
The condition is present at birth and caused by congenital hypoplasia
of the depressor anguli oris muscle (CHDAOM). Although the disor-
Box 85.1. Diagnosis of Craniofacial Anomalies der may occur in isolation, it may also occur in association with other
ww Cleft of the lip or palate anomalies, including congenital heart disease (40%–50%), head and
ww Small, atretic, or malformed ear neck anomalies (45%–50%), skeletal defects (22%), and genitouri-
ww Asymmetry of the face nary anomalies. Syndromes with which CHDAOM has been associ-
ww Misshapen skull ated include CATCH 22, Cayler cardiofacial, VACTERL, and DiGeorge.
ww Recurrent otitis media As with other craniofacial anomalies, the presence of CHDAOM indi-
ww Speech impairment cates the need for a thorough physical examination for other findings.
ww Nasal regurgitation of liquids or foods Microtia, a small atretic pinna of the ear, results from failure of
development of the pinna and portions of the external auditory canal.

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 Chapter 85: Craniofacial Anomalies 615

It is most likely caused by a vascular accident during the 12th week FGFR3P25OR mutation has been reported in patients with non-
of gestation. Similar anomalies have been created in laboratory ani- syndromic craniosynostosis, particularly with coronal or multisu-
mals by ligature of the stapedial artery. Microtia is considered in the ture synostoses.
spectrum of branchial arch defects. Premature closure of the lambdoid sutures results in plagioceph-
Craniosynostosis refers to the premature closure of the sutures, aly (ie, oblique head) (Figure 85.3). Plagiocephaly may also result
which should remain open until 2 to 3 years of age. The newborn from malpositioning in utero or after birth, a condition referred to
skull consists of membranous bones that meet at the suture lines. The as nonsynostotic, deformational, or positional plagiocephaly. The
newborn skull is therefore moldable, can change during the birthing skull has been likened to a parallelogram in appearance in cases that
process, and can expand in response to growth of the brain. Premature also include involvement of the facial structures. Torticollis, which
closure of the sutures is a pathologic process. What initiates this patho- often is attributed to injury to the sternocleidomastoid muscle at
logic ossification is unclear. Some evidence exists to suggest that skull birth (see Chapter 119) and abnormal positioning after birth, con-
compression, such as that which occurs in utero with breech presen- tributes to plagiocephaly. Plagiocephaly-torticollis sequence occurs
tation or twins, contributes to the process. The presence of other asso- in 1 in 300 live births. Malar and contralateral occipital flattening
ciated anomalies, such as syndactyly, is suspicious for embryologic related to preferential positioning by infants are characteristically
disturbances in fibrocartilaginous development. Abnormalities in seen in affected infants. Some affected babies also have hip dislo-
1 region of chromosome 10 are implicated in syndromic synostosis. cation or positional talipes (ie, clubfoot) from in utero constraint
Genes associated with fibroblast growth factor receptor have been (see Chapter 113).
implicated in some genetic syndromes with craniosynostosis. Any or Since 1994 with the advent of the Back to Sleep campaign (cur-
all of the sutures can be affected, and the closure may result in asym- rently called the Safe to Sleep campaign), the prevalence of positional
metry of the skull or microcephaly. Single suture synostosis is classi- plagiocephaly has increased significantly, with estimates of between
fied as simple; multiple synostosis is classified as compound. When 25% and 45% of infants affected. Most cases are mild and correct
closure is related to pathology at the suture, the condition is primary. over time. Positional plagiocephaly may encompass positional occip-
In the presence of underlying brain pathology, the disorder is sec- ital plagiocephaly (ie, unilateral flattening of parieto-occipital region,
ondary. Premature closure of all sutures is often associated with dis- compensatory anterior shift of the ipsilateral ear, bulging of the
eases of the central nervous system, with failure of the brain to grow. ipsilateral forehead) and positional brachycephaly (ie, symmetric
Microcephaly may result from premature closure of some or all of flattening of the occiput, foreshortening of the anterior dimension
the sutures as a primary event or from impairment of the brain and of the skull, compensatory biparietal widening) or any combination
its growth related to some other problem, such as hypoxic encepha- of these 2 deformities. An important strategy to help minimize the
lopathy or congenital infection. Other disorders involving head size development of positional plagiocephaly is to recommend “tummy
include macrocephaly, in which the head circumference is greater than time,” placing an infant in a prone position while awake (eg, with
the 97th percentile. Macrocephaly has numerous causes, including each diaper change) to help develop the muscles of the neck.
hydrocephalus, characterized by enlargement of the ventricular sys-
tem, and macrencephaly, the latter of which may be caused by enlarge- Differential Diagnosis
ment of the brain from anatomic or metabolic conditions, including Typically, the differential diagnosis of clefts of the lip and palate pre­
mucopolysaccharidoses. The child with a large head and who is neu- sents few problems. Submucosal clefts may be more difficult to diag-
rologically normal has benign or idiopathic macrencephaly. Measuring nose, however. The child with such a cleft may present with recurrent
parental head size is frequently a clue to the correct diagnosis. otitis media, hypernasal speech, or nasal regurgitation of liquids.
Fusion of individual sutures prevents growth of the skull perpen- Physical examination may reveal a bifid uvula and occasionally a
dicular to the suture, and skull expansion proceeds in an axis par- notch at the junction of the hard and soft palates.
allel to that of the suture (Figure 85.2). If the sagittal suture fuses Determining whether any physical finding represents an isolated
prematurely, the head is long and narrow, a condition referred to anomaly or is part of a genetic syndrome may be challenging. Any
as scaphocephaly (“boat head”). This is the most common type of associated anomalies (eg, syndactyly, atrial septal defect) suggest the
craniosynostosis, occurring in approximately 54% to 58% of cases possibility of a genetic problem (Boxes 85.2 and 85.3).
of craniosynostosis. If the coronal sutures fuse too soon, the head Microtia does not present a diagnostic dilemma. The anomaly
is flattened; this condition is called brachycephaly and occurs in usually appears sporadically as an isolated condition, although,
18% to 29% of cases of craniosynostosis. The prevalence is 1 in like a cleft, it may be part of some other syndrome. Microtia is
10,000 live births. Unilateral fusion of a coronal suture produces associated with midfacial hypoplasia and antimongoloid slant to
facial asymmetry and a characteristic appearance of the orbit on the eyes in Treacher Collins syndrome. Microtia may also occur in
the affected side, called a harlequin deformity, noted on facial radi- oculoauriculovertebral dysplasia (ie, Goldenhar syndrome), which
ography. Premature closure of the metopic suture results in the is characterized by several associated findings, including hemifa-
triangular-shaped head characteristic of trigonocephaly, reported cial microsomia (ie, 1 side of the face smaller than the other), epi-
in 4% to 10% of case of craniosynostosis. Familial cases have been bulbar dermoids, hemivertebrae, microphthalmos, and renal and
reported, as well as abnormalities of chromosomes 3, 9, and 11. The cardiac anomalies.

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 616 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Anterior fontanelle Metopic suture

Coronal suture

Sagittal suture
Posterior fontanelle

Lambdoid suture

Normocephaly Brachycephaly

Scaphocephaly (dolichocephaly) Oxycephaly

Plagiocephaly Trigonocephaly

Figure 85.2. Changes in the shape of the skull when sutures fuse prematurely. Growth occurs parallel to the fused suture.

Craniosynostosis may also be an isolated finding or associated History

with a condition such as Apert syndrome, in which clefts of the pal- A medical, family, and psychosocial history should be obtained
ate are also seen (Box 85.4). A careful neurodevelopmental assessment (Box 85.5). Whether the condition appeared at birth or some time later
helps determine whether microcephaly is related to an underlying is particularly significant in lesions affecting the skull, such as cranio-
neurodevelopmental disorder. Plagiocephaly may present a diagnostic synostosis. Maternal use of certain medications, such as diazepam,
dilemma: Is the condition related to unilateral craniosynostosis, torti- phenytoin, and isotretinoin (eg, Accutane, Claravis), and alcohol is
collis, or supine sleeping? A careful assessment of the neck for masses associated with an increased incidence of clefts of the lip and palate.
or mobility helps determine the role of the neck musculature in cranial Maternal smoking also increases the risk of clefting, especially in a
flattening and defines the management approach (eg, neck exercises). genetically vulnerable population. Maternal smoking and high alti-
tude are associated with an increased occurrence of craniosynostosis.
Evaluation
Care must be taken to assess the child and determine if the anom- Physical Examination
aly is an isolated finding or a component of a syndrome. This infor- Height, weight, and head circumference should be measured and
mation is important in terms of patient care and genetic counseling plotted at each visit. Head circumference is especially important in
for the parent(s) on the likelihood of having future offspring with the child with craniosynostosis or facial asymmetry. The skull should
similar anomalies. be palpated to detect perisutural ridging. Inner canthal distance

BPPCA6e_Ch085_0613-0620.indd 616 2/13/20 6:27 PM

 Chapter 85: Craniofacial Anomalies 617

Occipital
flattening

Malar
flattening

Plagiocephaly Malar flattening

Scaphocephaly Brachycephaly Trigonocephaly

Figure 85.3. Top row, Classic appearance of an infant with facial asymmetry secondary to plagiocephaly. Bottom row, Classic appearance of infants with
craniosynostosis.

may reveal hypotelorism, a finding that occurs in trigonocephaly.

Box 85.3. Anomalies Associated With Cleft Lips
The child’s face should be assessed when neutral and when crying
and Palates
or smiling to evaluate the status of the depressor anguli oris muscle.
The neck should be palpated for masses and neck range of motion ww Anencephaly
assessed. The growth of the child with craniofacial anomaly must ww Aniridia
be carefully monitored. Problems with adequate weight gain are ww Ankyloblepharon filiforme adnatum
frequently experienced by newborns and infants with clefts. ww Aplasia of trochlea
ww Cleft larynx
ww Congenital heart disease
ww Congenital neuroblastoma
Box 85.2. Genetic Syndromes Associated
ww Congenital oral teratoma
With Clefts
ww Foot deformities
ww Apert ww Forearm bone aplasia
ww Ectrodactyly–ectodermal dysplasia–clefting ww Laryngeal web
ww Goldenhar (ie, oculoauriculovertebral dysplasia) ww Lateral proboscis
ww Meckel ww Nasal glioma or encephalocele
ww Opitz ww Oral duplication
ww Oral-facial-digital, type I ww Persistent oropharyngeal membrane
ww Popliteal web ww Polydactyly
ww Stickler (ie, hereditary progressive arthro-ophthalmopathy) ww Sacral agenesis
ww Treacher Collins ww Spina bifida
ww Van der Woude ww Thoracopagus (ie, conjoined twins)

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 618 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

disorder is suspected (see Chapter 20). Referral to a geneticist is

Box 85.4. Genetic Disorders With Craniosynostosis
warranted to ensure testing for recently identified gene associated
ww Apert syndrome with craniofacial conditions and for genetic counseling with the par-
ww Crouzon syndrome ent or parents.
ww Jackson syndrome
ww Pfeiffer syndrome Imaging Studies
Radiographs and imaging studies may be indicated in children with
facial asymmetry, microcephaly and macrocephaly, and craniosyn-
Box 85.5. What to Ask ostosis. Three-dimensional computed tomography of the skull is
particularly helpful in defining which, if any, sutures are fused but
Craniofacial Anomalies is not warranted in the child with suspected positional plagioceph-
ww What was the birth like? aly. Such studies are helpful in differentiating craniosynostosis and
ww Was the delivery vaginal or by cesarean section? deformational disorders that will resolve on their own. Renal ultra-
ww Were forceps used during the delivery? sonography may be indicated in the child with microtia or other ear
ww Was the fetus in an abnormal position (eg, breech) in utero? anomaly because of the association between ear and renal anoma-
ww What is the child’s usual sleeping position? lies. Renal ultrasonography is usually normal in the child with iso-
ww Have the findings been present since birth, or did they appear later? lated microtia but is abnormal in approximately 30% of children
ww Has the child experienced other symptoms, such as recurrent ear with auricular as well as other congenital anomalies, particularly
infections or speech difficulties? when associated with certain syndromes.
ww Does the child have any family history of craniofacial anomalies?
ww Does the mother have a history of illness during pregnancy? Management
ww Was the mother exposed to any medications, particularly teratogenic The management of the child with craniofacial anomaly usually
agents, during pregnancy? requires the expertise of a multidisciplinary team, including a pedi-
ww Did the mother use alcohol during pregnancy? atrician, plastic surgeon, otolaryngologist, speech pathologist, social
ww Did the mother smoke during pregnancy? worker, psychologist, orthodontist, and prosthodontist. The primary
ww Is the child experiencing difficulties in school (eg, teasing)? care physician who is not part of the team can receive information
ww How is the child performing in school? about appropriate patient care and follow-up. It is important for the
ww Is development progressing normally? What are the child’s milestones? primary care physician to be familiar with the appropriate nomen-
ww Is the child’s speech understandable? clature to be able to communicate with consultants. In brief, clefts
ww Does the child have any trouble with eating or drinking? Are fluids of the lip are unilateral or bilateral and complete or incomplete.
expelled through the child’s nose with swallowing? A complete cleft extends into the nares (Figure 85.1). Clefts of the
palate may involve the entire palate or be confined to the second-
ary or soft palate.
The physical examination should focus on defining the extent of Routine well-child care, including monitoring of growth and
the anomaly and determining if associated abnormalities are pres- administration of immunizations, is most important in the treat-
ent. Such abnormalities may involve the face or other parts of the ment of the child with craniofacial anomaly. Some newborns and
body, including the skeleton. The pharynx should be carefully exam- infants with clefts are slow feeders. Mothers should be told that
ined for mobility of the palate. Although some children with nasal although breastfeeding can be carried out, it may present unique
regurgitation have a structural deformity, such as a submucosal cleft, challenges; however, the compliancy of the breast tissue creates a
others, including infants with DiGeorge syndrome (ie, 22q11 dele- natural seal for the lip and palate. If difficulties are encountered,
tion syndrome), have functional impairment with decreased palatal consultation with a lactation specialist may be especially helpful
mobility. In the child with microtia, the position of the ear should (see Chapter 29). Some formula-fed newborns and infants require
be noted. When the ear is displaced to the cheek, the condition is the use of special adaptive nipples or feeders. A specific cleft palate
called auricular dystopia. Cardiac murmur may be noted in the child feeder, which consists of a plastic bottle that allows for compression
with cleft, and the murmur should be carefully evaluated. of the unit during feeding, is available. A soft nipple typically used
The child with cleft should be carefully assessed for the presence by a preterm neonate can also be used. Long nipples, such as lamb’s
of otitis media at each visit. Periodic hearing assessments should be nipples, which are used to feed baby lambs, are not routinely rec-
carried out in the child with cleft or microtia. Speech and develop- ommended because they cause newborns and infants to gag. The
ment should also be evaluated. newborn or infant who gains weight slowly may need to be given
concentrated formula (see Chapter 146).
Laboratory Tests Hearing and speech should be monitored. Chronic prophylactic
Routine laboratory tests are not indicated in most children with antibiotics or the insertion of pressure equalization tubes may be
craniofacial anomalies. Genetic studies are indicated if a genetic necessary to manage recurrent otitis media (see Chapter 87). Speech

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 Chapter 85: Craniofacial Anomalies 619

problems require the expertise of a speech pathologist and place- many centers. The age of the infant and the site of the synostosis influ-
ment of the child in speech therapy in the community or school. ence the complexity of the surgical procedure, although a move is
Surgical correction is indicated for many anomalies. Clefts usu- afoot to perform endoscopic surgery in infants younger than 16 weeks.
ally are repaired as staged procedures during the first 2 years after Endoscopic strip craniectomy is usually followed by the use of a
birth. Repair of the cleft lip, the first procedure, is traditionally custom-made molding helmet for up to 7 months. A controversy about
scheduled when an infant weighs 10 lb and is 10 weeks of age and whether neurodevelopmental problems are related to craniosynos-
the hemoglobin is 10 (ie, rule of 10s). Infants, particularly those tosis or whether they represent a preexisting condition has arisen.
with widely separated complete bilateral clefts, may require tap- In the developmentally normal child with evidence of closure of all
ing or a prosthetic device to bring tissues in close proximity before sutures, surgical repair is believed to be warranted. In other cases,
surgery is attempted. Appropriate weight gain is therefore critical the procedure is thought to be reconstructive because it normalizes
to ensure timely surgery. If skilled anesthesiologists and nurses are the appearance of the child with a deformation.
available, cleft lip repair can be carried out within the first 2 weeks In the newborn or infant with plagiocephaly, when the deforma-
after birth. Early repair is recommended at some centers. Repair of tion is believed to be related to torticollis, passive stretching of the
the cleft palate, the second procedure, is usually undertaken when neck 5 to 6 times a day (with each diaper change) is used to manage
the child is between 12 and 18 months of age. Better speech devel- the condition. Additionally, it is recommended that bright objects,
ops with earlier palatal repair. Surgical correction of clefts does not such as mobiles, be placed over the child’s crib to encourage head
alter a child’s propensity to otitis media, although the incidence of turning. Changing the crib position or the position of the newborn
otitis media appears to have decreased among children with clefts or infant in the crib may also encourage movement of the head.
following the use of conjugated pneumococcal vaccine. The inci- Studies have shown that 90% of newborns and infants with con-
dence of otitis media decreases as children age, however. Refinement genital torticollis improve with manual stretching. The infant who
of the cosmetic results, including rhinoplasty, occurs throughout does not improve with stretching, who has a developmental delay
childhood. Orthodontia is frequently a key component to achieve in which the infant does not develop normal neck muscle strength
a cosmetically acceptable result and appropriate occlusion of the or tone, or who has a deformity that is still present at 6 months of
dentition for speech and chewing. Approximately 10% to 20% of age may benefit from the use of a specially designed helmet or band,
children will develop velopharyngeal insufficiency after repair of referred to as a dynamic orthotic cranioplasty device, that reshapes
a cleft palate. In these cases, the posterior soft palate fails to make the skull. The device is not used before 6 months of age and gener-
a tight seal with the pharynx. A child may experience nasal regur- ally is worn for a minimum of 4 months.
gitation of food or hypernasal speech. Surgical correction of velo- In an effort to reverse the trend of increasing positional pla-
pharyngeal insufficiency involves lengthening the shortened palate. giocephaly related to supine sleeping, the American Academy of
Additional surgery may also be required for the child with signifi- Pediatrics has recommended that parents and guardians rotate
cant jaw deformity. These may include the placement of bone grafts their infant’s position when they are awake and allow for tummy
or maxillary advancement. time, which is time when the infant is placed prone, sometimes
The child with isolated unilateral microtia often hears, and sur- with a rolled receiving blanket under the upper chest. Tummy time
gery is recommended to restore a normal anatomic appearance, even can be recommended with each diaper change. This promotes the
if hearing in the affected ear is not improved. Surgical correction of development of the neck musculature and head control. Most posi-
microtia usually is initiated when the child is 5 years of age, before tional plagiocephaly secondary to supine sleeping resolves over
the child starts school. At this time, the ear has achieved 90% of its time as the infant develops head control and spends less time in
growth, and the child is spared the potential embarrassment of the a supine position. In 2016 the Congress of Neurological Surgeons
deformity in the school setting. Surgical reconstruction can involve released guidelines about the management of positional plagio-
the implantation of the child’s costal cartilage or a porous polyethy­ cephaly that were later endorsed by the American Association of
lene framework shaped like the pinnae. In either case, several surgi- Neurological Surgeons and the American Academy of Pediatrics.
cal procedures usually are necessary. For the child with other facial These guidelines were based on an extensive review of the existing
anomalies as well, more extensive reconstructive surgery is indi- literature. The guidelines note that imaging studies are rarely indi-
cated. The infant with “outstanding ears” may benefit from taping cated. Repositioning of infants is effective, although the evidence
the ears back to the mastoid area early on while the ear cartilage is supports the use of physical therapy as preferred. Helmets can be
soft and malleable. Other ear anomalies may be amenable to molds used in refractory cases with persistent moderate to severe plagio-
that reshape flattened or folded areas. cephaly. Early initiation of a dynamic orthotic cranioplasty device
Craniosynostosis can be corrected surgically, and such correc- is usually indicated in the child with developmental delay, includ-
tion is best carried out before 1 year of age. Endoscopic cranio- ing Down syndrome, because of their failure to achieve neuromus-
synostosis repair (ie, endoscopic-assisted strip craniectomy) is cular control that precludes persistent supine posture.
minimally invasive and requires shorter surgical time (average time, Psychological counseling should be available to affected chil-
<1 hour) and a reduced length of hospital stay. Discharge can be dren and their families to help them adjust to anomalies and the
as early as the first postoperative day. It is the preferred approach in reactions of society. The parent(s)/guardian(s) may be referred to

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 620 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

national agencies and support groups, such as the American Cleft Graham JM Jr, Kreutzman J, Earl D, Halberg A, Samayoa C, Guo X. Deformational
Palate-Craniofacial Association (https://cleftline.org) to help them brachycephaly in supine-sleeping infants. J Pediatr. 2005;146(2):253–257 PMID:
cope with the potential stress related to giving birth to a child with 15689919 https://doi.org/10.1016/j.jpeds.2004.10.017

this anomaly and to advise them about the medical and surgical Hunt O, Burden D, Hepper P, Stevenson M, Johnston C. Self-reports of psy-
interventions that are available. FACES: The National Craniofacial chosocial functioning among children and young adults with cleft lip and pal-
ate. Cleft Palate Craniofac J. 2006;43(5):598–605 PMID: 16986986 https://doi.
Association (www.faces-cranio.org) is another referral source for
org/10.1597/05-080
parents.
Ishimoto S, Ito K, Karino S, Takegoshi H, Kaga K, Yamasoba T. Hearing lev-
Prognosis els in patients with microtia: correlation with temporal bone malformation.
Laryngoscope. 2007;117(3):461–465 PMID: 17334306 https://doi.org/10.1097/
Some anomalies, such as deformational plagiocephaly, resolve spon- MLG.0b013e31802ca4d4
taneously or with exercise and positioning. Most other anomalies Ludwig KU, Mangold E, Herms S, et al. Genome-wide meta-analyses of non-
can be surgically corrected, leaving little residual evidence of the syndromic cleft lip with or without cleft palate identify six new risk loci. Nat
deformity. School success and psychological well-being may be more Genet. 2012;44(9):968–971 PMID: 22863734 https://doi.org/10.1038/ng.2360
resistant to remediation and are highly dependent on the supportive- Mathijssen IMJ. Guideline for care of patients with the diagnosis of cranio-
ness of the family and its emotional resources. Children who grow synostosis: Working Group on Craniosynostosis. J Craniofac Surg. 2015;26(6):
up in settings in which the deformity is thought to be embarrassing 1735–1807 PMID: 26355968 https://doi.org/10.1097/SCS.0000000000002016
have long-term problems with low self-esteem. Mawji A, Vollman AR, Hatfield J, McNeil DA, Sauvé R. The incidence of posi-
tional plagiocephaly: a cohort study. Pediatrics. 2013;132(2):298–304 PMID:
23837184 https://doi.org/10.1542/peds.2012-3438
CASE RESOLUTION Proctor MR. Endoscopic craniosynostosis repair. Transl Pediatr. 2014;3(3):
The newborn has a cleft of the lip and palate. The mother is advised that her new- 247–258 PMID: 26835342 https://doi.org/10.3978/j.issn.2224-4336.2014.07.03
born can be given formula, and she is given a supply of special feeders. She is also Renju R, Varma BR, Kumar SJ, Kumaran P. Mandibulofacial dysostosis
given contact information for a parents’ support group and meets other parents (Treacher Collins syndrome): a case report and review of literature. Contemp
of children with similar anomalies. During her visit to the local craniofacial team, Clin Dent. 2014;5(4):532–534 PMID: 25395774 https://doi.org/10.4103/
she views pictures of children who have undergone a repair and feels relieved. 0976-237X.142826
The mother is advised about the timing of surgery and told that the surgery Rowland K, Das N. PURLs: helmets for positional skull deformities: a good idea,
will be scheduled when the infant is approximately 10 weeks of age. A follow-
or not? J Fam Pract. 2015;64(1):44–46 PMID: 25574506
up appointment in approximately 2 weeks is arranged. Weight gain is monitored,
and the adjustment between the mother and the newborn is assessed. Ruegg TA, Cooper ME, Leslie EJ, et al. Ear infection in isolated cleft lip: etiolog-
ical implications. Cleft Palate Craniofac J. 2017;54(2):189–192 PMID: 26153759
https://doi.org/10.1597/15-010
Schuster M, Maier A, Haderlein T, et al. Evaluation of speech intelligibility for
Selected References children with cleft lip and palate by means of automatic speech recognition. Int
Ashokan CS, Sreenivasan A, Saraswathy GK. Goldenhar syndrome—review with J Pediatr Otorhinolaryngol. 2006;70(10):1741–1747 PMID: 16814875 https://
case series. J Clin Diagn Res. 2014;8(4):ZD17–ZD19 PMID: 24959523 doi.org/10.1016/j.ijporl.2006.05.016

Bhattacharya D, Angurana SK, Suthar R, Bharti B. Congenital hypoplasia of Shkoukani MA, Chen M, Vong A. Cleft lip—a comprehensive review. Front
depressor anguli oris muscle (CHDAOM): an uncommon cause of asymmetric Pediatr. 2013;1:53 PMID: 24400297 https://doi.org/10.3389/fped.2013.00053
crying facies in childhood. BMJ Case Rep. 2018;bcr-2018-227240 PMID: 30355578 van Wijk RM, van Vlimmeren LA, Groothuis-Oudshoorn CG, Van der Ploeg CP,
https://doi.org/10.1136/bcr-2018-227240 Ijzerman MJ, Boere-Boonekamp MM. Helmet therapy in infants with positional
Damiano PC, Tyler MC, Romitti PA, et al. Health-related quality of life skull deformation: randomised controlled trial. BMJ. 2014;348:g2741 PMID:
among preadolescent children with oral clefts: the mother’s perspective. 24784879 https://doi.org/10.1136/bmj.g2741
Pediatrics. 2007;120(2):e283–e290 PMID: 17671039 https://doi.org/10.1542/ Wang RY, Earl DL, Ruder RO, Graham JM Jr. Syndromic ear anomalies and
peds.2006-2091 renal ultrasounds. Pediatrics. 2001;108(2):e32 PMID: 11483842 https://doi.
Flannery AM, Tamber MS, Mazzola C, et al. Congress of Neurological org/10.1542/peds.108.2.e32
Surgeons systematic review and evidence-based guidelines for the man- Wilkie AO, Byren JC, Hurst JA, et al. Prevalence and complications of single-
agement of patients with positional plagiocephaly: executive summary. gene and chromosomal disorders in craniosynostosis. Pediatrics. 2010;126(2):
Neurosurgery. 2016;79(5):623–624 PMID: 27759671 https://doi.org/10.1227/ e391–e400 PMID: 20643727 https://doi.org/10.1542/peds.2009-3491
NEU.0000000000001426 Zarate YA, Martin LJ, Hopkin RJ, Bender PL, Zhang X, Saal HM. Evaluation
Graham JM Jr, Gomez M, Halberg A, et al. Management of deformational of growth in patients with isolated cleft lip and/or cleft palate. Pediatrics.
plagiocephaly: repositioning versus orthotic therapy. J Pediatr. 2005;146(2): 2010;125(3):e543–e549 PMID: 20142284 https://doi.org/10.1542/peds.
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 CHAPTER 86

Common Oral Lesions

Charlotte W. Lewis, MD, MPH, FAAP

CASE STUDY
A 7-year-old girl is brought to the office for evalua- Questions
tion of a swelling on the inside of her lower lip of 4 to 1. What is the differential diagnosis of lip masses and
6 weeks’ duration. Her mother reports that it increases other oral lesions?
and decreases in size. The girl states that the swelling is 2. What laboratory tests or radiologic studies are
not painful, and she cannot remember hurting her lower useful in the evaluation of oral lesions?
lip. On examination, a raised, bluish, nontender swelling 3. What management strategies are used to treat cyst-
measuring 0.8 × 0.7 cm (0.31 × 0.28 in) is apparent on like and other intra-oral lesions?
the mucosa of the lower lip. 4. When should children with oral lesions be referred
to subspecialists?

Primary care physicians commonly evaluate lesions in the oral cav- early in childhood. Approximately 20% to 25% of the US population
ity. Knowledge of common congenital, developmental, infectious, experiences recurrent aphthous stomatitis (RAS). Oral lesions resulting
traumatic, and neoplastic conditions that affect the mouth and its from infections are also common. Approximately 35% of newborns and
structures can help physicians recognize and manage these lesions young infants develop oral candidiasis, commonly known as thrush.
appropriately. Although many oral lesions are benign or represent Oral herpes lesions are also common and are caused by human herpes-
normal variants, others may require specific medical or surgical virus, usually type 1. By young adulthood, more than 50% of US indi-
treatment. Some oral lesions offer clues to underlying syndromic viduals are seropositive for human herpesvirus 1. Approximately 20%
diagnosis, indicate more serious infectious or systemic disease, or to 40% of the population has experienced oral herpes at least 1 time.
occur as side effects of certain medications. Chronic (“ordinary”) gingivitis usually has its onset in the peri-
pubertal age group, and it ultimately affects as many as 90% of
Epidemiology adults. Smoking is a major risk factor for gingivitis and its sequelae–
Oral pathology is common and covers a broad range of lesions. Benign periodontal disease. In 2017, 20% of high school students reported
oral lesions, such as gingival cysts, occur in approximately 75% of new- current use of a tobacco product.
borns. Approximately 20% of the population has at least a small torus
palatinus, a benign bony overgrowth of the palate that usually begins in Clinical Presentation
childhood. Ankyloglossia, commonly referred to as tongue-tie, affects Oral lesions may come to the attention of the physician in any number
approximately 5% of newborns. Fissured tongue affects approximately of ways. Some may be obvious at birth, such as a congenital epulis (also
2% of the population. Fissured tongue may be associated with benign called congenital granular cell tumor), which typically presents as a
migratory glossitis (ie, geographic tongue), which occurs in approxi- mass arising from the maxillary alveolar ridge and protruding from the
mately 1% to 2% of children. Tobacco-associated keratosis occurs at the oral cavity in a neonate, potentially interfering with breathing or eating.
site of habitual placement of snuff or chewing tobacco and is estimated Oral lesions may be an incidental finding on physical examination. For
to affect more than 300,000 children in the United States. Leukoplakia, example, in examining a newborn, the pediatrician may notice small
a premalignant condition associated with smokeless tobacco, occurs (approximately 2–3 mm in size) yellow-white papules along the pal-
in approximately one-half of users. Although the resulting oral can- atal midline and can reassure the family that these are Epstein pearls,
cer is often diagnosed in the sixth or seventh decade of life, the habit common lesions of no clinical significance. Most oral vascular mal-
of oral tobacco use typically starts in childhood—typically between 9 formations are present at birth, become more noticeable over time,
and 16 years of age. Among US high school students surveyed in 2017, and rarely regress. One of 3 vessel types usually predominates in such
5.5% report current use of a smokeless tobacco product; use is higher malformations: arterial, venous, or lymphatic. Microcystic lymphatic
in boys than girls and in whites as well as American Indians/Alaska malformations often affect the tongue and surrounding soft tissue, can
Natives relative to other racial/ethnic groups. be friable, may interfere with eating and speaking, and can result in
Aphthous ulcers, commonly known as canker sores, are among the overgrowth of adjacent bones. When these lesions become infected,
most common oral lesions in developed countries, with typical onset they rapidly enlarge and may compromise the airway.

621

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 622 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

The physician or parent/guardian may be the first to notice Other oral lesions may present in conjunction with other symp-
thrush. The incidence of oral candidiasis peaks around the fourth toms. Acute onset of “strawberry tongue,” indicating glossitis, often
week after birth; thrush is uncommon in infants older than 6 to occurs with scarlet fever or Kawasaki disease. The initial herpes
9 months. Thrush can occur, however, at any age in predisposed simplex virus oral infection–primary herpetic gingivostomatitis–
patients (ie, immunosuppressed or deficient) and can affect the which typically affects infants and young children, is characterized
esophagus as well as the oropharynx. Candida albicans in combina- by multiple oral vesicular or ulcerative lesions, fever, malaise, cer-
tion with contact irritation has been implicated in angular cheilitis, vical lymphadenopathy, and decreased oral intake. Reactivation of
which appears as crusty or scaling erythematous fissures at the cor- prior human herpesvirus 1 infection often affects the vermillion bor-
ners of the mouth. Other benign oral lesions, such as benign migra- der of the lip, which is known as herpes labialis. Oral lesions may
tory glossitis, are brought to the attention of the physician because also indicate underlying serious systemic illness, such as Crohn dis-
parents or guardians are concerned that they represent pathology; ease, systemic lupus erythematosus, or acute myelogenous leukemia.
however, reassurance is appropriate. Some life-threatening, rapidly progressive infections begin
Concerns for ankyloglossia may arise when a newborn has dif- in the mouth. Ludwig angina (see Chapter 89) is a painful, rapidly
ficulty breastfeeding, particularly when the mother has persistent progressive, infectious process of the submandibular space, often
pain or trauma to her nipple with breastfeeding. Clinically signifi- presenting as induration and swelling of the floor of the mouth, neck
cant ankyloglossia interferes with an effective latch and with nor- swelling, a superiorly and posteriorly displaced tongue, difficulty
mal tongue movement needed to efficiently transfer milk from the swallowing, and subsequent airway obstruction. Ludwig angina is
breast. Anterior ankyloglossia refers to a sublingual attachment to a potential complication of a dental infection; in children, however,
the underside of the tongue that is close to the tongue tip. In pos- Ludwig angina can occur without a clear etiology, or it can compli-
terior ankyloglossia, the sublingual attachment is farther back on cate oral trauma or gingivostomatitis. Vincent infection or acute nec-
the tongue underside, but it still restricts motion of the tongue. The rotizing ulcerative gingivitis is painful, edematous, bleeding gums
maxillary labial frenulum can appear quite prominent in infants and with ulcers, necrosis, and pseudomembrane formation in affected
young children; however, it usually becomes much less obvious by areas. When this spreads to the pharynx and tonsils, the condition
the time the permanent central incisors erupt. is referred to as Vincent angina (also called trench mouth). Like
Physicians may be the first to note swollen, friable, erythematous Ludwig angina, Vincent angina can progress to life-threatening air-
gingiva along with plaque buildup on and between the teeth repre- way obstruction.
senting the initial presentation of chronic gingivitis. Chronic gin- Some genetic syndromes are first detected because of oral lesions.
givitis is the first and only reversible stage of periodontal disease. For example, lip pits or mounds in conjunction with cleft lip and/
Onset is typically in peripubertal children. Although young children or cleft palate are virtually pathognomonic of Van der Woude
experience gingivostomatitis from other causes, they do not usually syndrome, an autosomal-dominant cause of orofacial clefting.
harbor Actinobacillus actinomycetemcomitans or Porphyromonas Hyperpigmented lesions (brown or dark blue, similar to freckles)
gingivalis and thus do not commonly experience chronic gingivi- on the lips or buccal mucosa may provide a clue in the diagnosis of
tis or periodontal disease. Peutz-Jeghers syndrome, an autosomal-dominant condition of mul-
Thickening of the mucosa, usually in the labial vestibule, offers tiple intestinal hamartomas. Patients with Peutz-Jeghers syndrome
clues to smokeless tobacco use. The severity of tobacco-related oral may experience recurrent abdominal pain, intestinal obstruction,
lesions demonstrates a dose-response relationship with the amount, or bleeding, and have a 15-fold increased risk of intestinal cancer.
frequency, and duration of smokeless tobacco exposure. Tobacco-
associated keratosis is a predictable lesion that manifests as an area
Pathophysiology
of thickening at the site of habitual placement of snuff or chewing
tobacco. Chronic exposure to smokeless tobacco can result in the Neonatal and Other Developmental
development of opaque-white to yellow-brown lesions with a wrin- Lesions
kled appearance, known as leukoplakia and which is considered to Gingival cysts in the neonate include Epstein pearls, Bohn nodules,
be a premalignant condition. and dental lamina cysts; these are caused by entrapment of tissues
Recurrent oral mucosal trauma, such as habitual biting of the inside during embryologic development. Congenital epulis of the newborn
of the lip or recurrent irritation from orthodontics, can induce oral is a rare, gingival tumor of unclear etiology that occurs more com-
lesions anywhere in the mouth but most often does so on the buccal or monly in the maxilla than the mandible, with female predilection
labial mucosa. One such lesion is a mucocele, which is a saliva-filled cyst (8:1), and may occur as a single tumor or multiple tumors. The etiol-
that is usually less than 1 centimeter in diameter, round, painless, and ogy of fissured tongue and geographic tongue are unknown. Fissured
opaque white or slightly blue in color. Pyogenic granuloma is another tongue tends to cluster in families, suggesting a genetic etiology, and
lesion that can occur at a site of recurrent mucosal or skin irritation. can also occur in Down syndrome. Benign migratory glossitis results
These lesions are blood red or reddish-brown, and they bleed easily. from the loss of the tiny fingerlike projections, called papillae, on
Although they can be protuberant and look scary to parents/guardians, the surface of the tongue, giving the tongue a map-like appearance.
both mucoceles and pyogenic granulomas are benign. The inciting factors responsible for oral vascular malformations are

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 Chapter 86: Common Oral Lesions 623

not well understood. Ankyloglossia is thought to result from a local- White plaques involving the buccal, lingual, and palatal mucosa
ized failure of apoptosis. are suggestive of oral candidiasis. Thrush can sometimes be confused
with milk remaining in the child’s mouth after feeding. Scraping the
Traumatic lesion to determine if the white substance is readily removed (as
Some of the most common oral lesions noted on physical exami- milk is) helps differentiate this from oral candidiasis, in which the
nation result from minor accidental self-bites to the lip or buccal white plaques do not easily scrape off; additionally, after scraping,
mucosa. Most of these lesions resolve quickly, but recurrent trauma the base of the thrush lesion may be erythematous or may bleed.
may result in pyogenic granuloma or fibroma formation. A muco- Some infants and young children have a white coating to the tongue
cele results from traumatic rupture of a minor salivary gland with as a normal variant. The lack of white patches on other mucosal
subsequent cyst formation. surfaces should call into question the diagnosis of thrush. In fissured
tongue, grooves that vary in depth are noted along the dorsal and
Infectious lateral aspects of the tongue.
Although an infectious etiology to aphthous ulcers has been In scarlet fever, the tongue initially has a white coating overly-
proposed, their true etiology remains unclear. Oral herpes lesions ing the red swollen papillae of the tongue—the “white strawberry
usually are the result of infection with human herpesvirus 1. tongue,” which desquamates at approximately day 4 or 5 of illness,
Herpangina results from coxsackievirus A infection. Candida albicans leaving the “red strawberry tongue.” In Kawasaki disease, initial pre-
causes oral candidiasis. Thrush occurs when normal host immu- sentation usually includes a bright red strawberry tongue and red,
nity is immature (as in neonates) or suppressed (eg, during steroid dry, cracked lips. Other clinical features and select laboratory testing
treatment) or when normal flora is disrupted (eg, while on antibi- help differentiate Kawasaki disease from scarlet fever. Both
otics). Newborns may be colonized with C albicans during birth. Kawasaki disease and scarlet fever require specific treatment to
Other sources of transmission to neonates include colonized mater- avoid long-term complications. For scarlet fever, treatment is with
nal skin in contact during breastfeeding, pacifiers, and bottle nipples. penicillin to avoid rheumatic fever, and for Kawasaki disease, treat-
Chronic gingivitis occurs after buildup of bacterial plaque on the ment is with intravenous immunoglobulin to prevent coronary
teeth, adjacent gingiva, and pockets between teeth and gums. Bacteria artery aneurysms.
within plaque release toxins that cause an inflammatory response; the Common oral ulcers include aphthous ulcers, herpes gingivosto-
most commonly involved species are gram-negative anaerobic bacte- matitis, and herpangina. Oral herpes may be characterized by multi-
ria, including A actinomycetemcomitans and P gingivalis. ple vesicular lesions, which, after rupture, appear as ulcers involving
Both Ludwig angina and Vincent infection/angina result from the lips, skin around the mouth, tongue, and mucosal membranes,
polymicrobial infection, including anaerobes. typically in the anterior portion of the mouth. The initial infec-
tion may occur between 1 and 3 years of age. Aphthous ulcers
Other also involve the anterior mouth, typically along the wet vermillion;
Drug-induced gingival hyperplasia can occur in patients taking however, they usually first appear at a somewhat older age (ie, in
corticosteroids, phenytoin (most common cause in children), the preschool years or later) and with fewer lesions than oral her-
cyclosporine A, or nifedipine. It results from fibrous tissue over- pes. Factors that may predispose to the development of RAS include
growth but, much like ordinary gingivitis, is exacerbated by poor familial tendency, trauma, hormonal factors, food or drug hyper-
oral hygiene and presence of plaque. sensitivity, immunodeficiency, celiac disease, inflammatory bowel
disease, and emotional stress. Herpangina may present similarly to
Differential Diagnosis herpes, but it more typically involves the posterior pharynx and the
Age at onset, location and characteristics of the lesion, and accom- palate. Similar lesions on the hands or foot, as in hand-foot-and-
panying signs and symptoms often help narrow the differential mouth disease, may lend support to coxsackievirus A as the etiology.
diagnosis. The appearance of 1- to 3-mm cysts in the mouth Trauma to the salivary duct may result in a mucocele. In con-
of a neonate is indicative of Epstein pearls, which are the most trast, a pyogenic granuloma is an erythematous, nonpainful, smooth
common and usually are present along the palatal midline; dental or lobulated mass that often bleeds easily when touched, whereas
lamina cysts, which usually are located bilaterally along the crest a fibroma is a moderately firm, smooth-surfaced, pink, sessile or
of the dental ridge about where the first molars typically erupt; or pedunculated nodule, usually noted on the buccal mucosa in the
Bohn nodules, which are found on the buccal and lingual aspects occlusal plane. When located on the gingiva, a pyogenic granuloma
of the ridge, away from the midline. A protuberant mass from the can be confused with a periapical abscess, which is an erythematous,
anterior maxillary ridge of a newborn should prompt suspicion for a pus-filled cyst that occurs when infection spreads from the root of
congenital epulis; however, examination by a pathologist after resec- an infected tooth to surrounding tissues (also called a gum boil or
tion is important to confirm the diagnosis. A mucocele is a pain- a parulis). If the abscess ruptures, it often leaves a periapical fistula.
less, clear or bluish, fluid-filled cyst that results from damage to the Erythematous and friable gums often indicate the presence of
salivary duct, resulting in extravasation of mucus from the gland chronic gingivitis. Typically, plaque is seen on and between the
into the surrounding soft tissue. teeth. In contrast with plaque-associated chronic gingivitis, which

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 624 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

is usually painless or only mildly uncomfortable, acute necrotizing herpesvirus and coxsackievirus can be identified and differentiated
ulcerative gingivitis (Vincent infection) is quite painful, acute in with polymerase chain reaction testing. Likewise, oral candidiasis in
onset, and associated with ulcers, necrosis, and pseudomembrane an otherwise healthy infant usually is managed without diagnostic
formation in affected areas. Swollen and inflamed gingiva can be tests. However, a potassium hydroxide 10% microscopic slide prep-
presenting signs of leukemia in an ill-appearing child with an abnor- aration of scrapings from the lesion should demonstrate the charac-
mal complete blood count. teristic spherical budding yeasts and pseudohyphae. An excisional
biopsy or resection may be necessary to determine histology and
Evaluation diagnosis of other oral lesions.
Laboratory tests, including a complete blood cell count, may be
History
helpful in the initial evaluation of the ill-appearing child with oral
The history is very important in evaluating oral lesions and deter- lesions or in cases in which serious infection, systemic illness, or
mining the need for further treatment or referral. Key factors to inflammatory conditions are suspected.
include in the history are age at onset, duration, inciting factors,
other medical problems, medications, tobacco use, family history, Imaging Studies
ill contacts, and associated or systemic symptoms and signs, such Imaging studies are not indicated in the evaluation of most oral
as fever (Box 86.1). lesions unless the lesions are related to problems of dentition, such as
a periapical abscess, in which case radiographs are usually obtained
Physical Examination
by the dentist rather than the physician. Magnetic resonance imaging
Physical examination of the oral structures should start with the lip may be used for evaluation of oral vascular malformations.
(dry and wet vermillion) and surrounding skin (the “white lip”). The
examination should then turn to the mucosa, gingiva, teeth, and Management
palate; all aspects of the tongue (ie, superior, inferior, both sides);
Many of the common oral lesions are developmental or normal
sublingual structures; frena; and posterior pharynx. The physi-
variants or are self-limited, and management entails observation
cian should note the number, size, location, and characteristics of
to ensure the lesions follow their expected course. For example,
the lesions, because this information can be helpful in narrow-
gingival cysts in newborns typically regress spontaneously. Oral
ing the differential diagnosis. The presence and duration of fever
lesions such as torus palatinus or benign migratory glossitis do not
should be ascertained. The rest of the body should be examined with
require treatment. Although human herpesvirus 1 gingivostomati-
specific attention to the presence of other lesions, rashes, lymphad-
tis is self-limiting, primary infection can cause considerable pain
enitis, or arthritis.
and result in decreased oral intake. Early (within 72 hours) anti­
Anterior ankyloglossia may be obvious because of a notched
viral therapy in the form of acyclovir 5 to 10 mg/kg/dose 5 times
or heart-shaped tip of tongue. In more severe cases, the tight and
per day for 7 to 10 days has been shown to shorten the duration
short sublingual frenum makes it difficult to pass a finger under the
of fever, lesions, and odynophagia. Other oral lesions respond well
tongue. Difficulty lifting the tongue to the middle of the mouth and/
to supportive care. For example, a child with herpangina may
or difficulty extruding the tongue past the gingiva are other charac-
benefit from regular ibuprofen or acetaminophen; topical applica-
teristics suggestive of anterior or posterior ankyloglossia.
tion of a 1:1 mixture of attapulgite (eg, Kaopectate, Donnagel) and
Laboratory Tests diphenhydramine elixir (eg, Benadryl) to form a protective coating
over the lesion; avoidance of acidic beverages, such as orange juice,
In otherwise healthy children who present with oral ulcerative
that may cause pain on contact with the ulcers; and close attention
lesions, supportive care is typically implemented without pursu-
to fluid intake and signs or symptoms of dehydration. Amlexanox
ing a definitive etiology. If a specific diagnosis is required, human
5% oral paste or triamcinolone acetonide dental paste reduces pain
associated with, duration of, and size of aphthous ulcers and is used
in adults with RAS; however, safety of these treatments in children
Box 86.1. What to Ask has not been established. Viscous lidocaine has been associated
Common Oral Lesion with systemic absorption and subsequent dysrhythmia or seizure
ww How long has the child had the lesion? and should not be used in children.
ww Is the lesion painful? Complicated vascular and lymphatic malformations of the oral
ww Did the child recently injure the affected area? cavity require specialty consultation with a team experienced in the
ww Has the child had any fever? care of these lesions. Unlike infantile hemangiomas, complicated
ww Is the child eating as usual? vascular malformations usually do not involute. Typically, surgi-
ww Does the child have any other lesions? cal resection is the treatment of choice for lymphatic malforma-
ww Is the child currently taking any medications? Has the child recently tions; however, microcystic lymphatic malformations are difficult to
taken any medications? remove and may recur even after resection. Sclerosing therapy and
laser treatment are options in some cases. Supportive care includes

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 Chapter 86: Common Oral Lesions 625

treatment with antibiotics when the lesion becomes infected as well should be delayed until after the permanent maxillary (upper)
as ongoing, aggressive preventive oral hygiene. incisors erupt.
Other oral lesions require specific therapy. Thrush is typically Management of oral lesions may require consultation and col-
treated with nystatin suspension (100,000 units/mL) as 1 mL laboration with colleagues in dental, oral surgery, otolaryngology,
swabbed to lesions 4 times per day until lesions are resolved. It or other subspecialties. The recommended treatment of a congeni-
is important to consider the possibility of an underlying immu- tal epulis is early resection; however, if a large lesion with potential
nodeficiency when thrush occurs outside of infancy or without to obstruct the newborn’s airway is diagnosed antenatally, it may be
a reasonable explanation. Angular cheilitis can be managed with necessary to perform an ex-utero intrapartum procedure to establish
nystatin cream or ointment and a low-potency hydrocortisone an airway before interruption of the fetomaternal circulation. If the
cream. Some oral lesions, including congenital epulis, muco- etiology of a lesion is unclear or if an oral lesion does not follow its
cele, pyogenic granuloma, and fibroma, are best managed with expected course, referral is essential to ensure appropriate diagnosis
resection. and treatment.
Treatment of gingivitis should start with ensuring that a child
follows a regular home oral hygiene program, including twice-daily Prognosis
toothbrushing with fluoride toothpaste and flossing, and refer- Most oral lesions in children respond to appropriate intervention
ral for professional dental care. Regular rinsing with a mouth- without residual problems. However, oral lesions can signal the
wash containing essential oils (eg, Listerine Ultraclean, Vita-Myr onset of or occur in association with serious systemic conditions.
Mouthwash) or chlorhexidine gluconate oral preparation has been Although uncommon, rapidly progressive polymicrobial infections
shown to decrease plaque and inflammation. The goals of therapy of oral structures, such as Vincent angina or Ludwig angina, can
for gingivitis are to reduce clinical signs of inflammation and be life-threatening. Chronic gingivitis represents the first and only
gingival bleeding and arrest or reduce the risk of progression of reversible stage of periodontal disease, which is the leading cause of
the periodontal disease and maintain dentition. Drug-induced tooth loss in adulthood. Because chronic gingivitis has its onset dur-
gingival hyperplasia requires therapy similar to that for ordinary ing childhood, physicians can play an important role in the preven-
gingivitis. Additionally, if the causative medication cannot be tion of periodontal disease by promoting oral hygiene early in life.
discontinued or changed, patients can be referred for surgical After periodontal disease extends beyond the gums, it is no longer
removal of excess gingival tissue and fitting of a positive-pressure reversible and gradually destroys the bone and tissue that support
mouth guard to inhibit further tissue growth. Acute necrotizing the teeth, resulting in halitosis and tooth loss.
ulcerative gingivitis should be managed with débridement and
penicillin or metronidazole.
Vincent infection should be managed with débridement and CASE RESOLUTION
penicillin or metronidazole. Ludwig angina requires surgical drain- The child seems to have a mucocele. Mucoceles may spontaneously regress;
age as well as treatment with broad-spectrum antibiotics covering however, if the lesion persists the child should be referred to an oral surgeon
gram-negative, gram-positive, and anaerobic bacteria (eg, ampicillin, or head and neck surgeon for surgical excision of the lesion.
sulbactam). Securing the airway should be prioritized in both
Vincent angina and Ludwig angina.
When a woman reports persistent pain or experiences bruis- Selected References
ing or bleeding of the nipple with breastfeeding, prompt consid-
eration should be given for clinically significant ankyloglossia, American Academy of Pediatric Dentistry. Guideline on management con-
siderations for pediatric oral surgery and oral pathology. Pediatr Dent.
either anterior or posterior. Consultation with a lactation con-
2016;38(6):315–324 PMID: 27931471
sultant is often helpful as a first step when questions arise about
Belenguer-Guallar I, Jiménez-Soriano Y, Claramunt-Lozano A. Treatment
whether ankyloglossia is contributing to breastfeeding difficulty.
of recurrent aphthous stomatitis: a literature review. J Clin Exp Dent.
Not all infants with ankyloglossia have difficulty breastfeeding. 2014;6(2):e168–e174 PMID: 24790718 https://doi.org/10.4317/jced.51401
Systematic review indicates that sublingual frenotomy reduces
Centers for Disease Control and Prevention. Youth and tobacco use. Centers
maternal pain with breastfeeding and increases breastfeeding for Disease Control and Prevention website. https://www.cdc.gov/tobacco/data_
efficacy. Sublingual frenotomy, which involves incising the sublin- statistics/fact_sheets/youth_data/tobacco_use/index.htm. Accessed May 8,
gual frenum, is indicated when ankyloglossia is interfering with 2019
effective breastfeeding. Longitudinal data are insufficient to war- Congenital vascular lesions of the head and neck. Otolaryngol Clin North Am.
rant sublingual frenotomy in infancy as a means to prevent speech 2018;51(1):1–274
problems in later childhood. No research evidence exists to sup- Gibson AM, Sommerkamp SK. Evaluation and management of oral lesions in
port incising the superior (upper lip or maxillary) labial frenum the emergency department. Emerg Med Clin North Am. 2013;31(2):455–463
in infancy to improve breastfeeding, prevent dental caries, or pre- PMID: 23601482 https://doi.org/10.1016/j.emc.2013.02.004
vent future orthodontic problems. Cosmetic interventions for a Gonsalves WC, Chi AC, Neville BW. Common oral lesions: part I. superficial
prominent superior labial frenum and/or concerns for a diastema mucosal lesions. Am Fam Physician. 2007;75(4):501–507 PMID: 17323710

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 626 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Gonsalves WC, Chi AC, Neville BW. Common oral lesions: part II. masses and Power RF, Murphy JF. Tongue-tie and frenotomy in infants with breastfeeding
neoplasia. Am Fam Physician. 2007;75(4):509–512 PMID: 17323711 difficulties: achieving a balance. Arch Dis Child. 2015;100(5):489–494 PMID:
Jamal A, Gentzke A, Hu SS, et al. Tobacco use among middle and high 25381293 https://doi.org/10.1136/archdischild-2014-306211
school students—United States, 2011-2016. MMWR Morb Mortal Wkly Rep. Rowan-Legg A; Canadian Paediatric Society Community Paediatrics Committee.
2017;66(23):597–603 PMID: 28617771 https://doi.org/10.15585/mmwr. Ankyloglossia and breastfeeding [in English, French]. Pediatr Child Health.
mm6623a1 2015;20(4):209–218. Reaffirmed February 28, 2018 PMID: 26038641

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 CHAPTER 87

Otitis Media
Nasser Redjal, MD

CASE STUDY
An 18-month-old boy is brought to your office with a The child has a 10- to 15-word vocabulary, and no
2-day history of fever and decreased food intake. He has one smokes in the household.
had symptoms of an upper respiratory infection for the
past 4 days but no vomiting or diarrhea. Otherwise, he
Questions
1. What are the differences between acute, persistent,
is healthy.
and recurrent otitis media?
The child appears tired but not toxic. On physical exam-
2. What factors predispose to the development of ear
ination, the vital signs are normal except for a temperature
infections?
of 38.3°C (101°F). The left tympanic membrane is erythem-
3. What are the most common presenting signs and
atous and bulging, with yellow pus behind the membrane.
symptoms of ear infection in infants and children?
The light reflex is splayed, and mobility is decreased. The
4. How do the treatment considerations differ between
right tympanic membrane is gray and mobile, with a sharp
acute, persistent, and recurrent ear infections?
light reflex. The neck is supple with shotty anterior cervical
5. What are some of the complications of otitis media?
adenopathy, and the lungs are clear.

Otitis media (OM) is the second most common reason after well- for 2 to 3 months but usually resolves within 3 to 4 weeks in 60% of
child care for a visit to the pediatrician and the most common rea- cases. Recurrent OM is defined as frequent episodes of AOM with com-
son for which antibiotics are prescribed for children. An estimated plete clearing between each episode, although a more specific defini-
30 million office visits per year are for the evaluation and treatment tion of recurrent OM is 3 new episodes of AOM requiring antibiotic
of OM in the United States. More than 25% of all prescriptions writ- treatment within a 6-month period or 4 documented infections in
ten each year for oral antibiotics were for the treatment of middle 1 year. This condition affects approximately 20% of children with
ear infections. Many surgical procedures, such as myringotomy a propensity to otitis; such children typically have their first infection
with tympanostomy tube placement or adenoidectomy, were per- at younger than 1 year. Chronic OME, which is also known as serous
formed on children for treatment of recurrent disease. However, a OM, secretory OM, nonsuppurative OM, mucoid OM, and glue ear
dramatic decline has occurred in the prevalence of OM from the pre- OM, is characterized by persistence of fluid in the middle ear for
pneumococcal conjugated vaccine (PCV) 7 era to the post-PCV13 3 months or longer. The TM is retracted or concave with impaired
era, from 9.5% of office visits to 5.5%, respectively, and from 826 mobility but without signs of acute inflammation. The affected child
per 1,000 children to 387 per 1,000 children, respectively. Despite may be asymptomatic. The child with chronic OME is at increased
this decline, the primary care physician must have a good under- risk for developing hearing deficits, speech delay, and learning prob-
standing of these pediatric conditions, which remain quite common. lems. Chronic suppurative OM implies a non-intact TM (ie, perfora-
Otitis media can be classified into the following 5 categories: tion or tympanostomy tube present) with at least 6 weeks of middle
acute OM (AOM), OM with effusion (OME), recurrent AOM, chronic ear drainage.
OME, and chronic suppurative OM. It is important to distinguish
between each of these entities because their presentation and man- Epidemiology
agement differ. The prevalence of OM peaks in children 6 to 24 months of age. An
Acute OM (ie, acute suppurative or purulent OM) is the sudden additional smaller peak occurs at approximately 4 to 6 years of age.
onset of inflammation of the middle ear, which is often accompanied Otitis media is relatively uncommon in older children and adoles-
by fever and ear pain (ie, otalgia). The clinical findings of inflam- cents. The condition is more common in boys than girls.
mation noted on otoscopic examination are bulging of the tympanic Several epidemiologic risk factors for OM have been identified,
membrane (TM), limited or absent mobility of the TM, air-fluid level including age younger than 2 years; first episode before 6 months;
behind the TM, and otorrhea not resulting from acute otitis externa familial predisposition; siblings in the household; low socio-
(Box 87.1). Otitis media with effusion or serous OM is the persistence economic status; infant not breastfed; altered host defenses (ie,
of nonpurulent middle ear fluid after antimicrobial treatment fol- acquired or congenital immunodeficiencies); environmental factors
lowing resolution of acute inflammatory signs. Fluid may persist (eg, cigarette smoke); child care attendance; and the presence of

627

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 628 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

enterovirus develop AOM. The bacterial pathogens causing AOM

Box 87.1. Components of Acute Otitis Media
in the first 6 weeks after birth are essentially the same as those in
Definition older children. However, 10.5% of neonates with AOM have gram-
A diagnosis of acute otitis media requires the following: negative bacilli.
ww A history of acute onset of signs and symptoms Currently, approximately 50% of H influenzae and 100% of
ww The presence of middle ear effusion M catarrhalis isolated from the upper respiratory tract are positive
ww Signs and symptoms of middle ear inflammation for b-lactamase, and 15% to 50% (average, 30%) of S pneumoniae are
Findings on Examination not susceptible to penicillin. The mechanism of penicillin resistance
ww The presence of middle ear effusion that is indicated by any of the among isolates of S pneumoniae is associated not with b-lactamase
following: production but with an alteration of penicillin-binding proteins.
— Bulging of the tympanic membrane This effect varies widely by geographic location and results in resis-
— Limited or absent mobility of the tympanic membrane tance to penicillins and cephalosporins.
— Air-fluid level behind the tympanic membrane
— Otorrhea (not resulting from acute otitis externa) Clinical Presentation
Children with AOM often have a history of fever and ear pain.
Derived from Lieberthal AS, Carroll AE, Chonmaitree T, et al. The diagnosis and management of
acute otitis media. Pediatrics. 2013;131(3):e964–e999. Associated symptoms include URI, cough, vomiting, diarrhea,
and nonspecific symptoms, such as decreased appetite, waking
at night, generalized malaise, lethargy, and irritability. Purulent
an underlying condition, such as allergic disease of the upper air- otorrhea with minimal ear pain and hearing loss may also occur and
way, chronic sinusitis, a cleft palate, or other craniofacial anoma- signifies rupture of the TM. Fever occurs in approximately 30% to
lies. Children with Down, Goldenhar, or Treacher Collins syndrome 50% of patients. Temperatures exceeding 40°C (104°F) are uncom-
and ciliary dysfunction also are at increased risk for OM. American mon and are suggestive of bacteremia or another complication.
Indian/Alaska Native individuals have a higher incidence of AOM Verbal children may report tinnitus, vertigo, and hearing loss; Bell
than whites. Otitis media usually occurs during the winter and palsy is a rare finding. Nonverbal children may appear ataxic on
early spring, coinciding with peaks in the incidence of viral upper physical examination.
respiratory infections (URIs).
Worldwide, OM results in an estimated 50,000 deaths per year Pathophysiology
in children younger than 5 years because of the complications of The most important factor in the pathogenesis of OM is abnor-
chronic suppurative OM. Otitis media is estimated to affect 65 mil- mal function of the eustachian tube (Figure 87.1). Eustachian tube
lion to 133 million individuals worldwide, 60% of whom experience dysfunction occurs for 2 main reasons: abnormal patency and
significant hearing loss. Chronic suppurative OM is a rare entity in obstruction of the tube. Obstruction is functional (secondary
developed countries, in which most instances of OM are of the acute to collapse of the tube), mechanical (from intrinsic or extrinsic
presentation or with effusion. causes), or both. Functional obstruction or collapse of the eusta-
chian tube is common in infants and young children because the
Etiology tube is less cartilaginous and therefore less stiff than in adults; the
In general, 50% to 90% of cases of AOM culture are bacterial, 20% tube is also more horizontal and shorter in infants and young chil-
to 50% are viral, and 66% are both. Until the widespread immu- dren. Additionally, the tensor veli palatini muscle is less efficient
nization of children with PCV, the causative microorganisms for in this age group. Extrinsically, the presence of lymphoid follicles
AOM were Streptococcus pneumoniae (25%–50%), non-typable (eg, adenoidal enlargement) or, rarely, tumors surrounding the
Haemophilus influenzae (15%–30%), and Moraxella catarrhalis opening of the tube contributes to reflux, aspiration, or insufflation
(3%–20%). Other less common causative organisms include group of nasopharyngeal bacteria into the middle ear. Intrinsic mechani-
A streptococcus, Staphylococcus aureus, α-hemolytic Streptococcus, cal obstruction of the eustachian tube occurs as the result of inflam-
Pseudomonas aeruginosa, anaerobic bacteria, Mycoplasma pneu- mation secondary to a URI or allergy in patients older than 5 years.
moniae, chlamydia, and Mycobacterium tuberculosis. Bullous Viral infections may occur up to 6 to 12 times per year in children
myringitis has shown a 97% bacterial-positive rate, primarily with younger than 3 years. Subsequently, viral respiratory infections
S pneumoniae, in contrast to the previous belief that mycoplasma contribute to eustachian tube dysfunction, resulting in negative
was the causative agent in this condition. Respiratory viruses, such middle ear pressure, which occurs in 75% of children who have
as respiratory syncytial virus, adenovirus, rhinovirus, parainfluenza, viral URIs. The presence of a viral URI enhances the ability of
coronavirus, and influenza (A and B), also play a role. Respiratory bacterial pathogens to adhere to and ascend from the nasopharynx
syncytial virus, adenovirus, and coronavirus are associated with an to the middle ear via the eustachian tube. Viruses also can affect
increased rate of AOM, with 50% of children with URI caused by the local host immune response by impairing leukocyte function,
these viruses developing AOM; in contrast, only 33% of patients exposing receptors for bacteria, and decreasing the effectiveness of
who have URIs caused by rhinovirus, influenza, parainfluenza, or the mucociliary escalator (Figure 87.1).

BPPCA6e_Ch087_0627-0634.indd 628 2/13/20 6:26 PM

 Chapter 87: Otitis Media 629

Skull base Ossicles

(malleus, incus, Semicircular
stapes) canals Acoustic
nerve

Cochlea

Tympanic
membrane Eustachian
tube
External
canal

Figure 87.1. Relationship of middle ear to external and inner ears.

Hematogenous spread of microorganisms also can result in OM.

Box 87.2. What to Ask
Less often, primary mucosal disease of the middle ear from allergies
or abnormal cilia contributes to OM. Otitis Media
ww Does the infant or child have fever, ear pain, hearing loss, or otorrhea?
Differential Diagnosis ww Is the infant or child inconsolable or lethargic?
The most common cause of otalgia is AOM. Other causes include ww Has the infant or child had a previous ear infection? If so, when?
mastoiditis, which is almost always accompanied by OM; otitis externa; ww Did the child complete the course of prescribed antibiotics?
and referred pain from the oropharynx, teeth, adenoids, or posterior ww How many ear infections has the child had in the past year?
auricular lymph nodes. A foreign body in the canal can produce simi- ww Is the child taking any medication to prevent recurrent otitis media?
lar symptoms. In the child with ear pain, a search for any of these other ww Does the child attend child care?
conditions must be undertaken if the TM appears completely normal. ww Is the child exposed to passive smoke?
ww Is the infant breastfed?
Evaluation ww Does the child seem to hear?
History ww Is the child’s speech development normal?
The history should carefully delineate the symptoms of OM and
differentiate from those indicating a more serious condition, such of inflammation usually are absent and the TM may be retracted,
as meningitis (Box 87.2). with limited or no mobility. Smartphone apps exist that can be used
For the infant or child with a history of persistent or recurrent in assessing the mobility of the TM and can assist in diagnosing OM.
OM, it is important to discern when the last documented infection Associated physical findings with an uncomplicated middle ear
occurred and what treatment, if any, was administered. It is also infection may include posterior auricular and cervical adenopa-
critical to monitor development, particularly speech. thy. Other significant findings on physical examination are pain on
movement of the pinna, anterior ear displacement, posterior auric-
Physical Examination ular pain, and, rarely, evidence of peripheral facial nerve (ie, sev-
To diagnose OM, the TM must be completely visualized and its mobil- enth cranial nerve) paralysis. The presence of these findings suggests
ity assessed. Occasionally, this may be difficult because of the pres- other diagnoses, such as an associated otitis externa or mastoiditis.
ence of cerumen or otorrhea. In such cases, diagnosis is made on Positioning of the infant or young child for evaluation of the ear
the basis of the history, and treatment may be initiated without con- is critical for an adequate examination. Several methods have been
firmation by physical assessment. In all other cases, the position, described, including restraining the child on the examination table
color, degree of translucency, and mobility of the TM are evaluated. or allowing parents or guardians to hold the child in their arms or
Classically, in AOM the TM is full, bulging, hyperemic, opaque, or has on their laps (Figure 87.2). In the infant, it may be difficult to visu-
an air-fluid level, with limited or no mobility. Typically, the light reflex alize the TM because the external auditory canal is slightly angu-
is distorted or absent. In the case of persistent or chronic OM, signs lated. Lateral retraction of the pinna may help correct this problem.

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 630 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Figure 87.2. Three methods of positioning an infant or child for examination of the ear. Left, Restraining the infant on the examination
table. Middle, Holding the child in the arms. Right, Holding the child on the lap.

Laboratory Tests Immunoprophylaxis with influenza vaccine and PCV has proven
Although the diagnosis of OM is suspected on the basis of the effective in reducing the prevalence of OM. Avoiding supine bottle-
history and verified on physical examination, tympanometry feeding (ie, bottle propping) and reducing or eliminating pacifier
may be helpful in distinguishing the normal ear from the ear with use between age 6 and 12 months, as well as eliminating exposure
effusion. In acute cases, audiometry is of limited diagnostic value; to passive tobacco smoke, decreases the incidence of AOM.
however, it is helpful in evaluating the effects of a persistent, recur- Breastfeeding, which provides infants with immunologic protec-
rent, or chronic middle ear effusion (MEE) on hearing. tion against URIs, other viral and bacterial infections, and allergies,
Tympanocentesis is the most definitive method of verifying the also has a protective effect. Facial musculature may mature differ-
presence of middle ear fluid and of recovering the organism respon- ently in breastfed infants, thus influencing eustachian tube func-
sible for the infection. Indications for tympanocentesis or myringot- tion and reducing the risk of aspiration of fluid into the middle ear.
omy are listed in Box 87.3. Nasopharyngeal cultures are not helpful Positioning during breastfeeding also has some protective effect,
because they do not correlate with middle ear fluid cultures. although immune factors in human milk may serve as the most
important mechanism for the reduced prevalence of OM.
Prevention Increased antibiotic resistance has eliminated the utility of
routine antibiotic prophylaxis for recurrent AOM as a means of
During infancy and early childhood, the incidence of respiratory
disease prevention.
tract infections and recurrent OM can be reduced by altering child
care center attendance patterns.
Management
In 2013, the American Academy of Pediatrics released clinical
Box 87.3. Indications for Tympanocentesis or guidelines on the management of OM (Box 87.4). One of the per-
Myringotomy in the Child With Otitis Media haps more controversial recommendations was to, depending on
ww Otitis media in the patient with severe ear pain, serious illness, or the clinical findings in the child between 6 and 24 months of age,
appearance of toxicity not routinely initiate antibiotics but instead observe the child. The
ww Onset of otitis media in the child receiving appropriate and adequate American Academy of Pediatrics has not revised these guidelines.
antimicrobial therapy Some physicians, based on several subsequent meta-analyses detail-
ww Otitis media associated with confirmed or potential suppurative compli- ing the risk of serious complications, including TM perforation,
cations, such as facial paralysis, mastoiditis, or meningitis seventh cranial nerve palsy, subperiosteal abscess, mastoiditis, sinus
ww Otitis media in the newborn, ill neonate, or immunodeficient patient, vein thrombosis, labyrinthitis, bacteremia, and bacterial meningitis,
in each of whom an unusual organism may be present have opted to start antibiotics at the initial encounter.
ww Otitis media in the patient with severe illness in whom second-line High-dose amoxicillin is recommended as the first-line treatment
antibiotic management has been unsuccessful in most patients with AOM, although several medications are clinically
ww Otitis media in the patient with penicillin allergy in whom the first-line effective (Table 87.1). The justification for the use of amoxicillin relates
agent was unsuccessful to its effectiveness against common AOM bacterial pathogens as well
as its safety, low cost, acceptable taste, and narrow microbiological

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 Chapter 87: Otitis Media 631

cefdinir and cefuroxime indicate efficacy of only 70% to 80%, compared

Box 87.4. Indications for Antibiotics in the
with 84% to 92% amoxicillin efficacy. In vitro efficacy of cefdinir and
Management of Otitis Media
cefuroxime against H influenzae is approximately 98%, compared with
Criteria for Antibacterial Treatment or Observation in the Child with 58% efficacy of amoxicillin and nearly 100% efficacy of amoxicillin-
Non-Severe Illness clavulanate. A multicenter double tympanocentesis open-label study
1. Younger than 6 months: antibacterial treatment of cefdinir in recurrent AOM attributable to H influenzae showed
2. Six to 24 months: antibacterial treatment with certain diagnosis or severe eradication of the organism in 72% of patients.
illness; observation with unilateral acute otitis media without otorrhea, For children allergic to penicillin, recent data suggest that
although a 2015 meta-analysis showed better outcome with antibiotics cross-reactivity among penicillins and cephalosporins is lower
3. Two years and older: antibacterial treatment with severe illness or observa- than historically reported. Cross-reactivity is higher between
tion in the setting of uncomplicated unilateral or bilateral acute otitis media penicillins and first-generation cephalosporins but is negligible
Observation is an Appropriate Option if All of the Following Are Present: between penicillins and second- and third-generation cepha-
A. Caregiver is informed and agrees losporins. According to the Joint Council of Allergy, Asthma &
B. Caregiver can monitor the child and return should condition worsen Immunology, cephalosporin treatment of patients with a history
C. Systems are in place for ready communication with the physician, of mild delayed penicillin allergy (ie, maculopapular or morbilli-
reevaluation, and obtaining medication, if necessary form eruption after 6 hours of taking penicillin) shows a reaction
rate of less than 0.1% to cephalosporin antibiotics and can be pre-
Adapted with permission from Lieberthal AS, Carroll AE, Chonmaitree T, et al. The diagnosis and scribed to this subset of patients. This treatment is not appropri-
management of acute otitis media. Pediatrics. 2013;131(3):e964–e999.
ate for individuals with severe reaction histories to penicillin (eg,
anaphylaxis urticaria, angioedema, bronchospasm, serious delayed
spectrum. In children with a history of recurrent AOM or who have reaction [eg, Stevens-Johnson syndrome, toxic epidermal necroly-
taken amoxicillin in the previous 30 days, those with concurrent sis, hemolytic anemia]) or uncertain type of reaction where treat-
conjunctivitis, or those for whom coverage for b-lactamase-positive ment with another antibiotic (azithromycin, clarithromycin, or
H Influenzae and M catarrhalis is desired, therapy should be initiated erythromycin-sulfisoxazole) is recommended.
with high-dose amoxicillin-clavulanate (90 mg/kg per day of amoxicillin Currently, tympanocentesis is rarely performed before the
with 6.4 mg/kg per day of clavulanate in a ratio of amoxicillin to clavu- initiation of antibiotics. Indications for tympanocentesis include
lanate of 14:1, given in 2 divided doses; this preparation is less likely toxic appearance or severe ear pain, because the procedure pro-
than other amoxicillin-clavulanate preparations to cause diarrhea). vides relief through decompression. Tympanocentesis is occasion-
Alternative initial antibiotics include cefdinir (14 mg/kg per day ally performed in a child who has been receiving appropriate and
in 1 or 2 doses), cefuroxime (30 mg/kg per day in 2 divided doses), adequate antimicrobial therapy but in whom such treatment does
cefpodoxime (10 mg/kg per day in 2 divided doses), or ceftriaxone not result in improvement; in the setting of confirmed or poten-
(50 mg/kg, administered intramuscularly). Alternative antibiotics tial suppurative complications, such as facial paralysis, mastoiditis,
vary in their efficacy against AOM pathogens. For example, recent or meningitis; or in the newborn, ill neonate, or immunodeficient
data from the United States on in vitro susceptibility of S pneumoniae to patient in whom an unusual organism may be present (Box 87.3).

Table 87.1. Recommended Antibiotics for Initial or Delayed Treatment and for Patients Who Have Failed
Initial Antibiotic Treatment
Initial Antibiotic Treatment at AOM Diagnosis or After Observation Antibiotic Treatment After 48–72 Hours of Initial Antibiotic Treatment Failurea
Recommended First-Line Treatments Alternative Treatments Recommended First-Line Treatments Alternative Treatments
Amoxicillin (80–90 mg/kg per day) OR Cefdinir (14 mg/kg per day Amoxicillin–clavulanate (90 mg/kg Clindamycin (30–40 mg/kg per day in
Amoxicillin–clavulanateb (90 mg/kg in 1 or 2 doses) per day of amoxicillin, with 6.4 mg/kg 3 divided doses), with or without second-
per day of amoxicillin, with 6.4 mg/kg Cefuroxime (30 mg/kg per day of clavulanate) OR or third-generation cephalosporinb
per day of clavulanate) per day in 2 divided doses) Ceftriaxone (50 mg/kg per day IM or
Cefpodoxime (10 mg/kg IV for 3 days)
per day in 2 divided doses)
Ceftriaxone (50 mg/kg per
day IM or IV for 1–3 days)
a
If no improvement with second course of antibiotics, consider tympanocentesis and consultation with an otolaryngologist.
b
May be considered in patients who have received amoxicillin in the previous 30 days or who have the otitis–conjunctivitis syndrome.
Abbreviations: IM, intramuscular; IV, intravenous.
Adapted with permission from Lieberthal AS, Carroll AE, Chonmaitree T, et al. Erratum. The diagnosis and management of acute otitis media. Pediatrics 2014;133(2):346–347 DOI: https://doi.org/10.1542/
peds.2013-3791.

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 632 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Adjunctive medications, such as topical analgesics (antipyrine

and benzocaine ear drops) and antipyretic agents are important ther- CASE RESOLUTION
apeutic options and should be prescribed for the child with signifi- The child displays the classic signs and symptoms of AOM: fever, URI,
cant pain or fever. Antihistamines, decongestants, or steroids have decreased appetite, and an abnormal TM on physical examination. Because
no documented role in the management of OME. Most resolving of his age and fever and the certainty of diagnosis, he should be treated for
10 days with oral amoxicillin. The prognosis is good given his normal speech
MEEs that occur after AOM in otherwise healthy children do not
development.
need to be treated; the wait-and-watch approach is recommended.
Failure of antibiotic treatment and chronic effusions warrant a refer-
ral to an otolaryngologist for further evaluation for pressure equaliza-
tion tubes, especially if an effusion is present for more than 3 months Selected References
and associated symptoms, such as vestibular symptoms, school or
American Academy of Pediatrics. Antimicrobial stewardship: appropriate and
behavioral problems, ear discomfort, or decreased quality of life, are
judicious use of antimicrobial agents. In: Pickering LK, Baker CJ, Kimberlin DW,
present. The physician may recommend pressure equalization tubes Long SS, eds. Red Book: 2012 Report of the Committee on Infectious Diseases.
to manage recurrent OM (3 episodes in 6 months or 4 episodes in 29th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2012:802–806
1 year, with 1 episode in the preceding 6 months). Episodes of recur- American Academy of Pediatrics Section on Breastfeeding. Breastfeeding and the
rent OM should be well documented as separate acute infections. Other use of human milk. Pediatrics. 2012;129(3):e827–e841 PMID: 22371471 https://
indications for consultation include hearing loss; any anatomic abnor- doi.org/10.1542/peds.2011-3552
mality, such as a defect of the TM (eg, perforations, cholesteatomas) or Arguedas A, Emparanza P, Schwartz RH, et al. A randomized, multicenter,
intranasal problems (eg, deviated septum, polyp); signs and symptoms double blind, double dummy trial of single dose azithromycin versus high
of an OM but normal physical examination; and a predisposition to dose amoxicillin for treatment of uncomplicated acute otitis media. Pediatr
chronic recurrent OM (eg, child with a cleft palate or Down syndrome). Infect Dis J. 2005;24(2):153–161 PMID: 15702045 https://doi.org/10.1097/01.
inf.0000151024.11703.4a
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For the child younger than 2 years and any child with severe symp- the first two months of life: characteristics and diagnostic difficulties. Arch Dis Child.
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A 7-day course of oral antibiotic appears to be equally effec- Block SL, Heikkinen T, Toback SL, Zheng W, Ambrose CS. The efficacy of live
tive in children 2 to 5 years of age with mild or moderate AOM. attenuated influenza vaccine against influenza-associated acute otitis media in
For the child 6 years or older with mild to moderate symptoms, children. Pediatr Infect Dis J. 2011;30(3):203–207 PMID: 20935591 https://doi.
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a 5- to 7-day course of oral antibiotics is adequate.
Brunton S, Pichichero ME. Acute otitis media: influence of the PCV-7 vac-
Follow-up of the Patient With Acute cine on changes in the disease and its management. J Fam Pract. 2005;54(11):
961–968 PMID: 16266602
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Casey JR, Pichichero ME. Changes in frequency and pathogens causing acute
The physician may choose to reassess some children in 10 to 14 days,
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such as the young child with severe symptoms or recurrent AOM or 15361720 https://doi.org/10.1097/01.inf.0000136871.51792.19
when specifically requested by the child’s parent or guardian.
Centers for Disease Control and Prevention. National, state, and urban area vac-
Persistent MEE is common and can be detected by pneumatic cination coverage among children aged 19-35 months—United States, 2005.
otoscopy (with or without verification by tympanometry) after res- MMWR Morb Mortal Wkly Rep. 2006;55(36):988–993 PMID: 16971887
olution of acute symptoms. Two weeks after successful antibiotic Coco A, Vernacchio L, Horst M, Anderson A. Management of acute otitis media
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age decreases to 40% at 1 month and to 10% to 25% at 3 months Pediatrics. 2010;125(2):214–220 PMID: 20100746 https://doi.org/10.1542/
after successful antibiotic treatment. peds.2009-1115
Cohen R, Levy C, Chalumeau M. Shortened antimicrobial treatment for acute
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org/10.1056/NEJMc1700966
Complications associated with OM are uncommon with appropri-
Coker TR, Chan LS, Newberry SJ, et al. Diagnosis, microbial epidemiology, and
ate antibiotic therapy and follow-up. Such complications are either
antibiotic treatment of acute otitis media in children: a systematic review. JAMA.
extracranial or intracranial. Extracranial complications include 2010;304(19):2161–2169 PMID: 21081729 https://doi.org/10.1001/jama.2010.1651
perforation of the TM, conductive and sensorineural hearing loss,
Damoiseaux RA, van Balen FA, Hoes AW, Verheij TJ, de Melker RA. Primary care
ossicular fixation or discontinuity (eg, adhesive OM), cholestea- based randomised, double blind trial of amoxicillin versus placebo for acute oti-
toma, mastoiditis, petrositis, facial nerve (ie, seventh cranial nerve) tis media in children aged under 2 years. BMJ. 2000;320(7231):350–354 PMID:
paralysis, osteomyelitis of the temporal bone, and Bezold abscess. 10657332 https://doi.org/10.1136/bmj.320.7231.350
Intracranial complications are meningitis, extradural as well as DePestel DD, Benninger MS, Danziger L, et al. Cephalosporin use in treatment
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Gould JM, Matz PS. Otitis media. Pediatr Rev. 2010;31(3):102–116 PMID: Rovers MM, Glasziou P, Appelman CL, et al. Antibiotics for acute otitis media: a
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and Immunology; American College of Allergy, Asthma and Immunology; Joint Spiro DM, Tay KY, Arnold DH, Dziura JD, Baker MD, Shapiro ED. Wait-and-see
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peds.2007-2703 Whitney CG, Farley MM, Hadler J, et al; Active Bacterial Core Surveillance of
Piglansky L, Leibovitz E, Raiz S, et al. Bacteriologic and clinical efficacy of the Emerging Infections Program Network. Decline in invasive pneumococ-
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inf.0000065688.21336.fa NEJMoa022823
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 CHAPTER 88

Hearing Impairments
Patricia Padlipsky, MD, FAAP

CASE STUDY
A 15-month-old girl is brought to the office because points and grunts to indicate her needs. She does not
her parents are concerned that she has not yet begun to respond to loud noises by turning her head.
speak. The child was the product of a term uncompli- The child’s growth parameters, including head cir-
cated pregnancy. Her 25-year-old mother, who began to cumference, are normal for age. The remainder of the
receive regular prenatal care during the second month physical examination is unremarkable.
of gestation, had no documented infections during the
pregnancy, took no medications, and denies using illicit
Questions
1. When should deafness be suspected in infants and
drugs or alcohol. The child was delivered at home by a
children?
midwife, and a newborn hearing screening was never
2. What is the relationship between hearing loss and
done. The 27-year-old father is reportedly healthy. The
language development?
family history is negative for deafness, intellectual dis-
3. What are the major causes of deafness in children?
ability, and consanguinity.
4. Which neonates are at risk for the development of
The child, who is otherwise healthy, has never been
hearing deficits?
hospitalized, but she has had 3 documented ear infec-
5. What methods are currently available for evaluating
tions. She rolled over at 4 to 5 months of age, sat at
hearing in infants and children?
7 months, and walked at 13 months. She can scribble. The
6. What are the important issues to address with fam-
parents report that their daughter smiles appropriately,
ilies who have infants or children with suspected
laughs occasionally, and plays well with other children.
hearing impairment?
As an infant, the girl cooed and babbled, but she now

Any amount of hearing loss, whether unilateral or bilateral, can Epidemiology

cause a significant childhood disability that can compromise speech
The prevalence of congenital deafness in children is approximately
and language development, academic performance, and social
0.1%. In other words, 1 in 1,000 children is born with severe to pro-
and emotional development. It is essential to identify hearing
found hearing loss. The prevalence increases to 6 in 1,000 when
loss as soon as possible to implement early intervention, which
all degrees of hearing loss, mild to profound, are considered. By
has been shown to prevent many adverse consequences. Children
age 18 years, it is estimated that 17 in 1,000 children have some
may be born with a hearing deficit (ie, congenital deafness), or
degree of permanent hearing loss. This increase over time reflects
they may acquire the condition during childhood (ie, late-onset
the addition of patients with progressive, acquired, or late-onset
deafness). Hearing loss also may be progressive and not identified
genetic causes. Diagnostic findings for ANSD often are not conclu-
on early screening. Hearing loss can be classified as conductive,
sive in newborns because language skills are still developing and not
sensorineural, mixed, or auditory neuropathy spectrum disorder
aberrant at the time of newborn screening.
(ANSD). Hearing loss is further described by the degree of loss,
Prior to the initiation of newborn hearing screening, the aver-
whether mild (26–40 dB loss), moderate (40–70 dB loss), severe
age age at diagnosis of most children who were born with hearing
(71–90 dB loss), or profound (>90 dB loss). The most important
impairment was 2 to 3 years. Since the advent of such screening,
period for speech and language development is from birth to 3 years
however, the average age at diagnosis has dropped to 2 to 3 months.
of age. Reduced hearing acuity in both ears or even 1 ear during
Currently, all 50 states have a universal newborn hearing screen-
this time can significantly interfere with this important process
ing as well as early hearing detection and intervention programs.
(see Chapter 33). Therefore, the primary care physician must have
More than 95% of all newborns are screened, and 74% of those
a clear understanding of when to suspect impaired hearing in
found to have hearing impairment have entered an intervention
infancy and early childhood and must be familiar with the iden-
program by 6 months of age. As with any screening program, how-
tification of, evaluation methods for, and treatment options for
ever, some newborns are missed, especially with at-home births.
hearing loss.

635

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 636 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Additionally, as many as 40% of identified newborns are currently

Box 88.1. Risk Factors for Hearing Impairment
lost to follow-up, and some forms of early-onset hearing loss are
not apparent at birth. Therefore, careful assessment of hearing and ww Family history of congenital or early SNHL
language development by the medical professional is essential at ww Congenital infection known to be associated with SNHL
each patient encounter. ww Craniofacial anomalies
More than 90% of children with deafness are born to hearing ww Birth weight <1,500 g (<3.3 lb)
parents. An estimated 20% to 30% of children with hearing impair- ww Hyperbilirubinemia over the exchange level
ment develop the condition during childhood. Of these, 70% of ww Infectious diseases associated with SNHL
children with acquired hearing loss are initially identified by par- ww Exposure to ototoxic medications
ents rather than physicians. Any concern by parents that their child ww Bacterial meningitis
might have a hearing problem should be taken seriously by the phy- ww Low Apgar scores at birth
sician and objective testing performed. Risk factors for acquired ww Prolonged mechanical ventilation in neonatal period
hearing loss in childhood include persistent otitis media with ww Findings of a syndrome associated with SNHL
effusion, history of head trauma, bacterial meningitis, and iden- ww Any parental/caregiver concern about hearing, speech, language, or
tification of syndromes or neurologic disorders associated with developmental delay
hearing loss. ww Head trauma, especially with fracture of the temporal bone
Graduates of the neonatal intensive care unit are at significantly ww Neurodegenerative disorders
increased risk for sensorineural hearing loss (SNHL) and ANSD, with
Abbreviation: SNHL, sensorineural hearing loss.
reported rates of SNHL and ANSD of 16.7 and 5.6 per 1,000 infants,
respectively, compared with an estimated incidence of ANSD in a
well-baby population of 0.06 per 1,000 infants. Diagnostic find-
are approximately 90 dB. Therefore, children with a 50-dB hearing
ings for ANSD often are not conclusive in newborns because
loss may hear their mother when she yells at them and may startle
language skills are still developing and not aberrant at the time
when the telephone rings. However, they do not hear most conver­
of newborn screening. Associated factors, such as preterm birth
sational speech, and they may not always hear the teacher in a class-
(birth weight <1,500 g [<3.3 lb]), hyperbilirubinemia, prolonged
room, especially if they are not in the front and in the setting of
mechanical ventilation, extracorporeal membrane oxygenation
significant extraneous noise. This can result in behavioral problems,
treatment, perinatal asphyxia, exposure to ototoxic drugs, and neo-
such as inattention, temper tantrums, and aggressive play with other
natal sepsis increase the risk for ANSD. Other factors associated with
children (Box 88.2).
deafness in childhood include meningitis, parental consanguinity,
Worsening speech or school performance may herald long-
craniofacial malformations, congenital viral infections, exposure to
standing or progressive mild-to-moderate hearing loss. Other pre-
chemotherapy, and a family history of deafness.
sentations of mild or progressive hearing loss may consist of either
withdrawal from social activities and playing alone or playing the
Clinical Presentation television and music at increasingly loud volumes.
The newborn may present to the primary care physician having had Hearing impairment can be particularly difficult to recog-
an abnormal newborn hearing screening test. The initial newborn nize in infants younger than 6 months because they may have
screening is mandated by 1 month of age, with definite testing by no obvious symptoms of a hearing deficit. They may startle to
an audiologist for abnormal tests by 3 months of age. Intervention moderately loud noises and begin to vocalize as other infants
should begin by 6 months. For the infant who passes hearing test- do (Figure 88.1). If the history is suggestive of a hearing defi-
ing but has positive risk factors for hearing impairment (Box 88.1), cit or a parent or caregiver expresses concern, audiologic test-
communication skills should be assessed at every well-child visit ing should be performed.
and diagnostic audiologic assessment should be done by 24 to
30 months of age.
In the unscreened population or for children with progressive
or acquired hearing loss, parents are often the first to suspect Box 88.2. Diagnosis of Hearing Impairment
hearing loss. A parent may be concerned that the toddler is indi- in the Pediatric Patient
cating needs by grunting and pointing rather than using words ww Parental concern or suspicion of hearing loss
or that the child does not seem to respond to sounds. Children ww Delayed speech and language development
with hearing impairments frequently present to physicians with ww Associated risk factors, including preterm birth, exposure to ototoxic
delayed speech or speech impediments; children produce what drugs, congenital or acquired central nervous system infections, family
they hear. history of hearing loss, and craniofacial abnormalities
Normal speech volumes range from 30 to 50 dB, whereas typ- ww History of behavioral problems or poor school performance
ical street traffic volume is approximately 60 dB. Standard tele- ww Abnormal hearing test
phone rings and shouts are approximately 80 dB, and lawnmowers

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 Chapter 88: Hearing Impairments 637

Pitch (Hz)
Pathophysiology
250 500 1,000 2,000 4,000 8,000 Mechanism of Hearing
Sounds in the form of pressure waves are carried from the envi-
0 ronment through the external auditory canal to the tympanic
membrane (TM). These waves are converted to mechanical
10 vibrations by the ossicles, and the mechanical vibrations are
then transmitted from the TM to the inner ear, where they are
“ f”
20 transformed to fluid vibrations. Finally, these fluid vibrations
“s” fis h
are converted into nerve impulses by nerve endings within the
30
organum spirale located in the cochlea in the inner ear. These
“a” s i t impulses are conducted via the auditory nerve to the auditory
40
“o” cortex (Figure 88.2).
50
c at Hearing impairments can be classified according to the part of
Loudness (dB)

oh the auditory system affected or by the cause of the hearing loss.

60
Types of Hearing Loss
70 A conductive hearing loss (CHL) denotes an abnormality from the
pinna, external auditory canal, TM, or middle-ear ossicles. Conductive
80 hearing loss can occur in congenital anomalies, such as atresia,
impacted cerumen, otitis externa, serous otitis media, otitis media
Honk!
90 with effusion, TM perforation, ossicular discontinuity, and otosclero-
sis. An SNHL is an abnormality that affects the cochlea, inner ear, or
100
auditory nerve. Congenital infections, anomalies, genetic disorders,
and loud noise result in this type hearing loss. The loss of cochlear
110
function, usually from damage to sensory hair cells in the organum
spirale, is the main cause of permanent childhood hearing loss. A
120
mixed disorder has characteristics of both conductive and sensorineu-
ral losses. In retrocochlear hearing loss, the auditory nerve, brain stem,
or cortex is affected. This includes ANSD noted in neonates after neo-
Figure 88.1. Loudness of everyday sounds. natal intensive care unit stays. In auditory neuropathy, sound enters

Ossicles
Skull base
(malleus, incus, Semicircular
stapes) canals Acoustic
nerve
External
canal

Cochlea

Eustachian
tube

Tympanic
membrane

Figure 88.2. Sound waves (represented by arrows) passing through the external ear into the middle ear.

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 638 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

the ear normally, but because of damage to the inner ear or the hearing however, and result from a single gene defect encoding connexin
nerve, sound is not organized in a way that the brain can understand. 26 protein. This recessive disorder with mutations in the GJB2 gene
accounts for 30% to 50% of all cases of nonsyndromic hearing loss.
Etiology of Hearing Impairment Genetic malformations of the ear pinnae or ossicles do occur but
The causes of hearing loss can be broadly divided into genetic are the least common cause of hearing loss. Genetic mutations may
and acquired and further divided into congenital and/or progres- result in different types of deafness with various presentations and
sive (Table 88.1). Of the 1 in 1,000 individuals born with severe to outcomes, that is, hearing loss may be conductive, sensorineural, or
profound hearing loss, the cause is genetic in approximately 50%, mixed and may be static or progressive, with the initial presentation
nongenetic in approximately 25%, and idiopathic in approximately in infancy or later childhood.
25%. Of the 50% with a genetic cause, the hearing loss is syndromic Acquired environmental causes of hearing loss include prenatal,
in 30% and nonsyndromic in 70%. More than 500 forms of syn- perinatal, or postnatal events and exposures, such as congenital
dromic hearing loss exist, each with associated clinical features. infections, bacterial meningitis, hyperbilirubinemia, complications
Most of these syndromes are rare. Waardenburg syndrome is the of prematurity, and exposure to ototoxic drugs. Cytomegalovirus
most common type of autosomal dominant syndrome with SNHL. (CMV) likely is the most frequently unrecognized congenital infec-
Usher syndrome and Pendred syndrome with goiter are examples tion causing deafness. Even if an infant is asymptomatic from the
of autosomal recessive syndrome with SNHL. Down (ie, trisomy 21) CMV infection, a 10% to 15% chance exists that the infant will
syndrome and oculoauriculovertebral dysplasia (ie, Goldenhar syn- develop an SNHL. Other congenital infections, such as toxoplasmo-
drome) often have associated hearing impairment. Alport syndrome sis, measles, mumps, rubella, herpes simplex virus, HIV, and syphi-
with progressive SNHL and nephritis is also well recognized and lis can also cause hearing loss. Hearing loss associated with bacterial
is X-linked. Most cases of genetic hearing loss are nonsyndromic, meningitis accounts for as many as 20% of cases, with Streptococcus
pneumoniae as the prevalent responsible organism. The incidence
of S pneumoniae and Haemophilus influenza type b meningitis has
Table 88.1. Major Causes of Childhood Deafness decreased tremendously in young children following the advent of
Hearing conjugate vaccine, and the prevalence of postmeningitic hearing
Loss Type Conductive Hearing Loss Sensorineural Hearing Loss loss has similarly declined. The role of steroids in the management
of bacterial meningitis has also contributed to the decrease in SNHL
Congenital Microtia/atresia Genetic disorders (eg, syn-
in survivors.
Tympanic membrane dromic, connexin 26,
With the recognition and treatment of hyperbilirubinemia in
abnormalities mitochondrial)
term newborns, hyperbilirubinemia as a cause of hearing loss
Ossicular malformations In utero infection (eg, cyto- is now rare in the United States and other developed countries.
megalovirus, measles, mumps, Preterm birth as a cause of hearing loss is, however, not uncom-
rubella, varicella, syphilis) mon. Because of associated complications, preterm newborns have
Anatomic abnormalities of higher rates of severe hearing loss than do term newborns. Some
the cochlea or temporal bone antibiotics (eg, aminoglycosides) and other medications (eg, loop
Exposure to ototoxic drugs diuretic agents) can be irreversibly ototoxic; other drugs may cause
during pregnancy only transient effects.
(eg, alcohol, isotretinoin, Any head injury, especially if the injury damages the temporal
cisplatin) bone, can cause deafness in children. Fractures through the cochlea
Hyperbilirubinemia and vestibule can result in severe to profound hearing loss, and
Acquired Infection (eg, acute oti- Infection (eg, bacterial men- damage to the TM and/or ossicles can result in a significant CHL.
tis media, otitis externa, ingitis, measles, mumps, Acoustic trauma (ie, noise-induced hearing loss) from continuous or
ossicular erosion) rubella, Lyme disease) significant exposure to loud noise can also cause irreversible SNHL.
With children’s use of personal listening devices, the prevalence of
Otitis media with Trauma (eg, physical or
this cause of hearing loss is increasing.
effusion acoustic)
Hearing loss caused by middle ear effusions is the most com-
Foreign body (including Radiation therapy for head mon cause of childhood hearing loss. It is often not discussed
cerumen) and neck tumors because it is usually considered benign and transient in nature.
Cholesteatoma Neurodegenerative or However, of all ears with resolved otitis media persistent fluid
Trauma (eg, ossicular demyelinating disorders is exhibited in 40% at 1 month, 20% at 2 months, and 10% at
disruption, tympanic (eg, Alport syndrome, Cogan 3 months after infection or after the conclusion of treatment. It is
membrane perforation) syndrome) important to identify whether this effusion is affecting the child’s
Adapted with permission from Gifford KA, Holmes MG, Bernstein HH. Hearing loss in children. hearing. Speech development is greatest in the first 3 years after
Pediatr Rev. 2009;30(6):207–216. birth and can be affected if a child has chronic effusion that is

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 Chapter 88: Hearing Impairments 639

causing hearing loss. Any concern for hearing loss warrants objec- parent or guardian whether that individual is at all suspicious
tive testing, and consultation with an otolaryngologist should be or concerned about the child’s speech or hearing. Guidelines for
recommended. assessing language development are found in Table 88.2 (also see
Chapter 33). It is also important to assess for risk factors for deaf-
ness, such as a positive family history, infection during gestation,
Differential Diagnosis
history of prematurity, hyperbilirubinemia, neonatal sepsis, and
In addition to hearing loss, communication disorders should be asphyxia (Box 88.3).
considered in the infant or child with delayed speech and language
development. These include problems with speech perception, lan-
Table 88.2. Expected Speech, Language,
guage comprehension, formulation of language output, and speech
and Auditory Milestones
production. Unrecognized conditions, such as intellectual disabil-
ity or autism spectrum disorder, are responsible for some of these Age Receptive Skills Expressive Skills
disorders. Other etiologies include specific central nervous sys- Birth Turns to source of sound Cries
tem deficits as well as impairments of fine motor control of the Shows preference for voices
oropharynx. Shows interest in faces
2–4 months Turns to source of sound Coos
Evaluation Shows preference for voices Takes turns cooing
Newborn Hearing Screening Shows interest in faces
The earlier the diagnosis of hearing loss is made, the sooner inter- 6 months Responds to name Coos
ventions can be initiated to help the child develop. In 1994, the Joint Takes turns cooing
Committee on Infant Hearing (JCIH), composed of representatives 9 months Understands verbal rou- Babbles
from several professional organizations, endorsed universal newborn tines (eg, “wave bye-bye”) Points
hearing screening. The goal was the early identification of hearing
loss in newborns and infants before age 3 months and the imple- Says mama, dada
mentation of intervention services by age 6 months. As a result of 12 months Follows a verbal command Uses jargon
these recommendations, states have implemented legislation man- Says first words
dating newborn hearing screening and intervention programs. A 15 months Points to body parts by name Learns words slowly
subsequent position statement was issued in 2000. The American
Academy of Pediatrics endorsed this statement and promoted 18–24 Understands sentences Learns words quickly
newborn hearing screening as well as periodic hearing assessment months Uses 2-word phrases
for every child. As a result, hearing screening has been established as 24–36 Answers questions Phrases 50% intelligible
an essential newborn evaluation; however, a significant need exists months Follows 2-step commands Builds ≥3-word sentences
to improve infrastructure to ensure that physicians receive and pro- Asks “what” questions
cess screening results. The JCIH policy statement was most recently
36–48 Understands much of what Asks “why” questions
updated in 2007 and includes more specific guidelines for diagnostic
months is said Sentences 75% intelligible
audiologic evaluation, medical evaluation, and surveillance screen-
ing in the medical home. Per the policy, all infants and children— Masters the early acquired
regardless of hearing screening results—should undergo ongoing speech sounds: m, b, y, n, w,
assessment of communication skills beginning at 2 months of age. d, p, and h
Any child with evidence of hearing loss in 1 ear or both ears should 48–60 Understands much of what Creates well-formed sentences
be offered early intervention. In 2013, a supplement was published months is said, commensurate with Tells stories
to the 2007 JCIH position statement describing principles and guide- cognitive level 100% intelligible
lines for early intervention after a child is diagnosed with hearing
6 years Understands much of what Pronounces most speech
impairment.
is said, commensurate with sounds correctly; may have
History cognitive level difficulty with sh, th (as in
Because the primary symptom of hearing impairment or deaf- think), s, z, th (as in the, l, r,
ness is failure to learn to speak at the appropriate age, the most and s in treasure)
important aspect of the history in the child with possible hear- 7 years Understands much of what Pronounces speech sounds
ing loss is determining whether speech is developing normally. is said, commensurate with correctly, including consonant
Even an infant with deafness may begin cooing and babbling in cognitive level blends, such as sp, tr, bl
infancy, and these early attempts at verbalization are not useful Adapted from Feldman HM. Evaluation and management of language and speech disorders in
milestones for assessment of hearing deficits. It helps to ask the preschool children. Pediatr Rev. 2005;26(4):131–142.

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 640 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Box 88.3. What to Ask Box 88.4. Tests for Evaluating Hearing
Hearing Impairment ww Automated auditory brainstem response: used for newborn hearing
ww Does the child seem to respond to sounds? screening
ww Does the child attempt to repeat sounds? ww Behavioral observation audiometry
ww How does the child indicate his, her, or their desires or needs? ww Brainstem auditory evoked response
ww How are the parent(s)/guardian(s) currently communicating with the ww Evoked otoacoustic emissions (a newer type of newborn hearing
child? screening)
ww Does evidence exist of a congenital infection, structural anomaly of the ww Conditioned play audiometry
head and neck, or syndrome? ww Conventional audiometric testing
ww Is there a history of prematurity or other prenatal or perinatal
problem?
ww Has the child had any serious bacterial infection, such as meningitis?
ww Does the child have a history of repeated ear infections or exposure to specific stimulus via a small probe that contains a microphone
ototoxic drugs? that is placed in the ear canal. This test can be used for newborn
ww Aside from the hearing problem, is the child developmentally normal? hearing screening in the low-risk newborn. It requires no sedation
ww Is there a family history of deafness, consanguinity, or multiple miscar- and is inexpensive and quick; it can be completed in 10 minutes.
riages or stillbirths? It has been implemented quite successfully as the hearing screen-
ing test in hospitals and primary care physician offices and has
the advantage of being useful for all ages. ABR is an electrophys-
iological measurement of activity in the auditory nerve and brain
Physical Examination stem pathways. Electrodes are placed on the head of the newborn,
A complete physical examination should be performed on all infant, or child to record brain wave activity while a specific audi-
children. In particular, any dysmorphic facial features that may tory stimulus is presented through earphones to 1 ear at a time.
be suggestive of a syndrome with an associated hearing deficit Auditory thresholds can be estimated. ABR is used as a screening
should be noted. Other anomalies of the head and neck should be tool for newborn hearing screening and only takes approximately
noted as well. Abnormal pigmentary conditions may be impor- 10 minutes. As a screening tool, it delivers a preset intensity and
tant clues. The eyes should be evaluated for heterochromia and frequency. If an infant does not pass the screening test, a full
hypertelorism findings seen in Waardenburg syndrome, which ABR should be done in which different intensities and frequencies
also includes SNHL. The size and shape of the pinnae and exter- are used to help identify the degree of hearing loss and at what
nal ear canals should be carefully inspected for abnormalities and frequencies. This evaluation takes approximately 90 minutes. Both
patency, respectively. Preauricular pits or tags may be apparent. otoacoustic emissions and ABR results can be affected by the pres-
Additionally, the TMs should be visualized and assessed for the ence of outer ear or middle ear disease. ABR requires a calm,
presence of middle ear effusion that may influence subsequent resting infant. The older infant may require sedation. Behavioral
audiologic tests. Insufflation may be helpful in the assessment of observation audiometry measures a child’s response to speech and
middle ear effusion. The oropharynx should be examined for a cleft frequency-specific stimuli presented through speakers in a sound-
palate or bifid uvula, which may be associated with a submucosal proof room. This method of testing assesses hearing in the bet-
cleft. The manner in which the child communicates with the parent ter ear only and cannot detect unilateral hearing loss. It is used
or guardian should be noted, if possible, and the child should be for the child with a developmental age of younger than 6 months.
assessed for response to sound. Conditioned play audiometry, like conventional audiometric
testing, measures auditory thresholds in response to frequency-
Laboratory Tests specific stimuli presented through earphones to 1 ear at a time.
Tympanometry does not measure hearing, but it is useful in The patient is instructed to perform a particular task, such as
assessing the presence of middle ear fluid and the mobility of the putting a block in a container or raising his or her hand, when
TM. Tympanometry can be particularly helpful with the unco- the stimulus is heard. The child as young as 3 years can be tested
operative, crying child in whom assessing the appearance of the by conditioned play audiometry, and the child age 4 or 5 years
TM and insufflation is difficult. Different types of hearing tests can be assessed using conventional audiometric testing. Any
for evaluating infants and children for possible hearing deficits abnormal results on these screening tests should be used in con-
are available to the primary care physician (Box 88.4). The 2 tests junction with evaluation by an audiologist and an otolaryngolo-
used for the screening of newborns are otoacoustic emissions and gist. In the case of the high-risk infant, audiologic testing should
auditory brainstem response (ABR), the latter of which is also be repeated at least every 6 months until 3 years of age and at
referred to as brainstem auditory evoked response. Otoacoustic appropriate intervals thereafter, depending on the etiology of the
emissions testing measures cochlear function in response to a suspected hearing loss and test results.

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 Chapter 88: Hearing Impairments 641

Diagnostic tests to consider in evaluating the cause of deaf- Additional assessment for the child with identified hearing loss
ness include titers for congenital infections, such as CMV, toxo- includes an ophthalmologic evaluation and referral to a geneti-
plasmosis, HIV, and rubella, and fluorescent treponemal antibody cist. After an infant or child is found to have a hearing impair-
absorption tests for syphilis. In the newborn period, a CMV culture ment, careful follow-up is necessary so that any further reduction
may be helpful. Testing for the GJB2 gene, the mitochondrial in hearing is promptly identified. The role of the primary care
A1555G mutation that predisposes an individual to ototoxicity physician thus becomes even more crucial. Coordination of care
from drugs, and the SLC26A4 gene for Pendred syndrome, along with speech and language specialists as well as educators who
with CMV testing, would reveal an etiology for 40% of cases of con- have experience working with children with deafness is essential.
genital hearing loss and 60% of cases of late-onset hearing loss. Parents or guardians and other family members may initially be
Thyroid function tests are necessary if Pendred syndrome is sus- devastated by the diagnosis of a hearing impairment, especially
pected, especially in the school-age child with goiter. Proteinuria if the deficit is severe to profound. These individuals often have
and hematuria should also be ruled out by urinalysis, especially multiple questions about the child’s medical prognosis and edu-
in boys with a positive family history of deafness and renal fail- cational future. The possibility of further speech and language
ure, both of which are findings suggestive of Alport syndrome. development may also be a foremost concern in their minds. In
Electrocardiography is recommended for the detection of con- addition to providing the patient with comprehensive care, all
duction defects, such as QT prolongation in Jervell and Lange- the questions and concerns of the parent or guardian must be
Nielsen syndrome. addressed and anticipated.

Imaging Studies Future Audiologic Evaluations

Occasionally, computed tomography or magnetic resonance imag- In the newly diagnosed patient with SNHL, audiology testing should
ing of the temporal bone may be obtained to view the anatomy of the be repeated every 3 months in the first year after birth, every 6 months
middle and inner ear, particularly in cases of a suspected cochlear or when the child is in preschool, and at least annually after the child
vestibular malformation or fistula. Necessity for such studies should has begun school. Continued monitoring is essential to detect any
be determined by an audiologist or otolaryngologist. progression of the hearing deficit.

Assistive Devices
Management Although hearing aids may not restore normal hearing, all chil-
According to the 2013 supplement to the JCIH 2007 statement, dren with CHL as well as SNHL benefit from amplification. Several
“Screening and confirmation that a child is deaf or hearing different types of hearing aids are available for children; these
impaired are largely meaningless without appropriate, individ- devices should be fitted appropriately and adjusted regularly by
ualized, targeted and high-quality intervention. For the infant a specialist. Additionally, it is also recommended that all patients
or young child who is deaf or hard of hearing to reach his or receive bilateral hearing aids to improve auditory localization
her full potential, carefully designed individualized intervention and training, particularly in the context of different learning
must be implemented promptly, utilizing service providers with situations.
optimal knowledge and skill levels and providing services on the A frequency modulation system is an additional assistive listening
basis of research, best practices, and proven models.” Studies have device that can be used in a classroom. A speaker (eg, the teacher)
shown that the earlier a hearing deficit is detected and remedia- uses a microphone to transmit to a receiver worn by the child to
tion begun in an otherwise normal newborn, the greater the like- improve reception. Closed-caption television, whether signed or
lihood the child will have language development close to that of subtitled, is another method of auditory training. Teletype telephone
a hearing child. Initiation of intervention before 6 months of age systems are available for children who can read.
contributes to infants being able to develop language as well as Cochlear implants may be surgically placed in the cochlea to
social and emotional skills appropriate for their age. School per- improve hearing. Implants were first approved by the US Food and
formance and communication skills have been shown to be bet- Drug Administration for use in children in 1990. The implant consists
ter in those identified at a younger age. It appears as though the of an electrode array placed in the cochlea with a receiver-stimulator
critical period for hearing and speech development is from birth under the skin and a processor worn over the ear that transmits by
to 3 years of age. All newborns, infants, and children identified radio waves an impulse that produces an electrical discharge within
with a hearing deficit should be referred to an otolaryngologist the cochlea. These electrical pulses effectively stimulate the auditory
and audiologist for immediate assessment and recommendation system. The implant can be used in children 12 months or older with
for assistive devices. severe to profound SNHL. Its use is now considered to be standard of
If a child is diagnosed with bilateral otitis media with effusion, care for patients with SNHL and is being used in infants as young as
recent guidelines indicate that a hearing test should be done if the 6 months. Speech, language, and special education resources should
effusion lasts for 3 months or longer. If a hearing loss is identified, be provided. Most patients with implants show significant improve-
tympanostomy tubes should be inserted. ment in communication skills. Children with cochlear implants are

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 642 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

at increased risk for meningitis; therefore, pneumococcal and rou- The earlier the intervention, the more likely children are to
tine H influenzae type b vaccines are recommended. succeed and maximize their potential. With appropriate treat-
ment, the child with hearing impairment should be able to lead a
Education and Communication normal life.
Much controversy exists concerning the optimal method of com-
munication for children with deafness. Oral communication (ie, lip
reading) and sign language each has advantages and disadvantages CASE RESOLUTION
depending on the child’s age, type of deafness, and whether the def- The child has a history that is classically positive for a hearing deficit. She does
icit is congenital or acquired. Whether the child already knows a not turn to loud noises, she has not developed any specific words, and she
indicates her needs nonverbally. Although obvious historical risk factors for
language is also important to consider. The preferred methods seem
hearing loss are lacking, behavioral audiography or brainstem auditory evoked
to vary from region to region; therefore, schools, other institutions, response should be performed by an audiologist. The physician’s suspicion should
and resource groups often use the most popular communication be discussed with the family, and a follow-up visit should be arranged to review
method in a particular area. Generally, some authors recommend hearing test results as soon as possible.
that children with minimal hearing loss may do better with lip read-
ing than those with greater hearing loss, who will most likely benefit
more from sign language. Early intervention and education pro-
grams can be home-based or in a group setting, but it is recom- Selected References
mended that educators be familiar with working with children with American Academy of Pediatrics Joint Committee on Infant Hearing. Year
hearing impairment. 2007 position statement: principles and guidelines for early hearing detection
Whether to mainstream the child with severe hearing loss in a and intervention programs. Pediatrics. 2007;120(4):898–921 PMID: 17908777
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guardian(s) should be encouraged to explore the possibilities of each preschool children. Pediatr Rev. 2005;26(4):131–142 PMID: 15805236 https://
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cator who is knowledgeable in this field can be helpful when mak- screen young children for hearing loss in primary care settings. Pediatrics.
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and children with hearing deficits are to minimize possible long- Nikolopoulos TP. Auditory dyssynchrony or auditory neuropathy: under-
term sequelae of persistent speech and language problems and standing the pathophysiology and exploring methods of treatment. Int J
maximize cognitive development. An additional goal is to pre- Pediatr Otorhinolaryngol. 2014;78(2):171–173 PMID: 24380663 https://doi.
vent learning disabilities with subsequent educational failure. org/10.1016/j.ijporl.2013.12.021

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Papsin BC, Gordon KA. Cochlear implants for children with severe-to-profound Smith RJ, Bale JF Jr, White KR. Sensorineural hearing loss in children.
hearing loss. N Engl J Med. 2007;357(23):2380–2387 PMID: 18057340 https:// Lancet. 2005;365(9462):879–890 PMID: 15752533 https://doi.org/10.1016/
doi.org/10.1056/NEJMct0706268 S0140-6736(05)71047-3
Rosenfeld RM, Schwartz SR, Pynnonen MA, et al. Clinical practice guideline: Weichbold V, Nekahm-Heis D, Welzl-Mueller K. Universal newborn hearing
tympanostomy tubes in children. Otolaryngol Head Neck Surg. 2013;149(1): screening and postnatal hearing loss. Pediatrics. 2006;117(4):e631–e636 PMID:
8–16 PMID: 23818543 https://doi.org/10.1177/0194599813490141 16585279 https://doi.org/10.1542/peds.2005-1455
Ross DS, Visser SN. Pediatric primary care physicians’ practices regarding new- Yoshinaga-Itano C, Sedey AL, Wiggin M, Chung W. Early hearing detection and
born hearing screening. J Prim Care Community Health. 2012;3(4):256–263 vocabulary of children with hearing loss. Pediatrics. 2017;140(2):e20162964
PMID: 23804171 https://doi.org/10.1177/2150131912440283 PMID: 28689189 https://doi.org/10.1542/peds.2016-2964
Russell JL, Pine HS, Young DL. Pediatric cochlear implantation: expanding
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 CHAPTER 89

Sore Throat
Casey Buitenhuys, MD, FACEP, and Stanley H. Inkelis, MD, FAAP

CASE STUDY
An 8-year-old girl has had a sore throat and fever for exudate bilaterally, palatal petechiae, and tender cervical
2 days. She also has pain on swallowing, a headache, lymphadenopathy.
and a feeling of general malaise but no stridor, drool-
ing, breathing difficulty, or rash. Other than the cur-
Questions
1. What are the causes of sore throat in children?
rent illness, the girl is in good health. Although she has
2. What is the appropriate evaluation of the child with
had sore throats in the past, she has never had one this
sore throat? What laboratory tests are necessary?
severe. One week previously, her mother and father had
3. What is the appropriate management for the child
sore throat and fever that resolved after 5 days with no
with sore throat?
medication.
4. When should otolaryngologic consultation be
The child has a temperature of 39.0°C (102.2°F). The
obtained?
physical examination is normal except for red tonsils with

Sore throat, which is among the most common illnesses seen by Clinical Presentation
the primary care physician, is a painful inflammation of the phar-
The clinical presentation of sore throat is variable and often depends
ynx, tonsils, or surrounding areas. In most cases, children with
on etiology (Box 89.1; also see Differential Diagnosis). Most chil-
sore throat have mild symptoms that require little or no treat-
dren with sore throat present with sudden onset of pain and fever.
ment. However, sore throat may be the presenting symptom of
The height of the fever is variable and is typically higher in younger
a severe illness, such as epiglottitis or retropharyngeal abscess.
children. In the older child, especially if the sore throat is associ-
Young children may not able to define their symptoms very well,
ated with a common cold, fever is minimal or absent. The throat
which makes a careful history from parents or other caregivers and a
or tonsils are red, and the breath may be malodorous. Headache,
good physical examination essential for correct diagnosis. Optimal
nausea, vomiting, and abdominal pain may occur, especially if the
management of sore throat, especially if group A b-hemolytic strep-
child is febrile. Appetite may be decreased, and the child may be
tococcus (GABHS; Streptococcus pyogenes) is suspected, remains
less active than usual.
quite controversial.
In the child with the common cold, rhinorrhea and postnasal dis-
charge are present. A pharyngeal and tonsillar exudate is not typical.
Epidemiology Although the cervical lymph nodes may be enlarged, they are usu-
In the United States, sore throat accounts for approximately 15 million ally not very tender. In contrast, the child with streptococcal phar-
outpatient physician visits each year, and approximately 5% of all yngitis typically has high fever, pharyngeal and tonsillar exudate,
pediatric emergency department visits are for pharyngitis. Sore and tender cervical lymph nodes.
throat is most common in children between 5 and 15 years of age. It
is uncommon in infants younger than 1 year. Like other respiratory
infections, sore throat occurs most often in the late fall and winter Pathophysiology
months. Approximately 11% of all school-age children receive med- Various bacteria and viruses produce sore throat symptoms by caus-
ical care for pharyngitis. Twenty percent to 30% or more of cases of ing inflammation in the ring of posterior pharyngeal lymphoid tis-
pharyngitis in these children are caused by GABHS. The estimated sue that consists of the tonsils, adenoids, and surrounding lymphoid
medical and nonmedical costs for GABHS pharyngitis are $205 per tissue. This ring of tissue, called Waldeyer tonsillar ring, drains the
visit or approximately $224 million to $539 million per year, with oral and pharyngeal cavity and defends against infection of the mouth
much of the indirect costs related to parental loss of time from work. and throat. Other host defenses that protect against infection include
The organisms that cause bacterial and viral pharyngitis are pres- the sneeze, gag, and cough reflexes; secretory immunoglobulin A;
ent in saliva and nasal secretions and are almost always transmitted and a rich blood supply.
by close contact. Spread between children in school is the common Viral sore throat may be acquired by inhalation or self-inoculation
mode of transmission. from the nasal mucosa or conjunctiva. The local respiratory

645

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 646 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Box 89.1. Diagnosis of Sore Throat Box 89.2. Causes of Sore Throat
Viral Etiology Viral Infections
ww Pain in throat ww Adenovirus
ww Fever (variable)a ww Coxsackievirus
ww Rhinorrhea (common) ww Echovirus (enteroviruses)
ww Cough (common) ww Common cold
ww Erythema of pharynx or tonsils ww Cytomegalovirus
ww Follicular, ulcerative, exudative lesions of pharynx or tonsilsa ww Enteroviral infections
ww Conjunctivitis ww Epstein-Barr virus
ww Non-scarlatiniform rash ww HIV seroconversion syndrome
ww Occipital or posterior cervical adenopathy ww Human herpesvirus
Bacterial Etiology ww Influenza virus
ww Pain in throat, usually sudden onset ww Mononucleosis
ww Fever ww Rhinovirus
ww Marked erythema of pharynx, tonsils, or uvula ww Respiratory syncytial virus
ww Headache, nausea, vomiting, abdominal pain Bacterial Infections
ww Tonsillar and posterior pharyngeal wall exudate ww Arcanobacterium haemolyticum
ww Tender, swollen cervical lymphadenopathy ww Chlamydophila pneumoniae
ww Scarlatiniform rash ww Chlamydia trachomatis
ww Absence of rhinorrhea or cough ww Corynebacterium diphtheriae (diphtheria)
ww Positive rapid antigen test or throat culture result ww Francisella tularensis (tularemia)
ww Distortion of natural anatomy ww Fusobacterium necrophorum
ww Group A b-hemolytic streptococcus
a
Dependent on etiology (see Differential Diagnosis).
ww Group B, C, and G b-hemolytic streptococci (non-GABHS)
ww Haemophilus influenzae type B
epithelium becomes infected with the virus, and inflammation ww Mycoplasma pneumoniae
occurs. In some instances, inflammatory mediators may be respon- ww Neisseria gonorrhoeae
sible for the pain of sore throat. Group A β-hemolytic streptococcus ww Staphylococcus aureus
and other bacterial organisms directly invade the mucous mem- ww Streptococcus pneumoniae
branes. Enzymes produced by this organism, streptolysin O and ww Treponema pallidum (syphilis)
hyaluronidase, aid in the spread of infection. Other Causes
ww Abscess (peritonsillar or retropharyngeal)
Differential Diagnosis ww Allergic rhinitis with postnasal drip
ww Burns
Although most children who present with sore throat have com-
ww Candida albicans
mon viral or bacterial pharyngitis, other, less common disorders
ww Caustic material
should be considered, such as infectious mononucleosis, acute HIV
ww Cigarette smoke (including secondhand smoke)
seroconversion syndrome, epiglottitis, retropharyngeal abscess, and
ww Croup
peritonsillar abscess. See Box 89.2 for a list of causes of sore throat.
ww Kawasaki disease
Viral Infection ww Marijuana smoke
Viral infection, the most common cause of sore throat in children, is ww Odontogenic infections
most often associated with an upper respiratory infection caused by ww Trauma
a rhinovirus. Cough and rhinorrhea associated with a sore throat are ww Tumors
suggestive of this etiology. Influenza virus infections may present with ww Vaping
sudden onset of high fever, headache, cough, sore throat, and myalgia.
Adenovirus often results in exudative pharyngitis, frequently in
children younger than 3 years. Pharyngoconjunctival fever, caused by apparent on the anterior tonsillar pillars and soft palate. They may
adenovirus 3, is characterized by a high fever (temperature >39.0°C also be found on the tonsils, pharynx, or posterior buccal mucosa.
[>102.2°F]) for several days, conjunctivitis, and exudative tonsillitis. The child may have a high fever (temperature >39.0°C [>102.2°F]),
Coxsackievirus and echovirus, both of which are enteroviruses, be irritable, and refuse to eat or drink; dehydration may result.
are the usual cause of herpangina. Vesicles and ulcers are generally Coxsackievirus A16, coxsackievirus A6, and enterovirus 71 cause

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 Chapter 89: Sore Throat 647

hand-foot-and-mouth disease, which is characterized by ulcerative potato” voice. The abscess in the affected tonsil causes a bulge in the
oral lesions on the tongue and buccal mucosa and, less frequently, posterior soft palate and pushes the uvula away from the midline to
on the palate and anterior tonsillar pillars. Vesicular and papulove- the unaffected side of the pharynx. On palpation, the abscess may
sicular lesions are evident on the hands and feet and occasionally feel fluctuant. Peritonsillar cellulitis typically produces a bulge in the
on other parts of the body, most commonly the knees and buttocks. soft palate but does not cause deviation of the uvula.
It usually occurs in children younger than 5 years but can occur Parapharyngeal and retropharyngeal abscesses that typically
in older children as well. A more severe form of hand-foot-and- occur in children younger than 6 years are additional life-threatening
mouth disease is associated with coxsackievirus A6, a virus new to complications of GABHS. Sore throat is associated with these
the United States in 2012. Enterovirus 71 is sometimes associated conditions, but dysphagia is usually more evident when the child
with severe central nervous system disease. Enteroviral infections swallows. The child with a retropharyngeal or parapharyngeal
typically occur in the late spring, summer, and early fall. abscess is toxic-appearing, also reports trismus, has a fever, has
Human herpesvirus may lead to pharyngotonsillitis but can be dysphonia, refuses to swallow, and drools. Additionally, the child
distinguished from most of the enteroviral infections because human may have meningismus and may be short of breath. A fluctuant
herpesvirus almost always involves the anterior portion of the mouth mass may be palpated deep to the tonsils. The patient may have
and lips and is associated with gingivitis (ie, herpetic gingivosto- pain when the trachea is manipulated in a lateral direction. The
matitis). The lesions often appear as whitish-yellow plaques with neck may be stiff, and the patient may resist passive neck move-
an erythematous base and are sometimes ulcerative. This illness is ments. Stridor may be present but usually is an ominous sign of
characterized by a high fever (temperature >39.0°C [>102.2°F]) for impending airway compromise.
up to 7 to 10 days and frequent refusal to eat or drink because of the Group B, C, and G b-hemolytic streptococci (non-GABHS) have
painful lesions. Dehydration may occur. all been isolated from children with pharyngitis. Streptococcus pneu-
Epstein-Barr virus (EBV) may cause exudative pharyngotonsil- moniae and Arcanobacterium haemolyticum infrequently cause
litis alone or as part of the infectious mononucleosis syndrome that pharyngitis in children. The latter organism is associated with a
includes fever, malaise, lymphadenopathy, palatal petechiae, and scarlatiniform rash in some patients and is most common in ado-
hepatosplenomegaly. Fatigue, malaise, eyelid edema, organomeg- lescents and young adults. In contrast with scarlet fever, palatal
aly, and a maculopapular rash without the other characteristics of a petechiae and strawberry tongue are not present with the pharyn-
scarlet fever rash help distinguish between infectious mononucleo- gitis caused by this bacterium. Although Corynebacterium diphthe-
sis and GABHS infection. riae (diphtheria) rarely causes sore throat in immunized children,
Cytomegalovirus may cause an infectious mononucleosis syn- this organism should be considered in nonimmunized children or
drome similar to EBV but is less commonly associated with phar- children from developing countries with exudative pharyngoton-
yngitis and splenomegaly. sillitis and a grayish pseudomembrane that bleeds when removal
HIV seroconversion syndrome may present with low-grade fever, is attempted.
myalgia, nonexudative pharyngitis, diffuse adenopathy, anorexia, Chlamydia trachomatis may result in pharyngitis and tonsilli-
and weight loss. Generally, onset of symptoms is approximately tis in adolescents and young adults through sexual transmission.
1 week after exposure but may not appear until 1 month after exposure. The role of Chlamydophila pneumoniae as a cause of sore throat
in children remains unclear. Mycoplasma pneumoniae does not
Bacterial Infection usually produce sore throat in children unless they have lower
Group A b-hemolytic streptococcus is the most common cause of respiratory tract disease. Neisseria gonorrhoeae may cause sore
bacterial sore throat in children older than 3 years. The pharynx is throat in sexually active adolescents. Its occurrence in prepuber-
typically very red and sometimes edematous, and the tonsils are tal children is often secondary to sexual abuse. The appearance of
red, enlarged, and covered with exudate. Occasionally, the uvula the throat is not characteristic, and diagnosis is made by cultures
is quite inflamed as well. The child may also have dysphagia, fever, when the degree of suspicion is high. Tularemia is a rare cause
vomiting, headache, malaise, and abdominal pain. Swollen anterior of exudative pharyngitis in children but should be suspected if
cervical lymphadenopathy and petechiae on the soft palate and uvula contact with wild animals has occurred.
are usually apparent. Additionally, the occurrence of a scarlatiniform Fusobacterium necrophorum is a gram-negative anaerobe that
rash, strawberry tongue, and Pastia lines (ie, petechiae in the flexor may cause an exudative pharyngitis, tender adenopathy, and fever.
skin creases of joints) is indicative of scarlet fever, which is diag- Untreated, it may progress to Lemierre syndrome or septic thrombo-
nostic of group A streptococcal infection (see Chapter 139). Sore sis of the internal jugular vein. Direct extension of the bacterial phar-
throat from GABHS typically occurs in the winter and early spring. yngitis leads to perivenular inflammation and septic thrombosis of
Rheumatic fever and glomerulonephritis are nonsuppurative com- the internal jugular vein. The patient may present with fever, severe
plications of group A streptococcal infection. lateral neck pain, torticollis, and prominent internal and external
Peritonsillar abscess or cellulitis and cervical lymphadenitis are jugular veins with erythema and induration. The patient may also
suppurative complications of GABHS. Children with peritonsillar present with additional signs and symptoms if septic emboli prop-
abscess often experience trismus and drooling and speak with a “hot agate, including acute neurologic signs (eg, central nervous system

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 648 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

retrograde propagation), and shortness of breath as a result of mul-

Box 89.3. What to Ask
tilobar pneumonia with or without cavitation (ie, pulmonary prop-
agation). Paradoxical septic emboli may cause other symptoms if a Sore Throat
right-to-left cardiac shunt is present. ww How long has the child had a sore throat?
ww Does the child have fever, headache, or vomiting?
Other Causes
ww How rapid was the onset of fever?
Candida albicans may be responsible for sore throat in the infant or ww Does the child have pain on swallowing?
child who is immunocompromised or taking antibiotics. The child ww Are there any voice changes?
with oral candidiasis usually presents with whitish plaques on the ww Does the child have a rash or oral lesions?
labial or buccal mucosa that do not wipe off easily. When the phar- ww Does the child have abdominal pain?
ynx and tonsils are involved, some discomfort or dysphagia, but usu- ww Does the child have any ill contacts?
ally not significant pain, may occur. ww Are the child’s immunizations current?
Epiglottitis (ie, supraglottitis) may present as sore throat. Prior to ww Is the child having any difficulty breathing?
the Haemophilus influenzae type B (Hib) conjugate vaccine, epiglot- ww Does the child have a history of allergies?
titis typically affected children 2 to 7 years of age who would pre­ ww Has the child suffered any trauma to the throat or neck?
sent with signs of toxicity, stridor, difficulty swallowing, and drooling. ww Has the child been exposed to environmental smoke?
In the relatively well-appearing child with sore throat but no stri- ww For the sexually active adolescent or child with a history of sexual abuse
dor, neither epiglottitis nor retropharyngeal abscess is a likely cause with nonresponding sore throat, has there been any oral sexual activity?
of sore throat. Historically, epiglottitis was almost always caused by Any other risk-taking behavior?
Hib. With the widespread use of the Hib conjugate vaccine, however,
this organism is now rarely the etiology, the prevalence of epiglotti-
tis is diminished, and epiglottitis is rarely the cause of sore throat in of contact with other family members or classmates with similar
children. Although rare in adolescents, epiglottitis may present with symptoms suggest the most common causes of sore throat (eg, infec-
severe sore throat out of proportion to clinical findings. Other signs tions with viruses and GABHS). A history of rapid onset of fever,
and symptoms include dysphagia, odynophagia, a muffled voice, toxicity, difficulty swallowing, drooling, and respiratory distress is
and pain on palpation of the anterior neck around the hyoid bone. suggestive of epiglottitis and retropharyngeal abscess. Voice changes
Streptococcus pneumoniae, Staphylococcus aureus, and group A, B, are suggestive of peritonsillar abscess or tonsillar hypertrophy asso-
and C b-hemolytic streptococci are unusual but reported causative ciated with infectious mononucleosis (ie, EBV). Immunization his-
agents of epiglottitis. tory or history of immigration from a developing country is helpful
The child with croup may have sore throat and stridor but does in assessing the risk of diphtheria. Oral sexual activity suggests the
not usually appear toxic and does not have difficulty swallowing. possibility of a sexually transmitted infection. A history of allergies,
Affected children are usually between 6 months and 3 years of age trauma, and environmental smoke may help diagnose other causes of
(see Chapter 71). sore throat. Red eye or pinkeye with a rash, persistent fever (>5 days),
Odontogenic infections may cause localized infection, inflam- and sore throat are suggestive of Kawasaki disease. A teenager with
mation, and swelling of the submental and submandibular space. at-risk behavior and an influenza-like illness may be presenting with
Significant infection of this space may present with Ludwig angina, HIV seroconversion syndrome.
which is characterized by difficulty with secretions, dyspnea, air-
way compromise, and elevated position of the tongue and “woody” Physical Examination
induration and tenderness of the sublingual space. A general physical examination should be performed. It is impor-
Trauma from penetrating objects, burns, or exposure to caustic tant to note whether the child appears toxic or if respiratory distress
materials may cause sore throat in children. Household cigarette is present. A child in the tripod position or with drooling should be
smoking, marijuana smoking, and vaping may also result in pha- kept in a position of comfort, taking care not to agitate or further
ryngeal irritation. Additionally, allergic rhinitis with postnasal drip distress the child. The child with stridor at rest should be examined
may result in sore throat. Tumor rarely causes sore throat in chil- in a position of comfort. Further agitation, distress, or attempts at
dren but should be considered if a mass is present or pharyngeal examination of the oropharynx in these patients can precipitate air-
inflammation persists. Persistent sore throat may also be a symp- way obstruction. The skin should be examined for a scarlatiniform,
tom of Kawasaki disease. sandpaper-like rash; a vesicular rash involving the hands and feet;
or a generalized maculopapular rash. The eyes should be evaluated
Evaluation
for conjunctivitis and the nose for rhinorrhea (serous or purulent).
History The mouth, pharynx, and tonsils should be examined for vesicu-
A thorough history often reveals the etiology of the sore throat lar lesions, ulcers, and gingivitis. The pharynx should be checked
(Box 89.3). Questions about duration, fever, headache, vomiting, for redness, exudate, vesicles, edema, and foreign bodies. The tonsils
pain on swallowing, rash, oral lesions, abdominal pain, and history and uvula should be examined for these same findings as well as

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 Chapter 89: Sore Throat 649

asymmetry, and the neck should be checked for nuchal rigidity. The sudden onset of sore throat, fever, headache, swollen and erythem-
lymph nodes should be evaluated for enlargement (adenopathy) atous tonsils, tonsillar or posterior pharyngeal wall exudate, uvuli-
and tenderness (adenitis). The abdomen should be examined for tis, tender and enlarged cervical lymphadenopathy, absence of runny
hepatosplenomegaly. nose or cough, or exposure to an individual with streptococcal phar-
yngitis, may warrant further testing with RADT or throat culture to
Laboratory Tests confirm group A streptococcal pharyngitis because an accurate
Although many signs and symptoms may be suggestive of strepto- diagnosis cannot be made on clinical grounds alone. Approximately
coccal pharyngitis, diagnosis can be confirmed only with laboratory 20% of all children are asymptomatic carriers of GABHS in the phar-
tests. The throat culture is the standard for diagnosis. When done ynx, especially during winter and spring. If tested, these children
correctly, throat culture has a sensitivity of 90% to 95% in detecting will be positive for GABHS even though they may have a viral ill-
pharyngeal GABHS. Specimens should be obtained from the sur- ness and will be unnecessarily treated with antibiotics. Guidelines
faces of both tonsils and posterior pharynx without touching other for clinical prediction have been evaluated and have suggested
parts of the pharynx or mouth. The main disadvantage of throat different approaches to the need for RADT or throat culture and anti-
culture is that the results are not available for a day or more after biotic management. The Centor criteria were derived and validated
the specimen is obtained. Nevertheless, throat culture is the most in adult patients and overestimate the likelihood of GABHS pharyn-
reliable means of confirming streptococcal infection. gitis in children. A modified McIsaac score is more predictive than
Rapid antigen detection tests (RADTs) are available for on-the-spot the Centor criteria in children but is not sufficiently sensitive or spe-
diagnosis. False-positive results are uncommon (specificity ≥ 95%), cific enough to rely on alone. In a meta-analysis of signs and symp-
but false-negative results for most RADTs occur commonly (sensi- toms predicting GABHS, presence of a tonsillar exudate, pharyngeal
tivity 80%–90%). Because a negative test may not exclude a strep- exudate, or exposure to strep infection in the previous 2 weeks (posi-
tococcal infection, guidelines recommend that a negative result be tive likelihood ratios of 3.4, 2.1, and 1.9, respectively) and the absence
confirmed by throat culture. Because RADTs are highly specific, it is of tender anterior cervical nodes, tonsillar enlargement, or exudate
not necessary to confirm a positive test result with a throat culture. (negative likelihood ratios of 0.6, 0.63, and 0.74, respectively), were
Newer RADTs using optical immunoassay (OIA) and chemilu- most predictive. Guidelines that recommend identifying patients
minescent DNA probes boast sensitivities of greater than 99%. A who are likely to have group A streptococcal pharyngitis based on
recent Cochrane review, however, demonstrates a pooled sensitiv- clinical or epidemiologic findings and providing antibiotics for only
ity of RADTs of 85% with a specificity of 95%. A throat culture in those confirmed by RADT or throat culture decrease the unneces-
the setting of a negative RADT is still beneficial given the sensitivity. sary overuse of antibiotics.
Rapid and sensitive OIA RADTs reduce antibiotic prescription Viral throat cultures and acute and convalescent titers to deter-
rates by 50% in pediatric emergency care visits related to sore throat. mine viral pharyngitis are rarely indicated unless systemic infection
An antistreptolysin-O titer and an anti-deoxyribonuclease-B titer occurs (eg, herpes encephalitis). Epstein-Barr virus infection can be
are not useful for the acute diagnosis of GABHS infection because determined by specific serologic antibody assays, but nonspecific
these titers do not increase until 1 to 2 weeks after the onset of phar- tests for heterophile antibody (eg, mononucleosis spot [ie, mono-
yngitis and peak at 3 to 4 weeks. However, measurement of these spot] test) are most available and are usually the tests of choice for
titers may help confirm a prior streptococcal infection if the throat diagnosing infectious mononucleosis. However, it may be negative in
culture is negative, particularly in the child for whom exists a high children younger than 4 years or early in the course of the infection.
index of suspicion for acute rheumatic fever or acute poststreptococ- Only 75% of infected children between 2 and 4 years of age and less
cal glomerulonephritis. Diagnostic studies are typically not neces- than 30% of children younger than 2 years are identified by this test.
sary for children younger than 3 years because GABHS pharyngitis The monospot test, a rapid slide test for heterophil antibodies, may
is uncommon and the risk of developing acute rheumatic fever and remain positive for months after the infection and incorrectly may
suppurative complications is low. The role of antibiotic prophylaxis suggest the diagnosis of infectious mononucleosis in the child who
for household contact of patients with acute GABHS is not recom- no longer has this disorder. A complete blood cell count with more
mended. Symptomatic contact should be evaluated with an RADT than 50% to 60% lymphocytes or more than 10% atypical lympho-
or throat culture. cytes is suggestive of mononucleosis. When these tests are incon-
Differentiating between viral and bacterial pharyngitis is often clusive, the specific serologic antibody tests for EBV infection are
difficult, and rapid streptococcal antigen tests and throat cultures helpful in establishing the diagnosis. Cytomegalovirus-specific anti-
should be reserved for the patient with signs and symptoms com- body tests should be considered in the patient with a mononucleosis
mon for both illnesses. Some children have clinical findings that are syndrome and negative laboratory test results for EBV. Culture or flu-
not consistent with bacterial pharyngitis. For example, the afebrile orescent antibody evaluation of the pseudomembrane may be used
child with a sore throat, runny nose, and cough who has slight pha- to diagnose diphtheria. Culture or presence of serum agglutinins
ryngeal erythema almost certainly has viral pharyngitis and does confirms tularemia. Thayer-Martin culture plates should be used
not require further workup. However, the child with a constellation to diagnose suspected gonorrheal sore throat. HIV antibody tests
of signs and symptoms suggestive of bacterial pharyngitis, such as are of little use in the evaluation of acute seroconversion syndrome

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 650 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

because antibody titers take 4 to 6 weeks to become detectable. If used cautiously because it can suppress the gag reflex. The dose of
acute seroconversion is suspected, quantitative RNA polymerase lidocaine should never exceed 3 mg/kg. Excess lidocaine may result
chain reaction should be ordered for HIV. in seizures or arrhythmias. Magic mouthwash can also be prepared
without lidocaine, especially for the younger child. The parent or
Imaging Studies
guardian should be given instruction on how to monitor fluid intake
If epiglottitis or retropharyngeal abscess is suspected but not and the signs of dehydration.
clinically apparent, a lateral neck radiograph may be obtained. In the child without clear-cut evidence of streptococcal pharyn-
Radiography should be performed with a physician in attendance gitis, a positive rapid streptococcal antigen test helps direct antibi-
who is capable of performing endotracheal intubation in case the otic treatment. A negative test in the presence of positive symptoms
child has respiratory failure (see Chapter 71). Computed tomog- should be accompanied by a throat culture. The OIA rapid test may
raphy of the neck is indicated in the stable patient with suspected preclude the need for culture confirmation, however. The patient
deep parapharyngeal or retropharyngeal infection. Emergency bed- can await the results of culture before beginning antibiotic therapy.
side ultrasonography is an effective and sensitive tool for differen- Antibiotics without confirmation from rapid streptococcal tests or
tiating between peritonsillar cellulitis and a peritonsillar abscess. cultures are indicated in the child who appears toxic, who has scar-
let fever or peritonsillar cellulitis/abscess, or who has a history of
Management rheumatic fever. Most evidence suggests that early treatment results
Management of sore throat in children is based on the etiology in more rapid clinical improvement, although this is controversial.
of the condition. The early recognition of potentially serious con- Rheumatic fever can be prevented if treatment is started within
ditions based on history and physical examination is essential to 9 days of sore throat symptom development. Glomerulonephritis
providing optimal care. Otolaryngologic consultation should be likely is not affected by antibiotic therapy.
obtained for the child with peritonsillar abscess, retropharyngeal Evidence suggests that the child with streptococcal pharyngitis
abscess, parapharyngeal abscess, submental abscess, epiglottitis, should be treated with antibiotics to relieve symptoms, shorten the
significant pharyngeal trauma, or pharyngeal tumor. Recurrent course of the illness, and prevent disease dissemination, suppura-
tonsillitis, especially in the child who misses school, may be a reason tive complications, and rheumatic fever. Penicillin is the antibiotic
for referral to an otolaryngologist. of choice. It may be administered orally as penicillin V (ie, phenoxy-
methyl penicillin) in a dose of 250 mg 2 to 3 times a day for 10 days
Outpatient Treatment for children (<27 kg [<60 lb]) and 500 mg 2 to 3 times a day for older
For most children with sore throat, the physician must differentiate children, adolescents, and adults. Most patients will feel better after 2
between viral and streptococcal pharyngitis. Viral sore throat can be to 3 days, but it is important to stress to the parent(s)/guardian(s)
managed symptomatically. Treatment with analgesics, such as acet- that the children must complete the full 10-day course. Amoxicillin,
aminophen or ibuprofen to relieve pain, and to promote hydration given once a day orally (50 mg/kg; maximum, 1,000 mg) for 10 days
are the mainstays of therapy for the young child with viral or bac- is as effective as penicillin V given 2 to 3 times a day for 10 days, mak-
terial sore throat. Gargling with warm water and sucking on hard ing compliance more likely. Additionally, amoxicillin is more accept-
candy may provide additional symptomatic relief for the older child. able to young children because the oral suspension is better tasting.
The use of steroids for reducing pain for pharyngitis is controversial. No significant difference in treatment success exists between anti-
One systematic review demonstrated a relatively small reduction in biotics. (See Table 89.1 for dosages.)
time to significant pain relief of 4.5 hours and a negligible reduction If the risk of noncompliance is high or if the risk of complica-
in pain in 24 hours when a single dose of dexamethasone (0.6 mg/kg, tion is great (eg, child with a history of rheumatic fever), penicillin
maximum 10 mg) is given. However, an additional study in adult should be administered intramuscularly. Intramuscular penicillin
patients found a significant reduction in pain at 48 hours after treat- has 2 disadvantages: pain associated with the injection and increased
ment. The decision to administer steroids should be individualized incidence of a potentially more severe allergic reaction. The dose of
to the patient. benzathine penicillin for the child weighing less than 27 kg (<60 lb)
The pain from lesions of herpetic stomatitis often responds to is 600,000 U. The dose for larger children and adults is 1.2 million
acetaminophen or ibuprofen. Anesthetics, such as lidocaine, may also U. Bicillin C-R, which contains 900,000 U of benzathine penicillin
decrease the pain. A convenient means of delivering lidocaine is in a and 300,000 U of procaine penicillin, is a satisfactory alternative
mixture (1 part each) of lidocaine, diphenhydramine (eg, Benadryl), form of delivering penicillin intramuscularly in children and may be
and a liquid antacid. This mixture, called “magic mouthwash,” may preferable because it causes less pain and less severe local reaction.
be used in the older child. However, little evidence exists supporting This preparation has not been determined to be effective in heavier
a benefit. If used, it may be inserted into each side of the mouth, gar- patients (ie, adolescents and adults); therefore, the benzathine prepa-
gled, or placed on a gloved finger or cotton swab and applied directly ration noted previously is recommended for this group. The injection
on the oral lesions of the tongue and labial and buccal mucosa. It of benzathine penicillin is less painful if it is given after it reaches
is best used approximately 30 minutes before feeding or drinking, room temperature (Table 89.1). A first-generation oral cephalospo-
especially in the child who refuses to drink. Lidocaine should be rin, such as cephalexin, is recommended for most children with

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 Chapter 89: Sore Throat 651

Table 89.1. Antibiotics Used in the Management of Sore Throat in Children

Cause of Sore Throat Drug Dosage
Streptococcal pharyngitis Penicillin V 250 mg 2–3 times per day for children (<27 kg [<60 lb]) and 500 mg 2–3 times per day for
adolescents orally for 10 days
Amoxicillin 50 mg/kg once daily orally for 10 days (maximum 1,000 mg)
Cephalexin 25–50 mg/kg per day every 12 hours orally for 10 days (maximum 500 mg every 12 hours)
Clindamycin 30 mg/kg per day in 3 divided doses (maximum 900 mg per day) orally for 10 days (for chronic
streptococcal carriers or suspected Fusobacterium necrophorum)
Azithromycin 12 mg/kg once daily orally for 5 days (maximum 500 mg)
Clarithromycin 15 mg/kg per day every 12 hours orally for 10 days
Benzathine penicillin 600,000 U (<27 kg [<60 lb]); 1.2 million U (≥27 kg [≥60 lb]) as single intramuscular
injection
Bicillin C-R 900,000 U benzathine penicillin and 300,000 U procaine penicillin for children as single
intramuscular injection
Rifampin 10 mg/kg per dose orally every 12 hours for 4 days (given last 4 days of treatment with
penicillin V or benzathine penicillin for chronic streptococcal carriers)
Gonococcal pharyngitis Ceftriaxone 125 mg intramuscularly in a single dose if <45 kg (<99 lb), 250 mg intramuscularly in a single
dose if ≥45 kg (≥99 lb)
plus
Azithromycin 20 mg/kg (maximum 1 g) orally in a single dose
or
Erythromycin 50 mg/kg per day (maximum 2 g per day) orally every 6 hours for 14 days
or (if ≥ 9 years of age)
Doxycycline 100 mg orally 2 times per day for 7 days

penicillin allergy but should not be used in the child with an after treatment are generally not recommended, except for the child
immediate or type 1 hypersensitivity to penicillin. Clindamycin with recurring or persistent symptoms or with a previous history of
may also be used for the patient with penicillin allergy; how- rheumatic fever. In selected cases of children whose throat cultures
ever, the liquid preparation is not palatable and compliance remain positive, eradication of the pharyngeal carriage should be
may be poor. Azalides and macrolides, such as azithromycin or strongly considered. These indications are as follows: an outbreak
clarithromycin, may be substituted in the child with penicillin of acute rheumatic fever or poststreptococcal glomerulonephritis,
allergy. These agents are preferred to erythromycin because an outbreak of group A streptococcal pharyngitis in a closed or semi-
they are associated with fewer gastrointestinal side effects. closed community, a family history of rheumatic fever, or repeated
Azithromycin has the added advantage of once daily dosing episodes of documented symptomatic group A streptococcal phar-
and a shortened course of therapy of only 5 days. Additionally, yngitis within a family over several weeks despite appropriate ther-
shorter-duration alternatives to penicillin are superior in time apy. If cultures remain positive, these children may be treated with
to symptom improvement as well as duration of fever. Macrolide benzathine penicillin and oral rifampin for 4 days in an attempt
resistance, however, is 5% to 8% in most areas of the United to eradicate the organism (Table 89.1). Clindamycin is reportedly
States. Tetracyclines; sulfonamides, including trimethoprim- more effective in eradication of the organism from symptom-free
sulfamethoxazole; and fluoroquinolones are not recommended carriers (Table 89.1).
for the management of streptococcal pharyngitis. Mycoplasma pneumoniae pharyngitis usually is associated with a
If symptoms persist and the child has a persistently positive generalized infection. Because it is often a self-limited illness, anti-
throat culture after completing a course of therapy, the child may biotic therapy is unnecessary unless symptoms persist. Treatment
be re-treated with the same antibiotic, given another oral antibiotic with any of the macrolide antibiotics may be helpful. However, cla­
(as noted previously and in Table 89.1), or given an intramuscular rithromycin and azithromycin have fewer side effects and are likely
dose of penicillin, especially if compliance is in question. to produce better compliance. Macrolides are also the drugs of choice
As noted previously, approximately 20% of children are asymp- for children with A haemolyticum pharyngitis.
tomatic carriers of group A streptococci. Typically, these children Diphtheria, which occurs almost exclusively in developing coun-
do well, and eradication of the bacteria is not necessary. Cultures tries, is a life-threatening infection that requires prompt diagnosis

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 652 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

and treatment. Penicillin G or erythromycin must be given to kill from tonsillectomy. These guidelines, containing content based on
C diphtheriae; additionally, equine antitoxin must be administered previous work done by Paradise et al about children with recur-
to neutralize the exotoxin. Tularemia pharyngitis is unusual, but if rent sore throat, have been recently published. Most children with
suspected, it is treated with gentamicin. sore throat improve on their own, and recommendations are there-
For gonococcal pharyngitis caused by N gonorrhoeae, intra- fore for watchful waiting in the setting of fewer than 7 documented
muscular ceftriaxone is the drug of choice (Table 89.1). Additional sore throat episodes in the past year, fewer than 5 per year over
antibiotic coverage for associated C trachomatis infection should the past 2 years, and fewer than 3 per year over the past 3 years.
be administered. Oral azithromycin or doxycycline should also Parental or guardian report does not qualify as documentation. If the
be given to children 9 years or older (Table 89.1). Azithromycin number of documented sore throats meets or exceeds these numbers
or erythromycin may be used for the younger child. The child and associated findings exist (eg, temperature >38.3°C [>101°F],
should be examined and cultured for sexually transmitted infec- cervical lymphadenopathy, tonsillar exudate, positive test for
tions in other sites and should undergo a serologic testing for GABHS), the physician may recommend tonsillectomy. Consultation
syphilis at the first visit as well as a repeat test 6 to 8 weeks later. with an otolaryngologist and a period of watchful waiting should be
The child should also be evaluated for concurrent hepatitis B and considered. If a child with recurrent sore throat does not meet these
HIV infection. Sexual abuse should be considered in all cases of criteria, the child should be assessed for other factors that may favor
gonococcal pharyngitis, particularly in the prepubertal child (see tonsillectomy over observation, including, but not limited to, mul-
Chapters 60 and 145). tiple antibiotic allergy or intolerance, PFAPA syndrome (periodic
Children with croup usually respond to steroids. Oral nystatin fever, aphthous stomatitis, pharyngitis, and adenitis), or a history of
can be used in the child with oral candidiasis. The adolescent with peritonsillar abscess. Although these guidelines are evidence-based
uncomplicated peritonsillar abscess may be treated on an outpatient and the recommendations are better defined than before, each case
basis in selected cases with needle aspiration and oral antibiotics. should be individualized. As with all clinical decisions, a role exists
for shared decision-making with the child’s caregiver and primary
Inpatient Treatment care physician about the need for tonsillectomy.
The child with sore throat should be admitted to the hospital in
the setting of airway obstruction or a need for intravenous (IV) Education
hydration or antibiotics. The child with retropharyngeal abscess
Patients and families should receive general education about sore
and epiglottitis requires IV antibiotics and should be managed in
throat. Medication for pain with drugs such as acetaminophen
consultation with an otolaryngologist. The preadolescent child or
or ibuprofen is useful, especially if the child is having difficulty
adolescent with complicated peritonsillar abscess also requires IV
swallowing. Gargling with warm salt water or sucking on hard
antibiotics. Surgical intervention is indicated if the abscess is fluc-
candy may soothe the pain of sore throat. For the child with her-
tuant, the child is toxic or has severe trismus or airway compro-
petic gingivostomatitis, avoidance of acidic or spicy food products
mise, or no resolution occurs within 24 hours. Needle aspiration
may prevent pain during eating. The child with bacterial phar-
may be acceptable in selected cases but this is associated with an
yngitis may return to school after 24 hours of antibiotic therapy
increased rate of treatment failure. Intravenous hydration is occa-
and the disappearance of fever. Children are likely noninfectious
sionally needed for patients with severe herpetic stomatitis who will
12 hours after a single dose of amoxicillin. It should be recom-
not drink because of pain and who become dehydrated.
mended that symptomatic family members see a physician. The
The patient with Lemierre syndrome should be admitted and
parent(s)/guardian(s) should call or return to the physician if the
started on IV antibiotics covering a polymicrobial infection, includ-
child has respiratory or swallowing difficulties, drooling, severe
ing anaerobic organisms. If F necrophorum pharyngitis is suspected,
pain, or fever (temperature >38.3°C [>101°F]) for more than
antibiotics covering anaerobes are indicated.
48 hours after the initiation of appropriate antibiotics.
Suspected deep space infection of the pharynx other than uncom-
plicated peritonsillar abscess requires inpatient admission and
treatment. Retropharyngeal and parapharyngeal abscesses usually Prognosis
require incision and drainage during or after antibiotic initiation. The prognosis for the child with viral sore throat is excellent because
Ludwig angina requires IV antibiotics for an uncomplicated cellu- of its self-limited nature. The outlook for the child with streptococ-
litis or an incision and drainage procedure in the operating room. cal sore throat is also excellent. If the infection is not diagnosed
Meticulous monitoring of the patient’s airway is required if it is not and managed appropriately, however, suppurative (eg, periton-
secured preoperatively. sillar abscess) and nonsuppurative complications (eg, rheumatic
The role of tonsillectomy or adenotonsillectomy for the child with fever, acute glomerulonephritis) may occur. With early diagnosis
recurrent sore throat remains controversial. However, the American and prompt treatment, the prognosis for unusual, life-threatening
Academy of Otolaryngology-Head and Neck Surgery convened a causes of sore throat is also very good. The cross-immunogenicity
panel of clinicians from various disciplines to develop evidence- of GABHS presents a unique and challenging case for vaccine
based guidelines to identify children who are most likely to benefit development.

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 Chapter 89: Sore Throat 653

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Lippincott Williams & Wilkins; 2016:481–485
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 CHAPTER 90

Nosebleeds
Anna K. Schlechter, MD, Katherine E. Remick, MD, FACEP, FAEMS, FAAP; and
Stanley H. Inkelis, MD, FAAP

CASE STUDY
A 3-year-old boy is brought to the office on a winter day. Questions
He has had 4 nosebleeds in the past week as well as a 1. What are the common causes of nosebleeds in
cold with rhinorrhea and cough, which began the day children?
before the first nosebleed. The nosebleeds occur at night 2. What systemic diseases are associated with
or during sleep and stop spontaneously or with gentle nosebleeds?
pressure. Other than the cold and nosebleeds, the boy is 3. How should nosebleeds be evaluated in children?
in good health. He is active, with bruises over both tibias 4. How should minor and severe nosebleeds be man-
but none elsewhere. The many cuts and scrapes he has aged in children?
had in the past resulted in minimal bleeding. His fam-
ily has no history of a bleeding disorder or easy bruising.
The child’s physical examination is entirely normal
except for a small amount of blood in the left anterior naris.

Nosebleed, or epistaxis, occurs commonly in children, especially

Box 90.1. Diagnosis of Epistaxis
in those between 2 and 10 years of age. In most cases, nosebleeds
are secondary to local trauma and can be managed by primary care ww Blood in anterior nares, nasopharynx, or mouth
physicians. In rare instances, however, a nosebleed may be difficult ww History of any of the following:
to control or may be a manifestation of a serious systemic illness. — Frequent digital manipulation (ie, nose picking)
Referral to an otolaryngologist or a hematologist/oncologist is usu- — Upper respiratory infection (recent)
ally not required except in these situations, and hospitalization is — Allergic rhinitis
generally unnecessary. Parents and children often are frightened by — Dry climate
nosebleeds and frequently overestimate the amount of blood lost. — Foreign body in nose
Providing reassurance and a basic understanding of the most com- — Trauma to nose
mon causes are important in allaying anxiety. — Prolonged or difficult-to-stop bleeding, or easy bruising
ww Physical examination consistent with any of the following:
Epidemiology — Rhinorrhea
— Dry, cracked nasal mucosa
Thirty percent of children experience 1 nosebleed by 5 years of
— Foreign body in nose
age. In children between the ages of 6 and 10 years, the frequency
— Trauma to nose
increases to 56%. Nosebleeds are rare in infancy, with an estimated
— Multiple bruises
1 nosebleed per 10,000 patients younger than 2 years. Nosebleeds
are infrequent after puberty. They occur much more frequently in
the late fall and winter months, when upper respiratory infections prolonged bleeding, easy bruising, or multiple bruises in uncommon
(URIs) are common, environmental humidity is relatively low, and locations such as the gingivae or joints. Less commonly, nosebleeds
the use of heating systems may result in dryness. Nosebleeds are are among the first manifestations of an undiagnosed malignancy
also more common in children who live in dry climates, especially or other systemic illness. In unusual situations, children with gastro-
in the presence of a concomitant URI or chronic allergic rhinitis. intestinal or respiratory tract bleeding may present with blood exit-
ing through the nose. Alternatively, some children with nosebleeds
Clinical Presentation may present with hematemesis, hemoptysis, melena, or anemia. In
Most children with nosebleeds have a history of bleeding at these cases, a nasal source should be considered.
home and have minimal or no bleeding at the time of presentation Nosebleeds are generally categorized by anatomic location as
(Box 90.1). However, children with underlying clotting disorders anterior or posterior. Anterior epistaxis is most common, comprising
(eg, hemophilia) may have recurrent nosebleeds and a history of 90% of pediatric nosebleeds, and is almost always self-limited. With
655

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 656 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

anterior nosebleeds, vessels from the anterior portion of the nose Posterior nosebleeds are unusual in children and are most com-
rupture, resulting in readily visible blood loss through the nares. monly associated with trauma. If bleeding is vigorous or poorly
With posterior nosebleeds, most of the blood runs into the naso- controlled with anterior nasal packing or involves both nares, a
pharynx and mouth, although some blood may exit through the posterior source is likely to be responsible. Posterior nosebleeds
nose as well. Posterior nosebleeds, though uncommon in children, generally arise from the turbinate or nasal wall. Significant bleed-
tend to be heavier and more difficult to control, and children have ing, usually from a branch of the sphenopalatine artery, may occur.
a propensity to become hemodynamically unstable. Because of the posterior location, children often present with symp-
toms other than frank epistaxis (eg, hematemesis, hemoptysis,
Pathophysiology melena, anemia).
More than 90% of nosebleeds in children are anterior and easily
controlled. Anterior bleeding originates approximately 0.5 cm from Differential Diagnosis
the tip of the nose, known as the Kiesselbach area. This area is Trauma from nose picking and inflammation of the nasal mucosa
a confluence of small vessels supplied by the anterior and posterior from a URI are by far the most common causes of nosebleed in chil-
ethmoidalarteries, the sphenopalatine artery, and the septal branches dren. Repetitive, habitual nose picking (ie, epistaxis digitorum) results
of the superior labial artery (Figure 90.1). The mucosa covering in the formation of friable granulation tissue that bleeds when con-
the Kiesselbach area is thin and friable, providing little structural gested blood vessels are traumatized. As the nasal mucosa dries, crust
support to the small vessels supplying the nasal mucous membrane. formation and cracking may occur. Bleeding may occur spontane-
The congestion of vessels located in the Kiesselbach area as the ously or from nose rubbing, but more often it results from forceful
result of URIs or drying of the mucosa secondary to low environmen- nose blowing and sneezing, which increase venous pressure in the
tal humidity makes this area susceptible to bleeding. Viral respira- more vascularized nasal septum.
tory infections, such as infectious mononucleosis and influenza, may Foreign bodies may cause direct trauma or pressure necrosis
predispose children to nosebleeds because of the local inflammatory to the vessels of the nasal mucosa. Toddler-age children may place
effect of such infections. Nosebleeds in children with these infections small toys, beads, pebbles, or food items into the nares. Button bat-
are more common in areas of low environmental humidity. Even in teries are particularly troublesome and should be removed immedi-
the absence of URI-like symptoms, however, children may experi- ately to avoid septal perforation. Children with unilateral epistaxis
ence nosebleeds in such environments, especially in the winter, when with purulent or foul-smelling nasal drainage should be evaluated
inhaling dry, hot air from heating systems causes desiccation of the for a foreign body. External trauma secondary to falls or blunt force
nasal mucosa (ie, rhinitis sicca). Nosebleeds also occur more com- can cause tears to the nasal mucosa or nasal fractures. If bleeding
monly in children who have nasal colonization with Staphylococcus from mucosal vessels occurs but the mucosa remains intact, a septal
aureus. It is postulated that S aureus replaces existing flora and results hematoma may occur. Thus, it is important to carefully examine the
in inflammation and new vessel formation. nasal septum. Abscess formation or septal perforation may occur if
the septal hematoma is not drained. Non-accidental trauma, specif-
ically asphyxiation, should be considered in any child younger than
Anterior and posterior 2 years with a nosebleed.
ethmoid arteries Allergic rhinitis with inflammation and subsequent drying also
Sphenopalatine may result in nosebleeds. Airborne environmental pollutants have
Kiesselbach artery been associated with increased inflammation of the nasal mucosa.
area
Children with allergic rhinitis who take decongestants or use topi-
cal nasal decongestants or topical nasal steroid sprays may have an
increased likelihood of experiencing nosebleeds. In addition, the
dispenser tip of these sprays may traumatize the already dry and
friable mucosa, causing the nose to bleed.
Although nosebleeds are usually benign conditions, they may
be among the first signs of serious illness. Persistent or recur-
rent nosebleeds with no obvious cause should raise the suspi-
Labial cion of bleeding disorders (see Chapter 99). Thrombocytopenia
artery is the most common coagulation defect that results in nosebleeds.
Idiopathic thrombocytopenic purpura is the thrombocytopenic
disorder most frequently associated with nosebleeds. Leukemia,
aplastic anemia, and HIV infection also should be considered and
ruled out in children with nosebleeds and thrombocytopenia.
Figure 90.1. Vascular supply of the nasal septum. Note the confluence of Platelet aggregation disorders also may be a cause of recurrent
vessels that forms the Kiesselbach plexus. nosebleeds.

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 Chapter 90: Nosebleeds 657

The most commonly inherited bleeding disorder associated

Box 90.2. What to Ask
with nosebleeds is von Willebrand disease, an autosomal-dominant
bleeding disorder characterized by varying degrees of factor VIII Nosebleeds
deficiency and platelet dysfunction (ie, decreased platelet adhe- ww Does the child pick his or her nose?
siveness). Hemophilia (factor VIII, IX, or XI deficiency), factor ww Has the child suffered any trauma recently?
VII deficiency, Glanzmann thrombasthenia, and Bernard-Soulier ww Is there suspicion of non-accidental trauma?
syndrome are other inherited bleeding disorders that may result ww Has the child recently had an upper respiratory infection?
in nosebleeds. Hepatic disease, severe vitamin K deficiency, or mal- ww Has the child recently had any systemic viral or bacterial illness?
absorption syndrome are associated with acquired coagulopathy, ww Does the child have any allergies?
of which nosebleed may be a presenting sign. Administration of ww Is the child exposed to dry conditions (eg, dry climate, dry heat,
valproic acid has been associated with acquired von Willebrand dehumidified air)?
disease and nosebleeds. ww Has the child put or tried to put foreign objects in his or her nose?
Nosebleeds may be a manifestation of hereditary or acquired ww Is there a history of easy bruising or prolonged, difficult-to-stop
blood vessel disorders. Hereditary hemorrhagic telangiectasia (ie, bleeding in the child or family?
Osler-Weber-Rendu disease) is an inherited autosomal-dominant ww Does the child or anyone in the family use any aspirin, aspirin-containing
disease with multiple mucosal telangiectasias, especially in the nose. medications, nonsteroidal anti-inflammatory drugs, or warfarin?
Because telangiectasias are deficient in muscular and connective ww Does the child or any family member use cocaine, heroin, inhalants, or
tissue, they may rupture spontaneously and bleed profusely. An any other drugs of abuse?
association between migraine headaches and recurrent nosebleeds ww Does the child or any family member use complementary and alterna-
has also been reported. tive therapies, such as Ginkgo biloba?
Neoplasms, particularly malignancies, are uncommon causes ww Which side of the nose was bleeding? Was it bilateral?
of nosebleeds in children. Although nasal polyps generally are ww How extensive was the bleeding?
benign, they usually occur in association with cystic fibrosis or ww Did the child spit out or swallow blood? Was there blood in the mouth?
allergies. Capillary, cavernous, and mixed hemangiomas may ww What measures were used to stop the bleeding? How long did it take to
occur in the nose and be a source of bleeding. Juvenile naso- stop the bleeding?
pharyngeal angiofibroma occurs almost exclusively in adoles- ww Was this the first nosebleed? If nosebleeds are recurrent, how often do
cent males who present with nasal obstruction and bleeding. they occur and how long do they last?
Rhabdomyosarcoma, lymphoma, and squamous cell carcinoma
of the nose, sinuses, or nasopharynx are rare causes of nosebleeds
in the pediatric population.
Drugs such as aspirin and nonsteroidal anti-inflammatory Physical Examination
drugs, which interfere with platelet function, and warfarin and A general physical examination should be performed. In children with
heparin, which inhibit clotting factors, increase the risk for nasal significant blood loss, particular attention should be directed toward
hemorrhage with minor trauma, infection, or inflammation. the mental status and vital signs to determine hemodynamic stability. If
Unintentional ingestion of these medications should be suspected vital signs are normal, these children should also be evaluated for ortho-
if they are accessible to the child. Snorting cocaine or heroin may static changes. If blood pressure is elevated, it should be reassessed at a
cause nasal septal perforation and nosebleeds. Some complemen- time when the anxiety related to the nosebleed has dissipated.
tary and alternative therapies, such as Ginkgo biloba, may also be In addition, the skin and mucous membranes should be checked
associated with abnormal bleeding. carefully for petechiae, purpura, and ecchymoses, as well as pallor.
Hypertension is rarely associated with nosebleeds in children. The conjunctiva may reveal icterus, a sign of liver disease present-
Wegener granulomatosis and lethal midline granuloma are rare idio- ing as a coagulopathy. The abdomen should be examined for hepato-
pathic inflammatory diseases in children that result in nasal tissue splenomegaly and the child should be evaluated for lymphadenopathy,
destruction and bleeding. Nosebleeds during menstruation, that is, which is suggestive of leukemia. Telangiectasia in the oropharynx
vicarious menstruation, may be secondary to hormonal changes that or mucous membranes is associated with hereditary hemorrhagic
result in vascular congestion of the nasal mucosa. telangiectasia. The oropharynx and nasopharynx should be examined
for masses, foreign body, and blood dripping downward from a poste-
Evaluation
rior bleed. Next, the nose should be inspected for the site of bleeding,
History with particular attention paid to the Kiesselbach area in the anterior
A thorough history often reveals the etiology of the nosebleed septum. In children younger than 2 years with nosebleeds, suspicion of
(Box 90.2). Information concerning the side of the nose from which non-accidental trauma should be considered, especially if nosebleeds
the bleeding occurred, amount of bleeding, measures used to stop are recurrent, the event is not witnessed, or the story given by the care-
the bleeding, and time required to stop the bleeding may be helpful giver changes. In such cases, the patient should be evaluated for burns
to quickly assess the severity of the nosebleed. and other marks that may be indicative of non-accidental trauma.

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 658 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Diagnostic Studies nosebleeds. Health professionals should reassure parents and chil-
Laboratory tests are rarely indicated in most children with nose- dren that most nosebleeds are easily controlled. Children should
bleeds. They might be considered for those patients in whom sit upright and lean forward slightly while direct pressure is applied
bleeding lasted longer than 30 minutes, patients younger than 2 years, to the nose. External compression of the nasal alae between the
or patients who have experienced more than 2 to 3 episodes of epi- thumb and forefinger for 5 to 10 minutes typically is sufficient. Most
staxis each week for several weeks. Hematocrit and hemoglobin tests nosebleeds originate at the anterior and mid-portion of the nose;
should be performed if the nosebleeds are severe or recur frequently. thus, application of pressure here is more effective in stopping bleed-
For children with signs or symptoms of hypovolemia (ie, increased ing than application of pressure at the base of the nose.
pulse; cool, clammy skin; increased capillary refill; decreased blood Children who are actively bleeding through the nose at the
pressure) or a marked drop in hematocrit, blood should be tested time of evaluation by a primary care physician should be posi-
for type and crossmatch. If the history or physical examination are tioned sitting upright and leaning forward slightly, and they
suggestive of coagulopathy, a complete blood cell count with platelet should be given a basin and facial tissue. Direct pressure should
count and examination of peripheral smear, prothrombin time, and be applied by a provider or reliable parent to the anterior and
partial thromboplastin time should be obtained. If these laboratory mid-portion of the nose while following universal precautions.
tests are negative, screening for von Willebrand disease should be A cotton dental roll may be placed under the upper lip to com-
considered if the other laboratory tests are negative. press the labial artery in older children in whom concern about
Radiography and other imaging studies are rarely necessary displacement and possible aspiration of the cotton is minimal.
in children with nosebleeds. However, if a mass is visualized, fur- If the bleeding continues after external compression, children
ther imaging may be necessary to evaluate it. The most commonly should be instructed to blow their nose to remove as much clot
used modalities include magnetic resonance imaging and contrast- as possible. Fresh blood should be removed with suction. Cotton
enhanced computed tomography. Rarely, pseudoaneurysm of the pledgets moistened with a few drops of a topical vasoconstrictor,
internal carotid artery has been associated with epistaxis; in these such as 0.05% oxymetazoline (Afrin, OcuClear, Drixine), or topi-
cases, angiography may be required for further evaluation. cal thrombin should be inserted into the involved side of the nose.
Pressure should be applied for an additional 10 minutes. Because
phenylephrine has been associated with significant morbidity and
Management mortality when topically applied, it should be avoided in the man-
Children who present to primary care physicians with a history of agement of pediatric epistaxis.
1 or more nosebleeds that have resolved spontaneously or with appli- If the bleeding persists, cauterization of the bleeding site with a
cation of pressure to the nasal alae need no further treatment in the 75% silver nitrate stick is indicated. If not already applied, topical
office or emergency department setting, provided that the history anesthesia with 2% to 4% lidocaine should be applied before cau-
and physical examination are consistent with a benign cause. These terization. Continued bleeding is slowed by first cauterizing a small
children or their parents should be instructed to apply a lubricant ring around the bleeding point to interrupt flow from surrounding
(eg, petroleum jelly) or an antibiotic ointment, inside the septal por- vessels and then rolling the tip of the applicator onto the bleeding
tion of the involved naris twice a day for 3 to 5 days with a cotton- site for 5 seconds or less. Cauterization is often difficult in children,
tipped swab or little finger. Often the child’s little finger is used and consultation with an otolaryngologist is advised. Cauterization
because it is nonthreatening and it “knows where to go.” Further nose should not be performed in children with a bleeding diathesis. In
picking should be discouraged, and fingernails should be trimmed addition, cauterization should only be done unilaterally. Cauterizing
to minimize trauma. In addition, a bedside humidifier helps mois- both sides of the nasal septum can result in septal ischemia and pos-
turize the air, especially in dry climates or during the winter when sible necrosis and ultimately, septal perforation.
forced hot-air heat is used. Children whose nares moisten from If the bleeding continues, an absorbable nasal sponge made
rhinorrhea and then dry and crack also benefit from humidified air. of oxidized cellulose (eg, Merocel, Rhinocell, Surgicel) or gela-
Buffered saline nasal spray may also be helpful in humidifying the tin (eg, Gelfoam) may be directly applied to the bleeding site to
nose. Children who are prone to recurrent nosebleeds and in whom form an artificial clot. This also may be done to avoid cauterization.
serious causes have been ruled out may benefit from regular use of Hemostatic seals (eg, Floseal, Avitene) may also be used; these
some of the aforementioned measures when they have URIs or aller- agents are composed of collagen or thrombin derivatives and help
gic manifestations or are in a dry season or environment. Patients support platelet aggregation and clot formation when applied
with suspected allergic causes may benefit from the use of an oral to the bleeding site. Alternatively, nasal tampon, which is made
antihistamine or topical corticosteroids. In addition, for children of a dehydrated material that expands when it becomes moist,
with S aureus nasal colonization, eradication with a course of mupir­ may be inserted to tamponade the area of bleeding. This method
ocin nasal ointment should be considered. is less well tolerated than an absorbable nasal sponge, how-
Children or parents should be given advice about how to care ever. The application of antibiotic ointment, preferably mupiro-
for nosebleeds at home. These instructions can also be given to par- cin or chlorhexidine-neomycin, to the tampon allows for easier
ents who seek advice over the telephone about how to stop children’s insertion and removal and may prevent S aureus colonization

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 Chapter 90: Nosebleeds 659

and infection. Another option to stop the bleeding is insertion bleeding should be referred to an otolaryngologist for further care
of an inflatable balloon (eg, Rapid Rhino, Epi-Stat, Epi-max, and possible surgical intervention. Children with a documented or
Post-Stop) coated with a platelet aggregator. Continued uncon- suspected bleeding disorder should be referred to a hematologist.
trolled bleeding requires anterior nasal packing with gauze strips. Selective angiographic embolization (most commonly of the inter-
Antibiotic-impregnated (preferably with mupirocin), 1-inch pet- nal maxillary artery) by an interventional radiologist or sphenopal-
rolatum gauze strips may also be used, although these have not atine artery ligation may be indicated for patients with persistent,
been shown to prevent toxic shock syndrome. The nasal packing intractable nosebleeds. Intranasal laser surgery may be indicated in
should remain in place for approximately 2 to 3 days. Prophylactic patients with recurrent nosebleeds resulting from abnormal vascu-
antibiotics are not routinely recommended because they have not lar malformations, such as hereditary hemorrhagic telangiectasia.
been shown to prevent toxic shock syndrome. In the patient with In children with severe nosebleeds, an intravenous line should
evidence of an underlying sinus infection, however, it has been be started early, blood should be sent for type and crossmatch and,
shown that antibiotics that provide coverage for staphylococ- depending on the amount of blood loss and physical evidence of hypo-
cal organisms (in particular, methicillin-resistant S aureus) and volemia, fluid replacement therapy should be initiated. In children
sinusitis should be prescribed. For all patients with packing, an who are frightened or in whom certain procedures (eg, cauterization
otolaryngologist should be consulted, and the otolaryngologist of bleeding site, drainage of septal hematoma) are performed, proce-
should be present at the time of packing removal. In general, pack- dural sedation should be strongly considered. Intravenous pain med-
ing should not be done in patients younger than 1 year because ication should be used in children who need anterior or posterior
of the risk of aspiration. packing. If procedures that cause undue pain or discomfort are neces-
Posterior nosebleeds, which are more difficult to control than sary, general anesthesia in an operating room setting may be indicated.
anterior nosebleeds, should be suspected if the measures described Children with underlying systemic illness that is causative for nose-
previously are ineffective, bleeding is vigorous and the cause cannot bleeds may benefit from an individualized approach. For example,
be identified, or most of the bleeding is into the nasopharynx and platelets should be administered to patients with nonimmune throm-
mouth. A posterior nasal pack can be created using rolled gauze or bocytopenia, and appropriate factor should be administered to patients
a nasal tampon. Alternatively, a Foley catheter or an Epi-Stat inflat- with hemophilia. Patients with epistaxis who have von Willebrand dis-
able nasal balloon catheter may be used to control posterior nose- ease benefit from packing with cellulose soaked in topical thrombin
bleeds. Posterior packs should never be used without the presence as well as administration of desmopressin. Replacement therapy with
of a concurrent anterior nasal pack. Thus, a double-balloon catheter Factor VIII or with recombinant human Factor VIIa depends on the
(eg, Nasostat, Epi-Stat) can be used, obviating the need for 2 separate type of von Willebrand disease and their response to local treatment.
packing mechanisms. Posterior packing can result in significant dis- For patients with immune thrombocytopenia, intravenous immuno-
comfort for the patient, and appropriate analgesia should be provided. globulin or anti-D immune globulin in conjunction with high-dose
However, significant pain with balloon inflation should not occur, steroids has been shown to be effective. Cautery should be avoided in
and overinflation may result in ischemia if the balloon is not slightly patients with hereditary hemorrhagic telangiectasia; fibrin glue has
deflated. Posterior packing should be left in place for 2 to 3 days, and been shown to be effective in controlling bleeding.
antibiotics should be initiated to prevent sinusitis. Additionally, pos- Hospitalization is rarely necessary for children with nosebleeds.
terior packing can result in hypoventilation and hypoxia. Therefore, However, children who are hemodynamically unstable on presentation
all patients with posterior packing should be admitted to the hospi- usually require inpatient treatment. As mentioned previously, children
tal and placed on a cardiorespiratory monitor. This also allows for for whom placement of a posterior nasal pack is required should be
monitoring of potential complications, such as aspiration caused by admitted to the hospital for close airway observation. Hospitalization
unintentional dislodgment of the packing material; septal ischemia may be necessary for children with difficult-to-stop bleeds who need
secondary to packing; and the development of hypotension, brady- an anterior nasal pack or who have a bleeding disorder or underlying
cardia, or apnea secondary to a pronounced nasal-vagal response. chronic illness, such as leukemia, aplastic anemia, or HIV infection.
The need for consultation with an otolaryngologist is depen-
dent on the experience of the individual physician and availability of Prognosis
consultation. Prompt consultation, if available, should be obtained The prognosis for nosebleeds in children is excellent. Almost
for children with severe nosebleeds who need volume replacement; all nosebleeds are easily controlled with a minimal amount of
with nosebleeds that do not stop or that recur after implementation home care or medical management. Surgery is rarely indicated.
of the aforementioned measures; who may need anterior or posterior Complications associated with significant nosebleeds include hypo-
nasal packing; and with recurrent, difficult-to-stop nosebleeds. For volemia resulting from blood loss, and sinusitis and toxic shock
patients with suspected nasal fracture, surgical consultation should syndrome resulting from S aureus associated with anterior or pos-
be sought prior to placement of nasal packing. In some cases, obtain- terior nasal packing. Recurrent idiopathic epistaxis resolves with
ing consultation before cauterization with silver nitrate is advisable, time and is uncommon in children older than 14 years. Even for
particularly in patients with known bleeding disorders. Children rare causes of nosebleeds, the prognosis is very good with prompt
with septal hematoma, tumor, polyp, telangiectasia, and intractable diagnosis and treatment.

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 660 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Higgins TS, Hwang PH, Kingdom TT, Orlandi RR, Stammberger H, Han JK.
CASE RESOLUTION Systematic review of topical vasoconstrictors in endoscopic sinus surgery.
Laryngoscope. 2011;121(2):422-432 PMID: 21271600 https://doi.org/10.1002/
The boy has experienced several nosebleeds of short duration associated with a
lary.21286
URI and winter dryness. His history and physical examination are unremarkable
for a bleeding disorder or chronic illness. The small amount of blood in his nose Kasperek ZA, Pollock GF. Epistaxis: an overview. Emerg Med Clin North Am.
is consistent with an anterior nosebleed originating from the Kiesselbach area, 2013;31(2):443–454 PMID: 23601481 https://doi.org/10.1016/j.emc.2013.01.008
with inflammation and drying of the nasal mucosa. Laboratory tests are not Manning SC, Culbertson MC Jr. Epistaxis. In: Bluestone CD, Stool SE, Alper
indicated. The parents should be instructed to apply petroleum jelly to the sep- CM, et al, eds. Pediatric Otolaryngology. 4th ed. Philadelphia, PA: Saunders;
tal portion of the left side of the child’s nose twice a day for 3 to 5 days and to 2003:925–931
humidify the child’s bedroom. They should also be reassured that their child has
McIntosh N, Mok JY, Margerison A. Epidemiology of oronasal hemorrhage in
a common condition that he will outgrow.
the first 2 years of life: implications for child protection. Pediatrics. 2007;120(5):
1074–1078 PMID: 17893187 https://doi.org/10.1542/peds.2007-2097
Patel PB, Kost SI. Management of epistaxis. In: King C, Henretig FM, eds.
Selected References
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Bent S, Goldberg H, Padula A, Avins AL. Spontaneous bleeding associated Williams & Wilkins; 2008:604–614
with ginkgo biloba: a case report and systematic review of the literature. J Gen Rees P, Kemp A, Carter B, Maguire S. A systematic review of the probability
Intern Med. 2005;20(7):657–661 PMID: 16050865 https://doi.org/10.1007/ of asphyxia in children aged <2 years with unexplained epistaxis. J Pediatr. 2016;168:
s11606-005-0114-4 178.e10–184.e10 PMID: 26507155 https://doi.org/10.1016/j.jpeds.2015.09.043
Bernius M, Perlin D. Pediatric ear, nose, and throat emergencies. Pediatr Clin Riviello RJ. Otolaryngologic procedures. In: Roberts JR, Custalow CB, Thomsen
North Am. 2006;53(2):195–214 PMID: 16574522 https://doi.org/10.1016/j. TW, eds. Roberts and Hedges’ Clinical Procedures in Emergency Medicine and
pcl.2005.10.002 Acute Care. 7th ed. Philadelphia, PA: Elsevier; 2019:1338–1383
Briskin KB. Epistaxis. In: Baren JM, Rothrock SG, Brennan JA, Brown L, Sandoval C, Dong S, Visintainer P, Ozkaynak MF, Jayabose S. Clinical and lab-
eds. Pediatric Emergency Medicine. Philadelphia, PA: Saunders; 2008: oratory features of 178 children with recurrent epistaxis. J Pediatr Hematol
402–404 Oncol. 2002;24(1):47–49 PMID: 11902740 https://doi.org/10.1097/00043426-
Douglas R, Wormald PJ. Update on epistaxis. Curr Opin Otolaryngol Head 200201000-00013
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MOO.0b013e32814b06ed epistaxis and nasal colonization with Staphylococcus aureus. Otolaryngol Head
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 CHAPTER 91

Strabismus
Teresa O. Rosales, MD

CASE STUDY
The mother of an 8-month-old reports that every Questions
time her son looks to either side, his eyes seem 1. What is strabismus?
crossed. Otherwise, he is growing and developing 2. What conditions make an infant’s eyes appear
normally. Symmetric pupillary light reflex, bilateral crossed? What is the differential diagnosis?
red reflex, and normal extraocular eye movements 3. What tests are used in the office evaluation of
in all directions are noted on physical examination of the child with suspected strabismus?
the eyes. 4. Which infants with crossed eyes require referral
for further evaluation and treatment?

The term strabismus refers to any abnormality in ocular alignment, Strabismus may also be classified as congenital or acquired, inter-
whether the eyes go in or out or 1 eye is higher than the other. It mittent or constant, and alternating or unilateral. In nonparalytic
is among the most common eye problems observed in infants and strabismus, the extraocular muscles and the nerves that control them
children. The pediatrician plays an important role in the early detec- are normal. The degree of deviation is constant or nearly constant
tion and prompt referral of children with suspected ocular align- in all directions of gaze. Nonparalytic strabismus is the most com-
ment abnormalities. mon type of strabismus occurring in children, and congenital or
infantile esotropia is usually of this type. Ocular or visual defects,
Epidemiology such as cataracts or high refractive errors, occasionally cause non-
paralytic strabismus.
Strabismus affects approximately 3% of the population, and the con-
In paralytic strabismus, paralysis or paresis of 1 or more of the
dition occurs most commonly in children younger than 6 years.
extraocular muscles produces a muscle imbalance. The deviation is
Approximately 50% of all affected children have a positive family
asymmetric, and characteristically the degree of deviation is worse
history of strabismus, although the exact genetic mode of inher-
when gazing in the direction of the affected muscle. Paralytic stra-
itance is unclear. Up to 75% of otherwise healthy newborns and
bismus may be congenital or acquired. Congenital paralytic strabis-
infants have transient intermittent strabismus during the first
mus may be the result of birth trauma, muscle anomalies, abnormal
3 months after birth.
development of the cranial nerve nuclei, or congenital infections
affecting the eyes. Congenital strabismus may occur in association
Clinical Presentation
with neurodevelopmental disorders, such as cerebral palsy. Acquired
Children with ocular misalignment have an asymmetric corneal paralytic strabismus resulting from extraocular muscle palsies usu-
light reflex test. Eye movement is noted on cover testing. Children ally indicates the presence of a serious underlying condition, such
with paralytic strabismus may present with torticollis (ie, head tilt) as an intracranial tumor, a demyelinating or neurodegenerative dis-
in an effort to avoid double vision (ie, diplopia; Box 91.1). ease, myasthenia gravis, progressive myopathy, or central nervous
system (CNS) infection. Children may present with double vision
Pathophysiology or a compensatory head tilt to avoid double vision.
Normal binocular vision is the result of the fusion of images from Intermittent (ie, latent) misalignment of the eyes is referred to
both eyes working synchronously across the visual field. Six extra- as a phoria. Under normal conditions, the fusional mechanisms of
ocular muscles control all eye movements. Orthophoria is proper the CNS maintain eye alignment. Eye deviation is appreciated only
alignment of the eyes, and strabismus results from an imbalance in under certain conditions, such as illness, fatigue, or stress, or in cases
muscle movements. in which fusion is interrupted by occluding 1 eye (eg, during cover
testing). Some degree of phoria may be found in almost all individ-
Strabismus uals and typically, it is asymptomatic. Larger degrees of phoria may
The classification of strabismus is complex. Based on etiology, it may give rise to troublesome symptoms such as headaches, transient
be considered nonparalytic (comitant) or paralytic (noncomitant). diplopia, or asthenopia (eg, eyestrain).

661

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 662 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

associated with strabismus that manifests in children younger than

Box 91.1. Diagnosis of Strabismus
4 years. If the condition that causes amblyopia is not corrected while
ww Head tilt the brain’s visual pathways are still malleable (eg, before approxi-
ww Double vision (ie, diplopia) mately 6–7 years of age), children may experience some degree of
ww Squint permanent visual loss.
ww Asymmetric corneal light reflex
ww Eye movement with cover testing Differential Diagnosis
The differential diagnosis of strabismus may be divided into 3 cat-
egories: transient neonatal strabismus, congenital or infantile stra-
bismus, and acquired strabismus. It is important to differentiate true
Constant misalignment of the eyes is referred to as heterotro- strabismus from the illusion of deviation created by facial asymme-
pia (ie, strabismus). This condition occurs because normal fusional try or anatomic variations (ie, pseudostrabismus).
mechanisms are unable to control eye deviation; children are unable
to use both eyes together to fixate on an object. In alternating Transient Neonatal Strabismus
heterotropia, both eyes appear to deviate equally, and vision gener- Eye alignment in normal newborns and infants during the first 2
ally develops normally in each eye because children have no pref- to 3 months after birth may vary from normal to intermittent eso-
erence for fixation. If strabismus affects only 1 eye, the other eye is tropia or exotropia. These deviations are believed to result from
always used for fixation, and a danger exists for the development of CNS immaturity and resolve spontaneously in most infants by
amblyopia or vision loss in the deviating eye. 4 months of age. If such deviations are constant or persist beyond
Convergent deviation, which is a turning in or crossing of the this age, the child should be referred to an ophthalmologist for fur-
eyes, is an esodeviation (eg, esotropia, esophoria). Divergent devi- ther evaluation.
ation, which is a turning out of the eyes, is an exodeviation. The
term hypertropia refers to conditions involving upward vertical Congenital or Infantile Strabismus
deviations. Esodeviations are the most common type of ocular Congenital or infantile strabismus is deviation that occurs within
misalignment, accounting for 50% to 75% of all cases of stra- the first 6 months after birth. Because the deviation may not always
bismus. Vertical deviations represent less than 5% of all cases of be present at birth, the term “infantile” may be more accurate.
strabismus. The differential diagnosis of infantile strabismus is presented in
Box 91.2. Pseudoesotropia is an illusion or apparent deviation; it is
Amblyopia not a true deviation. In many infants, the broad, flat nasal bridge
Amblyopia is a potential complication if strabismus is not corrected and prominent epicanthal folds may obscure a portion of the sclera
in a timely manner. Amblyopia refers to poor vision in 1 eye or, near the nose and create the appearance of esotropia (Figure 91.1).
rarely, both eyes despite correction of any refractive errors. A child This illusion resolves as children mature. Symmetric corneal light
with no significant refractive error and with visual acuity of 1 eye reflexes or normal cover tests differentiate pseudoesotropia from
that is worse than the other has amblyopia. Diagnosis of amblyo- true esotropia.
pia is based on a difference in visual acuity of at least 2 lines (eg, Esotropia is among the more common types of childhood stra-
20/20 in 1 eye and 20/40 in the other), as measured by reading bismus. The constant deviation of infantile esotropia typically is
an eye chart. Amblyopia is the leading cause of preventable visual readily apparent because of the large angle of deviation. Affected
loss in children. children usually have good bilateral vision because of the alternation
Amblyopia may be classified into 3 major categories: depriva- of fixation from 1 eye to the other. Cross-fixation, in which children
tion amblyopia, refractive amblyopia, or strabismic amblyopia. look to the left with the adducted right eye and to the right with the
Generally, deprivation amblyopia is the result of obstruction of adducted left eye, may be evident because of the large angle of devi-
vision caused by a unilateral lesion or developmental defect in 1 of ation. Rarely, esotropia may be caused by abducens palsy (ie, palsy
the structures of the eye or its visual pathways. Causes of depriva- of the sixth cranial nerve) in isolation or association with other cra-
tion amblyopia include congenital cataract, ptosis, corneal opacity, nial nerve palsies (eg, Möbius syndrome).
retinal detachment, retinoblastoma, coloboma, optic nerve defect Infantile exotropia is less common than esotropia. Like esotro-
(eg, optic nerve hypoplasia), and orbital tumor. These conditions pia, exotropia manifests within the first 6 months after birth and is
cause a lack of formation of a retinal image or a blurred retinal characterized by a large angle of deviation. Causes of infantile exo-
image, usually in 1 eye. Refractive amblyopia refers to a blurring tropia include trochlear palsy (ie, congenital third nerve palsy, a type
of the retinal image resulting from large or asymmetric refractive of paralytic strabismus) and abnormalities of the bones of the orbit
errors. Amblyopia is usually considered a unilateral abnormality, (eg, Crouzon syndrome).
but it may be bilateral in cases of large refractive errors (usually,
astigmatism or hyperopia). In strabismic amblyopia, the imma- Acquired Strabismus
ture or developing brain suppresses images from the deviating Acquired strabismus may result from a variety of causes (Box 91.2).
eye to prevent diplopia. Strabismic amblyopia is most commonly Accommodative esotropia and intermittent exotropia are the 2 most

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 Chapter 91: Strabismus 663

common types of acquired strabismus. Accommodative esotro- when accommodation occurs, so does convergence. If children have
pia typically manifests in children between 2 and 4 years of age severe hyperopia (ie, farsightedness), the amount of convergence
but may occur as early as 6 months or as late as 8 years. Children that occurs with accommodation may be severe and may result in
with hyperopia use accommodation (ie, attempts to focus) to see the development of esotropia. Such esotropia is usually intermittent
clearly. The accommodative reflex is closely linked to convergence; initially and only gradually becomes constant. Often, the deviating
eye becomes amblyopic.
Intermittent exotropia, which is the most common form of exo-
Box 91.2. Differential Diagnosis of Strabismus
deviation in children, manifests between birth and 4 years of age.
Congenital or Infantile Strabismus Although it begins as an intermittent condition in which the eyes
Esophoria/Esotropia appear to deviate outward, especially when a child is tired, ill, or fix-
ww Infantile esotropia ating at a distance, the exotropia can become constant with time.
ww Pseudoesotropia A child also may close 1 eye in bright sunlight, presumably in an
ww Möbius syndrome attempt to prevent diplopia.
ww Abducens palsy (ie, congenital sixth nerve palsy)
ww Duane syndrome Evaluation
Exophoria/Exotropia
ww Congenital exotropia History
ww Trochlear palsy (ie, congenital third nerve palsy) Evaluation of infants or children with suspected strabismus
ww Abnormalities of the bony orbit (eg, Crouzon syndrome) should begin with a thorough family history, because strabis-
Esophoria/Esotropia and Exophoria/Exotropia mus often runs in families (Box 91.3). Parental description of
ww Duane syndrome (esotropia more common than exotropia) the ocular deviation is useful because misalignments, especially
intermittent deviations that may manifest only when children
Acquired Strabismus
are tired, may not always be evident during the office visit. A
Esophoria/Esotropia
history of head or orbital trauma may help in the evaluation of
ww Accommodative esotropia
acquired strabismus.
ww Abducens palsy (benign sixth nerve palsy)
Exophoria/Exotropia
ww Intermittent exotropia Physical Examination
ww Overcorrection after surgery for esotropia On physical examination, the presence of any dysmorphic fea-
Esophoria/Esotropia and Exophoria/Exotropia tures and structural abnormalities of the face or neck (eg, torti-
ww Poor vision collis) is noted. Children with paralytic strabismus (eg, trochlear
ww Orbital trauma causing entrapment of extraocular muscles palsy [ie, fourth nerve palsy], superior oblique palsy) may com-
ww Intracranial tumors or tumors involving the orbit (eg, retinoblastoma) pensate for their paretic lesion by tilting the head to avoid diplo-
ww Myasthenia gravis pia. It is important that visual screening of children begin during
ww Central nervous system infection (eg, meningitis) the neonatal period. Newborn screening should emphasize the
ww Central nervous system tumor presence of a bilateral red reflex. An abnormal red reflex or a
ww Orbital cellulitis white reflex may be indicative of a cataract or retinoblastoma,
both of which require immediate referral to an ophthalmologist.
Evaluation for ocular alignment should begin at the 4-month
health maintenance visit. Intermittent misalignment of the eyes
is often seen in otherwise healthy infants younger than 4 months.
Constant misalignment at any age, however, requires immedi-
ate attention.

Box 91.3. What to Ask

Suspected Strabismus
ww Does the infant or child have a family history of strabismus?
ww Does the infant or child constantly tilt the head toward one side?
ww Does the infant or child squint?
ww Has the infant or child reported blurred or double vision?
ww Does the infant or child close 1 eye in bright sunlight?
Figure 91.1. Illustration showing a child with pseudoesotropia. Note the wide ww Has the infant or child incurred any recent trauma to the eyes or head?
nasal bridge and prominent epicanthal folds.

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 664 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Vision Testing preferred by the primary care physician as a screening tool. The
Testing visual acuity is essential in the evaluation of children with cover-uncover test detects only manifest deviation or heterotropia.
suspected strabismus. Such testing may be performed as early as In the alternate cover test, first 1 eye and then the other is covered
3 years of age if children are cooperative. Charts with symbols, as the child fixates on an object at a distance. If neither eye moves as
figures, or letters can be used. The traditional Snellen chart with the cover is moved rapidly between the eyes, the eyes are in align-
letters can generally be used in children as young as 4 years of age. ment (ie, orthophoric). With heterotropia, the deviating eye moves
Decreased vision in 1 eye may be indicative of ocular abnormalities, when the fixating eye is occluded; in heterophoria, the deviating eye
including ocular deviations. moves when it is uncovered (Figure 91.2).
The 2 basic tests for strabismus that can be easily performed in The alternate cover test may be illustrated with the following
the office are the corneal light reflex test (ie, Hirschberg method) example. A child presents with constant esotropia of the left eye.
and the cover test. The pediatrician should be comfortable perform- When the right or fixating eye is occluded, the left eye is forced to
ing both tests. fixate so that the child can see, and the left eye moves outward as
The simplest and quickest test for the evaluation of strabismus is the right eye is occluded. In the case of a child with an esophoria
the corneal light reflex test, in which a penlight is projected simul- or latent deviation of the left eye, the eye deviates inward when it
taneously onto the corneas of both eyes as the child looks straight is occluded because it is not being forced to fixate. As the cover is
ahead. The examiner compares the placement of the corneal light moved from the left eye to the right, the left eye moves outward and
reflex in each eye with respect to the center of the pupil. If the eyes returns to a position of fixation.
are straight, the reflection appears symmetrically in the center of The alternate cover test may be more difficult to interpret in
both pupils or on the same point on each cornea. If the light reflex children with bilateral or alternating strabismus who use both
appears off center in 1 eye compared with the other, the test is posi- eyes in turn for fixation. It is not necessary for the pediatrician
tive for ocular deviation or heterotropia. Nasal deviation of the light to identify exactly what type of strabismus is present. Rather, it is
reflex on the cornea indicates exotropia on that side, temporal devia- sufficient to note abnormal movement and refer the child for fur-
tion signifies esotropia, and inferior deviation indicates hypertropia. ther evaluation. An ophthalmologist can perform a more detailed
Unlike the corneal light reflex test, which may be performed examination.
even in uncooperative children, cover tests require a child’s cooper-
ation and ability to fixate on a specified object. These tests are used Management
to detect heterophoria. Two types of cover test are used: the alter- The goals of management are the attainment of the best possible
nate cover test and the cover-uncover test. Only the alternate cover vision in each eye, straight eyes cosmetically, and fusion. The sooner
test detects both heterophoria and heterotropia. This test may be deviations are corrected, the better the child’s chances for equal

A Left eye esotropia Right or fixated eye covered, left eye

moves outward

B Left eye esophoria (eyes aligned) Left eye deviates inward when occluded

As the occluder is moved from the left to the right eye, the left eye moves outward to a
position of fixation.

Figure 91.2. Alternate cover test in the detection of strabismus. Normally, both eyes appear to be aligned and
centrally fixed. A, Detection of esotropia. The right eye is fixating, and a left esotropia is present. When the right
or fixating eye is covered, the left eye moves outward (away from the nose). B, Detection of esophoria. The eyes
are aligned with a left esophoria. When the left eye is covered, it deviates inward. As the cover is moved from the
left eye to the right eye, the left eye moves outward to a position of fixation.

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 Chapter 91: Strabismus 665

bilateral vision. Treatment includes correction of any underlying Selected References

refractive error with corrective lenses. Such lenses, which remedy
Campos EC. Why do the eyes cross? A review and discussion of the nature
the refractive error and minimize the need for accommodation, are
and origin of essential infantile esotropia, microstrabismus, accommodative
used in the management of accommodative esotropia. Glasses are esotropia, and acute comitant esotropia. J AAPOS. 2008;12(4):326–331 PMID:
also used in cases of anisometropic amblyopia in which the refrac- 18550403 https://doi.org/10.1016/j.jaapos.2008.03.013
tive error in 1 eye is significantly different from that in the other eye. Donahue SP, Baker CN; American Academy of Pediatrics Committee on Practice
Children who do not see equally well from both eyes may be and Ambulatory Medicine, Section on Ophthalmology; American Association of
at risk for amblyopia if they preferentially fixate with only 1 eye. If Certified Orthoptists; American Association for Pediatric Ophthalmology and
detected, amblyopia should be corrected with occlusion therapy of Strabismus; American Academy of Ophthalmology. Procedures for the evalu-
the fixating “good” eye. Occlusion therapy forces children to use the ation of the visual system by pediatricians. Pediatrics. 2016;137(1):e20153597
amblyopic eye. This treatment is best accomplished by constantly PMID: 26644488 https://doi.org/10.1542/peds.2015-3597
patching the eye with better vision during waking hours or penal- Ekdawi NS, Nusz KJ, Diehl NN, Mohney BG. Postoperative outcomes in chil-
ization with dilating drops in the better-seeing eye. Children require dren with intermittent exotropia from a population-based cohort. J AAPOS.
repeat evaluations and close monitoring during this therapy. 2009;13(1):4–7 PMID: 18848478 https://doi.org/10.1016/j.jaapos.2008.
06.001
If eye alignment is not achieved nonsurgically, surgical correc-
tion may be necessary. Surgery may be used to achieve the best pos- Greenberg AE, Mohney BG, Diehl NN, Burke JP. Incidence and types of child-
hood esotropia: a population-based study. Ophthalmology. 2007;114(1):170–174
sible ocular alignment and is usually required for the management
PMID: 17070595 https://doi.org/10.1016/j.ophtha.2006.05.072
of infantile esotropia. It is generally performed in infants between
Keech R. Practical Management of Amblyopia. San Francisco, CA: American
6 months and 1 year of age, while the visual system is still pliable
Academy of Ophthalmology; 2000:1–3. Focal Points: Clinical Modules for
enough to allow for the development of postoperative binocular Ophthalmologists
vision. Surgery also may be needed in children with intermittent
Louwagie CR, Diehl NN, Greenberg AE, Mohney BG. Long-term follow-up of
exotropia in whom the frequency of deviation is increasing. congenital esotropia in a population-based cohort. J AAPOS. 2009;13(1):8–12
PMID: 18993096 https://doi.org/10.1016/j.jaapos.2008.06.013
Prognosis Ludwig IH, Clark RA, Stager DR Sr. New strabismus surgical techniques.
Certain conditions, such as pseudoesotropia or infrequent intermit- J AAPOS. 2013;17(1):79–88 PMID: 23415038 https://doi.org/10.1016/j.
tent exotropia, may resolve as children mature. Others, such as infan- jaapos.2012.09.019
tile esotropia, require early detection and management to achieve Olitsky SE, Hug D, Plummer LS, Stass-Isern M. Disorders of eye movement
the best binocular vision. Amblyopia and permanent vision loss may and alignment. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds.
result if correction of strabismus is delayed. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Saunders Elsevier;
2011:2157–2162
Parks MM. Binocular vision. In: Tasman W, Jaeger EA, eds. Duane’s
CASE RESOLUTION Ophthalmology. 15th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2010
The infant has pseudoesotropia. Although the boy’s eyes appear to deviate, the Wallace DK, Chandler DL, Beck RW, et al; Pediatric Eye Disease Investigator
corneal light reflex and cover tests are normal. Physical examination reveals Group. Treatment of bilateral refractive amblyopia in children three to less than
prominent epicanthal folds and a broad, flat nasal bridge. 10 years of age. Am J Ophthalmol. 2007;144(4):487–496 PMID: 17707330 https://
doi.org/10.1016/j.ajo.2007.05.040

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 CHAPTER 92

Infections of the Eye

Teresa O. Rosales, MD

CASE STUDY
A 10-day-old neonate has a 1-day history of red, watery Questions
eyes and nonproductive cough with no fever. She is 1. What is the differential diagnosis of conjunctivitis
breastfed and continues to eat well. She was the 3,232-g during and after the neonatal period?
(7-lb, 2-oz) product of a term gestation, born via normal 2. What laboratory tests, if any, should be performed
spontaneous vaginal delivery without complications to a in neonates with conjunctivitis?
26-year-old woman. The pregnancy was also uncompli- 3. When is chest radiography indicated in the evalua-
cated. No one at home is ill. tion of the neonate with conjunctivitis?
On examination, the infant is afebrile with normal 4. What are management strategies for eye infection
vital signs. Examination of the eyes reveals bilateral con- in older infants and children?
junctival injection with only a mild amount of purulent
discharge. Bilateral red reflexes are present. The remain-
der of the physical examination is within normal limits.

Infections of the eye and surrounding structures are commonly Clinical Presentation
seen by pediatricians. Such infections range in severity from com-
Red eyes and discharge are the common presenting signs of infec-
mon problems, such as blepharitis and conjunctivitis, which lack
tion of the eyelids and conjunctivas. Eyelid edema and erythema
serious sequelae, to severe and less common infections, such as
surrounding the eye characterize periorbital and orbital cellulitis.
periorbital and orbital cellulitis. The presenting concern in many
Proptosis, abnormal extraocular movement, or loss of visual acuity
children with eye infection is a red-appearing eye. Familiarity with
may signal spread of the infection beyond the orbital septum, as in
the common causes of a red eye makes prompt diagnosis and treat-
orbital cellulitis (Box 92.1).
ment possible.

Epidemiology Pathophysiology
Eye infections may be divided into 2 types: those affecting the struc-
Conjunctivitis, which affects children of all ages, is perhaps the
tures surrounding the orbit and those involving the orbital con-
most common eye infection of childhood. The rate of conjunc-
tents themselves (Figure 92.1). Although all structures surrounding
tivitis in the newborn period is estimated to range from 1.6%
the eye may potentially become inflamed or infected, the eyelids;
to 12%. The prevalence of chlamydial conjunctivitis is approxi-
nasolacrimal drainage system, as in dacryocystitis (see Chapter 93);
mately 8 in 1,000 live births. Approximately two-thirds of acute
conjunctiva; and cornea are most commonly involved. Orbital cellu-
childhood conjunctivitis has a bacterial etiology, and one-third
litis is defined as an infection of the orbital structures posterior to
is viral. Haemophilus influenzae and Streptococcus pneumoniae
the orbital septum. The orbital septum, an extension of the perios-
are the most common bacterial agents and account for approx-
teum of the bones of the orbit, extends to the margins of the upper
imately 40% and 10% of culture-proven cases, respectively. The
and lower eyelids and provides an anatomic barrier to the spread
incidence of H influenzae is decreasing with the advent of the
of most infectious and inflammatory processes. Preseptal or perior-
H influenzae type b vaccine. The incidence of community-
bital cellulitis is localized to structures superficial to the orbital sep-
acquired methicillin-resistant Staphylococcus aureus (MRSA) is
tum, whereas postseptal or orbital cellulitis implies that the disease
increasing. Staphylococcus aureus is isolated from the conjunc-
process involves orbital structures extending beyond the septum.
tivas of children with acute conjunctivitis, but it is found with
approximately the same frequency in the eyes of children with-
out conjunctivitis. Adenovirus is the most common viral isolate. Differential Diagnosis
Most cases of acute conjunctivitis in young adults have a viral Infections of the eye are included in the differential diagnosis of
etiology. Serious eye infections, such as periorbital and orbital conditions presenting with red eye (Box 92.2). Also included in the
cellulitis, occur far less often. differential diagnosis are congenital, inflammatory, traumatic, and

667

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 668 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Box 92.1. Diagnosis of Eye Infection Box 92.2. Differential Diagnosis of Red Eye
Eyelid Infections Congenital Anomalies
ww Redness ww Nasolacrimal duct obstruction
ww Itching (blepharitis) ww Congenital glaucoma
ww Burning (blepharitis) Infection
ww Scales at the base of the lashes (seborrheic blepharitis) ww Keratitis
ww Swelling (hordeolum or chalazion) ww Conjunctivitis
ww Pain (hordeolum) ww Dacryocystitis
Conjunctivitis ww Corneal ulcer
ww Conjunctival injection and edema ww Periorbital and orbital cellulitis
ww Excessive tearing Inflammation
ww Discharge or crusting ww Blepharitis
ww Itching (allergic conjunctivitis) ww Hordeolum
Uveitis ww Chalazion
ww Conjunctival injection Trauma
ww Pain ww Corneal abrasion
ww Blurred vision ww Foreign body
ww Photophobia ww Blunt trauma: hyphema
ww Headache ww Perforating injuries
Periorbital Cellulitis ww Exposure to chemicals or other noxious substances
ww Unilateral eyelid edema Systemic Illnesses
ww Erythema surrounding the eye ww Kawasaki disease
ww Pain ww Varicella
ww Fever ww Measles
Orbital Cellulitis ww Lyme disease
ww Eyelid edema ww Stevens-Johnson syndrome
ww Proptosis ww Ataxia-telangiectasia
ww Decreased extraocular movements ww Juvenile rheumatoid arthritis
ww Loss of visual acuity
ww Fever
ww Ill appearance
ww Associated sinusitis systemic processes. Although infection and irritation are by far the
most common causes of an acute onset of red eye, other possibili-
ties, including trauma, glaucoma, or underlying systemic disease,
must be considered.

Orbital
Eyelid Infections
septum Common conditions affecting the eyelid and its related structures
Cul-de-sac Vitreous
are blepharitis, hordeolum, and chalazion.
Lens
Blepharitis is an inflammation of the lid margins. This condi-
Anterior
chamber Sclera tion, which is often bilateral, may be chronic or recurrent. The 2
Retina
Cornea most common causes of blepharitis are staphylococcal infection
and seborrheic dermatitis. The child with staphylococcal blephari-
tis often presents with scales at the base of the lashes, ulceration of
the lid margin, and loss of lashes. The infection may spread to the
Iris conjunctiva or cornea, producing conjunctivitis or keratitis. In con-
Optic trast, seborrheic blepharitis is characterized by greasy, yellow scales
nerve attached to the base of the lashes. Additionally, associated seborrhea
Orbital
septum of the scalp or eyebrows may be present. Mixed staphylococcal-
seborrheic infections, which occur as staphylococcal superinfection,
Figure 92.1. The eye and surrounding structures. may complicate seborrheic blepharitis. Less commonly seen forms

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 Chapter 92: Infections of the Eye 669

of blepharitis are parasitic blepharitis, which results from infesta- keratoconjunctivitis is a highly contagious form of adenoviral con-
tion of the lids by the head louse, Pediculus humanus capitis, or crab junctivitis. Affected children often report foreign body sensation
louse, Phthirus pubis, and primary or recurrent human herpesvirus beneath the lids or photophobia resulting from corneal involvement.
1 infections that may manifest as clusters of vesicles on the eyelids. Pharyngeal conjunctival fever, another presentation of adenoviral
Rosacea may rarely occur in childhood and can present very simi- conjunctivitis, usually manifests as conjunctivitis in association with
larly to chronic blepharitis. pharyngitis and fever.
The glands of the eyelid can also be infected. Staphylococcus The infant with chronic or recurrent conjunctival discharge may
aureus is the most common organism. A hordeolum, or common have an obstruction of the nasolacrimal duct, whereas the older
stye, results from an infection of the meibomian glands located along child with chronic conjunctivitis may have allergic disease, recur-
the lid margins. The glands become obstructed and an abscess can rent blepharitis, or chlamydial infection. Blepharitis is the most com-
form. The affected child presents with a well-circumscribed, pain- mon cause of chronic conjunctivitis in older children. Staphylococcus
ful swelling that may be at the lid margin or deeper in the lid tis- aureus is frequently implicated in these infections.
sue. These generally rupture or resolve without complications when Itching, tearing, and conjunctival edema are the hallmarks of
managed aggressively with hot compresses. allergic conjunctivitis, a noninfectious form of conjunctival inflam-
A chalazion is a hordeolum that has not resolved over weeks mation often occurring in children with other allergic disorders,
to months. It is no longer an infectious process but has become a such as asthma or hay fever. Conjunctival injection tends to be
chronic granulomatous inflammation of the meibomian glands. mild, bilateral, and seasonal. The etiology is most often a hyper-
The resulting firm, nontender, slow-growing mass within the sensitivity to pollens, dust, or animal dander. Vernal conjunctivitis
upper or lower eyelid may be painful if secondary infection is is a bilateral, severe form of allergic conjunctivitis seen primarily
present. during childhood. Most cases occur during the spring and summer.
Severe itching and tearing are the most frequent complaints. The
Infections of the Conjunctiva palpebral conjunctiva may have a cobblestone appearance result-
Conjunctivitis refers to any inflammation of the conjunctiva. The ing from the accumulation of inflammatory cells, or there may be
condition may be allergic, chemical, viral, or bacterial in etiology. small, elevated lesions of the bulbar conjunctiva at the corneal lim-
Additionally, it may be a sign of systemic disease, such as Kawasaki bus. The pathogenesis is unclear, but atopy seems to play a role.
disease or Stevens-Johnson syndrome. Chlamydial conjunctivitis frequently affects neonates and adoles-
Acute conjunctivitis, or pinkeye, is common during childhood cents. Inclusion conjunctivitis is an acute infection of the eyes caused
and can be extremely contagious. The usual signs are conjunctival by sexually transmitted Chlamydia trachomatis (usually serotypes
injection, tearing, discharge, crusting of the lashes, and conjuncti- D–K). This condition may be seen in the neonate or sexually active
val edema (ie, chemosis). Pain and decreased vision are uncommon adolescent. Trachoma, the most common cause of impaired vision
symptoms and may signal corneal involvement. and preventable blindness worldwide, is a chronic conjunctivitis usu-
Generally, it is difficult to distinguish bacterial conjunctivitis ally caused by C trachomatis serotypes A, B, and C. Although this
from viral conjunctivitis on clinical features alone. Certain clini- disease is rarely seen in North America, it is endemic among certain
cal characteristics may guide the diagnosis. The average age of chil- populations, especially Native Americans. Inclusion conjunctivitis
dren affected with bacterial conjunctivitis tends to be younger than and endemic trachoma are characterized initially by conjunctivitis
the age of those with viral conjunctivitis, which occurs more fre- with small lymphoid follicles in the conjunctiva.
quently in adolescents; however, considerable overlap occurs. The Neonatal conjunctivitis, or ophthalmia neonatorum, occurs dur-
child with bacterial conjunctivitis typically presents with an acute ing the first month after birth. In decreasing order of frequency, the
onset of unilateral or bilateral injection and edema of the palpebral major causes of neonatal conjunctivitis are chemical, chlamydial,
and bulbar conjunctiva, minimal to copious purulent discharge, and and bacterial. Ophthalmia neonatorum may be produced by the
crusting of the eyelashes. The child may have difficulty opening the same bacteria that cause childhood conjunctivitis but also results
eyes on awaking in the morning because of the exudate. An asso- from organisms such as C trachomatis and Neisseria gonorrhoeae.
ciation between conjunctivitis and concomitant otitis media has The newborn may acquire these latter pathogens following prema-
been well described. Haemophilus influenzae, which is often resis- ture rupture of membranes or passage through an infected or col-
tant to ampicillin, is the pathogen most commonly isolated from onized birth canal. Chlamydia trachomatis is the organism most
affected children. commonly identified. It has been isolated from 17% to 40% of neo-
The diagnosis of viral conjunctivitis is considered if signs of viral nates with conjunctivitis. The neonate born to a mother with active
upper respiratory infection (eg, low-grade fever, cough, rhinorrhea) cervical chlamydial infection has a 20% to 50% chance of devel-
are evident. Viral infection is associated with conjunctival injec- oping chlamydial conjunctivitis. Viruses are uncommon causes of
tion, watery or thin mucoid discharge, and only mild lid edema neonatal ocular infections. Human herpesvirus is the primary viral
and erythema. Adenoviral infection is usually bilateral, with signif- agent involved in neonatal conjunctivitis. The presence of charac-
icant conjunctival injection and chemosis of the conjunctiva, and is teristic vesicular skin lesions or corneal dendritic lesions helps in
often accompanied by a tender preauricular lymph node. Epidemic the diagnosis.

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 670 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Time of onset of symptoms is related to the etiologic agent. edema, proptosis, decreased extraocular movements, and loss of
Inflammation secondary to the silver nitrate drops instilled at birth visual acuity occur. As with periorbital cellulitis, the affected child
to prevent gonococcal infection presents as mild conjunctivitis 12 to is often febrile and ill-appearing. Contiguous spread of infection
24 hours after birth in 10% to 100% of treated newborns. This con- from adjacent sinusitis (most often ethmoid) is the most common
dition usually resolves spontaneously in 24 to 48 hours. This is more cause. The organisms most often involved are the same as those in
of historic interest because erythromycin ointment 0.5% has replaced acute sinusitis (ie, S aureus, S pneumoniae, non-typeable H influ-
silver nitrate in most hospitals. (Silver nitrate was ineffective against enzae). Untreated, the infection may progress to orbital abscess
C trachomatis.) Conjunctivitis resulting from N gonorrhoeae appears 2 formation or progress posteriorly in the orbit to the cavernous
to 5 days after birth and is associated with copious purulent discharge. sinus and brain.
Conjunctivitis caused by C trachomatis occurs at 5 to 14 days, a result Primary human herpesvirus infection can affect the skin sur-
of a longer incubation period. Time of onset and severity of symp- rounding the eyes as well as the eye itself. Most of these infections
toms of these 2 conditions may overlap, however. The presentation are caused by human herpesvirus 1, although human herpesvirus 2
of gonococcal infection may be delayed for 5 days or more because infections may occur in the newborn. The child with herpetic infec-
of the partial suppression of the infection by the prophylactic drops tion of the eye usually presents with unilateral skin vesicles and a
instilled at birth. Chlamydial infection can vary in severity from mild mild conjunctivitis or keratitis. Herpetic keratoconjunctivitis can
erythema of the eyelids to severe inflammation and copious purulent recur after fever, exposure to sunlight, or mild trauma. The charac-
discharge. Chlamydial infection is primarily localized to the palpebral teristic corneal lesion of herpetic keratitis is the dendritic corneal
conjunctiva and only rarely affects the cornea. Gonococcal conjuncti- ulcer, which appears as a tree branch pattern on fluorescein stain-
vitis is considered a medical emergency because the gonococcus can ing of the cornea. Although this lesion may occur with primary
penetrate the cornea, resulting in corneal ulceration and perforation infection, it is more common in recurrent infections. Skin vesi-
of the globe within 24 hours if untreated. cles may not appear with a recurrence, which makes it difficult to
Concomitant nasopharyngeal chlamydial infection is com- distinguish herpetic infection from other causes of conjunctivitis.
mon. Spread of the organism from the nasopharynx to the lungs Steroids may cause progression of the herpetic infection and per-
is a sequela of colonization. Ten percent to 20% of newborns and manent corneal scarring as well as cataracts and glaucoma. Empiric
infants with conjunctivitis have chlamydial pneumonia. It may occur topical steroid treatment for presumed viral conjunctivitis should be
simultaneously with the conjunctivitis or up to 4 to 6 weeks later. avoided for this reason. Neonatal herpetic infections of the eye pri-
The affected newborn or infant usually is afebrile and presents with marily result from human herpesvirus 2. Infections may be isolated
symptoms of increasing tachypnea and cough. to the eye, or the eye may be infected secondarily resulting from
Anterior uveitis may be confused with conjunctivitis. The uvea central nervous system or disseminated disease. Proper diagnosis
consists of the iris, ciliary body, retina, and choroid. Inflammation is important because disseminated herpetic disease has a mortal-
of the iris or ciliary body may produce conjunctival injection, ity rate of approximately 85%, and central nervous system disease
which may be associated with decreased visual acuity, pain, head- has a mortality rate of 50%. Isolated herpetic eye disease is quite
ache, and photophobia. Systemic conditions associated with uveitis rare in neonates.
include Kawasaki disease, juvenile idiopathic arthritis, Lyme disease,
tuberculosis, sarcoidosis, Toxocara infection, toxoplasmosis, and Evaluation
spondyloarthropathies. History
A careful history taken from the parent or primary caregiver
Infections of the Eye and Surrounding as well as the child can guide the diagnosis (Box 92.3). It is
Tissues important to exclude the possibility of ocular trauma or expo-
Preseptal cellulitis and orbital cellulitis are 2 serious infections of the sure to noxious chemicals when evaluating the child with red,
eyelids and surrounding structures. Although these infections are irritated eyes.
not as frequent as those that are limited to the eye, they have serious
sequelae. The preseptal space is defined by the skin of the eyelid on Physical Examination
one side and the orbital septum on the other. The child with presep- A thorough examination of the eyes should be performed. The eye-
tal cellulitis, or periorbital cellulitis, usually presents with acute onset, lids, conjunctiva, and cornea should be inspected for evidence of
unilateral upper and lower eyelid edema, erythema, and pain. The con- inflammation or foreign bodies. The presence of any discharge or
dition is often associated with systemic signs and symptoms, such as crusting of the eyelids as well as light sensitivity or pain should
ill appearance, fever, and leukocytosis. The eye itself usually appears be noted. Extraocular movements should be checked, and their
normal. Infection may follow hematogenous seeding of the preseptal symmetry should be noted. Visual acuity should be determined,
space, most often with H influenzae type b or S pneumoniae, or after and an ophthalmoscopic examination of the retina should be
traumatic breaks in the skin that usually result in S aureus infection. performed whenever possible. A slit-lamp examination of the eye
Orbital cellulitis is an infection of the contents of the orbit pos- is indicated if uveitis is suspected. Additionally, it is important
terior to the orbital septum. Usually an insidious onset of eyelid to perform a thorough head and neck examination, noting the

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 Chapter 92: Infections of the Eye 671

Box 92.3. What to Ask Management

Most common eye infections resolve spontaneously or respond read-
Eye Infections
ily to hygiene and topical antibiotics. Treatment of staphylococcal and
ww How long has the child had symptoms?
seborrheic blepharitis consists of daily lid hygiene (usually at bedtime),
ww Is the child having any difficulty seeing clearly?
including application of a warm compress and removal of the scales
ww Is the child reporting light sensitivity?
and crusts with a moistened, warm washcloth. The eyelashes and lid
ww Has the child had fever, cold symptoms, or purulent nasal discharge
margins may also be scrubbed with a washcloth soaked in a 50:50
(eg, green, yellow)?
mixture of baby shampoo and water. When staphylococcal blephari-
ww Are the eyes pruritic (ie, itchy) or painful?
tis is present, an ointment containing an anti-staphylococcal antibi-
ww Does the child have difficulty opening the affected eye on awaking in
otic agent, such as erythromycin, may be applied to the eyelids after
the morning or after naps?
cleansing. Treatment of parasitic blepharitis consists of application of
ww Has the parent or guardian noticed any discharge from the eyes or
petrolatum ophthalmic ointment (to smother the nits) several times
crusting around the eyelids?
a day for 1 week, followed by removal of the remaining parasites and
ww Is the child reporting earache or sore throat?
their ova with tweezers or forceps.
ww Does the child have allergies, asthma, or hay fever?
Treatment for hordeola consists of application of hot compresses
ww Has any trauma or bug bites occurred?
several times a day. This usually causes the abscess to come to a
point and drain. Occasionally, surgical excision and drainage may
be required if the abscess does not resolve. Unlike the hordeolum, a
presence of associated sinusitis, otitis media, pharyngitis, or pre- chalazion generally requires surgical excision because spontaneous
auricular nodes. resolution is uncommon.
Most cases of acute childhood conjunctivitis can be managed
Laboratory Tests successfully by the primary care physician. Acute conjunctivitis in
Laboratory assessment is guided by the history and physical exam- childhood is generally a self-limited disease. However, antibiotic
ination. Although clinical differentiation between a bacterial and treatment of bacterial conjunctivitis hastens recovery and may help
viral etiology is difficult, cultures usually are not required because prevent secondary cases by eradicating the bacterial pathogen. It
acute conjunctivitis in children is a self-limited disease. In neonatal is helpful to determine clinically whether the infection is bacterial
conjunctivitis, however, the time of onset of illness and clinical find- and initiate empiric treatment with topical antibiotic preparations.
ings overlap, and Gram stain and cultures are essential. Gonococcal Trimethoprim and polymyxin B sulfate (eg, Polytrim), sodium
infection is assessed by Gram stain and culture. Treatment may be sulfacetamide (eg, Bleph-10, Sodium Sulamyd), gentamicin (eg,
initiated on the basis of Gram stain alone because of the serious Garamycin, Gentak), tobramycin (eg, Tobrex), and moxifloxacin
potential for corneal involvement and subsequent loss of visual (eg, Vigamox) are some of the commonly prescribed antibiotics.
acuity. Trimethoprim and polymyxin B sulfate as well as gentamicin
If human herpesvirus is suspected, viral cultures should be remain highly effective against MRSA. Neomycin-containing
obtained. If chlamydial infection is suspected, a nasopharyngeal cul- products should be avoided, because sensitivity to neomycin
ture should be sent in addition to conjunctival scraping. Purulent occurs frequently. Antibiotic ointments may be easier to instill
material may be examined for gonococci, but conjunctival scrapings in the infant or young child. Antibiotic drops should be used in
are required for chlamydia because chlamydia is an obligate intracellu- the older child during the day because the ointments can blur
lar organism. Laboratory assessment of periorbital cellulitis includes a the vision; ointments may be used at bedtime. Systemic ther-
complete blood cell count, blood culture, and a lumbar puncture in the apy can be considered for concomitant conjunctivitis and
young infant or child with signs of meningeal irritation. The reported otitis media. Good hygiene with special attention to handwash-
prevalence of meningitis is 1% in children with periorbital cellulitis ing is important to prevent the spread of the infection to the
and 10% in children with bacteremia and periorbital cellulitis. As with other eye as well as family and friends. Even if the child is on
periorbital cellulitis, laboratory assessment of orbital cellulitis includes antibiotic drops, the child should not go to school until the dis-
a complete blood cell count and blood culture. charge and drainage have resolved. If symptoms persist for more
than 7 to 10 days or if the diagnosis is in question, appropriate
Imaging Studies cultures should be taken. Immediate referral to an ophthalmologist
Imaging studies are required infrequently in the assessment of is required for complicated cases, such as suspected herpetic, gono-
eye infections. Chest radiography to detect pneumonia should be coccal, or MRSA infection; foreign bodies that cannot be removed
obtained if the infant with neonatal conjunctivitis has respiratory easily; loss of visual acuity; presence of significant pain; those
symptoms. Characteristic features on chest radiography include involving a history of recent penetrating ocular trauma or surgery;
hyperinflation and diffuse or patchy interstitial infiltrates. In orbital and those involving contact lens use. Contact lens wearers with red
cellulitis, computed tomography of the orbit and sinuses may be use- eyes are at high risk for corneal ulcers. They should be instructed
ful for assessing the degree of involvement. to leave their lenses out until seen by the ophthalmologist.

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 672 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Allergic conjunctivitis can be managed with cool compresses. considered a true ophthalmologic emergency because the potential
Topical decongestant or antihistamine drops drops may provide for complications is high. The optic nerve may become involved,
symptomatic relief if treatment is indicated. Vernal conjunctivitis resulting in loss of vision or spread of the infection into the cra-
may be managed with topical cromolyn sodium drops or medica- nial cavity. This spreading may result in meningitis, cavernous sinus
tions designed to relieve redness and itching and stabilize mast cells, thrombosis, or brain abscess.
such as olopatadine hydrochloride (eg, Patanol, Pataday) or ketorolac
tromethamine (eg, Toradol, Acular). Caution should be used when
prescribing corticosteroid preparations for the eye because they may
cause progression of an undiagnosed herpetic eye infection. Chronic
CASE RESOLUTION
The newborn has neonatal conjunctivitis. A Gram stain of the purulent discharge
use of topical steroids can cause cataracts and glaucoma.
should be examined, and cultures should be taken from the eye and nasopharynx.
Management of neonatal conjunctivitis depends on the diag- If the Gram stain result is negative for gonococci, empiric treatment for chlamydia
nosis. If gonococcal infection is suspected and Gram stain result is may begin with oral erythromycin.
positive for gram-negative diplococci, immediate parenteral ther-
apy with ceftriaxone should be initiated. Chlamydial conjunctivi-
tis should be managed with systemic rather than topical treatment
to prevent systemic disease. Oral erythromycin is the drug of Selected References
choice. Although oral treatment provides adequate local antibiotic Amato M, Pershing S, Walvick M, Tanaka S. Trends in ophthalmic manifestations
levels, topical erythromycin ointment may be used in conjunction of methicillin-resistant Staphylococcus aureus (MRSA) in a northern California
with systemic therapy to provide more prompt relief of ophthalmic pediatric population. J AAPOS. 2013;17(3):243–247 PMID: 23623773 https://
symptoms. The parent(s) or guardian(s) should also be treated. doi.org/10.1016/j.jaapos.2012.12.151
Empiric parenteral antibiotic therapy (eg, cefuroxime) should Gold RS. Treatment of bacterial conjunctivitis in children. Pediatr Ann. 2011;40(2):
be initiated for periorbital cellulitis. Repeat evaluations for signs of 95–105 PMID: 21323206 https://doi.org/10.3928/00904481-20110117-09
progression should be performed frequently during the initial 24 to Golde KT, Gardiner MF. Bacterial conjunctivitis in children: a current review
48 hours. If orbital cellulitis is suspected, an ophthalmologist should of pathogens and treatment. Int Ophthalmol Clin. 2011;51(4):85–92 PMID:
21897142 https://doi.org/10.1097/IIO.0b013e31822d66a1
be consulted, and hospitalization and systemic antibiotics should be
instituted. Surgical drainage of the sinuses or an orbital abscess is Liesegang T, Skuta G, Cantor L. Infectious and allergic ocular diseases. J Pediatr
Ophthalmol Strabismus. 2006;17:215–238
sometimes necessary.
The child with suspected herpetic infection should be referred to Liu S, Pavan-Langston D, Colby KA. Pediatric herpes simplex of the ante-
rior segment: characteristics, treatment, and outcomes. Ophthalmology.
an ophthalmologist. Intravenous acyclovir is often recommended for
2012;119(10):2003–2008 PMID: 22796308 https://doi.org/10.1016/j.
the management of isolated herpetic eye infections in the neonate.
ophtha.2012.05.008
Nageswaran S, Woods CR, Benjamin DK Jr, Givner LB, Shetty AK. Orbital cel-
Prognosis lulitis in children. Pediatr Infect Dis J. 2006;25(8):695–699 PMID: 16874168
https://doi.org/10.1097/01.inf.0000227820.36036.f1
Most common eye infections, such as blepharitis, hordeolum, and
acute childhood conjunctivitis, resolve without sequelae. Recurrence Ohnsman CM. Exclusion of students with conjunctivitis from school: policies
of state departments of health. J Pediatr Ophthalmol Strabismus. 2007;44(2):
is common for hordeola, and periorbital cellulitis may be a potential
101–105 PMID: 17410961
complication in rare or untreated cases. Unlike acute conjunctivitis,
Patel PB, Diaz MC, Bennett JE, Attia MW. Clinical features of bacterial conjunc-
chronic conjunctivitis may not be self-limited. Appropriate diagnosis
tivitis in children. Acad Emerg Med. 2007;14(1):1–5 PMID: 17119185 https://
and management are extremely important to prevent serious sequelae doi.org/10.1197/j.aem.2006.08.006
in some children. For example, endemic trachoma may progress to
Pichichero ME. Bacterial conjunctivitis in children: antibacterial treatment
produce conjunctival scarring, pannus formation, and even blind- options in an era of increasing drug resistance. Clin Pediatr (Phila). 2011;50(1):
ness if not appropriately managed with systemic erythromycin or 7–13 PMID: 20724317 https://doi.org/10.1177/0009922810379045
tetracycline. (Generally, systemic tetracycline should not be used in Rimon A, Hoffer V, Prais D, Harel L, Amir J. Periorbital cellulitis in the era of
the child younger than 8 years to avoid discoloration of the teeth.) Haemophilus influenzae type B vaccine: predisposing factors and etiologic agents
Periorbital cellulitis generally resolves without sequelae if treated in hospitalized children. J Pediatr Ophthalmol Strabismus. 2008;45(5):300–304
promptly with systemic antibiotics. Orbital cellulitis should be PMID: 18825903 https://doi.org/10.3928/01913913-20080901-14

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 CHAPTER 93

Excessive Tearing
Teresa O. Rosales, MD

CASE STUDY
A 4-week-old girl has had a persistent watery discharge Questions
from both eyes since birth. Her mother has noticed 1. What is the differential diagnosis of excessive tear-
white, crusty material on her daughter’s eyelids for the ing in infancy?
past few days. The infant’s birth and medical history are 2. How do physical findings such as corneal enlargement
unremarkable. Examination of the eyes, including bilat- and haziness influence the differential diagnosis?
eral red reflexes and symmetric extraocular movements, 3. How should excessive tearing in infants be managed?
is normal, except that the left eye appears “wetter” than 4. When should a child with excessive tearing be
the right. referred to an ophthalmologist?

Excessive tearing or epiphora in 1 or both eyes in infants or young Most commonly, a persistent, thin membrane (Hasner membrane)
children is a common pediatric ophthalmologic concern. The pedia- obstructs the opening of the sac in the nose. Typically, the mem-
trician must be capable of differentiating benign causes of this com- brane is located in the distal or nasal segment of the duct rather
mon childhood condition from more serious illnesses (eg, glaucoma) than the proximal portion. The term dacryocystitis is used in cases
that have the potential to threaten vision. in which acute infection or inflammation is associated with the
obstruction. If the canaliculi and NLD are obstructed, a dacryocys-
Obstruction of the Nasolacrimal Duct tocele involving the nasolacrimal sac may be noted at birth. This
sac appears as a bluish, firm mass located over the lacrimal sac.
Epidemiology
Atresia of some portion of the drainage system is an extremely rare
Dacryostenosis, that is, congenital obstruction of the nasolacrimal occurrence. Infants with dacryocystocele, who have large intrana-
duct (NLD), occurs in 1% to 6% of newborns and infants and is the sal cysts, may present with respiratory symptoms because infants
most common cause of excessive tearing in infancy. Eighty percent are obligate nasal breathers. Symptoms range from difficulty
of cases of dacryostenosis resolve spontaneously by 6 months of age. during feeding (caused by obstruction of the mouth) to respiratory
Clinical Presentation distress.

Infants with dacryostenosis typically present with a history of

Differential Diagnosis
a mucoid discharge and crusting along the eyelid margins. The
affected eye appears “wetter” than the normal eye, and a small pool Box 93.2 outlines the differential diagnosis of excessive tearing in
of tears may be noted along the lower eyelid. Frequent tearing is infancy. Although obstruction of the NLD is the most common
reported. Commonly, the patient has repeated episodes of infection cause of excessive tearing in newborns and infants, it is important
with purulent discharge (Box 93.1). to consider and rule out glaucoma when evaluating neonates and
infants with excessive tearing. Infantile glaucoma may be unilat-
Pathophysiology eral or bilateral.
The lacrimal system produces and drains tears away from the Acute onset of excessive tearing in older children is usually the
eyes and into the nose (Figure 93.1). Typically, reflex tearing is result of ocular irritation. Any irritation of the conjunctiva, cornea,
present shortly after birth; however, it may be delayed for sev- or eyelids may produce tearing. Conjunctivitis and corneal abrasion
eral weeks to months until the lacrimal gland begins to function. (secondary to a foreign body in the eye or human herpesvirus
Tears drain away from the eyes through the superior and infe- keratoconjunctivitis) are the 2 most common causes. Eye infections
rior puncta into the superior and inferior canaliculi and finally are discussed in Chapter 92.
into the NLD, which drains beneath the inferior turbinate into
Evaluation
the nose.
Outflow obstruction, which typically results from dacryosteno- History
sis, is the most common cause of excessive tearing in infancy. The Evaluation of excessive tearing begins with a thorough patient history.
obstruction is usually bilateral and occurs during fetal development. The nature of any discharge (eg, watery, mucoid, purulent) is noted.
673

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 674 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Parents or other caregivers should be questioned about the appear-

Box 93.1. Diagnosis of Excessive Tearing
ance of the infant’s eyes (Box 93.3). The excessive mucoid discharge
ww Conjunctival edema or injection in the medial canthal region and on the eyelashes is noticeable to
ww Crusting of the eyelids the family, as is the increased tearing. Crusting along the eyelashes
ww Rhinorrhea caused by drying of the mucoid material is usually noted when infants
ww Photophobia awake in the morning or after a nap. Mucopurulent discharge may be
ww Corneal haziness noted if an associated acute infection is present (eg, dacryocystitis).
ww Reflux of tears with gentle pressure on the medial canthus
Physical Examination
ww Wetness of the eye
A careful examination of the eyes in infants includes inspection,
evaluation of extraocular movements, and funduscopic examination.
While funduscopic examination may be difficult in this age group,
it is easy to elicit bilateral red reflexes. Signs of dacryostenosis,
which usually appear days to weeks after birth, include tearing,
Puncta mucoid discharge, and crusting of the eyelids. Tearing may be as
mild as an increased wetness of the affected eye; this is best eval-
Canaliculi uated prior to disturbing the infant. Visible overflow of tears from
Nasolacrimal sac the affected eye is not unusual and is typically seen when the eye
is irritated (eg, from cold or wind). Infants with dacryosteno-
sis may present with associated erythema and edema of the eye-
lids as well. Dacryocystitis may also spread to the surrounding
Lacrimal tissues, producing a periorbital cellulitis; systemic signs of infec-
gland
Nasolacrimal tion, such as fever, may be noted. Gentle pressure along the medial
duct canthal region over the lacrimal sac may produce a reflux of tears or
Inferior mucoid discharge onto the surface of the eye, thus confirming the
turbinate diagnosis of obstruction.
Laboratory Tests
Typically, routine laboratory assessment is not necessary.

Management
Figure 93.1. The lacrimal system, showing massage of the lacrimal sac Early treatment of dacryocystoceles before 13 months of age is
(ie, Crigler massage). advised to prevent complications related to infection or respiratory
distress. Digital massage may be attempted to decompress the dac-
ryocystocele; occasionally, the condition resolves without surgery.
Dacryocystoceles associated with acute respiratory distress require
immediate surgical intervention.
Box 93.2. Differential Diagnosis of Excessive Nasolacrimal duct probing alone may be curative; however, in
Tearing in Newborns and Infants approximately 25% of patients the condition persists after probing.
Increased Production
ww Infantile glaucoma
ww Allergy Box 93.3. What to Ask
ww Conjunctivitis Excessive Tearing
ww Corneal abrasion ww How old was the infant when the excessive tearing began?
ww Foreign body under the eyelid ww Does the condition affect 1 eye or both eyes?
Outflow Obstruction ww How does the eye appear? How has its appearance changed?
ww Obstruction of the nasolacrimal duct (ie, dacryostenosis) ww Is there a family history of infantile glaucoma?
ww Anomalies of the lacrimal drainage system ww Does the infant have photophobia or light sensitivity (eg, closes eyes in
ww Mucocele of the lacrimal sac bright sunlight)?
ww Atresia of the lacrimal punctum or canaliculus ww Has the infant had any persistent, watery discharge?
ww Nasal congestion ww Does the infant have difficulty opening the affected eye on awaking in
ww Craniofacial anomalies involving the midface the morning or after a nap?

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 Chapter 93: Excessive Tearing 675

Nasolacrimal duct probing in conjunction with nasal endoscopy Clinical Presentation

and intranasal cyst removal is effective in more than 95% of infants. In addition to excessive tearing, other clinical signs of infantile glau-
Spontaneous resolution of dacryostenosis is common by 6 to coma include blepharospasm (ie, spasmodic blinking of the eyelids),
8 months of age. The primary medical treatment of uncomplicated photophobia, corneal enlargement and corneal haziness resulting
NLD obstruction consists of local massage and cleansing, beginning from corneal edema, progressive enlargement of the eye (ie, buph-
when symptoms are noted. Parents are instructed to massage the thalmos), and cupping and atrophy of the optic nerve (Box 93.1).
NLD several times a day by applying firm, downward pressure over
the medial canthal region with a finger and sliding the finger down Pathophysiology
toward the mouth (ie, Crigler massage; Figure 93.1). This maneuver Glaucoma is the most serious and potentially sight-threatening etiol-
is done in an attempt to move fluid trapped within the nasolacrimal ogy for excessive production of tears. Glaucoma refers to an increase
sac down through the duct to break the obstruction with hydrostatic in intraocular pressure that is severe enough to damage the eye and
pressure. Parents may be asked to cleanse the eyes with warm water alter vision. In infants and children, glaucoma usually is a result of
before performing this maneuver. Antibiotic ointments and drops a developmental abnormality of the iridocorneal angle, which inter-
should be prescribed only if the discharge is purulent or associated feres with drainage of aqueous humor from the anterior chamber.
conjunctivitis is evident. Young infants differ from adults in that the globe in infants’ eyes is
Controversy exists about when probing of the NLD should be distensible; the increased intraocular pressure not only produces
performed. Some ophthalmologists prefer probing the duct before corneal enlargement and edema but also expands the globe itself.
infants are 6 months of age, because before this age the procedure Breakdown of the corneal epithelium and resultant irritation of the
may be performed in the office without general anesthesia. Other eyes produce reflex tearing and photophobia. Untreated glaucoma
ophthalmologists state that more than 80% of obstructions resolve may result in optic nerve damage with resultant loss of visual acu-
with conservative medical management by 6 months of age and pre- ity, decreased visual field, and even blindness. Glaucoma in chil-
fer to delay probing until 9 to 12 months of age. At this age, how- dren younger than 3 years of age is referred to broadly as infantile
ever, the procedure must be performed under general anesthesia. glaucoma, and glaucoma that occurs in children between 3 and
Deciding when to proceed with probing depends on the standard of 20 years of age is called juvenile glaucoma. Infantile glaucoma is
practice within the community, severity of symptoms, response to further classified as primary or secondary. Primary glaucoma refers
medical management, and level of parental concern. to isolated abnormalities of the iridocorneal angle, whereas secondary
Ophthalmologic referral is necessary for affected infants by glaucoma may be associated with other ocular or systemic diseases.
6 months of age if the obstruction has not resolved. Referral should Juvenile glaucoma is a form of open-angle glaucoma.
be made sooner if symptoms are severe or infections recur.
Differential Diagnosis
Prognosis
Excessive tearing may be caused by increased production or out-
Most cases of dacryostenosis resolve spontaneously by age 1 year flow obstruction (see Box 93.2). Infants with increased production
without further sequelae. Tear duct probing, should it be necessary, of tears demonstrate rhinorrhea in association with epiphora, a find-
is 90% to 95% successful if performed before 13 months of age. The ing that distinguishes glaucoma from dacryostenosis. Rhinorrhea is
success rate drops to 70% between 13 and 15 months of age and to not associated with dacryostenosis because the NLD is obstructed
50% in those older than 18 months. and no tears drain out the nose. Ocular irritation, which occurs with
conjunctivitis, corneal abrasion, or a foreign body under the eye-
Congenital Glaucoma lid, also can produce excessive tearing, especially in older children.
Epidemiology In addition, allergies are associated with excessive tearing resulting
Congenital or infantile glaucoma, which is a serious cause of exces- from overproduction.
sive tearing, is rare, with a prevalence of approximately 1 in 10,000 Disorders associated with outflow obstruction include dacryoste-
live births. Although glaucoma may be present at birth, onset dur- nosis, anomalies of the lacrimal drainage system (eg, dacryocystocele
ing the first few weeks to months after birth is more common. of the lacrimal sac, atresia of the lacrimal punctum or canaliculus),
Approximately 25% of cases are diagnosed at birth, and 80% of cases nasal congestion, and craniofacial anomalies involving the midface.
are diagnosed by 1 year of age. The severity of the disease is worse
with earlier onset. Most affected infants are male; the male to female Evaluation
ratio in older infants is 2:1. Infantile glaucoma seems to have a mul- A corneal diameter greater than 12 mm in infants is suggestive of
tifactorial inheritance pattern, but most cases are sporadic. In fami- infantile glaucoma. Corneal edema is more common in newborns
lies with 1 affected infant or a parental history of infantile glaucoma, and in infants younger than 3 months and may cause the red reflex
the chance of having an infant with glaucoma is approximately 5%. to appear dull in the affected eye. The optic cup may be enlarged.
Although glaucoma may be unilateral or bilateral, 75% to 90% of A cup to disk ratio greater than 0.3 or an asymmetry of the ratio
infants who present before 3 months of age have bilateral glaucoma. between the eyes may be indicative of glaucoma.

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 676 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

As in adults, loss of visual fields occurs in children with glau- Selected References
coma. Visual fields are difficult to evaluate in infants and young chil-
Al-Faky YH, Al-Sobaie N, Mousa A, et al. Evaluation of treatment modalities
dren because of their inability to cooperate with the examination.
and prognostic factors in children with congenital nasolacrimal duct obstruc-
Management tion. J AAPOS. 2012;16(1):53–57 PMID: 22370666 https://doi.org/10.1016/j.
jaapos.2011.07.020
Suspected cases of glaucoma should be referred to an ophthalmolo-
Becker BB. The treatment of congenital dacryocystocele. Am J Ophthalmol.
gist immediately for further evaluation, including measurement of
2006;142(5):835–838 PMID: 16989760 https://doi.org/10.1016/j.ajo.2006.
intraocular pressure. Pressures greater than 20 mm Hg are sugges- 05.043
tive of glaucoma. (In persons of any age, normal intraocular pressure
Guez A, Dureau P. Diagnosis and treatment of tearing in infancy [in French].
is 10 to 20 mm Hg.) Arch Pediatr. 2009;16(5):496–499 PMID: 19324537 https://doi.org/10.1016/j.
Surgery is the primary treatment for glaucoma. The goal is to arcped.2009.02.011
normalize intraocular pressure and minimize irreversible damage Olitsky S, Medow N, Rogers G. Diagnosis and treatment of congenital naso-
to the cornea and optic nerve. lacrimal duct obstruction. J Pediatr Ophthalmol Strabismus. 2007;44(2):80–83
PMID: 17410956
Prognosis
Pediatric Eye Disease Investigator Group. Resolution of congenital naso-
Left untreated, infantile glaucoma may progress to blindness. The lacrimal duct obstruction with nonsurgical management. Arch Ophthalmol.
visual prognosis depends on several factors, including age at onset, 2012;130(6):730–734 PMID: 22801833
with the earlier the onset, the worse the prognosis; the amount of Repka MX, Chandler DL, Beck RW, et al; Pediatric Eye Investigator Group.
optic nerve damage; and the degree of myopia caused by the enlarge- Primary treatment of nasolacrimal duct obstruction with probing in chil-
ment of the eye. In addition, amblyopia secondary to deprivation dren younger than 4 years. Ophthalmology. 2008;115(3):577.e3–584.e3 PMID:
resulting from corneal opacities or unequal refractive errors is often 17996306 https://doi.org/10.1016/j.ophtha.2007.07.030
seen (see Chapter 91). Silbert DI, Matta N. Congenital Nasolacrimal Duct Obstruction. San Francisco,
CA: American Academy of Ophthalmology; 2016. Focal Points: Clinical Practice
Perspectives Module 6
CASE RESOLUTION Stamper RL, Lieberman MF, Becker B, Drake MV. Becker-Shaffer’s Diagnosis and
The infant has dacryostenosis. At this stage, it can be managed with medical Therapy of the Glaucomas. 7th ed. St Louis, MO: Mosby; 1999
treatment, such as local massage and cleansing. If her symptoms persist beyond Walton DS, Katsavounidou G. Newborn primary congenital glaucoma:
age 6 months, consultation with an ophthalmologist is recommended. 2005 update. J Pediatr Ophthalmol Strabismus. 2005;42(6):333–341 PMID:
16382557

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 CHAPTER 94

Neck Masses
Casey Buitenhuys, MD, FACEP, and Stanley H. Inkelis, MD, FAAP

CASE STUDY
A 2-year-old boy is brought to the office with a 1-day submandibular neck mass that is erythematous, warm,
history of an enlarging red, tender “bump” beneath and tender to palpation.
his right mandible. He has a fever (temperature 38.7°C
[101.6°F]) and sores around his nose, upper lip, and cheek.
Questions
1. What are the common causes of neck masses in
These sores have been present for 3 days and have not
children?
responded to an over-the-counter antibiotic ointment.
2. What steps are involved in the evaluation of the
He had an upper respiratory tract infection 1 week previ-
child with a neck mass?
ously, which has almost entirely resolved. He is otherwise
3. What clinical findings suggest that neck masses are
in good health. The family has no history of tuberculosis
neoplasms? When should neck masses be biopsied
or recent travel, and the child has not been playing with
or removed?
cats or other animals.
4. What is involved in the treatment of the different
The physical examination is completely normal
types of neck masses in children?
except for fever, mild rhinorrhea, honey-crusted lesions
5. When should the child with a neck mass be referred
on the nares and upper lip, and a 4- × 5-cm, right
for further consultation?

A neck mass is any swelling or enlargement of the structures in the The epidemiology of neck masses of infectious origin depends
area between the inferior mandible and the clavicle. Normal vari- on the infectious agent itself, geographic location of the child, and
ants, such as the angle of the mandible or tip of the mastoid bone, the child’s immediate environment. Neck masses of viral origin may
may occasionally appear as swellings, and the parent or guardian be related to focal infection of the oropharynx or respiratory tract
sometimes confuses these with neck masses. If the swelling is not a but often are associated with generalized adenopathy. Neck masses
normal structure, a well-directed history and physical examination of bacterial origin typically occur from normal bacterial flora of the
usually determine the etiology. nose, mouth, pharynx, and skin that secondarily spreads to lymph
Lymphadenopathy from viral or bacterial throat infections is the nodes. These organisms are not usually transmitted from person
most common cause of neck masses in children. Therefore, neck masses to person. Pathologic flora, such as group A streptococcus and
are common because children frequently have sore throats. Most par- Mycobacterium tuberculosis, that result in neck masses can spread
ents and guardians know about swollen lymph glands, and they usu- by human-to-human contact, however. Additionally, cat-scratch
ally do not seek medical advice unless the glands become quite large or disease is caused by Bartonella henselae, a vector-borne pathogen.
do not recede in a few days. Neck masses in children may have many
other causes besides lymphadenopathy. Most of these masses may be Clinical Presentation
categorized as inflammatory, neoplastic, traumatic, or congenital in Children with neck masses present in a variety of ways depending
origin. A well-described mnemonic in the adult literature, KITTENS on the etiology of the mass. Typically, a swelling or enlargement in
(congenital/developmental anomalies, infectious/inflammatory, the neck, which a parent or guardian often notices more than the
trauma, toxic, endocrine, neoplasms, systemic disease), can summate child, is evident. Associated signs and symptoms include fever, upper
many of the causes of neck masses in children as well (Box 94.1). respiratory tract infection, sore throat, ear pain, pain or tenderness
over the mass, changes in skin color over the mass, skin lesions
Epidemiology of the head or neck, and dental caries or infections (Box 94.2).
Most neck masses are benign. Almost 50% of all children 2 years Malignant tumors are usually slow-growing, firm, fixed, nontender
of age and up to 90% of children between 4 and 8 years of age have masses. Congenital neck masses and benign tumors, which have fre-
palpable cervical lymph nodes. Although more than 25% of malig- quently been present since birth or early infancy, are soft, smooth,
nant tumors in children are found in the head and neck region (this and cyst-like and may be recurrent. Neck masses associated with
is the primary site in only 5%), less than 2% of suspicious head and trauma are often rapidly evolving and may result in airway obstruc-
neck masses are malignant. tion. Temporal development of neck masses is a helpful predictor

677

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 678 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Box 94.1. KITTENS Mnemonic for Neck Masses Box 94.2. Diagnosis of Neck Masses
in the Pediatric Patient
K Congenital/Developmental anomalies
Thyroglossal duct cyst Inflammatory/Infectious
Branchial cleft cyst ww Swelling or enlargement in the neck
Dermoid cyst ww Fever
Vascular malformation ww Sore throat, dental infection, skin infection of head or neck
I Infectious/Inflammatory ww Pain or tenderness over the mass (usually)
Lymphadenitis/cervical adenopathy Neoplastic
Viral adenitis (multiple causes) ww Slowly enlarging mass
Bacterial adenitis (multiple causes) ww Unilateral, discrete
Retropharyngeal/parapharyngeal abscesses ww Firm or rubbery
T Trauma ww Fixed to tissue
Hematoma ww Deep within the fascia
Pseudoaneurysm ww Nontender (usually)
Laryngocele Traumatic
T Toxic ww Rapidly enlarging mass
Thyroid toxicosis ww Hematoma
Medications (eg, carbamazepine) ww Acute airway obstruction
E Endocrine Congenital
Thyroid neoplasms ww Enlargement in neck (usually present since birth or soon after)
Parathyroid neoplasms ww Soft, smooth, cyst-like
Thyroiditis ww Nontender (unless infected)
Goiter ww Recurrent
N Neoplasms
Hemangioma
Lipoma
Table 94.1. Rule of 7 for the Differential Diagnosis
Salivary gland
of Neck Masses
Parapharyngeal space
Lymphoma Mass Duration Likely Mass Etiology
S Systemic disease 7 minutes Trauma
Sarcoidosis 7 days Inflammation/infection
Sjögren syndrome 7 months Neoplastic
Kimura disease 7 years Congenital
Histiocytic necrotizing lymphadenitis (Kikuchi disease)
Adapted with permission from Skandalakis JE. Neck. In: Skandalakis LJ, Skandalakis JE,
Castleman disease
Skandalakis PN, eds. Surgical Anatomy and Technique: A Pocket Manual. 3rd ed. New York, NY:
Kawasaki disease Springer; 2009:17–91.
AIDS
apparent because of fluid collection or infection of the defect. The
parotid gland may be enlarged from inflammation (eg, blocked sali-
of mass etiology. The “rule of 7” proposed by Skandalakis may be
vary gland duct), infection (eg, mumps), or tumor (eg, pleomorphic
applied and adapted to pediatric neck masses (Table 94.1).
adenoma), but the swelling primarily involves the face rather than
Pathophysiology the neck and obscures the angle of the jaw. Other salivary glands
The pathophysiology of neck masses in children is dependent on may be infected or obstructed and may cause submandibular swell-
etiology. Most neck masses are related to inflammation or infec- ing, erythema, and tenderness.
tion of lymph nodes. Enlargement of lymph nodes usually results
from proliferation of intrinsic lymphocytes or macrophages already Differential Diagnosis
present in the lymph node (eg, lymphadenopathy caused by a viral Neck masses in children are usually the result of inflammation or
infection) or from infiltration of extrinsic cells (eg, lymphadenitis, infection of lymph nodes, tumors of lymph nodes and other neck
metastatic tumor). Neck masses from trauma occur from leakage of structures, trauma, and congenital lesions. The location of the mass
fluid into the neck, and congenital anatomic abnormalities become is often a clue to its etiology (Figure 94.1).

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 Chapter 94: Neck Masses 679

Cat-scratch disease, toxoplasmosis, fungal infection, and mycobac-

terial and atypical mycobacterial infections may be associated with
lymphadenopathy that may later progress to lymphadenitis. Viral
illnesses, including Epstein-Barr virus (ie, mononucleosis); adenovi-
rus; enterovirus; human herpesvirus, 1, 2, and 6; and cytomegalovi-
rus commonly cause lymphadenopathy and lymphadenitis.
Lymphadenitis is infection or inflammation of the lymph node
1 that occurs when microorganisms and neutrophils infiltrate the
2
node, resulting in necrosis and abscess formation. This condition
5
usually is associated with proximal bacterial infection that drains
4 to the affected nodes by connecting afferent lymphatic channels.
3 7
8 Lymphadenitis often is a progression of disease, resulting in enlarged
6 nodes that measure 2 to 6 cm. These nodes, which are typical of bac-
9
10 terial disease, are often termed “hot nodes,” and they are erythem-
11 atous, warm, tender, and sometimes fluctuant. Subacute or chronic
inflammation of lymph nodes typical in illnesses such as cat-scratch
disease, mycobacterial infection, or toxoplasmosis are not warm
Figure 94.1. Differential diagnosis of neck mass by location. 1, Parotid—cystic
to the touch and are usually not as tender. They are typically not
hygroma, hemangioma, lymphadenitis, parotitis, Sjögren syndrome, infantile
suppurative and may be difficult to distinguish from nodes that are
cortical hyperostosis (Caffey-Silverman syndrome), lymphoma.
simply enlarged (Figure 94.2).
2, Postauricular—lymphadenitis, branchial cleft cyst (first), squamous
epithelial cyst. 3, Submental—lymphadenitis, cystic hygroma, thyroglossal Staphylococcus aureus and group A β-hemolytic streptococcus are
duct cyst, dermoid, sialadenitis. 4, Submandibular—lymphadenitis, cystic responsible for 50% to 90% of cases of acute unilateral cervical lymph-
hygroma, sialadenitis, tumor, cystic fibrosis. 5, Jugulodigastric—lymphadenitis, adenitis. These organisms spread from a primary site to the lymph
squamous epithelial cyst, branchial cleft cyst (first), parotid tumor, normal- nodes, draining those sites. Common primary sites of infection are
transverse process C2, styloid process. 6, Midline neck—lymphadenitis, the throat, teeth and gums, and skin (lesions), particularly on the
thyroglossal duct cyst, dermoid, laryngocele, normal hyoid, thyroid. scalp or ears. Infections at these sites may result from trauma, such
7, Sternomastoid (anterior)—lymphadenitis, branchial cleft cyst (second, third) as scratches or scabs, or from primary infection, such as impetigo.
pilomatricoma, rare tumors. 8, Spinal accessory—lymphadenitis, lymphoma, Anaerobic oral flora constitute a small portion of causes for bacte-
metastasis from nasopharynx. 9, Paratracheal—thyroid, parathyroid, rial cervical adenitis, especially in older children with poor dentition.
esophageal diverticulum. 10, Supraclavicular—cystic hygroma, lipoma,
Staphylococcus aureus or group B streptococcus usually causes cervi-
lymphoma, metastasis, normal fat pad, pneumatocele of upper lobe.
cal lymphadenitis in neonates or very young infants. Such staphylo-
11, Suprasternal—thyroid, lipoma, dermoid, thymus, mediastinal mass.
coccal infections, which often are nosocomially spread from contact
Adapted with permission from May M. Neck masses in children: diagnosis and
treatment. Pediatr Ann. 1976;5[8]:517–535, with permission from SLACK Inc. in the newborn nursery, present as discrete masses. Group B strep-
tococcus causes the “cellulitis-adenitis” syndrome, which presents
as cervical adenitis associated with a facial cellulitis. Pseudomonas
Lymphadenopathy/Lymphadenitis aeruginosa is an unusual cause of cervical adenitis in newborns.
Lymphadenopathy is lymph node enlargement or hyperplasia sec- Mycobacterial disease must always be considered in children
ondary to localized infection or antigenic stimulation proximal of all ages who present with cervical adenitis particularly involv-
to the involved node or nodes. Because lymphoid tissue steadily ing posterior cervical nodes. The child with M tuberculosis cervi-
increases until puberty, palpable lymph nodes, including those in cal adenitis often has multiple nodes, sometimes bilateral, usually
the cervical area, are a common, normal finding in children. Palpable nontender, and usually not erythematous or warm. These children
cervical lymph nodes are present in up to 90% of children between 4 are typically older, commonly reside in an urban setting, have a his-
and 8 years of age. Any lymph node in the neck larger than 10 mm tory of tuberculosis exposure, and often have an abnormal finding
qualifies as cervical lymphadenopathy. or findings on chest radiography. Intradermal placement of a puri-
The most common cause of bilateral cervical lymphadenopathy fied protein derivative (PPD) often produces more than 15 mm of
is a viral infection of the upper respiratory tract. Lymphadenopathy induration in most children with typical mycobacterial infection.
usually begins and resolves with the acute infection. Occasionally, A reaction of 5 to 14 mm may be caused by tuberculous or nontu-
the swelling remains for several days or months, however, and a child berculous mycobacterial infection. Atypical mycobacterial infection
presents because of parental or guardian concern. Bacterial pharyn- usually occurs in children between 1 and 6 years of age with unilat-
gitis, usually from infection with group A b-hemolytic streptococ- eral rather than bilateral lymph node enlargement and normal find-
cus, is often associated with cervical lymphadenopathy. Cervical as ings on chest radiography.
well as generalized lymphadenopathy may occur in the child with Cat-scratch disease should be considered in children who have
systemic illness (eg, Kawasaki disease, sarcoid, HIV, mononucleosis). cats or kittens or who play with them. The infection may result

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 680 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

than 6 years. Non-Hodgkin lymphoma typically occurs in preado-

Preauricular
lescence, and Hodgkin disease and thyroid carcinoma are the most
Submandibular common malignancies in adolescents.
Benign neck tumors, with the exception of those mentioned
Mastoid later in the discussion of congenital lesions, are uncommon. They
Submaxillary
Jugulodiagastic include epidermoid inclusion cysts, lipomas, fibromas, neurofi-
Submental Occipital bromas, pilomatricomas (ie, benign skin neoplasms of hair follicle
origin), keloids, goiters, and ranulas (ie, intraoral mucocele) and
plunging ranula (ie, an extension of the oral ranula into the neck).

Deep cervical
Trauma
Superficial cervical
chain chain Trauma to the neck may be associated with bleeding and edema.
Large hematomas that affect vital structures are potentially life-
Figure 94.2. The lymphatic drainage and lymph nodes involved in infants and
threatening. Significant trauma and structural injury usually accom-
children with cervical lymphadenitis.
pany expanding neck hematomas. Neurologic deficits and stroke
Adapted with permission from Feigin RD, Cherry JD, eds. Textbook of Pediatric Infectious
Diseases. 5th ed. Philadelphia, PA: Saunders; 2004:186.
after neck trauma should alert the physician to consider cervical
arterial dissection. In the child with mild injury and neck hematoma,
bleeding disorders are a possible cause of the hematoma. Twisting
from a cat scratch or from a cat licking a child’s broken skin. If the injury to the neck may result in muscle spasm of the sternocleido-
inoculum is near the head and neck area, cervical adenitis mani- mastoid muscle (ie, torticollis) and an apparent mass that is the con-
fests. Contact with the hand may result in axillary lymphadenitis. tracted muscle. Additionally, intramuscular hematoma or bleeding
Occasionally, generalized lymphadenopathy is present. An asso- from vaginal delivery may cause torticollis in the neonatal period.
ciated nonpainful papule or papules where the cat scratch or lick The neck is bent toward the side of the affected sternocleidomastoid
occurred may be apparent. Bartonella henselae has been identified muscle. Child abuse should also be considered in children who have
as the organism causing cat-scratch disease. neck injuries that are not consistent with their histories.
Toxoplasmosis may be accompanied by adenitis, usually in the Atlantoaxial rotary subluxation may result in a torticollis-like
posterior cervical area. Nodes are painless and may fluctuate in syndrome in the patient with minimal to no trauma. Because of
size, and children often are asymptomatic. Multiple lymph nodes the relatively flat nature of the facets, rotation and subluxation of
are involved in approximately one-third to one-half of cases. C1 on C2 can occur. The patient generally presents with unilateral
The child who presents with recurrent cervical adenitis and neck pain and inability to turn the head. The head is tilted to 1 side
recurrent fever may have PFAPA syndrome (periodic fever, aph- with the chin rotated the opposite direction from the subluxation.
thous stomatitis, pharyngitis, and cervical adenitis). This entity usu- A foreign body in the neck may present as a mass because of the
ally occurs in children younger than 5 years and can be aborted foreign body itself (eg, piece of glass or metal, bullet) or surrounding
with steroids. inflammation. A crepitant neck mass following trauma to the neck
Kikuchi disease, or histiocytic necrotizing lymphadenitis, has or chest is suggestive of subcutaneous emphysema from tracheal
an Asian and female predilection and is characterized by fever, leu- injury or a pneumomediastinum. Crepitant neck masses may also
kopenia, and cervical lymphadenopathy. The illness is self-limited, occur secondary to pneumomediastinum in the child with obstruc-
and follow-up is recommended because of a possible association tive lung disease, such as asthma or cystic fibrosis.
with systemic lupus erythematosus.
Less common bacterial, viral, and fungal causes of cervical Congenital Lesions
adenitis are listed in Box 94.3. The child with a congenital neck lesion can present with a neck
mass in early infancy or later in childhood. Some congenital lesions
Tumors are not discovered until adulthood. The most common of these
Compared with other neck masses, malignant neck tumors occur benign lesions are thyroglossal duct cysts, branchial cleft cysts,
rarely; nevertheless, they should be considered in any child with lymphatic malformations (ie, cystic hygromas/lymphangiomas),
an enlarging or persistent neck mass. Hodgkin disease and non- and hemangiomas (Figure 94.3).
Hodgkin lymphoma are the most frequent cause of head and neck Thyroglossal duct cysts are almost always midline in the neck
malignancies in children, accounting for almost 60% of cases. and inferior to the hyoid bone. They usually move upward with
Rhabdomyosarcoma is the next most frequent head and neck malig- tongue protrusion or swallowing. Most branchial cleft cysts occur
nancy, followed by thyroid tumor, neuroblastoma, and nasopha- anterior to the middle third of the sternocleidomastoid muscle.
ryngeal carcinoma. Age is an important factor in determining the Less commonly, branchial cleft cysts may appear in the poste-
likelihood of specific tumors. Neuroblastoma, leukemia, and rhabdo- rior triangle of the neck and the preauricular area. Branchial
myosarcoma are the most common tumor types in children younger cleft sinus tracts appear as slit-like openings anterior to the

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 Chapter 94: Neck Masses 681

Box 94.3. Differential Diagnosis of Neck Masses

Cervical Lymphadenopathy/Lymphadenitis Tumor Immunologic Disorders
Bacterial Origin Malignant ww Local hypersensitivity reaction (sting or bite)
ww Staphylococcus aureus (methicillin sensitive) ww Hodgkin disease ww Pseudolymphoma (from phenytoin)
ww Staphylococcus aureus (methicillin resistant) ww Non-Hodgkin lymphoma ww Serum sickness
ww Group A b-hemolytic streptococcus ww Lymphosarcoma ww Sarcoidosis
ww Mycobacterium tuberculosis ww Rhabdomyosarcoma ww Infantile cortical hyperostosis (Caffey-Silverman
ww Atypical mycobacteria ww Neuroblastoma syndrome)
ww Cat-scratch disease (Bartonella henselae) ww Leukemia ww Kawasaki disease
ww Anaerobes ww Langerhans cell histiocytosis ww Systemic lupus erythematosus
ww Gram-negative enteric bacteria ww Thyroid tumors ww Juvenile rheumatoid arthritis
ww Haemophilus influenzae ww Nasopharyngeal squamous cell carcinoma ww Kikuchi disease (necrotizing lymphadenitis)
ww Plague ww Salivary gland carcinoma ww Kimura disease
ww Actinomycosis Benign Miscellaneous
ww Diphtheria ww Epidermoid cyst ww Sialadenitis
ww Tularemia ww Lipoma ww Parotitis
ww Brucellosis ww Fibroma ww Storage disorders
ww Syphilis ww Neurofibroma ww Niemann-Pick disease
ww Group B streptococcus (neonates) ww Pilomatricoma ww Gaucher disease
Viral Origin ww Keloid ww Obstructive airway disease (asthma, cystic
ww Epstein-Barr virus (infectious mononucleosis) ww Goiter fibrosis)
ww Adenovirus ww Osteochondroma
ww Cytomegalovirus ww Teratoma (may be malignant)
ww Human herpesvirus types 1 and 2 ww Ranula
ww Enterovirus Congenital Disorders
ww HIV Hemangioma
ww Measles ww Cystic hygroma (lymphangioma)
ww Rubella ww Branchial cleft cyst
ww Human herpesvirus 6 ww Thyroglossal duct cyst
ww Influenza virus ww Laryngocele
Fungal Origin ww Dermoid cyst
ww Histoplasmosis ww Cervical rib
ww Coccidioidomycosis ww Sternocleidomastoid tumor
ww Aspergillosis Trauma
ww Candidiasis ww Hematoma (acute or organized)
ww Sporotrichosis ww Subcutaneous emphysema
ww Cryptococcosis ww Foreign body
Parasitic Origin ww Arteriovenous malformation
ww Toxoplasmosis
ww Leishmaniasis

lower third of the sternocleidomastoid muscle and may present common on the left side of the neck. Cystic hygromas occasionally
as neck masses if they become infected. Thyroglossal duct cysts become secondarily infected, with findings of erythema, warmth,
and branchial cleft cysts may also present for the first time as and tenderness.
infected neck masses. Hemangiomas are usually not present at birth but appear in early
Cystic hygromas are usually large, soft, easily compressible infancy and may enlarge rapidly. In most cases, they recede sponta-
masses found in the posterior triangle behind the sternocleido- neously by 9 years of age. They are usually much smaller than cys-
mastoid muscle in the supraclavicular fossa. They transillumi- tic hygromas, do not transilluminate, and may be recognized by
nate well. Two-thirds of cystic hygromas are present at birth, and their reddish color (eg, capillary or strawberry hemangioma) or
80% to 90% are identified before 3 years of age. They are more by a bluish hue of the overlying skin (eg, cavernous hemangioma).

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 682 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

The small infant who presents with torticollis should be exam- Evaluation
ined for a sternocleidomastoid mass (“tumor”), which represents
History
fibrosis and contracture of that sternocleidomastoid muscle so that
the head tilts toward the affected side with the chin rotating to the A thorough history is important in establishing the etiology of the
opposite side. Contusion of the sternocleidomastoid muscle from neck mass (Box 94.4).
traumatic extraction of the head during delivery with subsequent
hemorrhage and healing has been implicated as the cause of the
Physical Examination
fibrotic mass. It is more likely, however, that this mass occurs before A general physical examination should be performed. The neck mass
birth, because it contains mature fibrous tissue. Additionally, the should be examined for anatomic location, color, size, shape, consis-
mass may be present following cesarean section and is associated tency, tenderness, fluctuation, and mobility. The mass should also
with hip dysplasia and other congenital lesions, which suggests that be measured. A mass that has an audible bruit or palpable thrill is
the condition is related to abnormal positioning in utero. Venous suggestive of a vascular malformation or traumatic injury. The air-
occlusion of the sternocleidomastoid muscle in utero or at the time way should be assessed for patency, including presence of stridor,
of delivery has also been proposed as a cause. trismus, drooling, or other signs of airway compromise. The head,
neck, and face should be examined for lesions, most often infections
that drain into neck lymph nodes. Lesions can frequently be found
on the scalp, neck, face, ears, mouth, teeth, tongue, gums, and throat.
Hairstyles, such as tight braids, can sometimes provide ports of entry
for bacteria. Occasionally, sinus tracts or fistulas may be the entry
point of infection. Additionally, other lymph node groups should
be examined to determine if the lymphadenopathy is local or gen-
eralized. Particular attention should be paid to the supraclavicular
area because enlarged supraclavicular nodes are more frequently
G associated with malignant pathology, such as Hodgkin disease. The
chest should be examined for use of accessory muscles, equality of
A F
breath sounds, and wheezing. The abdomen should be examined for
B hepatosplenomegaly.
Hyoid
bone
C
Laboratory Tests
D Laboratory tests are rarely indicated in the child with cervical lym­
E
phadenopathy or lymphadenitis of acute onset. Although a rapid
antigen detection test or throat culture for group A streptococci is
helpful in the child with suspected streptococcal sore throat, it may
be unnecessary if antibiotic therapy is empirically prescribed for
lymphadenitis. If the adenitis is fluctuant, aspiration and culture
may be helpful in determining the specific bacteriologic diagnosis.
G

F
A
Box 94.4. What to Ask
C
Neck Masses
B
D
ww How old is the child?
E
ww How long has the neck mass been present?
ww What signs and symptoms are associated with the neck mass?
ww Has the child been exposed to tuberculosis?
Figure 94.3. Frontal (upper left) and lateral (lower right) views of head and
ww Has the child consumed any unpasteurized cow’s milk?
neck congenital lesions that occur in children. The shaded areas denote ww Has the child been in contact with any cats or kittens? Rabbits? Other
the distribution in which a given lesion may be found. A, Dermoid cyst. animals?
B, Thyroglossal duct cyst. C, Second branchial cleft appendage. D, Second ww Has the child traveled to areas where endemic diseases, such as histo-
branchial cleft sinus. E, Second branchial cleft cyst. F, First branchial pouch plasmosis or coccidioidomycosis, are prevalent?
defect. G, Preauricular sinus or appendage. ww Has the child suffered any trauma recently?
Reproduced with permission from Fleisher GR, Ludwig S, Henretig FM, eds. Textbook of ww Does the child have any allergies?
Pediatric Emergency Medicine. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; ww Does the child have any risk factors for HIV?
2006:1594.

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 Chapter 94: Neck Masses 683

Fine-needle aspiration cytology and polymerase chain reaction Imaging Studies

assays may help differentiate between tuberculous and nontuber- A chest radiograph should be obtained in all children with suspected
culous mycobacterial disease. Superficial cervical nodes or axillary tuberculosis or tumor (eg, enlarged supraclavicular nodes) as well
nodes, if enlarged, are appropriate first-line aspiration sites because as in children with crepitant neck masses or in whom the diagno-
of their superficial nature. A PPD test should be applied to all children sis of the adenitis remains uncertain. If a foreign body in the neck
who present with lymphadenitis. is suspected, anteroposterior and lateral neck radiographs should
If the adenitis does not resolve or improve after 2 to 3 days of be obtained.
observation or therapy, laboratory tests directed by the history and Almost all neck masses can be evaluated by ultrasonography,
physical examination should be considered. A complete blood cell computed tomography (CT), or magnetic resonance (MR) imaging.
count with differential and a monospot test or serologic antibody Because there is no ionizing radiation associated with ultrasonography
tests for Epstein-Barr virus may be helpful in the diagnosis of infec- and because it is quickly available, is noninvasive, and does not need
tious mononucleosis. If the neck mass is a hematoma and a bleed- sedation, it is the initial imaging study of choice, particularly for thy-
ing disorder is suspected, a complete blood count with platelet roid masses, thyroglossal duct cysts, branchial cleft cysts, and parotid
count, prothrombin time, and partial thromboplastin time should masses. Ultrasonography is a reasonable screening tool for all super-
be obtained. Possible additional tests include an erythrocyte sedi- ficial neck masses. If malignancy is suspected or the mass cannot be
mentation rate and C-reactive protein. If indicated, serologic tests for delineated in the field of view, MR imaging or CT may be more defin-
toxoplasmosis, cytomegalovirus, human herpesvirus 6, coccidioido- itive studies. Color Doppler ultrasonography is helpful in differentiat-
mycosis, histoplasmosis, tularemia, B henselae, and syphilis should ing cystic from solid masses and determining if the mass is vascular.
be performed. Skin tests for fungi should be considered in those Additionally, demonstration of a normal thyroid gland by ultrasonog-
patients coming from endemic areas. These tests should be placed raphy in the patient with a thyroglossal duct cyst confirms a source
after serologic tests are performed to avoid false-positive results on of thyroid hormone. Consequently, a thyroid scan is not necessary.
serologic testing. Computed tomography and MR imaging provide better def-
Similar laboratory tests should be considered for the child who inition and extent of the mass than ultrasonography. Computed
presents for the first time with enlarged or enlarging lymph nodes tomography with contrast is the preferred method of imaging for
of long duration or who does not respond after 2 weeks of antibiotic deep neck abscesses that present with a neck mass and for enlarged
therapy. The physician may choose to do more of these laboratory salivary glands. Computed tomography or MR imaging is superior
tests at the initial evaluation or after a therapeutic trial because the to plain radiography for diagnosing rotary subluxation. Magnetic
likelihood of viral lymphadenopathy decreases and the possibility resonance imaging provides the best anatomic detail, and it is the
of a more unusual etiology increases. HIV testing should also be preferred imaging study for children with vascular and lymphatic
considered. malformations and neoplasms; however, it is not as available as CT
Thyroid function testing should be considered in the child with a and often requires a greater degree of sedation.
thyroid mass or suspected thyroglossal duct cyst. Ultrasonography
or thyroid scan should be considered for the child with a
thyroid mass. Management
Unless cat-scratch disease is very likely, urgent lymph node Neck masses are rarely acutely life-threatening. If a neck mass
biopsy is indicated for enlarged supraclavicular nodes or lymph impinges on the airway, the affected child may experience stridor,
nodes in the lower half of the neck, rapidly progressive and enlarg- hoarseness, drooling, increased effort of breathing, unequal breath
ing nodes, fixed nodes, nodes deep within the fascia, or firm or sounds, or evidence of shock. Resuscitation and stabilization should
hard nodes. The child with a persistent fever or weight loss should be initiated immediately. There should be a low threshold to estab-
undergo a biopsy at 1 week if a diagnosis has not been established. lish a secure airway in a child with an expanding traumatic neck
The asymptomatic child without an established diagnosis should mass. The algorithm in Figure 94.4 outlines the management of cer-
undergo a biopsy at 2 weeks if the node increases in size, at 4 to vical lymphadenitis.
6 weeks if the node is not increased in size but is persistent, and The child with lymphadenitis who does not appear toxic and
at 8 to 12 weeks if the node has not regressed to a normal size. has no evidence of sepsis may be treated empirically with oral
In some cases, the initial biopsy is nondiagnostic, but on subse- antibiotics that cover S aureus, particularly methicillin-resistant
quent biopsy, a specific diagnosis may be made. Consequently, the S aureus (MRSA), and group A b-hemolytic streptococcus.
child with persistent adenopathy should be followed closely and Clindamycin or a combination of cephalexin (administered until
undergo biopsy again if indicated. Fine-needle aspiration biopsy the culture from the lymph node, if available, for group A strep-
in children is controversial but has some demonstrated benefits. tococcus is negative) and trimethoprim-sulfamethoxazole may be
It is increasingly being used to differentiate between benign and used to provide coverage for these organisms. If methicillin-
malignant disease and to potentially decrease the need for open susceptible S aureus is cultured and sensitivities are determined,
biopsy. If malignancy is a consideration, however, consultation a first-generation cephalosporin, such as cephalexin (50 mg/kg per
with an oncologist is advisable to determine the biopsy method day), dicloxacillin (50 mg/kg per day), or amoxicillin-clavulanate
of choice. potassium (40 mg/kg per day based on the amoxicillin component)

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 684 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

surgeon to promote resolution of the lymphadenitis. Alternatively,

Cervical Lymphadenitis treatment by needle aspiration instead of incision and drainage
has been advocated by some physicians. If M tuberculosis, atypical
PPD
Aspiration if fluctuant mycobacterial infection, or infection with B henselae is suspected,
Antibiotic
Return for follow-up in 1–3 days
Admission if toxic appearing or a young infant
incision and drainage should not be done because persistent sinus
tracts may result.
Not resolving Resolving Atypical mycobacterial infection is generally unresponsive to
treatment with antituberculous medications. Surgical excision
CBC
Monospot
Complete
antibiotic
of all visibly affected nodes, deep as well as superficial, is recom-
Additional tests to consider
ESR
course
mended. In cases in which surgery is refused or is incomplete or
C-reactive protein
Chest radiograph disease is recurrent, however, macrolide or azalide monotherapy
Serologic tests for
Toxoplasmosis (clarithromycin or azithromycin) or a macrolide in combina-
Epstein-Barr virus
Cytomegalovirus
HHV-6
tion with ethambutol hydrochloride, rifampin, or rifabutin has
Coccidioidomycosis
Histoplasmosis
been helpful in managing nontuberculous adenitis. Tuberculous
Tularemia
Bartonella henselae mycobacterial adenitis usually responds to short-course medical
Syphilis
HIV therapy. Antituberculous medication for 6 to 9 months is
Ultrasonography? CT? MRI?
Aspiration if fluctuant recommended.
Consider admission for IV
antibiotics and/or further
evaluation
Lymphadenitis from cat-scratch disease usually resolves sponta-
neously in 2 to 4 months. Azithromycin may shorten the course and
Diagnosis Diagnosis not established
is recommended by some experts. Antibiotic therapy is indicated if a
established
child is severely ill or appears toxic with systemic cat-scratch disease,
Consider consultation with has hepatic or splenic involvement, is immunocompromised, devel-
Treat an oncologist and/or
accordingly otolaryngologist ops meningoencephalitis, or is admitted for a suppurative adenitis.
Consider biopsy
Treatment with oral antibiotics, such as azithromycin, rifampin,
Biopsy indications
trimethoprim-sulfamethoxazole, or ciprofloxacin, may be beneficial.
Location
Supraclavicular or lower half of neck If no improvement occurs, the addition of parenteral gentamicin to
Perform biopsy immediately unless strong history
of cat-scratch disease and negative chest radiograph the regimen may be considered. Aspiration is indicated to relieve
Clinical findings
Immediate biopsy if symptoms in the patient with fluctuant or painful suppurative nodes.
Rapidly progressive and enlarging
Fixed Incision and drainage should not be done. Total removal of the node
Deep within the fascia
Firm or hard
Persistent fever or weight loss
is sometimes necessary to effect a cure.
Biopsy by 1 week
Asymptomatic child
No specific therapy exists for lymphadenitis caused by
Node increased in size: biopsy at 2 weeks
Node is same size: biopsy at 4–6 weeks Toxoplasma gondii. Excision of the affected node may be indicated
Node not regressed to normal: biopsy at 8–12 weeks
if the diagnosis is questionable.
If a tumor is suspected, consultation with a pediatric oncologist
Figure 94.4. Management of cervical lymphadenitis.
is recommended. The mass should be biopsied for a definitive diag-
Abbreviations: CBC, complete blood cell count; CT, computed tomography; ESR, erythrocyte
sedimentation rate; HHV-6, human herpesvirus 6; HIV, human immunodeficiency virus;
nosis. If it is malignant, a treatment plan should be generated with
IV, intravenous; MRI, magnetic resonance imaging; PPD, purified protein derivative. the appropriate consultants.
Congenital torticollis from fibrosis of the sternocleidomastoid
muscle is best managed nonsurgically. Repetitive passive range-of-
may provide satisfactory coverage. Treatment should be continued motion exercises over several weeks usually result in loosening the
for at least 10 days but for no less than 5 days after resolution of tight muscle and increasing the range of motion. Rotary sublux-
acute signs and symptoms. Lack of clinical improvement after 36 to ation is generally treated conservatively with immobilization because
48 hours suggests that the diagnosis and proposed therapy need to most cases resolve. More severe cases are treated in traction by a
be reevaluated. neurosurgeon.
Infants and younger children, as well as those older children Fifty percent of hemangiomas will usually resolve by the
who do not respond to oral antibiotic therapy, may require admis- time children are 5 years of age, 70% by age 7, and 90% by age 9.
sion for intravenous antibiotics, such as clindamycin or vancomycin Propranolol may expedite evolution of hemangioma. Surgical
(especially for a potentially life-threatening infection) until culture intervention is rarely indicated. Recent literature has demonstrated
and sensitivity results are available. If methicillin-susceptible a profound benefit with oral propranolol therapy in the manage-
S aureus is cultured and sensitivities are determined, oxacillin or a ment of hemangioma. Oral propranolol therapy demonstrates a
cephalosporin may be satisfactory alternatives. Incision and drain- considerable flattening and effacement of cutaneous and mucous
age of large, fluctuant nodes that are clearly from bacterial dis- membrane hemangiomas within 24 hours and marked improve-
ease should be done in consultation with an otolaryngologist or ment after weeks of propranolol therapy. Multiple studies suggest

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 Chapter 94: Neck Masses 685

excellent efficacy with oral propranolol for the management of

life-threatening, vital-organ–impairing (eg, vision loss), refrac- CASE RESOLUTION
tory, or disfiguring hemangioma. Dosing is titrated up to 3 mg/kg The boy has signs and symptoms consistent with submandibular bacterial cervi-
per day and continued for up to 6 months until resolution of the cal lymphadenitis. The location of the neck mass in relation to the honey-crusted
hemangioma. The patient should be closely monitored for hypo- lesions (nonbullous impetigo) implicates spread of bacteria from the primary site
glycemia, bradycardia, hypotension, and congestive heart failure of infection to the lymph nodes. Laboratory tests are unnecessary because the
child does not appear septic. Culture of the impetigo may be helpful in deter-
during institution of therapy. Propranolol initiation is increasingly
mining if the organism causing the infection is MRSA. If the affected lymph node
performed in the outpatient setting, and evidence exists indicat- is fluctuant, aspiration of fluid for culture is indicated and incision and drainage
ing that initiation can be safely performed in the healthy child. should be considered. The child should be treated as an outpatient with an oral
Atenolol appears to work just as well as propranolol, but most stud- antibiotic directed against MRSA and group A b-hemolytic streptococcus, such as
ies are performed with propranolol. Corticosteroids, previously the clindamycin or a combination of cephalexin and trimethoprim-sulfamethoxazole,
as well as an analgesic for pain as necessary. If methicillin-susceptible S aureus
mainline treatment modality for hemangiomas, are now consid-
is cultured and sensitivities are determined, cephalexin, amoxicillin-clavulanic
ered an adjunctive treatment with propranolol. Additional ther- acid, or dicloxacillin may be administered. If the child appears toxic or if marked
apies include interferon-α-2a and various cytotoxic medications. lymph node enlargement is present, the child should be admitted for intravenous
Interferon-α-2a has been associated with transient or permanent antibiotics. A PPD skin test should be placed for tuberculosis. The child should be
neurologic disabilities, so it is important to balance the benefits followed up in 1 to 3 days for clinical improvement depending on the severity of
and risks of this medication. High-potency topical corticosteroids the infection.
(eg, clobetasol propionate) or topical beta blockers (eg, timolol)
may be used in selected cases.
Surgical excision is usually indicated for cystic hygroma (ie, Selected References
lymphangioma), branchial cleft cyst, and thyroglossal duct cyst.
Abarzúa-Araya A, Navarrete-Dechent CP, Heusser F, Retamal J, Zegpi-Trueba
Thyroid ultrasonography should be performed before removal MS. Atenolol versus propranolol for the treatment of infantile hemangiomas:
of a thyroglossal duct cyst to determine the presence of ectopic a randomized controlled study. J Am Acad Dermatol. 2014;70(6):1045–1049
thyroid tissue. Endocrinologic consultation is recommended in PMID: 24656727 https://doi.org/10.1016/j.jaad.2014.01.905
these cases. Thyroid nodules in children may be cancerous, espe- Acierno SP, Waldhausen JH. Congenital cervical cysts, sinuses and fistulae.
cially in children who have undergone irradiation to the head and Otolaryngol Clin North Am. 2007;40(1):161–176, vii–viii PMID: 17346566
neck region. Consultation with an endocrinologist and surgeon https://doi.org/10.1016/j.otc.2006.10.009
should be obtained. Goiters in children should be evaluated by an Bansal AG, Oudsema R, Masseaux JA, Rosenberg HK. US of pediatric superfi-
endocrinologist. cial masses of the head and neck. Radiographics. 2018;38(4):1239–1263 PMID:
29995618 https://doi.org/10.1148/rg.2018170165
Prognosis Chadha NK, Forte V. Pediatric head and neck malignancies. Curr Opin
The prognosis for children with neck masses is generally excellent Otolaryngol Head Neck Surg. 2009;17(6):471–476 PMID: 19745735 https://doi.
but differs depending on the cause. Children with an infectious eti- org/10.1097/MOO.0b013e3283323893
ology do quite well if diagnosed and treated with appropriate anti- Droitcourt C, Kerbrat S, Rault C, et al. Safety of oral propranolol for infantile
microbial agents. Mycobacterial lymphadenitis may result in sinus hemangioma. Pediatrics. 2018;141(6):e20173783 PMID: 29844139 https://doi.
tract formation or disseminated disease. Surgical excision usually org/10.1542/peds.2017-3783

cures atypical mycobacterial infection. Cat-scratch disease is usu- Frieden IJ, Drolet BA. Propranolol for infantile hemangiomas: promise, peril,
ally benign and self-limited. Encephalopathy is a rare complication pathogenesis. Pediatr Dermatol. 2009;26(5):642–644 PMID: 19840341 https://
doi.org/10.1111/j.1525-1470.2009.00977.x
of this disorder.
The prognosis for benign tumors of the neck is excellent. The Goff CJ, Allred C, Glade RS. Current management of congenital bran-
chial cleft cysts, sinuses, and fistulae. Curr Opin Otolaryngol Head Neck
outlook for the child with a malignant neck tumor depends on eti-
Surg. 2012;20(6):533–539 PMID: 23128685 https://doi.org/10.1097/MOO.
ology of the tumor and spread of the malignancy to other organs. 0b013e32835873fb
Early diagnosis and treatment are important in improving outcome.
Gross E, Sichel JY. Congenital neck lesions. Surg Clin North Am. 2006;86(2):
The child with a neck mass resulting from trauma usually has 383–392, ix PMID: 16580930 https://doi.org/10.1016/j.suc.2005.12.013
sustained significant injury. The outcome often depends on estab-
Healy CM, Baker CJ. Cervical lymphadenitis. In: Cherry JD, Harrison GJ, Kaplan
lishment of an airway, provision of ventilatory support, and manage- SL, Steinbach WJ, Hotez PJ, eds. Feigin and Cherry’s Textbook of Pediatric
ment of hemodynamic instability. Availability of surgical support is Infectious Disease. 8th ed. Philadelphia, PA: Elsevier; 2019:124–133
often essential to a good outcome. Holmes WJ, Mishra A, Gorst C, Liew SH. Propranolol as first-line treatment
The child with a congenital lesion of the neck usually has an for rapidly proliferating infantile haemangiomas. J Plast Reconstr Aesthet Surg.
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others require simple surgical excision. Cystic hygromas may Jackson MA, Long SS, Kimberlin DW, Brady MT, eds. Red Book: 2018 Report
require multiple operations for complete removal because of their of the Committee on Infectious Diseases. 31st ed. Itasca, IL: American Academy
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Lawley LP, Siegfried E, Todd JL. Propranolol treatment for hemangioma of Rajasekaran K, Krakovitz P. Enlarged neck lymph nodes in children. Pediatr
infancy: risks and recommendations. Pediatr Dermatol. 2009;26(5):610–614 Clin North Am. 2013;60(4):923–936 PMID: 23905828 https://doi.org/10.1016/j.
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Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Sans V, Dumas de la Roque E, Berge J, et al. Propranolol for severe infantile
Taïeb A. Propranolol for severe hemangiomas of infancy [letter]. N Engl J Med. hemangiomas: follow-up report. Pediatrics. 2009;124(3):e423–e431 PMID:
2008;358(24):2649–2651 PMID: 18550886 https://doi.org/10.1056/NEJMc0708819 19706583 https://doi.org/10.1542/peds.2008-3458
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Novoa M, Baselga E, Beltran S, et al. Interventions for infantile haemangiomas Tanphaichitr A, Bhushan B, Maddalozzo J, Schroeder JW Jr. Ultrasonography
of the skin. Cochrane Database Syst Rev. 2018;4(40):CD006545 PMID: 29667726 in the treatment of a pediatric midline neck mass. Arch Otolaryngol Head
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Pasha R, ed. Otolaryngology: Head and Neck Surgery Clinical Reference Guide. archoto.2012.1778
2nd ed. San Diego, CA: Plural Publishing; 2006:79, 207 Vogeley E, Saladino RA. Pharyngeal procedures. In: King C, Henretig FM, eds.
Pruden CM, McAneney CM. Neck mass. In: Shaw KN, Bachur RG, eds. Fleisher Textbook of Pediatric Emergency Procedures. 2nd ed. Philadelphia, PA: Lippincott
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 CHAPTER 95

Allergic Disease
Nasser Redjal, MD, and Niloufar Tehrani, MD

CASE STUDY
A 3-year-old girl is rushed to an urgent care center by including blood pressure, are normal. The girl has a dif-
her mother after the girl developed a pruritic rash, facial fuse, blotchy, erythematous rash with central wheals; a
swelling, and hoarseness shortly after eating a peanut hoarse voice; and a mild expiratory wheeze on auscul-
butter sandwich. She had eaten peanut butter once tation of her chest. The remainder of the examination is
before, and her parents noticed a few small hives on her normal.
cheek that self-resolved. Previously, the girl has been
well except for recurrent nasal congestion every spring
Questions
1. What are the various symptoms of allergic disease?
that has responded to antihistamines. She has also had
2. What is the appropriate evaluation of a child with
an intermittent skin rash that has been managed with
manifestations of allergic disease?
topical steroid creams. She has never before had an
3. What allergens are common triggers for allergic
acute reaction and has no history of asthma. Her father
symptoms?
had asthma as a child.
4. What treatment is helpful for the child with mani-
Physical examination reveals a well-developed,
festations of allergic disease?
3-year-old girl with marked facial swelling and a gener-
5. Can allergic disease be prevented?
alized rash who is in mild respiratory distress. Vital signs,

Allergic disease occurs frequently in the general population and Epidemiology

manifests in many ways. Types of allergic disease include asthma,
Allergic disease or some form of allergic symptoms occur in 12%
atopic dermatitis, allergic rhinitis, allergic conjunctivitis, urticaria,
to 20% of the general population in the United States. The prev-
angioedema, anaphylaxis, and food, insect, and drug allergies.
alence of symptoms varies depending on the population being
Allergic symptoms result from the production of specific immu-
investigated. Factors such as age, genetic background, and place of
noglobulin (Ig) E antibody after exposure to a foreign antigen. The
residence are significant. Allergic rhinitis occurs in 10% of children,
process has 2 steps. The first step is the sensitization or antibody
as does asthma. Up to 6% of children younger than 3 years develop
induction stage. The individual develops IgE antibody against an
food allergies. Urticaria occurs at some time in approximately 10%
inhaled, ingested, or injected substance. The process usually takes
to 20% of the population.
from several days to weeks for medications and foods to months to
It is generally accepted that if neither parent is atopic (ie, has
years for inhalant allergens, such as pollens. Newly formed antigen-
the allergic tendency to manufacture IgE on antigen exposure),
specific IgE antibody adheres to IgE receptors on circulating
the chance that a child of theirs will develop allergic symptoms is
blood basophils or to tissue mast cells. On reexposure, the aller-
less than 1 in 5. If 1 parent is atopic, however, the risk doubles. If
gen binds to several specific IgE antibodies on the surface of these
both parents are atopic, the chance of their child developing aller-
cells and triggers degranulation of preformed and rapidly formed
gic symptoms is better than 3 in 5.
mediators, such as histamine and leukotrienes, resulting in the
early phase of the reaction. Clinically, this is manifested by sneez-
ing, rhinorrhea, and pruritus in allergic rhinitis; wheal and/or
Clinical Presentation
flare in urticaria or angioedema; and shock in anaphylaxis. Mast Children with allergic disease frequently present with persistent, clear
cell degranulation also releases peptides that attract inflammatory rhinorrhea; sneezing; postnasal drip; or injected pruritic conjuncti-
peptides, such as interleukin-4 and interleukin-5, which release vitis. Skin manifestations include dry, scaling, erythematous rashes;
mediators that result in chronic inflammation. This late phase wheals; and subcutaneous swelling (Box 95.1). A recurrent cough or
of inflammation is responsible for congestion and hyperreactive wheezing on chest examination is further evidence of allergic disease.
mucosa in allergic rhinitis and delayed reactions in anaphylaxis.
The remaining manifestations of allergies, including allergic rhi- Pathophysiology
nitis, conjunctivitis, food allergies, and anaphylaxis are the focus Allergic rhinitis, like all allergic manifestations, is caused primarily
of this chapter. by an antigen-antibody reaction involving IgE. Antigen-specific

687

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 688 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

An urticarial rash appears as erythematous lesions of various

Box 95.1. Diagnosis of Allergic Disease
sizes with pale, papular centers that typically are not painful unless
in the Pediatric Patient
traumatized by scratching. Lesions may coalesce, the rash blanches
ww Chronic, clear rhinorrhea on pressure, and the skin is intact. Usually, individual lesions last
ww Nasal congestion less than 24 hours at a single location, although they often recur in
ww Conjunctival tearing and pruritus the same area.
ww Conjunctival injection Angioedema is the extension of the urticarial process deeper into
ww Wheezing the dermis of the skin, producing swelling. The mucous membranes
ww Chronic cough may be affected. Pathophysiology is similar to that of urticaria. A
ww Postnasal drip hereditary type of angioedema, which is not allergy related, is caused
ww Mouth breathing by an inherited deficiency of the C1 esterase inhibitor, resulting in
ww Snoring unopposed production of the potent vasoactive amine bradykinin.
ww Skin findings of atopic dermatitis or urticaria Unlike urticaria, no distinct rash exists and the skin is not pruritic.
ww Seasonal variability of symptoms Capillary leak and edema of the dermis results in tissue swelling, a
ww Occurrence of symptoms after exposure to an antigen sense of tightness, and sometimes pain. Involvement of the abdom-
ww Family history of allergic disease inal viscera may result in colicky abdominal pain.
ww Acute onset of symptoms following exposure to possible allergen Chronic urticaria and angioedema is that which persists beyond
6 weeks. More than 90% of chronic urticaria and angioedema in an
otherwise healthy patient is idiopathic and is termed chronic idio-
pathic urticaria. Up to one-half of patients with chronic idiopathic
IgE is produced by the B lymphocytes of allergic patients on expo- urticaria have the circulating mast cell anti-IgE receptor antibody
sure to a particular antigen, which attaches to immune cell recep- IgG, which repeatedly causes degranulation of cutaneous mast cells
tors located on basophils in the circulation and mast cells in the in these patients.
tissue. On reexposure, the antigen reacts with this specific IgE on Anaphylaxis is an acute, systemic allergic reaction resulting from
the mast cells, releasing vasoactive mediators, including histamine, antigen-specific IgE on mast cells and basophils. Pathophysiology
leukotrienes, kinins, and prostaglandins. These mediators produce is similar to that of allergic rhinitis, but the reaction occurs in mast
vasodilation and edema, and they also stimulate neural reflexes to cells in many locations simultaneously, and prior sensitization to an
produce mucous hypersecretion and sneezing. Eosinophils, baso- allergen is essential. Anaphylactic reactions may be life-threatening.
phils, and other inflammatory cells induced by chemotactic factors Massive vasoactive mediator release results in large reductions in
enter the affected organ, releasing mediators and thereby worsening peripheral resistance caused by capillary leak and vasodilation. This
the inflammation and damaging tissues. Secretions and released tis- eventually overwhelms compensatory increases in cardiac output
sue proteins exacerbate existing edema. Other immunologic mech- (blood pressure = cardiac output × total peripheral resistance),
anisms can also be involved. resulting in hypotension and shock. Pooling of blood in the periph-
Children with allergic rhinitis often present with rhinorrhea, ery also reduces venous return, resulting in diminished preload and
sneezing, nasal pruritus, and congestion. Allergic eye symptoms cardiac output. Reactions can occur in seconds or as late as 1 hour
(ie, allergic rhinitis, conjunctivitis) may accompany nasal rhinitis after exposure. Up to 20% of adults and 15% of children may expe-
with ocular pruritus, injection of the conjunctiva, and clear tear- rience a late or biphasic phase (ie, a recurrence of symptoms after
ing. Younger children may present with repeated sniffing, snorting, resolution of anaphylaxis without trigger reexposure) hours after
coughing, or scratching an itchy palate with their tongue (ie, palatal the first reaction because of late recruitment and activation of baso-
clicking) because they usually cannot blow their nose. phils and other inflammatory cells. Children with anaphylaxis have
Urticaria, the clinical rash produced by capillary leak vasodilation a reduced risk for late phase reaction, likely of approximately 3% to
and edema of the skin, occurs when histamine and other vasoactive 5%. Common causes of anaphylaxis include food, medications and
peptides, such as prostaglandins and leukotrienes, are released from biologic agents, and stinging insect venom. The drug most frequently
the epidermal mast cells. Basophils have been identified in biopsies implicated in anaphylaxis is penicillin. Patients initially experience
of these lesions. Mast cell degranulation occurs commonly in sensi- tightness and intense itching of the skin. Nausea, vomiting, and
tized patients on reexposure to antigens found in food, medications, abdominal pain may ensue, followed by the full spectrum of ana-
supplements, and insect venom when antigen-specific IgE found on phylaxis. Certain agents, such as radiocontrast media and blood
mast cells is triggered by these allergens. The mast cell, however, can products, may cause massive mast cell degranulation via an IgE-
also be activated and release mediators by a wide number of stimuli independent mechanism, resulting in clinical symptoms that are
independent of IgE mechanisms, including viral, bacterial, parasitic, indistinguishable from anaphylaxis; these reactions, termed anaphy-
and fungal infections; collagen vascular disease; malignancy; and lactoid reactions, are generally less severe than anaphylaxis.
endocrine disease. Some patients also experience urticaria to physi- Confusion exists among less experienced clinicians as to what
cal factors, such as heat, cold, pressure, sun, and vibration. combination of symptoms constitutes anaphylaxis, which may result

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 Chapter 95: Allergic Disease 689

in either overdiagnosis or underdiagnosis. Generally, anaphylaxis is shock on initial ingestion of cow’s milk, although soy and other foods
highly likely in the setting of cutaneous findings plus respiratory or have, in rare cases, been implicated. Allergic proctocolitis presents in
hemodynamic compromise; involvement of 2 or more of the follow- infants on first exposure to cow’s milk protein, including via human
ing systems: skin, respiratory, and gastrointestinal (GI) organ; and milk, but symptoms are mild and involve only blood-streaked stool.
hemodynamic compromise (Box 95.2). Heiner syndrome is a rare IgG-mediated reaction to cow’s milk pro-
A food allergy may produce IgE-mediated reactions and diseases, tein that results in anemia, wheezing, hemoptysis, melena, and pul-
including acute urticaria or angioedema, anaphylaxis, acute rhini- monary infiltrates. Older patients with pollen-food allergy syndrome
tis, and atopic dermatitis. The localization of IgE-sensitized mast to fruits and vegetables experience lip, oral mucosa, and tongue tin-
cells to that specific antigen determines the symptoms produced gling as well as minimal swelling. These reactions are local and mild
by an allergy. The antigen enters through the GI mucosal barrier. and rarely progress to anaphylaxis.
Intact food proteins may enter the circulation, stimulating the pro-
duction of antigen-specific IgE. Additionally, food allergens may Differential Diagnosis
result in some non–IgE-mediated diseases, including eosinophilic Most patients with allergic rhinitis have clearly identifiable signs
esophagitis, food protein-induced enterocolitis syndrome (FPIES), and symptoms consistent with a history of exposure; other etiologies
allergic proctocolitis, and Heiner syndrome, a rare reaction to cow’s should be considered, however, especially with poor response to treat-
milk protein. Finally, many patients have a nonimmune-mediated ment. Children experience many upper viral respiratory infections
reaction to foods termed food intolerance (eg, lactose intolerance). each year that can mimic allergic rhinitis. Many types of nonallergic
In young children, the most common food allergens are milk, rhinitis exist and the mechanisms responsible are unclear, although
egg, soy, wheat, peanuts, and tree nuts, whereas adults and older cholinergic pathways are likely involved. Vasomotor and cholinergic
children are allergic to peanuts, shellfish, and fish. Adults and older rhinitis often result in copious amounts of clear rhinorrhea in
children with atopic predisposition and allergic rhinitis or asthma response to cholinergic stimuli, such as cold air (ie, skier nose) or
often become sensitized to tree and weed pollens via the respira- spicy foods (ie, gustatory rhinitis). Medications such as angiotensin-
tory tract. They exhibit cross-reactivity between common elements converting enzyme inhibitors and nonsteroidal anti-inflammatory
of these inhalant tree or weed pollen peptides and similar peptides drugs may cause rhinitis, but this usually occurs in adults and older
found in fruits and vegetables, resulting in local (ie, oral) IgE symp- children. Similarly, hormone surges, such as during ovulation, may
toms of tingling and mild swelling when ingesting these foods. These result in nasal symptoms. Overuse of topical decongestants contain-
mild reactions are called pollen-food allergy syndrome. ing α-agonists results in rebound rhinitis (ie, rhinitis medicamen-
Patients with IgE-mediated food allergy may experience urticaria tosa). In children, foreign bodies produce unilateral nasal obstruction
or angioedema, eczematoid dermatitis, vomiting, wheezing, and, and often malodorous purulent discharge. Patients with a history of
in severe cases, anaphylaxis. Eosinophilic esophagitis presents with basilar skull fracture with cerebrospinal fluid leak may present with
nonspecific symptoms, including vomiting, reflux, dyspepsia, poor clear rhinorrhea.
appetite, and failure to thrive. Infants with FPIES present with severe Urticaria and angioedema have a distinct clinical presentation
vomiting and bloody stool, generalized edema, and, in some cases, that is usually easy to distinguish from other skin conditions. As
mentioned previously, numerous possible etiologies of urticaria and
angioedema exist, and determining the cause of a given instance is
Box 95.2. Practical Definition of Anaphylaxis challenging. Urticaria and angioedema may last for weeks; however,
individual lesions should persist for less than 24 hours, although
One of the following criteria is fulfilled: they can recur at 1 area. Urticarial vasculitis is a distinct form of
1. Acute onset of mucosa or cutaneous findings, such as hives, pruritus, lip urticaria that results from the persistence of antigens arising from
swelling, AND 1 of the following: collagen vascular disease, serum sickness, and neoplasia. The rash
ww Respiratory compromise from urticarial vasculitis lasts for more than 24 hours, has a burning
ww Cardiovascular compromise (eg, hypotension) or end-organ sensation, causes less pruritus than other forms of urticaria, and
dysfunction (eg, syncope) leaves an area of hyperpigmentation on resolution. Other rashes that
2. TWO of the following after likely exposure to allergen: mimic urticaria include insect bites, erythema multiforme, mastocy-
ww Respiratory compromise tosis, and contact dermatitis. Anaphylaxis is usually associated with
ww Mucocutaneous symptoms cutaneous, respiratory, cardiovascular, and systemic symptoms, such
ww Hypotension as skin rash, edema, wheezing, arrhythmia, occasionally fever, and
ww Persistent gastrointestinal symptoms shock. A history of an acute exposure to a potential allergen is more
3. Reduced blood pressure appropriate for age likely to be seen with anaphylaxis than with urticaria.
Signs and symptoms of food allergies vary. It is important to
Adapted from Sampson HA, Muñoz-Furlong A, Campbell RL, et al. Second symposium on the
definition and management of anaphylaxis: summary report—second National Institute of determine whether actual immune-mediated disease exists or if the
Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network symposium. Ann Emerg Med. patient has food intolerance. Food may contain toxic peptides, such
2006;47(4):373–380, with permission. as in scombroid fish poisoning, in which bacteria in unrefrigerated

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 690 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

fish convert amino acids into histamine, which results in allergy-like It is also important to obtain an environmental history of allergen
symptoms when ingested. Patients with enzyme deficiencies, such exposure, including pets and tobacco smoke, as well as a family his-
as lactose intolerance, experience GI symptoms when eating dairy tory of atopy.
products. Alternatively, food may have pharmacologic properties, The physical examination should be thorough. The skin should be
such as those found in caffeinated drinks. inspected for atopic dermatitis and the lungs for evidence of asthma.
Non–IgE-mediated food allergies may produce signs and symp- The nasal mucosa should be examined with an otoscope. In the child
toms similar to other disorders that can be life-threatening, such as with allergic rhinitis, the nasal mucosa is swollen, pale, and some-
FPIES, which often is indistinguishable from sepsis; Heiner syndrome, times cyanotic with copious clear discharge. Nasal polyps, if present,
which shares similarities with serious diseases (eg, pulmonary hemo- should be noted. Although polyps are most often present on an aller-
siderosis); and Wegener granulomatosis. These conditions must be gic basis, they may occur with cystic fibrosis. A transverse crease
ruled out during the evaluation of these forms of food allergies. across the nose (ie, allergic crease) can occur from repeatedly using
Most patients with symptoms associated with foods do not have the palm of the hand in an upward thrust on the nares to relieve itch-
an immune-mediated reaction but have food intolerances. Some ing and open the nasal airway (ie, allergic salute). Dark circles under
manifestations have well-defined mechanisms, such as in lactase the eyes (ie, allergic shiners) may be present from chronic peri-
deficiency or galactosemia, whereas others, such as gustatory rhi- orbital edema and venous stasis. Morgan fold (ie, Dennie-Morgan
nitis, are less clear. fold), a wrinkle just beneath the lower eyelids, is present from early
infancy and is associated with atopic dermatitis and allergic rhini-
Evaluation tis. Adenoid facies (ie, allergic gape) is secondary to chronic mouth
breathing during the first several years of age and results in a char-
History and Physical Examination
acteristic pattern of maldevelopment of facial bones, causing a high-
Allergic Rhinitis and Conjunctivitis arched palate, flat maxilla, and angulated mandible with a recessed
The child with possible allergic rhinitis has a history of sneezing, itch- chin and dental malocclusion (Figure 95.1). If affected, the conjunc-
ing, nasal discharge, and nasal blockage. The eyes, ears, palate, and tivae are erythematous with a clear discharge and may have a follic-
throat may itch. The child may also have a history of mouth breathing ular appearance. The mouth may reveal a high-arched palate from
and snoring at night, sleep disturbances, and daytime fatigue from chronic mouth breathing, and hypertrophic lymphoid follicles in the
nasal obstruction. Other signs include sinusitis, postnasal drip, hal- oropharynx often are seen.
itosis, cough, and morning sore throat. Symptoms may be seasonal Classification of allergic rhinitis, although not as stringent as that
or associated with a specific stimulus. Additionally, systemic symp- of asthma, is important because it guides the choice of optimal ther-
toms of fatigue, headache, anorexia, and irritability may be present. apy. According to the World Health Organization Allergic Rhinitis
The history should also include a search for other manifestations and its Impact on Asthma guidelines, allergic rhinitis is considered
of allergies (eg, wheezing, atopic dermatitis). Approximately 40% to be intermittent if symptoms occur fewer than 4 days a week or
of children who present with allergic symptoms also have asthma, fewer than 4 weeks in duration, whereas persistent rhinitis is defined
50% have atopic dermatitis, and about 30% have allergic rhinitis. as symptoms occurring 4 or more days a week or 4 or more weeks

A B C

Figure 95.1. Characteristic facial features in children with allergic diseases. A, Allergic shiner. B, Allergic salute. C, Adenoid facies.

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 Chapter 95: Allergic Disease 691

in duration. Further, mild allergic rhinitis is defined as having no many hours to days after exposure have been reported. Therefore,
sleep disturbances; normal activities, sports, and leisure; normal the history must be inclusive of this time frame.
school or work; and no troublesome symptoms. One or more of these Hemodynamically, patients have reduced peripheral resistance
symptoms results in classification of moderate/severe persistent aller- resulting from vasodilation and capillary leak. This results in warm
gic rhinitis (Table 95.1). Thus, allergic rhinitis may be classified as skin and flushing. Compensatory increases in cardiac output result
mild intermittent, moderate/severe intermittent, mild persistent, and in tachycardia and bounding pulses. Patients often feel a sense
moderate/severe persistent. of doom. Hypotension ensues if shock is untreated. Respiratory
Complications of chronic allergic rhinitis may be evident on symptoms include airway edema with upper airway obstruction
physical examination, including chronic serous otitis, recurrent oti- and stridor, and smooth muscle constriction results in wheez-
tis media, hearing loss secondary to otitis, sinusitis, nasal polyps, ing and respiratory distress. Some patients have GI symptoms,
sleep apnea, or dental malocclusion. including vomiting, abdominal discomfort or pain, and diarrhea.
Most patients with anaphylaxis have skin manifestations (typically
Urticaria and Angioedema
urticaria).
Because the most common causes of urticaria and angioedema The most common cause of death from anaphylaxis is respira-
in children are foods, medications, supplements, and viral infec- tory compromise followed by hemodynamic collapse.
tions, questions should focus on recent exposures to drugs, dietary
changes, new soaps or detergents, environmental agents, and recent Food Allergies
viral illnesses. Consideration should also be given to other seri- Diagnosing food allergy is not difficult if a reaction is clearly asso-
ous conditions, such as collagen vascular disease and neoplasm. ciated with ingestion of a specific food. Timing of the reaction is
Laboratory tests usually are not required unless the history and usually minutes to a few hours after ingestion. Diagnosis is less
physical examination are suggestive of a potential etiology; for clear after a meal with multiple ingredients. Maintaining an accu-
example, a patient with urticaria, joint swelling, pallor, and fatigue rate diet diary helps narrow the list of potential reactive foods.
requires testing for evidence of systemic lupus erythematosus and Negative food allergy test results via skin or in vitro serum IgE are
malignancy. reliable in ruling out a food; however, positive test results have lim-
ited predictive value unless values are high (Table 95.2). Therefore,
Anaphylaxis
a positive test result is suggestive of a food being responsible for
In the patient with anaphylaxis, the history is focused on identify- a reaction, and confirmation should be pursued via an elimina-
ing an acute exposure to a foreign antigen (eg, medication, food, tion period followed by food challenge. The double-blind, placebo-
venom). Although most patients react within minutes to hours after controlled food challenge is the diagnostic standard; however, it
exposure to the causative agent, anaphylactic reactions occurring is impractical in most community clinical settings. National food
allergy guidelines recommend using open food challenges to con-
Table 95.1. Classification of Allergic Rhinitis firm a diagnosis. An absolute exception is in the patient with a
serious systemic reaction (eg, anaphylaxis), in which case a strong
Frequency Severity
history and a positive skin or serum test result is sufficient for
Not applicable Mild: Moderate/severe (≥1 of diagnosis.
No sleep disturbances the following):
No impairment of Sleep disturbance
activities, sports, leisure Impairment of activities, Table 95.2. Specific Food Immunoglobulin E Levels
Normal school and work sports, leisure and Likelihood of Clinical Reactivity
No troublesome Abnormal school and Food IgE (kU/L) PPV (%) NPV (%)
symptoms work Egg 7 98 36
Troublesome symptoms Egg (<2 years of age) 2 95 —
Intermittent Mild intermittent: same Moderate/severe inter- Milk 15 95 53
(<4 days/week symptoms as above mittent: same symptoms
Milk (<1 year of age) 5 95 —
or <4 weeks’ as above
duration) Peanut 14 99 36
Persistent Mild persistent: same Moderate/severe persis- Soy 30 73 82
(≥4 days/week symptoms as above tent: same symptoms Fish 20 99 89
and ≥4 weeks’ as above Wheat 26 74 87
duration) Abbreviations: IgE, immunoglobulin E; NPV, negative predictive value; PPV, positive predictive
Derived from Brożek JL, Bousquet J, Agache I, et al. Allergic Rhinitis and its Impact on Asthma (ARIA) value.
guidelines—2016 revision. J Allergy Clin Immunol. 2017;140(4):950–958 PMID: 28602936 Adapted with permission from Adkinson NF Jr, Bochner BS, Burks AW, et al, eds. Middleton’s
https://doi.org/10.1016/j.jaci.2017.03.050. Allergy: Principles and Practice. 8th ed. Philadelphia, PA: Elsevier Saunders; 2013.

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Laboratory Tests Although serum IgE levels are elevated in 60% of patients with allergic
The diagnosis of an allergic disease can be made with a thorough rhinitis and asthma, they are not sensitive or specific and have limited
history and physical examination when there is resolution of value. Rhinoscopy in adults and older children provides a painless
symptoms with empiric therapy. Additional tests are performed method to detect pathology not visible by anterior inspection via the
when the diagnosis is in question, to provide optimal avoidance nares. Such pathology includes identification of nasal polyps, deviated
strategies, and if allergen immunotherapy is needed. Testing may nasal septum, adenoidal hypertrophy extending into the nares, sinus-
be necessary for the diagnosis of food allergy and further evaluation itis, vocal cord edema, and polyps and potential masses. Computed
of urticaria and angioedema. tomography is clinically quite useful because it reveals structural
History of exposure followed by symptoms can be suggestive of abnormalities and mucosal disease, including findings within sinus
causative agents; however, the combination of allergy testing with cavities. The need for such information must be weighed against the
history improves the positive predictive value of a specific anti- high doses of radiation associated with computed tomography. Plain
gen causing the symptoms. For example, a patient with year-round radiography of the sinuses is rarely indicated because of the limited
indoor symptoms of rhinorrhea and nasal pruritus may be allergic ability to detail nasal anatomy and the high rates of false-negative
to dust mites, cats, dogs, cockroaches, or mold. Subsequent allergen results for sinusitis along with exposure to radiation.
testing will elucidate which single allergen or combination of aller-
Management
gens is responsible for the symptoms. This results in a more focused
and effective approach to environmental control. Allergen testing General Principles
is also necessary if allergen immunotherapy is being considered. Avoidance of allergen triggers is a natural means of controlling
Allergy testing may be achieved with in vivo skin-prick testing or allergy symptoms without medication and should be encouraged
in vitro specific IgE testing. Skin tests are sensitive and accurate. A in all patients with allergic disease. Currently, avoidance of aller-
positive test result indicates the presence of an antigen-specific IgE. gen triggers is the only option available to patients with food allergy
Although skin testing may be less reliable in infants because their and anaphylaxis.
skin is less reactive than that of older individuals, these tests have For patients with allergic rhinitis, conjunctivitis, and asthma,
been performed in infants as young as 4 months. Typically, fewer allergic triggers consist of perennial and seasonal allergens. Perennial
allergens are tested in children younger than 2 years compared with allergens usually include house dust mites, warm-blooded animals
older individuals because those younger than 2 years of age have not with fur (eg, cats, dogs, rodents), cockroaches, and indoor pollens
been sensitized to a wide range of antigens. Currently, skin-prick (usually molds). Seasonal allergens include outdoor molds as well as
testing is the recommended method for skin tests. Intradermal test- pollen from trees, grasses, and weeds. Effective environmental con-
ing involving injection of allergen under the skin is used for testing trols for these are outlined in Box 95.3. In addition to allergic trig-
of stinging insect allergy and various medication hypersensitivity gers, nonallergic irritant triggers, such as tobacco smoke, automobile
and rarely is necessary for the diagnosis of inhalant or food aller- exhaust, smog, and perfumes, should be avoided.
gen sensitization. When evaluating food allergy, intradermal test- Although environmental controls are safe and effective, they may
ing should not be performed because of the risk of precipitating a be labor intensive, expensive, and, in rare cases, psychologically detri-
systemic reaction. mental. For instance, removal of carpeting is difficult and expensive,
In vitro allergen-specific IgE concentrations (ImmunoCAP, radio- especially in rented housing. Dust mite-proof coverings are expensive
allergosorbent tests) in sera, which are also available for the labora- and cumbersome to wash in hot water weekly. Removal of family pets
tory assessment of allergies, provide a measure of the amount of IgE may result in psychological issues if children have become attached.
specific for individual allergens. Generally, in vitro tests have simi- Patients are more likely to institute environmental controls if evidence
lar sensitivity and specificity as skin testing but are more expensive. exists of allergic sensitization via skin-prick or blood tests.
In vitro tests have an advantage in that they can be performed on Currently, no therapy is available to treat patients with food aller-
patients who are using medications that affect skin-testing reactivity, gies except avoidance of the triggering food. Food avoidance should
such as antihistamines and tricyclic antidepressants. Additionally, be undertaken only after a careful history, allergy testing, and a trial
they are the preferred test in patients with poor skin reactivity, such of food elimination and challenge to correctly identify the trigger. A
as the very young and very old; those who do not have skin findings, poor history and false-positive testing can result in the implication of
such as atopic dermatitis and dermographism; and those who may many foods, a draconian elimination diet, and undernutrition. The
have anaphylaxis to the allergens being tested. patient who is truly allergic to multiple foods will benefit from con-
For inhalant allergens, in vivo and in vitro tests have good neg- sultation with a registered nutritionist or dietitian. Additionally, such
ative and positive predictive values. Both tests have good negative patients should be taught to read food labels to avoid offending foods.
predictive value for food allergy but positive predictive value of Children generally lose sensitivity to milk, soy, egg, and wheat
approximately 50%. For certain foods, very high levels of specific by school age. For instance, a large study noted that 85% of milk-
IgE have good positive predictive values (Table 95.2). allergic and 66% of egg-allergic children lost their sensitivity by
Other screening tests may include a nasal smear for eosinophil 5 years of age. Only approximately 20% of patients with peanut
counts. More than 10% eosinophils is consistent with allergic rhinitis. allergy lose their sensitivity, and even fewer become tolerant of fish

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 Chapter 95: Allergic Disease 693

to 1:10,000. Because of the significant cardiac effects of IV epineph-

Box 95.3. Environmental Control Strategies for
rine, however, cardiac monitoring must be instituted. Because vaso-
Perennial and Seasonal Allergens
active mediators result in capillary leak of fluids into the peripheral
Dust Mites tissue, rapid volume expansion with normal saline or similar colloids
ww Cover mite reservoirs with mite-proof covers (eg, mattress, pillows, box should be instituted early, especially if response to epinephrine is
springs, comforter). poor. Administration of epinephrine must not be delayed; most
ww Wash linen in hot water once a week. deaths from anaphylaxis have been associated with delayed epi-
ww Remove mite reservoirs, such as carpets, and if possible, stuffed toys. nephrine administration.
ww Use HEPA filter in conjunction with mite reduction strategies. Oxygen therapy should be instituted and bronchodilator drugs
ww Use vacuum cleaner that has HEPA filtration. (eg, albuterol) given for bronchospasm and wheezing. H1 and H2
Pets (eg, dogs, cats) receptor antagonists, such as diphenhydramine and ranitidine,
ww Do not get a warm-blooded furry pet (even if initially not allergic to cats respectively, usually are given as well as systemic corticosteroids,
or dogs), because children may sensitize to the pet later, at which time it such as methylprednisolone. Intravenous administration of these
will be difficult to remove the pet from the home. drugs ensures systemic delivery, especially if the patient is vomiting.
ww Bathing the pet once a week reduces the allergen load. However, antihistamines and corticosteroids are rapidly absorbed
ww Keep the child’s room pet free or, if possible, have an outside cat or dog. orally, and absorption of diphenhydramine is also good intramus-
ww Use of HEPA filter may help. cularly. These routes should be considered if IV access cannot be
ww Re-home the pet, if possible. (Note: Cat dander may linger for many established in a timely fashion.
months, and continuous house cleaning is required even after removal Because of the risk of a late-phase reaction or a biphasic reaction,
of the pet.) the patient with anaphylaxis must be observed for a period of time,
usually 4 to 8 hours. Administration of systemic corticosteroids and
Cockroaches/Rodents antihistamines does not adequately reduce the risk of late-phase reac-
ww Use of boric acid for cockroach control is a pesticide-free strategy. tions. The patient must be discharged with an epinephrine auto-injector
ww Cockroaches and rodents often require professional extermination. and taught how to use it (0.15 mg for patients weighing <29.9 kg
Mold [<66 lb]; 0.3 mg for patients weighing ≥29.9 kg [≥66 lb]). Inpatient
ww Repair water damage. observation is appropriate for patients experiencing severe reactions
ww Reduce humidity. or who need prolonged resuscitation, when triggers are unclear, if it
Pollens is difficult to obtain an epinephrine auto-injector, and if the parents
ww Use HEPA filter. are not capable of recognizing anaphylactic symptoms or administer-
ww Close windows at night (plants usually pollinate in early morning). ing treatment. Medical alert bracelets should be issued if the offend-
ing agent is known.
Abbreviation: HEPA, high-efficiency particulate air. Management of urticaria and angioedema should begin with
the identification and elimination of the causal agent. Offending
and shellfish. Understanding the natural history of food allergies foods or medications should be avoided, underlying infections
allows the clinician to liberalize the diet if retesting shows negative should be managed, and more serious etiologies, such as collagen
or reduced reactivity and a supervised food challenge is negative. The vascular disease and malignancy, should be worked up and man-
decision to challenge depends on the nutritional and social impor- aged. Medications are required to manage bothersome symptoms
tance of a particular food. and include H1 and H2 receptor antagonists and, in severe or persis-
Many patients with FPIES, allergic proctocolitis, and Heiner syn- tent cases, corticosteroids. Tricyclic antidepressants are also used
drome also outgrow their food hypersensitivities. The natural history in adults and older children because of the potent antihistamine
of eosinophilic esophagitis is less clear. Rechallenge to more severe effects of these agents.
diseases, such as FPIES, must be performed in a monitored setting
with staff who are competent in resuscitation. Medications
Anaphylaxis is a medical emergency. Rapid reversal of cardio- Avoidance of allergens and other triggers is sufficient in mild disease,
vascular or respiratory compromise is essential. Epinephrine is the but often, addition of medications is necessary when symptoms
first agent given to increase systemic vascular resistance, reverse persist or are more severe.
bronchospasm, and reduce airway edema. A 0.01 mL/kg per dose Antihistamines or H1 receptor antagonists have been used exten-
of 1:1,000 aqueous solution is given intramuscularly in the lateral sively in the management of allergic diseases and are effective in
thigh. This route achieves high serum concentrations in the short- reducing symptoms of pruritus, rhinorrhea, and sneezing. They are
est time compared with other routes. Subcutaneous injection is less effective in relieving nasal congestion, although many patients
no longer recommended. If symptoms progress or do not improve, report that their nasal congestion improves with these agents. These
epinephrine may be repeated in 5 to 10 minutes. Refractory symp- agents are also effective in reducing hives, swelling, and pruritus in
toms have been managed with intravenous (IV) epinephrine diluted urticaria and angioedema. First-generation antihistamines include

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 694 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

diphenhydramine, chlorpheniramine, and brompheniramine; atopic dermatitis when symptoms are not controlled using avoid-
although these agents are effective, they have significant sedating ance strategies and medications. This treatment is effective for symp-
side effects. Even when administered at bedtime, patients show toms caused by dust mites, pollens, animal dander, molds, and insect
diminished concentration and attention the next day; sedating venom. Immunotherapy involves a series of injections with extracts
antihistamines can impair learning in schoolchildren. Newer H1 of allergens specific for individual patients, producing tolerance to
receptor antagonists, such as loratadine, cetirizine hydrochloride, particular antigens. The mechanism of action of immunotherapy is
and fexofenadine hydrochloride, are less sedating and should be related to the development of allergen-specific blocking antibody
used as first-line agents in children. Some patients report decreased (ie, IgG), increased allergen-specific T regulatory cells, decreased
efficacy of these less-sedating antihistamines compared with older lymphocyte cytokine response to an allergen, and decreased baso-
ones. Newer intranasal antihistamines, such as desmethylazelas- phil histamine release in response to an allergen. The results of skin
tine, have a rapid onset of action, are effective, and are quite safe testing dictate which allergens to use. Initially, injections are given
but also are sedating. weekly with increasing doses until maintenance concentrations are
Anti-inflammatory agents include intranasal corticosteroids, achieved, at which point the injections are given once every 4 weeks.
intranasal cromolyn sodium, and systemic corticosteroids. Intranasal Immunotherapy is given in a supervised setting with an obser-
corticosteroids have become the mainstay in the management of vation period of approximately 20 minutes, because of the small
allergic rhinitis because of their powerful, broad anti-inflammatory possibility for anaphylaxis.
properties and excellent safety profile. They are effective in reduc- In Europe, immunotherapy using sublingual delivery of anti-
ing pruritus, rhinorrhea, sneezing, and congestion when used on a gens has been used effectively for many years without the need
chronic preventive basis. Topical intranasal therapy has minimal to for injections. In the United States food allergen-specific therapies
no effects on the hypothalamic-pituitary-adrenal axis when newer currently under investigation include oral immunotherapy (OIT),
corticosteroids, such as fluticasone propionate and mometasone sublingual immunotherapy (SLIT), and subcutaneous immuno-
furoate, are used. Older agents, such as beclomethasone dipropio- therapy (SCIT). Early clinical trials for SCIT have shown efficacy
nate, have more bioavailability, and children may experience small in inducing oral tolerance to peanut allergy; however, most ther-
but significant growth delays. Cromolyn sodium is a mast cell stabi- apies have not been studied in humans. Oral immunotherapy and
lizer and has almost no side effects. Clinical efficacy is poor, however, SLIT have been shown to have lower rates of systemic reactions than
and it is now used infrequently. Although most patients with aller- SCIT. Moreover, although most studies have reported that OIT has
gic rhinitis do not require systemic corticosteroids, some patients a higher efficacy for desensitization than SLIT, OIT has been found
who are refractory to treatment may benefit from a short course to have a higher incidence of side effects.
of oral corticosteroids, such as prednisone. Systemic steroids are Additionally, epicutaneous immunotherapy, which solubilizes
usually used to control urticaria and angioedema and are mandatory the allergen and enters via the stratum corneum, has been shown
in the management of anaphylaxis. to be effective for cow’s milk protein and peanut allergy and is in
Alpha-adrenergic agents are used systemically as well as topically preclinical studies for egg allergy. Studies suggest that the risk for
in the management of rhinitis. Oral agents, such as pseudoephedrine systemic reactions may be lower with epicutaneous immunotherapy
and phenylephrine, are effective but cause α-adrenergic side effects, than with SCIT, OIT, or SLIT.
such as tachycardia and jitteriness. Topical agents, such as oxymetazo-
line hydrochloride, are potent vasoconstrictors and can dramatically Caveats About Treatment
improve nasal obstruction; however, routine use for more than 3 to Because patients with allergic rhinitis are repeatedly exposed to
5 days results in rebound congestion with persistent and worse symp- allergens, they may require use of anti-inflammatory agents, such
toms (ie, rhinitis medicamentosa). as intranasal corticosteroids, as first-line therapy. Current guidelines
Leukotrienes have biologic effects similar to those of hista- for the United States recommend anti-inflammatory agents as first-
mine and also recruit inflammatory cells into tissue. Therefore, line therapy for moderate/severe persistent disease and can be con-
the leukotriene receptor antagonist montelukast sodium can be sidered for mild persistent and moderate/severe intermittent disease.
used as monotherapy for the management of allergic rhinitis. A patient may require long-term use of anti-inflammatory agents in
Intranasal corticosteroids have better efficacy than montelukast combination with antihistamines (Figure 95.2).
sodium, but the latter is an oral agent that promotes better com- Allergic conjunctivitis often coexists with allergic rhinitis.
pliance. Additionally, allergic rhinitis is often comorbid with Control of nasal symptoms may result in improvement of eye
asthma, and montelukast sodium has the advantage of treating symptoms, but ocular medications may also be required. Available
both conditions. medications include antihistamines, mast cell stabilizers, vasocon-
Normal saline washes provide a medication-free adjunctive treat- strictors, and combination agents. The sight-threatening allergic
ment for allergic rhinitis, and their use has increased in popular- ocular conditions of vernal conjunctivitis and atopic keratocon-
ity among patients. junctivitis (both of which may cause corneal ulcerations) require
Immunotherapy is quite effective for the long-term management prompt referral to an ophthalmologist.
of allergic rhinitis, allergic conjunctivitis, and stinging insect venom In mild and sporadic cases of urticaria and angioedema, use of
hypersensitivity and is also recommended for allergic asthma and antihistamines is sufficient. With frequent and persistent eruptions,

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 Chapter 95: Allergic Disease 695

Intermittent Persistent

Mild Moderate/Severe Mild Moderate/Severe

(Not in preferred order) (Not in preferred order) (In preferred order)

Oral antihistamine Oral antihistamine Intra-nasal
Or Or corticosteroid
Intra-nasal antihistamine Intra-nasal antihistamine Antihistamine or
And/or And/or Montelukast
Decongestant Decongestant
Or Or Addition of
Montelukast Intra-nasal corticosteroid decongestant
Or if needed
Montelukast

Figure 95.2. Allergic rhinitis treatment algorithm.

Adapted from Bousquet J, Schünemann HJ, Samolinski B, et al; World Health Organization Collaborating Center for Asthma and Rhinitis.
Allergic Rhinitis and its Impact on Asthma [ARIA]: achievements in 10 years and future needs. J Allergy Clin Immunol. 2012;130[5]:
1049–1062, with permission.

however, the routine use of H1 blockers with the addition of H2 block- In 2000, the American Academy of Pediatrics recommended
ers may be required. In more severe and refractory cases, older first- delaying the introduction of certain highly allergenic foods in
generation antihistamines are necessary, with maximal dosages. In high-risk children based on early studies suggesting that delay may
adults and older children, tricyclic antidepressants, such as doxepin help prevent certain allergic diseases, specifically atopic dermati-
hydrochloride, can be added when high doses of H1 and H2 receptor tis. Recent studies have suggested, however, that delayed introduc-
antagonists do not provide relief. Systemic steroids are effective in tion of solid foods may not only increase the risk of allergy, but that
controlling symptoms of urticaria and angioedema; symptoms may early introduction of certain foods (eg, egg, peanut) between 4 and
recur after stopping, however, and patients may become dependent 6 months of age may decrease the risk of allergy to that food. An
on them for relief. interim guideline on the early introduction of peanut for infants at
Chronic urticaria and angioedema may last months to years, various risk levels was published in 2017 based on the Learning Early
some in waxing and waning fashion. The patient with chronic idio- About Peanut allergy (LEAP) trial and other studies (Table 95.3);
pathic urticaria and angioedema must be counseled that the disease formal guidelines have yet to be developed, however.
is not caused by an external agent. The aforementioned medications Strategies to prevent development of inhalant allergies have not
must be used long term to allow these patients to lead symptom-free been universally effective. For the patient at risk for the development
lives until the pathology self-extinguishes. of allergic rhinitis and asthma, early avoidance of allergens is reason-
able; however, this may merely delay sensitization until the patient is
Prevention older. Strategies include removal of dust mite reservoirs (eg, carpets),
The cost of medical care, lost school days and workdays, disability covering of mattresses and pillows with mite-proof covers, not hav-
from complications, and lives lost from allergic disease take a great toll ing a warm-blooded furry pet, and repair of water damage to reduce
on the population. Prevention significantly reduces the health burden. mold growth. Prevention of tobacco smoke exposure is essential.
Currently, evidence suggests that sensitization to foods can occur Recently, the hygiene hypothesis has received attention. In
in the first 6 to 12 months after birth and even in utero. Strategies this paradigm, exposure to bacterial components, such as endo-
to avoid allergic foods during the third trimester and first year after toxin, results in a natural shift of lymphocytes from IgE-facilitating
birth have not diminished the prevalence of food allergies. Although T helper type 2 cells and T helper type 1 cells. Global shifts away
breastfeeding is still recommended as the optimal form of nutrition from endotoxin-exposing agrarian socie­ties to more sterile urban
for infants in the first 6 months after birth and studies have suggested societies reduces this shift and may result in the increase in atopy
mother’s milk to have positive immune effects, data are insufficient worldwide. A common misconception is that the child exposed to
to support strong associations between exclusive feeding of moth- a dirty environment with multiple pets, respiratory infections, and
er’s milk and decreased development of specific allergic diseases dirt will be protected against atopy. The endotoxin-facilitated shift
(eg, eczema, allergic rhinitis). of T helper type 2 cells and T helper type 1 cells is likely to occur

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 696 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Table 95.3. Recommendations for the Introduction Selected References

of Peanuts in Allergic and Non-Allergic Infants Adkinson NF Jr, Bochner BS, Busse WW, Holgate ST, Lemanske RF Jr, Simons
Extent of allergic Earliest age of FER, eds. Middleton’s Allergy: Principles and Practice. 8th ed. St. Louis, MO:
symptoms Recommendation peanut introduction Mosby Elsevier; 2013
Severe eczema, Strongly consider evaluation by 4-6 months Armogida SA, Yannaras NM, Melton AL, Srivastava MD. Identification and quan-
egg allergy, or serum (peanut-specific IgE) and/ tification of innate immune system mediators in human breast milk. Allergy
both or skin prick test and if necessary Asthma Proc. 2004;25(5):297–304 PMID: 15603202
an oral food challenge. Introduce Bousquet J, Schünemann HJ, Samolinski B, et al; World Health Organization
peanut based on results. Collaborating Center for Asthma and Rhinitis. Allergic Rhinitis and its Impact
on Asthma (ARIA): achievements in 10 years and future needs. J Allergy Clin
Mild to moderate Introduce peanut-containing Around 6 months Immunol. 2012;130(5):1049–1062 PMID: 23040884 https://doi.org/10.1016/j.
eczema foods jaci.2012.07.053
No eczema or no Introduce peanut-containing Age-appropriate Boyce JA, Assa’ad A, Burks AW, et al; NIAID-Sponsored Expert Panel. Guidelines
food allergy foods and in accordance for the diagnosis and management of food allergy in the United States: report
with family prefer- of the NIAID-sponsored expert panel. J Allergy Clin Immunol. 2010;126
ences and cultural (6 suppl):S1–S58 PMID: 21134576 https://doi.org/10.1016/j.jaci.2010.10.008
practices Du Toit G, Roberts G, Sayre PH, et al; LEAP Study Team. Randomized trial
of peanut consumption in infants at risk for peanut allergy. N Engl J Med.
Derived from DuToit G, Roberts G, Sayre PH, et al. Randomized trial of peanut consumption in
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infants at risk for peanut allergy. N Engl J Med. 2015;372:803-813.
Ierodiakonou D, Garcia-Larsen V, Logan A, et al. Timing of allergenic food intro-
duction to the infant diet and risk of allergic or autoimmune disease: a system-
in a narrow window in utero and during the first year after birth, atic review and meta-analysis. JAMA. 2016;316(11):1181–1192 PMID: 27654604
however. Thus, exposure of older children to a dirty environment https://doi.org/10.1001/jama.2016.12623
is unlikely to protect against atopy, and exposure of children with Jones SM, Sicherer SH, Burks AW, et al; Consortium of Food Allergy Research.
allergic disease exacerbates symptoms. Epicutaneous immunotherapy for the treatment of peanut allergy in children
and young adults. J Allergy Clin Immunol. 2017;139(4):1242–1252.e9 PMID:
The method for preventing progression of allergic disease,
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sometimes referred to as allergic march, is immunotherapy.
Keet CA, Frischmeyer-Guerrerio PA, Thyagarajan A, et al. The safety and effi-
Immunotherapy against inhalant allergens in the child with allergic
cacy of sublingual and oral immunotherapy for milk allergy. J Allergy Clin
rhinitis significantly decreases the risk of asthma. The need for
Immunol. 2012;129(2):448–455.e5 PMID: 22130425 https://doi.org/10.1016/j.
weekly, then monthly injections reduces the acceptance of this jaci.2011.10.023
method, but the approval of SLIT may improve patient compliance.
Kwong KY, Leibel S. Update on allergen immunotherapy for treatment of aller-
gic diseases. Adv Pediatr. 2013;60(1):141–165 PMID: 24007843 https://doi.
Prognosis org/10.1016/j.yapd.2013.04.008
Prognosis for the child with allergic disease is good. Some patients Lee S, Bellolio MF, Hess EP, Erwin P, Murad MH, Campbell RL. Time of onset
lose their allergy naturally or following immunotherapy. For oth- and predictors of biphasic anaphylactic reactions: a systematic review and meta-
ers, allergic rhinitis, urticaria, and anaphylactic reactions persist analysis. J Allergy Clin Immunol Pract. 2015;3(3):408–416.e1-2 PMID: 25680923
https://doi.org/10.1016/j.jaip.2014.12.010
throughout life on exposure to antigens. With appropriate manage-
ment, however, the child can thrive and live a normal life. Methods Lieberman P, Kemp SF, Oppenheimer J, et al; American Academy of Allergy,
Asthma, and Immunology; American College of Allergy, Asthma and
to prevent development of inhalant and food allergies are less well
Immunology; Joint Council of Allergy, Asthma and Immunology Joint Task
understood; however, current research is focusing on strategies to Force on Practice Parameters. The diagnosis and management of anaphylaxis:
prevent the development of inhalant and food allergies. an updated practice parameter. J Allergy Clin Immunol. 2005;115(3 suppl 2):
S483–S523 PMID: 15753926 https://doi.org/10.1016/j.jaci.2005.01.010
Meltzer EO, Blaiss MS, Derebery MJ, et al. Burden of allergic rhinitis: results
CASE RESOLUTION from the Pediatric Allergies in America survey. J Allergy Clin Immunol. 2009;124
The symptoms of rash, swelling, and wheezing after exposure to an antigen (3 suppl 1):S43–S70 PMID: 19592081 https://doi.org/10.1016/j.jaci.2009.05.013
are suggestive of an anaphylactic reaction. Treatment with epinephrine, anti- Patel P, Philip G, Yang W, et al. Randomized, double-blind, placebo-controlled
histamines, and systemic corticosteroids is clearly indicated. The girl should be study of montelukast for treating perennial allergic rhinitis. Ann Allergy Asthma
observed for the occurrence of late-onset reactions. The child and family should be Immunol. 2005;95(6):551–557 PMID: 16400895 https://doi.org/10.1016/
counseled to avoid any foods that contain peanuts. The parents should read all food S1081-1206(10)61018-6
labels and carry an epinephrine auto-injector (eg, EpiPen) at all times for emer-
gency use. A medical alert bracelet indicating peanut allergy should be ordered Platts-Mills TA. Allergen avoidance. J Allergy Clin Immunol. 2004;113(3):
and the patient and parents advised that she should always wear it. The patient 388–391 PMID: 15007333 https://doi.org/10.1016/j.jaci.2003.12.027
should be referred to her primary care physician, who should consider referral to Rudders SA, Banerji A, Vassallo MF, Clark S, Camargo CA Jr. Trends in pediatric
an allergist for further evaluation. emergency department visits for food-induced anaphylaxis. J Allergy Clin Immunol.
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Sicherer SH, Allen K, Lack G, Taylor SL, Donovan SM, Oria M. Critical parameter. J Allergy Clin Immunol. 2008;122(2 suppl):S1–S84 PMID: 18662584
issues in food allergy: a National Academies consensus report. Pediatrics. https://doi.org/10.1016/j.jaci.2008.06.003
2017;140(2):e20170194 PMID: 28739655 https://doi.org/10.1542/peds.2017-0194 Wander AA, Bernstein IL, Goodman DL, et al. The diagnosis and management
Togias A, Cooper SF, Acebal ML, et al. Addendum guidelines for the prevention of urticaria: a practice parameter. Ann Allergy Immunol. 2005;85:525–544
of peanut allergy in the United States. report of the National Institute of Allergy
Wang J, Sampson HA. Food anaphylaxis. Clin Exp Allergy. 2007;37(5):651–660
and Infectious Diseases-sponsored expert panel. Pediatr Dermatol. 2017;34(1):
PMID: 17456212 https://doi.org/10.1111/j.1365-2222.2007.02682.x
e1–e21 PMID: 28054723 https://doi.org/10.1111/pde.13093
Zuraw BL, Christiansen SC. Pathogenesis and laboratory diagnosis of heredi-
Wallace DV, Dykewicz MS, Bernstein DI, et al; American Academy of Allergy,
tary angioedema. Allergy Asthma Proc. 2009;30(5):487–492 PMID: 19843402
Asthma & Immunology; American College of Allergy, Asthma and Immunology;
https://doi.org/10.2500/aap.2009.30.3277
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Parameters. The diagnosis and management of rhinitis: an updated practice

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 CHAPTER 96

Wheezing and Asthma

Kenny Y.C. Kwong, MD, and Nasser Redjal, MD

CASE STUDY
A 7-year-old boy is referred to the office after being seen Questions
in the emergency department for wheezing. He has 1. What are the most common causes of wheezing in
been treated in the emergency department for wheez- infants and children?
ing 4 times in the past month and was once hospitalized 2. What are the causes of reversible bronchospasm?
for 3 days. The boy’s father and paternal grandmother 3. What is the pathophysiology of reversible
both have asthma. bronchospasm?
The child’s physical examination is remarkable for 4. How should the child with asthma be treated?
end-expiratory wheezing on forced expiration.

Recurrent wheezing is a frequent symptom of obstructive airway with obstruction are caused by turbulence of the air as it is forced
disease in children that may be caused by intrinsic or extrinsic through a narrowed airway. Infants and young children are more
compression of the airway, bronchospasm, inflammation, or defec- prone to wheezing when they have airway obstruction because air
tive clearance of secretions. Ten percent to 15% of infants wheeze forced through smaller airways is more turbulent than air forced
during the first year after birth, and as many as 25% of children through the larger airways of older children and adults. Infection-
younger than 5 years present to their physician with wheezing dur- induced wheezing in children younger than 2 years is associated
ing a respiratory illness. Most infants and young children with recur- with RSV, especially in infants with passive exposure to smoke, and
rent wheezing have asthma; however, a wide variety of congenital with rhinovirus in children older than 2 years. The most common
and acquired conditions can cause narrowing of the extrathoracic causes of wheezing in infants and children are asthma, bronchiolitis,
or intrathoracic airways and may present with wheezing. Reactive and pneumonia. Less common causes include congenital structural
airway disease is the most common cause of wheezing in childhood. anomalies, gastroesophageal reflux and aspiration, cardiac failure,
Childhood asthma typically falls into 1 of 3 categories: transient cystic fibrosis, foreign bodies, and vocal cord dysfunction (Box 96.1).
wheezing, late transient wheezing, and atopic wheezing. The modified Asthma Predictive Index (mAPI) is a clinical
Transient wheezing occurs in infants who are born with smaller instrument used to predict persistence of asthma. Predictive fac-
caliber airways and who wheeze with viral lower respiratory tract tors include wheezing before 3 years of age and the presence of either
infections and bronchiolitis. These infants do not have atopy and 1 major risk factor (ie, parental history of asthma, personal history
usually have no more wheezing by 3 years of age. Most patients of atopic dermatitis, or patient sensitized to aeroallergen) or 2 of
with no atopic predisposition who wheeze in the early years after 3 minor risk factors (ie, patient sensitized to food, wheezing apart
birth fall into this category. Late transient wheezing occurs in chil- from colds, or eosinophilia). The mAPI has a positive predictive
dren who usually have a history of serious lung insult, such as severe value of 76% and a negative predictive value of 95%. More than 80%
respiratory syncytial virus (RSV) infections, and persistent wheez- of infants with a history of wheezing in the first postnatal years do
ing beyond 3 years of age. These children also have no atopy, and not wheeze after 3 years of age.
symptoms usually slowly resolve over time. Atopic wheezing occurs Asthma is a common chronic disorder of the airways character-
in children with a strong atopic predisposition. Such children are ized by variable and recurring symptoms, airflow obstruction, bron-
most likely to develop asthma that persists throughout the school- chial hyperreactivity, and underlying inflammation. Bronchospasm
age years. is reversible spontaneously or with treatment. In some patients, per-
Wheezes can originate from airways of any size, from the large manent alterations in the airway structure, referred to as airway
extrathoracic upper airway to the intrathoracic small airways. In remodeling, occur and are not prevented by or fully responsive to
addition to narrowing or compression of the airway, wheezing currently available treatment. Clinically, asthma is characterized by
requires sufficient airflow to generate airway oscillation and produce recurrent episodes of cough, chest tightness, dyspnea, prolonged
sound. Thus, the absence of wheezing in a patient who presents with expiration, wheezing, hyperinflation of the chest (ie, air trappings),
acute asthma may be an ominous finding suggestive of impending use of accessory chest muscles (ie, retractions), and, in severe cases,
respiratory failure. The audible musical or squeaking sounds noted cyanosis.

699

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 700 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

absenteeism. Over the past several decades, the prevalence of

Box 96.1. Causes of Wheezing
asthma has increased worldwide, an increase that varies from 40% in
Infection some areas of the United Kingdom and Australia to 3% in Indonesia,
ww Bronchiolitis China, and India. Asthma prevalence, mortality, and hospitaliza-
ww Pneumonia tion rates are higher in blacks than in whites. One-third of patients
ww Bronchitis initially experience symptoms in the first year after birth, and 80%
ww Laryngotracheobronchitis are diagnosed by the time they reach school age. In the United States,
ww Bacterial tracheitis asthma is a leading diagnosis for children admitted to children’s
ww Toxocariasis hospitals. Hospitalization rates have remained relatively stable,
ww Ascariasis with lower rates in some age groups but higher rates in children
Reactive Airway Disease 0 to 4 years of age. Additionally, asthma is a major cause of school
ww Asthma absence; 23% of school days missed can be attributed to asthma.
ww Exercise-induced asthma The male-to-female ratio is 2:1 until age 10 years and is equal from
ww Anaphylaxis ages 10 to 14 years; after puberty, asthma incidence is greater in
ww Nighttime cough asthma girls and women.
ww Toxic exposure (eg, smoke, organophosphate poisoning) In recent years, the incidence of more serious disease in younger
ww Allergic aspergillosis children and adolescents has increased. Low socioeconomic sta-
tus is associated with an increase in asthma prevalence, morbidity,
Laryngeal Dysfunction
and mortality. Inner-city blacks are most at risk, but studies suggest
Congenital Structural Anomalies
that socioeconomic class and health care disparities only partially
ww Vascular rings
account for these differences.
ww Bronchiectasis
ww Lung cysts
ww Laryngotracheoesophageal cleft Clinical Presentation
ww Tracheobronchomalacia The child with asthma may present with acute symptoms of cough,
Defective Secretion Clearance shortness of breath, prolonged expiration, use of accessory muscles
ww Cystic fibrosis of respiration, wheezing, reports of chest tightness or congestion,
ww Immotile cilia syndrome hyperinflation of the chest, cyanosis, exercise intolerance, tachycar-
dia, and abdominal pain.
Tumor (Mediastinal)
Wheezing may be audible and detected by the parent or guardian
ww Lymphoma
or may not be appreciated until the child is examined by a physician.
ww Teratoma
The child with severe bronchoconstriction may have no wheezing
ww Neuroblastoma
because the flow of air is impeded; however, wheezing may occur
ww Thymoma
after bronchodilator treatment resulting from partial opening of
Chronic Aspiration the airway.
ww Gastroesophageal reflux Some children have cough, which may be nocturnal or recurrent
ww Bulbar palsy as a predominant symptom. Some pediatric patients have symp-
ww Tracheoesophageal fistula toms, such as cough or wheezing, that are precipitated or exacer-
Other Causes bated by exercise (Box 96.2).
ww Bronchopulmonary dysplasia Abdominal pain and vomiting also are common in younger
ww α1-Antitrypsin deficiency children and may be followed by temporary relief of respiratory
ww Pulmonary hemosiderosis symptoms. During an acute asthma attack, a low-grade fever, pro-
ww Sarcoidosis fuse sweating, and fatigue from the hard work of breathing may be
apparent.
Foreign Body
Cardiac Disease
Immunodeficiency Pathophysiology
Asthma is a chronic inflammatory disorder of the airways. The
immunohistopathologic features of asthma include alteration and
denudation of the airway epithelium, thickening of the basement
Epidemiology membrane, fibrotic changes in the subbasement membrane, bron-
Asthma is the most common chronic childhood illness. In 2017, chial smooth muscle hypertrophy, edema and angiogenesis, mast
the National Health Interview Survey noted that 9.5 million cell activation, and inflammatory cell infiltration (ie, neutrophils,
children had asthma. Asthma is the leading cause of emer- lymphocytes, eosinophils), which release mediators such as hista-
gency department (ED) visits, hospital admissions, and school mine, prostaglandin, leukotriene, and major basic proteins. These

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 Chapter 96: Wheezing and Asthma 701

and Chlamydia infections can cause wheezing and persistence of

Box 96.2. Diagnosis of Asthma
asthma. Emotional stress may also play a role in the exacerbation
in the Pediatric Patient
of asthma.
ww Recurrent wheezing The physiological changes involved in asthma occur in 2 phases:
ww Shortness of breath early and late. An immediate response (early phase) to the offend-
ww Exercise intolerance ing agent causes edema and bronchial smooth muscle constriction
ww History of allergies that result in narrowing of the airway and plugging with secretions.
ww History of atopic dermatitis Air is trapped behind the narrowed airways, resulting in altered
ww Nasal polyps gas exchange, increased respiratory rate, decreased tidal volume,
ww History of nighttime cough and increased work of breathing. The late response, which occurs
4 to 12 hours after the initial symptoms, primarily involves infil-
tration of the airways with inflammatory cells. The end pathway in
the disease process is obstruction to airflow. The pathophysiology
changes result in airway hyperreactivity, airflow limitation, respi- of asthma is reviewed in Figure 96.1.
ratory symptoms, and disease chronicity. The limitation to the flow
of air results from acute bronchoconstriction, airway edema, mucus Differential Diagnosis
plug formation, and airway wall remodeling. The unique anatomy
An extensive differential diagnosis of asthma is listed in Box 96.1.
and physiology of the lung in infants compared with adults predis-
Most conditions are differentiated from asthma by the presence of
poses infants to obstructive airway disease. These anatomic differ-
associated symptoms or the child’s response to bronchodilators.
ences include reduced number and size of the alveolar pores and
canals of Lambert, causing deficient collateral ventilation and a pre-
disposition to atelectasis distal to the obstructed airway. Mucous
Evaluation
gland hyperplasia favors increased intraluminal mucus produc- History
tion. Decreased smooth muscle in the peripheral airway results in A thorough history, including how the child and family are coping
less support and narrower airways. Decreased number of fatigue- with the asthma, should be obtained (Box 96.3). If acute respiratory
resistant skeletal muscle fibers in the diaphragm, its horizontal distress is present, an abbreviated history focusing on potential pre-
insertion to the rib cage (versus oblique in adult), and a highly cipitating factors and medication use should be obtained first, sav-
compliant rib cage increase the work of breathing in children. ing the more detailed history for a later time.
Decreased static elastic recoil predisposes to early airway closure
during tidal breathing, resulting in ventilation-perfusion mismatch Physical Examination
and hypoxemia. Vital signs, including pulse oximetry, should be obtained.
Atopy, the genetic predisposition to the development of immu- Objective determination of pulmonary function involves mea-
noglobulin (Ig) E–mediated response to common aeroallergens, is suring the peak expiratory effort. Paradoxical pulse, the differ-
a predisposing factor for developing asthma. At least 50 genes influ- ence between systolic arterial blood pressure during inspiration
ence susceptibility to asthma and its clinical expression. Sites located and expiration, is usually under 10 mm Hg. This difference may
on chromosomes 6p, 12q, 5q, 11q, and 16p are known to be associ- be increased during an acute asthma exacerbation, but the mea-
ated with allergic diseases and encoding for major histocompatibil- surement is difficult in young children. Breath sounds, work of
ity complex, IgE, interferon, and cytokine. breathing, the inspiratory-expiratory ratio, use of accessory mus-
Environmental changes, such as wind, temperature fluctuations, cles, presence of retractions, quality of breath sounds (whether
and increased exposure to allergens or air pollutants (eg, tobacco decreased), presence of prolonged expiration, and quality of
smoke, ozone, sulfur dioxide, nitrogen dioxide), and particulate wheezing should be carefully assessed (Table 96.1). Polyphonic
matter (eg, diesel exhaust, biologic residues [eg, endotoxin]), may wheezing (ie, many different pitches, starting and stopping at
precipitate clinical attacks. Inhalant allergens, particularly indoors, varying points in the respiratory cycle) and cough are strongly
play an important role. The most important allergens are house dust suggestive of asthma. Monophonic wheezing (ie, a single, distinct
mite feces (Der p1 and Der p2), cat allergen (Fel d1), and cock- noise of 1 pitch and starting and stopping at 1 discrete time) and
roach saliva (Bla g2, Bla g4, and Bla g5). Other inhalant allergens, cough should always raise suspicion for large airway obstruction
such as dog dander, outdoor fungus (ie, Alternaria), and some pol- caused by foreign body aspiration, vascular ring, or tracheoma-
lens, also play a role. Infections, particularly respiratory ones, are lacia. The nose and associated nasal passages should be exam-
implicated, influencing not only the exacerbation of asthma but ined for secretions, edema, pallor, and polyps. The skin should
also its inception and persistence. Viral infections, such as RSV, be assessed for eczema and other rashes. The chest should be
parainfluenza, and rhinovirus, are the most frequent precipitants carefully checked for increased anteroposterior diameter of the
of asthma exacerbations in infancy. The frequency of lower tract chest wall, a sign of air trapping. Fingers should be examined
respiratory infection during early childhood with these viruses is a for signs of digital clubbing, which is suggestive of a diagnosis
strong independent factor in development of asthma. Mycoplasma other than asthma.

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 702 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Nonimmunologic stimuli:
Immunologic stimuli:
Viral infections Cell activation
Antigen
Physical and chemical stimuli

Mast cells
Epithelial cells
Macrophages
Eosinophils
Lymphocytes

Autonomic nervous system: Inflammatory mediators

Axon reflexes Smooth muscle constriction
Neuropeptides Chemotaxis

Granulocytic responses:
Neutrophils
Eosinophils
Basophils
Activated mononuclear cells
Macrophages
Lymphocytes

Inflammatory mediators

Airway edema
Cellular infiltration
Subepithelial fibrosis
Mucous secretion
Mucosal and vascular
permeability

Airway hyperresponsiveness

ASTHMA

Figure 96.1. Proposed pathways in the pathogenesis of bronchial inflammation and airway hyperreactivity.
Reprinted from the National Asthma Education and Prevention Program. Expert Panel Report: Guidelines for the Diagnosis and Management of
Asthma. Bethesda, MD: National Heart, Lung, and Blood Institute; 1991. NIH Publication No. 91-3042.

Laboratory Tests running followed by pulmonary function tests can be performed; a

Laboratory assessment of wheezing children is indicated if the decrease greater than 12% in FEV1 or 30% in forced expiratory flow
diagnosis is unclear or to eliminate disorders that mimic asthma. is diagnostic for exercise-induced asthma. Peak flow meters, which
Pulmonary function tests are noninvasive, objective, and cost-effective measure forced peak expiratory flow, are useful in the office and at
in the diagnosis and follow-up of patients with asthma. These tests home to monitor expiratory flow rate. A decrease in peak expira-
can be performed in children older than 5 years with appropriate tory flow may predict the onset of an exacerbation and suggests the
coaching. After the administration of an aerosolized bronchodilator, need for early intervention, using additional drug therapy. A com-
dynamic tests of airflow increase or return to normal. An improve- plete blood cell count with a differential count may be suggestive
ment in forced expiratory volume in 1 second (FEV1) greater than of infection or allergies. Peripheral eosinophil counts may be ele-
12% is nearly diagnostic, but lack of improvement in FEV1 does not vated in asthma. Pulse oximetry assesses the degree of oxygen satu-
preclude asthma. Exercise tolerance tests using a treadmill or free ration. Although not used as a parameter in North American asthma

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 Chapter 96: Wheezing and Asthma 703

of epithelial cells; and Charcot-Leyden crystals, which are derived

Box 96.3. What to Ask
from eosinophils.
Asthma Total serum IgE is not as helpful as antigen-specific IgE in the
ww What symptoms (eg, wheezing, exercise intolerance) does the child diagnosis of asthma and is elevated in 80% of children with allergen-
experience? induced asthma. Allergy skin testing or serologic testing, such
ww Does the child experience any nocturnal awaking or cough? as radioallergosorbent testing, is indicated to identify potentially
ww What time of day and year do the symptoms occur? important environmental allergens. Allergens may play a significant
ww When did the symptoms begin? How old was the child? role in asthma, and 85% of patients with asthma have a positive skin
ww Are the symptoms associated with any particular activity? Does anything test reaction to common aeroallergens. Extensive laboratory testing
seem to trigger the symptoms? should be reserved for the child with severe disease who may benefit
ww Are the child’s activities limited in any way? from consultation with an allergy specialist.
ww How often do asthma attacks occur?
ww What is the child’s living situation? Are there pets in the home? Imaging Studies
ww Does anyone smoke in the home? The child with suspected asthma rarely requires chest radiography
ww Do asthma attacks cause the child to be absent from school? unless concern exists for other pathology. Radiographic findings may
ww Does the child manage the condition at home with any particular range from normal to hyperinflation, increased bronchial marking,
treatments or medications? and atelectasis, especially during acute exacerbation. Infiltration,
ww Has the child visited any urgent care facility or emergency department pneumothorax, pneumomediastinum, and pneumonia are less
for treatment for asthma or related episodes in the past? Has the child common findings.
had any hospitalizations?
ww Does the child have a history of allergies? Management
The long-term goals of asthma therapy are listed in Box 96.4.
Assessment measures for asthma should include monitoring of
guidelines for classification or assessment of asthma control, fractional the following: signs and symptoms, pulmonary function through
exhaled nitric oxide (FeNO) has been shown to help differentiate diag- peak flow or respirometry, quality of life or functional status, acute
nosis of asthma from other conditions that may mimic asthma, such disease exacerbations, pharmacologic therapy, and satisfaction of
as upper airway disease and reflux (ie, chronic cough). Additionally, the child and family with the asthma care.
some specialists use serial FeNO measurements to help wean patients Selecting the appropriate therapy for the patient with asthma
off anti-inflammatory medications. depends on the age, disease severity (Table 96.2), and develop-
Sputum smear, stained with eosin-methylene blue agar, may mental level of the child; tolerance for a specific pharmacologic
show numerous eosinophils and granules from disrupted white agent; and routes of administration. The pharmacologic treatment
blood cells, eosinophils, and epithelial cells. The presence of more for symptom control and reduction of inflammation is predicated
than 5% to 10% eosinophils is suggestive of allergic inflammatory on the severity of the asthma, as categorized according to
disease. Other findings in the sputum include Curschmann spirals, National Heart, Lung, and Blood Institute (NHLBI) guidelines.
which are threads of glycoprotein; Creola bodies, which are clusters Box 96.5 contains an overview of these medications. Quick-relief

Table 96.1. Parameters Used to Estimate the Severity of Acute Asthma

Sign/Symptom Mild Moderate Severe
Respiratory rate Normal Increased Increased >2 standard deviations
Breath sounds Normal (some end-expiratory wheezes) Wheezing in inspiration and Decreased, with or without wheezing
expiration
Shortness of breath None (can speak in sentences) Can speak in phrases Speaks only single words
Skin color Normal Pale Pale to cyanotic
Work of breathing Normal Moderate retractions; some use of Severe retractions, nasal flaring, use of accessory
accessory muscles muscles
Pulse Normal Normal or increased Increased
Level of consciousness Normal Normal Diminished; may be lethargic or combative
Paradoxical pulse <10 mm Hg 10–20 mm Hg 20–40 mm Hg
Oxygen saturation >95% 90%–95% <90%
Pulmonary function 80% of predicted 50%–70% of predicted <50% of predicted

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 704 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

be referred to the ED. Equipment and supplies for resuscitation of

Box 96.4. Long-term Goals of Asthma Therapy
infants and children must be available in offices in which care is pro-
ww Prevent chronic and disabling symptoms (eg, coughing, sleep disturbances, vided for children with asthma.
exercise intolerance, shortness of breath). The goal of therapy is to relieve airflow obstruction and prevent
ww Maintain normal or near-normal pulmonary function. respiratory failure. All children with moderate or severe asthma
ww Maintain normal activity levels. should be placed in a position of comfort and given oxygen by nasal
ww Prevent recurrent exacerbations and minimize the need for emergency prongs or mask as tolerated. Assessment of work of breathing and
department visits and admission to the hospital. the use of pulse oximetry help guide oxygen therapy. Nebulized
ww Provide drug therapy that is effective with minimal side effects. b2 agonists (eg, albuterol) are used until symptoms subside. The
ww Meet the expectations of the child and family for the care of asthma. child may require drug therapy every 20 to 30 minutes. Not only
ww Encourage self-management of asthma. are nebulized b2-adrenergic agonists more effective than oral med-
ications, they are associated with fewer side effects. Ipratropium
bromide used in conjunction with a b agonist in the urgent care
medications (“rescue meds”) are used for acute exacerbations; setting has been shown to reduce risk of hospitalization. Systemic
the long-term control medications are used for chronic therapy. corticosteroids, which help reduce the inflammation associated
Nonpharmacologic measures are used in a preventive fashion and with clinical attacks, are indicated in most moderate and severe
serve as an adjunct to drug therapy. cases. Response to therapy is determined by clinical assessment
of work of breathing, respiratory rate, objective changes in pul-
Short-term Management monary function, and pulse oximetry. The child with incomplete
Acute attacks can be managed in the office if the staff is prepared response to initial therapy may require several hours of treatment
to manage a child in respiratory distress. Otherwise, the child can or hospitalization.

Box 96.5. Pharmacologic Therapy: An Overview of Medications Used to Treat Asthma

Long-term Control Medications (Controller Class)

Corticosteroids
Most potent and effective anti-inflammatory medication currently available. Inhaled form is used in the long-term control of asthma. Systemic corticosteroids
are often used to gain prompt control of the disease when initiating long-term therapy.
Cromolyn Sodium and Nedocromil
Mild anti-inflammatory medication. May be used as monotherapy in the setting of mild persistent asthma. Can also be used as preventive treatment before
exercise or unavoidable exposure to known allergens.
Long-acting β2 Agonists
Long-acting bronchodilator used concomitantly with anti-inflammatory medications for long-term control of symptoms, especially nocturnal symptoms.
Includes salmeterol and formoterol.
Methylxanthines
Sustained-release theophylline is a mild-to-moderate bronchodilator used principally as an adjuvant to inhaled corticosteroids for prevention of nocturnal
asthma symptoms. May have a mild anti-inflammatory effect.
Leukotriene Modifiers
Montelukast, a leukotriene receptor antagonist, or zileuton, a 5-lipoxygenase inhibitor, may be considered an alternative therapy to low doses of inhaled
corticosteroids, or cromolyn or nedocromil, in the setting of mild persistent asthma.
Systemic Corticosteroids
ww Used for moderate-to-severe exacerbations to speed recovery and prevent recurrence of exacerbations.
ww Omalizumab (anti-immunoglobulin E), a recombinant DNA-derived humanized monoclonal antibody of the Fc portion of the immunoglobulin E antibody,
binds to that portion, thereby preventing immunoglobulin E binding to its high-affinity receptor (FcεR1) on mast cells and basophils, resulting in a decrease
in the release of mediators in response to allergen exposure.
Quick-Relief Medications (Reliever Class)
Short-acting β2 Agonists
Albuterol and levalbuterol are therapy of choice for relief of acute symptoms and prevention of exercise-induced bronchoconstriction.
Anticholinergic Agents
Ipratropium bromide provides additive benefit to inhaled b2 agonists in acute exacerbations. May be an alternative bronchodilator for the patient who does not
tolerate inhaled b2 agonists.

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 Chapter 96: Wheezing and Asthma 705

Table 96.2. Classifying Asthma Severity

Classifying Asthma Severity and Initiating Treatment (0–4 years of age)
Persistent
Components of Severity Intermittent Mild Moderate Severe
Symptoms ≤2 days/week >2 days/week but not Daily Throughout the day
daily
Nighttime awakenings 0 1–2x/month 3–4x/month >1x/week
Short-acting b2 agonist use ≤2 days/week >2 days/week but not Daily Several times per day
Impairment
for symptom control (not daily
prevention of EIB)
Interference with normal None Minor limitation Some limitation Extremely limited
activity
Exacerbation requiring oral 0–1 per year ≥2 exacerbations in 6 months requiring oral systemic corticosteroids, or ≥4 wheezing
systemic corticosteroids episodes per 12 months lasting >1 day AND risk factors for persistent asthma
Risk
Consider severity and interval since last exacerbation. Frequency and severity may fluctuate over time.
Exacerbations of any severity may occur in a patient in any severity category.
Recommended Step for Initiating Step 1: SABA PRN Step 2: Low-dose ICS Step 3: Medium-dose ICS and consider short course
Therapy of OCS
(See Table 96.4 for treatment steps.) In 2–6 weeks, depending on severity, evaluate level of asthma control achieved. If no clear benefit is observed in
4–6 weeks, consider adjusting therapy or alternative diagnoses.
Classifying of Asthma Severity and Initiating Treatment (5–11 years of age)
Persistent
Components of Severity Intermittent Mild Moderate Severe
Symptoms ≤2 days/week >2 days/week but not Daily Throughout the day
daily
Nighttime awakenings ≤2x/month 3–4x/month >1x/week but not nightly Often 7x/week
Short-acting b2 agonist use ≤2 days/week >2 days/week but not Daily Several times per day
for symptom control (not daily
prevention of EIB)
Impairment
Interference with normal None Minor limitation Some limitation Extremely limited
activity
Lung function Normal FEV1 between FEV1 ≥80% predicted FEV1 = 60%–80% FEV1 <60% predicted
exacerbations FEV1/FVC >80% predicted FEV1/FVC <75%
FEV1 >80% predicted FEV1/FVC = 75%–80%
FEV1/FVC >85%
Exacerbation requiring oral 0–1 per year ≥2 per year
Risk systemic corticosteroids Consider severity and interval since last exacerbation. Frequency and severity may fluctuate over time.
Relative annual risk of exacerbations may be related to FEV1.
Recommended Step for Initiating Step 1: SABA PRN Step 2: Low-dose ICS Step 3: Medium-dose ICS Step 3, Medium-dose ICS
Therapy option option or step 4
(See Table 96.4 for treatment steps.) Consider short course of OCS.
In 2–6 weeks, evaluate level of asthma control that is achieved and adjust therapy accordingly.
(continued)

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 706 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

Table 96.2. Classifying Asthma Severity (continued )

Classifying of Asthma Severity and Initiating Treatment (≥12 years of age)
Persistent
Component of Severity Intermittent Mild Moderate Severe
Symptoms ≤2 days/week >2 days/week but not Daily Throughout the day
daily
Nighttime ≤2x/month 3–4x/month >1x week, but not nightly Often 7x/week
awakenings
Short-acting b2 ≤2 days/week >2 days/week but not Daily Several times per day
agonist use for daily and more than once
symptom control on any day
Impairment (not prevention
of EIB)
Interference with None Minor limitation Some limitation Extremely limited
normal activity
Lung function Normal FEV1 between FEV1 ≥80% predicted FEV1 >60%, but <80% FEV1 <60% predicted
exacerbations FEV1/FVC normal predicted FEV1/FVC reduced <5%
FEV1 >80% predicted FEV1/FVC reduced 5%
FEV1/FVC >85%
Exacerbation requir- 0–1 per year ≥2 per year
ing oral systemic
Risk corticosteroids
Consider severity and interval since last exacerbation. Frequency and severity may fluctuate over time.
Exacerbations of any severity may occur in a patient in any severity category.
Step 1: SABA PRN Step 2: Low-dose ICS Step 3: Low-dose ICS + Step 4/5: Medium-/
Recommended Step for Initiating LABA High-dose ICS + LABA
Therapy Consider short course of OCS
(See Table 96.4 for treatment steps.) In 2–6 weeks, depending on severity, evaluate level of asthma control that is achieved. If no clear benefit is
observed in 4–6 weeks, consider adjusting therapy or alternative diagnoses.
Abbreviations: EIB, exercise-induced bronchoconstriction; FEV1, forced expiratory volume in 1 second; FVC, forced vital capacity; ICS, inhaled corticosteroids; LABA, long-acting b2 agonist; OCS, oral cortico-
steroids; PRN, as needed; SABA, short-acting b2 agonist.
Adapted from the National Asthma Education and Prevention Program. Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma, 2007. Bethesda, MD: National Heart, Lung, and Blood
Institute; 2007. NIH Publication No. 07-4051.

Long-term Management Box 96.6. Therapeutic Goals for the Child

The goals of long-term management are shown in Box 96.6. With Chronic Asthma
Continuous and longitudinal primary care of children with
ww Maintain a normal, age-appropriate activity level
asthma can profoundly affect the disease course. The primary care
ww Maintain near-normal pulmonary function
physician, child, and family must work together to achieve good
ww Prevent symptoms such as exercise intolerance, chronic cough, and
control of symptoms. Prevention of exacerbations may be accom-
shortness of breath
plished by removal of offending allergens. Minimization of exposure
ww Prevent acute exacerbations of the disease that require acute therapy
to known allergens and irritants has been shown to decrease symp-
ww Minimize adverse effects of the drugs used to manage the disease
toms and exacerbation. The most commonly implicated irritants are
ww Promote self-esteem and a sense of well-being
tobacco smoke; fumes from gas, oil, and kerosene stoves and wood-
burning appliances; and sprays and strong odors. House dust mites
are microscopic insects that feed on human scales, require humid- Allergen immunotherapy may be considered for the patient
ity greater than 50%, and are found in stuffed furniture, carpets, and with asthma when evidence exists of a clear relationship between
mattresses. House dust mite infestation can be reduced by encasing symptom and exposure to an allergen, and symptoms are poorly
mattresses, blankets, and pillows in mite-proof covers; removing car- controlled with pharmacologic management (eg, medication inef-
peting; and decreasing humidity to less than 50%. fective, multiple medications are required, patient does not accept

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 Chapter 96: Wheezing and Asthma 707

or tolerate medication). Adherence to appropriate medications and course of the disease should be discussed, and all parties should
in-home peak-flow measurement helps in prevention and control of be allowed to express their concerns about the development of a
asthma. Periodic assessment ensures appropriate therapy and com- treatment plan. Other factors, such as rhinitis, sinusitis, and gastro-
pliance with treatment. The physician should make sure that the esophageal reflux, which may influence the severity of asthma or the
family can afford the necessary medications. During routine vis- child’s quality of life, should be assessed and treated appropriately.
its, home monitoring and therapy as well as any diaries and records Family members and the affected child should be given a written
should be reviewed, the child’s and family’s expectations about the action plan based on the patient’s personal best peak flow (Figure 96.2)

Figure 96.2. Asthma action/medicine plan.

Reprinted from National Heart, Lung, and Blood Institute. National Asthma Education and Prevention Program. Full Report 2007. NIH Publication No 07-4051,
Revised August 2007. https://www.nhlbi.nih.gov/sites/default/files/media/docs/asthgdln_1.pdf.

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 708 PART 6: HEAD, NECK, AND RESPIRATORY SYSTEM

and instructed in the use of peak flow meters to indicate when med- modifier agents, such as montelukast. Short-acting b2 agonists
ical treatment is necessary. Some meters have 3 color zones: a green are used for acute exacerbations. For moderate persistent asthma,
zone, which indicates good airflow; a yellow zone, which signals the medium-dose ICSs or low-dose ICSs plus a second agent is rec-
need for treatment; and a red zone, which suggests that a visit to the ommended, depending on the age group. Second agents include
ED may be indicated. long-acting b2 agonists, leukotriene modifiers, and theophylline.
For severe persistent asthma, usually high-dose ICS plus a second
Dynamic Monitoring and Treatment