ON CALL

Pediatrics
OTHER TITLES IN THE ON CALL SERIES
Adams, Forrester, Bresnick & Rosenberg: On Call Surgery,
4th Edition, Upcoming
Bernstein, Poag & Rubinstein: On Call Psychiatry,
4th Edition, 2018
Khan: On Call Cardiology, 3rd Edition, 2006
Marshall & Mayer: On Call Neurology, 3rd Edition, 2006
Marshall & Ruedy: On Call Principles and Protocols,
6th Edition, 2017
ON CALL
Pediatrics
4th Edition 0 Q
JAMES J. NOCTO
Professor of Pedia '
Section of Rheuma ,
Director, Pediatric RheumatoioQFellow ' Training Program
DepartmenLof Radia‘mcs
Medical ColEge Wisconsin
ital of Wisconsin
MI
‘ .au ,Wisconsin

G. GEDEIT, MD
’ Professor of Pediatrics
iedical College of Wisconsin;
' ical Director, Pediatric Intensive Care Unit
Children’s Hospital of Wisconsin
Milwaukee, Wisconsin

and the Pediatric Residents of Children’s

Hospital of Wisconsin

ELSEVIER
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899
ON CALL PEDIATRICS, FOURTH EDITION ISBN: 978-0-323-52905-1
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Notices
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Practitioners and researchers must always rely on their own experience and knowledge in
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In using such information or methods they should be mindful of their own safety and the safety
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With respect to any drug or pharmaceutical products identified, readers are advised to check
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Previous editions copyrighted 2006, 2001, and 1997.

Library of Congress Cataloging-in-Publication Data
Names: Nocton, James J., editor. j Gedeit, Rainer G., editor. j Children’s
Hospital of Wisconsin, issuing body.
Title: On call pediatrics / [edited by] James J. Nocton, Rainer G. Gedeit,
and the pediatric residents of Children's Hospital of Wisconsin.
Other titles: Pediatrics j On call series.
Description: 4th edition. | Philadelphia, PA : Elsevier, [2019] | Series:
On call series | Includes bibliographical references and index.
Identifiers: LCCN 2017046943 | ISBN 9780323529051 (pbk. : alk. paper)
Subjects: | MESH: Pediatrics | Child | Emergencies | Infant | Handbooks
Classification: LCC RJ370 | NLM WS 39 | DDC 618.92–dc23 LC record available
at https://lccn.loc.gov/2017046943

Content Strategist: James Merritt

Content Development Specialist: Katie DeFrancesco
Publishing Services Manager: Catherine Jackson
Senior Project Manager: Rachel E. McMullen
Design Direction: Amy Buxton

Printed in China
Last digit is the print number: 9 8 7 6 5 4 3 2 1
 To our children—
Amanda, Kay, Beth, and Claire Gedeit,
and Jeff and Sara Nocton—
who remain our greatest teachers.
Contributors

Laura Adams, MD Keli Coleman, MD

Resident Resident
Department of Internal Department of Pediatrics
Medicine-Pediatrics Medical College of Wisconsin
Medical College of Wisconsin and Children’s Hospital of
and Children’s Hospital of Wisconsin
Wisconsin Milwaukee, Wisconsin
Milwaukee, Wisconsin
Alison Coren, MD
Daniel Beacher, MD Resident
Resident Department of Pediatrics
Department of Pediatrics Medical College of Wisconsin
Medical College of Wisconsin and Children’s Hospital of
and Children’s Hospital of Wisconsin
Wisconsin Milwaukee, Wisconsin
Milwaukee, Wisconsin

Alina G. Burek, MD Rose Doolittle, MD

Resident Resident
Department of Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin

Brian Carroll, MD Danielle DuMez, MD

Resident Pediatric Resident
Department of Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin

vii
viii Contributors

Rainer G. Gedeit, MD Jennifer Lhost, MD

Professor of Pediatrics Resident
Medical College of Wisconsin; Department of Pediatrics
Clinical Director Medical College of Wisconsin
Pediatric Intensive Care Unit and Children’s Hospital of
Children’s Hospital of Wisconsin
Wisconsin Milwaukee, Wisconsin
Milwaukee, Wisconsin
Susan K. Light, MD
Michael Girolami, MD Resident
Resident Department of Pediatrics
Department of Internal Medical College of Wisconsin
Medicine–Pediatrics and Children’s Hospital of
Medical College of Wisconsin Wisconsin
and Children’s Hospital of Milwaukee, Wisconsin
Wisconsin
Milwaukee, Wisconsin Vanessa C. McFadden, MD,
PhD
Maja Z. Katusic Resident
Resident Department of Pediatrics
Department of Pediatrics Medical College of Wisconsin
Medical College of Wisconsin and Children’s Hospital of
and Children’s Hospital of Wisconsin
Wisconsin Milwaukee, Wisconsin
Milwaukee, Wisconsin
James J. Nocton, MD
Professor of Pediatrics
Karlo Kovacic, MD Section of Rheumatology,
Resident Director, Pediatric Rheumatology
Department of Pediatrics Fellowship Training Program
Medical College of Wisconsin Department of Pediatrics
and Children’s Hospital of Medical College of Wisconsin and
Wisconsin the Children’s Hospital of
Milwaukee, Wisconsin Wisconsin
Milwaukee, Wisconsin
Hema Krishna, MD
Resident Julia Richards, MD
Department of Internal Resident Physician
Medicine–Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin
 Contributors ix

Kent Rosenwald, MD Rachel T. Sullivan, MD

Pediatrics Resident Pediatric Resident
Department of Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin
Kathryn M. Rubey, MD Corinne Swearingen, MD
Resident Resident
Department of Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin
Anna Schmitz, MD Eric Velazquez, MD
Resident Resident
Department of Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin
Purabi Sonowal, MD Rachel Weigert, MD
Resident Resident Physician
Department of Pediatrics Department of Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin

Shela Sridhar, MD Amanda A. Wenzel, MD

Resident Resident Physician
Department of Internal Department of Pediatrics
Medicine/Pediatrics Medical College of Wisconsin
Medical College of Wisconsin and Children’s Hospital of
and Children’s Hospital of Wisconsin
Wisconsin Milwaukee, Wisconsin
Milwaukee, Wisconsin
Stephen E. Wilkinson, MD
Lea Steffes, MD Resident Physician
Resident Department of Internal
Department of Pediatrics Medicine–Pediatrics
Medical College of Wisconsin Medical College of Wisconsin
and Children’s Hospital of and Children’s Hospital of
Wisconsin Wisconsin
Milwaukee, Wisconsin Milwaukee, Wisconsin
Preface

We are very pleased to have the opportunity to write this revised

and updated edition of On Call Pediatrics. Over the 11 years since
the last edition was published, there have been considerable
advances in diagnostics and management of many of the problems
reviewed in this book. In addition, there have been significant
changes in the way in which care is delivered to patients, including
the staffing of residents and physicians within hospitals, the
increasing number of non-physician advanced providers, and the
growth of the electronic medical record. In fact, the entire concept
of being “on call” in the hospital has been changing, with residents
and staff moving away from intermittent long periods of being “on
call” and responsible for patients that are “cross-covered” to sys-
tems of team-based care and scheduled night shifts or day shifts.
Despite these trends, the concepts reviewed in this and previous
versions of On Call Pediatrics remain very relevant, and the
approach to patient problems discussed in this edition can con-
tinue to be applied effectively when evaluating hospitalized chil-
dren. Therefore we believe that this edition will continue to
serve as a valuable resource for all providers who care for children.

With this edition, we have included chapters on each of the

problems reviewed in previous versions. We hope that this will per-
mit this edition to remain most comprehensive and most helpful.
With the development of multiple and easily accessed web-based
resources, we decided to eliminate the formulary as well as several
of the appendices that were included in earlier editions. We trust
that the reader will not view this as an inconvenience.

We have again enlisted the assistance of the senior residents of

the Children’s Hospital of Wisconsin and the Medical College of
Wisconsin Affiliated Hospitals to serve as contributors to nearly
all of the chapters. These residents have been responding to calls
exactly like those reviewed in this book for several years, and we
believe that the knowledge and expertise that our residents are able
to share will allow this edition to be most helpful.

xi
xii Preface

It continues to be our intent that this book will remain a valu-

able and helpful resource to those who provide care to children,
permitting the delivery of the best care to children, and contribut-
ing to the education of all providers in the process.

James J. Nocton, MD
Rainer G. Gedeit, MD
Acknowledgments

We are indebted to Robert M. Kliegman, MD, whose advice, sup-

port, and encouragement have made every edition of this book pos-
sible, and to Mr. Jim Merritt, Ms. Lauren Willis, Ms. Stacy
Eastman, and Ms. Katie DeFrancesco of Elsevier, Inc., whose assis-
tance and guidance were greatly appreciated during the prepara-
tion of this edition. We thank our colleagues at the Medical
College of Wisconsin and Children’s Hospital of Wisconsin for
their support and wisdom, and the medical students and residents
at these respective institutions, who challenge us to improve the
care we provide to children, to become better teachers, and to
continue to learn ourselves.

xiii
Structure of the Book

The book is divided into three main sections: I (Introduction), II

(Patient-Related Problems), and III (Laboratory-Related Problems).
Section I covers introductory material in five chapters: The
Diagnosis and Management of On-Call Problems, Communicating
with Colleagues and Families, Common Mistakes, Remembering
Your ABCs, and Teaching (and Learning) While On Call.
Section II discusses the common calls associated with patient-
related problems. Each problem is approached from its inception,
beginning with the relevant questions that should be asked over the
phone, the temporary orders that should be given, and the major
life-threatening problems to be considered as one approaches the
bedside.
The setup of Section II is as follows.

PHONE CALL
Questions
Pertinent questions to assess the urgency of the situation.

Orders
Urgent orders to be carried out before the housestaff arrives at the
bedside.

Inform RN
Nurse to be informed of the time the housestaff anticipates arrival
at the bedside.

ELEVATOR THOUGHTS

The differential diagnosis to be considered by the housestaff while

they are on their way to assess the patient (i.e., while they are in the
elevator).

xv
xvi Structure of the Book

MAJOR THREAT TO LIFE

Identification of the major threat to life, which is essential in provid-
ing focus for the subsequent effective management of the patient.

BEDSIDE
Quick-Look Test
The quick-look test is a rapid visual assessment to place the patient
into one of three categories: well, sick, or critical. This helps deter-
mine the necessity of immediate intervention.

Airway and Vital Signs

Selective History and Chart Review
Selective Physical Examination

MANAGEMENT
Section III contains the common calls associated with laboratory-
related problems.
The Appendices consist of reference items that we have found
useful in managing calls.
The On-Call Formulary is a compendium of commonly used
medications that are likely to be prescribed by the student or res-
ident on call. The formulary serves as a quick, alphabetically
arranged reference for indications, drug dosages, routes of admin-
istration, side effects, contraindications, and modes of action.
Commonly Used
Abbreviations

ABCs Airway, breathing, and circulation

ABD Abdomen
ABG Arterial blood gas
AC Before meals
ACE Angiotensin-converting enzyme
ACLS Advanced cardiac life support
AIDS Acquired immunodeficiency syndrome
ANA Antinuclear antibody
A/P Anteroposterior
aPTT Activated partial thromboplastin time
ARDS Adult respiratory distress syndrome
ASD Atrial septal defect
ASO Antistreptolysin O
AV Atrioventricular
BID Twice a day
BP Blood pressure
BPD Bronchopulmonary dysplasia
BPM Beats per minute
BSA Body surface area
CBC Complete blood count
CF Cystic fibrosis
CHF Congestive heart failure
CMV Cytomegalovirus

xvii
xviii Commonly Used Abbreviations

CNS Central nervous system

CO Cardiac output
CO2 Carbon dioxide
CPK Creatine phosphokinase
CSF Cerebrospinal fluid
CT Computed tomography
CVAT Costovertebral angle tenderness
CVS Cardiovascular system
CXR Chest x-ray
DDAVP Desmopressin
DIC Disseminated intravascular coagulation
DKA Diabetic ketoacidosis
D5NS 5% dextrose in normal saline solution
DOE Dyspnea on exertion
DVT Deep venous thrombosis
D5W 5% dextrose in water
D25W 25% dextrose in water
ECG Electrocardiogram
EEG Electroencephalogram
ELISA Enzyme-linked immunosorbent assay
ENT Ears, nose, and throat
ESR Erythrocyte sedimentation rate
EXT Extremities
FIO2 Fraction of inspired oxygen
Fr French (unit of measurement for catheters
and tubes)
FUO Fever of unknown origin
GI Gastrointestinal
G-6-PD Glucose-6-phosphate dehydrogenase
GU Genitourinary
Hb Hemoglobin
 Commonly Used Abbreviations xix

HCT Hematocrit
HEENT Head, eyes, ears, nose, and throat
HIV Human immunodeficiency virus
HPI History of present illness
Hr Hour
HR Heart rate
HS At bedtime
HSV Herpes simplex virus
HUS Hemolytic-uremic syndrome
ICP Intracranial pressure
ICU Intensive care unit
IDDM Insulin-dependent diabetes mellitus
IgG Immunoglobulin G
IM Intramuscular
INR International normalized ratio
ITP Idiopathic thrombocytopenic purpura
IV Intravenous
IVIG Intravenous immunoglobulin
JRA Juvenile rheumatoid arthritis
LDH Lactate dehydrogenase
LOC Loss of consciousness
LP Lumbar puncture
LV Left ventricle
LVH Left ventricular hypertrophy
MAO Monoamine oxidase
MCV Mean corpuscular volume
mm Hg Millimeters of mercury
MRI Magnetic resonance imaging
NEC Necrotizing enterocolitis
NEURO Neurologic system
xx Commonly Used Abbreviations

NG Nasogastric
NIDDM Non–insulin-dependent diabetes mellitus
NPH Neutral protamine Hagedorn (insulin)
NPO Nothing by mouth
NS Normal saline
NSAID Nonsteroidal anti-inflammatory drug
O2 Oxygen
Osm Osmolality
P/A Posteroanterior
PAC Premature atrial contraction
PALS Pediatric Advanced Life Support
PC After meals
PCA Patient-controlled analgesia
PCO2 Partial pressure of carbon dioxide
PEEP Positive end-expiratory pressure
PICU Pediatric intensive care unit
PMI Point of maximal intensity
PO By mouth
PO2 Partial pressure of oxygen
PPD Purified protein derivative
PPHN Persistent pulmonary hypertension of the newborn
PR Per rectum
PRBCs Packed red blood cells
PRN As necessary
PT Prothrombin time
PTH Parathyroid hormone
PTT Partial thromboplastin time
PVC Premature ventricular contraction
Q Each
QHS Each night at bedtime
 Commonly Used Abbreviations xxi

QID Four times a day

RBC Red blood cell
RESP Respiratory system
RN Registered nurse
RR Respiratory rate
RSV Respiratory syncytial virus
RTA Renal tubular acidosis
RV Right ventricle
RVH Right ventricular hypertrophy
SBE Subacute bacterial endocarditis
SC Subcutaneous
Sec Second
SI International System of Units
SIADH Syndrome of inappropriate antidiuretic hormone
SL Sublingual
SLE Systemic lupus erythematosus
SOB Shortness of breath
STAT Immediately
SVT Supraventricular tachycardia
T3 Triiodothyronine
T4 Thyroxine
TB Tuberculosis
TCA Tricyclic antidepressants
TKVO To keep vein open
TORCH Toxoplasmosis, other infections, rubella,
cytomegalovirus, herpes (congenital infections)
TPN Total parenteral nutrition
TSH Thyroid-stimulating hormone
TTP Thrombotic thrombocytopenic purpura
UAC Umbilical arterial catheter
URI Upper respiratory infection
xxii Commonly Used Abbreviations

UTI Urinary tract infection

UVC Umbilical venous catheter
V/Q Ventilation/perfusion
VSD Ventricular septal defect
VT Ventricular tachycardia
WBC White blood cell
WPW Wolff-Parkinson-White syndrome
 CHAPTER

1
The Diagnosis and
Management of
On-Call Problems
James J. Nocton, MD

The approach to the evaluation and management of problems that

arise while on call is similar to that taken in other clinical situations.
Initially, information about the patient and the specific problem
must be collected. This information is acquired by obtaining a his-
tory, performing a physical examination, and potentially reviewing
pertinent laboratory data or imaging studies. While collecting
information, the physician is considering possible diagnoses. Even-
tually, a diagnostic impression is formulated, and appropriate man-
agement is undertaken. Although this general scheme is identical to
that used when admitting a new patient or evaluating a patient in
the ambulatory setting, the approach often needs to be modified
when one is informed about a problem that arises in a patient
already in the hospital.
The primary distinction between the approach to an on-call
problem and a problem in a “new” patient is that the goals are dif-
ferent. In most cases, patients who are in the hospital have already
undergone a complete history and physical examination, a diag-
nostic impression has already been formulated regarding their
problems at the time of admission, and management of these prob-
lems has begun. It is not the goal of the physician on call to repeat
this process. Instead, the physician on call is expected to evaluate
and manage acute problems that either are causing discomfort or
have the potential to lead to deterioration in the patient’s condition.
It is appropriate to ask the question, “What might happen today
that may be causing this problem and may adversely affect the
patient?” When one is admitting multiple patients and is respon-
sible for many others, time is always a factor. Some problems need
to be given priority over others. Rarely does one have time to

2
 The Diagnosis and Management of On-Call Problems 3

deliberate extensively about problems that are not going to lead to

immediate harm to the patient. These problems can and should be
addressed at a later time. In some cases the cause of a problem may
not be easily diagnosed, and specific management may therefore be
deferred until the problem “declares itself.” This approach may be
acceptable if one keeps in mind that the goals are to maintain the
comfort of the patient and exclude (or empirically treat) potential
imminent causes of significant morbidity or mortality.
Because the goal of a physician on call is limited, the approach
to a patient in whom a problem develops can be much more
focused. One does not need to take a complete history or perform
an exhaustive physical examination. Chart reviews may provide
helpful information but should also be directed at answering spe-
cific questions. In many cases, historical information may have
already been obtained during “sign-out.” The sign-out process is
the ideal time to enhance efficiency in managing on-call problems.
Potential problems can often be anticipated by those who have
been caring for a patient on a daily basis. Specific details about
patients can be relayed to the on-call house officer, and manage-
ment suggestions for anticipated problems can be discussed. Such
a sign-out process can be extremely helpful, for example, when a
problem is recurrent and previously successful or unsuccessful
management strategies are known. The key to addressing on-call
problems is efficiency. Being only as thorough as one needs to
be and prioritizing appropriately allow one to be a successful phy-
sician on call.
The approach suggested in this book is designed to be efficient,
yet appropriately thorough. For each problem, the sequence of
thought processes that the physician should go through is dis-
cussed from the time that a problem is identified (usually with a
phone call from a nurse) until the problem is managed. These
thought processes are discussed in four separate parts for each
chapter:
1. Phone call
2. Elevator thoughts
3. Major threat to life
4. Bedside

PHONE CALL
The phone call is usually how one first hears about a problem. The
initial call should not simply be for notification but should also
allow the physician to indirectly assess the severity of the problem,
to begin developing a differential diagnosis, and, when necessary,
to begin management. In this section of each chapter, questions
that should be asked immediately, before hanging up the phone,
4 Introduction

are listed. If the severity of the problem can be determined, the phy-
sician can then appropriately decide how to prioritize the call. If the
severity cannot be determined, the patient needs to be seen fairly
quickly. Orders that may enhance efficient evaluation or manage-
ment are also suggested in this section, and other information
important for the nurse to know is discussed, such as when the
physician will arrive at the bedside.
Not every call requires that the physician evaluate the patient
directly. For quick, minor problems that pose no immediate threat
to the patient, taking a history from the nurse may be sufficient to
allow one to determine appropriate management. In these
instances, it is reasonable to ask the nurse, “Do you think I need
to see the patient?” If there is any doubt in either the nurse’s or
the physician’s mind, the patient should be seen.

ELEVATOR THOUGHTS
After the physician has finished discussing the problem with the
nurse, some time is usually required to travel to the patient’s bed-
side. The time that it takes to walk or ride the elevator to see a
patient can be used to reflect on the situation and to think about
differential diagnoses. In this section of each chapter, the differen-
tial diagnosis for each problem is listed. The lists are not exhaustive
but are intended to present the most common possibilities and
those considered life threatening.

MAJOR THREAT TO LIFE

As part of the differential diagnosis, it is important for the physi-
cian on call to identify the possible diagnoses that are the most
severe or potentially life threatening. One should ask, “What
should I be considering that might be life threatening?” By asking
this question, one ensures that even if definitive solutions are not
found, the most serious possibilities have been considered and
either excluded or empirically treated.

BEDSIDE
This section describes the steps that should be taken on arrival
at the bedside. By this time, the chief complaint is known (the
reason for the phone call from the nurse), possible diagnoses
have been considered on the basis of the information available
(elevator thoughts), and the major threat or threats to life have
been identified (elevator thoughts). The steps to be taken at the
 The Diagnosis and Management of On-Call Problems 5

bedside are presented in the following subsections in each

chapter:
1. Quick-look test
2. Airway and vital signs
3. Selective history
4. Selective physical examination
5. Selective chart review
6. Management
The quick-look test and evaluation of the airway and vital
signs should always be the first things done on arrival at the bed-
side, regardless of the complaint. The quick-look test involves an
overall impression gained by observing the patient. The goal is to
rapidly evaluate the patient’s condition. The patient may appear
comfortable and in no distress (e.g., sitting in bed having a
conversation), mildly uncomfortable or distressed (e.g., crying,
worried), or severely ill (e.g., comatose). Combined with evalua-
tion of the airway and vital signs, the quick-look test enables
the physician to determine the urgency of the situation and
whether immediate intervention is necessary before obtaining a
history and performing a physical examination. If the patient
appears comfortable and has stable vital signs and an intact air-
way, the physician can proceed with less urgency. The sequence
of the subsections describing the history, physical examination,
chart review, and management varies among the chapters accord-
ing to the problems presented. For some problems (e.g., hypoten-
sion and shock), some form of management probably needs to be
undertaken before additional history is obtained or a selective
chart review is performed. In other instances (e.g., constipation),
the sequence is as expected, with management suggestions offered
after suggestions regarding the history, physical examination, and
chart review.
We believe that this approach to problems that may arise in a
pediatric patient is helpful for the physician on call. As outlined
here and presented in the individual chapters, this approach should
provide an organized way for the physician to thoroughly and effi-
ciently evaluate the common problems that arise in hospitalized
pediatric patients.
 CHAPTER

2
Communicating With
Colleagues and Families
James J. Nocton, MD

As with all other clinical situations, appropriate communication is

an essential component of the evaluation and management of a
patient while one is on call. The results of the evaluation and man-
agement of the problem, if any, must be effectively communicated
to the patient, the family, the nurse, and others responsible for care
of the patient. Communicating while on call occurs in two ways:
(1) discussions in person or by telephone and (2) documentation
in the patient’s chart. When one is busy admitting patients and
evaluating patients with problems, taking the time for appropriate
communication and documentation may often seem less important
than the actual process of caring for the patients. However, the time
spent talking with families, discussing the patient with the nurse,
writing a note, or making the extra phone call is much appreciated
by everyone, including your colleagues who will resume responsi-
bility for the patient when you are no longer on call.
Communicating with the child, and usually the parents of the
child, will begin as soon as you arrive at the bedside and will con-
tinue until you have addressed the problem and answered the
family’s questions. The family of a child in the hospital is under
great stress. Their child’s illness is a tremendous source of worry
and anguish and causes a significant disruption in their daily lives.
When a problem arises while unknown physicians are responsible
for the care of their child, the family may feel lost without the
physicians familiar to them. They may feel as though no one knows
what is happening with their child and may not trust that the
physician on call will know what to do. It will be up to you to
evaluate and manage the problem and to communicate to the fam-
ily in a manner that is comforting, gains their trust, and reassures
them that their child will receive excellent care.
Once you arrive to evaluate the child, you need to formally
introduce yourself to the family and the child and address the fam-

6
 Communicating With Colleagues and Families 7

ily by name, not as “Mom” or “Dad.” This takes very little time, and
it may make a difference in how the family reacts to you. Explain
who you are, your role as part of the medical team, and why you
have been called to see their child. Stating that you know why
the child is in the hospital, the issues that are being evaluated or
treated during this hospitalization, and that you have communi-
cated with the physicians that may be more familiar to them will
reassure the family that you do know something about their child
and will hopefully build their trust in you. Then, proceed with your
evaluation efficiently and thoroughly.
After you have finished your selective examination, history, and
chart review and have developed an assessment and plan of action,
this should be communicated directly to the family. The family will
want to know whether their child is becoming “sicker” or is in dan-
ger of becoming critically ill as a result of the problem for which
you have been called. You will need to address these issues and
inform the family if there appears to have been a change in the
child’s status. When the findings of your evaluation imply a serious
change in the child’s condition, the family needs to be informed in a
clear, understandable, and honest manner. Bad news is always dif-
ficult to deliver. The physician must never delegate this duty to a
student or nurse. If the family is not present, the on-call physician
should contact the family by telephone promptly, introduce him-
self or herself clearly, and truthfully explain the situation to the
family.
One decision that will need to be made when managing prob-
lems on call is when to notify a supervising resident or attending
physician. This is a process that must be individualized. The status
of the patient, the severity of the problem, and your experience and
degree of comfort are all factors that affect the decision to imme-
diately notify others who are supervising care of the patient. Early
in the first year of residency, nearly every problem is discussed
immediately with a supervisor. As experience is gained, the physi-
cian on call is able to manage many of these problems indepen-
dently, with discussion at a later time. In some cases, it is
obvious that a supervisor must be notified, such as when a patient’s
condition changes significantly, transfer to an intensive care setting
appears imminent, or diagnostic or therapeutic uncertainty exists.
As a general rule, if there is any doubt regarding a problem that
arises while one is on call, it is always best to discuss the problem
with a supervisor. Knowing one’s limitations is an extremely valu-
able asset that should not be forgotten while on call.
Discussing problems and their management with the nurse car-
ing for the child is also essential. In many instances the nurse
knows the patient well, may have more experience with specific
problems, and has seen how others have managed similar problems
8 Introduction

successfully. The nurse can be a great resource in these circum-

stances and may be able to offer very helpful suggestions. Listening
to the nurse, involving the nurse in the decision-making process,
and keeping the nurse informed of your plans will allow you to pro-
vide the best care to the patient and will keep everyone “on the
same page,” thereby minimizing the potential for errors.
Regardless of whether a particular problem requires immediate
discussion or notification of others, documentation of the problem,
evaluation, and action taken is mandatory. With the exception of very
minor and inconsequential problems that can be resolved over the
phone without seeing the patient, all other problems require at least
some documentation. It is extremely helpful for those caring for the
patient on a daily basis to know what happened in the middle of the
night and to have access to such information via the patient’s chart.
The written documentation of the evaluation and management of a
problem that arises while one is on call can take several forms. Simple
problems may require only a few sentences. For example:
Called to see patient for sore throat. Patient afebrile, HR 84, RR 20,
BP 120/75. Appeared comfortable. No complaints other than throat.
Posterior pharynx slightly erythematous with patches of exudate on
tonsils. Mild cervical adenopathy. Obtained rapid streptococcal test
and throat culture. Will not administer antibiotics until results of
tests known.
For other problems (e.g., new fever in an immunocompromised
patient), a more extensive note may be required, and the “SOAP”
format generally works well—listing the Subjective complaint, Objec-
tive findings, Assessment, and Plan of management. One should be as
concise as possible, describing only as much historical, subjective
information as is essential and providing a brief summary of your
assessment and management plan. It is necessary to perform and
document only the pertinent parts of the physical examination.
The goal is to notify those who will be seeing the patient the following
day that a problem arose and that the problem was evaluated and
managed. As with all other types of documentation, notes should
be dated and timed. All procedures should be described. If the
problem was discussed with the family, attending physician, or
consultants, this should be documented along with the time that
the discussion took place. Finally, your name should be indicated,
followed by a phone or pager number. If someone has a question
regarding what happened, that person should be able to reach you
easily.
Discussing problems with colleagues and families and docu-
menting the evaluation and management of problems are tasks that
are easy to ignore when one is on call and extremely busy. However,
maintaining communication should be a priority. Communicating
 Communicating With Colleagues and Families 9

with the family promptly, honestly, and in person when possible is

essential. Communicating with supervising colleagues in all situa-
tions in which there is any doubt is in the patient’s best interest. By
documenting problems, you are helping your colleagues and others
caring for the patient. Through verbal communication and docu-
mentation, you can also remind yourself that you are never truly
alone when you are on call.
 CHAPTER

3
Common Mistakes
James J. Nocton, MD

Everyone makes mistakes. Although some mistakes are the result of

fatigue and lack of sleep, the most common mistakes are made by
physicians in a hurry. Therefore many mistakes are preventable.
This chapter briefly discusses a few of the most common and most
preventable errors made in the care of hospitalized children,
including the following:
• Poorly entered orders
• Failure to communicate with the nurse
• Failure to communicate with a supervising physician
• Failure to listen to the family
• Failure to document in the chart while on call
The way you enter orders is important. An electronic health
record is now used in most hospitals, and this helps to avoid leg-
ibility issues and some calculation errors; however, the potential for
error remains, and one still needs to be compulsive about entering
orders. Calls requesting clarification of confusing or incomplete
orders will plague you and eat away at your time if you do not take
this issue seriously. Enter every order as simply and as clearly as
possible. Be meticulous. Specify the preparation of any medication,
the dose, the route of administration, and the dosing frequency
clearly. Be careful about decimal points! Always precede a decimal
point by a 0 when prescribing less than a milligram (e.g., digoxin,
0.25 mg by mouth every day). Never follow the tenths or hun-
dredths place with a 0; for example, captopril, 6.250 mg, could
be misread easily as 6250 mg. Do not assume that an electronic sys-
tem will autocorrect, and always double check the order you enter.
Try to avoid abbreviations. Instead of writing (or typing) “qd” or
“bid,” write “once a day” or “twice a day.” When ordering a liquid
preparation, check that the concentration of the liquid is correct.
Another way to save yourself time is to communicate what you
have ordered directly to the nurse. Having the nurse review the
orders allows immediate clarification and prevents annoyingly
time-consuming pages and clarification calls later. It also facilitates

10
 Common Mistakes 11

prompt and appropriate action on those orders by the nursing staff

and other ancillary personnel.
As mentioned in Chapter 2, Communicating With Colleagues
and Families, communication with supervising physicians should
occur whenever there is any doubt regarding a problem or its
management. Remember that notification does not always require
a telephone conversation. Do not hesitate to text-page your analysis
or plan of action. This allows supervising physicians to act at their
convenience. Remember, if you think that the supervising
physician might want to know, make the call, send a page, and
communicate!
The same policy applies for the family. The more willing you are
to communicate with the family, the more confident they will be
having you care for their child. Families have many reasons for
being present with their child in the hospital and equally many rea-
sons why they sometimes cannot be with their child. Do not judge
them. Involve them in their child’s care and communicate freely,
frequently, and honestly. A family would be very unlikely to com-
plain because they were told too much or because providers in the
hospital communicated too often with them.
Finally, as discussed in Chapter 2, Communicating With
Colleagues and Families, if you go to the effort of evaluating a
patient when on call or if you spend time communicating with
the attending physician or the family, document this in a note!
It takes only a moment to document, “Asked to evaluate [the
patient’s name] for [fever, chest pain, wheezing, etc.]. Vital signs
stable. Cardiorespiratory exam normal. No signs of sepsis, shock,
or meningitis. Tylenol ordered for fever control. No studies at this
time. Supervisor notified [or will be notified in AM]. Discussed with
family.” Always make sure that the date and time are recorded in
on-call documentation. Charting on-call encounters not only is for
your protection but also serves a vital role in communicating with
the team caring for the patient during the daytime. It is too impor-
tant to neglect.
Each of these mistakes occurs when physicians are busy, but
none of these actions is so time consuming that this is prohibitive.
Each is a fundamental part of conscientious care and ultimately
improves your efficiency. Improved efficiency will give you the
time to call home, grab dinner, or even potentially catch a little
sleep while on call.
 CHAPTER

4
Remembering
Your ABCs
Rainer G. Gedeit, MD

Nothing strikes fear in a house officer more than the “code page.”
When the “code team” is activated, there is a flurry of activity not
seen at any other time or place in the hospital. The “code team”
responds, as does everyone else within earshot of the page.
A frenzied group of doctors, nurses, medical students, respiratory
therapists, chaplains, pharmacists, phlebotomists, security guards,
and anyone else who is curious enough to see what the commotion
is all about show up. The team awaits orders from the “team
leader.” As you view the chaotic scene of a cardiac arrest in the
hospital, many people are busy doing things but may not truly
be helping. When you arrive at the scene, you must not be one
of these people. You need to be caring for the patient, not getting
in the way. The most important thing to remember comes from the
novel House of God by Samuel Shem, MD. “AT A CARDIAC
ARREST, THE FIRST PROCEDURE IS TO TAKE YOUR OWN
PULSE!” Stop, take a breath, assess the situation, determine what
you should be doing, and do it. Your role in the “code” will depend
on the situation. You may be the first person on the scene, the first
MD on the scene, the fifth MD on the scene, or the team leader.
Think about what you need to do and do it. If you are not needed,
move away.

THE ABCS
When you walk into a code situation, the things you learned in
kindergarten will come in handy. Go back and think about the
ABCs. You will probably not know the patient in front of you or
why that patient is dying; that is not what is important. The thing
to do is to try to reverse that process.

12
 Remembering Your ABCs 13

ELEVATOR THOUGHTS
Do I know the patient? If so, why would he or she be in trouble?
Think about what you know and how this could help in the treat-
ment of this patient.
What is my role in this situation? Am I a member of the code
team? Is this my patient? If you are a member of the code team,
remember your role and check in with the team leader to let
him or her know you are there and ready to assist. If this is your
patient, be ready to give a brief summary of the patient to the
team leader.

BEDSIDE
When coming upon the scene, assess the situation. Are you the first
physician there? If so, take the lead until others arrive. Otherwise,
assume your role or take the role you are assigned by the
team leader.
Quick-Look Test
Who is in the room, and what is being done for the patient? The
activity in the room can usually tell you how critical the problem
is. If you need to proceed with patient assessment and management,
start your ABCs.

A ¼ Airway
See whether the patient responds to stimulation. Call the child’s
name or gently shake the child; if there is no response, open the
airway. A jaw thrust or chin lift along with placing the patient
supine is the first thing to do. Make sure that suction equipment
is available in case the child vomits or has excessive airway secre-
tions. If it is not within reach, direct someone to get the
equipment needed.

B ¼ Breathing
Is the patient breathing? Look, listen, and feel. Is the chest rising,
can you feel air moving in and out, are there breath sounds? If yes,
maintain the airway and assess for cardiac output. If the patient is
not breathing, begin assisted breathing. Most hospital rooms will
have some type of resuscitation device (know where these are in
your institution), or the “code cart” that should have been brought
to the bedside will have a self-inflating bag and mask. Get the cor-
rect size mask and begin respirations. If this is not available, begin
mouth-to-mouth or mouth-to-mask rescue breathing. Two people
are often needed to accomplish effective bag-mask breathing: one
to open the airway and hold the mask in place and the other to
14 Introduction

ventilate with the bag. Once intubation equipment arrives, an arti-

ficial airway should be placed. This is the best way to assist breath-
ing and can be used as a portal to deliver drugs if intravenous (IV)
access is absent. The size of endotracheal tube that should be used
can be estimated by the formula (age in years/4) + 4. Make sure that
all the equipment works before attempting intubation. Nothing is
worse than placing a laryngoscope and having a dead lightbulb.
Use the personnel at the bedside to get the equipment; do not leave
the patient.
C ¼ Circulation
This is the most difficult part of the entire assessment. Feeling a
weak pulse in an infant who is receiving artificial respirations is
difficult, if not impossible. Your own adrenaline is rushing and
your hand is shaking. Take time to feel multiple areas for a pulse
(brachial, femoral, carotid). If there is no pulse, begin chest com-
pressions. Use the appropriate rate and method for the patient’s
age and size.
Placement of an IV catheter is required for drug delivery. Using
a large vein such as the antecubital should be attempted. If a cath-
eter is already in place, flush it to ensure patency. Placing a second
IV catheter is never wrong, in case the first one fails. Place the larg-
est catheter possible. If an IV catheter cannot be placed within 15 to
30 seconds, place an intraosseous catheter. If available, place the
patient on a cardiorespiratory monitor to evaluate the heart rate
and rhythm. If needed, give a dose of epinephrine.
As the basics are started, it is important to get an idea of
what might have precipitated this event. Someone familiar with
the patient (nurse, medical student, resident) must be available
to consult with the team leader, and the chart must be brought
to the bedside. Always show up if a patient you know is in
trouble.
If you are the team leader, you must take control of the situation
at the bedside. Your job is to ask for information and request that
medications be given, as well as control flow in the room. The most
difficult part of running the “code” is keeping control. Keep control
of your own emotions; a panicked leader makes a panicked team.
Make sure that everyone in the room is supposed to be there.
Excuse extraneous persons. If there are persistent “pests,” give these
people a job to do to keep them out of the way. The best team leader
I ever saw was a senior resident who looked the extraneous person-
nel in the eye and gave them each a job to do, outside the room (get
ice for labs, get the chart, check on the family, get equipment for a
blood draw). This left only the needed team members in the room.
Make sure that those performing ventilation and chest compres-
sions are doing an adequate job, and replace them every few
 Remembering Your ABCs 15

minutes as they fatigue (performing cardiopulmonary resuscitation

[CPR] is physically and emotionally draining).
As a team member, know your role (perform CPR, get informa-
tion, place an IV line, ventilate the patient, communicate with the
family). Do that job until you are told otherwise, or tell the leader
that you cannot perform the assigned duty or are becoming
fatigued (while performing CPR).
In some institutions, rapid-response extracorporeal support is
possible. Know your institution’s policies and procedures so that
this resource can be activated. Know what the response time will
be and what needs to be done to prepare the patient.
If the child is successfully resuscitated, transfer to a higher level
of care is required. The pediatric intensive care unit (PICU) should
be notified of the patient’s condition and needs (ventilator, pres-
sors, etc.). The team leader should accompany the patient to inform
the accepting team in the PICU of the situation.
The outcome of resuscitation becomes less favorable as time
goes on. At some point the team leader must “call the code.” This
is the worst time for any physician, and how to do this is important.
Verify how long the code has been ongoing, what medications were
given, and that there has been no response to resuscitation. Make
sure all agree that the code is to be stopped. Thank everyone for
their hard work. Stay with the patient and help clean up. If the doc-
tors help when it is over, it makes it easier for everyone, including
yourself.
When a code is over, whether the patient survived or not, those
involved are shaken. Take time to decompress. Get a drink of water,
coffee, or soda. Talk with those involved. At times a formal debrief-
ing may be needed, and most institutions can accommodate this.
Ask about a debriefing if you think it is needed.
 CHAPTER

5
Teaching (and Learning)
While On Call
James J. Nocton, MD

Although the first priority for physicians on call is always to care

for the patients, the hours spent on call are also of tremendous edu-
cational value. Every question, every call from a nurse, every dis-
cussion with a family, and every problem provide you with
opportunities to learn something new and to share things you
already know. The number of questions received from nurses
and families, the number of patients who require evaluation, and
the number of problems that you will need to manage will often
be far greater while you are on call than at other times. Although
you will probably be very busy responding to these situations, your
on-call experience will be much more rewarding and useful to you
if you remember to make an effort to teach, as well as remember to
learn, while you are taking care of patients.
Teaching while on call may initially seem to be an impossible
task. You may ask yourself, “How will I possibly find time to teach
when I will be taking care of so many patients, getting so many
pages, admitting so many new patients, and evaluating the other
problems that will invariably arise?” The answer to this question
involves understanding something about adult learning and using
some very effective methods of efficient teaching. Most of us think
of teaching as the active process of sharing information and learn-
ing as the passive process of receiving information from the
teacher. This is how we recall most of our formal education, with
heavy emphasis on “the lecture.” Needless to say, it will generally be
impossible for you to plan on giving lectures while you are on call,
and as it turns out, this is not a very effective way for adults to learn
anyway. Adults learn best when they are invested in the learning
process and take an active role in their own education. Therefore
the optimal way to teach is to allow your learners to be actively
involved in everything you are doing. When evaluating a problem,
evaluate it together with your student: inform the student of the

16
 Teaching (and Learning) While On Call 17

problem as it was presented to you, and give the student an

opportunity to think about it. Most importantly, give students
an opportunity to commit to a specific plan of action, whether it
be diagnostic testing, treatment, or both. Whenever possible, do
this before informing the student of your own plan of action. It
is the commitment on the part of students that gets them actively
invested in the learning process and permits you to teach more
effectively. After a student has thought the problem through inde-
pendently, developed an assessment, and formulated a plan, you
are then in a position to discuss that student’s thought process
and explain why or why not the assessment and plan are appropri-
ate. You will be a better teacher, and your student will learn far
more in this way than if you simply told the student what should
be done.
Two specific types of teaching can be very effective when time is
at a premium. The first is known as “priming.” Priming involves
setting a specific expectation for learners before engaging them
actively in the learning process. For example, if you are called to
see a child who has a sudden onset of abdominal pain, you might
ask your student, “When we go in to examine this patient, how will
we determine whether or not this may be appendicitis?” The stu-
dent can then respond, hopefully with a reasonable answer, and
you can comment on the answer and then proceed to examine
the patient. This process should not take a great deal of time, it
allows you to teach about a specific topic (the expected physical
findings in someone with appendicitis), and it permits the learner
to be invested in the learning process by making a commitment in
responding to your question.
“Modeling” is an even more efficient method of teaching.
Modeling is a more passive learning process than priming, but it
can also be very effective; it involves preparing the student for a
specific behavior or skill that you are about to demonstrate. For
example, if you are called to a child’s room by the nurse because
the child has a high fever, you may say to the student, “I want
you to watch how I discuss the significance of a fever with this
child’s parents.” Modeling requires that you choose a specific
teaching point and make sure that the student focuses on learning
about this specific topic. In this case the teaching point is the
significance of fever and how to explain it to families. You will
be discussing many other issues when you go into the room to
see the patient, but your student will understand what you would
like him or her to learn from this individual patient interaction.
You have been able to teach, while not spending any more time
than you would have had the student not been present.
When teaching an adult learner, particularly when using prim-
ing or modeling as a teaching method, it is important to keep two
18 Introduction

additional things in mind. First, teaching needs to occur at a suit-

able level for the learner. It may be appropriate to ask an average
third-year medical student about the expected physical findings in
someone with abdominal pain and appendicitis. It may not be
appropriate, even for the most advanced third-year student, to
ask about the expected physical findings, genetics, and treatment
of familial cold autoinflammatory syndrome. Of course, students
will vary with regard to their knowledge and skill, and you will need
to determine the individual level that is appropriate for each stu-
dent. This can be accomplished by asking students questions
and evaluating the appropriateness of their responses. Initially,
the questions may be very basic or superficial (“What are the phys-
ical findings in appendicitis?”). If the student answers the initial
questions easily, you can then determine the depth of the student’s
understanding by asking additional questions (“What are the
expected laboratory and radiographic findings in appendicitis?
Which patients should be taken to surgery? What is the prognosis
and long-term outcome of appendicitis?”). In this way, you should
be able to approximately determine the appropriate level at which
to teach.
The second principle to keep in mind is that teaching should
involve general rules that will be relevant to all learners. Physical
examination of the abdomen is a skill that nearly every physician
will need. Teaching about the genetics of familial cold autoinflam-
matory syndrome may be interesting, but it will not necessarily be
relevant to most medical students. They will remember a general
rule that they are able to apply repeatedly to the care of their future
patients. They will forget the specific minutiae regarding a rare dis-
ease that they are unlikely to see very often.
As you are making efforts to teach while on call, you may not
realize quite how much you have learned. When called regarding a
problem that you have never been confronted with before, you may
need to quickly review a textbook or an online database for infor-
mation. You may need to discuss the problem with a consultant. It
is to your educational benefit to take advantage of as many of these
resources as you can, as time permits. Sharing what you are learn-
ing from these resources with your student will sharpen your
understanding of the problem. Reviewing published information
and discussing the problem with those who have more experience
will also enable you to feel reassured regarding your evaluation and
treatment of the patient.
Much of the learning that you will experience while on call will
occur as you discuss problems with colleagues, either while you are
on call or at a later time, such as during morning rounds. Present-
ing your patients at rounds and discussing how you evaluated and
managed problems will give you an opportunity to ask how others
 Teaching (and Learning) While On Call 19

might have proceeded in certain situations. This will permit you to

reflect on your own decisions and allow you to ask the question to
yourself, “What could I have done differently?” By doing so, you
will increase your knowledge, improve your decision making,
and improve the care you will provide to your patients the next
time you are on call.
Teaching (and learning) can occur while you are on call. All that
is required is some effort on the part of the teacher and willingness
to invest in learning by the student. Hopefully, this chapter has
explained how teaching can be very effective without taking a great
deal of time away from patient care responsibilities. By making the
effort to teach, the physician on call can make the on-call experi-
ence more educational, rewarding, and satisfying.
 CHAPTER

6
Abdominal Pain
Rachel Weigert, MD

Abdominal pain in a hospitalized pediatric patient should always

be evaluated promptly. Although it is an extremely common and
often benign complaint in all children, abdominal pain should
never be dismissed as insignificant until a thorough evaluation
has excluded potentially serious causes. In addition, abdominal
pain should not be empirically treated with analgesics until a thor-
ough evaluation has determined that the patient does not have a
surgical or other life-threatening condition. In some instances
the cause of the abdominal pain may not be clear. After an initial
evaluation, it may be necessary to adopt an expectant approach that
involves performing serial examinations at regular intervals, pre-
scribing analgesics when necessary for discomfort, and continuing
to consider possible diagnoses until the cause becomes clear or the
pain resolves.

PHONE CALL
Questions
1. How old is the patient?
2. Why is the patient in the hospital?
3. What are the patient’s vital signs?
4. How does the patient rate the pain?
5. How long has it been present?
6. Is it localized?
7. Does the child have any nausea, vomiting, or diarrhea?
8. Is there blood in the emesis or stool?
9. Has the child complained of abdominal pain previously during
this admission?
The patient’s vital signs, the degree of severity, and the duration
of pain allow a quick determination of the urgency of the situation.
Stable vital signs with mild pain present for several hours are
reassuring and not suggestive of an acute intra-abdominal process.

22
 Abdominal Pain 23

Understanding the location of the pain, the age of the child, the rea-
son for hospitalization, and any associated symptoms will allow
you to begin to consider a specific diagnosis. Previous complaints
may signal that an evaluation has been performed before or that the
cause of the pain is already known.

Orders
1. Nil per os (NPO). The patient should have NPO until further
evaluated.
2. Place IV. If pain is severe and the vital signs indicate shock
(tachycardia and hypotension), an intravenous (IV) line should
be placed and a 20-mL/kg bolus of isotonic fluid (normal saline
or lactated Ringer’s solution) should be infused.
Inform Registered Nurse (RN)
If the pain is severe or the vital signs make you concerned about an
intra-abdominal emergency or shock, alert the RN that the patient will
be evaluated immediately. If the pain is mild and vital signs are normal,
the nurse should be informed of when you expect to see the patient.

ELEVATOR THOUGHTS
On your way to evaluate the patient, you can contemplate the many
causes of abdominal pain. You may consider the location of the
organs within the abdomen, pelvis, and retroperitoneum and the
potential pathologic processes that may affect each organ, the
source of blood supply to each area, and whether the pain is related
to that organ or referred. When abdominal pain is generalized or
cannot be localized (e.g., in an infant or toddler), a wide differential
should be considered. Abdominal pain may also result from systemic
metabolic abnormalities (e.g., diabetic ketoacidosis, porphyria), as
well as processes at distant anatomic sites (e.g., streptococcal
pharyngitis, pneumonia):
Epigastric pain Aortic dissection
Carditis (pericarditis, myocarditis)
Pancreatitis
Peptic ulcer (perforation)
Gastroesophageal reflux (heartburn)
Gastritis
Esophagitis
Left upper Splenic rupture
quadrant pain Splenic infarct
Splenic abscess
Subphrenic abscess
Left lower lobe pneumonia
24 Patient-Related Problems

Right upper Hepatitis

quadrant pain Cholecystitis
Biliary obstruction (gallstones)
Liver abscess
Subphrenic abscess
Fitz-Hugh–Curtis syndrome (gonococcal
perihepatitis)
Right lower lobe pneumonia
Left lower Ovarian torsion
quadrant pain Mittelschmerz (ovulation)
Ovarian cyst
Psoas abscess
Ectopic pregnancy
Pelvic inflammatory disease
Renal stone
Colitis (infectious or inflammatory)
Incarcerated hernia
Testicular torsion
Epididymitis
Right lower Appendicitis
quadrant pain Ruptured appendix/abscess
Mesenteric adenitis
Ovarian torsion
Ovarian cyst
Psoas abscess
Ectopic pregnancy
Pelvic inflammatory disease
Renal stone
Incarcerated hernia
Testicular torsion
Epididymitis
Hypogastric pain Cystitis
Bladder obstruction
Ovarian torsion
Pelvic inflammatory disease
Testicular torsion
Generalized pain Any of the preceding conditions
Mesenteric adenitis
Typhlitis
Constipation
Ileus
Gastroenteritis
Viral infection (e.g., mononucleosis)
Inflammatory bowel disease
Lactose intolerance
 Abdominal Pain 25

Vasculitis (Henoch-Sch€onlein purpura,

polyarteritis nodosa, systemic lupus
erythematosus)
Mesenteric thrombosis
Trauma (including nonaccidental)
Pharyngitis
Diskitis
Porphyria
Bezoar
Peritonitis
Sterile peritonitis (systemic juvenile
idiopathic arthritis, familial Mediterranean
fever, lupus)
Generalized pain Abdominal epilepsy
Hereditary angioedema
Abdominal migraine
Tumor
Adrenal insufficiency
Bowel obstruction (volvulus, malrotation)
Superior mesenteric artery syndrome
Sickle cell crisis
Pregnancy
Meckel diverticulum (with secondary
intussusception)
Intussusception
Psychologic (somatization)

MAJOR THREAT TO LIFE

• Obstruction leading to perforation
• Ischemic bowel (e.g., from volvulus, intussusception, or vasculitis)
• Infectious peritonitis (with or without a perforated viscus)
• Gastrointestinal (GI) bleed
• Ectopic pregnancy
• Splenic rupture
• Pericardial tamponade or myocarditis
• Aortic dissection
The biggest concern is shock. Exsanguination and infection
are the most immediate sequelae of the possible causes just listed
because hypovolemic or septic shock may occur quickly. Cardio-
genic shock may also occur if severe pericarditis or myocarditis
is the cause of the abdominal pain. In addition to the major threats
to life, torsion of an ovary or testicle can threaten their viability and
are considered emergencies requiring prompt intervention.
26 Patient-Related Problems

BEDSIDE
How Does the Patient Look?
Does the patient appear comfortable, uncomfortable, or severely ill?
An uncomfortable or severely ill patient should be examined
immediately to look for signs of peritonitis. If peritonitis is sus-
pected, empiric antibiotics, IV fluids, and immediate surgical eval-
uation are indicated. In general, patients with peritonitis appear
uncomfortable and may prefer to lie motionless and avoid any
movement of the peritoneum. A patient who has recently ruptured
a viscus (e.g., a perforated appendix) may suddenly appear more
comfortable because the obstruction has been relieved. The dis-
comfort may also be intermittent, as in intussusception or renal
colic, with episodic pain being punctuated by periods of relative
comfort. An infant or a child who has been receiving narcotics
or steroids may appear deceptively comfortable despite a signifi-
cant intra-abdominal pathologic condition, so it is important to
obtain a medication history.
Airway and Vital Signs
Hypotension and tachycardia are signs that shock may be present,
although tachycardia alone may be present due to fever, pain, or ane-
mia. Fever and abdominal pain should raise suspicion regarding
infectious causes, which could be relatively mild (gastroenteritis)
or life threatening (peritonitis). Tachypnea suggests pneumonia or
an attempt to compensate for a metabolic acidosis associated with
diabetic ketoacidosis, shock, or ischemic bowel. If a patient has
abdominal pain and is tachypneic with shallow respirations, they
may be splinting due to pain.
Selective History
When gathering information about pain in any location, including
the abdomen, precise characterization of the pain is essential.
Duration: How long has the pain been present?
If the pain has been ongoing for several days, it is most likely not
an intra-abdominal emergency. Acute onset or sudden worsening
of chronic pain is concerning and warrants immediate evaluation.
Location: Where is the pain? Does it radiate? Has it changed location?
As noted earlier, the location of the pain may help to narrow the
diagnostic possibilities. Pain moving from the umbilicus to the right
lower quadrant is strongly suggestive of appendicitis. Pain radiating
to the shoulder suggests pericarditis or irritation of the diaphragm, as
with a subphrenic abscess, perforated ulcer, or hepatobiliary pathol-
ogy including (Fitz-Hugh–Curtis syndrome or biliary colic). Pain
radiating to the back may occur with aortic dissection or pancreatitis.
Pain with radiation to the groin suggests ureteral irritation, as with
renal stones. In young children, the response to “Where does it
 Abdominal Pain 27

hurt?” invariably involves pointing directly to the umbilicus and

therefore may be less reliable than in older patients.
Character: Is the pain aching, burning, crushing, or sharp? Is it
constant or intermittent? Does it vary in intensity? What makes it
feel better? Worse?
Esophagitis, gastritis, or peptic ulcers may often be described as
burning. Aching pain generally indicates a more diffuse or distant
cause (e.g., pneumonia, porphyria), whereas sharp pain tends to be
indicative of a more localized process. Biliary or renal colic and
intussusception may cause severe but intermittent pain. If walking
or moving seems to provide relief, peritonitis is much less likely.
Associated symptoms: Does the child have any related symptoms?
If diarrhea is present, infectious causes need to be considered
further. In children, vomiting may occur with any intra-abdominal
process but should raise suspicion of infection or bowel obstruc-
tion. Hematemesis is strongly suggestive of gastritis, esophagitis,
or an ulcer. Bilious or feculent emesis implies bowel obstruction.
Melanotic stools are suggestive of bleeding in the upper GI tract.
Hematochezia indicates bleeding in the lower GI tract, whereas
streaks of blood on the stool may indicate constipation. Pharyngi-
tis, coughing, rashes, headache, fever, and other systemic symp-
toms may likewise help with the differential diagnosis. If the
child has been eating normally and appears well, the likelihood
of a significant problem is very small.

Selective Physical Examination

Vital signs Hypotension may ensue rapidly; therefore
repeating blood pressure and other vital sign
measurements is a good idea. Remember that
in infants and young children, tachycardia
alone may be indicative of shock. By
increasing the heart rate, cardiac output can be
enhanced enough to maintain “normal” blood
pressure; hypotension may not occur until
relatively late in the course of septic or
hypovolemic shock.
HEENT* Scleral icterus (suggestive of hyperbilirubinemia
and possible liver disease), pharyngeal
erythema or exudate (streptococcal infection,
infectious mononucleosis), periorbital edema
(angioedema).
Neck Adenopathy (streptococcal pharyngitis,
mononucleosis), jugular venous distention
(cardiac tamponade).

*HEENT, Head, Eyes, Ears, Nose, and Throat.

28 Patient-Related Problems

Chest Rales, wheezes, decreased breath sounds

(pneumonia, congestive heart failure [CHF]
from myocarditis, pericarditis).
Heart Muffled or distant heart sounds (pericardial
effusion), friction rub (pericarditis).
Abdomen 1. Observe. Protuberance may be a sign of bowel
obstruction, ascites, or an intra-abdominal
mass (intussusception, hernia).
2. Auscultate. The purpose of auscultation is
twofold. You can begin to listen to the
abdomen but also lightly palpate
simultaneously. The absence of bowel sounds
is consistent with ileus (which may occur with
any condition). High-pitched bowel sounds
are associated with obstruction, and
hyperactive bowel sounds may be appreciated
with gastrointestinal infection.
3. Palpate. Rigidity, rebound tenderness, and
guarding all suggest peritonitis. Localized
tenderness may be present even if the pain is
described as generalized. A fluid wave may be
appreciated if ascites is present. Cautiously
palpate the liver and spleen because an
enlarged spleen may be more easily ruptured.
4. Percuss. Evaluate the size of the liver. Shifting
dullness is indicative of ascites.
Rectal Tenderness (appendicitis, pelvic inflammatory
disease), impacted stool (constipation), positive
occult blood test result (gastritis, bleeding
peptic ulcer, ischemic bowel, Meckel
diverticulum, inflammatory bowel disease,
intussusception, vasculitis). Note: A patient
with abdominal pain has not been fully
evaluated without a rectal examination!
Genitourinary All adolescent girls should undergo a pelvic
examination for any unexplained abdominal
pain (pelvic inflammatory disease, ectopic
pregnancy); prepubescent and pubescent boys
should undergo testicular examination
(torsion, edema associated with vasculitis).
Skin Rashes (vasculitis, lupus, systemic juvenile
idiopathic arthritis, scarlet fever), Cullen
sign (purpuric discoloration of the skin of
the abdomen seen in hemorrhagic
pancreatitis), hyperpigmentation (adrenal
insufficiency).
 Abdominal Pain 29

Extremities Peripheral edema (CHF, renal disease,

vasculitis), pulses and capillary refill (assess for
potential shock).
Neurologic Altered mental status (shock, toxin, porphyria,
diabetic ketoacidosis).

Selective Chart Review

After the history and physical examination, the cause of the
abdominal pain may still be unclear. Selectively reviewing the med-
ical record may provide useful information.
What medications is the patient taking?
Some medications are notorious for causing abdominal
symptoms, and abdominal pain is listed as a potential side
effect of nearly all drugs. Attributing pain to a medication
may be reasonable but should be considered a diagnosis of
exclusion. Steroids and nonsteroidal antiinflammatory medica-
tions may potentially cause gastritis, esophagitis, and peptic
ulcer. Narcotics and other medications may induce constipation
and thereby result in abdominal pain. Pancreatitis and hepatitis
are also side effects of multiple medications. Remember that
Clostridium difficile infection, colitis, and subsequent abdominal
pain may develop in any patient who has been receiving anti-
biotics. Conversely, both narcotics and steroids can mask
abdominal pain.
If the child is an adolescent girl, when was the last menstrual
period? Is she sexually active?
Missed menses is suggestive of pregnancy, ectopic or intrauter-
ine. Known sexual activity confirms the possibility of pelvic inflam-
matory disease.
Has the child complained of this type of pain before?
If the same pain has prompted several previous calls, the prob-
lem is much less likely to be an acute one requiring extensive eval-
uation and emergency management in the middle of the night.
However, acute changes in chronic pain need to be evaluated.
The child may have a significant problem causing the pain (lactose
intolerance, inflammatory bowel disease), but further evaluation
may be able to be deferred after life-threatening possibilities are
excluded.

Management
At this point, either a specific diagnosis is evident or you will be able
to shorten the list of potential causes. If the specific cause of the
abdominal pain remains questionable, you should at least be able
to determine whether the patient (1) is critically ill and requires
immediate intervention, (2) is in discomfort but has no evidence
30 Patient-Related Problems

of an immediately life-threatening process, or (3) is only mildly

uncomfortable and life-threatening processes can be excluded. If
the patient is critically ill, you need to pursue management and addi-
tional diagnostic studies simultaneously. A patient who is stable but
uncomfortable may require further diagnostic studies before appro-
priate management is begun. A mildly uncomfortable patient may
not require any specific diagnostic tests or management at the
moment and may often be managed expectantly.
Critically Ill Patients
A critically ill patient with abdominal pain usually has hypovolemic
shock, septic shock, or a combination of both as a result of perfo-
ration, peritonitis, or exsanguination. Immediate management
involves addressing the issues of shock and infection simulta-
neously and necessitates consultation with pediatric surgeons.
Management should proceed as follows:
1. Volume
Immediate expansion of intravascular volume helps to improve tis-
sue perfusion. Normal saline or lactated Ringer’s solution can be
given as a 20-mL/kg IV bolus as fast as possible. Evaluate the
response of the heart rate, capillary refill, and blood pressure
and repeat if indicated. If cardiogenic shock is a possibility, volume
expansion should be performed cautiously, assessing for signs of
fluid overload including hepatomegaly, rales, and jugular venous
distention (JVD) after each bolus administration. If the patient is
known to be bleeding, whole blood can also be used to expand
intravascular volume. If time does not permit cross matching,
O-negative blood should be used.

2. Oxygenation
Oxygen should be administered and an arterial blood gas measure-
ment obtained to assess the adequacy of oxygenation and tissue
perfusion.
3. Laboratory Studies
Additional laboratory tests include a complete blood count with dif-
ferential, prothrombin time, partial thromboplastin time, blood
culture, urinalysis, urine culture, human chorionic gonadotropin-β
(HCG-β) if female, amylase, lipase, lactate dehydrogenase, blood
gas, and electrolyte determinations, as well as blood for typing
and cross matching. Additional labs are dictated by your differential
diagnosis. If concerned about a renal etiology, add blood urea nitro-
gen (BUN) and creatinine. If the liver or biliary tract is concer-
ning, aspartate transaminase (AST), alanine transaminase (ALT),
γ-glutamyltransferase (GGT), and bilirubin would be appropriate.
 Abdominal Pain 31

4. Paracentesis
If sepsis is suspected and the patient has ascites, diagnostic para-
centesis should be performed and the fluid cultured to evaluate
for bacterial peritonitis.
5. Antibiotics
After cultures are obtained, broad-spectrum antibiotics should be
started immediately to empirically treat gut anaerobes, as well as
gram-positive and gram-negative pathogens (the combination of
ampicillin, gentamicin, and metronidazole is one possible choice).
6. Radiography
Radiographic studies need to be performed with a portable machine
at the patient’s bedside and should include anteroposterior (AP)
views of the abdomen, supine and erect if possible. If the patient
is an infant, a cross-table lateral view allows the detection of free
air in the peritoneum. A patient who cannot stand should have a lat-
eral decubitus view. An AP view of the chest should also be obtained
to evaluate the lung fields (the potential exists for acute respiratory
distress syndrome, pneumonia) and heart size (to exclude pericardial
effusion, myocarditis, CHF) and to look for free air under the dia-
phragm indicating perforation. Air-fluid levels suggest bowel
obstruction or ileus. A “sentinel” loop suggests pancreatitis. Lead
may be seen as radiopaque “chips” throughout the bowel. Constipa-
tion should be fairly obvious, as should an intra-abdominal mass.
A fecalith in the right lower quadrant is suggestive of appendicitis.
7. Consultation
Surgical consultation is mandatory if a perforated viscus, splenic
rupture, intra-abdominal abscess, aortic dissection, appendicitis,
intussusception, volvulus, malrotation, psoas abscess, incarcer-
ated hernia, ischemic bowel, Meckel diverticulum, tumor, or tes-
ticular torsion is suspected. Gynecologic consultation may be
necessary if ovarian torsion, pelvic abscess, or ectopic pregnancy
is suspected.
8. Vasopressors
If fluid resuscitation alone does not improve the signs of shock,
vasopressors may be necessary (see Chapter 25, Hypotension
and Shock).

9. Pain Control
Once the initial work-up is underway, pain control is paramount.
IV opioid medications including morphine or hydromorphone
should be given and patient or nurse-controlled analgesia should
32 Patient-Related Problems

be considered if the cause of pain is determined to be ongoing, such

as in the case of pancreatitis or renal or biliary colic. Consideration
should be taken of the constipating nature of these medications.

Patients Who Are Uncomfortable but Do Not Have

Cardiorespiratory Compromise
If the patient is not in shock, additional evaluation may proceed
before any specific intervention is made. However, one should
remain alert for the possibility that the patient may suddenly
become critically ill (e.g., if ischemic bowel progresses to a perfo-
rated bowel or if intussusception progresses to ischemia and necro-
sis). Laboratory studies and radiographs as outlined for critically ill
patients may be helpful diagnostically. If gastroenteritis is a consid-
eration, stool may be tested for rotavirus, Salmonella, Shigella,
Yersinia, and Escherichia coli, and if the patient has been taking
antibiotics, C. difficile toxin. If laboratory testing and initial radio-
graphs do not lead to a diagnosis, consideration may be given to
performing abdominal or pelvic ultrasound studies (to exclude
an abscess, tumor, ovarian or testicular torsion, ectopic pregnancy,
urolithiasis, intussusception), computed tomography (CT) of the
abdomen and pelvis (abscess, tumor, appendicitis), angiography
(mesenteric thrombosis or vasculitis), or a Meckel scan. Whether
these tests are performed immediately or not depends on clinical
suspicion and the potential for morbidity if the diagnosis is
delayed. If significant abdominal pain persists, the patient should
remain NPO with IV hydration at a maintenance rate until the
cause is found. Further management depends on the diagnosis.

Patients With Mild Discomfort

In some cases a patient with mild discomfort may need additional
studies immediately to exclude the possibility of bacterial infection
or a surgical abdomen. Laboratory studies and radiographs, as out-
lined earlier, may provide additional evidence of infection (dra-
matic increase in the white blood cell count with a left shift) or a
surgical abdomen (free air), or they may help to exclude these pos-
sibilities. However, not every patient requires further study beyond
a history and physical examination. If a potentially serious cause of
the abdominal pain is unlikely, expectant management is reason-
able, provided that the patient is observed and examined fre-
quently. If the patient wants to eat, maintenance of NPO status
is usually unnecessary. Acetaminophen, 10–15 mg/kg every 4 to
6 hours, or a hot pack may be used to alleviate pain. Tramadol
may be considered if pain is not alleviated with initial measures.
 C HA P TE R

7
Altered Mental Status
Michael Girolami, MD

Altered mental status (AMS) may be defined as a change in con-

sciousness. Almost any pathologic process involving the central
nervous system (CNS) may cause AMS. Changes that may be
caused by these processes include irritability, lethargy, syncope, sei-
zures, and unresponsiveness. Immediately recognizing that there
has been a change in mental status is initially more important than
defining the exact type of alteration. The history of how the
patient’s mental status has changed and the physical examination
will help narrow the list of diagnostic possibilities and guide the
initial approach to management. This chapter discusses the evalu-
ation and management of children in whom irritability, lethargy,
syncope, delirium, or coma develop while in the hospital. Delirium
is defined as an acute change in attention, awareness, and cognition
and is usually manifested as inappropriate speech or bizarre behav-
ior. Lethargy implies profound fatigue and lack of interest in
any activity or conversation. If the lethargy is severe, the term
obtundation is sometimes used. Typically a lethargic child can be
awakened but then will return to a state of slumber. Stupor refers
to a state characterized by lapses of consciousness. A stuporous
child can only be awakened by repeated stimuli. Finally, coma is
a state of profound unconsciousness. Irritability in an infant (see
Chapter 12) and seizures (see Chapter 29) are discussed elsewhere.

PHONE CALL
Questions
1. How old is the patient?
2. What is the patient’s reason for admission?
3. What are the patient’s vital signs?
4. How is the child breathing? Is the airway compromised?
5. How is the child behaving? Was there a brief period of unre-
sponsiveness suggesting seizure or syncope?

33
34 Patient-Related Problems

6. Has this occurred in the past? What is the child’s baseline men-
tal status?
7. Were any new medications started, or stopped recently? When
was the last time any narcotics were given?
The patient’s reason for admission is vitally important
because it can help to acutely determine the etiology of the
AMS. Assessment of vital sign trends, respiratory pattern, and
current behavior help to determine whether the change in mental
status is associated with a critical ongoing process, such as
increased intracranial pressure (ICP), or a self-limited event, such
as a seizure or syncope. If the airway is compromised, plans
should be made to intubate the child immediately. The responses
to these questions also help to differentiate between the various
types of alterations in mental status. If this was only a brief epi-
sode and mental status is improving, an “event” may have
occurred that does not require immediate treatment. However,
if mental status remains altered, an ongoing process should be
considered and management started immediately. For example,
if the Cushing triad of systolic hypertension (widening pulse
pressure), bradycardia, and irregular respirations is present,
increased ICP should be suspected and management begun
immediately. If the child has a history of similar events, this
may help to direct your initial thoughts regarding the cause of
the change in mental status. Defining the child’s baseline mental
status and history of previous alterations in mental status helps to
clarify the significance of the current change. Review of the
patient’s medications can also be very helpful. New medications,
missed doses, and pain medications may all cause acute changes
in a patient’s mental status.

Orders
1. Immediate blood glucose should be checked. If hypoglycemia is
present (<40 mg/dL), give intravenous (IV) bolus of 2.5 mL/kg
of 10% dextrose (D10) in infants or children up to 12 years
of age; if older than 12 years, give 1 mL/kg of 25% dextrose
(D25), or 0.5 mL/kg of 50% dextrose (D50). If there is no IV
access, glucagon 0.03 mg/kg intramuscularly (IM) or subcuta-
neously (SQ; max 1 mg) may be administered. Keep in mind,
hyperglycemia may also be associated with AMS, as in diabetic
ketoacidosis.
2. Continuous pulse oximetry should be ordered if the
patient has abnormal respirations or a cardiac or respiratory
illness.
3. If vital signs changes indicate increased ICP or the airway
appears to be compromised, preparation should be made for
intubation.
 Altered Mental Status 35

4. If the history is suspicious for syncope, an electrocardiogram

(ECG) should be performed at the bedside to evaluate for car-
diac dysrhythmia. One should also consider continuous cardiac
or telemetry monitoring.
5. An intravenous (IV) line should be placedin patientswith delirium,
obtundation, stupor, or coma. If an IV line is to be placed, you may
ask the nurse to draw off some blood in anticipation of the need for
laboratory studies. A complete blood count (CBC), basic metabolic
panel (BMP), magnesium, and phosphate should be obtained, and,
depending on the clinical history, other labs may need to be
considered.
Inform RN
Acute AMS always requires immediate evaluation and may require
immediate treatment. It is critical that the etiology be determined as
soon as possible so that appropriate management can be initiated.

ELEVATOR THOUGHTS
As previously mentioned, AMS may have multiple etiologies. It
may be due to systemic infection or disorder, metabolic distur-
bances, or a primary intracranial process. In young children,
almost any illness can lead to irritability or lethargy. Therefore,
in this population, it is important to also consider illnesses localized
to organ systems other than the CNS. If consciousness is impaired,
the process must be affecting both cerebral hemispheres or the
brain stem. The mnemonic AEIOU TIPS (Table 7.1) covers most
of the etiologies of AMS. A more detailed list of potential causes:
Intracranial Infection (meningitis, encephalitis, abscess)
Processes Hemorrhage (subarachnoid, subdural,
epidural parenchymal) secondary to
aneurysm, trauma (including shaken baby
syndrome), coagulopathy, or arteriovenous
malformation
Tumor
Concussion secondary to head trauma
Cerebral edema secondary to trauma
Cerebral thrombosis
Cerebral vasculitis
Cerebritis
Psychosis
Hydrocephalus
Seizure disorder
Acute confusional migraine (“Alice in
Wonderland” syndrome)
Acute disseminated encephalomyelitis
Autoimmune encephalitis
36 Patient-Related Problems

Metabolic Diabetic ketoacidosis

disturbances Hyperammonemia (e.g., Reye syndrome, urea
cycle disorders, liver disease)
Hypoglycemia
Hyponatremia, hypernatremia
Hypocalcemia, hypercalcemia
Hypokalemia
Hypoxemia (e.g., carbon monoxide poisoning,
cardiac failure, respiratory failure)
Drugs and toxins (e.g., narcotics, lead,
barbiturates, alcohol, salicylates,
acetaminophen)
Mitochondrial encephalomyopathies
(e.g., Leigh disease, Zellweger syndrome)
Systemic Hypothyroidism, hyperthyroidism
illnesses Uremia (e.g., hemolytic-uremic syndrome)
Addison disease
Congestive heart failure
Cardiac dysrhythmia
Pulmonary failure
Hypertension with encephalopathy
Heat stroke
Shock (distributive, obstructive, hypovolemic,
cardiogenic, or combined)
Fever
Human immunodeficiency virus
encephalopathy
Systemic lupus erythematosus with cerebritis
Malnutrition (with thiamine deficiency)
Thrombotic thrombocytopenic purpura
Burn encephalopathy
“Hospital” or “intensive care unit”
encephalopathy
Posterior reversible encephalopathy syndrome
(PRES)
In children and adolescents whose history is consistent with
syncope, the following should also be considered:
Vasovagal episode
Cardiac conduction disturbance (heart block)
Dysrhythmia (e.g., supraventricular tachycardia, long QT syndrome)
Narcolepsy
Cough syncope
Severe anemia
Breath-holding
Hyperventilation
Cervical vertebral anomalies
 Altered Mental Status 37

TABLE 7.1 Differential of Altered Mental Status

A Alcohol, Abuse
E Epilepsy, Encephalopathy, Electrolytes, Endocrine
I Ingestion, Insulin, Intussusception, Inadequate Fluid
O Overdose, Occult Trauma, Obstructed ventriculoperitoneal (VP) shunt,
Oxygen Deficiency
U Uremia
T Trauma, Temperature Abnormality, Tumor
I Infection
P Poisonings, Psychiatric, Postictal
S Shock, Stroke, Space Occupying Lesion (Intracranial)

MAJOR THREAT TO LIFE

• CNS infection
• Intracranial hemorrhage
• Increased ICP
• Metabolic disturbance
• Shock
• Dysrhythmia
• Organ failure (e.g., heart, lung, liver, or kidney)
• Status epilepticus
• Aspiration resulting from an inability to protect the airway
The most worrisome etiologies in the acute phase include
infection, hemorrhage, and mass lesions leading to increased
ICP and possible herniation. Other etiologies with high morbidity
and potential mortality include metabolic disturbances, shock,
dysrhythmias, and organ failure. Status epilepticus may also have
substantial deleterious effects on the CNS. Regardless of the
underlying cause, a stuporous, obtunded, or comatose patient
may not be able to protect their airway and is therefore at greater
risk for aspiration.

BEDSIDE
Quick-Look Test
Is the patient currently alert, comfortable, and oriented (suggesting
an episodic event, e.g., a self-limited seizure or syncope), or is the
patient actively seizing, unconscious, lethargic, irritable, combative,
or sleepy (suggesting an ongoing process)?
The latter patient needs an immediate focused exam with atten-
tion to signs that would suggest increased ICP, shock, infection,
brain herniation, or a combination of these conditions. If the
patient is actively seizing, management to control the seizure
38 Patient-Related Problems

should begin simultaneously with further examination (see

Chapter 29). Isolated irritability is present in many hospitalized
children and, in the absence of other abnormal neurologic findings,
is not necessarily an indicator of CNS pathology. However, keep in
mind that mental status may change rapidly and a child who is
irritable may progress to stupor, obtundation, or coma and should
be closely monitored.

Airway and Vital Signs

Can the child protect his or her airway? (Are cough and gag reflexes
present?)
If the answer is no, the child should be intubated as soon as
possible.
A full set of vital signs should be evaluated, with particular
attention paid to signs of infection or increased ICP—two of the
major threats to life. Fever should raise the suspicion of CNS infec-
tion, certain toxins, or septic shock. Although the absence of fever
makes infection less likely, patients with brain abscesses are not
necessarily febrile. Cushing triad is an ominous finding that occurs
as a late sign of significantly increased ICP and suggests impending
herniation. Normal vital signs may be present in a patient in whom
increased ICP is developing. Management should begin immedi-
ately if Cushing triad is present.
Hypertension may also be the cause (rather than the result) of
an encephalopathy, usually associated with renal disease. Hypoten-
sion implies shock (distributive, obstructive, hypovolemic, cardio-
genic, or combined) and also requires immediate management (see
Chapter 25). Tachycardia may be present in several etiologies,
including sepsis and hyperthyroidism, but may be an early finding
in shock. Keep in mind, heart rates greater than 220 beats per
minute (BPM) in infants and 180 BPM in older children are sug-
gestive of supraventricular tachycardia. Tachypnea may reflect
pulmonary disease, pain, intoxication/ingestion, or compensation
for acidosis, as in diabetic ketoacidosis.

Focused Physical Examination I

After the quick-look test and a check of vital signs, an initial
focused physical examination should be performed with the goal
of determining the likelihood of increased ICP, impending brain
herniation, or CNS infection (meningitis, encephalitis, or abscess)
because immediate management is necessary if any of these condi-
tions is suspected.
 Altered Mental Status 39

Head Bruising over the mastoid (Battle sign), “raccoon

eyes,” or depressions or deformity of the skull (all
indicators of significant head trauma); bulging or
sunken fontanelle
Eyes Pupil size and reactivity to light. A single dilated,
unreactive pupil may indicate herniation of the
ipsilateral temporal lobe. Bilateral dilatation is
associated with a postictal state and certain drugs
(e.g., atropine, cocaine, mydriatic agents). The
pupillary reflex is typically intact in medical causes
of AMS; however, if there is a structural CNS
insult, one would expect the pupils to be unequal,
sluggish, or unreactive.
Fundi retinal hemorrhage is associated with
trauma. Papilledema suggests increased ICP
(Fig. 7.1)
Extraocular movements. Third nerve palsy (dilated
pupil, lateral and inferior displacement of the eye,
ptosis) may be associated with temporal lobe
herniation. Sixth nerve palsy (absence of lateral
movement) may be associated with increased ICP
and may be unilateral or bilateral
Ears Hemotympanum or blood in the external canal
(head trauma)
Nose Cerebrospinal fluid rhinorrhea (trauma)
Neck Nuchal rigidity (meningitis), Kernig and Brudzinski
signs (meningitis)
Chest Respiratory pattern (Table 7.2) (may indicate
herniation)
Cardiac Bradycardia (increased ICP), extreme tachycardia,
or abnormal rhythm
Extremities Posturing (see Table 7.2) (may indicate
herniation)
Neurologic Level of consciousness, orientation, focality,
Glasgow Coma Scale score (asymmetry in muscle
tone, strength, spontaneous movement, reflexes)
What is the Glasgow Coma Scale (GCS) (Table 7.3)? GCS is a
15-point neurologic scale used to record and describe the general
level of consciousness in patients. A GCS score less than 8 is
an indication that severe CNS abnormalities are present and
the child is at risk for respiratory compromise. Intubation is
indicated.
40 Patient-Related Problems

FIGURE 7.1 Disk changes seen in papilledema. A, Normal. B,

Early papilledema. C, Moderate papilledema with early hemor-
rhage. D, Severe papilledema with extensive hemorrhage. (From
Marshall SA, Ruedy J: On Call: Principles and protocols, ed 4, Phil-
adelphia, 2004, Elsevier, p 123.)

Management I
Increased Intracranial Pressure
If elevated ICP is suspected, treatment must be initiated in an
attempt to reduce the volume of the intracranial contents (i.e.,
brain, cerebrospinal fluid [CSF], and blood) and ensure adequate
cerebral perfusion pressure (CPP). Mean arterial pressure
(MAP) must be kept greater than ICP to maintain CPP
(CPP ¼ MAP  ICP). This is done by the following techniques:
1. Positioning. Elevate the head of the bed to 30 degrees. Maintain
the head in the midline position. Do not elevate above 40
degrees because this can lead to decreased CPP.
2. Treat hypoxia, hypercarbia, and hypotension if present. Vaso-
pressors may be necessary to maintain appropriate MAPs.
 TABLE 7.2 Localization of Cerebral Dysfunction

Level of Hemispheres Respiratory Pattern Pupils Vestibuloocular Reflex Motor Response

Diencephalon Regular or Small reactive Present, normal Localized noxious stimuli with nonparetic limb;
Cheyne-Stokesa later decorticate posturing
Midbrain-upper pons Hyperventilation or Mid position, Absent or abduction only Decerebrate or no movement
Cheyne-Stokes fixed
Lower-pons to upper Ataxic Mid position, Absent Triple flexion responseb or no movement
medulla fixed
Medulla Irregular or none Mid position, Absent Absent

Altered Mental Status

fixed
a
Cheyne-Stokes breathing refers to alternating hyperpnea and apnea.
b
Triple flexion reflex is flexion of the thigh, leg, and dorsiflexion of the foot upon noxious stimulation of the foot.
Modified from Plum F, Posner JB: The Diagnosis of Stupor and Coma III, Philadelphia, FA Davis, 1995, p 103.

41
42 Patient-Related Problems

TABLE 7.3 Modified Glasgow Coma Scale

Score Eyes Opening

>1 Year Old <1 Year Old
4 Spontaneously Spontaneously
3 To verbal command To shout
2 To pain To pain
1 No response No response
Best Motor Response
6 Obeys Spontaneous
5 Localizes pain Localizes pain
4 Flexion: withdrawal Flexion: withdrawal
3 Flexion: abnormal (decorticate rigidity) Flexion: abnormal
(decorticate
rigidity)
2 Extension (decerebrate rigidity) Extension
(decerebrate
rigidity)
1 No response No response
Best Verbal Response
>5 Years Old 2-5 Years Old 0-23 Months Old
5 Oriented Appropriate words Smiles, coos
and phrases appropriately
4 Disoriented Inappropriate Cries, consolable
words
3 Inappropriate Persistent cries Persistent,
words inappropriate cries
2 Incomprehensible Grunts Grunts, agitated, or
restless
1 No response No response No response

3. Osmotherapy. Increasing serum osmolarity to 300 to

320 mOsm/L may establish a gradient that allows brain water
to be drawn into the circulation. Mannitol 0.25 to 1.0 g/kg
can be given over 20 to 30 minutes; it can be given every 4 hours,
and serum osmolality should be monitored with each dose.
Typically a patient given mannitol should also have a urinary
catheter because it will cause significant diuresis. Three percent
saline, 5 mL/kg bolus, may also be given and may be repeated
hourly until serum Na reaches 160 mEq/L. After this threshold
is reached, there is typically minimal additional effect on ICP.
Mannitol is usually preferred over 3% saline because mannitol
lowers ICP more rapidly.
4. Hyperventilation. This method reduces blood flow to the brain
and is the quickest way to decrease ICP; however, it also may
reduce cerebral blood flow enough to cause cerebral ischemia
 Altered Mental Status 43

and therefore is reserved for acute situations. If the patient is

intubated, they may be ventilated with the goal of keeping
the arterial partial pressure of CO2 between 30 and 35 mm
Hg. One should ventilate only below 30 mm Hg if there are
signs of acute herniation.
After these steps have been performed, computed tomography
(CT) should be performed to help confirm the presence of a mass
lesion, hemorrhage, or cerebral edema as a potential cause of the
increased ICP. Neurosurgical consultation may be helpful because
surgery and/or an ICP monitor may be necessary to further man-
age the increased ICP.

Infection
If meningitis, abscess, or encephalitis is suspected, further evalua-
tion should be expedited and empiric treatment begun as quickly as
possible. Ideally, blood and CSF cultures should be obtained before
the administration of antibiotics. However, in a critically ill child,
there should be no more than a 15-minute delay in the administra-
tion of antibiotics for suspected meningitis. If suspicion of
increased ICP necessitates a CT scan before lumbar puncture,
blood cultures should be drawn, the antibiotics should be admin-
istered immediately after, and the lumbar puncture performed after
the CT scan. An opening pressure should be obtained during the
lumbar puncture because high pressure is suggestive of bacterial
meningitis. IV ceftriaxone (200 mg/kg/day, max 4 g/day divided
every 12 hours) and IV vancomycin (60 mg/kg/day divided every
6 hours) are the empiric treatments of choice for suspected bacte-
rial meningitis. The administration of dexamethasone (0.15 mg/
kg) should be individualized. Studies have not shown that dexa-
methasone improves mortality or survival; however, there has been
evidence that it decreases hearing loss in patients with Haemophi-
lus influenzae meningitis. In geographic areas that are endemic for
Rocky Mountain spotted fever, the addition of IV doxycycline may
be considered. The presence of focal abnormalities on physical
examination, hemorrhagic CSF, or associated focal seizures should
raise suspicion of herpes encephalitis, and empiric treatment with
acyclovir (30 to 45 mg/kg per day divided every 8 hours) is then
indicated.

Selective Physical Examination II

If the patient has no signs of significant cardiorespiratory compro-
mise, there is no evidence of increased ICP or CNS infection, and
the patient is not actively seizing, a more detailed physical exam-
ination can be performed before further evaluation and manage-
ment. The goal should be to identify findings that can help to
narrow the list of diagnostic possibilities.
44 Patient-Related Problems

HEENT* Intracranial bruits (arteriovenous malformations),

periorbital edema (anaphylaxis), or mastoid
edema and erythema (cellulitis); perioral cyanosis
(hypoxemia), fruity breath (diabetic ketoacidosis)
Neck Jugular venous pulsations (congestive heart failure),
goiter (thyroid disease)
Chest Rales, intercostal retractions, tachypnea (respiratory
failure, pneumonitis)
Cardiac Murmurs, friction rub, muffled heart sounds
(endocarditis, pericarditis, pericardial effusion)
Abdomen Hepatomegaly (liver disease, congestive heart
failure), splenomegaly (portal hypertension)
Extremities Paresis, paralysis, arthritis (systemic lupus
erythematosus, vasculitis), edema (congestive
heart failure)
Skin Palpable purpura (vasculitis), petechiae,
ecchymoses (hemolytic-uremic syndrome,
thrombotic thrombocytopenic purpura,
coagulopathy, abuse), jaundice (liver disease,
hemolytic diseases)
Neurologic Responsiveness (see Table 7.3, Modified Glasgow
Coma Scale); cranial nerves; doll’s eyes reflex;
muscle bulk, tone, and strength; reflexes (to
evaluate the extent of and potentially localize a
nervous system lesion); if possible in an
adolescent, assessment of mood, affect, and
thought processes (psychosis)
HEENT, Head, eyes, ears, nose, throat.
*

Selective History and Chart Review

After the airway and vital signs have been stabilized, treatment of
increased ICP or infection has been initiated if necessary, and the
patient has been examined, further information should be obtained
from the patient, family members, and the chart.
Is there a known history of trauma?
Keep in mind that a traumatic event may have occurred before
hospitalization. Symptoms due to a subdural hematoma may occur
well after the injury.
Are there any concerns for shaken baby syndrome?
Recall that infants may not have any external signs of injury.
Has the child been complaining of headaches? Nausea or vomit-
ing? Visual disturbances?
Symptoms of increased ICP that have been present for some
time suggest a mass lesion such as tumor or abscess.
Has the child had any recent infections?
 Altered Mental Status 45

A recent infection raises the suspicion of encephalitis, meningi-

tis, or abscess.
Does the child have any underlying illnesses that may affect the
CNS? Have there been any symptoms to suggest a systemic illness
that might be affecting the CNS?
Diabetes; chronic pulmonary, cardiac, renal, or liver disease; HIV
infection; and lupus are examples of underlying conditions that may
affect the CNS. Approximately 25% of diabetic children are diag-
nosed with diabetes upon presentation with ketoacidosis.
What medication is the child receiving? Do any of these med-
ications have CNS effects? What medications are other family
members taking (ask this to elicit what medications may be avail-
able to the child)? In an adolescent, is there a history of drug or
alcohol use?
Accidental or intentional overdosing of numerous medications
may alter mental status. In addition, secondary metabolic effects
from medications, such as hypoglycemia or hypokalemia, may
be contributing to the AMS.
Is there a family history of migraine or psychosis? Has the child
been acting depressed or abnormal lately?
Acute confusional migraine or psychosis may begin suddenly
but should be considered after the more life-threatening possibil-
ities have been ruled out. In addition, keep in mind that a psychotic
or depressed child is at increased risk for toxic ingestion.
If known, what was happening at the time of and prior to the
event? Did the child feel anxious, flushed, nervous, or sweaty? Is there
a family history of heart disease or premature death? Did the child
feel palpitations?
Such symptoms suggest vasovagal syncope. A family history
of early death raises the suspicion of long QT syndrome or
cardiomyopathy. Palpitations also increase suspicion for a
dysrhythmia.

Management II
Nearly all children and adolescents who have had an alteration in
mental status require additional laboratory or radiographic evalu-
ation. The exceptions to this generalization include young children
who are irritable but consolable, have normal neurologic examina-
tion findings, and have been hospitalized for an illness that might
be expected to cause some irritability. Such children may be
observed as their illness is being treated, with the expectation that
the irritability will resolve as they are feeling better. However, if
there is suspicion of CNS pathology, additional evaluation is
warranted.
46 Patient-Related Problems

The following tests should be performed in patients in whom

syncope has been excluded and the cause of the AMS remains
unclear:
1. CBC and differential (infection, anemia, thrombocytopenia)
2. Electrolytes, glucose, calcium, ammonia
3. Blood and urine toxicology screen
4. Head CT or magnetic resonance imaging (mass lesion). (CT is
preferred in the acute phase.)
5. Lumbar puncture with an opening pressure. (Unless there are
signs of increased ICP, focal neurologic findings, risk of cardio-
respiratory compromise, or a skin/soft tissue infection overly-
ing the site of the lumbar puncture.)
Additional blood and CSF should be collected and saved
because further studies (e.g., autoantibodies) may be necessary if
the diagnosis remains unclear.
The following tests may be helpful if some evidence suggests a
specific illness:
1. Hepatic transaminases, blood urea nitrogen, creatinine, urinal-
ysis (liver or renal disease)
2. Arterial blood gas (hypoxemia, hypercapnia)
3. Chest radiographs (cardiac or pulmonary failure)
4. Peripheral blood smear, prothrombin time, partial thrombo-
plastin time (microangiopathy, coagulopathy)
If the alteration in mental status is thought to be consistent with
syncope or if cardiac failure with decreased cerebral perfusion is
suspected, an ECG should be obtained. In addition, a patient
should be placed on telemetry monitoring because an ECG may
not capture the inciting rhythm.
Additional diagnostic evaluation such as thyroid studies, cere-
bral angiograms, electroencephalograms, echocardiograms, anti-
nuclear antibody studies, and other tests aimed at excluding
specific diseases should be considered for individual patients. Sim-
ilarly, further evaluation of a patient with syncope, such as electro-
encephalographic studies or tilt table testing, may need to be
scheduled the next day. In most cases the cause of the AMS is
apparent after the history, physical examination, and laboratory
evaluation, as outlined earlier. At a minimum, the major threats
to life are considered, and if necessary, the child is receiving treat-
ment aimed at reducing increased ICP or resolving a possible CNS
infection. Definitive treatment depends on the cause of the AMS.
Infection
As outlined previously, suspected bacterial meningitis is treated
with antibiotics. IV ceftriaxone and vancomycin are a good choice
of initial empiric therapy beyond the neonatal period because it is
effective against the most common pathogens: Streptococcus
 Altered Mental Status 47

pneumoniae (including penicillin- and cephalosporin-resistant

strains) and Neisseria meningitidis. Brain abscess may be caused
by a large group of aerobic and anaerobic bacteria, and therefore
empiric treatment with appropriate broad-spectrum IV antibiotics
is necessary. In addition, neurosurgical consultation is helpful
because most children require excision or drainage of the abscess.
Encephalitis should be treated empirically with acyclovir if herpes
infection is suspected.

Mass Lesion
Intracerebral hemorrhage or tumor requires surgical evacuation or
resection, and a neurosurgeon should be consulted promptly.
Any alteration in mental status associated with trauma is also an
indication for neurosurgical consultation.
Metabolic Disturbance
Diabetic ketoacidosis should be managed with insulin, hydration,
and correction of associated electrolyte abnormalities. Likewise,
other electrolyte disturbances should be corrected. Inborn errors
of metabolism (particularly partial/incomplete forms) can occur
at any age and require prompt evaluation and possible consultation
with a geneticist to ascertain the cause and begin emergency ther-
apy (i.e., reducing the serum ammonia level in patients with urea
cycle defects).
Drugs and Toxins
If overdose is suspected, treatment should be directed at decontam-
ination and supportive therapy. Treatment of ingestions includes
activated charcoal, diuresis, and specific antidotes, depending on
the substance ingested. Dialysis or exchange transfusion is reserved
for the most severe cases.
Psychosis
A psychotic child should be managed in consultation with psychi-
atrists. Chlorpromazine, haloperidol, or diazepam may be consid-
ered in an extremely agitated or violent child.
Systemic illnesses such as liver or renal disease, thyroid disease,
lupus, or vasculitis should be managed appropriately while keeping
in mind that associated increased ICP may also need to be treated.
 CHAPTER

8
Analgesics and
Antipyretics
Danielle DuMez, MD

Analgesics and antipyretics are two classes of medications that are

among the most commonly prescribed in hospitalized pediatric
patients. Although writing orders for these medications can
become very routine, it is important to remember that like all med-
ications, these drugs are to be prescribed with careful thought and
attention to detail. When asked to write an order for analgesics
and/or antipyretics, it is important that you (1) determine the need
for the medication, (2) understand why the child needs these med-
ications, and (3) realize the potential consequences of using these
medications in the individual patient, including adverse effects, as
well as the effect that limiting pain and/or fever will have on your
ability to accurately evaluate a patient’s status.

Analgesics

PHONE CALL
Questions
1. Why is an analgesic being requested?
2. Where is the pain?
3. How severe is the pain?
4. Is this pain new?
5. What interventions have been tried thus far?
6. What is the child’s admitting diagnosis?
7. How old is the child?
8. How much does the child weigh?
9. Does the child have any allergies?
10. Are there other symptoms in addition to the pain?
The answers to these questions should allow you to consider
potential causes of the pain and understand the child’s need for
an analgesic, as well as the urgency of the situation.

48
 Analgesics and Antipyretics 49

In children, it is sometimes difficult to know whether the prob-

lem is truly one of pain. Parents, nurses, and physicians may
assume that pain is present because a young child is crying or
screaming. Temper tantrums, night terrors, nightmares, and/or
fear may be the problem rather than pain. Similarly, pain may
be the result of behavioral issues, depression, anxiety, or other psy-
chogenic and psychosocial factors, in which case analgesics may be
ineffective. It is important to consider these possibilities before
immediately ordering medication.
If the child’s diagnosis is unclear or if masking the pain will
make it difficult to evaluate the child’s status or make an accurate
diagnosis (e.g., abdominal pain that may require surgery or joint
pain that may be associated with rheumatic fever), you must care-
fully consider these factors before immediately prescribing an
analgesic.

Orders
Table 8.1 lists selected analgesics and antipyretics. In most situa-
tions, acetaminophen is the best initial choice because it is well tol-
erated, it does not usually mask significant pain, and there are no
concerns regarding dependency. Ibuprofen or another nonsteroi-
dal antiinflammatory drug (NSAID) is generally the next best
choice if acetaminophen is insufficient. However, in children with
abdominal pain, liver disease, or renal disease, one needs to be
aware of the potential for gastritis or, less commonly, hepatotoxic-
ity or nephrotoxicity with these drugs. Because of their antiinflam-
matory effect, NSAIDs also have greater potential to mask
symptoms and signs associated with disease.
Narcotics should not be ordered unless the cause of the pain is
clearly understood and not until either (1) the child has already
failed a trial of acetaminophen and NSAIDs or (2) acetaminophen
and NSAIDs are contraindicated or felt to place the child at signif-
icant risk. Narcotics have greater potential for serious adverse events
such as respiratory depression. They may alter mental status;
their potency results in significant masking of signs and symptoms;
and they may result in dependency.
Inform RN
“Will arrive at the bedside in …minutes to assess the patient.”
Any child with a new need for analgesics should be evaluated.
The urgency of this evaluation will depend on the severity of the
pain and the potential for life-threatening causes of pain.

ELEVATOR THOUGHTS
If there is any doubt regarding the origin or cause of the pain, if the
pain is a new complaint, if the character of the pain has changed, or
 TABLE 8.1 Commonly Used Analgesics and Antipyretics

Generic Name Brand Name Dose Max Dosing Use of Drug

Acetaminophen Tylenol 10-15 mg/kg PO q4-6 h Max daily dose: 75 mg/kg/day or 400?' *- Analgesic and antipyretic
Ibuprofen—Avoid Motrin or 5-10 mg/kg PO q6-8 h Max single dose: 400 mg Max daily (119’ Analgesic and antipyretic
in children less Advil kg/day up to 1200 mg
than 6 months old
Naproxen Aleve Analgesia: 5-7 mg/kg q8—12 h Antipyretic: Max daily dose: 1 my® Mostly used for analgesia and antiinflam-
10-15 mg/kg/day P0 divided twice a day matory but also has antipyretic effects
Codeinea 0.5-1 mg/kg PO q4-6 h; Requires dose Max single dose: Analgesic can be used in combination with
adjustment in renal impairment acetaminophen: Tylenol No. 2: 15 mg
codeine+300 mg acetaminophen;
Tylenol No. 3230 mg codeine+300 mg
acetaminophen; Tylenol No. 4260 mg
codeine+300 mg acetaminophen

@‘s
Oxycodonea Oxycontin a Less than 6 months old: 0.025-0.05 mg/kg P0 q4- se: 10 mg if <50 kg & 20 mg if Analgesic can be used in combination with
6 h Greater than 6 months old: 0.1—0.2 m . acetaminophen (Brand: Percocet)
PO q4—-6 h
Morphinea Less than 6 months old. 0.08-0. 1 mg/kg/d ; ax single P0 dose: 15-20 mg; Max single IV/lM/ Analgesic
q3-4 h or (1025-003 mg/k SO dose for infants greater than 6 months old: 10 mg of morphine=100 mg meperidine
2 mg dose; lV/lM/SO dose for infants greater Antidote = naloxone
than 1 year old: 5-10 mg
Meperidinea—Use Demerola Infants less than 6 month Max single PO dose for infants less than 6 months Analgesic
with caution in P0 (13-4 h and 0. - ~ ‘ old: refer to dose section; max single dose for Antidote = naloxone
renal and liver q2-3 h; infants infants greater than 6 months: 150 mg P0 and
failure patients 75 mg IM/IV/SO

aNarcotics are addictive and may cause respiratory distress; th refore, they should be used only for severe pain and when the cause of pain is understood.
 Analgesics and Antipyretics 51

if the pain is severe, you must evaluate the child at the bedside
before ordering an analgesic.
Remember
1. It is easy to both overestimate and underestimate the severity of
pain in pediatric patients. You need to make sure that you
understand the cause of the pain as clearly as possible while
making the patient as comfortable as possible. Overprescribing
narcotics and other analgesics and withholding medication
from a child in pain should both be avoided. The severity of
pain can be assessed and monitored over time with a simple
0 to 10 scale for older children or with the Bieri faces scale
(see Fig. 21.1) for younger children.
2. Escalating the potency of the analgesic you order without ree-
valuating the patient should also be avoided. After ordering
acetaminophen or an NSAID for a patient, if the nurse calls
you several hours later to tell you the child is still in pain,
you must see that patient before simply ordering a more potent
analgesic, such as a narcotic.
3. When the cause of pain is poorly understood, you should not
order analgesics as “PRN pain.” You must instead be notified
if the patient remains in pain and how severe it is. In this situ-
ation, ask the nurse to call you back in several hours if the child
remains in pain.
4. When the cause of pain is clearly understood and there is an
anticipated need for multiple doses of intravenous (IV) narcotic
medication, patient-controlled analgesia (PCA) should be con-
sidered. PCA should be ordered after consultation with an anes-
thesiologist or other pain specialist.
5. Narcotic overdosage can be reversed with naloxone, 0.01 to
0.1 mg/kg per dose intramuscularly (IM), IV, or subcutaneously
(SQ; maximum, 2 mg per dose). This dose can be repeated every
2 to 3 minutes as needed. Reversal may induce nausea and
vomiting. Therefore, be prepared to protect the airway because
the child is at risk for aspiration.

Antipyretics

PHONE CALL
Questions
1. How old is the child?
2. How high is the fever?
3. What is the child’s admitting diagnosis?
4. How much does the child weigh?
52 Patient-Related Problems

5. Is the fever a new finding?

6. Any other associated symptoms?
7. When did the patient receive the last dose of an antipyretic?
Orders
See Table 8.1 for dosing of antipyretics.
Inform RN
“Will arrive at the bedside in … minutes to assess the patient.”
Any child with a new fever must be evaluated (see Chapter 18)
before ordering antipyretics.

Remember
1. Although you are ordering these medications for fever, they also
have an analgesic effect, and therefore you need to consider this
potential effect on your ability to evaluate the patient as
described earlier under “Analgesics.”
2. There is no longer any need to use aspirin as an antipyretic.
Aspirin has been associated with Reye syndrome and therefore
should be avoided. Acetaminophen and ibuprofen are alterna-
tives that in general are better tolerated and entail less risk.
3. There are fewer concerns with the use of antipyretics in a
patient in whom the diagnosis remains unclear. Decreasing
the fever rarely results in difficulty arriving at a diagnosis,
and it allows the child to be much more comfortable.
4. When fever is persistently high and unresponsive to acetamin-
ophen alone, acetaminophen and ibuprofen may be ordered
together (acetaminophen every 4 to 6 hours and ibuprofen
every 6 hours). You may alternate acetaminophen and ibupro-
fen every 3 hours (therefore each is administered every 6 hours)
to maximize benefit if needed.
 C HA P TE R

9
Bleeding
Laura Adams, MD

Bleeding may potentially occur at any anatomic location in a hos-

pitalized child. When evaluating a patient who has bled or is bleed-
ing, the goals are simple: (1) stop the active bleeding, and (2)
prevent further bleeding. How one achieves these goals depends
on the answer to a basic question: “Is the bleeding secondary to
an anatomically localized problem (e.g., trauma), or is it secondary
to a generalized bleeding disorder (coagulopathy)?” Coagulopa-
thies are further discussed in detail in Chapter 33. Epistaxis,
hemoptysis, hematemesis, melena, hematochezia, rectal bleeding,
hemarthrosis, and hematuria are terms used to describe bleeding
from various locations. In addition, bleeding into the skin and soft
tissues may produce ecchymoses, petechiae, or purpura. This chap-
ter discusses bleeding that does not involve the gastrointestinal or
urinary tract. These subjects are addressed in detail in Chapter 19
and Chapter 23.

PHONE CALL
Questions
1. What are the child’s vital signs?
2. Where is the bleeding?
3. What is the child’s underlying illness and reason for
hospitalization?
4. What medications is the child receiving?
The vital signs allow you to quickly determine whether the
blood loss has been severe enough to result in depletion of intra-
vascular volume. Tachycardia and/or hypotension might indicate
such a state. If the child is febrile and has petechiae or purpura,
one must immediately consider the possibility of sepsis with asso-
ciated coagulopathy. The location of the bleeding allows you to
develop a more specific differential diagnosis. In addition, the
patient’s underlying illness may give you clues regarding potential

53
54 Patient-Related Problems

causes of the bleeding. Discovering that the patient is receiving a

medication that might affect platelets or clotting factors is obvi-
ously of significance.
Orders
If the vital signs suggest shock, an intravenous (IV) bolus of either
normal saline or lactated Ringer’s solution should be given (10 to
20 mL/kg), and blood should be sent to the blood bank for typing
and cross matching. If bleeding is massive, type O-negative whole
blood (as well as plasma and platelets if a coagulopathy is suspected)
should be ordered immediately. A complete blood count, prothrom-
bin time (PT), and partial thromboplastin time (PTT) should also be
ordered to determine the degree of anemia and the potential need to
correct deficiencies in platelets or clotting factors.
If active bleeding is apparent from an accessible site (e.g., skin,
external nares, or oral cavity), firm pressure should be applied
and held.
If the child is receiving anticoagulating medications, his or her
use should be suspended.

Inform RN
Active bleeding, abnormal vital signs, and fever with purpura and
petechiae all require immediate attention. In the absence of these
concerns, you should inform the RN when you will be at the bedside.

ELEVATOR THOUGHTS
Potential causes of bleeding depend on the site of the blood loss. The
basic question of whether the bleeding reflects a localized problem or
a systemic one should be kept in mind. Local trauma to a blood ves-
sel or vessels is in general far more common than a coagulopathy,
particularly with epistaxis and hemoptysis. Generalized petechiae
or ecchymoses should make you consider a systemic coagulopathy
more carefully. Potential causes are listed by anatomic site:
Epistaxis Blunt trauma
Self-inflicted trauma (picking)
Nasal congestion, crusting
Excessive sneezing
Foreign body
Hypertension
Polyp
Tumor (e.g., angiofibroma)
Nasal hemangioma
Congenital syphilis (“snuffles”)
Granulomatosis with polyangiitis
 Bleeding 55

Hemoptysis Aspiration of blood from the mouth and

upper airway
Foreign body
Infection
Tuberculosis
Pneumonia
Pneumonitis
Lung abscess
Bacterial tracheitis
Cystic fibrosis
Chest trauma
Pulmonary vasculitis (systemic lupus
erythematosis, granulomatosis with
polyangiitis, eosinophilic
granulomatosis with polyangiitis,
Goodpasture syndrome)
Pulmonary embolus
Pulmonary hypertension
Severe congestive heart failure
Idiopathic pulmonary hemosiderosis
Heiner syndrome
Arteriovenous malformations
Petechiae, purpura, Trauma (e.g., blood pressure cuff)
ecchymoses Sepsis
Septic emboli (endocarditis)
Vasculitis (e.g., Henoch-Sch€onlein
purpura)
Viral infections
Drugs (salicylates, steroids,
nonsteroidal agents)
Scurvy
Any site Thrombocytopenia
Decreased platelet production
(e.g., marrow failure, leukemia)
Increased platelet destruction
(disseminated intravascular
coagulopathy [DIC], idiopathic
thrombocytopenic purpura [ITP],
hemolytic-uremic syndrome [HUS],
thrombotic thrombocytopenic purpura
[TTP], splenic trapping)
Abnormal platelet function (inherited
defect, drugs, uremia)
Clotting factor abnormality
Hemophilia (factor VIII or IX deficiency)
56 Patient-Related Problems

Other factor deficiency (inherited or from

liver disease)
Consumptive coagulopathy (DIC,
cavernous hemangioma)
Von Willebrand disease
Vitamin K deficiency

MAJOR THREAT TO LIFE

• Exsanguination
• Hypoxia from pulmonary hemorrhage or embolism
• Sepsis
• Intracranial hemorrhage secondary to a clotting abnormality
• Severe systemic necrotizing vasculitis
Exsanguination is possible as long as bleeding remains active.
Similarly, persistent or recurrent hemoptysis indicates active pul-
monary bleeding and is dangerous. A febrile child with petechiae
and/or purpura needs immediate evaluation and empiric treatment
of presumed sepsis. Severe clotting defects should be corrected as
quickly as possible because they may lead to secondary bleeding in
other locations, including intracranially. Vasculitides are rare but
must be considered and treated promptly because they may quickly
lead to multiorgan failure.

BEDSIDE
Quick-Look Test
Is the patient comfortable, alert, and in no distress, without any
evidence of active bleeding?
If so, your evaluation may proceed in a more relaxed manner
without the need for immediate intervention. However, if the child
is actively bleeding, has persistent hemoptysis (particularly if the
vital signs are abnormal), or is febrile with petechiae or purpura,
immediate intervention is necessary.
Airway and Vital Signs
A child with epistaxis or hemoptysis may rarely have such excessive
bleeding that the airway becomes compromised. Labored respira-
tions, cyanosis, tachypnea, grunting, and retractions of accessory
muscles may all be signs that the airway is compromised. In many
instances, simple suctioning and turning the child to the side to
clear the airway of blood alleviate the problem. However, if the air-
way cannot be maintained in this manner, the child needs to be
intubated. Tachycardia and/or hypotension is indicative of shock
secondary to either hypovolemia or sepsis and requires immediate
 Bleeding 57

management (see Chapter 25). Hypertension may result in rupture

of small intranasal blood vessels and can thus lead to epistaxis.
Fever may be associated with infection or vasculitis. Fever with
petechiae or purpura should be treated as sepsis (e.g., meningococ-
cemia) until proven otherwise.
Selective Physical Examination I
After a quick look and check of the airway and vital signs, the child
should be examined briefly to determine the likelihood of a major
threat to life, in which case immediate intervention is necessary
before obtaining more historical information and reviewing the
chart. The goal of this initial selective examination is to search
for signs that (1) bleeding remains active (usually obvious), (2) pul-
monary bleeding or emboli are causing significant hypoxia and/or
respiratory distress, (3) sepsis is a possibility, or (4) the child is also
bleeding intra-abdominally or intracranially.
HEENT Pupil size and reactivity, papilledema (intracranial
bleeding with increased intracranial pressure
[ICP]), persistent epistaxis, meningeal signs
(sepsis with meningitis)
Chest Grunting, retracting, breath sounds (respiratory
distress); chest wall trauma
Abdomen Distention, tenderness, bruising, rebound
tenderness, rigidity
Skin Petechiae, purpura (meningococcemia, vasculitis)
Neurologic Alterations in consciousness, focality (intracranial
bleeding)
HEENT, Head, Eyes, Ears, Nose, Throat.

Management I
After the quick look, check of the airway and vital signs, and brief
examination, the next step is intervention. You may need to inter-
vene quickly if the patient is actively bleeding or if you suspect pul-
monary embolism, pulmonary hemorrhage, sepsis, or intracranial
bleeding.
Active Bleeding
Bleeding from the nose, oral cavity, or mucocutaneous sites usually
abates with the application of pressure. Pressure should be applied
firmly for 5 minutes or longer while trying to avoid the temptation
to frequently visualize the site of bleeding. With epistaxis, the child
should be positioned on the side, gauze should be placed or packed
within the nares, and pressure should be held over the bridge of the
nose. Epistaxis most often occurs as a result of ruptured vessels in
Kiesselbach plexus, an area of anastomosed arterioles just within
the nares on the septum. If the bleeding remains active or is
58 Patient-Related Problems

profuse, blood should be sent for a stat complete blood count,

platelet count, prothrombin time (PT), partial prothrombin time
(PTT), and typing and cross matching. While these tests are per-
formed, pressure should continue to be applied.
If bleeding is massive and/or the child is in shock, type
O-negative blood should be requested from the blood bank and
transfused as soon as possible. A consultation to general surgery
or otolaryngology (depending on the site of bleeding) should
be made immediately to assess for the need for operative
management.
Coagulopathies detected by laboratory testing should be cor-
rected with appropriate blood products (platelets, fresh frozen
plasma, or cryoprecipitate; see Chapter 33). If bleeding persists
after correction of clotting abnormalities, surgical consultation
should be considered.

Pulmonary Embolus
Pulmonary embolism is a rare childhood event that should be sus-
pected in a child with hemoptysis who is tachypneic and hypoxic,
although these signs are not invariably present. Pulse oximetry
analysis or arterial blood gas determinations help to establish the
degree of hypoxia. If the child has a known deep venous thrombosis
or hypercoagulable state, suspicion should be high and anticoagu-
lation with heparin considered while further evaluation is proceed-
ing. Once the child’s airway and vital signs are stabilized, a helical
computed tomographic (CT) scan of the chest should be per-
formed as soon as possible to determine the probability of an
embolus. If the diagnosis remains uncertain, pulmonary angiogra-
phy should be considered to definitively diagnose or exclude an
embolus. After pulmonary embolism is diagnosed, anticoagulation
is the treatment of choice. Large emboli may require surgical
intervention.
Pulmonary Hemorrhage
Cystic fibrosis and vasculitides are conditions that may lead to
acute pulmonary hemorrhage. With cystic fibrosis, persistent
inflammation of the lung parenchyma may eventually lead to ero-
sion into a major vessel and the sudden onset of massive pulmo-
nary hemorrhage and hemoptysis. Emergency bronchoscopy,
pulmonary angiography, and percutaneous catheter embolization
of bronchial arteries may be necessary to localize and treat the
involved vessels. If the patient has a history of systemic vasculitis
or systemic lupus erythematosus or if such a diagnosis is being con-
sidered because of associated symptoms and physical findings,
aggressive treatment of the underlying illness with corticosteroids
and cytotoxic agents may be effective.
 Bleeding 59

Sepsis
A child with fever and petechiae or purpura should be considered to
have sepsis, and further evaluation and empiric treatment of sepsis
should begin immediately. Blood, urine, and cerebrospinal fluid (if
signs suggestive of meningitis are present) should be obtained and
antibiotics administered as quickly as possible. If the child is critically
ill, antibiotics should not be delayed while awaiting the performance
of a lumbar puncture. Ceftriaxone, 50 mg/kg per dose every 12 hours
empirically, covers pneumococcus, meningococcus, and Haemophi-
lus influenzae. Vancomycin should be added if there are concerns
regarding resistant organisms. In a child younger than 2 months,
the combination of ampicillin and cefotaxime should be used to pro-
vide additional coverage of the potential neonatal pathogens Listeria
monocytogenes, group B streptococcus, and Escherichia coli.

Intracranial Bleeding
If intracranial bleeding is suspected because of focal neurologic
findings or altered mental status, immediate CT scanning and neu-
rosurgical consultation are required. If signs of increased ICP are
present, management should proceed as outlined in Chapter 7.
Immediate measurement of the platelet count, PT, and PTT is nec-
essary with appropriate correction of deficiencies (see Chapter 33,
for guidelines regarding the administration of blood products).

Selective Physical Examination II

If the child is not actively bleeding and your suspicion of pulmo-
nary hemorrhage, pulmonary embolism, sepsis, or intracranial
bleeding is not high, you should proceed with a more detailed phys-
ical examination:
HEENT Fundi (retinal hemorrhage), hemotympanum,
nasal deformity (“saddle nose” of
granulomatosis with polyangiitis),
palatal petechiae, dental trauma
Neck Adenopathy (infection, malignancy), Kernig
and Brudzinski signs
Chest Breath sounds, rales, external trauma,
friction rub
Heart Murmurs (endocarditis), friction rub
Abdomen Bowel sounds, tenderness (vasculitis),
hepatosplenomegaly (malignancy, DIC, ITP,
liver disease, secondary coagulopathy), rectal
examination for occult blood
Musculoskeletal Swollen joints (hemarthrosis, vasculitis), bone
pain (malignancy), absent radii
(thrombocytopenia–absent radii syndrome)
60 Patient-Related Problems

Genitourinary Testicular swelling, tenderness (vasculitis)

Skin Rashes, splinter hemorrhages (vasculitis,
infectious), hemangiomas (Kasabach-Merritt
syndrome), Grey Turner sign (pancreatic
hemorrhage or retroperitoneal hemorrhage)
Neurologic Abnormal sensation, motor deficits
(peripheral neuropathy or weakness with
vasculitis)
DIC, Disseminated intravascular coagulopathy; ITP, idiopathic thrombocytopenic purpura.

Selective History and Chart Review

Has the child bled excessively before?
A history of previous episodes should raise suspicion of an
inherited coagulopathy or chronic thrombocytopenia. Recurrent
epistaxis may be self-inflicted and is also suggestive of a potential
anatomic lesion (e.g., polyp or hemangioma).
Has the patient had a recent infection?
If the patient has been hospitalized with a bacterial infection,
sepsis with associated disseminated intravascular coagulopathy
(DIC) is a possibility. A recent viral infection might suggest idio-
pathic thrombocytopenic purpura or Henoch-Sch€onlein purpura.
Recent diarrhea, particularly if bloody, might indicate hemolytic-
uremic syndrome (HUS).
What drugs has the patient received?
Multiple drugs may be associated with thrombocytopenia or
decreased platelet function, including salicylates, nonsteroidal anti-
inflammatory drugs, and antibiotics. Oral contraceptives may
increase the risk for pulmonary embolism.
Is there a family history of coagulopathies? Has there been bleed-
ing after childbirth or circumcision, menorrhagia, or epistaxis in
family members?
If yes, this is a clue to a potential inherited coagulopathy.
Is there any reason to suspect liver or renal failure, either sec-
ondary to the child’s underlying illness or as a consequence of
treatment?
The answers to these questions may be diagnostically helpful.
If hemoptysis is present, has the child been exposed to tuberculo-
sis? Have there been numerous upper and/or lower airway infec-
tions? Has the child undergone recent dental treatment? Is there
any history of heart disease?
Numerous infections should raise suspicion of cystic fibrosis.
Recent dental procedures might suggest lung abscess. A history
of heart disease might increase the risk for endocarditis.
Has the child been hospitalized with severe traumatic injuries?
Chest trauma may result in hemoptysis. Crush injuries, burns,
or severe head trauma may lead to DIC.
 Bleeding 61

If the child is a newborn, is there a history of maternal thrombo-

cytopenia or lupus? Has the mother been treated with any drugs that
may cause vitamin K deficiency (e.g., anticonvulsants) or suppress
platelet production? Has the neonate received vitamin K?
Neonatal immune thrombocytopenia may be the result of
transplacental passage of maternal immunoglobulin G (IgG) anti-
platelet antibodies, such as those seen in lupus or ITP. Vitamin K
deficiency typically results in bleeding on the second or third day
of life.
Management II
After the selective history and physical examination, further labo-
ratory testing may be necessary to determine the cause of the bleed-
ing or to exclude possible diagnoses. In many instances the
bleeding is minimal, self-limited, and not life threatening. If epi-
staxis has occurred and resolved, additional testing may be unnec-
essary. It may be presumed that the bleeding was secondary to local
trauma, and a simple “wait and see” approach may suffice. Simi-
larly, self-limited bleeding localized to one area of the skin may
often be presumed to be secondary to trauma and frequently does
not require further evaluation. Excessive bleeding, hemoptysis,
hemarthrosis, and generalized petechiae and purpura should be
further evaluated with laboratory testing. Likewise, recurrent epi-
sodes of bleeding should be additionally evaluated. The following
tests should be performed to assess the degree of anemia, possible
thrombocytopenia, possible microangiopathy, or possible clotting
factor abnormality:
1. Complete blood count
2. Platelet count
3. Peripheral blood smear
4. PT
5. PTT
Although not usually necessary immediately, a bleeding time
may also be useful to determine whether abnormalities in platelet
function are present.
For those with hemoptysis, a chest radiograph should be per-
formed. Additional testing may be done selectively, depending
on the clinical situation. If DIC is suspected, fibrinogen and D-
dimer determination would be helpful. Hepatic enzymes, blood
urea nitrogen, creatinine, and urinalysis are indicated if liver or
renal disease is a consideration. Helical CT scanning should be per-
formed if pulmonary embolism is suspected. Specific clotting factor
assays should be performed if unexplained PT and/or PTT abnor-
malities are present.
Definitive management of bleeding depends on the cause. Man-
agement of life-threatening conditions has been outlined earlier.
Less acute disorders should be managed as follows.
62 Patient-Related Problems

Infections
Chest radiographic studies should be performed in all patients with
suspected lung infection. If possible, sputum should be sent to the
laboratory for culture (including mycobacterial), Gram staining,
and acid-fast staining. A purified protein derivative skin test should
be performed if tuberculosis is suspected. Empiric treatment of sus-
pected pneumonia can be initiated with ceftriaxone. The decision
to empirically treat for Mycoplasma pneumoniae with erythromy-
cin or azithromycin should be individualized. Cold agglutinins,
often present in patients with M. pneumoniae infection, can be
detected at the bedside by placing a small amount (2 to 3 mL) of
blood in a purple-top tube and placing it on ice for several minutes.
In the presence of cold agglutinins, clumping of cells can be seen
along the glass walls of the tube as it is rolled in the hand. Lung
abscesses should be treated with antibiotics effective against Staph-
ylococcus aureus and anaerobes, such as clindamycin.

Vasculitis
Granulomatosis with polyangiitis, eosinophilic granulomatosis
with polyangiitis, lupus, Goodpasture syndrome, and less com-
monly, polyarteritis nodosa and Henoch-Sch€onlein purpura may
result in pulmonary hemorrhage. Prompt treatment with cortico-
steroids may be life saving. Consideration should also be given to
the use of cytotoxic therapy. Consultation with a rheumatologist is
recommended.
Coagulopathies
Disorders of coagulation may be divided into thrombocytopenias,
abnormalities in platelet function, and clotting factor deficiencies.
Depending on the cause (or presumptive cause), the treatment of
these conditions varies (see Chapter 33).
 C HA P TE R

10
Chest Pain
Stephen E. Wilkinson, MD

Acute chest pain is a nonspecific complaint with etiologies stem-

ming from many organ systems. Although children generally have
noncardiac causes of chest pain, each case must be evaluated for
possible life-threatening pathology (see later). As with other types
of pain described by children, the imprecision of the term may
include any abnormal sensation, such as palpitations or dysphagia.
A systematic approach to determining the etiology of the pain is
generally done either by mentally reviewing all thoracic organ sys-
tems or by “thinking through the chest” in an anteroposterior man-
ner. When evaluating patients for chest pain, consider if transfer to
a higher level of care is needed. If more resources are needed during
bedside evaluation and treatment, consider calling a rapid response
team (RRT) or code team to assist.

PHONE CALL
Questions
1. What are the vital signs, including temperature?
2. How severe is the pain? How uncomfortable is the child? When
did the pain start?
3. What is the child’s underlying illness and reason for
hospitalization?
4. Has the child complained of chest pain before?
The vital signs and degree of pain severity help to triage the
urgency of the complaint. If vitals are severely abnormal, immedi-
ately calling for an RRT or code team to assist you is likely war-
ranted; having such support often expedites medication
availability, lab draws, imaging, electrocardiographic (ECG) stud-
ies, and, if needed, transfer to a critical care unit. Tachycardia may
be present regardless of the cause of the pain. Extreme tachycardia
(>200 beats per minute) suggests a tachyarrhythmia, often a sup-
raventricular tachycardia (SVT). Bradycardia in a patient with

63
64 Patient-Related Problems

chest pain is an ominous sign that often denotes impending cardiac

arrest. This is symptomatic bradycardia, and Pediatric Advanced
Life Support protocols should be initiated immediately. Tachypnea
may likewise be secondary to the pain or to associated anxiety
about the pain. However, organic causes of tachypnea are often
concurrent and should be suspect, particularly cardiopulmonary
pathology. Respiratory rate trends may help you to determine
whether a pulmonary process was “brewing” before the chest pain
became apparent. Bradypnea may connote impending respiratory
failure. Hypertension may reflect anxiety, although organic, partic-
ularly cardiovascular and systemic causes should be ruled out. As
with bradycardia, hypotension is an ominous sign and should be
treated as an indicator of shock until this is ruled out. A fever should
raise suspicion of an infectious cause for the chest pain, such as
pneumonia, pleuritis, myocarditis, pericarditis, or osteomyelitis.
The child’s underlying illness and reason for hospitalization may
offer clues to the cause of the pain. Sickle cell disease with acute
chest syndrome (ACS), systemic juvenile rheumatoid arthritis
(JRA) with pericarditis, and cystic fibrosis with pneumothorax are
examples of illnesses associated with specific causes of chest pain.
Orders
If the vital signs reveal bradycardia or hypotension, have the RN
call for an RRT or code team to assist. The patient should be placed
on a cardiopulmonary monitor. An intravenous (IV) line should be
placed, if not yet present, and a 10 mL/kg bolus of normal saline or
lactated Ringer’s solution should be prepared. You should evaluate
the patient for congestive heart failure before administering fluids,
given of the risk of exacerbating this condition if it is present. Signs
to look for include pulmonary crackles, jugular venous distension,
and hepatomegaly. Further fluid boluses (normally dosed at
20 mL/kg each, if no congestive heart failure or other fluid overload
syndrome is present) may be needed to maintain blood pressure if
the patient is septic or in noncardiogenic shock. When the IV line is
placed, labs should be drawn, including a stat complete blood count
(CBC), basic metabolic panel (BMP), troponin, and fractionated
creatine phosphokinase (CK or CPK along with CK-MB); some
labs run the troponin and the fractionated CK/CK-MB together
as a “cardiac panel.” If a hematocrit can be determined on the ward,
this should be done because a decreasing hematocrit raises suspi-
cion of hemorrhage, such as from a ruptured vascular aneurysm or
a perforated gastrointestinal (GI) viscus. Keep in mind that slow
bleeds may have compensated, and that normal hematocrits and
such pathology should not be ruled out on the basis of this lab
alone. Continuous pulse oximetry should be going with the cardio-
pulmonary monitoring; if the patient is in extremis, an arterial
 Chest Pain 65

blood gas analysis should be obtained and oxygen administered,

initially 100% by mask. Often, bedside ECG and chest radiographic
studies may need to be performed soon after your evaluation. A
portable supine anteroposterior chest x-ray should be obtained if
the child is hemodynamically or otherwise clinically unstable. If
the child is hemodynamically stable but tachypneic without a sus-
picion of impending respiratory failure, consider ordering poster-
oanterior and lateral chest views. These films may reveal
pulmonary infiltrates and consolidations (including findings sug-
gestive of pneumonia, ACS, and acute respiratory distress syn-
drome), pleural effusions, an enlarged cardiac silhouette,
pneumothorax, pneumomediastinum, or rib fractures (additional
views may be needed for rib evaluation).
Inform RN
A patient with abnormal vital signs or severe discomfort requires
immediate evaluation. Otherwise, let the nurse know when you
plan to arrive. If the patient’s condition is severe, nursing staff
should stay with the patient until you have arrived.

ELEVATOR THOUGHTS
Potential causes of acute chest pain in children are most easily
categorized according to organ system or anatomic location. Dur-
ing inspiration, the superior liver edge goes as high as the nipple
line, so upper abdominal and diaphragmatic pathology should also
be considered.
Cardiac Pericarditis (viral, inflammatory, metabolic
[uremia, thyrotoxicosis], bacterial, fungal)
Myocarditis (viral, inflammatory conditions
including juvenile rheumatoid arthritis
[JRA], systemic lupus erythematosus [SLE],
and rheumatic fever)
Dysrhythmias (consider metabolic and
electrolyte causes)
Bradycardia
Supraventricular tachycardia
Ventricular tachycardia
Myocardial ischemia or infarction
secondary to:
Sickle cell disease
Kawasaki disease with coronary artery
aneurysms
SLE (with or without a lupus anticoagulant)
Antiphospholipid antibodies
66 Patient-Related Problems

Oral contraceptives
Cocaine or amphetamines
Exertion/physiologic stress with a coronary
artery anomaly/aberrancy
Severe aortic stenosis, pulmonary stenosis,
mitral valve prolapse
Cardiomyopathy (including hypertrophic
obstructive cardiomyopathy)
Aortic dissection or rupture (Marfan syndrome
or Takayasu arteritis)
Pulmonary Pneumonia
Pneumothorax (including tension
pneumothorax)
Pleuritis (infectious, JRA, SLE, familial
Mediterranean fever, medication induced)
Pulmonary embolism
Pulmonary infarction (acute chest syndrome)
Asthma exacerbation
Foreign body aspiration
Gastrointestinal Esophagitis (gastroesophageal reflux, pill
esophagitis, eosinophilic esophagitis)
Esophageal rupture (known as Boerhaave
syndrome)
Gastritis
Peptic ulcer (including hemorrhages and
perforations)
Biliary colic
Musculoskeletal Costochondritis
Rib fracture
Pectoral insertion pain or muscle strain
Clavicular fracture
Osteomyelitis
Precordial catch syndrome (“Texidor’s
Twinge”)
Mastalgia
Trauma (both accidental and nonaccidental)
Neurologic Thoracic radiculopathy
Neurotoxins from black widow (Lactrodectus)
spider bites
Psychogenic Anxiety
Somatization
Other regional considerations:
Mediastinum
Mediastinitis (infectious, caustic ingestion with
ruptured esophagus, trauma including recent
thoracic surgery)
 Chest Pain 67

Pneumomediastinum
Thymus cancers
Thymitis
Diaphragmatic (irritation can come from
above or below the diaphragm)
Diaphragmatic hernia
Subdiaphragmatic abscess
Splenic infarction or abscess
Pancreatitis

MAJOR THREAT TO LIFE

• Pericarditis with tamponade
• Pneumothorax, pneumomediastinum, or pneumopericardium
• Aortic dissection or rupture
• Myocardial ischemia or infarction
• Unstable dysrhythmias
• Pulmonary embolism (PE)
• Pulmonary infarction/ACS
• Perforated or hemorrhaging GI viscus
All of these conditions are rare in children, but each must be
considered for every patient complaining of chest pain given the
high rates of morbidity and mortality if missed. Often, these
pathologies occur in children with predisposing risk factors (e.g.,
aortic dissection with Marfan syndrome, pneumothorax with cystic
fibrosis).

BEDSIDE
Quick-Look Test
Does the child appear well and comfortable, with no signs of
distress?
If so, suspicion of major threat to life pathology is low. An ill-
appearing child requires prompt attention. Providers should stay at
the bedside until clinical stability is ensured. Body position may
offer clues to the source of the chest pain because pericarditis
and pericardial effusions may make it difficult for the child to
lay supine; patients with these pathologies often prefer to sit and
lean forward, sometimes in a tripod position.

Airway and Vital Signs

Labored respirations, tachypnea, central (oral) and peripheral cyano-
sis, nasal flaring, retracting, grunting, and hypoxemia (suggested by
pulse oximetry or definitive by lab study) are signs of respiratory com-
promise. If there is any concern regarding the current or future ability
to protect the airway, intubation must be considered; call a code for
68 Patient-Related Problems

critical care support. As noted earlier, fever suggests infectious causes

of pain. Blood cultures, chest imaging, and an ECG should be included
in the work-up. Tachycardia and tachypnea are nonspecific and may
occur as a result of the pain or may be indicative of cardiac or pulmo-
nary processes. Cardiopulmonary monitoring should be used.
Extreme tachycardia suggests SVT, and an ECG study should be
obtained immediately (see Chapter 22). Hypotension should be con-
sidered a result of shock until proven otherwise. Critical care support
is warranted. Key in treating this will be determining the underlying
type of shock (often categorized as distributive [including septic and
neurogenic], cardiogenic, hypovolemic, and obstructive). Fluids
should be given serially to help maintain adequate perfusion pressure
except in cases of cardiogenic shock; vasopressor or inotropic
therapy may be needed. Critical care staff should help in the manage-
ment of these patients. Adrenal insufficiency should be considered
in hypotensive patients. Narrow pulse pressure suggests pericardial
tamponade or tension pneumothorax. Pulsus paradoxus, an exagger-
ated decrease in systolic arterial pressure during inspiration (>10 mm
Hg), is also suggestive of these conditions. During inspiration, there is
normally a slight decrease in the filling of the left ventricle. This
decrease becomes exacerbated by the presence of a large amount of
pericardial fluid or by a tension pneumothorax. Normally, when
manual blood pressure readings are obtained, if the pressure is
decreased slowly, a systolic Korotkoff sound will initially be heard
only as the patient is exhaling. Continuing to decrease the pressure
slowly (2 to 3 mm Hg per heartbeat), the provider will hear the
systolic Korotkoff sound with both inspiration and expiration. The
degree of difference between these two points will indicate if pulsus
paradoxus is present (Fig. 25.2).

Selective Physical Examination

General Distressed breathing or cyanosis (initiate critical care
efforts)
Distressed posture (particularly tripod/anterior lean
position)
Confusion (uremia, other electrolyte and metabolic
abnormalities, drugs)
Marfanoid body habitus: tall, thin, long arms and
fingers (marfan.org for physical exam calculator
once stabilized)
HEENT Pupillary dilatation (cocaine, amphetamine)
Conjunctivitis (Kawasaki disease is classically bulbar
and nonexudative; infectious can lead to systemic
disease)
HEENT, Head, Eyes, Ears, Nose, Throat.
 Chest Pain 69

Uveitis (primary inflammatory conditions: juvenile

rheumatoid arthritis, systemic lupus
erythematosus)
Dislocated lenses (Marfan syndrome)
Blood in oropharynx (esophagitis, gastritis,
gastrointestinal viscus perforation, pulmonary
embolism [PE], acute chest syndrome,
pneumonia)
Neck Distended neck veins with or without prominent
venous pulsation (pericardial effusion)
Deviation of the trachea (tension pneumothorax)
Goiter (thyrotoxicosis)
Lymphadenopathy (Kawasaki disease, lymphoma,
leukemia)
Subcutaneous emphysema (pneumomediastinum
with tracking)
Chest Reproducible chest wall tenderness (costochondritis,
wall trauma, radiculopathy)
Localized point tenderness (rib or clavicular fracture,
osteomyelitis)
Suprasternal subcutaneous emphysema
(pneumomediastinum)
Pectoral stress maneuvers (Fig. 10.1; pectoral muscle
strain)
Erythema (cellulitis, myositis, mastitis)
Ecchymoses (trauma)
Heart Arrhythmias (intrinsic electrical, ischemia,
myocarditis)
Muffled or distant heart sounds (pericardial
effusion, pneumopericardium)
Murmur (numerous causes)
Rub (pericarditis)
Gallop (cardiomyopathy)
Crunching or grating sound (Hamman sign for
pneumomediastinum or pneumopericardium)
Lungs Grunting, nasal flaring, muscle retractions
(respiratory distress)
Absent breath sounds (pneumothorax until proven
otherwise, pleural effusion)
Rales (pneumonia, congestive heart failure, acute
chest syndrome, pulmonary embolus)
Wheezing (asthma, foreign body aspiration, acute
chest syndrome)
Pleural rub (pleuritis: infectious and primary
inflammatory)
Intrathoracic borborygmus (diaphragmatic hernia)
70 Patient-Related Problems

1 1

2 2

FIGURE 10.1 Pectoral stress maneuvers. With the patient’s arms

extended and elbows bent, ask the patient to move against resis-
tance, first upward and then downward.
Abdomen Rigid with guarding and rebound tenderness
(surgical abdomen with peritonitis)
Abdominal tenderness (gastrointestinal ulcer, viscus
perforation, biliary pathology, intra-abdominal
infection or infarction, empyema)
Extremities Swollen or tender extremity (deep venous
thromboses and PE)
Pulses Unequal right upper extremity to other pulses
(coarctation, dissection)
Absent femoral pulses (aortic dissection)
Skin Rash (Kawaski disease, infectious pericarditis or
myocarditis)
Livedo reticularis (antiphospholipid syndrome)

Selective History
What does the pain feel like? Where is it?
No descriptor of chest pain is pathognomonic, although many
are suggestive of certain etiologies. Acute dyspnea may often be
 Chest Pain 71

called chest pain by children. This complaint should raise suspicion

for pneumothorax, PE or infarction, ACS, and pneumonia. Pericar-
dial pain is usually sharp or stabbing; it is frequently referred to the
shoulder. Ischemic or infarction cardiac pain is often described as
crushing or squeezing and may be described with a clenched hand
(Levine sign). Tearing pain radiating to the back, classically intra-
scapular, or neck suggests aortic dissection, although this should be
highly suspected with any tearing chest pain. Pleuritic pain is sharp
and intermittent with position and respiratory cycle dependency.
Biliary pain is colicky and may have a postprandial temporal rela-
tionship. As mentioned previously, “pain” may also imply dyspnea,
palpitations, dysphagia, or anxiety. Pain from chest wall pathology
often feels superficial and may be localized. Attempting to elicit the
specific characteristics of what the child is feeling may require care-
ful questioning. Care should be taken not to use leading questions.
What exacerbates the pain?
Pain from the major threat to life pathology identified previ-
ously is usually progressive, often with no known exacerbating
factors. Pericarditis is worse in the supine position and is relieved
by leaning forward. Pain with inspiration suggests pleuritis or
a musculoskeletal cause. Pain with palpation of the chest is consis-
tent with musculoskeletal causes. Dysphagia suggests esophagitis.
Pain after eating may be secondary to esophageal reflux, biliary
pathology, or pancreatitis.
Has the child complained of chest pain previously?
A history of previous complaints and the results of previous
evaluations, if any, may provide clues to the cause of the current
complaints.
Selective Chart Review
What medications has the child received?
Amphetamines may cause tachyarrhythmias. Oral contraceptives
predispose to thrombosis and may lead to cardiac or pulmonary
infarction. Corticosteroids and nonsteroidal antiinflammatory agents
may cause esophagitis, gastritis, and peptic ulceration.
If not already done, a urine drug screen should be obtained if
there is any suspicion of recent amphetamine or cocaine use.
Review stat labs listed in Orders earlier. Correct electrolyte
abnormalities as needed to prevent arrhythmias. Be mindful of kid-
ney function when considering imaging studies with contrast. See
Chapter 32 regarding ABG interpretation.

Management
Pericarditis With or Without Tamponade
If your work-up suggests pericarditis, a pericardial effusion may be
present. Chest radiography may reveal an enlarged cardiac
72 Patient-Related Problems

silhouette or a “water bottle”–shaped heart. Pericardial effusions

are confirmed by echocardiography. Various ECG abnormalities
may be present, notably decreased voltage, particularly in the
QRS complexes, and generalized, nonregional ST-segment
changes. Therapeutic management of pericarditis depends on
the presence or absence of an effusion and the suspected cause.
Tamponade physiology (which clinically shows a narrow pulse
pressure, hypotension, pulsus paradoxus, and distended neck veins,
as well as distinct echo findings) is an emergency, and a therapeutic
and diagnostic pericardiocentesis should be done by a cardiologist
or an interventional radiologist. A drain is often left in place. If
needed, a pericardial window may be placed by a surgeon. Pericar-
diocentisis may be done on smaller, nontamponade effusions for
diagnostic work-up, if needed. This is particularly the case with
concerns for infectious pericarditis if it is presumed to be nonviral
and a source eludes other detection. Studies on the fluid should at
least include Gram staining, culture, and cell counts. Polymerase
chain reaction (PCR) studies and cytology may also be performed.
Antibiotics may need to be empirically started and later geared
to findings. Pericarditis secondary to viral infections or primary
systemic inflammatory diseases, such as JRA, may respond to IV
nonsteroidal antiinflammatory drugs (NSAIDs; ketorolac or indo-
methacin are generally used). Similarly, oral prednisone may be
sufficient for pericarditis secondary to systemic lupus erythemato-
sus, although if the pericarditis is severe, IV methylprednisolone
may be needed. Pericardial effusions secondary to thyroid disease
or uremia should respond to treatment of the underlying
condition.
Pneumothorax
Absent lung sounds in one hemithorax in a patient with respiratory
or cardiac distress, or a patient with acute chest pain, should be
treated emergently and empirically (before imaging confirmation)
at bedside for a presumed tension pneumothroax. This is done by
needle decompression. To accomplish emergency pneumothorax
evacuation:
1. Either the second intercostal space along the midclavicular line
or the third or fourth intercostal space along the midaxillary
line (this spot may be easier in infants) should be chosen and
cleaned in rapid but sterile fashion.
2. A 20- or 22-gauge angiocatheter should be used. Some new
models of angiocatheters have a mechanism that allows blood
to flash followed by a damming of the line to prevent blood loss;
these should be strictly avoided because this will prevent
decompression. The needle and catheter should be inserted
directly perpendicular to the skin along the upper surface of
 Chest Pain 73

the inferior rib (i.e., at the bottom of the intercostal space). This
protects the nerves and vessels that run adjacent to the inferior
margin of the ribs. Once the pleural space is entered, a “pop”
may be felt, and a rush of air may be heard through the angio-
catheter. The risk of causing a pneumothorax, should one hit
lung parenchyma, is low when using these gauges; that worry
should not prohibit nor delay this emergency procedure.
3. The needle should then be withdrawn while keeping the cath-
eter in place. The catheter should be secured against the
chest tightly. Do not stopcock the line, because tension would
accumulate.
4. Chest radiography should be done to evaluate the pneumo-
throax and catheter placement. The patient should be trans-
ferred to a critical care unit for monitoring and possible chest
tube placement.
If breath sounds are reassuring but a pneumothorax is seen on
imaging, treatment depends on its size. Giving 100% oxygen by
mask (or by hood in an infant) may result in quicker resolution
of the pneumothorax. Small pneumothoraces may resolve sponta-
neously. Large pneumothoraces require closed thoracostomy with
chest tube placement as prophylaxis against tension pneumothorax
and to aid in healing.
Pneumomediastinum and Pneumopericardium
Pneumomediastinum may be secondary to a pneumothorax; in
these cases, it should be treated as an extension of the pneumotho-
rax and may resolve with hemithorax decompression. Spontaneous
pneumomediastinum lacks an associated pneumothorax and is
often related to underlying pulmonary disease, particularly asthma
and lower respiratory tract infections. This develops when a small
parenchymal leak allows for air to track up along bronchovascular
bundles into the mediastinum without developing a pneumo-
throax. Tracking often continues into neck and upper extremity
soft tissues, causing palpable crepitus. Tracking may also continue
into the pericardial sac, causing a pneumopericardium. Pneumo-
mediastinum may also occur with esophageal perforation (often
caused by medication overuse, alkali or other caustic ingestion,
or as a complication to intraesophageal studies) and mechanical
ventilation. It may be iatrogenic from intrathoracic procedures
or surgery or may be idiopathic. Complications from tension accu-
mulation are rare, particularly in non-neonates. Chest radiographs
reveal a sharper cardiac border and may also disclose subcutaneous
air. ECG studies may have low voltages and nonspecific segment
and interval changes. Echocardiography may be helpful in deter-
mining if pneumopericardium is present, particularly if tamponade
physiology is suspected. Treatment is directed at the underlying
74 Patient-Related Problems

cause. Unless there is cardiovascular compromise, the air in the

mediastinal and soft tissue space need not be evacuated and
resolves spontaneously. Pneumomediastinum after esophageal
perforation requires emergent surgical repair with empiric aerobic
and anaerobic antibiotics for presumed concurrent mediastinitis.
Blood cultures should be obtained in these cases.
Aortic Dissection or Rupture
These conditions have notably high mortality and morbidity rates.
Life is preserved with early high indices of suspicion. Any patient
with a suspected dissection or rupture requires critical care man-
agement. Imaging is required to make the diagnosis. Computed
tomography (CT) angiography is generally the fastest modality
for making a radiographic diagnosis. Magnetic resonance angio-
gram has a role. For patients who are hemodynamically unstable
or unable to tolerate IV contrast, bedside echocardiography may
be used. A widened mediastinum on a chest film raises suspicion
but is not diagnostic, and this should not be used as a screening
test. Any dissection should have a stat cardiothoracic surgery con-
sult. Blood for a CBC, BMP, prothrombin time (PT), partial throm-
boplastin time (PTT), and blood typing and cross matching should
be sent to the laboratory immediately. Large-bore IV access in at
least two sites should be ensured. Propagation of the dissection
may be limited with decreasing shear stress through beta-blockade
or other medical suppression of cardiac output. Pain control
should be ensured because this will help to decrease adrenergic
drive. The surgery team will help to decide if emergent surgical
intervention is indicated or if medical management will suffice.
Myocardial Ischemia and Infarction
These pathologies are generally due to sickle cell vasoocclusion,
vasculitides, cocaine or methamphetamine use, or compression
of an aberrant coronary artery. When myocardial ischemia or
infarction is suspected, an ECG should be obtained to look for pat-
terns consistent with these diagnoses (Table 10.1). Serum troponin
and fractionated CPK (CK or CPK and CK-MB) levels should also
be determined; as previously stated, many hospitals bundle these
studies as a “cardiac panel.” These levels should be trended over
time (generally with three studies at 4- to 6-hour intervals) because
enzymatic leak takes time to occur after myocardial insult. A CBC,
BMP, PT, PTT, and a sample for blood typing and cross matching
should be sent to the lab. A stat cardiology consult should be placed
if ECG or lab studies are in any way concerning or if clinical judg-
ment leads to a high suspicion of ischemia. The patient should be
immediately transferred for critical care and cardiology manage-
ment. Nitroglycerin may relieve the chest pain. Morphine should
 Chest Pain 75

TABLE 10.1 Myocardial Infarction Patterns

Patterns of Changes (Q Waves, ST

Elevation, or Type of Infarct Inversion)a Depression, T Wave
Inferior Q in II, III, AVF
Inferoposterior Q in II, III, AVF, and V6
R > S and positive T in V1
Anteroseptal V1 to V4
Anterolateral to posterolateral V1 to V5; Q in I, AVL, and V6
Posterior R > S in V1, positive T, and
Q in V6
a
A significant Q wave is greater than 40 msec wide or greater than a third of the QRS height.
ST-segment or T wave changes in the absence of significant Q waves may represent a non–Q
wave infarct.
From Marshall SA, Ruedy J: On Call: Principles and Practices, 2nd ed. Philadelphia, WB
Saunders, 1993.

generally be avoided given the risk for respiratory depression but

may be used in small doses if necessary. Aspirin should be given
empirically. Oxygen should be administered to all patients, initially
at 100%; this will help with potential sickling and will decrease
myocardial demand. Patients with sickle cell disease may require
an exchange transfusion and those with hypercoagulable states
may require heparinization.
Stable and Unstable Dysrhythmias
See Chapter 22.
Pulmonary Embolism
The diagnosis of PE is often delayed due to low clinical suspicion.
Suspicion should be raised for patients who are immobile (includ-
ing those with recent surgery), those with central venous lines, and
those with hypercoaguable states (including those using oral con-
traception). Approximately half of patients also have deep venous
thromboses (DVTs). Signs and symptoms do not have high sensi-
tivity or specificity but may include acute chest pain, hypoxemia,
tachypnea or other signs of respiratory distress, and extremity evi-
dence of DVT. Chest x-rays have no role in the work-up. Likewise,
a negative D-dimer has been shown to not rule out PE in children
and is not specific for PE if positive. No diagnostic probability
scoring systems (such as the Wells score) have been validated
in children to this point. CT angiography of the chest or
ventilation-perfusion scan should be used for diagnosis. Oxygen
should be given to all patients with suspected PE. Rapid response
or critical care support may be needed if the child is unstable.
76 Patient-Related Problems

Systemic thrombolysis with tissue plasminogen activator may be

needed in unstable patients; the critical care team will help guide
the decision to use this. Initial therapy is usually a heparin
drip or therapeutic Lovenox subcutaneous injections. Long-term
anticoagulation will be needed.
Pulmonary Infarction/Acute Chest Syndrome
Pulmonary infarction may be caused by thrombi but is most com-
monly caused in children by vasoocclusion secondary to sickle cell
disease exacerbation, which leads to a condition called the ACS.
This is a common cause for hospitalization and death in children
with sickle cell disease. Infections, asthma exacerbations, nonpul-
monic vasoocclusion events, and anything leading to atelectasis
may trigger the intrapulmonary sickling. Patients with ACS often
have fevers, hypoxemia, and respiratory distress. Chest radiogra-
phy is key in diagnosing this condition with consolidations being
seen in many cases, sometimes in a diffuse pattern. A stat hematol-
ogy consult should be placed for any patient suspected to have
ACS. Continuous pulse oximetry should be monitored and any
hypoxemia treated with supplemental oxygen. Respiratory distress
should prompt a call for rapid response assistance with an ABG
draw for evaluation. Although dehydration can lead to sickling
and should be corrected, care should be taken not to overcorrect
because pulmonary edema will worsen the patient’s respiratory sta-
tus. Bronchodilators should be given, particularly if asthma is trig-
gering the event. Empiric antibiotics (a third generation and a
macrolide) should be given. A CBC, CMP, and a sample for blood
typing and cross matching should be obtained. Hematology should
help guide decisions about transfusions. The patient will require
critical care.

Perforated or Hemorrhaging Gastrointestinal Viscus

The presentation of these pathologies is usually obvious with severe
pain and possible hematemesis and hemodynamic instability.
These conditions very rarely happen without an overt underlying
cause including caustic ingestion, medication (particularly NSAID)
overuse, recent surgery or intralumenal endoscopic studies, con-
nective tissue disorders, or severe retching (Boerhaave syndrome).
Initial management should be focused on maintaining the airway
and hemodynamic stability; rapid response assistance is appropri-
ate if any help is needed. Stat surgery and GI consults should be
placed. Blood work should include a CBC, BMP, PT, PTT, and
blood typing and cross matching. Plain films of the chest and abdo-
men in the left lateral decubitus or the cross-table positions may be
helpful in evaluating for free air. Empiric antibiotics should be
 Chest Pain 77

started as soon as possible and should cover aerobic and anaerobic

organisms; blood cultures may be useful but should not delay
treatment.
The following diagnoses should be considered and treated only
after the above diagnoses have been clinically ruled out.
Myocarditis
This diagnosis is often seen with concurrent pericarditis. When
isolated, it is generally due to a viral infection, although it may
be seen with primary inflammatory conditions or bacterial infec-
tions. Help with diagnosis and management should be sought
through a cardiology consult. Because myocarditis may lead to
arrhythmias, children suspected to have this diagnosis should
be monitored on telemetry. ECG findings often suggest myocar-
dial injury with ST segment changes and T wave changes. Cardiac
enzymes (troponin, CK, and CK-MB) are generally elevated.
Chest radiography is nondiagnostic. Echocardiography and car-
diac magnetic resonance imaging (MRI) may provide strong evi-
dence of myocarditis. Endomyocardial biopsy via a cardiac
catheterization is the gold standard for diagnosis. Patients with
fulminant myocarditis have heart failure, and diuretic, afterload
reducing (angiotensin-converting enzyme [ACE]-I), and inotro-
pic therapies may be needed.

Severe Valvular Disease and HOCM

A careful, thorough cardiac examination will at least suggest these
etiologies and may allow for clinical diagnosis. Echocardiography
will delineate the pathology and the degree of its severity. Medical
manipulation of preloads or afterloads will likely be indicated in
children with chest pain. In severe cases, interventional cardiology
or surgical management may be needed. The patient should be
monitored on telemetry and have frequent vital checks. A cardiol-
ogy consult should be used to help with management.
Pneumonia
Focal adventitial lung sounds, hypoxemia, consolidation(s) on
chest radiography, fevers, respiratory distress, and hemodynamic
instability may be seen in patients with pneumonia. A CBC with
differential and blood cultures, as well as sputum Gram stain
and culture, if possible, should be obtained. If bacterial pneumonia
is suspected from the clinical and chest radiography findings, anti-
biotics are required. Antibiotic choice should be determined based
on whether the infection is considered community acquired, hos-
pital acquired, or due to aspiration. Review of any past infections
and Pseudomonas infection risk factors will also help to guide
78 Patient-Related Problems

therapy. Continuous pulse oximetry with supplementation as

needed is indicated. See Respiratory Distress, Chapter 28, for fur-
ther management guidance.

Pleural Effusion
If the effusion is leading to hypoxemia, supplemental oxygen
should be given. Incentive spirometry is indicated for all patients
with effusions. Effusions in patients with any signs of infection,
especially respiratory infections, should be considered empyemas
until proven otherwise. Thoracentesis should be strongly consid-
ered for diagnostic purposes in all patients with new effusions and
is indicated if any concerns exist about it being an empyema. If the
effusion is large, thoracentesis may be therapeutic as well. This
procedure is usually done under imaging, often by interventional
radiology. Critical care support may help to perform this as a bed-
side procedure if the patient is unstable. Studies on the fluid
should include Gram stain, culture, cell count, lactic acid dehy-
drogenase (LDH), and total protein determination. Concurrent
serum LDH and total protein should be measured for evaluation
using Light’s criteria. If the tap is bloody, obtain a hematocrit on
the sample. If it is turbid, obtain a triglyceride level on the sample.
If there are concerns for malignancy, obtain cytology and fluid
pH. If pancreatitis is suspected, a fluid amylase level may be
helpful.

Pleuritis
This usually responds well to conservative, symptomatic treatment
(e.g., NSAIDs).

Asthma Exacerbation
See Chapter 28.

Foreign Body Aspiration

See Chapter 28.

Esophagitis, Gastritis, or Peptic Ulcer

A “GI cocktail” of viscous lidocaine, liquid diphenhydramine,
and an antacid may be therapeutic, with an improvement in
symptoms suggesting these pathologies. Maintenance antacid,
H2-receptor antagonist, or proton pump inhibitor therapy
may be used. Consultation with a gastroenterologist and endo-
scopic examination may be required to evaluate the underlying
cause of pathology. A day-to-day trend of the hemoglobin/
hematocrit (Hgb/Hct) may help to identify bleeding; a markedly
 Chest Pain 79

elevated blood urea nitrogen (BUN), especially without a reflec-

tive rise in the creatinine, is suggestive of an upper GI bleed. If
bleeding is suspected, more frequent Hgb/Hct checks may be
appropriate.

Diaphragmatic and Peridiaphragmatic Pathology

Irritation of the diaphragm from either side may present as chest
pain. Pleural effusions are discussed previously. Diaphragmatic
hernias may be identified by intrathoracic borborygmus and chest
radiography showing stomach or bowel contours above the dia-
phragm. Subdiaphragmatic abscess is usually diagnosed only if
there is a high suspicion; CT imaging of the chest and abdomen
with contrast may identify such pathology. Splenic infarction or
abscesses are rare. Infarction may take place during sickle crises
or with hypercoaguable states, including myeloproliferative can-
cers. Abscesses usually develop secondary to a bacteremia or endo-
carditis. Blood counts with differential, a peripheral smear, and
blood cultures should be obtained if splenic pathology is consid-
ered. CT studies are often needed. Pancreatitis generally leads to
abdominal pain, although chest pain may be concurrent. Serum
lipase and amylase levels along with abdominal imaging (ultraso-
nography or CT) help make the diagnosis. Biliary colic has a dis-
tinct intermittent nature. Liver panel chemistries and right upper
quadrant ultrasonography are helpful in making the diagnosis.
See Chapter 23.

Chest Wall Musculoskeletal and Neurologic Pain

These conditions may lead to guarded or weak inspiratory
effort, tachypnea with possible hyperventilation, and anxiety.
The pain is often reproducible with palpation and positional
changes. Pain control is key; NSAIDs and topical therapies
are often helpful. Many of the underlying conditions spontane-
ously resolve. Consideration should be given to possible
infections.

Psychogenic
Reassurance and efforts aimed at relaxing the patient are the best
approach. As with abdominal pain, headaches, and extremity pain,
idiopathic chest pain is common in children, particularly during
the preadolescent years. Concern for family members who have
heart disease may contribute to the child’s fears and subsequent
pain. Reassurance that they have a “normal heart,” once clinically
determined, may be all that is required to result in the resolution of
the pain.
80 Patient-Related Problems

REMEMBER
Rapid response and critical care assistance should be sought
quickly when needed. All life-threatening causes of chest pain
should be considered and ruled in or out for every patient com-
plaining of chest pain. Thinking through all of the organ
systems in the thorax or mentally going “through the chest” in
an anterior to posterior manner helps to make sure differential
diagnoses are not overlooked.
 C HA P TE R

11
Constipation
Maja Z. Katusic, MD

Constipation is a common problem in both outpatient and inpa-

tient pediatrics. An estimated 3% to 5% of all pediatrician visits
are due to constipation. Calls about hard stools or no stools are
frequent in the hospital. Constipation is most often functional
due to stool retention. However, additional rare etiologies must
always be considered, especially in the hospital setting.

PHONE CALL
Questions
1. Is the stool hard with painful defecation or has there been no
stool at all? When was the last time the child had a stool?
2. How old is the child?
3. Why is the child hospitalized?
4. Are there any associated gastrointestinal symptoms or signs,
such as abdominal pain, poor oral intake, nausea, vomiting
(especially bilious), or abdominal distention?
5. Does the child have a history of constipation?
6. What medications is the child taking?
Characterizing the “constipation” is the most important step.
It may be normal for a formula fed infant to go up to 2 to 3 days
without a stool. Breastfed infants generally have stools more often.
Infants on average go 3 to 4 times per day. Toddlers have approx-
imately 2 to 3 stools per day. By age 4 or older, stool patterns are
about the same as adults. Constipation tends to happen in times of
transition. For infants, this can occur when they go from breastfed
to formula fed. In toddlers, difficulty with stools may develop when
going through toilet training. Finally, school-age children may
become more constipated when entering school and they have to
use unfamiliar bathrooms.
In general, the nature of the stool is much more indicative of
constipation than the frequency is. Straining in infants is a

81
82 Patient-Related Problems

common concern for parents. Infant dyschezia occurs in healthy

infants younger than 6 months who strain excessively due to dif-
ficulty coordinating increasing intra-abdominal pressure with
relaxing the pelvic floor to allow passage of stool. Straining alone
usually requires no further evaluation or intervention.
Once it is clear that the child does in fact appear to be consti-
pated, the age of the child allows you to consider the likelihood of
possible causes for the constipation. In addition, the reason for hos-
pitalization and other clinical signs may suggest specific etiologies,
including potential serious causes of constipation, such as bowel
obstruction. A previous history of constipation suggests a chronic
process, and medication side effects (i.e., secondary to opioids) are
a relatively frequent cause of constipation.
Orders
Most calls regarding infrequent or absent stools do not require urgent
action. If the problem is brought to your attention in the middle of
the night and the child is stable with no associated symptoms, it
may often be appropriate to defer further evaluation if you are busy
with more urgent matters. In this situation, symptomatic treatment
may be reasonable if it appears that it will be a while until you are able
to evaluate the child further. The management section below discusses
treatment options, including laxatives, stool softeners, and enemas. It
should be emphasized that these orders should not simply be reflexively
given. A child who has associated symptoms such as abdominal pain,
vomiting, or abdominal distention needs to be evaluated promptly
because a significant intra-abdominal process is then more likely.

ELEVATOR THOUGHTS
Potential causes of constipation differ depending on the age of the
child and can be divided into general subgroups:

Infant
1. Anatomic
• Imperforate anus or anal atresia
• Malrotation of gut
• Hirschsprung disease
• Spinal cord lesions (meningomyelocele)
2. Dietary
• Insufficient fluid intake
• Insufficient fiber intake
3. Endocrine/electrolyte imbalance
• Hypothyroidism
• Hypokalemia
• Hypercalcemia
 Constipation 83

4. Syndromes
• Prune belly syndrome
• Trisomy 21
• Meconium ileus (cystic fibrosis)
5. Other
• Infant botulism
• Pseudo-obstruction
Older Child
1. Functional (i.e., withholding)
2. Dietary
• Insufficient fluid intake
• Insufficient fiber intake
3. Anatomic
• Bowel obstruction
• Painful defecation due to anal fissure
• Hirschsprung disease
• Spinal cord lesion (e.g., tumor)
• Abdominal or pelvic mass
4. Endocrine/electrolyte abnormalities
• Hypothyroidism
• Hypokalemia
• Hypercalcemia
5. Neurologic
• Guillain-Barre syndrome
• Food-borne botulism
6. Other
• Drugs or toxins (e.g., opioids, anticholinergics, iron supple-
ments, antacids, antidepressants, lead poisoning)
• Ileus (postoperative, postviral)
• Dysmotility

MAJOR THREAT TO LIFE

Constipation as the sole complaint is not suggestive of a
life-threatening illness. The major threats to life are associated
with other signs and symptoms, which should suggest a specific
diagnosis.
• Bowel obstruction (abdominal pain, bilious vomiting)
• Botulism (weakness, hypotonia)
• Guillain-Barre syndrome (weakness, decreased deep tendon
reflexes)
• Exposure to drugs or toxins (altered mental status)
• Hypokalemia (e.g., in patients admitted with eating disorder
and at risk for refeeding syndrome)
84 Patient-Related Problems

BEDSIDE
Quick-Look Test
In nearly all cases the infant or child will likely be comfortable if
constipation is an isolated symptom. If an infant is irritable or
an older child appears distressed, one of the aforementioned major
threats to life should be considered.

Airway and Vital Signs

A child with isolated constipation is expected to have normal vital
signs and a stable airway. If this is not the case, a prompt search for
signs to suggest one of the threats to life listed previously should be
undertaken. Fever, tachycardia, and hypotension suggest bowel
obstruction with perforation and consequent peritonitis. Labored
respirations may be a sign of respiratory muscle weakness second-
ary to botulism or Guillain-Barre syndrome. Arrhythmias or
altered heart rate may be seen with hypokalemia or a number of
toxic syndromes (opioids, anticholinergics).

Selective History and Chart Review

Does the child have abdominal pain, nausea, vomiting, or abdom-
inal distention?
Relatively mild pain or rarely severe pain may occur as a result
of constipation. Other gastrointestinal symptoms such as disten-
tion or bilious vomiting should raise concern about bowel
obstruction.
In a neonate, has there been a normal stool yet? Is there a history
of a meconium plug?
Meconium plug at birth suggests Hirschsprung disease or cystic
fibrosis. These disorders, as well as anatomic anomalies of the
gastrointestinal tract, are possibilities if the neonate has not yet
had their first stool.
What is the patient’s diet? Has the infant (<12 months old) had
honey?
Breastfed infants should have several relatively loose, light
yellow or green stools every day, often after each feeding. Therefore
constipation in an exclusively breastfed infant is rare and suggests
either inadequate intake of breast milk or an explanation other
than diet. Insufficient volume of intake because of either diet or
intercurrent illness may cause defecation difficulties. In both
infants and older children, low fecal bulk may result in an
inadequate stimulus to peristalsis and subsequent constipation.
A high-starch, high-protein diet or the continued use of pureed
foods beyond infancy may not provide adequate fiber to promote
defecation. Honey is a notorious source of Clostridium botulinum
spores, the cause of infant botulism.
Has there been a history of constipation or fecal soiling?
 Constipation 85

Chronic constipation may lead to encopresis, characterized by

retention of stool with leakage of fluid stool involuntarily around a
large fecal mass. This may occur as a result of withholding second-
ary to a traumatic toilet training experience, painful defecation due
to anal fissures, or another psychologic disturbance. Occasionally,
Hirschsprung disease is diagnosed later in childhood after years of
constipation. A history of never having a normal stool pattern may
suggest such an underlying anatomic defect.
What medications has the child received?
Constipation may be secondary to a long list of possible
medications. This includes opioids, iron supplements, or
anticholinergics.

Selective Physical Examination

HEENT Mydriasis (anticholinergic, botulism); miosis
(opioids)
Cardiovascular bradycardia (opioids, hypothyroid); tachycardia
(anticholinergic); hypertension
(anticholinergic, Guillain-Barre); hypotension
(opioids, Guillain-Barre)
Lungs Tachypnea (abdominal distention due to bowel
obstruction); respiratory insufficiency
(Guillain-Barre, botulism, bowel obstruction)
Abdomen Hyperactive or hypoactive bowel sounds;
rebound; rigidity; distention (bowel
obstruction)
Rectal Nonpatent rectum (imperforate anus); impacted
stool in the vault or anal fissure (functional
constipation)
Neurologic Absent or delayed reflexes (Guillain-Barre,
spinal cord lesion); hypotonia (spinal cord
lesion, Guillain-Barre, botulism)
HEENT, Head, Eyes, Ears, Nose, Throat.

Management
When you are on call, most patients with constipation can be treated
symptomatically with plans to address a potential systemic cause at a
later point. The exceptions would be if the patient appears to have
signs or symptoms suggestive of a bowel obstruction, Guillain-Barre
syndrome, a spinal cord lesion, an electrolyte disturbance, or botu-
lism. If there are any concerns about bowel obstruction, the patient
should be nil per os and a surgeon should be consulted as soon as
possible. If Guillain-Barre syndrome or botulism is a concern, close
monitoring, especially of respiratory status, and potential treatment
with intravenous immunoglobulin (IVIG) or botulism antitoxin
86 Patient-Related Problems

therapy, respectively, would be appropriate. If there is concern for a

spinal cord lesion, imaging studies such as spinal magnetic reso-
nance imaging (MRI) should be performed and a neurosurgeon
should be consulted. Electrolyte disturbances should be identified
with laboratory testing if the patient is at risk for these and corrected
appropriately (see Chapter 34).
Imaging of a constipated child may be necessary if it is unclear
whether constipation is present or to further evaluate a potential
bowel obstruction. An abdominal radiograph may confirm a
large amount of stool in the bowel. If bowel obstruction is sus-
pected, an abdominal radiograph may reveal free air due to bowel
perforation. Further imaging in such a case should be discussed
with a surgeon. Additional imaging studies, such as computed
tomography or MRI of the spine, abdomen, or pelvis, may be pur-
sued if there is concern for spinal, abdominal, or pelvic masses
leading to constipation.
Laboratory tests rarely need to be ordered urgently in the setting
of constipation. If Guillain-Barre is suspected, lumbar puncture
should be done to look for elevated protein in the cerebrospinal
fluid prior to starting IVIG. If botulism is a possibility, C. botulism
toxin in the stool can be ordered, but in most laboratories this result
will likely not be available for several days. Therefore electromyog-
raphy (EMG) may be performed when the diagnosis is suspected,
and if the EMG reveals characteristic findings, treatment of botu-
lism may be initiated while awaiting the results of the stool testing.
Consultation with a neurologist in this situation will likely be
necessary to perform and interpret the EMG. Rectal biopsy will
confirm Hirschsprung disease. Serum thyroid-stimulating hor-
mone and free thyroxine (T4) can be tested if hypothyroidism is
suspected. A serum metabolic panel may be obtained to assess
for electrolyte imbalances if there is concern that such abnormal-
ities may be contributing to constipation.
Symptomatic treatment of constipation includes laxatives
(osmotic or stimulant), stool softeners, and rectal enemas. Osmotic
laxatives include polyethylene glycol (PEG) (Miralax), lactulose, or
milk of magnesia. Mineral oil is an excellent stool softener. Some
evidence suggests that PEG is more effective for functional consti-
pation compared with lactulose, milk of magnesia, mineral oil, or
placebo. Stimulant laxatives include bisacodyl (Dulcolax) and
senna (Senokot). Enema options are sodium phosphate (also
known as Fleet enema) or normal saline. For infants, a glycerin
suppository or normal saline enema can be used. If these measures
are unsuccessful, manual disimpaction may be necessary. Lubi-
prostone (Amitiza) is a recently developed oral chloride channel
protein activator that has been well tolerated in the pediatric
 Constipation 87

population; however, experience thus far with this treatment

remains limited.
An inadequate diet is often a significant contributor to consti-
pation. Several options are available to increase bulk or soften stool.
Increasing the volume of fluid intake, as well as dietary supple-
ments such as prune juice, olive oil, or mineral oil, may be helpful.
Vegetables, fruits, bran, and whole grains add bulk. An easy way to
remember the recommended grams of daily fiber in a child’s diet is
that it is equal to the child’s age plus five.
Most of the time while on call, constipation can be treated
symptomatically. However, it is always important to evaluate the
patient for potential warning signs that might indicate the need
for further evaluation. If there are no concerning symptoms or
signs, initial treatment with laxatives and enemas as needed may
be most appropriate, with further evaluation of potential systemic
causes performed at a later time if necessary.
Bibliography
Biggs WS, Dery WH: Evaluation and treatment of constipation in infants
and children, Am Fam Physician 73(3):469–477, 2006.
Colombo JM, Wassom MC, Rosen JM: Constipation and encopresis in
childhood, Pediatr Rev 36(9):392–402, 2015.
Hyman PE, Di Lorenza C, Prestige LL, et al: Lubiprostone for the treatment
of functional constipation in children, J Pediatr Gastroenterol Nutr
58(3):283–291, 2014.
Tabbers MM, et al: Evaluation and treatment of functional constipation in
infants and children: evidence-based recommendations from ESPGHAN
and NASPGHAN, J Pediatr Gastroenterol Nutr 58(2):258–274, 2014.
 CHAPTER

12
Crying and the
Irritable Infant
Julia Richards, MD

Crying and irritability may be considered expected findings in hos-

pitalized infants because these signs are frequently associated with
any condition causing pain or discomfort. Usually it will be clear
that the infant is irritable because of a known underlying illness.
However, in some hospitalized infants the irritability or crying
may be difficult to explain. There may not be a readily apparent
cause for the irritability, or the character or degree of the irritability
may differ from that present on admission. Evaluation of an irri-
table infant must be particularly thorough to search for clues that
suggest a source of the irritability. Sepsis and other infections
should always be at the top of the list of considerations, even in
the absence of fever, but this should not keep you from thinking
of other possible explanations. The age of the patient (whether they
are younger or older than 2 months old) is also an important factor
when considering infection as a potential cause because at a very
young age, there may be few or no additional signs and symptoms.
Crying in an infant in the absence of illness, such as that seen in
infants with colic, should always be a diagnosis of exclusion.

PHONE CALL
Questions
1. What are the vital signs and trends?
2. Has the infant’s degree of irritability changed since admission?
Is this a new sign or simply a persistent one?
3. Why is the infant hospitalized?
The development of a new fever with irritability is strongly sugges-
tive of infection and requires immediate action. Some tachycardia
is expected in an infant who is irritable, but extreme tachycardia or

88
 Crying and the Irritable Infant 89

progressive abnormalities in the vital signs should also raise your level
of concern. Because at least some degree of irritability is likely to be
present in most hospitalized infants, it is important to determine
whether the current status of the infant is different. For example,
an infant admitted with bacterial meningitis who has received only
a few doses of antibiotics may continue to be irritable for several days
until the infection and meningeal inflammation begin to resolve. In
contrast, in an infant who has been gradually responding to treatment
and then becomes increasingly irritable, a new condition may have
developed to explain the change in status. It may be serious, such
as a subdural effusion, or simple, such as an infiltrated intravenous
(IV) site.

Orders
No orders should be given until the infant is further evaluated.

Inform RN
An infant with a change in the degree of irritability or new onset of
irritability needs to be evaluated immediately.

ELEVATOR THOUGHTS
What might cause irritability in the infant? As mentioned earlier,
almost any condition may be associated with irritability, and infec-
tion is a very common cause. The following list contains some of
the more common possibilities, as well as some that may often be
overlooked. Remember to prioritize this list based on the age of the
infant:
Infection Sepsis
Meningitis
Encephalitis
Brain abscess
Lymphadenitis
Pneumonia
Gastroenteritis
Myocarditis
Pericarditis
Viral syndrome
Cellulitis
Mastitis
Otitis media
Urinary tract infection
Pyomyositis
Osteomyelitis
Septic arthritis
90 Patient-Related Problems

Gastrointestinal and intra- Gastritis

abdominal conditions Gastroesophageal reflux
and esophagitis
Intussusception
Volvulus
Bowel obstruction
Appendicitis
Peptic ulcer disease
Constipation
Anal fissure
Inguinal hernia
Inflammatory disorders Kawasaki disease
Systemic juvenile idiopathic
arthritis
Metabolic and endocrine Hypocalcemia or hypercalcemia
disorders Hyponatremia or hypernatremia
Hypokalemia
Hypoglycemia
Urea cycle disorders (early)
Reye syndrome (early)
Zinc deficiency
Protein malnutrition
Scurvy
Hyperthyroidism
Cardiac processes Supraventricular tachycardia
Congestive heart failure
Pericarditis
Anomalous left coronary—artery
from the pulmonary artery
Intracranial processes Increased intracranial pressure
Subdural hematoma
Subdural effusion with meningitis
Brain tumor
Seizures
Toxic processes Lead ingestion
Vitamin A poisoning
Narcotic withdrawal
Fetal alcohol syndrome
Other disorders Trauma, including nonaccidental
Respiratory failure
Foreign body ingestion
Teething
Colic
Hunger
 Crying and the Irritable Infant 91

Mechanical problems Infiltrated intravenous (IV) site

Entanglement in monitor wires
or IV tubing
Skin irritation from monitor lines
Blood pressure cuff
Too small a diaper
Lying on a foreign body in the bed
“Hair tourniquet” of a digit

MAJOR THREAT TO LIFE

• Infections (sepsis, meningitis)
• Intra-abdominal conditions (bowel obstruction, appendicitis)
• Metabolic disturbances
• Increased intracranial pressure (ICP)
• Seizures
• Cardiac disorders (dysrhythmia, pericarditis, myocarditis)
• Respiratory failure
Immediate evaluation of an irritable infant should focus on
searching for signs suggestive of one of the aforementioned threats
and/or recognizing that the infant is at risk for one or more of these
conditions because of an underlying illness or its treatment.

BEDSIDE
Quick-Look Test
Irritability can be intermittent, persistent, or progressive. If the
infant is quiet by the time of your arrival, this is not necessarily
reassuring. Intussusception, anal fissure, seizures, supraventricular
tachycardia, gastroesophageal reflux, systemic juvenile idiopathic
arthritis, teething, and colic should be greater considerations if
the irritability is intermittent. As mentioned before, your differen-
tial should also be considered in the context of the child’s age.

Airway and Vital Signs

Fever can be associated with irritability in an infant. Fever and irri-
tability, especially in very young infants (younger than 2 months),
is sepsis until proven otherwise. This warrants further evaluation,
including blood, urine, and cerebrospinal fluid culture and the
administration of IV antibiotics (see management section later).
92 Patient-Related Problems

In a critically ill infant, the risks of delaying antibiotic treatment

need to be weighed against the benefit of obtaining as many of
the cultures mentioned as possible.
Hypothermia: In an infant, hypothermia can also be a sign of
infection.
Tachycardia: Tachycardia can be associated with fever. Extreme
tachycardia can be suggestive of a cardiac etiology, such as supra-
ventricular tachycardia (see Chapter 22), as well as myocarditis.
Tachypnea: Tachypnea may be secondary to respiratory distress or
respiratory failure, infection, such as bronchiolitis or pneumo-
nia, congestive heart failure, or metabolic acidosis.
Hypotension: Hypotension may be related to sepsis or may be
related to hemorrhage.
Keep in mind that tachycardia and tachypnea can be secondary to
crying and irritability.

Comprehensive Physical Examination

When evaluating an irritable hospitalized infant, the physical
exam should be the initial step in your evaluation rather than
the usual approach of history taking followed by physical exam-
ination. It is critical that the physical examination be especially
thorough rather than selective. Particular attention needs to be
paid to potential sources of infection (e.g., ears), the abdominal
examination, and a careful examination of the extremities. It is
very easy to overlook the fact that an infant is not moving an
extremity normally because of pain. This may be seen with septic
arthritis, osteomyelitis, “hair tourniquets,” and trauma (including
occult fractures). Carefully inspect any IV sites by removing tape
and other coverings so that you can clearly visualize the skin. An
infiltrated or infected IV site can easily be overlooked. Remember,
the fontanelle exam is as important as the vital signs in an irritable
infant. Meningeal signs are not reliable indicators of meningitis in
an infant.
HEENT Fontanelle (flat or bulging?), pupils, conjunctivitis
(infections, Kawasaki disease), periorbital soft tissue
(cellulitis), tympanic membrane (otitis), auditory
canal (foreign body, trauma), nose (rhinorrhea),
mouth, pharynx (stomatitis, trauma)
Neck Adenopathy (Kawasaki disease); erythema, warmth
(adenitis)
Chest Breath sounds (pneumonia, foreign body aspiration,
congestive heart failure); breast swelling, warmth
(mastitis); erythema, tenderness (rib fracture)
HEENT, Head, Eyes, Ears, Nose, Throat.
 Crying and the Irritable Infant 93

Cardiovascular Heart sounds (myocarditis, pericarditis,

supraventricular tachycardia); thrills, heaves
(congestive heart failure)
Abdomen Bowel sounds, distention, masses (bowel
obstruction)
Genitalia Masses (inguinal hernia), testicular swelling or
tenderness
Rectal Masses, stool, bleeding
Extremities Swelling, warmth (infections); bruising
(trauma); cyanosis (respiratory failure), “hair
tourniquet”; limited use, limited range of
motion, tenderness (trauma, infection)
Neurologic Focal signs (brain abscess, tumor), seizures
(metabolic disturbance)
Skin Rash, petechiae, purpura (systemic juvenile-
idiopathic arthritis, meningococcemia);
wounds (trauma)

Selective History and Chart Review

What has the infant’s previous behavior been like? How is the infant
described in previous notes?
An attempt should be made to determine whether the infant’s
status has changed. If so, this suggests that either a new problem
has developed or the underlying illness being treated during this
hospitalization is progressing.
Does the infant have a known infection that is currently being
treated?
If so, you need to consider the possibilities of inadequate treat-
ment and/or a complication of the initial infection (e.g., mastoiditis
following otitis; meningitis, osteomyelitis, or septic arthritis follow-
ing bacteremia). Look carefully at the fever pattern during the
hospitalization. A recurrence following initial defervescence
suggests “seeding” of a distant site after bacteremia.
What has the infant’s oral intake been? Has there been any
vomiting, constipation, or diarrhea?
The answers may suggest a gastrointestinal cause of the irrita-
bility. A change in oral intake may also suggest worsening of an
underlying illness.
Has the infant received IV fluids?
Review the type and amount of fluids, as well as any recent elec-
trolyte measurements, to determine the likelihood of a metabolic
abnormality to explain the irritability.
What medications has the infant received?
94 Patient-Related Problems

Aspirin, as well as other drugs, may lead to Reye syndrome.

Numerous other medications may result in seizures or metabolic
disturbances. Recent narcotic or benzodiazepine use raises the pos-
sibility of withdrawal symptoms.

Management
Further evaluation and management of an irritable infant depends
on the differential diagnosis that you develop after the quick look,
physical examination, and history and chart review. It may be
apparent at this point that the infant’s irritability is not a significant
change in status or can easily be attributed to the underlying reason
for admission. If this is the case, continuing the present manage-
ment with observation of the infant may be the most appropriate
next step. However, it needs to be emphasized that continued and
frequent observation is the key element of this approach and that
further evaluation may become necessary if the irritability either
changes in character or persists.
If the irritability of the infant is believed to be either “new” or
different in character, further evaluation is necessary and depends
on the suspected diagnoses. As mentioned earlier, if new or wors-
ening fever is present, especially in infants less than 2 months of
age, a complete evaluation for sepsis should be initiated. This
includes obtaining a blood culture, urine culture, and cerebrospinal
fluid (CSF) culture, followed by immediate administration of
empiric antibiotics. The choice of antibiotics depends on the
infant’s age, as well as special considerations, such as the presence
of a venticuloperitoneal shunt, recent surgery, or immunodefi-
ciency (see Chapter 18 for further discussion). If an intra-
abdominal process is suspected, the infant should be given nothing
orally, and radiographs and surgical consultation may be necessary
(see Chapter 6). If metabolic disturbances remain a possibility,
serum electrolyte, calcium, glucose, and ammonia measurements
are helpful, followed by appropriate correction of the disturbance
if present. Neurologic signs suggesting seizures or increased ICP
should be managed appropriately (see Chapter 7).
In some cases, no other identifiable clues may be present to help
guide further evaluation. In these circumstances, it may be best to
pursue additional studies and management, with the goal being to
exclude and empirically treat the major threats to life. Ask yourself
“What could be happening at the moment that is potentially life
threatening?” Remembering that sepsis is always a possibility,
blood, CSF, and urine cultures should be obtained and antibiotics
begun empirically in a young infant (<2 months) and in an older
infant who appears particularly ill. In an older infant, this decision
requires clinical judgment and potentially a discussion with super-
visors or consultants. Serum electrolyte, calcium, and glucose levels
 Crying and the Irritable Infant 95

should be determined if a recent result is unavailable. As empha-

sized previously, repeated evaluations of the infant are necessary
in this situation to ensure that the abdominal, cardiac, respiratory,
or neurologic status is not changing. Additional studies depend on
your findings as the process evolves.
Evaluation of an irritable infant requires a careful, thorough
approach and is therefore often a time-consuming and potentially
frustrating process. Remember that your goals while on call are to
(1) identify a cause and treat it when possible and (2) exclude or
empirically treat life-threatening illness. This should help you
prioritize and prevent you from becoming as “irritable” as the
patient.
 CHAPTER

13
Cyanosis
Rachel T. Sullivan, MD

Cyanosis is blue to purple discoloration of the skin and is typically a

clinical diagnosis made by visualization of the skin. Cyanosis is a
result of decreased arterial oxygen saturation of hemoglobin or
increased oxygen utilization in tissues. As with many problems
in pediatrics, appropriate evaluation of a patient with cyanosis
depends on the age of the child. The diagnoses you consider when
confronted with cyanosis in a neonate differ from those you con-
sider when evaluating an older child or adolescent. Regardless of
age, the key distinction should be made between peripheral cyano-
sis and central, or generalized, cyanosis.
Peripheral cyanosis is, by definition, present in the extremities,
with sparing of the central regions of the body. It is usually the result
of localized vascular changes that lead to poor perfusion and/or
venous stasis. Peripheral cyanosis may be secondary to vascular phe-
nomena (e.g., “physiologic” acrocyanosis of the newborn, Raynaud
phenomenon, sepsis), obstructive processes (superior vena cava
syndrome, deep venous thrombosis, tourniquets), or blood disorders
such as hypercoagulability (with subsequent thrombosis) and hyper-
viscosity (e.g., polycythemia). Typically, the presence of peripheral
cyanosis without generalization suggests that primary lung or heart
disease is not present.
In contrast, generalized cyanosis is more commonly associated
with primary heart disease or respiratory insufficiency but may also
occur if some of the processes that cause peripheral cyanosis are
severe enough (e.g., sepsis). Generalized cyanosis indicates that a
large amount of hemoglobin is in its reduced state (>5 g/dL) or
that oxygen saturation is less than approximately 85%. This chap-
ter focuses on generalized cyanosis because it is more common,
more likely to be life threatening, and more likely to require exten-
sive evaluation when on call.
The many causes of central cyanosis can be broadly divided into
two groups: (1) decreased oxygenation of hemoglobin (often as a
consequence of either respiratory insufficiency or cardiac disease)

96
 Cyanosis 97

and (2) abnormalities of hemoglobin (methemoglobinemia or

hemoglobin with reduced affinity for oxygen). The vast majority
of cases encountered in infants and children are a result of
decreased oxygenation because of lung or heart disease, but hemo-
globin abnormalities should always be considered. In a full-term
newborn, cardiac and pulmonary disease should be considered
equally, whereas in an older child, congenital heart disease is a less
likely consideration. This chapter is divided into two sections, with
evaluation of a newborn discussed separately from evaluation of an
older child.

Cyanosis in a Newborn

PHONE CALL
Questions
1. What are the vital signs?
2. Is the cyanosis central or peripheral?
3. Are there signs of respiratory distress (tachypnea, grunting,
nasal flaring, retracting)?
4. Are there signs of poor perfusion (delayed capillary refill, cold
extremities, weak pulses)?
5. How old is the child?
6. Is the infant alert and active and able to feed or lethargic and
refusing to feed?
The vital signs and differentiation between central or peripheral
cyanosis allow you to determine the urgency of the situation and
may also provide clues to the potential cause of the cyanosis.
Tachycardia, tachypnea, signs of respiratory distress, poor perfu-
sion, and/or hypotension may indicate sepsis, cardiogenic shock
associated with congenital heart disease, or severe respiratory
insufficiency. Fever or hypothermia may raise suspicion of septic
shock. The age of the infant may provide a clue to the cause.
Peripheral cyanosis in the early newborn period may be “physio-
logic” and of little concern in an otherwise well-appearing neonate.
Because the ductus arteriosus normally closes functionally by the
third day of life, the onset or worsening of cyanosis at this age
may indicate the presence of a congenital heart lesion that depends
on ductal flow to perfuse the lungs (i.e., obstructions to pulmonary
blood flow, such as with tricuspid atresia or pulmonary atresia) or
to provide oxygenated blood to the systemic circulation (e.g., trans-
position of the great arteries with an intact ventricular septum).
Congenital heart lesions with complete mixing of venous and arte-
rial blood may present with cyanosis and hypoxia in the early new-
born period without any significant associated respiratory distress.
98 Patient-Related Problems

Exacerbation of the signs of distress with feeding may suggest con-

gestive heart failure. This occurs with congenital heart lesions that
result in an increase in pulmonary blood flow (e.g., transposition of
the great arteries, truncus arteriosus, and total anomalous pulmo-
nary venous return) or with lesions associated with obstructed left
heart outflow (as seen with coarctation of the aorta, valvular aortic
stenosis, and hypoplastic left heart syndrome). Lethargy is expected
if central nervous system (CNS) depression and secondary respira-
tory insufficiency are occurring.
Orders
1. If possible, an arterial blood gas sample should be obtained
from a site distal to the ductus arteriosus (i.e., the left arm or
either leg) while the infant is breathing room air. The infant
should then be placed on 100% oxygen, and after at least
10 minutes, a second arterial blood gas sample should be
obtained. This test, the hyperoxia test, may help to distinguish
primary cardiac disease from lung disease and other potential
disorders. In cyanotic congenital heart disease, the partial pres-
sure of O2 (PaO2) is not expected to rise to a value greater than
150 mm Hg for mixing lesions or 100 mm Hg in the case of
severely restricted pulmonary blood flow (Table 13.1). Pulse
oximetry (oxygen saturation analysis) cannot be a substitute
for measurement of PaO2 because saturation reaches the max-
imum of 100% at approximately 90 mm Hg (see Appendix D,
The Oxyhemoglobin Dissociation Curve of Normal Blood).
Caution should be used in interpreting the hyperoxia test too
strictly. Exceptions to the generalization may occur, and there-
fore the results should always be interpreted in conjunction
with other clinical information.
2. A serum glucose (or Dextrostix) measurement, chest radio-
graph, and 12-lead electrocardiogram (ECG) should be ordered
stat. Additional laboratory measurements that should be

TABLE 13.1 Hyperoxia Test Interpretation

PaO2 in Room Aira PaO2 in 100% FiO2 (mm Hg)a

Healthy 70 >200
Lung disease 50 >150
Cyanotic Heart Disease
Decreased pulmonary blood flow <50 <100
Increased pulmonary blood flow 50 <150
Methemoglobinemia 70 >200
a
Values are approximations.
 Cyanosis 99

obtained include a complete blood count and serum electrolyte

determinations.
3. The infant should be placed on a cardiorespiratory monitor
with continuous pulse oximetry.
4. An intravenous (IV) line should be placed, and the infant
should be maintained without oral intake pending results of
the work-up outlined previously.
Inform RN
“I will be there as soon as possible.”
A newborn with cyanosis needs to be evaluated immediately.

ELEVATOR THOUGHTS
What is the differential diagnosis of cyanosis in a newborn?
Central cyanosis See Table 13.2
Peripheral cyanosis Physiologic acrocyanosis
Arterial thrombosis
Vasomotor instability

MAJOR THREAT TO LIFE

Cyanosis in a newborn is nearly always a threat to life, and all of the
conditions just listed and in Table 13.2 (with the exception of met-
hemoglobinemia and physiologic acrocyanosis) are potentially
fatal. In particular, cardiac conditions that depend on a patent duc-
tus arteriosus for pulmonary or systemic circulation may be fatal
within the first few days of life if appropriate interventions are
not undertaken in a timely manner.

BEDSIDE
Quick-Look Test
Is the infant alert, active, and breathing comfortably?
If so, you may proceed with slightly less urgency. An infant in
obvious distress may need urgent intervention, such as intubation,
blood pressure support, and/or initiation of a prostaglandin infu-
sion (see later).
Airway and Vital Signs
Severe compromise of the airway by congenital masses (e.g., goiter,
cavernous hemangioma, tumor) is an unusual cause of cyanosis
and is obvious on physical examination. Hypotension is the most
significant sign and suggests cardiogenic or septic shock (or rarely,
salt-wasting congenital adrenal hyperplasia). If hypotension is
 Patient-Related Problems

TABLE 13.2 Differential Diagnosis of Neonatal Cyanosis

Disease Mechanism
Pulmonary
Respiratory distress syndrome Surfactant deficiency
Sepsis, pneumonia Inflammation, pulmonary hypertension,
shunting R —> L
Meconium aspiration Mechanical obstruction, inflammation,
pneumonia pulmonary hypertension, shunting R —> L
Persistent fetal circulation Pulmonary hypertension, shunting R —> L
Diaphragmatic hernia Pulmonary hypoplasia, pulmonary
hypertension
Transient tachypnea Retained lung fluid
Cardiovascular
Cyanotic heart disease with R —> L shunt as in pulmonary atresia,
decreased pulmonary tetralogy of Fallot
blood flow
Cyanotic heart disease with R —> L shunt as in dtranspos' ' c
increased pulmonary arteriosus
blood flow
Cyanotic heart disease with
congestive heart failure

Heart failure alone

’- ock; high-output failure, as in

ductus arteriosus, vein of Galen,
.' other arteriovenous malformation
Central Nervous S
Hypoventilation, apnea
Asphyxia CNS depression
lntracranial w CNS depression, seizure
Phrenic nerve palsy, hypotonia,
hypoventilation, pulmonary hypoplasia

Acute blood loss Shock

Chronic blood loss Congestive heart failure
Polycythemia Pulmonary hypertension
Methemoglobinemia Low-affinity hemoglobin or red blood cell
enzyme defect
Metabolic
Hypoglycemia CNS depression, congestive heart failure
Adrenogenital syndrome Shock (salt losing)
CNS, Central nervous system; H —> L, right-to-Ieft intracardiac (foramen ovale), extracardiac
(ductus arteriosus), or intrapulmonary shunting.
From Behrman RE: Nelson Textbook of Pediatrics, 14th ed. Philadelphia, WB Saunders,
1992, p 464.
 Cyanosis 101

present, support of blood pressure should proceed while you are

pursuing your evaluation (see Chapter 25). In addition to standard
support with maintenance of intravascular volume and vasopressors
if necessary, consideration may need to be given to the use of pros-
taglandin E1 infusion if any ductal-dependent lesion (e.g., hypoplas-
tic left heart syndrome or critical coarctation of the aorta) is a
possibility (see “Management,” later). As noted earlier, tachypnea
and/or tachycardia can be expected in many cardiac and pulmonary
disorders. Weak respiratory effort may suggest CNS depression from
maternal drugs, birth asphyxia, or neuromuscular disorders.
Selective Physical Examination
When evaluating a cyanotic newborn, you should ask three
questions to help guide your further evaluation and treatment:
Are there signs of respiratory distress?
If none are present, primary lung disease is unlikely.
Are there signs of congestive heart failure and/or hypotension
and poor perfusion?
Congestive heart failure suggests congenital heart disease associ-
ated with increased pulmonary blood flow; the additional findings of
poor perfusion and/or hypotension suggest obstruction to left heart
outflow. Sepsis, myocarditis, supraventricular tachycardia, adrenal
insufficiency, or complete heart block may also produce these signs,
but the degree of cyanosis is not usually as great in these conditions.
Are there signs of CNS depression?
The infant’s mental status should be assessed immediately, with
simultaneous questioning regarding irritability, lethargy, and gen-
eral responsiveness. Any sign of a depressed level of consciousness
is concerning.
General Evaluation of a cyanotic newborn requires a fairly quick
but complete physical examination.
The presence of any dysmorphic features or
extracardiac congenital malformations, especially
midline defects (e.g., cleft lip or palate), increases the
likelihood of congenital heart disease.
HEENT Pupillary constriction (maternal narcotics); cyanosis of
the oral and mucous membranes, usually the most
easily recognized sites, implies generalized cyanosis;
nasal flaring (respiratory distress); midline defects
such as cleft lip or palate
Neck Masses (airway compromise), deviation of the trachea
(congenital heart disease, tension pneumothorax)
Chest Grunting, retractions (respiratory distress); abnormal
breath sounds (pneumonia, congestive heart failure)
HEENT, Head, Eyes, Ear, Nose, Throat.
102 Patient-Related Problems

Cardiac Hyperdynamic precordium, thrills, heaves

(ventricular hypertrophy, volume overload from
left-to-right shunting); point of maximum impulse
(situs inversus, cardiomegaly); silent precordium
(pericardial effusion, cardiomyopathy); murmurs
(congenital heart disease)
Lungs Breath sounds (pneumonia, respiratory distress
syndrome, meconium aspiration), absent breath
sounds (pulmonary hypoplasia, diaphragmatic
hernia, pneumothorax)
Abdomen Scaphoid (diaphragmatic hernia); location of the
liver, spleen (situs inversus)
Pulses Weak (coarctation, hypoplastic left heart, aortic
stenosis, sepsis), femoral and brachial pulses
unequal (coarctation)
Genitalia Ambiguous (congenital adrenal hyperplasia)
Neurologic Lethargy, hypotonia, lack of response to stimuli
(central nervous system depression from maternal
drugs, birth asphyxia)
Skin Cyanosis, petechiae, purpura (sepsis)

Selective History and Chart Review

Review the maternal history. Did the neonate’s mother have any ill-
nesses during pregnancy? What is the gestational age of the infant?
Did the mother receive antibiotics or narcotics during labor and
delivery? If so, why?
A maternal infection perinatally is a risk factor for neonatal sep-
sis. Maternal narcotics may be passed transplacentally and affect
the infant postnatally, especially if administered close to the time
of delivery. Maternal systemic lupus erythematosus is a risk factor
for congenital heart block in a neonate. The gestational age can give
you insight into how well developed the newborn’s lungs are. When
infants are born prematurely, they have a higher chance of incom-
plete lung development and subsequently higher risk of respiratory
distress as a result. Whether antenatal steroids were given can be
helpful as well because administration of steroids to a pregnant
mother who is expecting a preterm delivery can help to promote
lung development.
Was the delivery complicated? Was meconium present, and if so,
was it visualized below the vocal cords of the infant at the time of
delivery? What were the Apgar scores? What interventions were
required as part of the neonatal resuscitation?
Meconium aspiration, which may lead to pneumonia and/or
persistent fetal circulation, should be specifically investigated.
 Cyanosis 103

The Apgar scores may suggest birth asphyxia and subsequent CNS
impairment.
Is there a family history of congenital heart disease?
A previous family history increases the risk for disease in the
child being evaluated.
Management
By the time you have completed the quick-look test, check of the
airway and vital signs, and selective physical examination and his-
tory, it may remain unclear whether the newborn has a primary
respiratory problem, cardiac disease, or one of the other potential
causes of cyanosis. An algorithm for the further evaluation of a cya-
notic newborn is presented in Fig. 13.1. An abnormal hyperoxia
test result (failure to detect an appropriate rise in PaO2) effectively
excludes causes other than lung or heart disease, makes congenital
heart disease most likely, and allows you to concentrate on further
differentiating heart from lung disease. The chest radiographic
findings and ECG results can be extremely valuable tools to help
differentiate congenital heart disease from lung disease and also
to allow preliminary discrimination of the various forms of cya-
notic heart disease. The presence of a specific congenital heart
lesion can then be confirmed with echocardiography.
Abnormal Hyperoxia Test Result
Chest Radiograph
When interpreting the chest radiograph, several questions should
be asked.
Is a primary lung process responsible for the cyanosis?
Infiltrates and/or consolidation, suggesting pneumonia or
meconium aspiration, may be present. A diaphragmatic hernia
may not be obvious by physical examination and should be ruled
out by chest radiograph. If meconium aspiration, pneumonia, or a
diaphragmatic hernia is present, persistent fetal circulation (persis-
tent pulmonary hypertension) should be strongly suspected. This
condition can be detected by obtaining arterial blood gas samples
from both a preductal (right arm) and a postductal (left arm or
legs) vessel. A decrease in PaO2 in the postductal sample relative
to the preductal sample suggests right-to-left shunting across the
ductus as a result of pulmonary hypertension. If both determina-
tions are extremely low (<40 mm Hg) and the other clinical find-
ings suggest persistent fetal circulation, there may be significant
intracardiac right-to-left shunting across the foramen ovale as a
result of the same process.
If the lung fields do not reveal signs of inflammation, consoli-
dation, or diaphragmatic hernia, heart disease is more likely (if the
104 Patient-Related Problems

Cyanosis

Hyperoxia Test
(FIO2 = 1.00)

PaO2 < 150 PaO2 > 200 PaO2 > 150

Risks for PPHN*? Methemoglobin CXR

determination Cultures
Electrolytes
(serum, urine)
Yes No

Diagnoses
Pre- and post- CXR Pulmonary disease
ductal ABGs ECG Neurologic disease
CXR Echocardiogram Sepsis
Adrenal hyperplasia

Increased Decreased
pulmonary blood flow pulmonary blood flow

ECG ECG

LVH RVH LVH RVH

Truncus arteriosus Transposition Tricuspid atresia Tetralogy
Transposition/VSD Total anomalous Pulmonary atresia of Fallot
Single ventricle pulmonary
venous return
Hypoplastic left heart
syndrome

*PPHN, persistent pulmonary hypertension (also known as persistent fetal circulation)

ABG, arterial blood gases
CXR, chest radiograph
ECG, electrocardiography
LVH, left ventricular hypertrophy
RVH, right ventricular hypertrophy
VSD, ventricular septal defect

FIGURE 13.1 Approach to cyanosis in a newborn.

hyperoxia test result is abnormal), and one should then ask the next
question.
Are there increased pulmonary markings, suggesting increased
pulmonary blood flow, or is there a paucity of markings, suggesting
diminished pulmonary blood flow?
As shown in Fig. 13.1, increased pulmonary blood flow is asso-
ciated with a different set of congenital heart lesions than decreased
pulmonary blood flow is.
 Cyanosis 105

A B C
FIGURE 13.2 Abnormal cardiac silhouette. (A) “Boot-shaped”
heart seen in a cyanotic child with the tetralogy of Fallot or tricus-
pid atresia. (B) “Egg-shaped” heart seen in transposition of the
great arteries. (C) “Snowman” sign seen in total anomalous pul-
monary venous return (supracardiac type). (From Park MK: Pedi-
atric Cardiology for Practitioners, 2nd ed. Chicago, Year Book,
1988, p 54.)

What is the shape of the heart?

A few lesions may result in a characteristic cardiac silhouette
(Fig. 13.2).
Shape Defect
Boot Tetralogy of Fallot
Tricuspid atresia
Egg on a string Transposition of the great arteries
Snowman Total anomalous pulmonary venous return
Where is the aortic arch?
A right-sided aortic arch is associated with intracardiac defects
40% of the time.
Electrocardiogram
The most distinguishing features of the ECG in the various forms of
congenital heart disease are the presence and pattern of ventricular
hypertrophy (see Fig. 13.1).

Echocardiogram
Once congenital heart disease is suspected, you should consult with
a pediatric cardiologist and arrange for an echocardiogram to
confirm the diagnosis.
Normal Hyperoxia Test Result
If the hyperoxia test result is normal and the infant has no signs
that suggest heart or lung disease, other potential causes need to
be considered further. In this case, sepsis remains a possibility
and appropriate cultures should be obtained before starting
broad-spectrum antibiotic coverage for the most common neonatal
pathogens (see Chapter 18). CNS disease should be easily excluded
106 Patient-Related Problems

if there are no signs of CNS depression in the infant and the mater-
nal and delivery history is unremarkable. Hypoglycemia should
have been detected by Dextrostix testing and corrected appropri-
ately. The salt-wasting variant of congenital adrenal hyperplasia
in a female infant is suggested by the presence of abnormal geni-
talia. Because a male infant with this disorder may have normal
genitalia, the diagnosis depends on laboratory measurement of
serum and urine electrolytes. Methemoglobinemia should be sus-
pected if the cyanotic infant appears well, the infant has an other-
wise normal examination, and the infant’s blood appears
“chocolate brown” when exposed to room air.

Treatment
Further management depends on the diagnosis made after the eval-
uation just presented.

Cyanotic Heart Disease

If the clinical findings, arterial blood gas results, chest radio-
gram, and ECG findings are consistent with congenital heart dis-
ease, immediate consultation with a pediatric cardiologist is
necessary. Admission to an intensive care unit, where appropri-
ate monitoring can occur, is also necessary. If the infant has a
suspected lesion that is dependent on flow through the ductus
arteriosus, consideration should be given to initiation of a pros-
taglandin E1 infusion. Prostaglandin E1 vasodilates the ductus
arteriosus and improves pulmonary or systemic blood flow,
depending on the specific cardiac lesion that is present. The
infusion can be initiated at 0.05 to 0.1 μg/kg/min and increased
to 0.4 μg/kg/min if necessary to achieve therapeutic response
with increased oxygenation or increased systemic perfusion.
Once therapeutic response is achieved, reduce the rate to the
lowest dosage where clinical effectiveness is obtained. The rate
of infusion should be titrated according to the response in
PaO2 or the improvement in peripheral perfusion. The infant
should be monitored closely because apnea is a potential com-
plication. The infusion often adequately stabilizes the infant’s
condition until a full cardiac evaluation can be completed. Oxy-
gen should be administered and respiratory status and oxygen
saturations monitored closely.
Pneumonia or Respiratory Distress
Ventilatory support, antibiotics, selective pulmonary vasodilators,
and extracorporeal membrane oxygenation may all be required.
Admission to an intensive care unit and neonatology consultation
may be necessary depending on the level of respiratory support that
is required (see Chapter 28). If a diaphragmatic hernia is present,
 Cyanosis 107

intensive care monitoring and surgical consultation are also neces-

sary. Antibiotics are required as are cultures of blood and urine.
Sepsis
As mentioned earlier, appropriate cultures and broad-spectrum
antibiotic coverage are required.
Hypoglycemia
Hypoglycemia may be seen alone or in connection with one of the
other disorders causing cyanosis and should be appropriately cor-
rected (see Chapter 35).

Central Nervous System Disorders

Brain or spinal cord injury from birth trauma can be confirmed by
computed tomography (CT) or magnetic resonance imaging
(MRI). Hypoxic-ischemic injury (asphyxia) may also result in cere-
bral edema that can be detected by CT scanning. Care should be
supportive. Neurologic injury places these infants at higher risk
for seizures. If clinical concerns arise for the presence of seizures,
the infant may require monitoring with an electroencephalogram
(EEG).
Narcosis causing cyanosis, hypotonia, and slow, shallow respi-
rations is a result of heavy doses of opioids, meperidine, or barbi-
turates taken by or given to the mother shortly before delivery. It
can be treated with naloxone, 0.1 mg/kg, intravenously, intramus-
cularly, subcutaneously, or intratracheally. If the initial dose is
unsuccessful, repeated doses may be given at 2- to 3-minute
intervals.
Methemoglobinemia
Methemoglobin is the product of oxidation of hemoglobin to the
ferric state. It is present in healthy people, but the erythrocytic
reducing system maintains amounts at less than 2% of the total
hemoglobin content. Methemoglobinemia may be hereditary
(a deficiency of the reducing enzyme nicotinamide adenine dinu-
cleotide (NADH)_ cytochrome b5 reductase) or may occur second-
ary to exposure to a toxin (aniline dye, benzocaine, nitrites).
Methylene blue, 1 mg/kg intravenously, can be used to treat both
hereditary and toxin-related methemoglobinemia. To confirm the
diagnosis, methemoglobin levels in the blood can be measured.
Congenital Adrenal Hyperplasia
The salt-losing form of 21-hydroxylase deficiency causes viriliza-
tion, vomiting, dehydration, and potentially cyanosis in the new-
born period. As noted earlier, male infants may have normal
genitalia. Low serum sodium and chloride and high potassium
108 Patient-Related Problems

levels may be present. Plasma renin levels are elevated, as are serum
levels of 17-hydroxyprogesterone and urinary 17-ketosteroids.
Contrast imaging of the urogenital tract in virilized females may
be helpful when the genitalia are particularly ambiguous. Treat-
ment is aimed at replacing glucocorticoid and mineralocorticoid,
as well as sodium. A dehydrated infant needs volume and sodium
replacement initially (see Chapter 15, Diarrhea and Dehydration)
and then hydrocortisone (10–15 mg/m2/day orally in three divided
doses), fludrocortisone 0.05 to 0.3 mg daily in one or two divided
doses, and sodium chloride (1–3 g/day) as maintenance therapy.

Cyanosis in an Older Child

PHONE CALL
Questions
1. What are the vital signs?
2. Are there signs of respiratory distress?
3. Are there signs of altered mental status?
4. Is the cyanosis generalized or localized?
5. Why is the child hospitalized? Is there a history of lung or heart
disease?
Cyanosis in an older child is seen most often in the context of
lung disease, such as pneumonia, reactive airway disease, or an
exacerbation of cystic fibrosis. Changes in mental status may sug-
gest a neurologic cause for hypoventilation and secondary cyanosis.
Alternatively, mental status changes may be a result of hypoxia.
Remember, cyanotic older children frequently become acidotic
and symptomatic more quickly than newborns do because they
lack the adaptations that allow newborns to tolerate lower levels
of oxygen, such as fetal hemoglobin, higher hematocrit, and
increased levels of 2,3-diphosphoglycerate (2,3-DPG).
Orders
1. An arterial blood gas determination should be done as soon as
possible. Oxygen should be administered and the patient placed
on continuous cardiorespiratory monitoring and pulse oximetry.
2. An IV line should be placed if not already present.
3. A complete blood count and serum glucose (or Dextrostix)
measurement should be obtained and extra blood drawn into
a red-top tube and saved (if toxicology screening studies or
serum chemistry studies are thought to be necessary after your
further evaluation).
4. A stat chest radiograph and ECG should be obtained.
5. If the child is febrile or there are significant infectious concerns,
appropriate cultures should be obtained.
 Cyanosis 109

Inform RN
“I will be there right away”
Cyanotic patients should be evaluated immediately.

ELEVATOR THOUGHTS
What are the causes of cyanosis beyond the newborn period?
Pulmonary Pneumonia
Reactive airway disease
Foreign body
Cystic fibrosis
Pneumothorax
Pulmonary hemorrhage
Bronchiectasis
Pulmonary embolism
Aspiration
Primary pulmonary hypertension
Cardiac Congenital heart disease
Myocarditis
Tetralogy of Fallot “spells”
Dysrhythmia
Cardiomyopathy
Neurologic Encephalopathy
Encephalitis
Toxins
Metabolic disease
Neuromuscular disease
Seizure
Hematologic Polycythemia
Hypercoagulable state
Methemoglobinemia
Peripheral or localized Arterial thrombosis
Raynaud phenomenon
Compartment syndrome (traumatic)
Superior vena cava syndrome
Septic shock with poor peripheral perfusion
Infectious Sepsis

MAJOR THREAT TO LIFE

Cyanosis in an older child nearly always indicates a critical under-
lying process. With the exception of Raynaud phenomenon
and methemoglobinemia, the other possibilities are all life
threatening.
110 Patient-Related Problems

BEDSIDE
Quick-Look Test
A cyanotic older child commonly has other signs of respiratory dis-
tress or may also appear lethargic or obtunded if neurologic disease
is present or the hypoxia is severe and prolonged. The paroxysmal
hypercyanotic episodes experienced by young children with the
tetralogy of Fallot (“tet spells”) may result in syncope after a short
period of respiratory distress.
Airway and Vital Signs
Because most older children with cyanosis have lung disease, par-
ticular attention should focus on the airway and the presence of
tachypnea. If there is any compromise, intubation may be required.
Bradycardia and hypotension are ominous signs in a cyanotic
child. Shallow, slow respirations suggest neurologic dysfunction
and hypoventilation.

Selective Physical Examination

HEENT Edema, proptosis, localized cyanosis (superior vena
cava syndrome); pupillary constriction or dilation
(narcosis or other ingestion); ptosis
(neuromuscular disease); nasal flaring (respiratory
distress)
Neck Distended neck veins (congestive heart failure,
superior vena cava syndrome), deviation of the
trachea (tension pneumothorax), use of accessory
muscles
Lungs Breath sounds, grunting, retracting (respiratory
distress, congestive heart failure, pneumothorax,
foreign body); stridor; wheezing; weak respiratory
effort (neuromuscular disease, central nervous
system disease)
Heart Rate and rhythm (dysrhythmias), single loud S2
sound (pulmonary hypertension), murmurs
(a temporary decrease in the intensity of a systolic
murmur is associated with hypercyanotic spells in
the tetralogy of Fallot), S3 or S4 sound (heart
failure)
Extremities Weak pulses (myocarditis or cardiomyopathy and
congestive heart failure, arterial thrombosis,
compartment syndrome, sepsis), edema
(congestive heart failure), local cyanosis
(thrombosis, vasospasm, compartment
syndrome), clubbing (chronic lung or cyanotic
 Cyanosis 111

heart disease), transient pallor of the digits

changing to cyanosis and then hyperemia
(Raynaud phenomenon), muscle tenderness
(compartment syndrome)
Neurologic Weakness, decreased reflexes (neuromuscular
disease); altered mental status (encephalitis, toxin)

Selective History and Chart Review

Has the cyanosis been chronic or recurrent, or is this an acute
episode? Is there a history of chronic heart or lung disease?
Mild cyanotic heart disease may go undetected for years; there-
fore a history of previous cyanosis may be helpful. The same is true
of chronic lung disease, such as cystic fibrosis or primary pulmo-
nary hypertension. Suspicion of a “tet spell” is obviously high in
those with a known history. These spells are characterized by rapid
and deep respiration pattern, irritability, cyanosis, and decreased
intensity of cardiac murmur. Cyanotic heart disease may be asso-
ciated with polycythemia. Pneumothorax may occur in those with
known lung disease, such as reactive airway disease or cystic
fibrosis.
In a patient with respiratory distress, is the history suggestive of
foreign body or chemical aspiration?
This should be considered, especially in a toddler.
Does the patient have any other known chronic medical condi-
tions, in particular chronic CNS disease (seizure disorder, cerebral
palsy, severe developmental delays, etc.)? How is the patient fed,
orally or via tube? Is there any association of the onset of symptoms
with timing of an episode of emesis, a choking event, or an oral feed?
Patients with known CNS disorder and developmental delays
are at higher risk for aspiration. History and chest radiograph find-
ings can be used in combination to determine the likelihood of
aspiration as the etiology of cyanosis.
Has the patient had fever, upper respiratory symptoms, or “flu”
symptoms?
This raises suspicion of lung infection, sepsis, or myocarditis. In
a child with neurologic findings, this would lead more toward a
diagnosis of encephalitis or postinfectious Guillain-Barre syn-
drome and secondary respiratory insufficiency.
What medications has the patient received? Has there been expo-
sure to dyes or chemicals?
One should specifically look for narcotics, barbiturates, or sub-
stances known to produce methemoglobinemia.
Is the patient predisposed to a hypercoagulable state?
This may cause pulmonary embolism or arterial thrombosis.
Oral contraceptive use, antiphospholipid antibodies, and immobi-
lization are risk factors for hypercoagulability.
112 Patient-Related Problems

If cyanosis is localized to the distal end of an extremity and is

associated with pain, is the patient at risk for compartment
syndrome?
Trauma (e.g., fractures) or excessive exercise and muscle swell-
ing can precipitate compartment syndrome.
Management
In nearly all cases of cyanosis in an older child, a respiratory, car-
diac, or neurologic cause is readily apparent, and management
should proceed as indicated by the underlying disorder (see Chap-
ters 7, 10, 22, and 28). Management of a few selected processes that
are associated with hypercyanosis and are not discussed elsewhere
is presented here.
Tetralogy of Fallot
Placement of the child in the knee-chest position compresses the
femoral arteries and increases systemic vascular resistance, thereby
decreasing right-to-left shunting across the ventricular septal
defect and improving pulmonary blood flow. Oxygen should be
administered and the child calmed as much as possible. Morphine,
0.05 mg/kg to a maximum of 0.2 mg/kg subcutaneously or intra-
muscularly, may aid in relaxing a young child. If these measures
are unsuccessful or if the attack is particularly severe, administra-
tion of IV sodium bicarbonate should be considered to correct the
metabolic acidosis that quickly develops as the PaO2 is maintained
below 40 mm Hg. IV propranolol (0.1–0.2 mg/kg) may also be
considered to alleviate the tachycardia that often accompanies
episodes and may impede adequate pulmonary blood flow. IV
ketamine (1–2 mg/kg IV or 3–5 mg/kg IM) may help by increasing
systemic vascular resistance and providing beneficial sedating
effects to the infant.
Polycythemia
If polycythemia is severe enough (hematocrit of 65% to 70%) and
associated with hyperviscosity, plethora, and/or cyanosis, consider-
ation should be given to phlebotomy and replacement of whole
blood with plasma, 5% albumin, or normal saline solution. The
volume replaced can be calculated as follows:
Volume ðmLÞ ¼
Estimated blood volume ðmLÞ  Desired hematocrit change
Starting hematocrit
Arterial Thrombosis
If there is concern for thrombosis, ultrasound study should be
obtained to aid in diagnosis. Heparinization may relieve the clot
 Cyanosis 113

and prevent further thrombosis in those with a hypercoagulable

state. An initial IV bolus of 50 U/kg should be followed by a main-
tenance infusion of 10 to 25 U/kg/h. The partial thromboplastin
time should be maintained at 1.5 to 2.5 times normal.

Raynaud Phenomenon
An episodic, triphasic (white-blue-red) color change of the digits,
usually bilateral and symmetrical, associated with cold exposure or
anxiety should suggest vasospasm. Most cases can be managed by
simple rewarming. Refractory cases can be treated with nifedipine,
10 mg 1 to 3 times a day.

Compartment Syndrome
Immediate surgical consultation is required because fasciotomy is
necessary to relieve pressure and restore adequate blood flow.

Summary
Cyanosis is a challenging problem and usually represents a true
emergency. A stepwise approach as outlined here that combines
a selective physical examination and a few laboratory and radio-
graphic studies usually allows you to determine a cause in an effi-
cient manner and begin appropriate treatment. It should be clear
that by thinking logically about the problem, you can take the
actions necessary to ensure that neither you nor the patient is
“blue.”
 CHAPTER

14
Delivery Room
Problems
Kathryn M. Rubey, MD

A call requesting the presence of a pediatrician at the delivery of a

newborn may be received for several reasons. The call may be “rou-
tine” (e.g., request for a pediatrician to be present for cesarean sec-
tion or instrument-assisted delivery), for premature delivery,
multiple births, or because fetal monitoring during labor reveals
fetal distress. For any type of call, you should always be prepared
to resuscitate the newborn and to manage several other potential
problems discussed in this chapter.

PHONE CALL
Questions
1. What is the expected gestational age?
2. Is the amniotic fluid clear?
3. How many babies are expected?
4. Are there any additional risk factors (e.g., no prenatal care,
maternal infection, maternal substance abuse)?
The younger the gestational age, the more likely the presence of
immature lungs in the newborn and subsequent respiratory dis-
tress at birth. Fetal tachycardia (>160 beats per minute [bpm])
or bradycardia (<120 bpm) or a fetal scalp blood pH less than
7.25 suggests fetal distress. Tachycardia may be seen with fetal hyp-
oxia, maternal fever, or anemia. Bradycardia may be seen with hyp-
oxia, inadvertent administration of anesthetic to the fetus, or
congenital heart block. Understanding the relationship of brady-
cardia to uterine contractions (decelerations) may be helpful
(Fig. 14.1). Late decelerations reflect fetal hypoxia from lack of suf-
ficient uteroplacental blood flow, whereas early and variable decel-
erations are less worrisome. Hypoxia results in acidosis and a fall in
fetal scalp blood pH. A value less than 7.20 indicates significant

114
 Delivery Room Problems 115

FIGURE 14.1 Three types of decelerations. (A) Early decelera-

tions reflect head compression with contractions of the uterus.
(B) Late decelerations occur when there is uteroplacental insuffi-
ciency as a result of compression of the blood supply to the pla-
centa during uterine contraction. (C) Variable decelerations
occur with umbilical cord compression during uterine contraction.
FHR, Fetal heart rate; UC, uterine contraction.

distress and is generally a reason for immediate early delivery. Pas-

sage of meconium into amniotic fluid may be a sign of fetal distress
and presents an added risk to the infant because this meconium
may be aspirated into the lungs at delivery and result in obstruction
of small airways and perhaps secondary complications (persistent
fetal circulation, pneumonia).
Several maternal illnesses may potentially complicate delivery.
Maternal infections may place the newborn at risk for sepsis, dia-
betes may result in macrosomia and a difficult delivery, and
116 Patient-Related Problems

maternal systemic lupus erythematosus may give rise to congenital

heart block.
Orders
You should ask the delivery room nurse to make sure that the
infant warming table is prepared in the event that resuscitation
is necessary. A laryngoscope, laryngoscope blades, several sizes
of endotracheal tube (sizes 2.5 to 4.5), an umbilical catheterization
tray, intravenous (IV) angiocatheters, butterfly needles, and syrin-
ges, in addition to oxygen and wall suction, should be present. Nal-
oxone as well as epinephrine, atropine, sodium bicarbonate,
surfactant, and dopamine should be readily available.

Inform RN
“I will be there right away.”
You need to go directly to the delivery room. It is optimal to
arrive well before the delivery so that you have time to briefly
review the mother’s chart.

ELEVATOR THOUGHTS
What possibilities do I need to anticipate?
Respiratory failure Hyaline membrane disease
(in a premature infant)
Secondary to birth asphyxia
or CNS depression
Meconium aspiration
Sepsis
Choanal atresia
Diaphragmatic hernia
Mandibular hypoplasia
Pneumothorax
Cystic adenomatoid
malformation
Phrenic nerve paralysis
Pulmonary hypoplasia
Severe anemia (and
secondary hydrops fetalis)
Plethora (polycythemia)
Seizures
Congenital malformations
(e.g., cleft lip or palate)
Birth injury
Shock

CNS, Central nervous system.

 Delivery Room Problems 117

MAJOR THREAT TO LIFE

All of the aforementioned are major threats.

BEDSIDE
Quick-Look Test
You should look to see whether delivery is imminent. If not, you
should find and review the maternal history.
Selective Maternal History
Does the mother have any medical illnesses?
Specifically, diabetes, hypertension, systemic lupus erythemato-
sus, chronic renal disease, chronic lung or heart disease, and sickle
cell anemia may affect the developing fetus and potentially predis-
pose to problems at birth.
Does the mother use narcotics, alcohol, cocaine, tobacco, or other
drugs? Has the mother received narcotics during labor?
All may affect the fetus and newborn infant. Narcotics admin-
istered within 1 hour of delivery may result in significant central
nervous system (CNS) depression in the newborn.
Is there a history of previous high-risk pregnancies?
Such a history may increase the risk associated with the current
pregnancy.
Has the mother had any infections during pregnancy? Does she
have a current infection? Is she receiving or has she received antibi-
otics? Has a cervical culture for group B streptococci been performed,
and if so, what are the results?
Rubella, human parvovirus B19, human immunodeficiency
virus, cytomegalovirus, toxoplasmosis, herpes simplex, syphilis,
tuberculosis, varicella, and hepatitis C may all be transmitted trans-
placentally and affect the newborn if present during pregnancy.
Group B streptococci, Escherichia coli, hepatitis B, and herpes sim-
plex may be acquired perinatally.
Have the maternal membranes ruptured, and for how long?
What is the maternal white blood cell count?
The risk for neonatal infection secondary to ascension from
the cervix and genital tract is greater if the membranes have been
ruptured for 18 hours or more and/or maternal leukocytosis is
present.
What is the mother’s blood type and Rh status?
ABO or Rh incompatibility may result in hemolytic anemia in
the newborn.
Has polyhydramnios or oligohydramnios been present?
118 Patient-Related Problems

Polyhydramnios is associated with anencephaly, hydroceph-

aly, bowel atresia, tracheoesophageal fistula, cleft lip or palate,
cystic adenomatoid malformation, and diaphragmatic hernia.
Oligohydramnios is associated with pulmonary hypoplasia, renal
agenesis, growth retardation, twin-twin transfusion, and fetal
anomalies.
Has amniocentesis been performed?
Chromosome abnormalities, neural tube defects (elevated
α-fetoprotein level), and the degree of fetal lung maturity
(a lecithin-sphingomyelin ratio >2:1 generally indicates maturity)
may all be determined with amniocentesis.
Has a nonstress test, a contraction stress test, or a biophysical
profile been performed?
Abnormal results of such testing may indicate fetal hypoxia.
Interpretation of the nonstress test and contraction stress test
may be difficult because of high false-positive rates with these tests.
Fetal Vital Signs
As noted earlier, tachycardia or bradycardia may indicate fetal dis-
tress. The pattern of decelerations and the beat-to-beat variability
of the fetal heart rate should also be observed. Decreased beat-to-
beat variability reflects fetal distress. Maternal fever may indicate
infection, in which case the newborn is also at risk.

Management I
Three questions to determine if a newborn needs initial steps at the
radiant warmer:
1. Is the newborn term?
2. Is the newborn breathing or crying?
3. Does the newborn have good muscle tone?
At the majority of deliveries, the newborn is crying and vigor-
ous once removed from the perineum; and therefore does not
require any immediate intervention other than drying and warm-
ing (skin-to-skin contact with mother is recommended), but they
may need a period of observation (e.g., if there is prematurity, con-
genital malformation, or any question of meconium aspiration or
sepsis). All infants whose delivery you attend should have an Apgar
score assigned (see later) and should be examined thoroughly.
If there are meconium-stained fluids, but the infant is vigorous,
the child may stay with the mother and requires only bulb suction-
ing of the mouth and nose.
Newborns who are not breathing, are not vigorous, or are in dis-
tress need intervention. The key is to be an astute observer of the
transition period to judge which infants need help and which
do not.
 Delivery Room Problems 119

Respiratory Failure
If the infant is received from the obstetrician and is not crying, is
cyanotic, is limp, and has not initiated respirations, initial efforts
should be directed toward suctioning the mouth then nose and vig-
orously stimulating the infant in an effort to provoke respiration.
Of note, the seventh edition Neonatal Resuscitation Program
(NRP) recommendations for nonvigorous newborns (depressed res-
pirations or poor muscle tone) with meconium-stained fluids are to
follow these initial steps of newborn care. Stimulation is best accom-
plished by rubbing the back of the torso while the infant is in a supine
position. Throughout this time, another person should be monitor-
ing the newborn’s pulse by auscultating the left side of the chest.
Although pulsations may be felt by holding the umbilical stump with
the thumb and forefinger, this is less accurate and may underesti-
mate the true heart rate. This person can help your evaluation by
tapping out the heart rate on the surface of the warming table. Esti-
mate the heart rate by counting the number of beats for 6 seconds
and then multiplying by 10. If the child is not vigorous, a pulse oxim-
etry sensor (placed on the right hand or wrist) and/or electronic car-
diac (ECG) monitor leads should be connected to evaluate the heart
rate. Continuous positive airway pressure (CPAP) may be used for
infants who are breathing with a heart rate of at least 100 bpm but
with labored respirations or oxygen saturation below target. If the
infant does not respond with a spontaneous breath, improved color,
and improved muscle tone within several seconds or if the heart rate
is less than 100 bpm, positive pressure ventilation (PPV) with bag
and mask should be started at a rate of 40 to 60 breaths/min with
flowmeter set to 10 L and 21% oxygen (start between 21% and
30% for infants <35 weeks’ gestation) should be administered while
you continue to stimulate. Oxygen concentration should be titrated
to goal oxygen saturation (see Fig. 14.2 for saturation goal by age in
minutes). If you are alone, you should call for help when beginning
PPV. Heart rate should be reassessed after 15 seconds. If heart rate is
rising, continue PPV and recheck heart rate in 15 seconds. If heart rate
is not increasing, assess ventilation. If chest rise is noted, continue
PPV and recheck heart rate in 15 seconds. Address ventilation with-
out chest movement with corrective steps (known as “MR. SOPA;”
Table 14.1), and reassess after 30 seconds of ventilation with chest
movement. If heart rate remains less than 60 bpm after 30 seconds
of PPV that moves the chest, intubation with endotracheal ventilation
is indicated. Providing 30 seconds of ventilation is strongly recom-
mended; and then if heart rate remains less than 60 bpm, oxygen
concentration should be increased to 100% and chest compressions
begun. If the infant’s mother received a narcotic during labor, they
should be managed as above. There is insufficient evidence for the
120 Patient-Related Problems

Antenatal counseling,
team briefing, and equipment check.

Birth

Stay with mother for routine care:

Term? Tone? Warm and maintain normal
Yes temperature, position airway,
Breathing or
crying? clear secretions if needed, dry,
ongoing evaluation.
No
Warm and maintain normal
1 temperature, position airway, clear
minute secretions if needed, dry, stimulate.

Labored breathing
Apnea, gasping, or No
or
HR below 100
persistent
bpm?
cyanosis?
Yes
Yes
PPV, Position and clear airway,
SpO2 monitor, SpO2 monitor,
Consider ECG monitor. Supplemental O2 as needed,
Consider CPAP.

No Post-resuscitation care,
HR below 100
bpm? team debriefing.

Yes
Check chest movement,
Ventilation corrective steps if
needed, Pre-ductal SpO2 Target
ETT or laryngeal mask if needed. 1 min 60%–65%
2 min 65%–70%
3 min 70%–75%
No
HR below 50 bpm? 4 min 75%–80%
5 min 80%–85%
Yes 10 min 85%–95%
Intubate if not already done,
chest compressions,
coordinate with PPV,
100% O2,
ECG monitor.

HR below 60 bpm?

Yes

IV epinephrine.
If HR persistently below 60 bpm;
consider hypovolemia,
consider pneumothorax.

FIGURE 14.2 Seventh edition Neonatal Resuscitation Program

flow diagram. CPAP, Continuous positive airway pressure; ECG,
electrocardiogram; ETT, endotracheal tube; HR, heart rate; IV,
intravenous; PPV, positive pressure ventilation.
 Delivery Room Problems 121

TABLE 14.1 The Six Ventilation Corrective Steps: MR. SOPA

Corrective
Steps Actions
M Mask adjustment Reapply the mask. Consider the two-hand
technique.
R Reposition Place head neutral or slightly extended.
airway
Try PPV and reassess chest movement.
S Suction mouth Use a bulb syringe or suction catheter.
and nose
O Open mouth Open the mouth and lift the jaw forward.
Try PPV and reassess chest movement.
P Pressure increase Increase pressure in 5-10 cm H2O increments,
maximum 40 cm H2O.
Try PPV and reassess chest movement.
A Alternative Place an endotracheal tube or laryngeal mask.
airway
Try PPV and assess chest movement and breath sounds.
PPV, Positive pressure ventilation.

safety and efficacy of using naloxone, the narcotic antagonist, in the

delivery room with the neonatal population. At this point, you need to
call for more “hands” because an umbilical venous catheter or periph-
eral IV line should be placed and samples drawn for arterial blood gas
measurement. Fluid volume, epinephrine, sodium bicarbonate, and
vasopressors may all be necessary to resuscitate the infant. Remember,
pulmonary hypoplasia, diaphragmatic hernia, or pneumothorax may
interfere with adequate ventilation.
If the infant begins to breathe spontaneously and does not
require immediate intubation, you should carefully observe the
pattern of respiration and look for signs of respiratory distress.
Labored breathing, intercostal and subcostal retractions, and
tachypnea are worrisome signs and can quickly evolve in a new-
born who is initially breathing comfortably. These signs may indi-
cate any of the problems associated with respiratory failure in the
newborn, and you should then proceed with a physical examina-
tion to determine the probable cause.
Shock
Internal hemorrhage from birth trauma, fetomaternal transfusion,
placental abruption, hemolytic anemia, or umbilical cord trauma
may result in shock in the newborn. Pallor, poor perfusion, cyano-
sis, respiratory distress, and cold extremities may all be present at
birth. Resuscitation should begin immediately by maintaining the
122 Patient-Related Problems

airway and respirations (see earlier) and supporting the circulation.

Hypovolemia can be corrected with normal saline solution, plasma,
or type O-negative blood after placement of a peripheral IV line or,
ideally, umbilical catheters (both arterial and venous). Vasopres-
sors may be necessary to support the blood pressure. Once the
infant is stabilized, further evaluation, with a complete physical
examination and laboratory studies, can proceed.
Physical Examination
HEENT Swelling (caput succedaneum, cephalohematoma),
indentations (skull fracture), subconjunctival
hemorrhages (often considered a “normal” event
with delivery), colobomas, ear anomalies,
encephaloceles (may obstruct the nose or airway),
cataracts, dysmorphic facies
Neck Masses (goiter, cystic hygroma), tracheal position
Chest Symmetry and adequacy of chest rise; equal breath
sounds; rales; rhonchi; grunting, flaring, retracting
(respiratory distress)
Heart Location of heart sounds (right-sided sounds may
indicate congenital heart disease or a shift
secondary to diaphragmatic hernia, tension
pneumothorax, or a lung mass), murmurs
(congenital heart disease), bradycardia (congenital
heart block, hypoxia)
Abdomen Scaphoid, flat, distended (scaphoid suggests
diaphragmatic hernia); liver and spleen position
(situs inversus may be associated with cyanotic
heart disease); umbilical cord inspection for two
arteries and one vein (lack of a vessel may be
associated with other congenital anomalies,
including cardiac defects)
Genitalia Testes palpable and descended in a male, external
genitalia normal in a female
Extremities Tone (generalized decrease associated with asphyxia
or hypoxia, poor perfusion, or neuromuscular
disorder), movement (nerve palsy secondary to
birth injury), presence of all digits, color (cyanosis,
plethora), perfusion
Neurologic Alert, active, moving all extremities, responsive to
stimuli

HEENT, Head, Eyes, Ears, Nose, Throat.

An Apgar score should be assigned at 1 and 5 minutes of life for

all infants (Table 14.2). In those requiring resuscitation, 10-, 15-,
 Delivery Room Problems 123

TABLE 14.2 Apgar Evaluation of a Newborn Infant

Sign 0 1 2
Heart rate Absent Less than 100 bpm Over 100 bpm
Respiratory Absent Slow, irregular Good cry
effort
Muscle tone Limp Some flexion Active motion
Reflex No response Grimace Cry
irritability
Color Pale Body pink, extremities All pink
blue

Sixty seconds after complete birth of the infant (disregarding the cord and placenta), the five
objective signs above are evaluated, and each is given a score of 0, 1, or 2. A total score of 10
indicates an infant in the best possible condition. An infant with a score of 0 to 3 requires
immediate resuscitation.
Modified from Apgar V: A proposal for a new method of evaluation of the newborn infant. Curr
Res Anesth Analg 32:260-267, 1953.

and 20-minute scores may also be necessary. The first Apgar score
indicates the need for resuscitation, and later scores are more
indicative of the potential for morbidity and mortality.

Management II
Severe Anemia
If the infant is born with pallor and shock and appears to be in dis-
tress, acute blood loss from a perinatal problem (abruption of the
placenta, placenta previa, internal hemorrhage) is more likely than
chronic intrauterine anemia. Chronic intrauterine anemia is more
likely to produce fetal hydrops and signs of congestive heart failure
at birth. In contrast to acute blood loss, laboratory measurements
of hemoglobin, the reticulocyte count, and mean cell volume are
often abnormal. A newborn infant who is symptomatic and anemic
is likely to require a transfusion. Additional evaluation of a new-
born with anemia is outlined in Fig. 14.3 and should include a com-
plete blood count, reticulocyte count, Coombs test, peripheral
smear review, and a determination of infant and maternal
blood types.
Plethora (Polycythemia)
Delayed umbilical cord clamping, twin-twin transfusion, maternal
diabetes, and chromosomal abnormalities are a few of the condi-
tions that may result in polycythemia in the newborn, which is
defined as a central hematocrit greater than 65%. Many newborns
124 Patient-Related Problems

FIGURE 14.3 Algorithm for the evaluation of a newborn with

anemia. DIC, Disseminated intravascular coagulation; G6PD,
glucose-6-phosphate dehydrogenase; MCV, mean cell volume.

are asymptomatic, but respiratory distress, cyanosis, lethargy, and

seizures may all occur. An infant who appears ruddy or reddish
purple should be suspected of having polycythemia and should
have a hematocrit determination immediately. If polycythemia is
present, a partial exchange transfusion should be performed with
the aim of reducing the hematocrit to 50%. The volume of the
exchange is calculated as follows:
Volume ðmLÞ ¼
Estimated blood volume ðmLÞ  Desired hematocrit change
Starting hematocrit

Seizures
Seizures in the delivery room are most likely secondary to asphyxia
and hypoxic or ischemic injury to the CNS. Other possibilities
include a structural anomaly of the brain, intracranial bleeding,
electrolyte disturbance, hypoglycemia, drug withdrawal, and men-
ingitis or sepsis. A point of care blood glucose, hematocrit, arterial
blood gas analysis, and serum electrolyte measurements should be
 Delivery Room Problems 125

obtained, oxygen administered, and the neonate treated with anti-

convulsants (see Chapter 29). Blood cultures, a lumbar puncture,
and administration of antibiotics to empirically treat potential
causes of meningitis in the neonatal period are also necessary.

Congenital Malformations
Any malformations of the infant noted should be discussed imme-
diately with one or, ideally, both of the parents. If you are unsure of
the presence of an anomaly, you should state this to the parents and
let them know that you will be discussing it with other physicians
(senior residents, neonatologists, the infant’s pediatrician) who will
be available to examine the infant. If you are unsure of the signif-
icance of an anomaly, you should also say so to the parents and
again let them know that other physicians will be available to them
to fully discuss the particular anomaly and its implications. As the
first physician to evaluate their child, the parents will be looking to
you for an initial confirmation that all is “perfect” with their new-
born. It is imperative that any potential problem be discussed with
them immediately. If this is not done, their initial sense of reassur-
ance will be crushed by bad news sometime in the next few days.
Many trips to the delivery room end with your handing over a
healthy, swaddled infant to a mother or father, with little or no
intervention required on your part. If there has been a problem,
you should remember to phone and inform the infant’s
pediatrician-to-be (if one has been identified) of the problem.
 CHAPTER

15
Diarrhea and
Dehydration
Karlo Kovacic, MD

Diarrhea is a common problem in the pediatric population. It is

generally defined as multiple watery stools in a day. Although usu-
ally the result of a gastrointestinal infection, and usually mild and
self-limited, it is important to recognize that diarrhea may have a
number of potential causes and that the potential complications of
diarrhea will vary according to the volume, frequency, and content
of stool, as well as the duration of the symptoms and the presence
of associated comorbidities.
When asked to evaluate a child with diarrhea, one needs to be
aware of the potential for dehydration and anemia (if there is also
blood in the stool). Significant anemia, hypovolemia, and electro-
lyte abnormalities can lead to increased morbidity or even mortal-
ity. When called regarding a child with bloody or nonbloody
diarrhea, your priorities should be to (1) quickly assess the overall
volume status of the child and the need for fluid resuscitation and/
or a blood transfusion, (2) appropriately correct volume deficits,
(3) appropriately correct electrolyte abnormalities, and (4) identify
the most likely cause of the diarrhea. This chapter discusses
diarrhea, as well as the general fluid management of hospitalized
children. Specific electrolyte abnormalities are discussed in detail
in Chapter 34 (Electrolyte Abnormalities).

PHONE CALL
Questions
1. Clarify whether diarrhea is present. Both increased frequency
and increased water content define diarrhea.
2. Is there blood in the stool?
3. What are the child’s vital signs?

126
 Diarrhea and Dehydration 127

4. How old is the child?

5. How long has the child had diarrhea?
6. Is there mucus or pus in the stool?
7. Why is the child in the hospital?
Hypotension implies significant hypovolemia and requires
urgent attention and intervention (see Chapter 25). The infant
may be able to maintain normal blood pressure in the face of
significant hypovolemia; tachycardia may be the only finding
initially. The duration of diarrhea may allow you to estimate
the risk for dehydration. Blood or mucus in the stool and the
reason for admission may provide clues to the cause of the
diarrhea.

Orders
1. If hypovolemia is a concern, the patient should have an intra-
venous (IV) line placed.
• If the patient is hypotensive, an immediate 10- to 20-mL/kg
bolus of normal saline or lactated Ringer’s solution should
be ordered (see Chapter 25).
2. Labs: electrolytes, blood urea nitrogen (BUN), creatinine,
venous pH, complete blood count with differential, and
urinalysis.
• The serum sodium concentration and hemoglobin are nec-
essary for you to plan appropriate rehydration and/or blood
transfusion if needed.
3. The patient should not receive any oral intake until your further
evaluation.

Inform RN
“I will arrive at the bedside in … minutes.” A child with abnormal
vital signs or bloody diarrhea should be evaluated immediately.

ELEVATOR THOUGHTS
What causes diarrhea?
Mild diarrhea without dehydration is a nonspecific finding that
may be present with nearly any illness in childhood. Diarrhea as a
manifestation of a pathologic condition of the gastrointestinal tract
is usually more severe and has greater potential to lead to
dehydration.
Infections
Diarrhea in children may result from infection of the gastrointes-
tinal tract itself (gastroenteritis, colitis) or from acute infections
128 Patient-Related Problems

elsewhere, such as an upper respiratory infection, pneumonia,

hepatitis, or a urinary tract infection.
Viral gastroenteritis Rotavirus
Norwalk virus
Adenovirus
Influenza
Enteroviruses
Bacterial colitis Salmonella, Shigella, Yersinia, or
Campylobacter (“SSYC”)
Enteropathogenic Escherichia coli
Staphylococcal food poisoning
Clostridium difficile (pseudomembranous
colitis)
Vibrio cholerae
Mycobacterium tuberculosis
Parasitic infection Giardiasis
Amebiasis
Cryptosporidiosis
Worms (strongyloidiasis, ascariasis,
trichuriasis, hookworm, tapeworms)

Malabsorption
Secondary lactase deficiency (e.g., after gastroenteritis)
Eosinophilic gastroenterocolitis
Cystic fibrosis
Celiac disease
Primary immunodeficiencies (including human immunodefi-
ciency virus)
Shwachman-Diamond syndrome
Abetalipoproteinemia
Diarrhea in the Neonate
Milk protein intolerance
Necrotizing enterocolitis
Overfeeding
Congenital diarrhea

Miscellaneous Causes
Medications
Illicit drugs
Laxative abuse
Starvation stools
Inflammatory bowel disease
 Diarrhea and Dehydration 129

Irritable bowel syndrome

Henoch-Sch€onlein purpura
Typhlitis
Appendicitis
Neuroendocrine tumors
Malignancy

MAJOR THREAT TO LIFE

• Dehydration
• Electrolyte abnormalities
• Severe blood loss (if diarrhea is bloody)
• Sepsis

BEDSIDE
Your initial goal should be to determine the degree and type of
dehydration, if any. Dehydration can be classified as hypona-
tremic, isonatremic, or hypernatremic, depending on the serum
sodium concentration. The appropriate management of each
type of dehydration differs; therefore, it is critical to identify
which type is present. Determining the degree of dehydration
can usually be accomplished with a brief physical examination
and the answers to a few selected questions. If dehydration is
present, you should initiate therapy before returning to per-
form a more detailed physical examination, history, and chart
review.

Quick-Look Test
Does the child look well (comfortable), sick (uncomfortable or dis-
tressed), or critical?
Infants are often irritable or lethargic if dehydration is
present. Older children generally appear ill if the diarrhea is
significant.

Airway and Vital Signs

Look carefully at the heart rate and blood pressure. These values
provide you with a quick assessment of the degree of hypovolemia,
if any. A child with a normal heart rate and blood pressure is
unlikely to have severe hypovolemia or anemia. Orthostatic blood
pressure should be determined in older children if tachycardia is
present in the absence of hypotension. Fever suggests gastroenter-
itis or colitis.
130 Patient-Related Problems

Selective Physical Examination I

What is the child’s volume status?
In addition to the vital signs, estimation of the hydration sta-
tus of the child can be made by selectively examining the
following:
1. Mucous membranes
Are they moist or dry? How dry? Absence of tears suggests
severe dehydration, as do very sunken eyes.
2. Skin turgor
Normal or decreased? Is there “tenting”? Is the skin “doughy”?
Skin turgor can be assessed by gently pinching and releas-
ing the skin over the abdomen between the thumb and
forefinger. Normally, in an adequately hydrated person,
the skin retracts immediately and quickly. Slow retraction
suggests moderate dehydration, and “tenting,” or the lack
of retraction, suggests severe dehydration. “Doughy” skin
is suggestive of hypernatremic dehydration, a condition
in which intracellular volume decreases as a means of
attempting to maintain equal osmolality between the extra-
cellular and intracellular spaces. In the case of hypernatre-
mic dehydration, the child’s appearance may be deceptive.
Clinical signs of dehydration may not be as obvious as in
the other two forms of dehydration, hyponatremic and
isonatremic dehydration, which result in a decrease in
extracellular volume and have an insignificant effect on
intracellular volume.
3. Capillary refill and temperature of the extremities
Normal or delayed (>2 seconds) refill? Warm or cool extrem-
ities? Delayed capillary refill and/or cool extremities imply
inadequate distal perfusion secondary to hypovolemia.
4. Anterior fontanelle (in an infant)
Flat or sunken?
5. Weight
Determining the current weight of the child is necessary, and
comparing it with a previous recent weight (if known) is also
very useful. It is conventional to assign a “percent dehydra-
tion” to a dehydrated child (Table 15.1). Either a known
amount of weight loss or the estimated percentage can then
be used to determine the volume deficit.
Selective History and Chart Review I
Has a previous weight been recorded with which you can compare
the current weight?
This allows you to most accurately estimate the volume deficit.
What has the child’s urine output been?
 Diarrhea and Dehydration 131

TABLE 15.1 Estimation of Volume Deficit in a

Dehydrated Child

Infant 5% 10% 15%

Child 3% 6% 9%
Mucous Normal Dry Parched
membranes
Tears Present Decreased Absent
Eyes Normal Slightly sunken Severely sunken
Skin turgor Normal Slow retraction Tenting
Skin temperature Normal Slightly cool Cool, clammy
Capillary refill <2 s 2-3 s >3 s
Heart rate Normal Mild tachycardia Severe tachycardia
Fontanelle Flat Slightly Sunken
depressed
Urine output Normal Decreased Severe oliguria,
anuria

Normal urine output (approximately 1–2 mL/kg/hr and similar

to recent intake) suggests that the child is either euvolemic or only
mildly dehydrated. Severe oliguria or anuria suggests a large volume
deficit. In infants, you may need to base an estimate of urine output
on the number of wet diapers per day that the child has had.
Has the child also been vomiting?
Vomiting and diarrhea often coincide, thereby potentially add-
ing to the volume deficit.
Are there other factors that may contribute to volume loss, such
as fever or recent surgery?

Management
Principles
Fluid therapy can be divided into three categories:
1. Maintenance therapy
2. Deficit replacement
3. Replacement of ongoing losses
Maintenance therapy is aimed at providing the body’s normal
daily requirements for fluid and electrolytes. In a healthy person,
water is physiologically “lost” through urine, stool, and so-called
insensible losses (pulmonary and cutaneous losses). Electrolytes
(primarily sodium and potassium) are also lost in urine and stool.
These water and electrolyte losses are usually replaced through
eating and drinking; however, in those who are ill and hospital-
ized, oral intake is often greatly reduced or absent, and therefore
IV replacement of maintenance fluids is necessary. Several
132 Patient-Related Problems

methods are available for estimating a person’s maintenance

requirements. The simplest method is based on caloric require-
ments and assumes that 100 mL of water, 2 to 4 mEq of sodium,
and 2 to 4 mEq of potassium are necessary for each 100 kilocal-
ories (kcal) expended. The calories expended for a 24-hour period
depend on body weight and are estimated by using the following
rules for a hospitalized child:
100 kcal/kg for the first 10 kg
50 kcal/kg for the next 10 kg
20 kcal/kg for each kg above 20
Therefore a 30-kg child would expend approximately 1700 kcal/
day and require 1700 mL of water and 34 to 68 mEq each of sodium
and potassium. A solution of 5% dextrose and 0.2 normal saline with
20 mEq of potassium per liter at a rate of 70 mL/hour adequately
approximates these requirements (0.2 normal saline solution con-
tains 0.2
154 mEq/L ¼ 31 mEq/L of sodium). This method can
be used to calculate the maintenance requirements for a child of
any weight, and by determining the appropriate volume (with the
rules just presented), appropriate amounts of sodium and potassium
can also be provided.
Deficit replacement is aimed at replacing the amount of water
and electrolytes that have already been lost. A deficit is present
when physiologic and pathologic losses are greater than oral or
IV intake. As noted earlier, your initial goal when evaluating a
potentially dehydrated child is to determine the severity and type
of dehydration.
The severity of dehydration, or the percentage of body weight
lost, is determined either by the known difference in weight or
by the estimated “percent dehydration” based on clinical findings
(see Table 15.1). In infants, total body water is a larger percentage
of body weight than in children, and therefore clinical estimates of
mild, moderate, or severe dehydration represent a slightly larger
deficit.
The type of dehydration (hyponatremic, isonatremic, or hyper-
natremic) depends on the relative losses of water and sodium, as
well as on attempts that have already been made to correct a deficit
(e.g., replacement of losses with free water). Hyponatremic dehy-
dration can be defined as a serum sodium concentration less than
130 mEq/L, isonatremic dehydration as a serum sodium concen-
tration of 130 to 150 mEq/L, and hypernatremic dehydration as
a serum sodium concentration greater than 150 mEq/L. Most
infants and children with diarrhea and dehydration have the iso-
natremic form (approximately 70%), but certain clues may lead
you to suspect either hyponatremia or hypernatremia. An infant
who has been given large amounts of free water to replace diarrheal
losses is probably hyponatremic. Conversely, an infant with fever
 Diarrhea and Dehydration 133

and diarrhea who has ingested an inappropriately mixed, highly

concentrated formula may have hypernatremia.
Replacement of ongoing losses is appropriate when there are
reasons to believe that these losses are significant. For example, a
patient with excessive vomiting may continue to lose large amounts
of gastric fluid. A child with an ileostomy or excessive burns may
also continue to have large fluid losses. These losses must be con-
sidered when determining appropriate fluid therapy, or the amount
of water and electrolyte replacement may be significantly
underestimated.
Treatment
For all but the most mildly dehydrated patients, management
should begin with an initial IV fluid bolus of 10 to 20 mL/kg of
normal saline solution. The goal is to rapidly expand the extracel-
lular fluid volume, particularly intravascular volume. In severely
affected children, the bolus may need to be repeated until signs
of improved peripheral perfusion and stable hemodynamics are
present. This initial approach is appropriate for hyponatremic,
isonatremic, and hypernatremic dehydration and therefore should
not be delayed while waiting for the serum sodium result. Hypo-
tonic solutions and those containing potassium should not be
used during this initial phase of therapy because the goal is to
provide fluid that remains intravascular and potassium may be
dangerous if renal function is abnormal or hyperkalemia is
already present.
After the initial replenishment of intravascular volume, subse-
quent therapy is aimed at replacing the calculated deficits of water
and electrolytes while also providing ongoing maintenance
requirements and replacement of ongoing losses. When determin-
ing appropriate subsequent therapy, it is assumed that sodium is
the only electrolyte to be replaced. Because calculations are based
on the sodium deficit, the extracellular space is preferentially
replenished (which is desirable).
The water deficit is determined by a known weight loss
(1 kg ¼ 1 L) or estimated by your clinical examination. For children
with hyponatremic or isonatremic dehydration, the sodium deficit
can be calculated by using the following formula, once the current
serum sodium concentration is known:
Deficit ðmEqÞ ¼ ð135  NaÞ ð0:6Þ ðNormal Weight in kgÞ +
ðWeight Loss in kgÞNa
where Na is the current measured serum sodium concentration (in
mEq) and weight loss is either known or estimated from clinical
findings.
134 Patient-Related Problems

Subsequent management depends on the type of dehydration

present and is discussed in the following sections.
Isonatremic and Hyponatremic Dehydration
Isonatremic and hyponatremic dehydration are clinical states
resulting primarily from loss of extracellular volume. They should
be managed as follows:
1. Calculate or estimate the volume deficit (based on weight or
clinical examination, respectively).
2. Calculate the sodium deficit with the formula presented earlier.
3. Estimate the daily maintenance requirements (based on normal
weight) and add these to the deficit determination (step 1).
4. Subtract the amounts of fluid and sodium already given as
boluses to determine the amount of fluid and sodium to be
administered over the next 24 hours.
5. Replace half of these amounts in the first 8 hours and half in the
remaining 16 hours with a concentration of saline that provides
the appropriate amounts of water and sodium as determined by
your calculations.
6. Add potassium to the fluids only after the child has voided and
you have established that renal function is normal.
7. Replace ongoing losses at 8-hour intervals if they continue to be
significant. Table 15.2 lists the estimated electrolyte composi-
tion of various body fluids that may be lost abnormally; such
charts can be used to determine the concentration of replace-
ment fluids.
8. Check the serum sodium and potassium concentration again in
4 to 6 hours. Also monitor the child’s clinical findings and urine
output and specific gravity closely as a guide to hydration status.
This plan assumes that symptomatic hyponatremia (altered
mental status, seizures) is not present. If the child has symptoms
secondary to severe hyponatremia, hypertonic saline infusion
may be required initially (see Chapter 34). As noted earlier, the

TABLE 15.2 Estimated Electrolyte Composition of

Body Fluids

Fluid Na (mEq/L) K (mEq/L) Cl (mEq/L)

Gastric 20-80 5-20 100-150
Pancreatic 120-140 5-15 40-80
Bile 120-140 5-15 80-120
Small bowel 100-140 5-15 90-130
Ileostomy 40-135 3-15 20-115
Diarrhea 10-90 10-80 10-110
Burns 140 5 110
 Diarrhea and Dehydration 135

amount of sodium administered should then be subtracted when

calculating the deficit to be replaced.

Hypernatremic Dehydration
Hypernatremic dehydration results from an excessive loss of free
water in the absence of a significant sodium deficit. In contrast
to the other types of dehydration, replacement of the water deficit
must occur slowly, over a period of 48 hours or more. Rapid
decreases in the extracellular fluid sodium concentration may lead
to cerebral edema secondary to a large intracellular shift of fluid.
For this reason, the goal should be to reduce the serum sodium con-
centration by no more than 10 mEq/L/day. Management should
proceed as follows:
1. Calculate the free water deficit. As noted previously, clinical
findings in those with hypernatremic dehydration can be
deceptive and may not reflect the severity of dehydration.
Therefore, it is often reasonable to estimate a 10% loss of weight
unless the weight loss is known. The free water deficit can also
be calculated by using the following formula:
Deficit ðLÞ ¼ Normal Total Body Water  Current Total Body Water

where
Normal Total Body Water ¼
ðCurrent Total Body WaterÞ ðCurrent OsmolalityÞ
Normal Osmolality
BUN Glucose
Osmolality ¼ 2ðNaÞ + +
2:8 18
Therefore
ðCurrent Weight in kgÞ ð0:6Þ ðCurrent OsMÞ
Deficit ðLÞ ¼
290
 ðCurrent Weight in kgÞ ð0:6Þ

2. Subtract the amount of water given as boluses. Add mainte-

nance requirements for 48 hours.
3. Replace the remaining water deficit over a 48-hour period with a
solution that is either a one-fourth or one-third normal saline
solution initially and check the serum sodium level frequently
(i.e., every 2–4 hours initially). It is usually necessary to make
frequent adjustments in the concentration and/or rate to ensure
an appropriate, slow steady fall in the serum sodium concentration.
136 Patient-Related Problems

4. Add potassium to the IV fluids only after the child has voided
and you have established that renal function is normal.
5. Replace ongoing losses every 8 hours if they are significant.
6. After initiating therapy, you should return to perform a more
detailed physical examination, history, and chart review to look
for clues to the cause of the diarrhea.
Selective Physical Examination II
Physical exam
findings to look
for Conditions to consider
General Wasted appearance Starvation tools, laxative
abuse, immunodeficiency,
malignancy, celiac disease
Cardiac Murmurs, rubs or Primary or secondary heart
gallops failure
Pulmonary Rales, wheezes, Cystic fibrosis, protein-
cough, increased losing enteropathy,
work of breathing tuberculosis
Abdomen Bowel sounds, Gastroenteritis, colitis,
distention, appendicitis, celiac disease,
tenderness, inflammatory bowel
organomegaly, disease, malignancy,
masses and Henoch-Sch€onlein
protuberance purpura
Lymph Adenopathy Immunodeficiency,
nodes malignancy, tuberculosis
Extremities Edema, tenderness, Protein-losing enteropathy,
limited range of inflammatory bowel
motion disease, Henoch-Sch€onlein
purpura
Skin Rashes Viral and bacterial
gastroenteritis,
inflammatory bowel
disease, Henoch-Sch€onlein
purpura

Selective History and Chart Review II

Has the child been exposed to others with diarrhea?
If so, a common infection or perhaps staphylococcal food poi-
soning is suggested.
Is there blood, leukocytes, or eosinophils in the stool?
Blood and/or fecal leukocytes are more consistent with a bacte-
rial cause than a viral one but may also be present with inflamma-
tory bowel disease or some parasitic infections. The stool can be
 Diarrhea and Dehydration 137

examined easily for leukocytes by preparing a thin smear on a slide

and adding a drop of methylene blue. A Wright stain allows you to
identify eosinophils, which may lead to a diagnosis of milk protein
allergy in an infant or a parasite in an older child.
In addition to these bedside tests, stool cultures should be con-
sidered if bacterial colitis is suspected. A stool sample for ova and
parasite detection should also be considered if there is any possi-
bility of this diagnosis.
Has the child received antibiotics? What other drugs has the child
received?
Clostridium difficile infection should always be considered in a
child older than 12 months in whom diarrhea develops while in the
hospital or if the child has received antibiotics recently. There
should be a low threshold for sending stool to the laboratory for
toxin assays. Mild diarrhea is a common adverse effect of many
drugs including antibiotics.
Is there a history of recent gastroenteritis?
Secondary lactase deficiency is a common sequela of gastroen-
teritis and may lead to persistent mild diarrhea.

REMEMBER
1. Your primary goal should be to assess the degree of dehydration
and begin appropriate rehydration when dehydration is
present.
2. All of the management strategies discussed in this chapter are
based on multiple estimations. The most critical aspect of man-
aging a dehydrated infant or child is frequent monitoring and
reassessment, with appropriate adjustments in therapy. This is
especially true in cases of hypernatremic dehydration.
3. Initial management is aimed at rapidly replenishing intravascu-
lar volume. After this has been accomplished, you have time to
plan your subsequent management.
 CHAPTER

16
Extremity Pain
Jennifer Lhost, MD

Extremity pain is a frequent complaint in children. The list of

potential causes of extremity pain is extensive; however, very few
of these causes are immediately life threatening. When called
regarding a child with extremity pain, your primary goal while
on call should be to determine the likelihood of an illness that is
either life threatening or associated with significant morbidity if
the diagnosis is delayed.

PHONE CALL
Questions
1. How old is the child?
2. What is the severity of the pain?
3. Is there pain in one site or in multiple sites?
4. Is there swelling, warmth, or erythema at the affected site?
5. Is the child febrile?
6. Why has the child been hospitalized?
The age of the child may help you narrow the list of possible
causes. For example, benign nocturnal leg pains, commonly
referred to as “growing pains,” are more common in younger chil-
dren. Similarly, the various malignancies that may produce extrem-
ity pain tend to affect children of different ages (e.g., neuroblastoma
and leukemia in a younger child, osteogenic sarcoma in an adoles-
cent). The severity of the pain helps you determine the need for an
urgent evaluation, but it may not necessarily reflect the seriousness
of the underlying problem, particularly in a young child. Multiple
painful sites should raise suspicion of a systemic process, whereas a
single site makes a localized process more likely. If the affected area
appears erythematous and swollen, trauma or infection is sug-
gested. Fever increases the likelihood of an infectious cause, and
the child’s reason for hospitalization or past history allows you

138
 Extremity Pain 139

to consider some specific causes (e.g., sickle cell crisis, deep venous
thrombosis in a postoperative patient, hypertrophic osteoarthropa-
thy in those with chronic lung disease).

Orders
If not recently administered, acetaminophen or ibuprofen may be
given in an attempt to relieve the pain.

Inform RN
A child in severe pain, with fever, or with an extremity that appears
abnormal should be seen immediately.

ELEVATOR THOUGHTS
What causes extremity pain? It may help to think anatomically and
classify causes according to whether bone, muscle, joint, nerve,
blood vessel, or skin and connective tissue are involved. Benign
nocturnal pains (growing pains) and behavioral causes of pain
are diagnoses of exclusion.
Bone Osteomyelitis
Fracture
Infarction (e.g., in sickle cell crises)
Malignancy (leukemia, neuroblastoma,
primary bone tumor)
Avascular necrosis
Osteoid osteoma
Hypertrophic osteoarthropathy
Histiocytosis
Joint Septic arthritis
Transient synovitis (hip)
Viral arthritis
Benign hypermobility
Slipped capital femoral epiphysis (hip)
(9-16 years old)
Legg-Calve-Perthes disease (hip)
(4-9 years old)
Serum sickness
Traumatic arthritis
Hemarthrosis (e.g., hemophilia)
Juvenile idiopathic arthritis
Rheumatic fever
Lyme disease
Reactive arthritis
Systemic lupus erythematosus
140 Patient-Related Problems

Muscle Myositis
Infectious
Traumatic
Inflammatory (dermatomyositis,
polymyositis)
Electrolyte disturbances
Cramps
Muscle strain
Nerve Neuropathy
Radiculopathy
Complex regional pain syndrome
Guillain-Barre syndrome
Vascular Deep venous thrombosis
Coarctation of the aorta
Arterial thrombosis
Vasculitis (which may also cause secondary
neuropathy, arthritis, or myositis)
Connective tissue Fasciitis
Compartment syndrome
Skin and Herpes zoster
subcutaneous Cellulitis
tissue Trauma
Infiltrated IV line
Restrictive tape or bandages
“Hair tourniquet” (infants)
Other causes Benign nocturnal pain
Behavioral or psychogenic
IV, Intravenous.

MAJOR THREAT TO LIFE

1. Infection
Either a localized infection (e.g., osteomyelitis, pyomyositis, or
septic arthritis) or a systemic infection (e.g., meningococce-
mia, Rocky Mountain spotted fever, or toxic shock syn-
drome) may result in significant extremity pain.
2. Malignancy
Malignancy is not usually an immediate threat unless cell lysis
results in severe hyperuricemia, hyperkalemia, and other
metabolic disturbances.
3. Sickle cell disease—vaso-oclusive pain crisis
If the crisis is accompanied by signs and symptoms of infection
or acute chest syndrome, it may be life threatening.
4. Compartment syndrome (compromising the vascular supply)
5. Deep venous thrombosis leading to pulmonary embolism
 Extremity Pain 141

6. Arterial thrombosis leading to peripheral gangrene

7. Vasculitis
If also involving major organ systems (lungs, heart, central ner-
vous system [CNS], gut, kidneys), vasculitis may threaten
life. Kawasaki disease may result in coronary artery aneu-
rysms and a subsequent risk for rupture or myocardial
infarction.
8. Rheumatic fever
If carditis is also present, congestive heart failure may ensue.
9. Guillain-Barre syndrome (may compromise respiratory function)

BEDSIDE
As noted earlier, your primary goal while on call is to determine
whether the child has one of the major threats just listed. If you
can exclude a life-threatening possibility, a secondary goal should
be to narrow the extensive differential diagnosis for extremity pain
to a few likely possibilities. Regardless of the cause, improving the
comfort of the child should be a priority.

Quick-Look Test
Does the child look well (comfortable), sick (uncomfortable), or crit-
ical? Is the child moving the affected extremity or extremities?
If the child appears critically ill, you should immediately suspect
a multisystem illness (sepsis, vasculitis). With most causes of
extremity pain, the child appears well or only mildly uncomfort-
able. However, in a young child response to even minimal discom-
fort may be severe. Note the posture of the child. The child may
protect or position the extremity in a way that minimizes discom-
fort. For example, children with a septic hip usually prefer to hold
the hip flexed, externally rotated, and abducted (Fig. 16.1).

Airway and Vital Signs

As with any pain, mild tachycardia and tachypnea may be expected.
Severe abnormalities in the heart rate or respiratory rate or abnormal
blood pressure should not be expected and may be clues that the
extremity pain is only one manifestation of a systemic process. Sim-
ilarly, a compromised airway suggests a more significant problem
affecting the lungs, heart, CNS, and/or abdomen. Fever may indicate
infection or any inflammatory condition (e.g., vasculitis, lupus, der-
matomyositis, rheumatic fever, malignancy).
Selective History and Chart Review
Where is the pain?
In a young child, this question may not be easy to answer.
Young children may not be able to localize pain well or may give
142 Patient-Related Problems

FIGURE 16.1 Posturing of a child with a septic left hip. In an

attempt to relax the joint capsule, the child holds the affected
hip flexed, externally rotated, and abducted.

inconsistent answers. In many instances, you need to rely on a care-

ful examination to localize the affected area or areas. Remember
also that pain may be referred from other sites. Knee or thigh pain
may represent a pathologic condition of the hip. Hip pain may be a
symptom of lower back or intra-abdominal disease. You should
therefore fully examine the joints proximal and distal to the site
of pain.
Is the pain felt at a single site or at multiple sites? Is the pain well
localized or diffuse?
Osteomyelitis, septic joints, fractures, pyomyositis, osteoid oste-
omas, hemarthroses, traumatic injuries, primary bone tumors, and
 Extremity Pain 143

cellulitis are most often well localized and at a single site. Isolated,
unilateral hip pain (or referred thigh or knee pain) is suggestive of a
septic hip, transient synovitis, slipped epiphysis, Legg-Calve-
Perthes disease, avascular necrosis, or a spondyloarthropathy (in
an older child), but you should also consider the other localized
processes just listed. Pain in multiple sites usually indicates a sys-
temic process.
Has the child been irritable, lost weight recently, or had intermit-
tent fever, malaise, and poor appetite?
These findings should raise suspicion of a systemic inflamma-
tory condition such as infection, rheumatic disease, inflammatory
bowel disease, or malignancy.
Is there a history of sickle cell disease or hemophilia, either in the
patient or in the family?
Painful vaso-occlusive crises are the most frequent manifesta-
tion of sickle cell disease. These patients tend to have recurrences
of pain in the same sites; therefore the development of pain in a
new site should raise suspicion of an alternative cause. Hemarthro-
sis may be the initial manifestation of hemophilia, and these
patients also tend to have recurrences in the same joints.
Is there a history of recent extremity trauma?
Such a history raises suspicion of a fracture, hematoma, or com-
partment syndrome.
Has the child undergone prolonged immobilization?
Immobilization may predispose to deep venous thrombosis.
Is there a hypercoagulable state (birth control pills, lupus antico-
agulant, protein C or S deficiency, antithrombin III deficiency,
nephrotic syndrome, or malignancy)?
A hypercoagulable state is a risk factor for venous or arterial
thrombosis.
Has there been a recent upper respiratory infection or
pharyngitis?
This may suggest Guillain-Barre syndrome or rheumatic fever.
Selective Physical Examination
Your examination should initially focus on the affected extremity.
The source of the pain may often be unclear from the history alone,
and your goal should be to determine whether the pain is the result
of a problem in the bone, joint, muscle, nerve, vasculature, or skin
and soft tissue. You should first inspect and note the position of the
extremity and whether the child is willing to move the extremity
spontaneously. Look for swelling, erythema, warmth, or deformity
at all the joints, as well as over the long bones and the surface of the
skin. Without moving the extremity, attempt to palpate over each
of the bones and muscles and around the joints and carefully local-
ize any tenderness as best as you can. Osteomyelitis, bone tumors,
144 Patient-Related Problems

and bone infarctions should cause discrete point tenderness. A sep-

tic joint may also be tender, but the tenderness may not necessarily
be severe unless the joint is moved. Because the hips and shoulders
are deep joints surrounded by muscle and soft tissue, it is frequently
not possible to appreciate tenderness or warmth at these sites, even
in the presence of joint inflammation. Compartment syndrome,
myositis, and thrombosis (venous or arterial) may all result in
exquisite muscle tenderness. Neuropathies may result in dysesthe-
sia, paresthesia, or hyperesthesia. The passive range of motion of all
of the joints of the extremity should be evaluated to determine
whether limitations suggestive of arthritis are present. Muscle
strength testing in the affected extremity should also be performed
and the findings compared with strength elsewhere. If pain is in the
lower extremity, your examination should include an assessment of
the ability to bear weight and an observation of gait. Weakness is a
clue that the underlying process may involve muscle and/or nerve.
Deep tendon reflexes should also be measured to further evaluate
this possibility. Pulses in the extremity should be palpated and tests
of distal sensation performed, particularly if compartment syn-
drome or arterial thrombosis is suspected. Homans sign is sugges-
tive of deep venous thrombosis in the calf and is detected by
flexing the knee with the patient supine and then quickly dorsiflex-
ing the ankle to assess whether the patient experiences pain in the
calf muscle. However, a negative finding does not rule out the
diagnosis.
After your evaluation of the extremities, a general physical
examination should be performed to look for signs that may
indicate a systemic process. If the child has hip pain, a careful
abdominal examination, as well as examination of the spine and
lower part of the back, should be performed because pathologic
conditions in these sites may result in referred pain (e.g., psoas
abscess).

HEENT Conjunctivitis (Kawasaki disease); pharyngitis (viral,

streptococcal, rheumatic fever); oral ulcers (lupus,
inflammatory bowel disease); swollen, cracked lips
(Kawasaki disease)
Neck Stiffness (meningitis)
Lungs Respiratory distress (pulmonary embolism from deep
venous thrombosis, acute chest syndrome with
sickle cell disease)
Heart Murmurs of aortic or mitral insufficiency (rheumatic
fever)
Abdomen Tenderness, mass (psoas abscess, appendicitis,
vasculitis)
HEENT, Head, Eyes, Ears, Nose, Throat.
 Extremity Pain 145

Back Tenderness (diskitis, vertebral osteomyelitis),

scoliosis
Skin Rashes, vesicles, nodules (sepsis, herpes zoster,
vasculitis, juvenile idiopathic arthritis, rheumatic
fever, dermatomyositis); pustules (disseminated
gonococcal infection); erythema, warmth, edema
(cellulitis, fasciitis); cyanosis (arterial thrombosis,
compartment syndrome); inspection of IV sites for
infiltration or restrictive taping; inspection of
fingers and toes (“hair tourniquet,” foreign body,
paronychia)
IV, Intravenous.

Management
Further evaluation and management of major threats to life and
other selected causes of extremity pain are discussed here.

Septic Arthritis
A child with fever and a single swollen, painful joint should be con-
sidered to have a septic joint until proven otherwise. Septic poly-
arthritis is also possible but much less common, and other signs
and symptoms of sepsis are usually apparent. Staphylococcus
aureus is the most common cause in young children. Streptococcus
pneumoniae is also prevalent. In neonates, group B streptococci,
Escherichia coli, Listeria monocytogenes, and Candida albicans
are likewise common pathogens. Neisseria gonorrhoeae infection
is an additional consideration in sexually active adolescents. Most
septic joints are swollen, erythematous, and warm and have limited
range of motion. However, if the affected joint is the hip, pain and
limitation of motion may be the only objective signs of arthritis.
Remember that the subjective pain may be referred to the thigh
or the knee; therefore any patient with fever and pain in these areas
needs to undergo a careful evaluation of the ipsilateral hip. Plain
radiographs or ultrasound studies of the hip may be helpful when
it is unclear from your physical examination whether a hip effusion
is present. Once you suspect septic arthritis, the synovial fluid
needs to be aspirated immediately, before starting antibiotic treat-
ment. You should proceed as follows:
1. Obtain radiographs of the affected area, a peripheral blood cul-
ture, complete blood count (CBC) and differential, C-reactive
protein level, and an erythrocyte sedimentation rate. The blood
tests may help you monitor the response to treatment. The
radiographs should be reviewed to look for evidence of a con-
tiguous osteomyelitis.
146 Patient-Related Problems

2. Aspirate synovial fluid. You may need to consult a rheumatol-

ogist, interventional radiologist, or orthopedic surgeon to help
with this procedure, and the child may need sedation. The
orthopedic surgeon on call should be notified of any potential
septic hip because if it is confirmed, open drainage of the hip in
the operating room is necessary.
3. The fluid obtained at aspiration should be sent for the following:
Gram stain
White blood cell (WBC) count and differential
Glucose
Aerobic and anaerobic culture
Mycobacterial culture (if there are risk factors for tuberculosis)
Acid-fast staining
Gonococcal culture (in those who are sexually active)
The appearance of the fluid should be noted. Septic joints
usually result in cloudy or purulent fluid. WBC counts in the
fluid are generally greater than 50,000/mm3, with predomi-
nantly neutrophils, and the glucose concentration may be
low. Exceptions to these generalizations are common with gon-
ococcal arthritis.
4. After the foregoing procedures have been implemented, intra-
venous (IV) antibiotics should be empirically started. In chil-
dren younger than 5 years the combination of nafcillin and
cefotaxime provides adequate coverage until culture results
are available. In older children, nafcillin alone should be suffi-
cient. If the Gram stain is positive, the choice of antibiotics can
be more directed. The use of vancomycin instead of nafcillin
should be considered if resistant organisms have been prevalent
in the community.
5. A sexually active adolescent should also have throat, rectal, and
cervical or urethral cultures obtained before starting antibiotics
to further evaluate the possibility of gonococcal infection. The
organism is more often cultured from these sites than from
synovial fluid.
6. If tuberculosis is suspected from the exposure history, a tuber-
culin skin test and chest radiographic studies should be
performed.
7. If your physical examination (point tenderness) or radio-
graphs suggest osteomyelitis adjacent to the joint, the bone
itself should also be aspirated, with any fluid obtained sent
for the same studies as listed earlier for synovial fluid.
Magnetic resonance imaging (MRI) may also be helpful in
distinguishing osteomyelitis from septic arthritis. This distinc-
tion is important because a longer duration of antibiotic ther-
apy may be necessary if osteomyelitis is present.
 Extremity Pain 147

Osteomyelitis
The distal metaphysis of the long bones is a common site for oste-
omyelitis to develop. Localized point tenderness of a bone in a
febrile child should be considered osteomyelitis until proven oth-
erwise. Warmth and soft tissue swelling may be apparent if the
periosteum has been penetrated and the infection has spread to
adjacent soft tissue. If osteomyelitis is present without contiguous
septic arthritis, passive range of motion of the joint is normal.
Further evaluation of osteomyelitis is similar to that for septic
arthritis, except that bone should be aspirated. MRI may aid in
identifying the optimal site to be aspirated and should be consid-
ered if physical examination fails to adequately localize the area of
maximal tenderness. Plain radiographs are often unhelpful
because changes may not be seen within the first week of onset
of osteomyelitis. The most common pathogens are the same as
those that cause septic arthritis. Salmonella should be considered
in children with sickle cell disease, Pseudomonas should be sus-
pected in those with foot osteomyelitis after puncture wounds
through shoes, and Kingella should be considered in toddlers
or early school-age children with indolent onset. After aspiration
of the bone, IV nafcillin and cefotaxime (or ceftazidime if Pseu-
domonas is suspected) should provide adequate empiric coverage
for the most likely pathogens.

Pyomyositis
Localized muscle abscesses containing staphylococci may occur
either from hematogenous spread or from penetrating trauma to
the muscle (including immunizations). Local warmth, tenderness,
and a palpable mass within the muscle should raise suspicion of
this disorder. MRI or ultrasonography of the muscle may be help-
ful. Surgical consultation for drainage is necessary before starting
IV antibiotics.
Systemic Infections
Diffuse arthralgias and myalgias may be associated with numer-
ous bacterial and viral systemic illnesses. You need to carefully
and thoroughly evaluate the child for other signs of sepsis and
potential sources of infection (see Chapter 18). Treatment
depends on the specific cause. Influenza A may frequently cause
severe pain and tenderness in the calf muscles, often associated
with extreme elevations in muscle enzymes (creatine phospho-
kinase, aldolase). This process is self-limited and usually
resolves within a few days. Other viral illnesses, Rocky Moun-
tain spotted fever, or leptospirosis may result in similar
findings.
148 Patient-Related Problems

Malignancy
Primary bone tumors (Ewing sarcoma, osteogenic sarcoma), leu-
kemia, and neuroblastoma may all cause extremity pain. The
severity may range from mild pain or limping to severe, debilitat-
ing pain. Symptoms are often chronic but may be deceptively
intermittent. Pain is frequently worse at night, and symptoms
may be out of proportion to the objective findings. Plain radio-
graphs may reveal lytic bone lesions, periostitis, or metaphyseal
radiolucency. If you suspect malignancy, your goal while on call
is to prevent any potential complications until the diagnosis
can be confirmed and definitive treatment begun. Potential com-
plications include metabolic disturbances (e.g., hyperuricemia,
hyperkalemia), hematologic abnormalities (especially thrombo-
cytopenia or neutropenia), infection (particularly if neutropenia
is present), and the effects of space-occupying lesions (e.g., spinal
cord compression). You should determine whether the CBC, elec-
trolytes, calcium, uric acid, and phosphate have been checked
recently. Consultation with the oncologist on call is necessary,
with arrangements made for further diagnostic tests (biopsy, bone
marrow aspiration) as soon as possible.

Sickle Cell Crisis

Dactylitis in an infant, also known as hand-foot syndrome, may
be the first clinical manifestation of sickle cell anemia. Painful
swelling of the hands, feet, and digits is most often symmetric.
In a young child, extremity pain is a frequent manifestation of
the disease and is due to ischemic necrosis of bone as a result
of vaso-occlusion from sickled cells. Affected sites tend to remain
the same in the individual child, and the frequency of episodes can
vary considerably. Your first goal should be to confirm that the
current episode is secondary to vaso-occlusion and not another
process. Osteomyelitis may mimic a vaso-occlusive crisis, and if
there is any concern regarding infection, you should arrange to
aspirate the affected site as described earlier before starting IV
antibiotics. Careful evaluation of the child’s respiratory status is
mandatory to exclude a concurrent acute chest syndrome, and
if the child is febrile, a thorough search for sources of infection
is necessary. A CBC and reticulocyte count should be obtained,
along with appropriate cultures if the child is febrile. If the diag-
nosis of sickle cell disease has not been confirmed, a peripheral
blood smear should be reviewed and hemoglobin electrophoresis
performed. Treatment consists of IV hydration and analgesia.
Dehydration and acidosis should be corrected when present
(see Chapter 15). You need to reevaluate the child frequently,
and adjustments to the rate of hydration may be necessary
 Extremity Pain 149

because fluid overload may lead to pulmonary edema and the

potential for acute chest syndrome. Oxygen should be adminis-
tered if the patient is hypoxic. Vaso-occlusive crises often require
narcotic analgesics, but acetaminophen and/or nonsteroidal anti-
inflammatory drugs may sometimes be sufficient. Ketorolac may
be given in a dose of 0.5 mg/kg (maximum of 30 mg) every
6 hours. IV morphine or hydromorphone (Dilaudid) are the most
commonly used narcotics. Morphine may be given at 0.1 to
0.2 mg/kg per dose intravenously every 2 to 4 hours and hydro-
morphone at 0.015 mg/kg per dose every 3 to 4 hours. Alterna-
tively, patient controlled analgesia (PCA) may be used, with a
continuous infusion at a basal rate, a bolus amount, and a lock-
out interval preprogrammed. A child receiving narcotics also
needs frequent reevaluation. Remember that respiratory depres-
sion is a serious potential consequence of narcotic administration,
and acute chest syndrome may ensue quickly.

Compartment Syndrome
A recent arm or leg fracture or other trauma to an extremity can
lead to compartment syndrome. Injury to muscles gives rise to
swelling, which if severe enough, becomes limited by the tight-
fitting fascia encasing some of the muscle groups in the arm or
leg. Increased pressure within this compartment may compress
blood vessels and lead to decreased circulation to the muscles
and nerves. Pain, tenderness, distal sensory loss and/or weakness,
and overlying tension, erythema, or edema may all occur. Pulses
are not necessarily affected. If the patient has been placed in a cast
for a recent fracture and is experiencing pain, the cast needs to be
removed to adequately evaluate the extremity. Immediate surgical
consultation and fasciotomy are necessary because irreversible
necrosis may occur.

Deep Venous Thrombosis

Deep venous thrombosis is unusual in the pediatric age group but
may occur in children who have been immobilized, are on pro-
longed bed rest, or have a hypercoagulable state. The goals of treat-
ment are to prevent embolization and potential pulmonary
infarction. Heparinization should be implemented if the child
has any of these risk factors and physical examination reveals ten-
derness, warmth, edema, distended veins, and/or a positive
Homans sign suggestive of thrombosis. After a baseline partial
thromboplastin time (PTT) is obtained, an 80-U/kg IV loading
dose should be followed by a continuous infusion at 18 U/kg/hour
and the dose adjusted to produce a PTT of 1.5 to 2.5 times the
control value.
150 Patient-Related Problems

Arterial Thrombosis
Also unusual in childhood, arterial thrombosis or embolism is sug-
gested by the four Ps: pain, pallor, pulselessness, and paresthesias.
Hypercoagulable states are the major risk factors in childhood,
along with complications of indwelling catheters or attempts at
placement of arterial lines. Heparin should be initiated as described
earlier for venous thrombosis and vascular surgery consultation
obtained immediately.

Vasculitis
Vasculitis may result in extremity pain for a number of reasons,
including vaso-occlusion, neuropathy (ischemic as a result of
effects on the blood supply to the nerve), myositis, skin and soft
tissue involvement, and arthritis. The vasculitis may be life threat-
ening if other organ systems are also involved. Consultation with a
pediatric rheumatologist is necessary, with consideration given to
treatment with steroids or cytotoxic agents.

Rheumatic Fever
Migratory polyarthritis or arthralgias and a history of recent phar-
yngitis or known streptococcal pharyngitis should raise your sus-
picion of rheumatic fever. A careful cardiac examination should be
performed to listen for murmurs of aortic or mitral insufficiency.
You should also look for signs and symptoms of associated conges-
tive heart failure. A chest radiograph, echocardiogram, and strep-
tococcal antibody tests (antistreptolysin O [ASO], anti-DNAse B)
will be useful. The arthritis of rheumatic fever tends to affect large
joints; the pain is often severe and disproportionate to objective
findings and responds dramatically to salicylates or other nonste-
roidal agents. Prednisone (if severe congestive heart failure is pre-
sent) may also be necessary.

Guillain-Barre  Syndrome
Pain and tenderness of muscles may be a feature accompanying
the ascending weakness or paralysis of Guillain-Barre syndrome.
The deep tendon reflexes should be carefully evaluated if the diag-
nosis is suspected, as should the airway and respiratory status.
Cerebrospinal fluid analysis is necessary, with an albuminocyto-
logic dissociation (protein greater than twice normal with <10
WBCs/mm3) in a patient with a subacute polyneuropathy being
diagnostic for Guillain-Barre syndrome. Consultation with a
pediatric neurologist is usually necessary, and consideration
should be given to treatment with IV immunoglobulin, steroids,
or plasmapheresis.
 Extremity Pain 151

REMEMBER
When evaluating an inpatient with extremity pain, first isolate the
site and then organize your approach: bones, joints, muscles, blood
vessels, nerves, skin. Use the opposite extremity as a control for
your physical examination. When on call, concentrate on two
goals:
1. Exclude a life- or limb-threatening process.
2. Make the child more comfortable.
 CHAPTER

17
Eye Problems and
Visual Abnormalities
Rose Doolittle, MD

Acute eye problems in children are less common than most of the
other problems discussed in this book, and isolated problems of
the eye are rarely life threatening. However, any problem involving
the eye must be evaluated promptly for two reasons. First, eye and/
or visual complaints may herald significant central nervous system
(CNS) disease, including meningitis, encephalitis, and increased
intracranial pressure (ICP). Second, any process involving the
eye may potentially threaten vision.

PHONE CALL
Questions
1. Does the child appear well or sick?
2. How old is the child?
3. Is there drainage from the eye? What is the appearance of the
drainage?
4. Is there periorbital swelling and erythema?
5. Is vision affected?
6. Is there pain (pain with eye motion or tenderness to palpation)
or photophobia?
7. Is there a sensation of a foreign body? Does the patient wear
contacts?
8. What was the reason for admission?
A child who appears ill should be suspected of having a systemic
illness with associated eye manifestations. A number of systemic
infections (viral, bacterial, rickettsial) may produce conjunctivitis
in addition to other signs and symptoms. The age of the child,
the appearance of any drainage, the presence of periorbital swelling,
and any pain or loss of vision will help you to differentiate the
possible causes of eye inflammation. Pain or photophobia in a

152
 Eye Problems and Visual Abnormalities 153

normal-appearing eye suggests migraine or early uveitis. If the call is

regarding a newborn with eye drainage, ophthalmia neonatorum is a
major consideration.
Orders
None.
Inform RN
“Will arrive at the bedside in … minutes.” Ill-appearing children,
those with swelling or erythema around the eye, those with pain or
abnormal vision, and newborns all need to be seen immediately.

ELEVATOR THOUGHTS
What causes eye redness, drainage, pain, or swelling?
Conjunctivitis or keratoconjunctivitis
Bacterial (Neisseria gonorrhoeae and Chlamydia trachomatis in
the newborn)
Viral (herpes simplex in the newborn)
Chemical (silver nitrate in the newborn)
Allergic
Kawasaki disease
Other infections (e.g., Rocky Mountain spotted fever, leptospirosis)
Periorbital or orbital cellulitis
Uveitis
Traumatic corneal injury (abrasion, foreign body)
Nasolacrimal duct obstruction (infant)
Subconjunctival hemorrhage
Glaucoma
Panophthalmitis
What causes abnormal or acute loss of vision?
Many of the conditions just listed may lead to abnormalities
in vision, but in the absence of any of these causes, you should
consider the following:
Migraine
Increased ICP
Posterior reversible encephalopathy syndrome (PRES)
Retinal artery thrombosis
Optic neuritis
Retinal detachment (e.g., traumatic)
Psychogenic

MAJOR THREAT TO LIFE OR VISION

• Infectious conjunctivitis or keratoconjunctivitis in the newborn
• Periorbital or orbital cellulitis
154 Patient-Related Problems

• Panophthalmitis
• Severe trauma
• Increased ICP
• Retinal artery thrombosis (secondary to vasculitis, emboli, or
hypercoagulability)
• Meningitis (if photophobia is the primary complaint)
Treatment of each of these conditions should begin as soon as
possible after diagnosis. Empiric treatment of N. gonorrhoeae
conjunctivitis (in a newborn) and periorbital or orbital cellulitis
(in an older child) should be considered if these conditions cannot
be immediately excluded.

BEDSIDE
Quick-Look Test
Does the child look well (comfortable), sick (uncomfortable or
distressed), or toxic (critically ill)?
A child who looks ill needs an immediate, thorough evaluation
for a systemic illness.
Airway and Vital Signs
The vital signs should not be affected by a localized eye problem
unless the child is also systemically ill. Fever should prompt a
search for other potential sources and increase your concern about
potential cellulitis or ophthalmitis.

Selective History and Chart Review

When did the eye inflammation begin?
In a newborn, onset within 12 hours of birth suggests chemical
conjunctivitis from silver nitrate rather than an infectious cause. You
should determine whether the infant received silver nitrate or eryth-
romycin ocular prophylaxis (or neither). Gonococcal conjunctivitis
typically begins 2 to 5 days after birth, although it may be delayed by
partial treatment with ocular prophylaxis. Conjunctivitis secondary
to C. trachomatis may not appear for 5 to 14 days.
If the child is a newborn, were there any maternal infections
during pregnancy?
A history of gonorrhea, chlamydia, or herpes infection should
raise your suspicion of these infections as causes.
Has the child been systemically ill or had a recent infection?
Periorbital and orbital cellulitis, as well as panophthalmitis, may
occur secondary to hematogenous spread of bacteria, direct exten-
sion from sinusitis, or penetrating trauma and subsequent infection.
Staphylococcus aureus, pneumococcus, and group A streptococcus
are the most common pathogens. Conjunctivitis and uveitis may
 Eye Problems and Visual Abnormalities 155

be associated with a number of systemic illnesses. If there has been

sudden visual loss in a child with renal disease or hypertension or in
a child recently receiving cytotoxic medications, PRES should be a
consideration.
Are there other associated symptoms?
The presence of headache or neurologic symptoms may suggest
migraine or CNS disease with increased ICP or associated optic
neuritis.
Does the patient wear contacts?
Contact lenses have been associated with bacterial keratitis,
especially when improperly worn overnight.
Is there a history of trauma to the eye?
Corneal abrasions may easily occur from trauma that is unrec-
ognized or is thought to be insignificant. If the child has recently
undergone surgery, incorrect taping of the eyelids during surgery
may lead to eye dryness and/or pain during the recovery period.
Cellulitis may also develop after infection of a relatively minor
abrasion or laceration of the skin near the eye.
Selective Physical Examination
Look carefully at the eyes and surrounding soft tissues, and note the
characteristics of any drainage. Observe the sclerae, conjunctivae, and
extraocular movements of the child. The presence of pus (hypopyon)
or blood (hyphema) in the anterior chamber may produce a visible
fluid level between the inferior pole of the iris and the cornea and sug-
gests infection and trauma, respectively. Visualize the retina as best as
you can to look for papilledema, hemorrhages, and venous pulsations.
If necessary, dilate the pupils so that you can adequately examine the
retina. Remember to tell the nurse and document that you have
dilated the pupils, so the dilation will not be misinterpreted.
In an infant who appears well, with clear, thin drainage or small
amounts of mucoid drainage from the eye, you should suspect
nasolacrimal duct obstruction. Thick, purulent drainage with
marked injection and hyperemia of the sclerae and conjunctivae
suggests gonococcal conjunctivitis in a neonate and other bacterial
causes (Haemophilus influenzae, pneumococcus, staphylococcus,
or streptococcus) in an older child. C. trachomatis may result in
similar drainage but is generally less severe than that seen with gon-
ococcus. Swelling and erythema of the soft tissues around the eye
suggest periorbital cellulitis, especially if the child is febrile. Prop-
tosis or any abnormalities in extraocular movement should make
you very suspicious of orbital cellulitis. Papilledema warrants a
computed tomography (CT) scan of the orbits to rule out a mass
lesion, hemorrhage, cerebral edema, and hydrocephalus.
An eye that is injected but without mucopurulent drainage in an
irritable child should raise your suspicion of uveitis, keratitis,
156 Patient-Related Problems

trauma, Kawasaki disease, or glaucoma. Childhood glaucoma is

rare and produces the classic triad of tearing, photophobia, and
spasm of the eyelids secondary to corneal irritation. Corneal abra-
sions may be detected by instilling fluorescein dye onto the surface
of the cornea and visualizing it with a Wood lamp. If herpes is a
consideration, consultation with an ophthalmologist is necessary
to determine whether characteristic dendritic lesions of the corneal
epithelium are present.
Visual acuity should be tested with a Snellen chart, and visual
field testing should be performed if the child is old enough to coop-
erate. Objective abnormalities in vision should be an indication to
consult with an ophthalmologist.

Management
Any eye drainage should be Gram stained and cultured. Additional
laboratory evaluation depends on the suspected diagnosis. Urgent
ophthalmologic consultation should be obtained if you cannot ade-
quately examine the eyes, if there are abnormalities in vision, or if
the diagnosis is unclear. Definitive management of many condi-
tions involving the eye needs the expertise of an ophthalmologist.
A few of the most likely diagnoses you may need to address while
on call are discussed here.
Ophthalmia Neonatorum
If the Gram-stained drainage reveals gram-negative diplococci
characteristic of N. gonorrhoeae, give ceftriaxone, 25 to 50 mg/kg
intravenously (IV) or intramuscularly (IM) (maximum 125 mg/
dose), along with irrigation of the eye with normal saline solution
at 15-minute to 2-hour intervals. Typically a one-time dose of
ceftriaxone is sufficient; however, treatment may be continued
for 48 to 72 hours if the clinical response appears to be inadequate.
Blood should be drawn for culture and additional cultures (such as
urine and cerebrospinal fluid [CSF]) considered before instituting
systemic antibiotic treatment if the infant appears ill. Chlamydia
infection can be treated with oral erythromycin for 14 days.
Remember that coinfection with other sexually transmitted dis-
eases must be suspected in an infant with gonococcal or chlamydial
infection, including syphilis and human immunodeficiency
virus (HIV).
Conjunctivitis in an Older Child
Distinguishing viral from bacterial conjunctivitis may be difficult,
and many physicians elect to treat all cases of isolated conjunctivitis
that are believed to be infectious. “Pink eye” may be treated with
topical antibiotics such as bacitracin–polymyxin B or erythromycin
for 5 to 7 days.
 Eye Problems and Visual Abnormalities 157

Herpetic Keratitis
Intravenous acyclovir should begin immediately in a newborn in
whom herpetic keratitis is suspected because the infant may also
be at risk for disseminated herpes infection. The addition of topical
antiviral therapy should be considered and discussed with an
ophthalmologist. Older infants and children may be treated with
topical antivirals alone.
Periorbital and Orbital Cellulitis
If periorbital cellulitis is suspected, empiric intravenous antibiotic
treatment directed against the most likely pathogens should be ini-
tiated. The combination of clindamycin and ampicillin-sulbactam
is a reasonable choice to cover skin flora including methicillin-
resistant S. aureus (MRSA) and the usual bacterial etiologies of
sinusitis, respectively. Blood cultures can be considered prior to
giving antibiotics; however, these are typically low yield in older
children. In an ill-appearing infant, CSF, blood, and urine should
be obtained for culture before starting antibiotics. If proptosis or
any abnormality or pain with eye movement is noted, an emer-
gency CT scan of the orbits should be obtained to look for orbital
cellulitis or a frank orbital abscess. When in doubt, perform the CT
scan. Otolaryngologic and ophthalmologic consultations should be
obtained immediately and surgical drainage of the infected orbit
considered. Orbital cellulitis has potential for severe complications.
Pressure within the orbit may affect the optic nerve and lead to
visual loss. Alternatively, extension of the infection may result in
cavernous sinus thrombosis or epidural or cerebral abscess.

Trauma
Corneal abrasions can be treated with topical antibiotics such as
erythromycin or tobramycin applied three times a day. If the abra-
sion is large, the eye should be patched for 24 hours to promote
healing. More severe forms of trauma should be managed by an
ophthalmologist.

REMEMBER
1. Eye complaints may indicate a systemic or a CNS process.
2. Infection, trauma, and vascular occlusion of the eye are major
threats to vision and should be excluded or appropriately
managed.
3. Urgent ophthalmologic consultation may frequently be neces-
sary and should be obtained if you suspect a process that may
threaten life or vision.
 CHAPTER

18
Fever
Vanessa C. McFadden, MD, PhD

One of the most common and potentially serious problems that the
pediatrician manages while on call is fever. Fever can be a symptom
of mild self-limited infections, serious infections, malignancies, or
inflammatory illness. In a child who has already been hospitalized
with appropriate evaluation, fever may be an expected finding that
can be anticipated and treated. However, fever should never be
ignored.
In general, the accepted temperature that constitutes fever is
38.5°C, which corresponds to approximately 101.5°F. Regardless
of age, this temperature is considered above normal. In neonates
we often consider 38°C or higher as a fever and an indication
for evaluation to rule out sepsis. Therefore fever must be consid-
ered in the context of the specific patient’s signs and symptoms,
the child’s age, and the underlying diagnosis that led to the child’s
admission to the hospital.
Fever is a sign of potentially life-threatening illness and deserves
prompt, hands-on evaluation.

PHONE CALL
It is important that the pediatrician obtain accurate information
when notified about a child with fever. This allows prioritization
of the call. The following questions are suggested:
1. How old is the child?
2. What is the child’s admitting diagnosis?
3. What are the child’s vital signs?
4. What is the child’s appearance? How is the child acting? Is the
child alert? Oriented? Distressed? Agitated? Are the extremities
well perfused?
5. Has the child been febrile previously during this admission?
6. Are there standing orders for an antipyretic and/or laboratory
tests in the event of fever?

158
 Fever 159

7. Does the child have any underlying condition that may com-
promise the immune system (e.g., cancer, neutropenia, sickle
cell disease, rheumatic disease)?
High fevers (>39.5°C) warrant immediate hands-on evalua-
tion in any child younger than 36 months. This is the age group
at highest risk for occult bacteremia. Neonates and young infants
are at an even higher risk of serious infection and will require a
more extensive evaluation, regardless of other signs and symp-
toms, than older children. Laboratory tests that were to be
ordered in the event of a fever should be obtained, and an anti-
pyretic should be ordered immediately over the telephone. The
nurse should also be informed of the intent to evaluate the child
immediately.
If the child, regardless of age, shows any signs of hemodynamic
decompensation, a normal saline intravenous (IV) fluid bolus of 10
to 20 mL/kg should be started. If the child does not have adequate
IV access, it is imperative to obtain at least one reliable IV line.
In an older child (>3 years) the same questions should be
asked, and the antipyretic should be administered. Confusion occa-
sionally arises regarding the administration of antipyretics. Regard-
less of whether laboratory tests will be performed, it is important to
give the antipyretic to control the febrile response and make the
child more comfortable. An antipyretic does not affect a blood
culture or a complete blood count, and antipyretics do not cause
significant physical findings to disappear.

ELEVATOR THOUGHTS
The approach to fever in children is very age dependent. Neonates
are far more susceptible to bacterial illness and have greater vulner-
ability to morbidity and mortality. In addition, the pathogens most
commonly encountered in neonates differ from those found in
older children. This difference is caused by two phenomena. The
first is passive transmission of immunity via the placenta in the
third trimester of pregnancy. This immunity conveys relative
protection from viral illnesses such as varicella, rubella, and rube-
ola. Humoral factors transmitted in breast milk may also be pro-
tective and can last up to 2 to 3 months. The second major
reason for the difference in pathogenic flora is immunization
against both viral and bacterial pathogens, especially Haemophilus
influenzae type B (HIB), Bordetella pertussis, Clostridium tetani,
and hepatitis B virus.
If the child is a neonate and has been admitted to “rule out
sepsis,” cultures and other tests must be reviewed. Antibiotics
are commonly started empirically in neonates, and it is important
to know the antibiotics, the doses, the dosing interval, and their
160 Patient-Related Problems

spectrum of antibacterial coverage. Could the antibiotic therapy

already begun be inappropriate or inadequate?
It is important to carefully consider the vital signs in infants. Is
the child appropriately tachycardic for the fever? Tachypnea must
be distinguished from hyperpnea, which may indicate respiratory
compensation for metabolic acidosis. Because blood pressure is
generally preserved in infants until late in the course of shock,
the blood pressure measurement must be viewed in the context
of the child’s peripheral perfusion, urine output, and mental status.
The child’s hydration status should also be assessed. Circulatory
collapse and shock, with their resultant metabolic acidosis and
end-organ failure, must be avoided.
In an older child with any underlying conditions that predis-
pose to infection, the same considerations apply. For example, a
child with sickle cell disease who is older than 3 years should be
considered functionally asplenic and therefore more susceptible
to encapsulated bacteria. These children should be receiving daily
antibiotic prophylaxis against such organisms. Many older chil-
dren have underlying conditions that may directly affect their
immune system or their general health and nutrition status.
Congenital heart disease, cystic fibrosis, inflammatory bowel dis-
ease, short gut syndrome, and neuromuscular disorders are chronic
illnesses that can have a profound effect on the immune system of
older children and adolescents.
On the way to evaluate the child, consider the child’s age and
the urgency of the vital signs. Always prioritize to rule out life-
threatening conditions first: septic shock and meningitis.

MAJOR THREAT TO LIFE

• Septic shock
• Meningitis
The cascade of humoral factors released in response to fever can
cause hemodynamic instability and jeopardize the function of mul-
tiple organ systems. Meningitis can compromise central nervous
system function and result in altered mental status, seizures, deaf-
ness, and permanent disability.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable or playful), sick (distressed,
agitated), or critical (lethargic, unresponsive)?
Appearing sick or critically ill generally reflects hemodynamic
instability.
 Fever 161

Airway and Vital Signs

What are the heart rate, respiratory rate, and blood pressure?
Bradycardia in a febrile child is an ominous sign of impending
circulatory collapse. Likewise, tachycardia out of proportion to the
level of fever can be a sign of the child’s desperate effort to preserve
cardiac output.
Tachypnea and hyperpnea can reflect primary pulmonary
disease, as well as respiratory compensation for metabolic acidosis.
Blood pressure must be viewed in the context of the child’s
perfusion, urine output, mental status, and volume status. Multiple
mechanisms in the body interact to preserve blood pressure.
History and Physical Examination I
What is the volume status? Are there signs of shock? What is the
child’s mental status?
Repeat vital signs, including temperature.
HEENT* Fundi, photophobia, mucous membranes,
presence of tears
Neck Stiffness
Cardiovascular Heart rate, blood pressure, perfusion, murmurs,
pulses (upper and lower)
Lungs Quality of breath sounds and respiratory effort
Neurologic Sensorium change
Skin Turgor
*
HEENT, Head, eyes, ears, nose, throat.

The physical examination begins on first glance or at least

simultaneously with obtaining additional history. If it is at night,
turn on the lights to adequately see the child as you ask the nurse
or other caretaker for additional history. Review the questions
asked on the telephone. What has the child’s fluid status been
for the past 8 to 12 hours? Has the nurse or caretaker noticed
any other changes in behavior, mood, or feeding? What laboratory
studies were performed at the time of admission, and what are the
results? What medications is the child currently receiving (with
dose and interval for each)?
Vital signs should be determined again at the time of your eval-
uation. This is especially critical if any of the initial vital signs were
abnormal. Retake the temperature as well, preferably a rectal tem-
perature. Other methods are adequate for screening but lack both
the sensitivity and specificity of a rectal temperature. A thorough
age-appropriate physical examination should follow, including
funduscopic examination, pneumatic otoscopy, and rectal and/or
pelvic examination if appropriate. Regardless of the findings of pre-
vious examinations, a complete examination must be performed
162 Patient-Related Problems

and documented thoroughly. Special consideration should be given

to general appearance and mental status, quality and rate of respi-
rations, quality and rate of pulses, capillary refill time, hydration of
the mucous membranes, and skin turgor. Remember that the major
threat to life is septic shock and/or meningitis.
Management I
What measures need to be taken to prevent septic shock or to recog-
nize meningitis?
Any known source of infection should be reassessed. Such
assessment may require laboratory studies in the case of a child
with bacteremia, pneumonia, or meningitis. Venous blood should
be obtained for culture, from any febrile patient hospitalized for
more than 24 hours. Resistance to obtaining blood for culture is
encountered among both nurses and parents, but it is necessary
to detect occult bacteremia. Frequently, a complete blood count
is also obtained and can yield helpful information regarding the
white blood cell count and differential, platelet count, and hemo-
globin and hematocrit, which can often indicate the presence of
underlying chronic illness. The yield of blood culture is greatest
in the setting of a patient with fever higher than 39.5°C and a total
white blood cell count greater than 15,000.
Any sign of hemodynamic compromise must be addressed
immediately. Perfusion, capillary refill, pulses, blood pressure,
and heart rate, along with urine output, help to determine the need
for fluid resuscitation. Normal saline or lactated Ringer’s solution is
appropriate, generally in volumes of 10 to 20 mL/kg. If signs of cir-
culatory compromise persist, the patient should be transferred
expeditiously to the pediatric intensive care unit (PICU) for inotro-
pic support.
In a neonate or toxic-appearing child, antibiotics should be
promptly given as soon as cultures of blood, urine, and cerebrospi-
nal fluid (CSF) have been obtained. If there is difficulty in obtaining
samples for culture quickly and the child appears toxic, empiric
antibiotics should be started. In a neonate with fever and no known
source, ampicillin and either gentamicin or cefotaxime are given.
Older children may receive cefotaxime or ceftriaxone alone unless
there is a reason to be specifically concerned regarding staphylo-
coccus or resistant gram-positive organisms, in which case vanco-
mycin may be added. The use of aminoglycosides must be
accompanied by assessment of peak and trough levels, as well as
blood urea nitrogen and serum creatinine levels, to monitor for
nephrotoxicity.
If meningitis is suspected, a lumbar puncture is indicated. How-
ever, especially in infants, this procedure is not without risk. Be sure
that the patient is hemodynamically stable and in no respiratory
distress before placing the patient in a compromising position.
 Fever 163

(See Appendix A for how to perform a lumbar puncture.) Be sure

that oxygen and airway support supplies are readily available. If
there are any lateralizing signs on neurologic evaluation or suspi-
cion of a space-occupying lesion, antibiotics should be given and an
emergency head computed tomography (CT) scan without con-
trast obtained before the lumbar puncture. Whenever possible,
record an opening pressure as soon as CSF is obtained, especially
in an older child.
A chest radiograph should be obtained in any patient with
respiratory distress. For a toddler or preschool child with a sore
throat and dysphagia, a lateral neck film should be performed to
evaluate the retropharyngeal space, as well as the epiglottis, tonsils,
and adenoids. Above all, a careful, thorough physical examination
of the child will help you to determine what further work-up may
be necessary.

Selective Chart Review

If the patient is stable and does not have signs of meningitis, look
for localizing clues in the patient’s history and physical examina-
tion, progress notes and/or consultations, and laboratory results.
Additional information that will be helpful includes the following:
Temperature graph since admission
Recent white blood cell count and differential
Evidence of immunodeficiency (e.g., sickle cell disease, asplenia,
malignancy, human immunodeficiency virus [HIV] infection,
use of steroids)
Allergies to antibiotics
Current medications

Selective Physical Examination II

Target areas suggested by the chart review of the patient’s current
complaints.
Vital signs Repeat now
HEENT
Fundi Check for papilledema (intracranial abscess),
Roth spots (infective endocarditis)
Ears Otitis media
Nose Purulent drainage (sinusitis, foreign body)
Mouth Dental abscess, pharyngitis, peritonsillar
abscess
Neck Stiffness (meningitis), cervical adenopathy
(adenitis, retropharyngeal abscess)
Lungs Crackles, wheezes, friction rub, consolidation
(pneumonia, empyema)
Cardiac New murmur (infective endocarditis)
164 Patient-Related Problems

Abdomen Localized tenderness

Musculoskeletal Erythema, swelling, tenderness
Skin Rash, petechiae, purpura, IV sites
Pelvic If indicated

Management II
Besides blood, urine, and CSF cultures if indicated, cultures should
be obtained from central lines, potentially affected bones and/or
joints, bullous skin lesions, the pharynx, or any other site of appar-
ent inflammation or infection. In addition, a Gram stain should be
performed on any such culture material. Examination of the Gram
stain can be very useful in making decisions regarding antibiotic
coverage. In young children, remember to assess for a urinary tract
infection in any boy less than 6 months of age, uncircumcised
males 6 to 24 months of age, and all females up to 2 to 3 years
of age, when fever source has not been identified.
Which patients need antibiotics now?
1. Patients with signs of sepsis, with or without shock, need broad-
spectrum antibiotic coverage promptly.
2. Patients who are immunocompromised (i.e., neutropenic
patients, patients receiving chemotherapy, HIV-positive
patients, and asplenic patients [e.g., sickle cell patients])
Which patients need specific antibiotics now?
1. Patients with meningitis or any other localized infection that is
usually associated with specific bacteria will need specific anti-
biotic therapy.
2. Patients with a known positive culture
3. Patients with specific antibiotic allergies
Which patients do not need antibiotics until a specific pathogen
is diagnosed?
Patients older than 60 days who are not toxic, who are immu-
nocompetent, and who have no specific source identified for
their fever
What antibiotic should be administered?
In a neonate, ampicillin is chosen to cover Listeria monocyto-
genes and group B streptococci, and gentamicin or cefotaxime is
added to cover gram-negative enteric pathogens. Coverage for
Staphylococcus species is needed in any postoperative patient,
patients with indwelling catheters, gastrostomy tubes, or tracheos-
tomies, and young patients with cystic fibrosis. Older children with
cystic fibrosis require Pseudomonas coverage. Likewise, immuno-
compromised patients require Staphylococcus coverage, as well as
gram-negative coverage and potentially additional specific cover-
age depending on their underlying illness and past history.
 Fever 165

REMEMBER
1. Fever requires hands-on assessment and can be an ominous
finding in a young child.
2. Noninfectious sources of fever (i.e., drug-associated fever) are
diagnoses of exclusion.
3. Antipyretics do not alter the yield of blood cultures or the white
blood cell count or differential.
4. Fever in an immunocompromised patient requires consider-
ation of potential infection with unusual pathogens.
5. Any sign of hemodynamic compromise must be treated
quickly, and the patient must be reassessed promptly for
improvement.
6. Seizures are commonly associated with a rapidly rising temper-
ature in young children and do not, by themselves, suggest
meningitis or other more serious infection.
7. Base empiric selection of antibiotics on the probable organisms
for that patient’s age, underlying illness, and localizing signs, if
present.
8. Document your findings thoroughly, explain them to the parent
or family, and notify other providers of the patient’s condition
and your diagnosis and plan.
 CHAPTER

19
Gastrointestinal
Bleeding
Kent Rosenwald, MD

In the pediatric population, gastrointestinal (GI) bleeding can

occur at any age, with many potential causes throughout the GI
tract. GI bleeding can be generally categorized into upper and lower
GI bleeding. The differential diagnosis will vary substantially
depending on the age of the child, the presence or absence of
chronic GI or hepatic disorders, and other risk factors.

PHONE CALL
Questions
1. Age, clinical history, and known medical conditions?
2. Attempt to clarify the source and rapidity of bleeding: Is it fresh
(bright red blood) or old (melena, “coffee grounds”)?
Upper GI bleeding may manifest as vomiting bright red blood
or “coffee grounds,” or as melena, whereas bright red
blood from the rectum indicates lower GI tract bleeding.
(There can occasionally be bright red blood per rectum in
the case of rapid upper GI bleeding.)
3. How much blood has been lost?
Estimates of blood loss may be very inaccurate, but trace blood
will be managed differently than grossly bloody output.
4. What are the current vital signs?
Watch for tachycardia (early sign of hemodynamic instability)
before hypotension (late sign).
5. Does the child appear to be in pain? Appear to be ill?
6. Is the bleeding a new problem or issue?
Acute infectious illnesses (e.g., bacterial enteritis) cause blood in
the stool, as do chronic inflammatory conditions such as
Crohn disease and ulcerative colitis.

166
 Gastrointestinal Bleeding 167

7. What was the patient’s last hemoglobin or hematocrit and

platelet count?
8. Is the patient receiving an anticoagulant, such as heparin, war-
farin (Coumadin), aspirin, nonsteroidal antiinflammatory drug
(NSAID), or fibrinolytic therapy?
Orders
In the case of brisk GI bleeding or hemodynamic instability:
1. Establish intravenous (IV) access with at least one IV, as
large a line as possible. You may need to consider two
peripheral IV lines in situations in which the blood loss is
significant and ongoing.
2. If the patient is hypotensive or the volume of blood loss is
large, a 20-mL/kg bolus of normal saline or lactated Ringer’s
solution should be given immediately over a 5- to 10-minute
period. Repeat as necessary while obtaining additional sup-
port. Consider transfusion if volume resuscitation is still
needed after several fluid boluses.
3. Obtain new or repeat complete blood count (CBC). The
drop in hemoglobin and hematocrit may lag in a rapid bleed.
Obtain prothrombin time/partial thromboplastin time/
international normalized ratio (PT/PTT/INR) if concerned
about hepatic dysfunction. Make sure a blood specimen is
sent to the blood bank for typing and cross matching.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Patients
who are hemodynamically unstable (tachycardic, hypotensive,
poorly perfused) or in pain must be examined without delay,
and the senior resident should be informed immediately.

ELEVATOR THOUGHTS
Upper GI Epistaxis or nasal trauma (vigorous suctioning,
bleeding “picking”)
Oral or pharyngeal trauma (including dental)
Esophagitis
Gastritis
Esophageal varices (liver disease, portal
hypertension)
Mallory-Weiss tear, prolapse gastropathy
(vomiting)
Peptic ulcer disease
Swallowed maternal blood (newborn)
Hemorrhagic disease of the newborn (confirm
that vitamin K was given)
Foreign body ingestion
168 Patient-Related Problems

Lower GI Anorectal fissure (constipation)

bleeding Colitis (ischemic, infectious)
Hemorrhoids
Meckel diverticulum
Intussusception
Malrotation with volvulus
Hemolytic-uremic syndrome
Inflammatory bowel disease
Milk protein allergy (infants)
Necrotizing enterocolitis (premature infants)
Polyps
Henoch-Sch€onlein purpura (and other
vasculitides)
Vascular malformation
Hirschsprung disease
GI, Gastrointestinal.

MAJOR THREAT TO LIFE

• Hypovolemic shock
• Ischemic bowel with secondary perforation, peritonitis, and
sepsis
Blood loss into the GI tract is often insidious and results in ane-
mia, sometimes severe, but it does not usually cause hemodynamic
compromise. However, large-volume blood loss can occur, espe-
cially with ulcers, which can erode into arteries. Intussusception
or bowel ischemia in particular can be associated with circulatory
collapse. Likewise, in a premature infant, lower GI bleeding from
necrotizing enterocolitis can be accompanied by septic shock. Chil-
dren with chronic liver dysfunction before or after liver transplan-
tation may have the potentially fatal combination of esophageal
varices and coagulopathy.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable), or
unstable (critical)?
Children who have had significant blood loss appear pale and
poorly perfused and frequently have other signs of hypovolemic
shock, such as tachycardia, cold clammy extremities (increased
sympathetic tone), and tachypnea.
Airway and Vital Signs
Are there postural changes in blood pressure?
 Gastrointestinal Bleeding 169

Vital signs should be obtained in the supine and sitting

positions, when possible. There should be less than a 20-mm
Hg fall in systolic blood pressure. Likewise, diastolic blood pres-
sure should not fluctuate with position changes. Such changes
suggest significant blood loss or distributive shock secondary
to sepsis.
Selective Physical Examination
What is the child’s volume status? Is the child in shock?
Cardiovascular Pulse quality, capillary refill, warmth of
extremities
Abdomen Rigidity, guarding, rebound tenderness,
masses, abnormal bowel sounds, rectal
examination, look at the emesis or stool
(trace vs. frank blood)
Central nervous Mental status changes
system
Skin Jaundice, spider nevi, petechiae, purpura,
A rectal examination with heme testing of the stool is absolutely
necessary regardless of age or suspected cause of GI bleeding.
Management
What must be done immediately to treat shock (or prevent progres-
sion to shock)?
If vital signs and examination indicate hypovolemia or shock
from other causes, quickly place a reliable and reasonably large IV
line, and give a 20-mL/kg bolus of normal saline. Repeat isotonic
fluid boluses as needed while escalating care. In premature infants
and neonates, 5% albumin is expensive but preferred if readily
available. If the amount of blood loss is large, typing and cross
matching should be done immediately. Because the magnitude
of the blood loss must be assessed, a CBC and platelet count
should be obtained, as well as electrolyte, blood urea nitrogen,
creatinine, amylase, and liver transaminase levels. In a crisis sit-
uation, non–cross-matched type O-negative blood can be given,
although this is rarely necessary. If there is any suggestion of
sepsis, a bleeding disorder, and/or a hypoxic or ischemic insult,
coagulation studies should also be performed (screening for
disseminated intravascular coagulation). Also consider blood cul-
tures and empiric broad-spectrum antibiotics if the child appears
septic.
What can be done to stop the source of the bleeding?
Active GI bleeding is difficult to localize and treat. You must
treat or prevent hypovolemia immediately and then pursue the
underlying cause.
170 Patient-Related Problems

When is surgical consultation appropriate?

• Persistent bleeding requiring transfusion
• Presence of signs and symptoms of bowel obstruction (intussus-
ception or volvulus) or ischemia
When can endoscopy be completed to localize the site of bleeding?
Endoscopic assessment is the test of choice for upper and lower
GI bleeding. Before endoscopy the child must be kept without oral
intake (NPO) and must be hemodynamically resuscitated.
Is there an underlying coagulopathy?
Coagulopathies can result in heme-positive stools or overt
bleeding. Correction of the PT or PTT and discontinuation of anti-
coagulant therapy should be undertaken immediately.

Upper Gastrointestinal Bleeding (Hematemesis or

Melena)
Examine the child for sources of bleeding in the nose, mouth, and
throat. Keep in mind that small children put all sorts of objects in
their mouths and frequently walk around and fall with objects in
their mouths, which can result in significant trauma. Pens, pencils,
and various sharp implements can cause lacerations and/or punc-
ture wounds to the tongue, lips, or pharynx when children fall with
these objects in their mouths. Consider iatrogenic trauma as well,
such as suctioning or recent procedures (e.g., tonsillectomy).
Esophageal bleeding can result from caustic ingestion, varices,
or Mallory-Weiss tears and prolapse gastropathy associated with
vomiting. Gastritis (including cases due to Helicobacter pylori)
and peptic ulcer disease (may have epigastric pain relieved with
meals) can cause upper GI bleeding as well.
Laboratory Data
CBC, platelet count, reticulocyte count
Electrolytes, blood urea nitrogen, creatinine
• Blood urea nitrogen/creatinine (BUN/Cr) ratio >30 sup-
ports upper GI bleed
Liver function tests
PT, PTT, INR
Flat, upright, and/or lateral decubitus abdominal films, especially if
the child is uncomfortable
Upper GI (malrotation with midgut volvulus)
An Apt test (to distinguish maternal blood from the infant’s blood
in the newborn period)

Management
Place an nasogastric (NG) tube if large volume hematemesis. Con-
sider upper endoscopy to identify source of bleeding and poten-
tially intervene. H2 blockers or proton-pump inhibitors (PPIs)
 Gastrointestinal Bleeding 171

usually help in alleviating esophagitis and gastritis. For rapid upper

GI bleeding, start an IV PPI.
Lower Gastrointestinal Bleeding (Hematochezia and
Occasionally Melena)
The classic description of a “currant jelly” stool in an infant or tod-
dler with intermittent irritability strongly suggests intussusception.
A mass may sometimes be palpable in the right lower quadrant or
on rectal examination.
If there is associated diarrhea, evaluation for bacterial enteritis
may begin with stool nucleic acid testing or culture for Salmonella,
Shigella, Yersinia, Campylobacter, Escherichia coli, and other spe-
cies. A history of frequent health care system encounters or pro-
longed antibiotic exposure will predispose to Clostridium difficile
enterocolitis. Stool should be examined for the presence of leuko-
cytes, ova, and parasites, especially Giardia lamblia. Eosinophils
seen on Wright stain of the stool suggest milk protein allergy in
infants. Growth failure may suggest inflammatory bowel disease.
Endoscopy or magnetic resonance (MR) enterography should be
considered if inflammatory bowel disease is suspected. Painless
lower GI bleeding implies a Meckel diverticulum, whereas associ-
ated abdominal pain suggests intussusception in infants and tod-
dlers, inflammatory bowel disease in children and adolescents,
or infectious causes if associated with diarrhea.
In infants, careful examination of the perineum may reveal
estrogen-withdrawal vaginal bleeding in females or the presence
of small fissures around the anus. Premature infants (<35 weeks)
are at higher risk for necrotizing enterocolitis. Milk protein allergy
may present as bloody mucous stools and increased stool fre-
quency. Also in newborns, passage of stools can cause small tears
in anorectal tissue and give rise to fissures that result in small
amounts of blood coating the stool.
Bismuth compounds (e.g., Pepto-Bismol) and iron supplements
can turn stools black. True melena is pitch black, tarlike, and sticky
and has an odor that is not soon forgotten. Iron supplements can
also make stools test heme positive.
Laboratory Data
CBC, platelet count, reticulocyte count
Electrolytes, blood urea nitrogen, creatinine
• BUN/Cr ratio >30 supports upper GI bleed instead of lower
GI bleed
Liver function tests
PT, PTT, INR
Flat, upright, and/or lateral decubitus abdominal films, especially if
the child is uncomfortable
172 Patient-Related Problems

Meckel scan
Ultrasound of bowel for intussusception
Stool cultures/nucleic acid amplification testing (NAAT) for
bacterial enteritis, Ova and Parasite (O&P) testing
Proctoscopy
Management
Consider colonoscopy to identify source of bleeding and poten-
tially intervene. For suspected intussusception, nonsurgical reduc-
tion (e.g., air enema) is frequently both diagnostic and therapeutic.

REMEMBER
1. Give the child NPO orders and document when the last oral
intake was in case surgical intervention or endoscopy is
indicated.
2. Resuscitate the child before you pursue diagnostic studies.
3. Insertion of a nasogastric tube for upper GI bleeding can be crit-
ically important
4. Medications that may result in bleeding or exacerbate GI bleed-
ing include NSAIDs, steroids, heparin, and warfarin.
 C HA P TE R

20
Genitourinary Problems
James J. Nocton, MD

Several problems related to the genitourinary system may arise

while a child is hospitalized and require prompt attention. Some
of these problems, such as hematuria (see Chapter 23) and urine
output problems (see Chapter 30), are reviewed elsewhere in this
book. In this chapter the approach to evaluating dysuria, scrotal
pain, and vaginal bleeding while on call is discussed.
Dysuria

PHONE CALL
Questions
1. Is the child febrile?
2. What is the child’s diagnosis?
3. How old is the child?
4. Is the child a boy or a girl?
5. Is there gross hematuria?
The answers to these questions will allow you to begin to think
about potential explanations for the problem. If fever is present, a
urinary tract infection is much more likely. The child’s diagnosis or
the presence of gross hematuria may suggest specific causes (such
as hemorrhagic cystitis related to cyclophosphamide treatment as
part of chemotherapy protocols). Likewise, some problems may be
more common at different ages (e.g., sexually transmitted diseases)
or occur only in boys (balanitis).
Orders
Ask the nurse to collect the next urine for urinalysis and culture.
If the child is an infant, the nurse will need to collect the urine
by catheter to avoid contamination.
Inform RN
Tell the RN, “Will arrive at the bedside in … minutes.” A child with
fever, severe discomfort, or gross hematuria should be seen
immediately.
173
174 Patient-Related Problems

ELEVATOR THOUGHTS
What causes dysuria?
Urinary tract infections
Cystitis (bacterial, adenoviral, drugs)
Pyelonephritis
Urethritis
Sexually transmitted infection (STI; gonococcus)
Inflammatory (reactive arthritis, Kawasaki disease)
Vaginitis
STI
Group A streptococcus
Foreign body
Passage of renal calculi
Passage of a blood clot (trauma)
Urethral irritation
Girls
Bubble bath
Pinworms
Urethral prolapse
Sexual abuse
Hypercalciuria
Trauma
Boys
Hypercalciuria
Urethral stricture
Balanitis
Trauma/foreign body insertion

MAJOR THREAT TO LIFE

Few life-threatening problems are associated with dysuria. If infec-
tion is present, it may progress to sepsis and shock. If hemorrhage
from bleeding along the genitourinary tract is present, it could
eventually lead to hypovolemia and shock.

BEDSIDE
Quick-Look Test
Most children will appear well. If they are distressed, lethargic, or
unresponsive, shock from sepsis or hemorrhage and hypovolemia
may be imminent.

Airway and Vital Signs

Tachycardia, if present, will most likely be secondary to pain or
fever. An increased respiratory rate may also be related to pain
 Genitourinary Problems 175

or may reflect acidosis from infection. Hypotension will indicate

potential shock.
Selective Physical Examination
HEENT* Conjunctivitis may be seen with reactive arthritis or with
Kawasaki disease, both of which may cause urethritis
Abdomen Flank tenderness (pyelonephritis), suprapubic
tenderness (cystitis)
Genitals Urethral or vaginal discharge, ulcerations, penile
lesions (balanitis), vesicles (herpes simplex)
*
HEENT, Head, eyes, ears, nose, throat.

Selective History and Chart Review

Is the child at risk for STIs?
If the child is sexually active or has previously had such infec-
tions, the potential for an STI to be the cause is increased.
Is the child at risk for renal calculi?
A family history of renal calculi or conditions leading to poten-
tial hypercalcemia (see Chapter 34) increase the risk.
Has there been a history of trauma or kidney tumor?
Trauma to the kidney or Wilms tumor can lead to bleeding into
the urinary tract and the passage of blood clots.
Has the child received medications that can cause hemorrhagic
cystitis?
Review the child’s list of medications and potential adverse
effects to answer this question.
Management
Your priority while on call is to establish whether infection or hem-
orrhage is a probable cause of the dysuria because these are the two
potential threats to life. Urinalysis should be performed as quickly as
possible. A quick dipstick test at the bedside will determine whether
there is heme in the urine, potentially from hemorrhage in the uri-
nary tract, or whether leukocytes and nitrites are present, potentially
indicative of infection. The presence of nitrites is more specific for
bacteria in the urine because leukocytes may be present with nonin-
fectious urethritis, as well as with urinary tract infections.
Microscopic analysis of urine will allow you to determine
whether red blood cells are present, indicative of hemorrhage,
rather than hemoglobinuria from hemolysis or myoglobinuria
from rhabdomyolysis as the cause for heme in the urine. It will also
allow quantitation of the number of white blood cells in the urine,
with large numbers being more suggestive of infection.
Urinary Tract Infection
If the child has fever, flank pain and tenderness, and large numbers of
white blood cells in the urine, pyelonephritis should be suspected. If
176 Patient-Related Problems

there is no fever or flank pain and large numbers of white blood cells
are found in the urine with positive nitrites, it is more likely to be a
lower urinary tract infection (i.e., cystitis). Cystitis is more common
in adolescent females, whereas younger children are more likely to
have pyelonephritis. In some instances, it is difficult to determine con-
clusively whether the infection has ascended the urinary tract and led
to pyelonephritis. In either case, if the suspicion of infection is strong,
empiric antibiotic treatment should be started after ensuring that an
adequate specimen for urine culture has been obtained (catheteriza-
tion, suprapubic aspiration, or clean-catch specimen). Escherichia coli
and other enteric pathogens will be the most likely cause, and there-
fore a third-generation cephalosporin is often the best choice. Pyelo-
nephritis should be treated with intravenous antibiotics, whereas
cystitis may be treated orally with a third-generation cephalosporin,
trimethoprim-sulfamethoxazole, or, in older children, ciprofloxacin.

Hemorrhagic Cystitis
In the absence of a history of administration of medications that
can cause hemorrhagic cystitis, hemorrhage in the urine associated
with dysuria may be the result of renal calculi, hypercalciuria, or
viral hemorrhagic cystitis. Adenovirus is a common cause of hem-
orrhagic cystitis and requires only supportive treatment, with res-
olution generally occurring within several days. However, if
cyclophosphamide or another medication that can cause hemor-
rhagic cystitis has been administered, the child is frequently in sig-
nificant pain and requires prompt attention. Consultation with a
urologist will be necessary, and bladder irrigation should be initi-
ated. A Foley catheter should be placed and normal saline irrigation
begun. In some instances, cystoscopy or administration of alum or
silver nitrate into the bladder may be necessary. These steps should
be undertaken only after consultation with a urologist.
Sexually Transmitted Infections
If the patient is an adolescent with dysuria, an STI is possible. Cer-
tainly, a history of previous similar infections or the presence of a
vaginal or urethral discharge increases the likelihood of an STI. An
adolescent female should undergo a pelvic examination, and
appropriate cervical specimens for culture or nucleic acid amplifi-
cation of gonococcus and Chlamydia should be obtained. A wet
preparation should be performed with analysis for clue cells and
Trichomonas. If there is cervical motion tenderness or adnexal ten-
derness on examination and/or significant purulent cervical dis-
charge, treatment should be considered before confirmation of
infection by culture. In males a urethral swab can be performed
to obtain specimens for culture of gonococcus and Chlamydia. Per-
forming polymerase chain reaction (PCR) for gonococcus and
 Genitourinary Problems 177

Chlamydia on urine samples is an alternative in both males and

females. Treatment regimens for uncomplicated STIs and those
for pelvic inflammatory disease are presented in Tables 20.1 and
20.2, respectively.

TABLE 20.1 Treatment of Uncomplicated Sexually Trans-

mitted Infection in Adolescents

Chlamydia Azithromycin, 1 g orally in a single dose

trachomatis or
Doxycycline,a 100 mg orally twice daily for 7 days
Neisseria Ceftriaxone, 250 mg intramuscularly in a single dose
gonorrhoeae or
Cefixime, 400 mg orally in a single dose
plus
Treatment of C. trachomatis if indicatedb
a
Eight years of age or older.
b
The Centers for Disease Control and Prevention recommend treating persons with a positive
gonorrhea test result for both gonorrhea and chlamydia unless a negative result has been
obtained with a sensitive chlamydia test.
Modified from Workowski KA, Bolan GA: Sexually transmitted diseases treatment guidelines
2015. MMWR Recomm Rep 64(3):1-137, 2015.

TABLE 20.2 Treatment of Pelvic Inflammatory Disease

Parenteral Regimens (One of the Following)

Cefotetan, 2 g IV q12h, or cefoxitin, 2 g IV q6h, plus doxycycline, 100 mg IV or
PO q12h
or
Clindamycin, 900 mg IV q8h, plus gentamicin, loading dose (2 mg/kg-body
weight) IV or IM followed by maintenance dose (1.5 mg/kg q8h; single daily
dosing [3-5 mg/kg] of gentamicin can be substituted).
Parenteral therapy may be discontinued 24-48 hours after clinical improvement
and continue doxycycline, 100 mg PO bid, or clindamycin, 450 mg orally qid,
continued for 14 days of total therapy
For tubo-ovarian abscess, addition of either metronidazole, 500 mg PO bid, or
clindamycin, 450 mg PO qid, to oral doxycycline provides better coverage
against anaerobes
Outpatient Regimens (One of the Following)
Ceftriaxone 250 mg IM in a single dose, or cefoxitin 2 g IM—and probenecid
1 g PO in a single dose once, or other parenteral third-generation
cephalosporin (e.g., ceftizoxime or cefotaxime), plus doxycycline 100 mg PO
bid for 14 days with or without metronidazole 500 mg PO bid for 14 days
bid, Twice daily; IM, intramuscularly; IV, intravenously; PO, per os (orally); qid, four times daily.
Modified from Workowski KA, Bolan GA. Sexually transmitted diseases treatment guidelines
2015. MMWR Recomm Rep 64 (3):1-137, 2015.
178 Patient-Related Problems

Scrotal Pain

PHONE CALL
Questions
1. How long has the pain been present?
2. Has there been any history of injury or trauma?
3. Is there any radiation of the pain?
4. Are there systemic or other symptoms?
5. Is there swelling?
The sudden onset of pain or pain after minor injury is sugges-
tive of torsion of the testes or the appendix testis. Radiation of the
pain may suggest inguinal hernia, and systemic symptoms such as
fever and chills might indicate infection. Nausea and vomiting can
be associated with testicular torsion, and dysuria may be seen with
urinary tract infection or epididymitis.
Orders
Ask the nurse to collect and save a urine sample if possible.
Inform RN
Tell the RN, “Will arrive at the bedside in … minutes.” Scrotal pain
is an emergency and should be evaluated immediately.

ELEVATOR THOUGHTS
What causes scrotal pain?
Testicular torsion (adolescent > prepubertal)
Torsion of testicular appendage (prepubertal > adolescent)
Trauma
Incarcerated inguinal hernia
Epididymitis (adolescents)
Orchitis (mumps)
Vasculitis (Henoch-Sch€onlein purpura, polyarteritis nodosa)
Referred pain (nephrolithiasis, appendicitis)
Malignancy
Fournier gangrene

MAJOR THREAT TO LIFE

Interruption of vascular flow to the testes with risk of subsequent
infarction and loss of the testes is the major immediate threat. Tes-
ticular torsion results in the greatest risk for infarction, but severe
trauma or vasculitis may rarely also place the patient at risk. Fournier
gangrene is unusual in children, but it is a life-threatening infection.
 Genitourinary Problems 179

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (distressed, agitated),
or critical (lethargic, unresponsive)?
A child with scrotal pain will generally appear very uncomfort-
able regardless of the cause. Those with torsion of the appendix testis
will not be as uncomfortable as those with testicular torsion. In the
rare event of Fournier gangrene, the child may be in septic shock.
Airway and Vital Signs
The airway and vital signs should not be compromised, with the
exception that tachycardia and mild hypertension may be present
secondary to pain. If fever is noted, infection is obviously a
consideration.
Selective Physical Examination
The examination will focus on the scrotum, its contents, and the
inguinal canal, as well as the abdomen. The abdomen should be
inspected and palpated for signs of an acute abdominal process
with potential referred pain to the scrotum. Any scars should be
noted because they will indicate a possible previous hernia or unde-
scended testes. When examining the scrotum, note the position of
the testis. A high-riding, swollen, exquisitely tender testis is sugges-
tive of testicular torsion. The scrotum may be erythematous, and
the cremasteric reflex should be absent. If a firm mass is palpated
at the upper pole of the testis and the cremasteric reflex is present,
torsion of the appendix testis is much more likely. In some
instances the “blue dot sign” may be seen in which the twisted
appendix testis is visible through the skin. With epididymitis,
the epididymis itself will be firm, tender, and swollen.
Attention should be directed to the inguinal area to look for evi-
dence of hernia. Swelling and pain in the scrotum and inguinal
canal, especially with additional signs indicative of potential bowel
obstruction, may occur with an incarcerated inguinal hernia.
Selective History and Chart Review
If the patient is an adolescent, is there a history of STI or dysuria?
This will increase the likelihood of epididymitis.
Has there been intermittent pain in the scrotum in the past?
Such pain might reflect previous intermittent episodes of testic-
ular torsion.
Are there rashes, abdominal pain, and other systemic features?
Vasculitides such as Henoch-Sch€onlein purpura and polyarter-
itis nodosa may cause scrotal pain and swelling secondary to vas-
culitis within the spermatic cord.
180 Patient-Related Problems

Management
It may be difficult to distinguish testicular torsion, torsion of the
appendix testis, and other causes of scrotal pain by the history
and physical findings alone. This is critical because testicular tor-
sion is managed surgically and the other conditions may be man-
aged nonoperatively. Imaging studies may help to determine
whether blood flow to the testis is reduced, as seen in testicular tor-
sion. A color Doppler ultrasound is quick and often the most read-
ily available imaging study. A radionuclide testicular flow scan can
also be performed, but it requires more time and is often less con-
venient. When testicular torsion is a consideration, prompt consul-
tation with a pediatric urologist is most helpful.

Testicular Torsion
Testicular torsion is a surgical emergency. If there is a reasonable
likelihood of testicular torsion, the patient should be taken to the
operating room for surgical exploration because time spent on
imaging studies may be detrimental. Even if the torsion can be
reduced manually by the pediatric urologist, surgical fixation is
necessary to prevent recurrence.

Torsion of the Appendix Testis

Torsion of the appendix testis will eventually lead to infarction of
the appendix with subsequent resolution of the pain and swelling.
Bed rest plus analgesics for several days is usually sufficient. Imag-
ing studies will most often reveal increased blood flow to the testis.
Incarcerated Hernia
If an incarcerated hernia is suspected, immediate pediatric surgical
consultation is necessary, with surgical correction performed. Man-
ual reduction of the hernia may be attempted before surgical repair.

Epididymitis
In prepubertal boys, epididymitis is most often secondary to an
anatomic abnormality of the lower genitourinary tract and is usu-
ally caused by the same organisms that cause urinary tract infec-
tions. In these young boys, urinalysis with culture should be
performed, appropriate antibiotics administered when indicated,
and consultation with pediatric urology obtained. In adolescent
boys, epididymitis is most often an STI, with gonococcus and Chla-
mydia being most common, and should be treated as described ear-
lier in this chapter under “STIs.”

Fournier Gangrene
Systemic symptoms of fever, chills, and potentially shock often
accompany this severe necrotizing infection of the perineum.
Multiple organisms, including staphylococcus, streptococcus,
 Genitourinary Problems 181

anaerobes, and gram-negative organisms, have been associated

with Fournier gangrene. Surgical debridement and broad-
spectrum antibiotics should be instituted promptly.
Vaginal Bleeding
Vaginal bleeding is always abnormal in the absence of secondary
sexual characteristics and in girls younger than 8 years. The excep-
tion is in a newborn, in whom small amounts of vaginal bleeding
may occur as a result of withdrawal from circulating maternal
estrogens. In adolescents, it may also be the result of irregular men-
struation. Vaginal bleeding is rarely an emergency, but you may
need to be prepared to evaluate this problem should it begin while
you are on call.

PHONE CALL
Questions
1. How old is the child?
2. Has the child been menstruating previously?
3. Has there been any trauma?
4. What medications is the child receiving?
5. Is there bleeding at other sites?
The age and menarchal status of the child will affect your dif-
ferential diagnosis, as will a history of trauma, medications, and the
presence of bleeding at multiple sites.
Orders
A complete blood count (CBC) with differential, prothrombin time
(PT), and partial thromboplastin time (PTT) should be ordered if
the bleeding is heavy or persistent and if these laboratory tests have
not been performed recently.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Unless the
child is unstable or bleeding profusely, vaginal bleeding is rarely an
emergency, and if other situations are a priority, the patient can be
evaluated when time allows.

ELEVATOR THOUGHTS
What causes vaginal bleeding?
Prepubertal child Vaginal foreign body (toilet paper)
Infectious vulvovaginitis
Urethral prolapse
Trauma
Lichen sclerosus
182 Patient-Related Problems

Pinworms
Hemangioma
Malignancy
Precocious menarche
Pubertal child Foreign body
Trauma
Malignancy
Cervical polyp
Coagulopathy
Hemangioma
Cervicitis
Endometrial polyp
Pelvic inflammatory disease

MAJOR THREAT TO LIFE

Massive bleeding with exsanguination is the primary threat to life.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (distressed, agitated),
or critical (lethargic, unresponsive)?
If the child is distressed or agitated, excessive bleeding, infec-
tion, trauma, or an uncomfortable foreign body should be
considered.

Airway and Vital Signs

Fever, tachycardia, or hypotension should increase your suspicion
of infection or massive hemorrhage.
Selective Physical Examination
The examination will focus on the external genitalia, and in ado-
lescents, consideration will need to be given to performing a pelvic
examination if there is considerable bleeding and the cause is not
apparent after examining the external genitalia. In addition, the
general examination should focus on the mucous membranes of
the nasal and oral cavities and the skin in a search for other signs
of hemorrhage that might be indicative of a systemic coagulopathy.
The labia should be examined for signs of trauma, such as lac-
erations. Any discharge from the vagina and any lesions should be
noted, such as hemangiomas or urethral prolapse. The presence of
a foreign body should be obvious. The Tanner stage of the child
should be documented. Lichen sclerosus of the vulva leads to thin-
ning of the epidermis and has a characteristic appearance.
 Genitourinary Problems 183

Selective History and Chart Review

Has the child had menarche?
If other signs of pubertal development are apparent, the current
bleeding may represent menarche.
If the child has begun menstruating, what has the pattern of the
menstrual periods been and when was the last one?
Soon after menarche, menstruation can be very irregular.
Is there a history of bleeding disorders in the family?
Coagulopathies, including von Willebrand disease or a platelet
function defect, may cause menorrhagia without necessarily caus-
ing bleeding at other sites.
Management
The cause of the vaginal bleeding may be obvious after your phys-
ical examination and selective history, particularly if a laceration,
vaginal discharge, foreign body, or mass is evident. If signs of vag-
inal discharge are present, cultures should be obtained and appro-
priate antibiotics begun. If the cause is not easily discerned, further
evaluation will be necessary, but it does not necessarily need to
occur in the middle of the night. Checking the results of the
CBC, PT, and PTT can help to reassure you that the bleeding is
not excessive and that the patient is not at risk for severe bleeding
from a coagulopathy. The patient may require frequent evaluation
to ensure that the bleeding does not worsen and that her vital signs
do not suggest impending hypovolemia. As long as this is the case,
further assessment, including evaluation for precocious puberty
and potential consultation with a gynecologist, pediatric hematol-
ogist, or pediatric endocrinologist, can proceed in a less urgent
manner. Remember, your goal while on call is to ensure that noth-
ing will occur immediately to endanger the patient.

Summary
Genitourinary problems are infrequent while on call and are rarely
an emergency. However, a few life-threatening and organ-
threatening processes can cause dysuria, scrotal pain, and vaginal
bleeding and may need to be addressed while on call. Although it is
always optimal to make a definitive diagnosis, when this is not pos-
sible, evaluating the patient frequently, providing supportive care,
and remaining alert for potential life-threatening processes will
allow you to keep your patient safe and comfortable while you plan
potential further evaluation.
 CHAPTER

21
Headache
Susan K. Light, MD

Headache is a frequent complaint of hospitalized children. It may

be a symptom secondary to a life-threatening intracranial process,
or it may be a relatively benign and self-limited complaint. The goal
while on call is not necessarily to definitively diagnose the cause of
the headache. Instead, the more immediate goal is to exclude con-
ditions that require urgent attention and treatment. After this is
accomplished, headache can be managed symptomatically and
expectantly while planning for further diagnostic evaluation when
a clear cause cannot be immediately established.

PHONE CALL
Questions
1. How old is the child?
2. Why is the patient in the hospital?
3. How severe is the headache? Can the child rate the pain such as
with a pain scale (Fig. 21.1)?
4. Was the onset sudden or gradual?
5. What are the vital signs?
6. Has the child had a headache like this before?
7. Is the headache positional (is it worse when lying down flat)?

Orders
If a recent set of vital signs have not been recorded, ask the nurse to
obtain them, including temperature.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.”
Increased intracranial pressure (ICP) is the most concerning
possible explanation for headache and needs to be considered in
all patients with this complaint. Headaches related to increased
ICP are associated with nausea, vomiting, mental status changes,

184
 Headache 185

0 1 2
Very happy; no Hurts just a Hurts a little
hurt at all little bit more

3 4 5
Hurts even more Hurts a whole Hurts as much as
lot you can imagine

FIGURE 21.1 The Bieri Faces Scale. (Modified from Bieri D, Reeve
RA, Champion GB, et al: The faces pain scale for the
self-assessment of the severity of pain experienced by children:
development, initial validation, and preliminary investigation
for ratio scale properties. Pain 41:139-150, 1990.)

and eventually vital sign abnormalities, such as bradycardia and

hypertension. Recurrent or chronic headaches should be addressed
within a reasonable time but do not warrant an immediate assess-
ment if the vital signs are stable, the pain is not severe, and the child
has no other symptoms.

ELEVATOR THOUGHTS
What causes headaches?
Many of the causes are the same as in adults, but the relative
frequencies may be very different. For example, brain tumors
are the most commonly diagnosed solid tumor of childhood,
and many, especially posterior fossa tumors, are associated with
characteristic headaches.
186 Patient-Related Problems

Headaches may be a symptom of a disorder outside the nervous

system or may arise directly as a result of dysfunction within the
nervous system. The pain-sensitive structures in the head are as
follows:
Intracranial Cerebral and dural arteries
Large veins and venous sinuses
Dura at the base of the brain
Periosteum of the skull
Extracranial Cervical roots
Cranial nerves
Extracranial arteries
Muscles attached to the skull
Periosteum/paranasal sinuses
Eyes/ears
Mouth/dental structures
Skin or soft tissue over the skull
Acute Headache
1. Increased ICP: Headache results from compression and distor-
tion of pain-sensitive dural and vascular structures surrounding
the brain. It is worse after a few hours of being recumbent or
asleep and decreases after a period of being awake and upright
(positional headache). When more severe, it increases with
coughing, straining, and bending over and may be associated
with visual obscuration. Causes include:
Brain tumor
Subdural or epidural hematoma
Malignant hypertension
Pseudotumor cerebri
Trauma (closed head injury)
2. Infectious: Headache results from inflammation of pain-
sensitive structures surrounding the brain (meningismus). It
is usually generalized, severe, throbbing, and associated with
nuchal rigidity and photophobia. Causes include:
Meningitis
Encephalitis
Sinusitis or mastoiditis
Brain abscess
3. Vascular: Subarachnoid hemorrhage causes an explosive, sud-
den onset, “worst headache of my life.” Pain is followed by
meningismus and later by headache secondary to increased
ICP. Causes include:
Intraparenchymal hemorrhage
Vasculitis
Migraine
Arteriovenous malformation with bleeding
 Headache 187

Cerebral venous sinus thrombosis: seizures, increased ICP,

altered mental status
4. Posttraumatic
Concussion
Subdural or epidural hematoma
Cerebral contusion
5. Other
Acute angle-closure glaucoma
Alcohol or drug ingestion
Low-pressure headache: Because of loss of cerebrospinal fluid
(such as after a lumbar puncture [LP]), the brain’s buoyancy is
decreased such that the organ descends when the individual is in
the upright position; as a result, traction is exerted on structures
at the apex, and structures at the base are compressed. Pain is
relieved by lying flat.
Pituitary apoplexy: Acute hemorrhage or infarction of the pitu-
itary gland can cause acute headache referred to the temples/ears,
meningismus, visual field changes, and raised ICP-type headache.

CHRONIC (RECURRENT) HEADACHE

Progressive
1. Vascular
Migraine
Cluster headaches
Hypertension
Subdural hematoma
2. Metabolic
Hypoglycemia
3. Drugs
Alcohol
Nitrates
Calcium channel blockers
Nonsteroidal antiinflammatory drugs (NSAIDs)
4. Increased ICP
Tumor
Pseudotumor
Central nervous system vasculitis
Hydrocephalus
5. Infectious
Abscess (intracranial, dental)
Nonprogressive
1. Psychogenic
Tension headaches
Stress
188 Patient-Related Problems

Depression
Anxiety
2. Other
Temporomandibular joint disease
Posttraumatic
School avoidance/attention seeking

MAJOR THREAT TO LIFE

Headaches caused by increased ICP can be a presenting symptom
of the following life-threatening diagnoses:
• Intracranial bleeding: subarachnoid, subdural, epidural
hemorrhage
• Meningitis
• Herniation (transtentorial, cerebellar, central)
• Tumor
• Cerebral venous thrombosis
• Acute obstructive hydrocephalus
All of these conditions can progress rapidly and are associated
with a poor outcome if unrecognized. Herniation is a significant
cause of death secondary to cerebral edema after trauma, intra-
parenchymal bleeding, or hypoxic-ischemic encephalopathy
(Fig. 21.2).

BEDSIDE
Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable,
distressed), or critical (about to die)?
Most patients with chronic or recurrent headaches are fairly
comfortable. Those with migraines, meningitis, subarachnoid hem-
orrhage, or subdural or epidural hematomas generally appear ill.

AIRWAY AND VITAL SIGNS

What is the temperature?
Fever in the setting of headache should prompt a search for
infectious causes, including meningitis, abscess, encephalitis, and
sinus disease.
What is the blood pressure?
Significant hypertension of any origin can cause headache.
What is the heart rate?
Cushing triad of hypertension, bradycardia, and respiratory
changes is a most ominous and generally very late finding that is
accompanied by significant mental status change. Tachycardia
would be expected in any child complaining of severe headache.
 Headache 189

FIGURE 21.2 Central nervous system herniation. a, Cingulate

herniation; b, Uncal herniation; c, Cerebellar herniation. (From
Marshall SA, Ruedy J: On Call: Principles and Protocols, 4th ed.
Philadelphia, Elsevier, 2004, p 122.)

SELECTIVE PHYSICAL EXAMINATION

HEENT Funduscopic examination is ABSOLUTELY

ESSENTIAL for detecting vascular changes,
hemorrhages, and papilledema (Fig. 21.3) (loss of
the optic nerve margin and lack of venous
HEENT, Head, Eyes, Ears, Nose Throat.
190 Patient-Related Problems

FIGURE 21.3 Disk changes seen in papilledema. (A) Normal.

(B) Early papilledema. (C) Moderate papilledema with early
hemorrhage. (D) Severe papilledema with extensive hemor-
rhage. (From Marshall SA, Ruedy J: On Call: Principles and
Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 123.)

pulsations are specific signs of increased ICP); also

check visual acuity, symmetry of pupils,
photophobia, extraocular movements, ptosis,
sinus tenderness, hemotympanum (basilar skull
fracture), mastoid tenderness, depressed skull
fractures, contusions, jaw pain, or restriction of
movement.
Neck Nuchal rigidity, positive Kernig or Brudzinski sign
(Fig. 21.4).
Neurologic Cranial nerve examination; symmetry of reflexes,
tone, and strength; cerebellar function, including
balance and gait; mental status examination. MAKE
THE CHILD WALK IF AT ALL POSSIBLE!
 Headache 191

FIGURE 21.4 (A) Brudzinski sign. The test result is positive when
the patient actively flexes his hips and knees in response to pas-
sive neck flexion by the examiner. (B) Kernig sign. The test result
is positive when pain or resistance is elicited by passive knee
extension from the 90-degree hip-knee flexion position. (From
Marshall SA, Ruedy J: On Call: Principles and Protocols, 4th ed.
Philadelphia, Elsevier, 2004, p 124.)
192 Patient-Related Problems

MANAGEMENT I
If the patient has a condition associated with hydrocephalus, such
as new diagnosis of brain tumor or intracranial bleeding, neurosur-
gical consultation should be obtained as the patient is being pre-
pared for computed tomography (CT) scan.
In a child with nuchal rigidity, altered mental status, or focal
neurologic findings, order an immediate CT scan of the head.
If meningitis is suspected, order an LP tray at the bedside and
appropriate intravenous broad-spectrum antibiotics to be given as
soon as the CT scan and LP are completed. The CT scan and LP
should be completed within 1 hour. If there is to be any delay, give
the antibiotics and complete the studies thereafter.
The necessity for a head CT scan is controversial in patients
with a completely nonfocal neurologic examination, normal men-
tal status, and no signs of increased ICP, including papilledema.
One can proceed either by performing the LP and administering
the antibiotics (if you feel comfortable that there is no increase
in ICP) or by administering the antibiotics empirically and per-
forming the LP after the head CT scan has been obtained (if
increased ICP remains a concern).
In children with nuchal rigidity and signs of increased ICP, LP
is absolutely contraindicated because of the risk of brain hernia-
tion. Meningitis, subdural empyema, and brain abscess can all pro-
duce increased ICP and be manifested as headache. Head CT helps
to distinguish among these conditions. In general, when evaluating
for acute blood and ventricular size, CT of head without contrast is
better than magnetic resonance imaging (MRI). If inflammation is
suspected, CT of the head with contrast helps. To evaluate the pos-
terior fossa, order an MRI. When evaluating arteries, order MRI/
magnetic resonance angiography. To evaluate the venous sinuses,
order magnetic resonance venography. The empiric antibiotic
coverage suggested for children and adolescents is cefotaxime,
50 mg/kg intravenously every 6 hours, along with vancomycin,
15 mg/kg every 6 hours. Vancomycin is a recent addition to the
recommended empiric antibiotics because of the alarming increase
in resistant Streptococcus pneumoniae. Higher doses of antibiotics
are needed to allow for meningeal penetration. If abscess or sub-
dural empyema is suspected, clindamycin or metronidazole is
added to cover for anaerobes.
In addition to immediate antibiotic treatment, children with a
brain abscess or subdural empyema require the expertise of a pedi-
atric neurosurgeon. Moreover, it is prudent to anticipate a thera-
peutic plan for seizure control (see Chapter 29). If any signs of
altered mental status and/or increased ICP are present, therapy
should begin immediately (see Chapter 7).
 Headache 193

SELECTIVE HISTORY AND CHART REVIEW

If the headache is a new complaint, have the child describe in detail
what it feels like, where it hurts the most, and what makes it better
or worse. Was there a warning or an aura? Are there associated
symptoms? Did it start suddenly or gradually? Has the child ever
had a headache like this before?
Characterize the onset, duration, frequency, and pattern of
chronic headaches. Do they awaken the child from sleep or keep
the child from falling asleep? Are the headaches present as soon
as the child awakens in the morning? What time of day does the
headache occur? Are there known precipitants, such as foods,
change in sleep pattern, trauma, toxins, medications, or psychoso-
cial stressors? Are there any associated symptoms such as an aura,
tinnitus, visual changes, mental status changes, seizure activity,
nausea, or vomiting?
The chart may contain additional information about past com-
plaints of headache, as well as reports of a family headache history.
A medication history plus a history of head trauma over the past
6 to 8 weeks should be obtained. Headaches secondary to subdural
hemorrhage may be delayed for days or even weeks, and therefore
the patient and family may not associate the headache with a “dis-
tant” head injury.

MANAGEMENT II
Tension Headaches
Also known as stress headaches, muscle tension headaches are the
most common headaches in childhood. Frequently described as
“bandlike,” they are usually bilateral. Pain tends to be mild to mod-
erate, and the duration is highly variable, from 2 to 72 hours. This
type of headache is frequently related to undiagnosed refraction
defects in school-aged children straining to see the blackboard.
Chronic exposure to loud music or noise can also provoke this type
of headache. Patients are generally treated conservatively with
acetaminophen or ibuprofen and reevaluated in the morning.
Migraine Headaches
Migraine headaches can be incapacitating regardless of the patient’s
age. Migraines may be preceded by an aura, which can consist of
homonymous visual disturbances, unilateral weakness or sensory
changes, aphasia or other language disturbances, or the “Alice in
Wonderland” syndrome of spatial disorientation. Be aware that
these symptoms are similar to those of ischemic stroke. Migraines
are frequently unilateral but can be bilateral. Pain intensity is
194 Patient-Related Problems

moderate to severe, and the duration is typically approximately

8 hours. Acute therapy should begin with acetaminophen or
NSAIDs. Narcotic analgesics should be avoided if possible. The acute
administration of vasoconstricting agents such as ergotamine or
sumatriptan succinate (Imitrex) should be avoided in children youn-
ger than 10 years, if possible. β-Blockers are extensively used for
migraine prophylaxis but are of little use as acute therapy.
Posttraumatic (Postconcussive) Headache
Given a history of trauma but no signs of intracranial edema or
hemorrhage, postconcussive headaches can occur in the acute post-
traumatic phase or at much later times. Mild analgesics such as
acetaminophen or NSAIDs, which do not adversely affect the
child’s mental status or level of consciousness, should be adminis-
tered. If the headache continues to worsen, consider a CT scan of
the head to look for subdural or intraparenchymal blood.
Complicated Migraines
Migraines with brain stem aura, commonly known as basilar
migraines, are typically characterized by onset in adolescence and
occurrence in females more frequently than males. They are fre-
quently accompanied by visual disturbances, ataxia, vertigo, nausea,
vomiting, loss of consciousness, and/or drop attacks. Cranial nerve
deficits can be observed. In treatment, avoid sumatriptan because it
will constrict vessels in an already ischemic area. Hemiplegic
migraine must be distinguished from stroke. There is a slow progres-
sion of unilateral weakness and/or sensory changes usually preced-
ing the headache. Symptoms may last hours to days, and in recurrent
attacks the alternate side may be affected. Associated symptoms
include aphasia, paresthesias, and rarely seizures. Permanent deficits
can result from repeated attacks. Ophthalmoplegic migraines gener-
ally have an age of onset of less than 10 years. Unilateral eye pain is
followed by third nerve palsy, a dilated pupil, and downward and
outward deviation of the eye. The fourth and sixth cranial nerves
are frequently involved. Ophthalmoplegia resolves in 1 to 4 weeks.
Permanent third nerve injury can result from multiple attacks.
Cluster Headaches
Cluster headaches are nonfamilial and tend to afflict males more
than females. These headaches are rare before 10 years of age.
The headache tends to be rather brief, 30 to 60 minutes, but is severe
to excruciating. Often, there is unilateral nasal stuffiness and tearing
attributed to histamine release, hence the term histamine cephalgia.
Brain Tumor Headaches
Although headaches can be an initial symptom of a brain tumor,
brain tumors are generally an uncommon cause of headache
 Headache 195

in children. Brain tumor headaches tend to be chronic and progres-

sive, and their onset commonly has a positional component, with
maximal pain in the early morning on first rising from bed. The
vast majority of children with brain tumors have abnormal findings
on neurologic or ophthalmologic examination. A meticulous his-
tory and neurologic examination detect most brain tumors, espe-
cially in the setting of chronic, progressive headache.
Hemorrhages and Effusions
Subdural, epidural, and subarachnoid hemorrhages can result in
headache and are generally diagnosed by CT scan. Therapy may
be surgical, with decompression required to avoid herniation.
Prompt neurosurgical consultation is warranted. Chronic subdural
effusions can occur after meningitis, as well as after trauma, espe-
cially child abuse.

Malignant Hypertension
Malignant hypertension is unusual in children. Prompt but careful
reduction in blood pressure should be undertaken, as discussed in
Chapter 24.
Hydrocephalus
Although more common in younger children than in adolescents,
hydrocephalus, like brain tumors, tends to cause recurrent, pro-
gressive headache. Therapy requires neurosurgical consultation.
Obtaining the opening pressure when performing an LP is very
important and may suggest the diagnosis of pseudotumor cerebri
if the ventricles are not dilated on CT scan. Pseudotumor is most
often seen in obese, adolescent girls but is also associated with sev-
eral systemic illnesses.

Summary
Headache can be a common complaint in hospitalized children.
Most headaches are due to benign causes that require only analge-
sic therapy. The house officer on call must distinguish these mild
headaches from those that are life threatening. Thus headaches
warrant prompt evaluation. It is important in the history to distin-
guish an isolated acute headache from a recurrent acute headache,
as well as a pattern of chronic progressive versus chronic nonpro-
gressive headaches. Box 21.1 can be helpful in organizing the dif-
ferential diagnosis. Key physical examination findings include
meningismus, funduscopic irregularities, and abnormal ambula-
tion of the child.
196 Patient-Related Problems

BOX 21.1 Differential Diagnosis of Headache

Acute isolated headache

Meningitis
Subarachnoid hemorrhage
Systemic infection with fever
Acute recurrent headache
Brain tumor
Vascular malformation
Migraine
Hypertension
Sinusitis (rare in younger children)
Chronic progressive headache
Brain tumor
Hydrocephalus
Brain abscess
Subdural hemorrhage
Pseudotumor cerebri
Chronic nonprogressive headache
Depression
Stress, tension headache
Posttraumatic
School avoidance or attention seeking
 C HA P TE R

22
Heart Rate and Rhythm
Abnormalities
Daniel Beacher, MD

When evaluating a heart rhythm disturbance, the primary concern

is the hemodynamic status of the patient. A patient who is pulseless
should be treated according to the Pediatric Advanced Life
Support (PALS) cardiac arrest algorithm. A patient who is hemo-
dynamically unstable—weak pulses, poor perfusion, hypotension,
altered mental status—requires immediate intervention. Investiga-
tions into the underlying etiology should not delay support of
blood pressure (BP) and oxygen-carrying capacity and possible
cardioversion.
If the patient is hemodynamically stable, there is more time to
investigate the underlying etiology and tailor therapeutic interven-
tions accordingly. In this case, it is best to think of rhythm distur-
bances in general categories: too fast versus too slow, regular
versus irregular, and wide complex versus narrow complex. The task
for a house officer confronted by a rhythm disturbance is to find a
cause, if possible, and to intervene to preserve cardiac output (CO)
before damage to vital organs occurs, including the brain, kidney,
liver, and heart itself. CO is equal to stroke volume (SV) times heart
rate (HR) (CO ¼ SV  HR), and rhythm abnormalities have a pro-
found effect on both factors in that equation. Loss of atrial and ven-
tricular synchrony compromises ventricular filling and adversely
affects SV. Likewise, very high HRs decrease ventricular filling time
and compromise SV.
In pediatrics, rhythm abnormalities with a rapid HR are far
more common than rhythm abnormalities with a slow HR. The
abnormality may be in response to an extracardiac disturbance
(e.g., hypoxemia causing sinus bradycardia or fever causing sinus
tachycardia), an intrinsic electrical problem, or a combination of
both. Regardless of the cause, rhythm disturbances can be life
threatening and must be evaluated without delay.

197
198 Patient-Related Problems

Rapid Heart Rates

PHONE CALL
Abnormal heart rhythms provoke an immediate reaction from the
nursing staff. Questions should include the following:
1. What are the patient’s BP and perfusion status?
2. Are there any associated symptoms (chest pain, palpitations,
respiratory distress)?
3. What is the HR?
4. Is the rhythm regular or irregular?
5. Is the QRS complex narrow or wide?
6. How old is the patient?
7. Why is the patient in the hospital?
8. What are the rest of the patient’s vital signs (temperature,
respiratory rate)?
Orders
1. If the child is hypotensive or has signs of poor perfusion, insert a
peripheral intravenous (IV) line and start a bolus of normal
saline, 20 mL/kg, run as fast as possible.
2. Ask the nurse to attach the patient to a cardiorespiratory mon-
itor immediately.
3. Call for a stat 12-lead electrocardiogram (ECG) and rhythm
strip, if available.
4. Obtain another set of vitals, including BP reading, now.
5. Start the child on supplemental oxygen, 1 to 2 L/min by nasal
cannula.

Inform RN
Tell the RN, “I will arrive at the patient’s bedside in … minutes.”
Rapid HR with perfusion abnormalities is an emergency.

ELEVATOR THOUGHTS (CAUSES OF RAPID

HEART RATES)
Rhythm abnormalities with rapid HRs are best categorized by three
features: (1) whether the QRS complex is wide or narrow; (2) the
presence and location of P waves; and (3) whether the rhythm is
regular or irregular.
A narrow QRS complex is seen when the rhythm originates
somewhere above the ventricles (i.e., supraventricular), either in
the sinoatrial (SA) node, atria themselves, or atrioventricular
(AV) node, and is conducted normally down the His-Purkinje sys-
tem to the ventricles. P waves preceding narrow QRS complexes
suggest an origin in the SA node or the atria (e.g., sinus tachycardia,
atrial tachycardia, atrial flutter). Lack of P waves or P waves
 Heart Rate and Rhythm Abnormalities 199

following narrow QRS complexes suggest either a junctional origin

or reentrant rhythm.
A wide QRS complex is seen when the rhythm originates in the
ventricles or when there is a supraventricular origin with abnormal
conduction to the ventricles (aberrancy). However, distinguishing
between these two can be difficult and ventricular arrhythmias pose
an immediate threat to life. Therefore a wide complex tachycardia
should be assumed to be ventricular tachycardia (VT) unless there
is clear and convincing evidence otherwise. The regularity of the
rhythm will also help to discriminate between different types of
narrow or wide complex rhythms.

MAJOR THREAT TO LIFE

• Hypotension leading to shock
• Congestive heart failure leading to pulmonary compromise
and hypoxia
As noted previously, keep in mind the following formulas:
CO ¼ HR  SV
BP ¼ CO  SVR
Once the HR becomes so high that ventricular filling is compro-
mised, SV and therefore CO fall. The body tries to preserve BP by
increasing systemic vascular resistance (SVR) through vasocon-
striction, especially in the extremities, thereby resulting in compro-
mised peripheral perfusion.

BEDSIDE
Quick-Look Test
Does the patient look well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
Tachycardia is uncomfortable and may cause agitation and irri-
tability in infants and toddlers. Older children describe palpita-
tions, pounding of the heart or chest, or racing of the heart.
A patient in shock may have mental status changes.

Airway and Vital Signs

What are the HR and rhythm, temperature, and BP?
Hypotension requires immediate action, including a fluid
bolus. Fever is a common cause of sinus tachycardia and may be a
clue to an underlying etiology (e.g., sepsis causing hypotension).
Of note, sinus tachycardia may be a response to hypotension, but
it rarely occurs at rates high enough to be a primary cause of
hypotension.
200 Patient-Related Problems

Identification of the abnormal rhythm is best accomplished

using a 12-lead ECG; however, many rhythms can be identified
using the rhythm strip or cardiorespiratory monitor. One should
note that the paper speed on the ECG machine and sweep speed
on the cardiorespiratory monitor are set to standard speed
(25 mm/s). A lower speed (e.g., 12.5 mm/s) makes the QRS com-
plexes narrow and a higher speed (50 mm/s) makes the QRS com-
plexes wide. Recognition of the rhythm disturbance is critical. The
following table may be helpful in classification:
Regular Irregular
Narrow • sinus tachycardia • atrial fibrillation with
QRS (Fig. 22.1) rapid ventricular
complex • atrial tachycardia response (Fig. 22.6)
(Fig. 22.2) • atrial flutter with
• atrial flutter with variable block
regular block (Fig. 22.3) (Fig. 22.7)
• atrioventricular reentrant • multifocal atrial
tachycardia (AVRT), tachycardia
including Wolff- (Fig. 22.8)
Parkinson-White (WPW) • sinus tachycardia
syndrome (Fig. 22.4) with premature
• atrioventricular nodal atrial contractions
reentrant tachycardia (Fig. 22.9)
(AVNRT; Fig. 22.5)
• junctional ectopic
tachycardia
Wide • ventricular tachycardia • sinus tachycardia with
QRS (Fig. 22.10); the variant premature ventricular
complex torsades de pointes can be complexes (Fig. 22.12)
seen with long QT • any irregular wide
syndrome (Fig. 22.11) complex tachycardia
• any regular narrow with aberrancy
complex tachycardia with
aberrancy

FIGURE 22.1 Sinus tachycardia. (From Marshall SA, Ruedy J: On

Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 137.)
 Heart Rate and Rhythm Abnormalities 201

FIGURE 22.2 Atrial tachycardia. (From Marshall SA, Ruedy J: On

Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 138.)

FIGURE 22.3 Atrial flutter with regular block. (From Marshall SA,
Ruedy J: On Call: Principles and Protocols, 4th ed. Philadelphia,
Elsevier, 2004, p 138.)

FIGURE 22.4 Wolff-Parkinson-White syndrome. This condition is

characterized by a regular rhythm, a PR interval less than 0.12 sec-
ond, a QRS complex longer than 0.11 second, and a delta wave
(i.e., slurred beginning of the QRS). This predisposes to reentrant
tachycardia. (From Marshall SA, Ruedy J: On Call: Principles and
Protocols, 2nd ed. Philadelphia, WB Saunders Co, 1993, p 121.)
FIGURE 22.5 Atrioventricular nodal reentry tachycardia. (From
Marshall SA, Ruedy J: On Call: Principles and Protocols, 4th ed.
Philadelphia, Elsevier, 2004, p 138.)

FIGURE 22.6 Atrial fibrillation with rapid ventricular response.

(From Marshall SA, Ruedy J: On Call: Principles and Protocols,
4th ed. Philadelphia, Elsevier, 2004, p 136.)

FIGURE 22.7 Atrial flutter with variable block. (From Marshall

SA, Ruedy J: On Call: Principles and Protocols, 4th ed. Philadelphia,
Elsevier, 2004, p 136.)

FIGURE 22.8 Multifocal atrial tachycardia. (From Marshall SA,

Ruedy J: On Call: Principles and Protocols, 4th ed. Philadelphia,
Elsevier, 2004, p 137.)
 Heart Rate and Rhythm Abnormalities 203

FIGURE 22.9 Sinus tachycardia with premature atrial contrac-

tions. (From Marshall SA, Ruedy J: On Call: Principles and Proto-
cols, 4th ed. Philadelphia, Elsevier, 2004, p 137.)

FIGURE 22.10 Ventricular tachycardia. (From Marshall SA, Ruedy

J: On Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 135.)

FIGURE 22.11 Torsades de pointes. (From Marshall SA, Ruedy J:

On Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 149.)

Selective History and Chart Review

Identification of sinus tachycardia should prompt investigation
into extracardiac causes such as fever, pain, and medications (espe-
cially sympathomimetic drugs, such as albuterol, pseudoephedrine,
caffeine, and theophylline). A history of heart disease and/or
204 Patient-Related Problems

FIGURE 22.12 Sinus tachycardia with premature ventricular

contractions. (From Marshall SA, Ruedy J: On Call: Principles
and Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 137.)

cardiac surgery is pertinent and may help to narrow the differential.

Any previous ECGs are also helpful and may reveal baseline char-
acteristics that predispose to arrhythmia (e.g., Wolff-Parkinson-
White syndrome).
Management
Management of Hemodynamically Unstable Patient
Regardless of the cause of the tachycardia, if there are signs of poor
perfusion, ventricular filling must be improved by immediately
increasing preload (with IV fluids, packed red blood cells, etc.).
However, if the patient has significant cardiovascular compromise
(especially if the QRS complex is wide), emergency electrocardio-
version is indicated. Give the following instructions:
• Page the senior resident and pediatric intensive care unit
physician immediately, or call for the “code team.”
• Bring the cardiac arrest resuscitation cart to the patient’s
bedside, and attach the ECG leads of the defibrillator to
the child.
• Make sure that the child is receiving oxygen by nasal cannula or
mask and that airway management equipment is on hand.
• Ensure that an adequate IV line is in place.
• Select the appropriate energy (begin with 0.5 to 1.0 joule/kg)
and synchronize to the patient’s R wave (select the monitor lead
with the most obvious R wave).
• Clear everyone from contact with the patient, and deliver the
shock while recording the ECG.
• If sinus rhythm is obtained, proceed further stabilization.
• Continue to follow the PALS protocol for resuscitation.
Management of Hemodynamically Stable Reentrant
Supraventricular Tachycardia
If the patient has no evidence of significant cardiovascular compro-
mise, other methods, such as temporary AV nodal blockade, may
 Heart Rate and Rhythm Abnormalities 205

be used to convert the rhythm. For atrioventricular reentrant

tachycardia (AVRT) or atrioventricular nodal reentrant tachycar-
dia (AVNRT), this may include vagal maneuvers: ice to the face in
an infant, having a young child blow through an occluded straw, or
eliciting a Valsalva maneuver in an older child. If these are unsuc-
cessful, IV adenosine (0.1 mg/kg, maximum dose ¼ 6 mg) given by
rapid push and followed immediately by a large-volume flush is
effective in causing the same short-term AV nodal blockade
achieved by vagal stimulation. If the initial dose is ineffective,
the dose should be doubled, to a maximum of 12 mg. The most
common reasons for failure of adenosine is administration in an
inadequate IV line or an inadequate flush. The half-life of adeno-
sine in the bloodstream is 6 to 10 seconds; therefore an IV site
above the diaphragm is preferable, with at least a 10 mL normal
saline flush.
Refractory SVT can be treated with synchronized cardioversion
or with IV antiarrhythmic medications. These therapies should not
be started without cardiology or critical care input.
Note that the calcium channel blocker verapamil should be
avoided in infants and younger children with acute SVT because
of its long duration and the possibility of persistent high-grade
(life-threatening) AV block.
Management of Hemodynamically Stable Atrial
Fibrillation and Atrial Flutter
Atrial fibrillation is a very rare rhythm in children with anatomi-
cally normal hearts. It is usually seen in children with congenital
heart disease (CHD) and dilated atria (e.g., tricuspid or mitral valve
stenosis or regurgitation, pulmonary hypertension, or dilated car-
diomyopathy) or as a late complication of corrective surgery. Atrial
flutter is more common than atrial fibrillation in children and is
also often seen in children with structurally abnormal hearts
(although infants with atrial flutter often have structurally normal
hearts).
Both atrial fibrillation and flutter become hemodynamically sig-
nificant if the ventricular response rate is very high or when atrial
systole is needed to augment filling of a noncompliant ventricle;
both cases result in reduced ventricular filling.
Temporary AV nodal blockade (by vagal maneuver or adeno-
sine) will not terminate atrial flutter or fibrillation but may slow
the ventricular response showing fibrillation or flutter waves, thus
allowing proper diagnosis. Because of variable ventricular response
rates in children, these rhythms are often not well tolerated. They
may resolve spontaneously but more often require synchronized
cardioversion and/or chemical cardioversion with antiarrhythmic
medications. If the patient is hemodynamically stable, these
206 Patient-Related Problems

interventions should be performed in consultation with a cardiol-

ogist. Particular care should be taken when the abnormal rhythm
has been present for more than 24 hours because there is a risk of
thrombus formation in the fibrillating or fluttering atria that could
be embolized during cardioversion.

Management of Hemodynamically Stable

Ventricular Tachycardia
VT is a dangerous rhythm and must be treated immediately. As
mentioned previously, any wide complex tachycardia must be
assumed to be VT, unless there is clear and convincing evidence
otherwise. If the patient is hemodynamically stable, IV amiodarone
is an effective treatment for VT; lidocaine is the alternative. Pedi-
atric dosing of amiodarone is 5 mg/kg intravenously infused over a
period of 20 to 60 minutes (which may be repeated up to a max-
imum of 15 mg/kg per day). Synchronized cardioversion may also
be used if medical therapy alone does not convert the patient to
sinus rhythm.
Once the patient is converted to sinus rhythm, it is important
to search for possible causes of the VT, such as myocardial
injury or ischemia, hypoxia, electrolyte imbalance (hyperkale-
mia, hypokalemia, hypomagnesemia, hypocalcemia), cardiomy-
opathy (arrhythmogenic right ventricular dysplasia), and drugs,
including quinidine, disopyramide, tricyclic antidepressants, and
phenothiazines

Slow Heart Rates

PHONE CALL
Questions
1. What are the patient’s BP and perfusion status?
2. Are there any associated symptoms (respiratory distress,
presyncope, or syncope)?
3. What is the HR?
4. Is the rhythm regular or irregular?
5. How old is the patient?
6. What are the rest of the patient’s vital signs (temperature,
respiratory rate)?
7. Why is the patient in the hospital?
8. What medications is the child receiving?
Digoxin, β-blockers, and calcium channel blockers can prolong
AV nodal conduction and result in bradycardia with a prolonged
PR interval; they can also inhibit sinus node automaticity.
 Heart Rate and Rhythm Abnormalities 207

Orders
1. If the child is hypotensive or has signs of poor perfusion, insert a
peripheral IV line and start a bolus of normal saline, 20 mL/kg,
run as fast as possible
2. Ask the nurse to attach the patient to a cardiorespiratory mon-
itor (including a continuous pulse oximeter) immediately.
3. Call for a stat 12-lead ECG and rhythm strip, if available.
4. Call respiratory therapy immediately, administer 100% oxygen,
and have a bag-valve mask and intubation supplies at the bedside.
5. Bring the resuscitation “code” cart to the bedside, and attach the
patient to the defibrillator monitor.
6. If the HR is less than 60 beats per minute (bpm) in an infant or
40 bpm in a child or adolescent, a dose of 0.02 mg/kg atropine(-
minimum dose 0.1 mg, maximum dose 0.5 mg in a child and
1 mg in an adolescent) and a dose of 0.01 mg/kg epinephrine
should be prepared. If the patient is hypotensive and has an
HR < 60 bpm, the code team should be called.
Inform RN
Tell the RN, “Will arrive at the bedside in … minutes.”
Bradycardia is an indication of imminent circulatory collapse
and must be evaluated immediately.

ELEVATOR THOUGHTS (CAUSES OF SLOW

HEART RATES)
Bradycardia in children is often secondary to, or exacerbated by, extra-
cardiac causes, especially hypoxemia and drugs. However, sick sinus
syndrome (sinus node dysfunction) and AV block may be secondary
to CHD or a consequence of cardiac surgery. With slow sinus rates, one
may see junctional (narrow QRS complex) or ventricular (wide QRS
complex) escape beats, which may make the rhythm appear irregular.
An irregularly slow rhythm may also be seen in atrial fibrillation with a
slow ventricular response (Fig. 22.13). Asymptomatic sinus bradycar-
dia is common in well-conditioned athletes and during sleep.

FIGURE 22.13 Atrial fibrillation with slow ventricular

response rate. (From Marshall SA, Ruedy J: On Call: Principles
and Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 152.)
208 Patient-Related Problems

Selected Etiologies
Rhythm Drugs Cardiac Other
Sinus Digoxin, Neurocardiogenic Hypothyroidism,
bradycardia β-blocker, (vagal) increased ICP
(Fig. 22.14) calcium bradycardia, sick (Cushing triad),
channel sinus syndrome respiratory
blockers compromise
(hypoxemia),
anorexia
nervosa,
electrolyte
disturbances
Second-degree Digoxin, CHD, cardiac Head trauma,
AV block, β-blocker, surgery, acute electrolyte
Mobitz type calcium myocardial disturbances
I/Wenckebach channel infarction, blunt
(progressively blockers trauma,
prolonged PR myocarditis
interval until (including Lyme
nonconducted disease)
P wave and
absent QRS
complex;
Fig. 22.15)
Second-degree Digoxin, CHD, cardiac Head trauma,
AV block, β-blocker, surgery, acute electrolyte
Mobitz type II calcium myocardial disturbances
(normal PR channel infarction, blunt
interval with blockers trauma,
sudden myocarditis
nonconducted (including Lyme
P wave and disease)
absent QRS
complex;
Fig. 22.16)
Third-degree/ Digoxin, CHD, cardiac Autoimmune
complete AV β-blocker, surgery, acute disorders (e.g.,
block (none of calcium myocardial infants born to
the P waves are channel infarction, mothers with
conducted; blockers myocarditis anti-SSA or
QRS (including Lyme anti-SSB
complexes are disease) antibodies)
independent of
P waves;
Fig. 22.17)
AV, Atrioventricular; CHD, congenital heart disease; ICP, intracranial pressure;
SSA, Sj€ogren syndrome antigen A; SSB, Sj€ogren syndrome antigen B.
 Heart Rate and Rhythm Abnormalities 209

FIGURE 22.14 Sinus bradycardia. (From Marshall SA, Ruedy J: On

Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier, 2004,
p 150.)

FIGURE 22.15 Second-degree atrioventricular block (type I).

(From Marshall SA, Ruedy J: On Call: Principles and Protocols,
4th ed. Philadelphia, Elsevier, 2004, p 151.)

FIGURE 22.16 Second-degree atrioventricular block (type II).

(From Marshall SA, Ruedy J: On Call: Principles and Protocols,
4th ed. Philadelphia, Elsevier, 2004, p 151.)

FIGURE 22.17 Third-degree atrioventricular block. (From

Marshall SA, Ruedy J: On Call: Principles and Protocols, 4th ed.
Philadelphia, Elsevier, 2004, p 152.)
210 Patient-Related Problems

MAJOR THREAT TO LIFE

• Hypotension
• Asystole
Especially in infants, CO is very dependent on HR; conse-
quently, bradycardia significantly decreases CO and thereby leads
to end-organ dysfunction. Bradycardia secondary to myocardial
infarction (e.g., in those with coronary artery aneurysms from
Kawasaki disease) or contusion may deteriorate into a more omi-
nous rhythm, such as ventricular fibrillation or asystole.

BEDSIDE
Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable or
distressed), or critically ill (about to die)?
Unless the patient is very comfortable without signs of poor
perfusion, the “code” cart and other resuscitative measures should
be close at hand. Make sure that the patient is attached to a monitor
and that the sweep speed of the monitor is set at the standard
25 mm/s and that there is IV access. Make sure that the monitor
and the patient’s pulse correlate with one another.

Airway and Vital Signs

First, be sure that the patient is ventilating adequately—address air-
way and breathing first of all.
What is the HR?
Analyze the rhythm strip, and identify the rhythm. Profound
bradycardia may require immediate intervention, including the
administration of 0.1 mg/kg atropine or 0.02 mg/kg epinephrine(mi-
nimum dose 0.1 mg, maximum dose 0.5 mg in a child and 1 mg in an
adolescent) via an IV line or endotracheal tube if necessary.
What are the BP and perfusion status?
Regardless of the rhythm, poor perfusion and an HR < 60 means
that cardiopulmonary resuscitation must be started, including
chest compressions if necessary. Remember: electromechanical disso-
ciation gives a false sense of security because an electrical rhythm is
seen despite inadequate contraction. Volume resuscitation is required.

Selective History and Chart Review

Look for a cause or for previous episodes of bradycardia. Remem-
ber that the most common causes of bradycardia are related to
medications and respiratory compromise.
Does the child have a history of CHD and/or cardiac surgery?
Does the child have a past history of Kawasaki disease with
 Heart Rate and Rhythm Abnormalities 211

coronary artery aneurysms? Has the child been in an area endemic

for Lyme disease? Is there a family history of hyperlipidemia?
Was there associated syncope that might point to a vasovagal
episode? Was the bradycardia associated with vagal maneuvers,
such as straining, micturition, or Valsalva maneuvers?

Selective Physical Examination

Again, look for clues to the cause of the patient’s bradycardia
(if stable).
Vital signs Bradypnea (hypothyroidism),
hypothermia(hypothyroidism, exposure),
hypertension and irregular breathing
(ominous in combination, indicating
increased ICP, Cushing triad)
HEENT Coarse facial features, macroglossia, loss of the
lateral third of the eyebrows
(hypothyroidism); papilledema
(increased ICP)
Neck Goiter, jugular venous distention
Cardiovascular New S3 or S4, mitral regurgitation murmur
(myocardial infarction with papillary
muscle injury)
Abdomen Hepatosplenomegaly
Extremities Poor perfusion and/or peripheral pulses
Neurologic Mental status changes, evidence of trauma,
delayed return phase of deep tendon reflexes
(hypothyroidism)
Skin Annular erythematous papules (neonatal lupus)
HEENT, Head, ears, eyes, nose, and throat; ICP, intracranial pressure.

Management
Management of Hemodynamically Unstable Patient
As mentioned previously, regardless of the rhythm, poor perfusion
and an HR < 60 means that cardiopulmonary resuscitation must be
started, including chest compressions if necessary. The code team
should be called, IV access should be obtained, and a defibrillator
should be brought into the room. Atropine and epinephrine should
be available. Transcutaneous pacing may need to be used.
Hemodynamically Stable Patient
Management of Hemodynamically Stable
Sinus Bradycardia
If the child has normal perfusion and BP, no acute intervention is
necessary. Search for a cause. If the child has been receiving
digoxin, β-blockers, or calcium channel blockers, no further doses
212 Patient-Related Problems

should be given until the HR normalizes. Abrupt discontinuation

of β-blockers may cause rebound hypertension, angina, or myocar-
dial infarction. The drug should be reinstituted at a lower dose once
the HR has normalized.

Management of Hemodynamically Stable

Second-Degree Atrioventricular Block Type I
This rhythm does not degenerate into complete heart block. If the
patient is hemodynamically stable, no acute intervention is
required. Cardiotonic drugs should be withheld until the rhythm
normalizes. Continuous monitoring is advisable. If the child is
unstable, consider isoproterenol infusion.

Management of Hemodynamically Stable

Second-Degree Atrioventricular Block Type II
This rhythm can degenerate into complete heart block. If the
patient is hemodynamically stable, no acute intervention is
required. If the child is unstable, consider isoproterenol infusion.
Given the risk for development of complete heart block, cardiology
should be consulted promptly.

Management of Hemodynamically Stable Third-Degree

Atrioventricular Block
If the child is hemodynamically stable, no acute intervention is
required. If the child is unstable, begin resuscitative measures
including atropine and/or epinephrine. Transcutaneous/transve-
nous pacing and/or isoproterenol may be required. In the setting
of acute digoxin ingestion with high blood levels, bradycardia,
and second- or third-degree heart block, the digoxin-specific bind-
ing antibody Digibind must be given to reduce the toxic effects.

REMEMBER
1. Regardless of the underlying rhythm, if a patient is hemody-
namically unstable, initial management should focus on
improving CO and oxygen-carrying capacity.
2. Any wide complex tachycardia should be treated as life-
threatening VT unless there is clear and convincing evidence
otherwise.
3. In a hemodynamically stable patient with narrow complex
tachycardia, vagal maneuvers or adenosine may terminate the
rhythm or help with diagnosis.
4. Bradycardia is often secondary to respiratory compromise or
medications.
5. Proper performance and interpretation of the ECG is essential.
You cannot get the answer if you do not get the data!
 C HA P TE R

23
Hematuria
Anna Schmitz, MD

Hematuria is often described by patients or parents as “red urine”

or “brown urine.” The first step in evaluating these patients is
determining whether the child actually has hematuria, defined as
red blood cells in the urine on microscopic urinalysis. Frankly
red or pink urine suggests macroscopic hematuria. Tea- or cola-
colored urine may reflect hemoglobinuria, myoglobinuria, or
excretion of organic dyes, such as those in beets and some confec-
tionery dyes. An orange stain in a diaper is often mistaken for blood
when it is more likely to be urate crystals.
Hematuria can be a sign of injury to the lower genitourinary
tract, as in trauma or infection, or it may represent glomerular
injury as a result of trauma, infection, or an autoimmune condition.

PHONE CALL
Questions
1. How old is the patient?
2. Is the child male or female?
3. Why is the patient in the hospital?
4. Has there been any recent trauma?
5. Are there any associated symptoms, such as frequency, urgency,
fever, dysuria, oliguria, or polyuria?
6. If female, is the child menstruating?
Orders
Ask the nurse to obtain a sterile urine specimen for dipstick, micro-
scopic analysis, culture, and Gram stain. For children unable to
provide a clean-catch sample, a catheterized specimen should be
obtained.
Repeat a full set of vital signs immediately.

213
214 Patient-Related Problems

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Hematuria
requires evaluation but rarely is an emergency. In the setting of a
trauma patient, it deserves prompt attention because significant
kidney injury or pelvic fracture can precipitate internal bleeding
and shock.

ELEVATOR THOUGHTS
The differential diagnosis of hematuria (red blood cells in the
urine) includes the following:
Infections
Urinary tract infection
Viral bladder infection
Trauma
Perineal or meatus irritation
Nephrolithiasis
Glomerular injury
Postinfectious glomerulonephritis
Hemolytic-uremic syndrome
Lupus
Immunoglobulin A (IgA) nephropathy
Henoch-Sch€onlein purpura
Antineutrophil cytoplasmic antibody-associated vasculitis
Goodpasture syndrome
Anatomic
Polycystic kidney disease
Tumor
Vascular
Renal artery/vein thrombosis
Exercise induced
Interstitial nephritis
Acute renal tubular injury (shock; medications)
Sickle cell trait
Fictitious
Menses

MAJOR THREAT TO LIFE

Blunt trauma to the flank, back, or abdomen of a child may result in
significant renal injury. In addition to hematuria, this may cause
intra-abdominal or retroperitoneal bleeding leading to shock.
The urine of every trauma victim should be tested for occult blood.
Hematuria in the setting of a known bleeding disorder should
also be considered an indication of potentially life-threatening
coagulopathy.
 Hematuria 215

BEDSIDE
Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable or
distressed), or critical?
Pain, in the absence of trauma, may be seen with pyelonephritis,
nephrolithiasis, cystitis, or perineal irritation. Painless hematuria
can be seen in patients with glomerulonephritis, as well as tumor.

Airway and Vital Signs

What are the temperature, heart rate, blood pressure, and respira-
tory rate?
Hypertension may suggest renal artery stenosis with high renin
production or glomerulonephritis. Tachycardia may accompany
pain. Respiratory distress can be seen with renal failure and fluid
overload. Fever may indicate pyelonephritis.

Selective History and Chart Review

See Table 23.1.

Selective Physical Examination

HEENT Carefully examine the fundi for signs associated
with hypertension; look for periorbital edema
Neck Check for jugular venous distention and
thyromegaly
Lungs Rales and/or consolidation with hemoptysis
suggests Goodpasture syndrome or vasculitis
Cardiovascular Tachycardia, hypertension, or an S3 gallop
rhythm can imply fluid overload
Abdomen Flank pain, tenderness, fullness, masses, ascites
Genitourinary Discharge, edema, erythema, trauma
Skin Petechiae, purpura
Extremities Edema
HEENT, Head, Ears, Eyes, Nose, Throat.

Initial Management
The initial step should be to assess the patient’s urine output and
adjust fluid intake appropriately. Hematuria accompanied by oli-
guria requires fluid restriction to cover only insensible losses due
to the possibility of renal failure with resultant electrolyte distur-
bances, hypertension, and fluid overload. Hypertension must be
managed to avoid complications, and strict dietary protein and
potassium restriction may be necessary (see Chapter 24). In renal
insufficiency, dialysis may be necessary because of uremia, fluid
overload, or severe electrolyte disturbances.
216 Patient-Related Problems

TABLE 23.1 History and Chart Review

Question Etiology (from History)

Dysuria/frequency/fever? Urinary tract infection/urethritis
Abdominal or flank pain? Stones/pyelonephritis
Trauma/exercise? Direct trauma/exercise induced
Bleeding tendency? Coagulopathy
Impetigo/pharyngitis? Postinfectious glomerulonephritis?
Family History
Kidney disease? Alport syndrome/polycystic kidneys
Deafness? Alport syndrome
Stones? Nephrolithiasis
Other: medications/menses? Medication induced/fictitious

Laboratory Data
It is important to determine why the urine is colored. Hemoglobin,
myoglobin, and red blood cells all can cause red to brown urine, but
the differential diagnosis and management differs for each.
If the patient has true hematuria (red blood cells in the urine),
reviewing the elements of the urinalysis might provide some clues:
Finding Possible Diagnosis
Proteinuria Glomerular disease
White blood cells Urinary tract infection; interstitial nephritis
Crystals Stones
At the same time that management initiatives are begun, diag-
nostic tests should be performed:
Lab Test Possible Diagnosis
Creatinine/serum K+ Kidney dysfunction
C3 low (C4 normal) Postinfectious glomerulonephritis
C3 and C4 low Lupus
Antistreptolysin O, Postinfectious glomerulonephritis
anti-DNAse B
Urine calcium and Hypercalciuria (urine calcium/
urine creatinine creatinine ratio >0.2 mg/mg)
Urine culture Urinary tract infection
Complete blood count Hemolytic-uremic syndrome
Immunoglobulin A IgA nephropathy
(IgA)
Antinuclear antibody Lupus
Albumin/cholesterol Nephrotic syndrome
Hemoglobin Sickle cell disease
electrophoresis
 Hematuria 217

Imaging Test Possible Diagnosis

CT of abdomen/pelvis Trauma
Renal ultrasound or CT Stones
Renal or bladder ultrasound Anatomic abnormalities, tumor
CT, Computed tomography.

Management
The pediatric nephrologist should be called for:
• Red blood cell casts on microscopic examination (may need
biopsy)
• Significant proteinuria (may need biopsy)
• Hypertension, edema
• Abnormal renal function
• Renal structural abnormality
The pediatric urologist should be called for:
• Traumatic kidney or urethra injury
• Kidney stones with urinary infection or fevers
• Tumors

Summary
Hematuria, although rarely presenting a major threat to life, may
occur in a variety of conditions associated with significant morbid-
ity. As with many conditions, a diagnostic work-up should be per-
formed simultaneously with management of any potentially
associated problems, such as hypertension or severe edema.
 CHAPTER

24
Hypertension
Hema Krishna, MD

Hypertension is a very common problem in adults, but it is rela-

tively rare in children. The normal ranges for systolic and diastolic
blood pressure differ based on age and height percentile, thus mak-
ing the definition of “high blood pressure” variable. Nomograms
have been developed by conducting blood pressure screening pro-
grams in very large populations of “normal” children. Persistent
measurements (three or more) above the 95th percentile have gen-
erally been used to define hypertension. It is important to remem-
ber that a single measurement recording a high blood pressure does
not necessarily indicate hypertension.
Probably the most common “cause” of hypertension in children
is the use of an inappropriate cuff size. If the cuff is too small, an
artificially high reading is obtained. The cuff width should be two-
thirds the length of the upper part of the arm and have a bladder
that encircles the arm. Similarly, in obtaining a leg pressure, the cuff
should cover two-thirds the length of the thigh. A second common
“cause” of high readings is agitation and movement during the blood
pressure reading. Even in toddlers, a blood pressure reading can usu-
ally be obtained without undue agitation if the child is approached
slowly and patiently and reassured that the cuff will squeeze his or
her arm for only a short time. Ideally, the child has avoided stimulant
drugs or foods, has rested quietly for 5 minutes, and is seated with
the back and right arm supported, cubital fossa at heart level. Due to
the possibility of coarctation of the aorta resulting in falsely low
blood pressure readings, the right arm is the preferred site for obtain-
ing measurements. It is also important to remember that blood pres-
sures obtained in the hospital may be spuriously elevated because of
the child’s concomitant illness, anxiety or pain, or iatrogenic causes
such as medications. As such, an isolated blood pressure reading in
this setting should be interpreted with caution.
Approximately 80% to 90% of true hypertension in children
results from renal disease, but other treatable causes must also
be considered in any evaluation for hypertension.

218
 Hypertension 219

PHONE CALL
Questions
1. Why is the child hospitalized? What are his or her chronic
medical problems?
2. How high is the blood pressure and how was it obtained
(manual cuff or Dynemap, arm or leg)?
3. Is this a new finding? What have previous blood pressure read-
ings been?
4. Is the child having any other associated symptoms (chest pain,
tachycardia, sweating, shortness of breath, nausea, vomiting,
headache, vision/hearing changes, focal numbness or weakness,
urine output changes, edema)? Is the child anxious or in pain?
5. If the patient is female, could she be pregnant?
6. What medications has the child received?
Orders
Ask the nurse to have a manual blood pressure cuff of the appro-
priate size at the patient’s bedside.
If the child does not have an intravenous (IV) line, ask the nurse
to have IV supplies ready at the bedside.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Situations
requiring emergency evaluation include severely elevated blood pres-
sure (in children, systolic pressure above 200 and/or diastolic pressure
above 110 mm Hg), hypertension in a pregnant adolescent female,
altered mental status, focal neurologic deficits, or pain of any kind.

ELEVATOR THOUGHTS
Major etiologic categories of hypertension include the following:
Renal (glomerulonephritis, vasculitis, tumors, polycystic kid-
neys, renal scarring, renal artery stenosis)
Coarctation of the aorta (upper extremity pressure > lower
extremity pressure)
Increased intracranial pressure (ICP)
Eclampsia
Hyperthyroidism
Drugs (sympathomimetic agents, corticosteroids, oral
contraceptives)
Hypercatechol states
Although rare, some life-threatening conditions in children may
result in hypertension. Preeclampsia in a pregnant adolescent
is accompanied by proteinuria and edema. Headache is fre-
quently associated with the rise in blood pressure.
220 Patient-Related Problems

Catecholamine crisis may be precipitated by a number of

conditions:
Drug overdose
Especially common with cocaine, phencyclidine (PCP), and
amphetamines; overdose must be suspected regardless
of age
Drug interactions
Monoamine oxidase (MAO) inhibitors and indirect-
acting catechols (wine, cheese, ephedrine)
Tricyclic antidepressants and direct-acting catechols
(epinephrine, norepinephrine, pseudoephedrine)
Pheochromocytoma
Neoplasm overproducing catechols
Burns
Transient hypertension develops in some patients with second-
or third-degree burns because of high levels of circulating
endogenous catecholamines, renin, and angiotensin II.
Head trauma may result in increased ICP secondary to expand-
ing subdural or epidural hematomas or cerebral edema. The
need for the cerebral perfusion pressure to exceed ICP
causes the release of endogenous catechols to preserve cere-
bral blood flow.
The consequences of hypertension include intraventricular
hemorrhage in low-birth-weight premature infants. Cere-
brovascular accidents are less common in full-term infants
and older children but are still a possible complication of
extreme hypertension. Hypertensive encephalopathy can
include nausea, vomiting, headache, lethargy, confusion,
visual disturbances, and seizures.

MAJOR THREAT TO LIFE

The major immediate threat to life is the rapid increase in blood
pressure that can occur with eclampsia, drug ingestion, and head
injuries, especially with extra-axial hemorrhage and hypertensive
encephalopathy.

BEDSIDE
Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable or
distressed), or critical (about to die)?
A patient having end-organ damage requires immediate action
to normalize the blood pressure. Such damage may include neuro-
logic complications such as encephalopathy, focal deficits, or sei-
zures; marked respiratory distress; renal insufficiency, possibly
 Hypertension 221

presenting as anuria or oliguria; or cardiac complications such as

heart failure or ischemia. Similarly, hypertension caused by sus-
pected aortic dissection (tearing chest pain radiating to the back,
possible cocaine use) must be treated rapidly. Patients with surpris-
ingly high blood pressure can appear quite comfortable and in little
or no distress.
Airway and Vital Signs
What is the blood pressure?
Check that the appropriate cuff size has been used and then pro-
ceed to take the blood pressure yourself from both arms and at least
one leg. Coarctation of the aorta is a common source of upper
extremity hypertension. The lower extremity blood pressure
should always equal or exceed the upper extremity blood pressure.
Comparison of upper and lower extremity blood pressures is the
definitive diagnostic technique for coarctation of the aorta.
What is the heart rate?
Bradycardia and hypertension in a patient not taking β-blockers
may indicate increased ICP. When high blood pressure is accom-
panied by irregular respirations, it is known as Cushing’s triad.
Tachycardia with hypertension is consistent with catecholamine-
mediated changes.
Is the patient in pain or anxious?
Pain is a very powerful stimulant for catecholamine release,
regardless of the source. A patient hospitalized after trauma may
have undiagnosed injuries, such as fractures or abdominal injuries,
that can be overlooked, especially if he or she is unconscious on
arrival and admission.
Selective History and Chart Review
What has the patient’s blood pressure been up to this point?
Any previous recorded blood pressure readings are useful to put
the patient’s current pressure into perspective. Remember, how-
ever, that how and where the pressure was obtained are not usually
recorded, thus making the previous results less reliable as a com-
parison. A normal leg pressure may be reassuring except in an
infant or child with coarctation of the aorta, whose arm pressure
may be significantly higher.
What risk factors does the patient have for high blood pressure?
Does the patient have a history of heart disease?
Could the patient be pregnant? Is a drug ingestion a possibility?
Does the patient have a history of renal disease, especially reflux
nephropathy, chronic or recurrent pyelonephritis, nephrotic syn-
drome, or any of the glomerulonephropathies?
Does the patient have a recent history of sore throat or upper
respiratory symptoms?
222 Patient-Related Problems

Postinfectious glomerulonephritis is a common cause of sudden

hypertension in children. Probably 80% to 90% of hypertension is a
complication of infectious and/or inflammatory renal disease. Pri-
mary injury to the renal arteries can occur after umbilical artery
cannulation in a newborn and results in altered renal perfusion
and high renin production. Proximal tubular disease can result
in poor sodium excretion and inappropriate loss of bicarbonate
with poor urine acidification.

Selective Physical Examination

Does the patient have evidence of a hypertensive emergency?
HEENT Assess the fundi for hypertensive changes
(generalized or focal arteriolar narrowing,
hemorrhages, exudates). Papilledema is an
ominous and late finding and is the hallmark
of malignant hypertension and hypertensive
encephalopathy.
Neck Jugular venous distention may suggest heart
failure; thyromegaly may indicate
hyperthyroidism.
Respiratory Rales, pleural effusion (congestive heart failure
[CHF])
Cardiovascular Increased precordial activity, loud S2
(pulmonary hypertension), S3 gallop (CHF),
continuous murmur in the back (collaterals
secondary to coarctation of the aorta), weak or
absent femoral pulses, brachiofemoral delay,
diffusely weak or absent pulses (Takayasu
arteritis).
Abdomen Presence of bruits over the kidneys (renal artery
stenosis), enlarged kidneys (ureteropelvic
junction obstruction, renal vein thrombosis).
Neurologic Confusion, lethargy, headache, visual
disturbances, delirium, agitation, focal
neurologic signs.
HEENT, Head, Eye, Ears, Nose, Throat.

Management
Remember, the object is to treat the patient, not a number. Hyper-
tension must be viewed in the context of the entire patient. If the
patient is asymptomatic, there is less urgency to normalize the blood
pressure than if he or she is encephalopathic. There is a very real risk
of overshooting the mark during acute reduction of blood pressure
in patients with long-standing hypertension and high levels of auto-
regulated cerebral blood flow. Do not treat a blood pressure reading!
Treat the condition underlying it or associated with it.
 Hypertension 223

Very High Blood Pressure

Emergency No emergency

Funduscopic examination

Hemorrhages, Arteriolar
exudates, narrowing only
papilledema

Lower BP
over minutes Lower BP Lower BP
(IV medications over hours slowly
as per text) (IV or oral) (over days orally)
FIGURE 24.1 Approach to the management of very high blood
pressure (BP).

True emergencies require special management. Such emergen-

cies include eclampsia, intracranial hemorrhage, and hypertensive
encephalopathy (Fig. 24.1).
It is important to involve your senior resident and an attending
physician in such crisis situations and to inform the pediatric
intensive care unit (PICU) that your patient requires transfer to
a higher level of care and monitoring. No matter how “comfort-
able” you may feel with managing hypertension, it is important
to recognize the critical condition of these patients and the many
potential complications inherent in their management.

Hypertensive Encephalopathy
Hypertensive encephalopathy is almost always accompanied by
papilledema, retinal hemorrhages, and exudates. Focal neurologic
signs, although unusual early on, suggest the presence of a stroke. It
is important to remember that lowering the blood pressure precip-
itously can cause a stroke as well as syncope.
1. Transfer the patient to the PICU for electrocardiographic
(ECG) and intra-arterial blood pressure monitoring.
2. While the transfer is being arranged, an initial dose of oral nifed-
ipine can be administered and adequate IV access obtained.
3. If there is evidence of hypertensive encephalopathy, IV infu-
sions of medications such as nitroprusside may be useful
because they can be titrated gradually. It is absolutely necessary
to have intra-arterial monitoring to use such medications safely.
4. Labetalol, a combination α- and β-blocking agent, may also be
administered and can be particularly useful in a patient whose
hypertension is mediated by overproduction of endogenous
224 Patient-Related Problems

catecholamines or increased sympathetic tone. Bradycardia

may result, and if the patient has a history of reactive airway
disease, the respiratory findings must be monitored closely.
5. Once the blood pressure is under control by parenteral means, a
suitable oral medication regimen must be initiated in order to
discontinue the IV medications and allow the patient to leave
the PICU.
Malignant Hypertension
Unless malignant hypertension is accompanied by other emer-
gency conditions such as encephalopathy, control of blood pres-
sure can be accomplished more gradually. Control of blood
pressure must often be pursued while a search for the cause is
ongoing. As you are gaining control of the blood pressure, begin
to arrange the workup to document renal perfusion and function
(ultrasonography with Doppler flow study of the renal artery and
vein, renal arteriography if necessary, dimercaptosuccinic acid
[DMSA] renal scan with or without captopril challenge, blood urea
nitrogen, creatinine, urinalysis, creatinine clearance, electrolytes,
calcium, phosphate), echocardiogram, serum and urine steroid
metabolite levels, and serum renin and angiotensin II levels. Espe-
cially if renal disease is suspected, include streptococcal antibody
(antistreptolysin-O [ASO], anti-DNAse B) and complement (C3
and C4) studies.
Preeclampsia and Eclampsia
A pregnant adolescent presents a variety of special problems and
can be difficult to manage. Hypertension poses a risk to both the
adolescent and her unborn fetus. Obviously obstetric consultation
is required and, frequently, transfer to a labor and delivery unit.

Intracranial Hemorrhage
Your index of suspicion should be high in the setting of a child with
altered mental status, suspicion of child abuse, focal neurologic
signs, or associated bradycardia. Subarachnoid bleeding and sub-
dural or epidural hemorrhage can cause increased ICP and second-
ary hypertension. Intraparenchymal bleeding is a risk in children
with known coagulopathies and requires close monitoring and a
judicious approach.
Catecholamine Crisis
Pheochromocytoma presents the classic syndrome of pallor, palpi-
tations, and diaphoresis associated with intermittent and alarm-
ingly high blood pressure. Other circumstances that can mimic
this syndrome include drug ingestion, especially of cocaine and
PCP. Food (cheese), drug (ephedrine), and drink (wine)
 Hypertension 225

interactions with MAO inhibitor antidepressant medications can

result in similar findings, as can the interaction of tricyclic antide-
pressants with pseudoephedrine in over-the-counter cold prepara-
tions. MAO inhibitors are not widely used in children. Symptoms
of catecholamine crisis should prompt immediate transfer to the
PICU for close ECG and intra-arterial monitoring. Besides nitro-
prusside and labetalol, phentolamine mesylate, a powerful direct
β-blocker, may be given to decrease systemic vascular resistance
(afterload) and venous capacitance by directly relaxing smooth
muscle. Alpha blockade is especially important with cocaine and
PCP ingestion because of the generalized increase in sympathetic
nervous system activity that most children exhibit. When amphet-
amines have been taken, psychosis and hyperactivity may require
the use of chlorpromazine (Thorazine) or haloperidol (Haldol) to
control hallucinations and delirium.

Summary
Hypertension is unusual in children and must be taken seriously.
Be sure that the correct cuff size has been used and that the con-
ditions for checking the blood pressure have been optimized. Take
four-extremity blood pressure readings manually, and carefully
record the results to rule out coarctation of the aorta. Acute hyper-
tension implies an intracranial process, drug ingestion or interac-
tion, or another source of catecholamine overproduction. Treat
acute hypertension carefully and always in the context of the entire
patient. Rapid normalization of blood pressure can itself precipitate
problems.
 CHAPTER

25
Hypotension and Shock
Amanda A. Wenzel, MD

A variety of disease processes in pediatric patients follow a com-

mon pathway of hypotension, decreased tissue perfusion, acidosis,
and shock. The physician’s task is to quickly assess the magnitude
of the problem, intervene to prevent progression of shock, and dis-
cover the underlying cause of the hemodynamic compromise. This
may seem like a tall order, but remember that fundamentally the
blood pressure must be adequate to perfuse the brain, heart, and
kidneys. Accordingly, assessment of the child’s mental status,
pulses and perfusion, and urine output will provide the informa-
tion needed. Remember too that blood pressure is preserved by
a variety of mechanisms and falls only as a late consequence; there-
fore early recognition of impending hypotension is essential to
appropriate management.

PHONE CALL
Questions
1. How old is the patient?
2. Why is the patient in the hospital?
3. What is the blood pressure? What method of measurement was
used and from what site on the body was the blood
pressure taken?
4. What has been the blood pressure trend over the past
several hours?
5. What are the heart rate, temperature, and respiratory rate?
6. What is the child’s mental status (agitated, somnolent,
unresponsive)?
Orders
1. If the child does not have an intravenous (IV) line, the largest
possible IV line should be started immediately. Normal saline

226
 Hypotension and Shock 227

or lactated Ringer’s solution, 10 to 20 mL/kg, should be admin-

istered over a period of 5 to 10 minutes by IV push if necessary.
2. Oxygen should be administered.
3. If the child is not on a cardiorespiratory monitor, one should be
obtained immediately. Request frequent repeat measurements
of blood pressure (every 5 minutes).
4. If the child is febrile but alert enough to take medication by
mouth, acetaminophen or ibuprofen should be given. Infants
or very small children may be given rectal acetaminophen if
they cannot take anything by mouth.
Inform RN
Tell the RN, “I will arrive at the bedside immediately.”
There should be no delay in seeing a child with impending
shock.

ELEVATOR THOUGHTS
Shock is caused by the following conditions:
Sepsis
Hypovolemia
Cardiogenic causes
Anaphylaxis
Adrenal insufficiency
Trauma
Toxins
Two formulas are useful in considering the cause of
hypotension:
Blood pressure ðBPÞ ¼ cardiac output ðCOÞ
 systemic vascular resistance ðSVRÞ
Cardiac output ðCOÞ ¼ heart rate ðHRÞ  stroke volume ðSVÞ
Hypotension results from a decrease in either cardiac output or
systemic vascular resistance (SVR), and cardiac output is reduced by
a decrease in heart rate or stroke volume. Sepsis and anaphylaxis
cause hypotension as a result of systemic vasodilatation (decreased
SVR) and relative hypovolemia (because of capillary leak), both of
which decrease cardiac output. Hypovolemia decreases cardiac fill-
ing and lowers stroke volume, thereby leading to tachycardia and
ultimately resulting in decreased cardiac output and hypotension.
Cardiogenic causes include decreased stroke volume because of
decreased ejection fraction, as in dilated cardiomyopathies, dys-
rhythmias, and cardiac ischemia; decreased heart rate, as in heart
block; restriction of cardiac filling (and therefore stroke volume),
228 Patient-Related Problems

as in hypertrophic states, mitral stenosis, restrictive cardiomyopathy,

and pericardial tamponade; and loss of systemic output via left-
to-right shunts when pulmonary vascular resistance is less than SVR.
The most common cause of decreased cardiac output is lack of
intravascular volume. This can be caused by external losses, as seen
with vomiting and diarrhea, or by internal losses from third spacing,
as seen with intestinal obstruction or after major abdominal surgery.

MAJOR THREAT TO LIFE

Shock is the major threat to life. Hypotension becomes life-
threatening when there is evidence of inadequate end-organ perfu-
sion. Making the diagnosis of shock or impending circulatory
collapse is not usually difficult, but treating shock and its underly-
ing cause can be a challenge. The duty of the on-call physician is to
prevent end-organ damage and to find and correct the cause of
hemodynamic compromise.

BEDSIDE
Quick-Look Test
Does the patient look well (comfortable), sick (uncomfortable or
distressed), or critical (about to die)?
A child who is hypotensive but not in shock appears quite well.
However, as soon as perfusion of vital organs is compromised, the
patient appears quite ill. The blood pressure may be normal in a
child in shock, so attention to end-organ function is of utmost
importance.
Airway and Vital Signs
Is the airway clear?
If the child’s mental status is compromised, the ability to protect
the airway may be impaired. Airway support should be readily at
hand for any patient in shock.
Is the child ventilating adequately?
Children in shock are often tachypneic (to blow off accumu-
lated CO2), grunting (to provide positive end-expiratory pressure
and prevent atelectasis), and retracting (to maximize tidal volume).
Assess the respiratory rate, aeration, breath sounds, and chest
movement. If the work of breathing is excessive, intubation and
ventilatory support are indicated.
Assess the Circulation
1. Mild hypotension may be manifested as postural dizziness.
Assess for postural hypotension by checking the pulse and
 Hypotension and Shock 229

blood pressure in the supine position and again after the patient
has been standing for 3 minutes. A postural rise in heart rate of
more than 15 beats per minute, a fall in systolic blood pressure
of more than 15 mm Hg, or any decrease in diastolic blood
pressure suggests hypovolemia.
2. What is the heart rate? Sinus tachycardia is the first response to
stress, hypovolemia, sepsis, and decreased myocardial function
in infants and children. Nonsinus tachydysrhythmias can lead
to circulatory collapse and shock; therefore an electrocardio-
graphic rhythm strip should be checked to rule out a supraven-
tricular tachydysrhythmia (see Chapter 22). Bradycardia in the
setting of shock is an ominous, life-threatening sign of circula-
tory collapse. If bradycardia is present, make sure that the
patient is not in heart block and therefore unable to respond
to his or her own catecholamine signals (Fig. 25.1). Vagal stim-
uli can produce profound bradycardia and even asystole in
young patients. Episodes are usually short-lived and respond
promptly to laying the patient supine with the legs elevated
or even in the Trendelenburg position. Prolonged bradycardia
should respond to IV atropine (0.02 mg/kg).
Children who are receiving β-blockers or calcium channel
blockers or who have ingested such medications accidentally
are not able to respond normally to their intrinsic catechol-
amine signals. Likewise, children with sick sinus syndrome
may not respond to β1-chronotropic stimulation and do not
compensate with tachycardia in response to stress.
3. Is the child in shock? This should take less than 30 seconds to
determine, and shock, if present, requires immediate action
regardless of the underlying cause.

Vital signs Repeat immediately (include urine output)

Cardiovascular Heart rate, blood pressure, pulse pressure,
pulse quality, capillary refill (normal, <2 s)
Neurologic Mental status

Shock is a clinical diagnosis. No single sign or test establishes

the diagnosis. The constellation of low systolic blood pressure
for age, cool clammy extremities, poor capillary refill, acrocya-
nosis, altered mental status (confusion, delirium, lethargy,
coma), and decreased urine output (number of wet diapers in
infants) indicates progressive circulatory compromise.
4. Is the child febrile? Obviously fever frequently means sepsis, and
prompt administration of antibiotics may be indicated. Fever also
results in peripheral vasodilation, which decreases SVR and causes
decreased cardiac output. A neonate with sepsis may not have sig-
nificant fever and may indeed be hypothermic.
 230
Patient-Related Problems
FIGURE 25.1 See legend next page.
 Hypotension and Shock 231

Selective Physical Examination

Determine the severity of the hypotension by assessing volume sta-
tus and end-organ function. Cardiogenic shock may be manifested
as volume overload, but in general most shock states include evi-
dence of hypovolemia.
Vital signs Repeat and document regularly
HEENT Pupils (narcosis), mucous membranes, tears,
fontanelle, periorbital edema, lip edema
Neck Jugular venous distention (congestive heart
failure [CHF], tamponade), deviation of the
trachea (tension pneumothorax)
Respiratory Grunting, retracting, stridor, or wheezing
(anaphylaxis); rales (CHF)
Cardiovascular Displaced point of maximal impulse (dilated
cardiomyopathy), distant heart sounds
(pericardial effusion, myocarditis), gallop
rhythm, holosystolic regurgitant murmurs
(ventricular septal defect, mitral or tricuspid
regurgitation), loud S2 (pulmonary
hypertension), weak femoral pulses and/or
brachiofemoral delay (coarctation of the
aorta). Also assess perfusion, skin
temperature, and color.
HEENT, Head, Ears, Eyes, Nose, Throat.

Algorithm for discerning the cause of decreased perfusion.

Although the heart rate is usually increased in response to poor
systemic perfusion, the presence of bradycardia should provoke
a search for a primary dysrhythmia (e.g., heart block) or auto-
nomic dysfunction (e.g., spinal cord trauma) or raise concern that
there is severe myocardial failure. Alternatively, when the heart
rate is increased, one should attempt to determine whether signs
of systemic venous engorgement are present. When the heart’s
silhouette is enlarged and there is venous distention, it is impor-
tant to distinguish whether the heart is well filled and suffering
from poor inotropic function or there is impedance to venous
return (e.g., cardiac tamponade or tension pneumothorax). In
these circumstances, an echocardiogram is an invaluable tool.
When there is tachycardia with no sign of venous distention,
inadequate perfusion can be caused by decreased preload and
insufficient cardiac filling (e.g., hemorrhage or dehydration) or
by diminished effective circulation, as in sepsis (with diffuse
inflammation, venodilatation, and maldistribution of blood
flow). It is also important to recognize that many of the problems
that cause decreased perfusion can do so by more than one mech-
anism. Sepsis is a good example of a process that can diminish pre-
load and produce myocardial failure simultaneously. (From
Behrman RE: Nelson Textbook of Pediatrics, 15th ed. Philadelphia,
WB Saunders, 1995, p 248.)
232 Patient-Related Problems

Abdomen Hepatosplenomegaly, ascites, distention and/or

tenderness (ischemia or perforation)
Extremities Presacral and/or ankle edema (CHF); cold,
clammy hands and feet; poor capillary refill
Neurologic Mental status
Skin Urticaria (anaphylaxis), skin turgor, burns or
wounds, anasarca (capillary leak secondary to
sepsis), petechiae or purpura (sepsis and
disseminated intravascular coagulopathy)

Selective History and Chart Review

Anaphylaxis has a preceding inciting event, such as food, drug, or
radiologic dye exposure, with an abrupt onset and rapidly progres-
sive course. Angioedema and urticaria are often present, as well as
wheezing with or without stridor. A neonate with sepsis may have
been exposed to an older child with a specific infectious illness.
Children with known immunologic compromise—such as HIV
infection, sickle cell (SS) disease, asplenia, or neoplasms—may
be more susceptible to septic shock. In addition to sepsis or distrib-
utive shock, cardiogenic shock may develop in children with
known cardiac disorders. A history of significant steroid therapy
for autoimmune illnesses or cystic fibrosis increases susceptibility
to infection. Rapid withdrawal of steroids may precipitate adrenal
crisis and subsequent shock. Recent surgery can result in hemor-
rhage and hypovolemia. Spinal trauma may lead to neurogenic
shock (disturbance in vasomotor tone). Children with dehydration
secondary to diarrhea must be assessed carefully. The fact that the
patient is hospitalized does not mean that the diarrhea is gone.
Children in diapers must be carefully assessed for intake and out-
put. Watery diarrhea can be confused with urine output because
modern diapers absorb extremely well. Make sure that the docu-
mented urine output is really urine and not watery stool.

Management
What immediate measures must be taken to restore circulatory
function and prevent progression of shock and end-organ
hypoperfusion?
Normalize intravascular volume. The heart needs preload to
function high on the Frank-Starling curve and maximize ejection
fraction and therefore stroke volume. Even in some forms of car-
diogenic shock, volume expansion is indicated initially. (Children
with dilated or hypertrophic cardiomyopathy require high filling
volume and pressure to compensate for low ejection fraction and
high end-diastolic volume or for poor diastolic compliance with
high end-diastolic pressure.)
 Hypotension and Shock 233

Volume expansion should start by positioning the patient

supine with the legs raised 15 degrees or in the 15-degree head-
down Trendelenburg position. In infants, volume replacement
should be in the form of either normal saline or other isotonic solu-
tion; 10 to 20 mL/kg should be administered over a 5- to 10-minute
period by IV push if necessary. Reassess volume status and end-
organ function (mental status, heart rate, urine output, perfusion)
after each intervention.

Anaphylactic Shock
If the patient is in anaphylactic shock, treat rapidly as follows:
1. Remove inciting antigen if possible (e.g., medication infusion).
2. Epinephrine, 0.01 mg/kg as a 1:1000 solution intravenously
immediately (intramuscularly only if necessary)
3. Oxygen via OxyMask
4. 10 to 20 mL/kg of normal saline bolus (repeat as necessary)
5. Diphenhydramine, 1 mg/kg intravenously (maximum 50 mg)
administered over 5 minutes
6. Methylprednisolone sodium succinate, 1 to 2 mg/kg intrave-
nously immediately and every 6 hours; steroids have a slower
onset of action but may be helpful in mitigating or preventing
the biphasic allergic reaction that can occur 6 to 8 hours after
the initial reaction.
7. Other adjunctive therapies (H1 or H2 blockers [e.g., ranitidine],
β2 agonists)
8. Note that although adjunctive therapies are helpful, they are not
lifesaving; therefore administration of epinephrine takes prece-
dence. Treat signs of hypovolemia and simultaneously assess
the magnitude of end-organ damage by obtaining an arterial
blood gas reading.

Cardiogenic Shock
Although much less common in pediatric patients, cardiogenic
shock can occur because of intrinsic cardiac disease (including
structural lesions and dysrhythmias), injury to cardiac muscle
(myocardial infarction, trauma, myocarditis), and extrinsic pro-
cesses such as sepsis. A 12-lead electrocardiogram and chest radio-
graph obtained promptly will help in assessing the cause and
gravity of cardiac failure. It is critical that the physician determine
whether the child is preload-dependent to maximize cardiac out-
put, as mentioned earlier. Other clues should exist to suggest con-
gestive heart failure, including jugular venous distention,
hepatomegaly, a history of congenital heart disease, and differential
pulses. Other conditions, however, can be manifested similarly and
result in hypotension and shock. Cardiogenic shock may have to be
managed with inotropic support, diuresis, afterload reduction, or
234 Patient-Related Problems

TABLE 25.1 Catecholamines Used for Cardiopulmonary

Resuscitation

Direct Positive Positive

Pressor Inotrope Chronotrope Vasoconstrictor Vasodilator
Dopamine ++ + +/– ++a ++b
Dobutamine ++ +/– – – +
Epinephrine +++ +++ +++ ++a –
Norepinephrine +++ +++ +++ +++ –
a
High dose.
b
Primarily splanchnic and renal in low doses (3 to 5 μg/kg/min).
From Behrman RE, Kliegman R: Nelson Essentials of Pediatrics, 7th ed. Philadelphia, WB
Saunders, 2015.

some combination of these. Consultation with a cardiologist may

be most helpful. Table 25.1 lists some of the common inotropic
agents that may be used.
Acute Pericardial Tamponade
Tamponade physiology results in poor cardiac filling, which com-
promises stroke volume, elevates right-sided pressure, and causes
jugular venous distention and tachycardia (Beck’s triad: arterial
hypotension, jugular venous distention, and soft or distant heart
sounds). Tamponade can be seen in postoperative cardiac patients,
children with viral pericarditis, and patients with blunt chest
trauma; it can also occur after cardiac catheterization and with
inflammatory conditions such as systemic juvenile idiopathic
arthritis and systemic lupus erythematosus. Suspect tamponade
in patients who appear well hydrated, nonvasodilated, and poorly
perfused. They may have a narrow pulse pressure and a pulsus
paradoxus of greater than 10 mm Hg during relaxed respirations
(Fig. 25.2). If the patient has hypotension that is unresponsive to
volume and tamponade is suspected, a code should be called and
preparations made for emergent pericardiocentesis. In this proce-
dure, pericardial fluid is removed to relieve the tamponade. The
area around the xyphoid process should be prepared in sterile fash-
ion. The skin and subcutaneous tissues should be anesthetized with
1% lidocaine. An 18- or 20-gauge angiocatheter can be placed on a
three-way stopcock and attached to a 20-mL syringe. The angio-
catheter is then inserted lateral to the xyphoid, aiming for the left
shoulder and aspirating as it is inserted and advanced slowly. Once
straw-colored fluid is visualized, the catheter can be threaded and
the needle withdrawn. The stopcock can be attached directly to the
catheter and the fluid aspirated with the syringe.
 Hypotension and Shock 235

FIGURE 25.2 Determination of pulsus paradoxus. BP, blood pres-

sure. (From Marshall SA, Ruedy J: On Call: Principles and Proto-
cols, 4th ed. Philadelphia, Elsevier, 2004, p 266.)

Tension Pneumothorax
A rather rare entity, tension pneumothorax precipitates circulatory
collapse by inhibiting cardiac filling and therefore stroke volume
because of high intrathoracic pressure, which decreases venous return
to the heart. The result is jugular venous distention, severe dyspnea,
unilateral hyper-resonance, tracheal deviation away from the affected
side, and unequal breath sounds. Trauma is the most common cause,
but spontaneous pneumothorax can progress to tension pneumotho-
rax as well. Tension pneumothorax is a life-threatening emergency,
and there may not be time to wait for a radiograph. If the patient
has hypotension that is unresponsive to volume and tension pneumo-
thorax is suspected, a code should be called and preparations made for
236 Patient-Related Problems

emergent decompression. Oxygen is then administered immediately

and an 18- to 20-gauge needle prepared on a three-way stopcock with
a 20-mL syringe. The second intercostal space of the affected side is
quickly scrubbed with povidone-iodine (Betadine), alcohol, or chlor-
hexidine and the needle is inserted at the midclavicular line in a
slightly lateral direction, aspirating the while. Insertion should be just
above the third rib to avoid damaging any neurovascular structures
coursing below the rib. The fifth intercostal space at the midaxillary
line may also be used. When air is aspirated, the syringe is filled
and flushed out with the stopcock; this is repeated until air is no longer
freely aspirated. These maneuvers should rapidly improve the
patient’s clinical condition. Tension pneumothorax is a true medical
emergency and requires prompt, definitive action. Follow-up treat-
ment with a chest radiograph is then mandatory to ensure resolution
of the pneumothorax.

Pulmonary Embolism
Though rather unusual, pulmonary embolization can occur in
children, especially in adolescents, and is a cause of sudden, severe
hypotension and circulatory collapse. Because of the acute
obstruction to pulmonary blood flow, left atrial filling decreases,
which causes compromised left ventricular filling, decreased
stroke volume, acute tachycardia, tachypnea, and chest pain. Car-
diac output can rapidly decrease, leading to shock. Preexisting
coagulopathy, immobility secondary to trauma or burns, birth
control pills, and indwelling central venous catheters may predis-
pose young patients to pulmonary embolization. Fat emboli are a
well-known, serious consequence of long-bone fractures, espe-
cially of the femur. Embolization or thrombosis and subsequent
infarction may also result in the acute chest syndrome of hemo-
globin SS disease.
The pathophysiologic consequences of these diverse processes
are essentially the same: ventilation/perfusion mismatch, hypox-
emia with or without hypercapnia, increased pulmonary vascular
resistance, decreased left heart filling, and decreased cardiac out-
put. Secondary decreased perfusion and tissue hypoxemia result
in acidemia, which worsens the ventilation/perfusion mismatch
(intrapulmonary shunt), and an elevation in pulmonary vascular
resistance.
Hypotension from pulmonary embolism is a medical emer-
gency. If it is suspected as the cause of hypotension, immediate
therapy can be lifesaving. A code should be activated and evalua-
tion for the use of thrombolytic therapy should begin. Laboratory
values should be obtained (complete blood count, prothrombin
time, partial thromboplastin time, D-dimers or fibrin split products,
and a type and screen) and an emergent computed tomography
(CT) of the chest as well as echocardiogram should be performed
 Hypotension and Shock 237

if available. Acute therapy for pulmonary embolism is largely sup-

portive and includes oxygen with positive-pressure ventilation if
necessary, volume and inotropic support as needed, and close
monitoring of arterial blood gases for gas exchange and acid-base
status. The diagnosis may be suggested by the history and chest
radiograph. Definitive diagnosis requires chest CT, pulmonary per-
fusion scanning, ventilation scintiphotographic studies, and/or
pulmonary angiography. Any patient with hypotension from a pul-
monary embolism and/or needing thrombolytic therapy requires
monitoring in the intensive care unit. Patients with SS disease
require at least partial exchange transfusion to lower their percent-
age of SS hemoglobin.

Hypovolemia
In infants and children, fluid losses from diarrhea, vomiting, exces-
sive sweating, acute blood loss, chronic gastrointestinal bleeding,
polyuria, and third-space losses (capillary leak syndromes, includ-
ing sepsis and systemic inflammatory response syndrome [SIRS])
all can result in hypovolemia and shock. A number of medica-
tions—including diuretics, β-blockers, calcium channel blockers,
acetylcholinesterase inhibitors, and other antihypertensive medica-
tions—can lead to relative or actual hypovolemia. Normal compen-
satory mechanisms for hypovolemia include increased sympathetic
tone with resultant tachycardia, increased myocardial contractility,
and peripheral vasoconstriction. Increased myocardial work and
oxygen consumption constitute the price paid for this compensa-
tion. Therefore the first goal of therapy is to reduce myocardial
work by fluid resuscitation.
Practicality, availability, and the type of fluid losses that the
child has suffered should guide the choice of fluid for resuscitation.
Normal saline and lactated Ringer’s solution are readily available
and economical; however, crystalloid solutions have a greater pro-
pensity for leaking from the tissues; thus colloid solutions are
sometimes indicated, as in infants and patients with obvious cap-
illary leak syndromes (Table 25.2). Likewise, a multiple-trauma
patient may require whole blood during resuscitation. Children
in severe hypovolemic shock may require a fluid bolus of 60 to
80 mL/kg within the first 30 to 60 minutes. However, the risk of
fluid overload must be continually reassessed.
Volume expansion may not be enough to sustain cardiac output
and perfusion. In this case inotropic and/or vasopressor support is
indicated. Table 25.1 lists the inotropic agents used. Dopamine is
generally given first; however, epinephrine may be more effective
and can be given safely through a peripheral IV or intraosseous
(IO) catheter.
The use of inotropic and vasopressor therapy generally requires
transfer to an intensive care unit for appropriate, usually invasive
238 Patient-Related Problems

TABLE 25.2 Intravenous Fluids Available for Pediatric

Volume Resuscitation

Crystalloids Colloids
0.9% sodium chloride 5% human serum albumin in 0.9% sodium chloride
Lactated Ringer’s 25% human serum albumin in 0.9% sodium
solution chloride
Hypertonic saline (3%) Fresh frozen plasma
Whole blood

From Behrman RE, Kliegman R: Nelson Essentials of Pediatrics, 2nd ed. Philadelphia, WB
Saunders, 1994, p 118.

monitoring. It is important to remember that inotropic support is

not a replacement for volume resuscitation.

Sepsis
Sepsis is defined as any life-threatening organ dysfunction resulting
from the body’s impaired response to infection. Any kind of infec-
tion can result in circulatory collapse and shock; therefore early
recognition and goal-directed therapy are key to decreasing mor-
bidity and mortality.
As soon as sepsis is recognized by clinical and/or supporting
laboratory evidence, IV access should be established (IO access
may also be used if an IV cannot be placed). Continuous pulse
oximetry is put in place and cardiorespiratory monitoring initiated
if not already done. Oxygen supplementation should be applied,
and support with respiratory ventilation as appropriate. Once IV
access has been established, fluids should be initiated. Boluses of
20 mL/kg isotonic fluid are continued until perfusion improves
or signs of fluid overload (rales, hepatomegaly) develop. At the
same time, hypoglycemia or electrolyte derangements (potassium,
magnesium, and/or calcium), if present, are corrected and empiric
broad-spectrum antibiotics are begun. This should all be done rap-
idly, within the first 15 minutes of recognition.
If the patient requires more than 60 mL/kg of fluid without
improvement in hemodynamics, this may indicate a need for the
initiation of IV pressors and closer hemodynamic monitoring.
A code should be called and the patient should quickly be trans-
ferred to the pediatric intensive care unit (PICU) for further eval-
uation and management.

Adrenal Crisis
Adrenal insufficiency is characterized by hyponatremia, hyperka-
lemia, acidosis, hypoglycemia, and hypotension or shock. It may
 Hypotension and Shock 239

occur in combination with other causes of shock (e.g., meningococ-

cemia and secondary adrenal infarction, sepsis in a steroid-
dependent patient). In addition to fluid resuscitation, if adrenal
crisis is suspected, prompt treatment with IV hydrocortisone, 1
to 2 mg/kg, can result in dramatic improvement. Continuation
of IV hydrocortisone, 25 to 250 mg/day divided into two or three
doses, is usually necessary. Hydrocortisone is preferred to other IV
steroid preparations because of its mineralocorticoid effect.

REMEMBER
1. Shock is a clinical diagnosis characterized by inadequate end-
organ perfusion and subsequent dysfunction. One must assess
its severity by noting the child’s mental status (brain), perfu-
sion, heart rate and blood pressure (heart), and urine output
(kidney). Blood pressure may be normal and the child may still
be in shock. Compensatory mechanisms (tachycardia, vasocon-
striction) will maintain blood pressure, but end-organ perfu-
sion will be inadequate.
2. Although the skin is not generally considered a “vital” organ, it
can provide valuable information regarding volume status and
tissue perfusion. Do not be misled or falsely reassured by a well-
perfused patient who has other manifestations of shock.
So-called warm shock is no less threatening and frequently
precedes circulatory collapse. (Be alert for a patient with tachy-
cardia, wide pulse pressure, and warm extremities.)
3. Hypovolemia is the most common cause of shock in infants and
children, with sepsis following close behind.
 CHAPTER

26
Lines, Tubes, and Drains
Stephen E. Wilkinson, MD

Nearly every child admitted to the hospital requires an intravenous

(IV) line, a catheter, a tube, or a drain of some sort. These devices
may be necessary for a variety of reasons, but each may also be a
source of trouble. Complications include soft tissue injury (includ-
ing to vascular and nervous tissues) and pain with placement,
blockages, leaks, and full malfunctions. This chapter discusses
the placement of a few of these devices, as well as a troubleshooting
approach to some of the problems that may arise while you are on
call. The placement of IV and intraosseous (IO) lines are discussed
in Appendix A. Chest tube placement is not outlined because this
will rarely need to be done at the bedside and critical care support
will perform or assist you with this procedure if an emergent need
arises. Similarly, techniques of central line placement are beyond
the scope of this book and are not discussed. Given the risks of
complication, placement of lines, tubes, and drains should be
supervised by attending physicians or appropriate fellows until
competency is recognized by your program and hospital. The
New England Journal of Medicine maintains an online archive
series entitled “Videos in Clinical Medicine”; the video clips there
are helpful reviews of procedures. Many hospitals have policies
governing lines, tubes, and drains; nursing staff often will be able
to locate these quickly.
This chapter is divided into the following sections:
IV lines:
• Infiltrated IV lines
• Medication reactions
Umbilical catheters
• Placement
• Obstructed umbilical arterial or venous catheter
Central lines
• Bleeding at the entry site
• Obstructed central line

240
 Lines, Tubes, and Drains 241

Nasogastric (NG) and enteral feeding tubes

• Placement
• Obstructed NG tube and enteral feeding tube
• Displaced NG tube and enteral feeding tube
Urethral catheters
• Placement
• Obstructed urethral catheter
• Gross hematuria in catheterized patients
Chest tubes
• Persistent bubbling in the water seal (air leak)
• Loss of fluctuation of the water seal (tube obstruction)
• Bleeding at the chest tube entry site
• Drainage of blood
• Dyspnea
• Subcutaneous emphysema
• Traumatic or accidental removal of a chest tube

Intravenous Lines: Infiltrated Intravenous

Given their frequency, the most common line issue you will likely face
is IV infiltrations. Extravasation of IV fluids or medications into peri-
vascular tissues has variable consequences based on the pH, other
redox potentials, osmolality of the infusion, and mechanism of action
of the medication being infused; these consequences range from tem-
porary discomfort to permanent tissue damage. In general, nonsevere
infiltrates are handled by nursing staff without house staff involve-
ment, so calls regarding infiltration should be treated seriously.

PHONE CALL
Questions
1. What fluid or medication was being infused?
2. What external signs are there of infiltration?
Orders
1. Stop the infusion, if not already stopped, but leave the line
in place.
2. Attempt to aspirate as much fluid as possible out of the line and
subcutaneous tissues. Do not flush the line.
3. Elevate the extremity above the heart to improve venous flow.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” If a nurse
feels a need to call about an infiltrated line, the patient should be
evaluated as soon as possible.
242 Patient-Related Problems

ELEVATOR THOUGHTS
How noxious is the fluid or medication that infiltrated?
The following lists are helpful, although not exhaustive:
1. Higher-risk medications: Vasopressors/inotropes, chemotherapy,
electrolytes (calcium, potassium, sodium bicarbonate, hypertonic
normal saline), hypertonic sugars (D12.5 or greater; mannitol;
total parenteral nutrition (TPN) with >900 mOsm/L), acyclovir,
hydroxyzine.
2. Intermediate-risk medications: Acetazolamide, amikacin,
amphotericin B, arginine, D10-D12.5, diazepam, doxycycline,
erythromycin, fosphenytoin, ganciclovir, lorazepam, midazo-
lam, morphine, ondansetron, nonionic radiology contrast,
pantoprazole, phenobarbital, phenytoin, potassium (60 mEq/
L or less), TPN (<900 mOsm/L).
3. Lower-risk medications: Most other fluids and medications.

MAJOR THREAT TO LIFE OR LIMB

• Tissue ischemia due to vasoconstriction.
• Tissue necrosis due to caustic medication-tissue chemical
interactions.

BEDSIDE
Quick-Look Test
Does the child appear to be in extreme pain? Is there any hemorrhage
at the infusion site?
Airway and Vital Signs
In general, the airway will be stable. Vital signs may be augmented
secondary to pain and anxiety.
Management
Again, the infusion should have been stopped and all possible fluid
should have been aspirated from the tissue. For medications that are
intermediate- or higher-risk medication, calling for rapid response
team assistance may expedite some medication availability and
administration. For any vasoconstrictive medications, subcutaneous
administration of phentolamine should be strongly considered to
improve blood flow; a sterile technique should be used if this is done.
For other higher-risk fluids and medications, talk with your pharma-
cist about antidotes. Cold packs may help with pain control (use
warm packs for vinca alkaloid chemotherapeutics). The severity of
damage from the infiltration should be assessed. Blistering, particu-
larly hemorrhagic blistering, erythema that is not blanchable, and
diminished or lost pulses are red flags indicating a need for surgical
 Lines, Tubes, and Drains 243

evaluation. For intermediate-risk fluids and medications, talk with

your pharmacist about hyaluronidase subcutaneous injections to
buffer the effects of the infiltrate; sterile technique should be used
if this is done. For low severity, lower-risk infusions, warm packs
may help to improve circulation and clearance of the infiltrate.
Umbilical Catheters: Umbilical Arterial Catheter
Placement
Umbilical arterial catheters (UACs) are placed in critically ill new-
borns to monitor blood pressure and for easy access for arterial blood
gases draws. They may also be used to obtain blood samples for
hematologic and chemistry studies, although this is not an indication
for placement. For infants with a body weight less than 1500 grams, a
3.5-French (Fr) catheter may be used. For larger infants, a 5-Fr cath-
eter is appropriate. Once in place, the tip of the catheter should rest in
one of two places. A “high UAC” will be in the aorta at the approx-
imate level of the diaphragm, at a level between the sixth and ninth
thoracic vertebrae on chest radiography. A “low UAC” will be just
above the iliac bifurcation of the aorta, between the second and
fourth lumbar vertebrae on an abdominal radiography. If the cath-
eter is inserted to a point between these two sites, it may obstruct one
or more of the major aortic branches (celiac axis, superior mesenteric
artery, renal arteries, inferior mesenteric artery).
Placement should proceed as follows:
1. Place the infant supine on a well-lit, prewarmed warming table.
2. Estimate the length of catheter to be inserted in the following
way:
a. Measure the length of the infant.
b. For a high UAC, the catheter is inserted one-third the
length of the infant.
c. For a low UAC, the catheter is inserted one-sixth the length
of the infant.
d. Note the marking on the catheter that corresponds to the
appropriate length. This mark will be aligned at the orifice
of the umbilical stump once the catheter is in place.
3. Restrain the infant’s legs by extending a diaper or small blanket
across the thighs and securing it to the warming table with tape
so that that the infant is not able to raise the legs.
4. Tie a heavy string or umbilical cord tape around the umbilical
stump just proximal to where the infant’s skin meets the soft
tissue of the cord. Ensure this is snug enough to prevent blood
from oozing out of the umbilical arteries.
5. Sterile technique should be used throughout the catheter’s
placement. Apply povidone-iodine to the entire umbilical
stump and the cord distally to the point where the cord is
clamped. Drape the infant’s abdomen so that only the stump
and cord are exposed.
244 Patient-Related Problems

6. Prepare the catheter by attaching a three-way stopcock and a

10-mL syringe that is filled with a heparinized solution. Fill the
entire length of the catheter with the solution, and then turn
the stopcock to the catheter off.
7. Using a scalpel, cut the cord transversely approximately 1 cm
above the string or tape-tie and remove the distally clamped cord.
This exposes the two umbilical arteries and umbilical vein in cross
section so that they can be visualized clearly. Leaving some of the
cord itself allows you to shave some off at a later time, should place-
ment be difficult or the distal umbilical arteries become frayed.
8. Grasp the wall of the cord with a hemostat in one hand and
pull up gently so that the cord is vertical and perpendicular
to the abdomen. (Alternatively, ask a nurse or colleague to
do this for you. This procedure is notably easier if a team
approach is possible.) With curved forceps in the other hand,
insert the tip of the forceps into the lumen of one of the umbil-
ical arteries. These arteries are constricted; therefore the lumen
may initially be difficult to visualize. Gently move the tip of the
forceps in a circular fashion to gradually dilate the lumen of the
artery. Patience is the key to success here, and rushing to dilate
the artery may result in fraying and destruction of the distal
end of the artery and make it impossible to insert the catheter
(Fig. 26.1).
9. Once the lumen is slightly dilated, both tips of the closed for-
ceps may be inserted and the artery dilated further by gently
opening the forceps to stretch the walls of the artery. As this
is being done, also gradually insert the tips of the forceps dee-
per and deeper into the lumen to dilate as far as possible.
10. When the artery has been dilated enough to accommodate the
umbilical catheter, grasp the catheter near the tip with forceps.
While holding the lumen of the umbilical artery open with the
other pair of forceps (again, this is much easier if two people
work together), insert the catheter tip into the artery and grad-
ually feed the catheter into the vessel with the forceps. Stop
when you have reached the estimated length (the marking that
you previously noted is positioned where the catheter enters
the lumen of the vessel) that places the tip at either a high level
(the level of the diaphragm) or a low level (just above the iliac
bifurcation of the aorta).
11. With successful catheterization, pulsating blood is usually visible in
the catheter. Opening the stopcock and aspirating on the
syringe should result in blood being easily drawn into the catheter.
12. While feeding the catheter into the umbilical artery, obstruction
may be encountered at the point where the vessels turn at the
umbilical wall or because of vasospasm. When obstruction
occurs, steady, gentle pressure on the catheter while pulling
 Lines, Tubes, and Drains 245

Catheter

Curved forceps
dilating the
vessel

Sterile
drape Umbilical tape

B
FIGURE 26.1 A, Position of the infant for umbilical vessel cathe-
terization. B, Procedure for inserting an umbilical catheter.
246 Patient-Related Problems

up on the umbilical stump often allows the catheter to pass. If it

does not pass, a false tract external to the lumen of the vessel may
have been created, and it may be necessary to withdraw the cath-
eter and try again. If, after several attempts, the catheter still can-
not be passed, it may be necessary to attempt insertion in the
other umbilical artery. Alternatively, shaving the cord again
with the scalpel may expose the artery proximal to the false tract
and allow you to attempt insertion again.
13. Placement of the catheter should always be confirmed with a
radiograph. If the sterility of the field and the catheter is main-
tained during radiography, the catheter may be adjusted if nec-
essary. Once positioning is confirmed, the catheter should be
secured to the cord with a suture and then taped in place.
14. It is common for pallor or cyanosis to appear in a lower
extremity after placement of a UAC. This occurs secondary
to vasospasm in response to the catheter and is often relieved
by warming the opposite leg. Warmth increases blood flow to
both extremities as a result of reflex vasodilatation.
Umbilical Catheters: Umbilical Venous Catheter
Placement
Umbilical venous catheters (UVCs) are placed for IV access and for
monitoring central venous pressures (CVPs). The umbilical vein is
a readily available site for IV access in a critically ill newborn. The
procedural steps for a UVC are identical to that for an umbilical
arterial catheter, although notably easier and faster to perform.
UVCs should be placed so that the tip lies within the inferior vena
cava, just above the diaphragm. To achieve this, the catheter should
be inserted one-sixth the length of the infant. The umbilical vein is
thin walled and does not usually require dilatation. Once identified,
the lumen is typically easily opened, and the catheter tip may pass
freely with gentle pressure. As with a UAC, proper positioning
should be confirmed with a radiograph and the catheter secured
with a suture and tape.

Umbilical catheters: obstructed umbilical arterial

or venous catheter

PHONE CALL
Questions
1. How long has the line been blocked?
2. What are the vital signs? Hypertension or narrowing of the
recorded pulse pressure may be a sign of a thrombus at the
tip of the arterial catheter.
 Lines, Tubes, and Drains 247

3. Is there adequate blood flow to the legs (capillary refill, color,

distal pulses)?
Orders
None
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” A blocked
umbilical catheter should be evaluated immediately.

ELEVATOR THOUGHTS
What causes an umbilical catheter to be blocked?
1. Thrombus at the catheter tip
2. Kinked or compressed tubing

MAJOR THREAT TO LIFE

• Ischemia of an extremity secondary to thrombosis of an
arterial line.
• Pulmonary or cerebral embolism (via a patent foramen ovale,
atrial septal defect (ASD), or ventricular septal defect (VSD)
secondary to thrombosis of a venous line.

BEDSIDE
Quick-Look Test
If the infant appears ill, suspect a complication such as emboliza-
tion or distal ischemia.
Airway and Vital Signs
As noted earlier, hypertension or a narrowed recorded blood pres-
sure may be an indication of a thrombus at the tip of an arterial
catheter.
Management
Inspect the external portion of the catheter for kinks or compres-
sion. Unless there is an obvious kink or compression that can be
relieved, the line must be removed. After pulling the line, inspect
the tip for a thrombus. Reevaluate the need for the catheter to
be replaced before replacing it.
Central Venous Lines: Bleeding at the Entry Site
As previously stated, placement of central venous lines (CVLs)
should be directly supervised by an attending or an appropriate
fellow.
248 Patient-Related Problems

PHONE CALL
Questions
1. What are the vital signs?
2. What was the reason for admission?
3. Is there bleeding anywhere else?
Orders
Ask the nurse to have a dressing set, sterile gloves, saline flushes or
a bottle of saline, and chlorhexidine or povidone-iodine at the
child’s bedside.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Bleeding at
the central line site needs to be evaluated immediately.

ELEVATOR THOUGHTS
What causes bleeding at the entry site?
1. Bleeding from skin capillaries
2. Tracking of venous blood up the line secondary to disordered
coagulation
a. Medications (anticoagulant, antiplatelet, and thrombolytic
agents)
b. Coagulopathy (thrombocytopenia, inherited or acquired
factor deficiencies, microangiopathy)

MAJOR THREAT TO LIFE

• Upper airway obstruction secondary to bleeding into the soft
tissues of the neck with a jugular catheter.
• Hemorrhagic shock
• Central line–associated bloodstream infection (CLABSI)

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
The child should appear well unless airway compromise or
shock has occurred.

Airway and Vital Signs

Check the airway and the respiratory rate carefully for signs of airway
obstruction. Look for hemodynamic changes suggestive of shock.
 Lines, Tubes, and Drains 249

Selective Chart Review

Review past history for bleeding diatheses. Review labs for last
hemoglobin/hematocrit (Hgb/Hct), platelet count, prothrombin
time/international normalized ratio (PT/INR), and partial throm-
boplastin time (PTT). See Chapter 33.

Selective Physical Examination and Management

1. Remove the dressing and try to localize the bleeding.
2. If you cannot localize the site, clean the area with saline where
the line enters the skin and reinspect.
3. If possible, elevate the site above the heart as tolerated.
4. Apply, or have the nurse or another team member apply, con-
tinuous, firm pressure to the site for 20 minutes (Fig. 26.2).
5. Reinspect. If the bleeding has stopped, clean the area with an
antiseptic and secure the line with an occlusive dressing. If
bleeding persists, continue to apply pressure for another

FIGURE 26.2 Continuous firm local pressure for 20 minutes is

required to stop oozing of blood from the central line
entry site. (From Marshall SA, Ruedy J: On Call: Principles and Pro-
tocols, 4th ed. Philadelphia, Elsevier, 2004, p 204.)
250 Patient-Related Problems

20 minutes. If the bleeding has still not stopped, a coagulation

defect should be suspected, and blood should be sent for a stat
platelet count, PT, and PTT. Refer to Chapter 33 for further
evaluation and management of coagulopathies. Review the
child’s medications for any offending agents.
6. Consider removal of the line if the bleeding is excessive and
resistant to the aforementioned measures. Discuss with your
team if a smaller catheter line or another location may provide
better results. If a line is required, the new line should be placed
prior to removing the old line.

Central Venous Lines: Obstructed Central Line

PHONE CALL
Questions
1. How long has the line been obstructed?
2. What was being infused through the line?
3. What are the vital signs, including recent CVP?
4. Are there any changes in flow or vitals with postural changes?

Orders
Ask the RN for a dressing set, sterile gloves, antiseptic (povidone-
iodine or chlorhexidine), a 5-mL syringe, and a 21-gauge needle to
be placed at the child’s bedside. You may need to remove the sterile
dressing that is in place, but this should be kept in place until you
evaluate the patient.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” The child
needs to be seen immediately.

ELEVATOR THOUGHTS
What causes a central line to become occluded?
1. Kinked or compressed tubing (Fig. 26.3A)
2. Thrombus at the catheter tip (see Fig. 26.3B)
3. Medication precipitate

MAJOR THREAT TO LIFE

• Loss of access for medication administration
• Inability to titrate medications with loss of CVP monitoring.
• Embolism of thrombi or foreign objects.
• Bacteremia
 Lines, Tubes, and Drains 251

FIGURE 26.3 Causes of blocked central lines. A, Kinked tubing.

B, Thrombus at the catheter tip. (From Marshall SA, Ruedy J: On Call:
Principles and Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 200.)

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
A blocked central line should not cause the child to appear ill
unless the obstruction halts administration of vasoactive medications.
If the child does appear ill, and no vasoactive medications were being
given, search for an alternative explanation for the clinical decline.
Airway and Vital Signs
The airway and vital signs are not affected by a blocked central line
unless the line carries a vasoactive or inotropic substance (such as
dopamine, dobutamine, epinephrine, norepinephrine, vasopressin,
isoproterenol).

Selective Physical Examination and Management

1. Evaluate the need for the line. If the line can be removed with-
out further management, remove it.
2. Inspect the line. Is there obvious kinking or compression? If so,
remove the dressing and attempt to straighten the line. If flow is
restored, clean the site and secure the line with an occlusive
dressing.
3. If there is no compression or kinking, proceed as follows:
a. Turn the IV line off.
b. Clean the entry port closest to the insertion site and attach a
5-mL syringe with sterile saline to the central line and clamp
the line distal to this site.
252 Patient-Related Problems

c. Draw back gently on the syringe pulling 3 mL of blood, if

possible. This may dislodge a small thrombus and restore
flow to the line.
d. Blocked central lines should never be flushed because flush-
ing may dislodge a clot on the tip of the catheter and pro-
duce an embolus.
e. If no blood will pull back during the previous attempt, a tis-
sue plasminogen activator (tPA) dwell may be put into the
line to try to dissolve a presumed thrombus. The volume
required will be dependent on the size of the CVL; discuss
this with nursing and pharmacy staff.
4. If the preceding measures are unsuccessful, determine the neces-
sity for the central line. If the central line is essential, a new cen-
tral line needs to be placed at a different site. A new central line
should not be reinserted over a guidewire at the same site
because this may disrupt any clots leading to embolization.
Nasogastric and Enteral Feeding Tubes: Placement
of a Nasogastric Tube
NG tubes are used for controlled feeding and hydration, aspiration
of stomach contents, and, less frequently, for gastric lavages. Place-
ment is a relatively simple procedure but may be made more chal-
lenging by the child’s overall size, his or her willingness to
cooperate, and any facial, oral, pharyngeal, esophageal, or gastric
anatomic abnormalities. Expert help (such as from an otolaryngol-
ogist) should be sought for any child with facial trauma. Small
tubes (5 to 10 Fr) may be used in neonates. Larger tubes (12 to
16 Fr) are necessary in older children. In general, smaller tubes
are placed for enteral feeding while larger tubes are required for
gastric lavage. Generally larger tubes are needed for gastric drain-
age and are not appropriate for feeding. Nursing staff who will be
managing the tube may be helpful in determining the most appro-
priate size and type of tube to be used.
1. Make sure that you have help to restrain an uncooperative child.
Sedation should not ordinarily be necessary unless the child is
especially combative or insufficient help is available to provide
restraint.
2. Approximate the required length of tubing by holding the tube at
the tip of the child’s nose, looping it to the tragus of the ear closest
to you, and then running it down along the neck and the thorax
to the inferior margin of the xyphoid process. This length should
be sufficient for gastric placement; mark the length.
3. Examine the nares for any obvious nasal septum deviation or
deformities. Choose the naris that appears the most open.
4. Lubricate the end of the tube and the child’s external naris.
5. Ask older children to sip some water or juice while the tube is
inserted. This closes the epiglottis, protecting the trachea and
 Lines, Tubes, and Drains 253

easing placement. This should not be attempted in little chil-

dren or those who may not be able to protect their airway.
6. Quickly and smoothly insert the end of the tube into the naris,
directing it toward the child’s occiput. Remember that the pas-
sage through the nasal cavity is nearly perpendicular to the
esophagus; the tube therefore should not be angled superiorly
or inferiorly.
7. The tube should be advanced in a smooth, continuous motion.
Once through the nasal passage, resistance should be minimal
and the tube should advance easily down the posterior of the
pharynx, through the esophagus, and then into the stomach.
Tilting the head forward slightly opens the esophagus wider,
thus making it less likely for the tube to pass into the trachea.
Although there may be a gag reflex when the tube touches the
oropharynx, notable coughing or choking may indicate passage
into the trachea; the tube should be withdrawn and placement
should be attempted after the coughing or choking subsides.
8. When the tube is advanced to the point where your marking
reaches the external naris, stop advancing and secure the tube
in place with tape. A bridle may need to be used to anchor the
tube to the nasal septum. These devices have instructions on or
in the packaging.
9. Verify the location of the tube within the stomach. This is gen-
erally done with an abdominal radiograph. However, it may be
clinically evaluated by pushing air through the tube with a
syringe (5 mL in neonates; 20 mL in larger children) while aus-
cultating the epigastrum. Alternatively, aspirating the tube with
the same syringe may bring up gastric fluid, which may be ver-
ified by a low pH on litmus paper.

Nasogastric and Enteral Feeding Tubes:

Obstructed Nasogastric Tube or Enteral Feeding
Tube

PHONE CALL
Questions
1. How long has the tube been blocked?
2. What type of tube is it?
3. Is the tube dislodged?
4. What are the vital signs and status of the patient?
Orders
Ask the nurse to place several 20- and 50-mL syringes, normal
saline solution, and an emesis basin at the bedside.
254 Patient-Related Problems

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” A blocked
tube used for feeding is not an emergent concern, but they should
be evaluated as soon as possible in an infant and within several
hours in an older child. A blocked tube used for drainage is a
greater problem and requires prompt evaluation to avoid compli-
cations from the accumulation of gastric fluid or gas.

ELEVATOR THOUGHTS
What causes blocked NG or enteral feeding or draining tubes?
1. External clamps or compression
2. Debris, including blood clots, within the lumen of the tube
3. Failure to irrigate the tube leading to desiccated formula
4. Precipitated medication
5. Suction against mucosa

MAJOR THREAT TO LIFE

• Aspiration: This may happen with any enteral tube, even if
functioning properly. If a suction NG tube is blocked, gastric
contents may rise around the tube and be aspirated into the
lungs, leading to pneumonitis and possibly pneumonia.
• Perforation: Although a rare complication, perforation may
happen with excessive suction against an injured mucosa.

BEDSIDE
Quick-Look Test
Does the patient look well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
Aspiration or perforation may quickly lead to a toxic
appearance.
Airway and Vital Signs
The airway and vital signs should not be affected by a blocked tube
unless aspiration or perforation has occurred, or if the blocked tube
has led to nausea, vomiting, or dehydration.
Management
1. Turn off the pump or suction.
2. Examine the tubing to ensure it is not clamped, kinked, or com-
pressed externally. Also look for visible blockages in the tubing.
Very rarely, patients or others may place nonprescribed crushed
medications in the tubing; if this is suspected, removed the tube.
 Lines, Tubes, and Drains 255

3. If the obstruction is due to desiccated formula, a pancrelipase-

sodium bicarbonate dwell may dissolve the obstruction. After
letting this dwell or if no blockage is seen, irrigate the tube with
5 to 10 mL of normal saline solution for an infant, 20 mL for an
older child, and 25 to 50 mL for an adolescent. As you irrigate,
listen with a stethoscope over the stomach to ensure proper
placement of the tube.
4. If irrigation is unsuccessful, remove the tube and replace it.
Blood clots may come from bleeding anywhere in the sinuses,
nasal cavity, or the upper alimentary tract. Place the new tube in
the contralateral naris. If it is felt that the bleeding is due to
esophagitis or gastritis, work that up. If bleeding is felt to be
due to varices, get GI to help evaluate the cause and place the
tube endoscopically.

Nasogastric and Enteral Feeding Tubes: Displaced

Nasogastric Tube and Enteral Feeding Tube

PHONE CALL
Questions
1. How long has the tube been displaced?
2. What type of tube is it?
3. What are the vital signs and the status of the patient?

Orders
Immediately stop using the tube until you have a chance to evaluate
the patient.
If a permanent enteral tube (a G-tube or J-tube) is dislodged,
have the RN place a red Robinson catheter through the tract to pro-
tect it from closure by reepithelialization. The red Robinson tube
should be of a small enough caliber that it will not hurt the patient
going in. This should be securely taped in place.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” As with
blocked tubes, infants with dislodged tubes should be evaluated
as soon as possible.

ELEVATOR THOUGHTS
What causes an NG or feeding tube to become dislodged?
1. Failure to secure the tube
2. Uncooperative child (normal for infants and young children)
256 Patient-Related Problems

MAJOR THREAT TO LIFE

• Aspiration: Dislodged tubes pose a greater risk of aspiration
than a blocked tube, particularly if used for feeding or fluid
and medication administration.

BEDSIDE
Quick-Look Test
Does the patient look well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
Aspiration may cause the patient to appear toxic.
Airway and Vital Signs
If the airway or vital signs are compromised, you should suspect
that aspiration has occurred.
Management
1. Inspect the tube. The markings may allow you to estimate the
positioning of the tube.
2. Aspirate the tube to determine whether you can obtain gastric
fluid. Alternatively, instill a small amount (10 to 20 mL) of air
into the tube and listen to the epigastrium with a stethoscope for
the rush of air to confirm positioning.
3. If the tube is an enteral feeding tube (e.g., a jejunal tube), it
needs to be removed and replaced. It should not be pushed far-
ther down if it has become dislodged.
4. If there are any concerns for a possible aspiration, obtain a chest
radiograph; keep in mind that it may take hours of inflamma-
tion for imaging to show pneumonitis.
5. Tubes that are in place via a surgically created stoma (G-tube
and J-tube) may require replacement under fluoroscopic guid-
ance. Discuss the need for this with a supervising physician.
Urethral Catheters: Placement
Urethral catheters are used to obtain sterile urine specimens, relieve
bladder outlet obstructions and retention, prevent incontinent urine
from getting into open wounds, and allow accurate measures of
urine output in the critically ill. Sterile technique should be ensured
when placing a urethral catheter. As with other procedures in pedi-
atric patients, having an experienced “holder” to distract and gently
restrain the child during the procedure can be a tremendous help.
1. Have a kit at bedside.
2. Position the child in the supine position. A frog-leg position
may help with females. Cleanse the urethral opening thor-
oughly with a povidone-iodine solution.
 Lines, Tubes, and Drains 257

3. Lubricate the end of the catheter with petroleum jelly and insert
it into the urethral opening; advance the tube with gentle, steady
pressure.
4. Once urine flow is visualized in the tube, stop advancing the cath-
eter if this is a one-time catheterization to obtain a sterile urine
specimen. If the catheter is to remain in place, advance the cath-
eter farther to ensure that the tip is fully within the bladder;
in higher Tanner stage males, advance the catheter to the hub.
5. If a Foley catheter is being used, inflate the balloon on the end of
the catheter with a few milliliters of water of saline. This should
not hurt the patient. Pain with balloon expansion may be sug-
gestive of placement within the urethra and inflation should
immediately stop.
6. Exert gentle traction to make sure that the balloon is against the
bladder trigone.
7. Secure the catheter to the medial aspect of the thigh with tape or
a clamp (check your kit). Leave a generous portion of the cath-
eter tubing between the anchor point and the urethral opening
to allow some slack in the tube as the child moves the leg.

Urethral Catheters: Obstructed Urethral Catheter

PHONE CALL
Questions
1. How long has the catheter been blocked?
2. What are the vital signs?
3. Is the child complaining of suprapubic or abdominal pain?
4. Has there been any hematuria?
Orders
Ask the nurse to try flushing the catheter with 10 mL of normal
saline solution if this has not been done already.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” If the child
is uncomfortable, you should evaluate the child immediately.

ELEVATOR THOUGHTS
What causes blocked urethral catheters?
1. Urinary sediment
2. Blood clots
3. External clamping, kinking, or compression
4. Catheter displacement
258 Patient-Related Problems

MAJOR THREAT TO LIFE

• Bladder rupture
• Progressive renal insufficiency
• Urosepsis (also known as sepsis with a urinary tract
infection [UTI])
Bladder rupture may occur if the bladder is unable to drain an
increasing volume of urine. Pain may be expected to precede rup-
ture; therefore an uncomfortable child is worrisome. However, some
children may be incapable of sensing a distended bladder (e.g., those
with spinal cord lesions and comatose or sedated children).
Renal failure may occur secondary to hydronephrosis from
chronic urinary tract obstruction. Urinary stasis also increases
the risk for infection of the urinary tract, which may potentially
progress to sepsis.

BEDSIDE
Quick-Look Test
Does the child look well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
Most patients look well unless the bladder has been distended
enough to cause pain.
Airway and Vital Signs
Unless pain or infection is present, the vital signs are not usually
affected by a blocked urethral catheter.
Management
1. Reevaluate the need for the urethral catheter; if no longer
needed or if a trial without the catheter is appropriate,
remove it.
2. Carefully inspect the catheter and drainage tubing for claps,
kinks, compression, or an obvious blockage in the external por-
tion of the tube.
3. Palpate the suprapubic area for fullness suggestive of bladder
distention.
4. Aspirate and irrigate the catheter as follows:
a. Using sterile technique, disconnect the catheter from the
drainage tubing.
b. With a 10- or 30-mL syringe, aspirate the catheter to dis-
lodge and extract any sediment or blood clot that may be
obstructing the lumen.
c. If aspiration does not reveal anything, flush the catheter with
10 to 20 mL of normal saline solution and check for return
of flow.
 Lines, Tubes, and Drains 259

5. If aspiration and flushing fail to relieve the obstruction, remove

the catheter and insert a new one, again, only if continued use is
indicated.

Urethral Catheters: Gross Hematuria

Discolored urine may be due to bleeding from anywhere in the uri-
nary tract from the glomeruli to the urethral meatus; such bleeding
may be secondary to trauma (catheterization, instrumentation, or
stones), UTIs, glomerular disease, hemorrhagic cystitis, or even neo-
plastic processes. Discoloration may also be due to pigmented metab-
olites (such as with rifampin, beets, and porphyria), myoglobinuria
(as a byproduct of rhabdomyolisis), or hemoglobinuria. In catheter-
ized patients, trauma from the catheter and catheter-associated UTIs
should be initially suspected with an open mind for other diagnoses.

PHONE CALL
Questions
1. Why does the child have a urethral catheter?
2. How long ago was the catheter placed?
3. Is the child in pain?
4. What are the vital signs?
5. Has the patient received heparin, warfarin, or cyclophosphamide?

Orders
Urine dipstick and urine macro-evaluation/micro-evaluation with
reflex urine culture.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Gross
hematuria should be evaluated urgently.

ELEVATOR THOUGHTS
What causes gross hematuria in a child with a urinary catheter?
See earlier. In addition to those causes, keep in mind that coa-
gulopathies and anticoagulant, antiplatelet, and thrombolytic ther-
apies may predispose to bleeding with even small traumatic events
from catheterization.

MAJOR THREAT TO LIFE

Perforated bladder or urethra.
Hemorrhagic shock: Although gross hematuria is generally fright-
ening to the child (and often their parents and medical staff), it
260 Patient-Related Problems

is exceedingly rare for any patient to bleed enough from their

urinary tract to result in hemodynamic compromise.

BEDSIDE
Quick-Look Test
Does the child look well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
A perforated urinary tract will make the child look sick or crit-
ical and will generally be exquisitely painful. An ascending UTI or
pyelonephritis may also make the patient appear sick. Sepsis due to
a UTI may make the child appear critical.

Airway and Vital Signs

Vitals are generally normal unless severe pain, fright or anxiety, or
infection are present.
Selective History and Chart Review
Has the child received any medication that may cause hematuria or
discolor the urine?
Do prior laboratory findings suggest a coagulopathy (particularly
abnormal PT, PTT, and platelet levels) or hemolysis (Hgb/Hct with
mean corpuscular volume [MCV] and mean corpuscular hemoglo-
bin content MCHC, bilirubin, lactic acid dehydrogenase [LDH],
haptoglobin levels)? Reviewing the peripheral smear for evidence
of DIC or a microangiopathy may be helpful.
Is there a history of urethral trauma?
Recent surgery, difficulty inserting or removing a urethral cath-
eter, or an obstructed catheter suggests the possibility of trauma.
Management
1. Examine the catheter contents. True hematuria will often,
although not always, have red sediment.
2. Physically examine the patient. Evaluate the urethral meatus.
Bleeding around the tubing is suggestive of urethral trauma.
Evaluation for costovertebral angle tenderness should be per-
formed on every patient with gross hematuria given the possi-
bility of pyelonephritis.
3. If the patient appears ill and trauma is the suspected cause, a
period of observation with frequent monitoring of the vital
signs and degree of hematuria allows time for the bleeding to
spontaneously subside, which it may do. Consultation with a
urologist should be considered if the bleeding does not dimin-
ish, the vital signs are compromised, or the degree of pain is
concerning for a perforation.
4. Review the UA dipstick and macro-/micro-analysis. UTIs are
suggested by the presence of white blood cells (WBC), leukocyte
 Lines, Tubes, and Drains 261

esterase, nitrates, and bacteria on dipstick; however, the pres-

ence or absence of these markers varies by the infecting organ-
ism and its concentration in the urine. If a UTI is suspected,
start empiric antibiotics and ensure a urine culture is obtained.
If the patient is very ill, also obtain blood cultures. Other diag-
noses that urine studies help with include glomerular disease,
for which the presence of red blood cells (RBC) casts has a very
high specificity. Myoglobinuria classically shows a positive
marker for RBC on dipstick study, whereas none are seen on
microscopic evaluation of the urine.
Consider prerenal causes of discolored urine (metabolites, DIC,
etc.) and work up and treat appropriately.

Chest Tubes: Placement

Chest tubes are placed in the pleural or mediastinal spaces to
evacuate air (pneumothorax, pneumomediastinum), fluid (pleural
or pericardial effusions), purulent collections (pleural or peri-
cardial empyemas), or blood (hemothorax, hemomediastinum;
Fig. 26.4). They are generally placed by surgeons, although critical

FIGURE 26.4 Chest tubes are inserted to drain air (pneumothorax),

blood (hemothorax), fluid (pleural effusion), and pus (empy-
ema). (From Marshall SA, Ruedy J: On Call: Principles and Proto-
cols, 4th ed. Philadelphia, Elsevier, 2004, p 208.)
262 Patient-Related Problems

care or emergency medicine teams or interventional radiologists

will place them if needed. These tubes are always connected to
an underwater seal, a device that allows for unilateral air movement
out of the cavity. With each expiration (similarly with a cough or
Valsalva maneuver), increased intrathoracic pressure may expel a
small portion of the collected air or fluid. Air, whether from the
cavity itself or displaced out of the tubing or collection box by
drained fluid, escapes through the water seal. This drainage may
be augmented with suction. The seal prevents air from entering
back into the system and chest during inspiration (Fig. 26.5).
A functioning water seal should have some bubbling with expira-
tion and the water level should oscillate during the respiratory
cycle. Thus abnormalities at the water seal may be the first indica-
tor of tube dysfunction. Common drainage setups are depicted in
Fig. 26.6; common problems with chest tubes are shown in
Fig. 26.7.

FIGURE 26.5 Loss of fluctuation of the underwater seal. Ask the

patient to cough, and observe for any fluctuation or bubbling.
(From Marshall SA, Ruedy J: On Call: Principles and Protocols,
4th ed. Philadelphia, Elsevier, 2004, p 212.)
 Lines, Tubes, and Drains 263

FIGURE 26.6 Chest tube apparatuses. a, Suction control chamber.

b, Underwater seal. c, Collection chamber. d, To suction. e, From
patient. f, Height equals amount of suction in cm H2O. g, Height
equals underwater seal in cm H2O. (From Marshall SA, Ruedy J:
On Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 209.)

Chest Tubes: Persistent Bubbling in the Water Seal

(Air Leak)

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs?
264 Patient-Related Problems

FIGURE 26.7 Common chest tube problems. a, Shortness of

breath. b, Subcutaneous emphysema. c, Bleeding at the entry site.
d, Loss of fluctuation. e, Excessive drainage. f, Persistent bub-
bling. (From Marshall SA, Ruedy J: On Call: Principles and Proto-
cols, 4th ed. Philadelphia, Elsevier, 2004, p 210.)

Orders
Two clamps at bedside

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” The patient
needs to be seen as soon as possible and immediately if in respira-
tory distress.

ELEVATOR THOUGHTS
What causes persistent bubbling in the drainage container?
1. Loose tubing connection
2. Air leaking into the pleural space around the chest tube at the
insertion site
 Lines, Tubes, and Drains 265

3. Traumatic tracheobronchial injury (in children with traumatic

pneumothorax, there may be additional injuries)
4. Persistent leak into the pleural space from the bronchoalveolar
tree
a. Postsurgical
b. Ruptured bleb (spontaneous pneumothorax; seen particu-
larly in patients with asthma, cystic fibrosis)
c. Bronchopleural fistula
5. Externalization of a proximal side hole on the chest tube
(may be visually identified or seen on chest radiography)

MAJOR THREAT TO LIFE

• Tension pneumothorax: If a pneumothorax is not being
appropriately evacuated, air may collect and tension may
develop leading to hemodynamic collapse (Figs. 26.8 and 26.9).
• Hypoxia: Buildup of air or fluids may lead to lung collapse and a
ventilation-perfusion mismatch with severe hypoxia.

FIGURE 26.8 Pneumothorax. x, Edge of visceral pleura or lung.

(From Marshall SA, Ruedy J: On Call: Principles and Protocols,
4th ed. Philadelphia, Elsevier, 2004, p 218.)
266 Patient-Related Problems

FIGURE 26.9 Tension pneumothorax. a, Shifted mediastinum. b,

Edge of collapsed lung. c, Low flattened diaphragm. (From Mar-
shall SA, Ruedy J: On Call: Principles and Protocols, 4th ed. Phila-
delphia, Elsevier, 2004, p 219.)

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
A tension pneumothorax may be developing in a child who
appears ill. Quickly evaluate the need for a needle thoracotomy
decompression or an additional chest tube in an ill-appearing child
by listening over the lung fields. If no sounds are heard on the side
with the possible tension pneumothorax, treat emergently before
getting imaging (see Chapter 10). If the exam is reassuring, obtain-
ing a stat portable chest radiograph may be helpful.

Airway and Vital Signs

If all connections are tight and the chest tube dressing is airtight, a
persistent air leak means that the patient has a pneumothorax. As
long as air continues to bubble through the collection chamber, the
air should drain from the pleural space and not alter the vital signs
or compromise the airway. Augmenting air removal with suction
should be considered.
 Lines, Tubes, and Drains 267

Selective History and Chart Review

Why was the chest tube placed?
If the tube was placed for pneumothorax, the collection cham-
ber should be bubbling during expiration unless the lung is fully
expanded and the leak into the pleural space has sealed; a contin-
uous bubbling may indicate a worsening pneumothorax.
If the tube was placed to drain fluid (effusion, empyema, or
blood) without suction, a new onset of bubbling indicates loose
connections external to the chest, air leaking into the pleural space
from around the insertion site, or the development of a spontane-
ous or traumatic pneumothorax.
Selective Physical Examination and Management
1. Inspect the tubing connections and ensure that all seals are
airtight.
2. Remove the dressing at the entry site, listen for the sound of air
being sucked into the chest while observing the area around the
site. If the incision for the tube is inadequately closed to form a
seal around the tube, placing sutures to seal the thoracotomy
tract may be necessary. If this stops the continuous bubbling,
clean the site and reapply the sterile dressing.
3. Inspect the tubing for air leaks along its entire length. Do this
by placing a clamp on the tube adjacent to the thoracotomy
site. If the bubbling stops, the air leak is coming from within
the chest; if the bubbling continues, there is a leak in the line,
likely a small hole. If you find the latter to be the case, place a
second clamp on the tube 5 to 10 inches away from the first.
Continue to move this down in serial increments until the
bubbling stops. The leak will be proximal to the patient from
the second clamp. Once the spot is found, multiple layers of
tape may seal off the leak and allow the tube to continue to
be functional. Alternatively, the tubing may be cut and spliced
together with a new drainage kit using a connector. Ensure the
tubing is clamped close to the patient before cutting any tubing
to prevent a pneumothorax via retrograde suction of air into
the chest.
4. If the tubing and entry site appear airtight, obtain a chest radio-
graph to confirm proper chest tube placement. The tube holes
should be within the chest, and the tip of the tube should be seen
clearly away from the mediastinal and subclavian structures.
Comparing this film with the previous film taken after insertion
of the chest tube allows you to determine whether a pneumo-
thorax has developed or if an existing one has increased in size.
If the pneumothorax is larger or the lung has not reexpanded,
the single chest tube may not be adequate to evacuate the air
leak. You may need to consider augmenting evacuation of
the collection with suction or placing a second chest tube. This
268 Patient-Related Problems

should be discussed with the patient’s attending physician. Sur-

gical consultation may also be necessary if the air leak occurred
secondary to trauma or surgery.

Chest Tubes: Loss of Fluctuation of the Water Seal

(Tube Obstruction)

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs?
4. How long has it been dysfunctional?
Orders
None

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” You need to
evaluate the child immediately.

ELEVATOR THOUGHTS
What causes loss of fluctuation of the underwater seal? Loss of fluc-
tuation means that the tube is not functioning to evacuate air or
fluid from the intrathoracic space.
1. External compression or kink in the chest tube
2. Intraluminal chest tube obstruction

MAJOR THREAT TO LIFE

• Tension pneumothorax: If a pneumothorax is not being appro-
priately evacuated, air may collect and tension may develop
leading to hemodynamic collapse (see Figs. 26.8 and 26.9).
• Hypoxia: Buildup of air or fluids may lead to lung collapse and a
ventilation-perfusion mismatch with severe hypoxia.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
 Lines, Tubes, and Drains 269

A tension pneumothorax may be developing in a child who

appears ill. Quickly evaluate the need for a needle thoracotomy
or an additional chest tube in an ill-appearing child by listening
over the lung fields. If no sounds are heard on the side with the
possible tension pneumothorax, treat emergently before getting
imaging (see Chapter 10). If the exam is reassuring, obtaining a stat
portable chest radiograph may be helpful.
Airway and Vital Signs
A displaced trachea, tachycardia, tachypnea, hypoxia, and hypo-
tension may all be markers of a tension pneumothorax or lung col-
lapse with a ventilation-perfusion mismatch.

Selective History and Chart Review

• Why was the chest tube placed?
• How long ago did it stop fluctuating?
• What and how much has been drained in the past 24 hours?
What was the trend of drainage?
Selective Physical Examination and Management
1. Again, ensure you have examined the trachea and the lung
fields to clinically reassure against a tension pneumothorax.
2. Reevaluate the need for the chest tube and remove if
possible.
3. Inspect the water seal. Is there any fluctuation? Ask the child
to cough, and watch for fluctuation. Poor respiratory effort
may lead to minuscule fluctuations. If this is the case, clini-
cally evaluate the child for possible intubation for airway
protection.
4. Inspect the tube for external compression or kinks in the tubing.
You may need to remove the dressing at the insertion site. If a
kink is found, reinspect for fluctuation after repositioning
the tube.
5. If the chest tube appears to be clogged, try milking or stripping
the tube to relieve obstruction by blood clots or other debris.
However, realize that frequent milking may lead to a buildup
of negative pressure on the system and should be done mini-
mally and with care. tPA may be used in select circumstances
to lyse thrombi, but this should be guided by experts (surgeons,
pulmonologists, intensivists).
6. Obtain a portable stat chest radiograph to determine the posi-
tion of the tube. Reposition the tube if it appears to be close to
structures that may be causing an obstruction. Remember that
shear force may traumatize intrathoracic vasculature.
270 Patient-Related Problems

Chest Tubes: Bleeding at the Chest Tube Entry Site

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs?
Orders
Ask the RN for sterile gloves, a dressing set, a suture kit, and
povidone-iodine at the bedside. You need to remove the sterile
dressing around the chest tube site.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” You need to
see the child immediately.

ELEVATOR THOUGHTS
What causes bleeding around the chest tube entry site?
In general, bleeding around chest tube entry sites (thoracotomy
sites) comes from the soft tissues along the tract; even so, intratho-
racic bleeding must be considered.
1. Inadequate pressure dressing
2. Inadequate closure of the incision
3. Coagulation disorders
4. Trauma to intercostal or intrathoracic arteries or veins during
tube placement or manipulation
5. Blocked chest tube in a child with a hemothorax

MAJOR THREAT TO LIFE

• Hemorrhagic shock: Usually the bleeding is not rapid though
direct trauma to any vasculature can lead to shock and
exsanguination.
• Hemothorax with subsequent ventilation perfusion defect due
to decreased lung volume.
• Sepsis: Active bleeding indicates an opening in the circulatory
system. Bacteremia and sepsis may develop; therefore one
should watch for signs and symptoms of infection after hemo-
stasis has been achieved.
 Lines, Tubes, and Drains 271

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
If the child appears more ill than would be suggested by the vol-
ume of extracorporeal blood, a developing hemothorax should be
suspected until proved otherwise.

Airway and Vital Signs

The airway is almost always stable. Vital signs are usually stable
unless significant hemorrhage (including hemorrhage leading to
hemothorax) has occurred. Rapid decompensation indicates mas-
sive blood loss, lung collapse, or intrathoracic tension.

Selective History and Chart Review

Why was the chest tube placed?
If it was placed for hemothorax, the main concern is inadequate
evacuation of blood from the pleural space. Augmenting drainage
with suction may be needed.
Review the patient’s personal and family histories with an eye
for any coagulopathies, their medications for any anticoagulant,
antiplatelet, or thrombolytic therapeutics, and their labs for any
coagulation disorders, anemia, or thrombocytopenias.

Selective Physical Examination and Management

1. Do a quick but full chest examination.
2. Remove the dressing and inspect the incision. See if the incision
is inadequately closed; if so, additional sutures may tamponade
the bleeding. If the incision is adequately closed, reapply a pres-
sure dressing at the site.
3. If the patient’s history or medication regimen suggest an etiol-
ogy, address that after ensuring additional pressure has been
placed around the thoracotomy site.
4. If the chest tube appears to be clogged, try milking or stripping
the tube to relieve obstruction by blood clots or other debris.
However, realize that frequent milking may lead to a buildup
of negative pressure on the system and should be done mini-
mally and with care. tPA may be used in select circumstances
to lyse thrombi, but this should be guided by experts (surgeons,
pulmonologists, intensivists).
5. If bleeding persists in a patient with hemothorax, consider plac-
ing a larger chest tube.
272 Patient-Related Problems

6. In those without hemothorax, apply firm pressure by hand over

the site for 20 minutes. Repeat this step if necessary. If the bleed-
ing still continues, further evaluation for an unknown coagulo-
pathy may be indicated.

Chest Tubes: Drainage of Blood

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs? Any orthostatic symptoms of changes
in vital signs?
4. How much blood has drained?

Orders
None
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” You need to
see the child immediately.

ELEVATOR THOUGHTS
What causes blood to drain from the chest tube?
1. Injury to intercostal or intrathoracic vasculature
2. Pulmonary hemorrhage

MAJOR THREAT TO LIFE

• Hemorrhagic shock: Usually the bleeding is not rapid though
direct trauma to any vasculature can lead to shock and
exsanguination.
• Respiratory insufficiency secondary to pulmonary hemorrhage:
This would usually be due to an underlying condition, not the
catheter itself.
• Sepsis: Active bleeding indicates an opening in the circulatory
system. Bacteremia and sepsis may develop; therefore one
should watch for signs and symptoms of infection after hemo-
stasis has been achieved.
 Lines, Tubes, and Drains 273

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
If the child appears more ill than would be suggested by the vol-
ume of extracorporeal blood, a developing hemothorax should be
suspected until proved otherwise.

Airway and Vital Signs

The airway is almost always stable. Check the heart rate as this is
often the first marker of hemodynamic stress. Also review the
blood pressure and, if possible, a CVP.

Selective Chart Review and Management

Was the chest tube placed postoperatively? Any recent operations or
procedures? Any recent trauma?
If so, determine if the bleeding is within an expected
volume range.
What is the trend of volume removed?
An increasing volume over time should raise your suspicions of
serious pathology.
What medications has the child received?
Check the list for any anticoagulant, antiplatelet, or thrombo-
lytic medications.
How much blood has been lost?
If the amount of blood loss is small, you may continue to care-
fully monitor the loss and ask to be informed if the amount lost
exceeds 10 mL/hour in an infant or 25 mL/hour in an older child.
Increase the frequency of vital sign observations so that the heart
rate and blood pressure can be monitored; consider transferring
the patient to a critical care unit for close monitoring. In addition
and in short order:
1. Order a chest radiograph to evaluate for potential sources of
intrathoracic bleeding.
2. Send blood for typing and cross matching for 4 adult units of
packed red blood cells.
Also obtain Hgb, Hct, platelet count, PT, and PTT measurements.
3. Consult cardiothoracic surgery. If the bleeding persists or is
excessive, surgical exploration may be necessary to localize
and stop the bleeding.
4. If there is any hemodynamic compromise, start aggressive
intravenous fluid (IVF) resuscitation until blood products are
ready for transfusion.
274 Patient-Related Problems

Chest Tubes: Dyspnea

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs?
Orders
A medical provider should stay with the patient until you arrive.
There should be a low threshold for calling for rapid response team
or critical care assistance. Ask the nurse for a dressing set, gloves,
several sizes of angiocatheters, and povidone-iodine at the bedside.
You may need an angiocatheter (one that does not lock after the
flash) to evacuate a tension pneumothorax. Ask the nurse to call
for an immediate portable chest radiograph.
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” You need to
see the child immediately.

ELEVATOR THOUGHTS
What causes dyspnea in a patient with a chest tube?
1. Tension pneumothorax
2. Expanding pneumothorax
3. Subcutaneous emphysema (see later)
4. Expanding pleural effusion or hemothorax
5. Reexpansion pulmonary edema (after rapid evacuation of the
pleural space)
6. Other causes unrelated to the chest tube (see Chapter 28)

MAJOR THREAT TO LIFE

• Tension pneumothorax: If a pneumothorax is not being appro-
priately evacuated, air may collect and tension may develop
leading to hemodynamic collapse (see Figs. 26.8 and 26.9).
• Hypoxia: Buildup of air or fluids may lead to lung collapse and a
ventilation-perfusion mismatch with severe hypoxia.

BEDSIDE
Quick-Look Test
Children with dyspnea usually appear ill.
 Lines, Tubes, and Drains 275

Airway and Vital Signs

1. Protecting the airway is paramount. Evaluate for stridor or
other signs of extrathoracic airway compromise, including sub-
cutaneous emphysema that may be obstructing the upper air-
way. If needed, call a code to expedite intubation.
2. A displaced trachea, tachycardia, tachypnea, hypoxia, and
hypotension may all be markers of a tension pneumothorax
or lung collapse with a ventilation-perfusion mismatch.
3. Tachypnea suggests hypercapnia, hypoxia, pain, or anxiety.

Selective Physical Examination

Examine for signs of tension pneumothorax as described
previously.
Evaluate the chest tube water seal for bubbling; if the bubbling
has stopped, obstruction is suggested (see previous section).

Management
1. Again, if there are any questions about the safety of the airway,
call a code to expedite intubation and transfer the patient to a
critical care unit.
2. If you suspect tension pneumothorax, it needs to be evacuated
(see Chapter 10, Chest Pain).
3. If you suspect an enlarging pneumothorax, look for a cor-
rectable cause (blocked or compressed tubing, inadequate
suction on the chest tube, or dislodged chest tube). A chest
radiograph helps to determine whether the tube is properly
positioned.
4. If the chest tube appears to be functioning and no tension or
expanding pneumothoraces are present, management should
be directed at other causes of dyspnea unrelated to the chest
tube (see Chapter 28, Respiratory Distress).

Chest Tubes: Subcutaneous Emphysema

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs?
Orders
Ask the nurse for a dressing set, gloves, and povidone-iodine at the
bedside. You need to remove the sterile dressing around the
insertion site.
276 Patient-Related Problems

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” You need to
see the child immediately.

ELEVATOR THOUGHTS
What causes subcutaneous emphysema in a patient with a chest
tube?
1. Inadequate size of tube
2. Inadequate suction
3. Chest tube aperture in the chest wall
4. Chest tube in the chest wall or abdominal cavity
5. Minor subcutaneous emphysema localized to the insertion site
(not uncommon)

MAJOR THREAT TO LIFE

• Upper airway obstruction: Subcutaneous emphysema may
extend into the soft tissues of the neck and compress the airway.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
If the emphysema is leading to upper airway obstruction, the
child will appear ill.
Airway and Vital Signs
1. Inspect and palpate the neck to feel for the crepitus of subcuta-
neous emphysema. If present, it will feel and may sound like a
“crunching” as you palpate.
2. Check the respiratory rate, blood pressure, and heart rate. Sub-
cutaneous emphysema may be associated with a concurrent
tension pneumothorax.
Selective Physical Examination and Management
1. If the airway is nearly obstructed (stridor, tachypnea, dyspnea),
call for critical care support to expedite intubation to protect
their airway.
2. Examine the size of the chest tube. If the tube is too small, air
may escape from the pleural space into the chest wall. A new,
larger tube may be necessary.
3. Is the suction connected? A tube left only to straight drainage
may be inadequate to evacuate a large pneumothorax.
 Lines, Tubes, and Drains 277

4. Remove the dressing and inspect the insertion site and the chest
tube. None of the holes in the tube should be visible. They should
all be within the pleural space. If the tube has become misplaced
and the holes now lie outside the chest wall, a new tube needs to be
inserted. Do not push an extruded tube back into the pleural
space, because this increases the risk of infecting the pleural cavity.

Chest Tubes: Traumatic or Accidental Removal of a

Chest Tube

PHONE CALL
Questions
1. Is the child in respiratory distress?
2. Why was the chest tube placed?
3. What are the vital signs?
Orders
Ask the nurse for occlusive dressing and tape. A medical provider
should stay with the patient until you arrive. The RN should also
call the provider who will replace the tube (generally the team who
initially placed it; if placed in the emergency department (ED) or
outside of the hospital, then have general surgery or critical care
help). There should be a low threshold for calling for rapid
response team or critical care support.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” You need to
see the child immediately.

MAJOR THREAT TO LIFE

• Hypoxia: Loss of a pressure differential between the hemithorax
and the external environment may lead to lung collapse and a
ventilation-perfusion mismatch with severe hypoxia.
• Tension pneumothorax: A small opening may lead to air entry
into the pleural space and prevent exit therefrom, leading to a
buildup of tension. However, if the hole is large, it will act as a
decompression tract allowing for air to enter and exit the space.
Should this be the case, if the tract is iatrogenically or sponta-
neously closed, a tension pneumothorax may develop as air
from the lung (if perforated) builds up in the space.
• Sepsis: The open tract may lead to infection; therefore one
should watch for signs and symptoms of infection after hemo-
stasis has been achieved.
278 Patient-Related Problems

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
An open tract may lead to collapse of the lung and acute respi-
ratory and hemodynamic collapse.
Airway and Vital Signs
1. If there is any respiratory distress, call for critical care support
because an expedited intubation may be required.
2. Continuous cardiorespiratory monitoring and frequent blood
pressure measures should be followed.

Selective Physical Examination and Management

1. Quickly examine the patient. Again, if they are in distress, call
for critical care support.
2. If the patient is stable from hemodynamically and respiratory
standpoints, tape three sides of an occlusive dressing over
the thoracotomy tract to protect it until the team responsible
for replacing the tube arrives. Do not tape all four sides as this
could lead to a tension pneumothorax.
 C HA P TE R

27
Rashes
Anna Schmitz, MD

Rashes can develop for a wide variety of reasons in a hospitalized

child—some very serious and others benign. When you are called
by the nurse to evaluate a rash while on call, remember that your
goal is not necessarily to arrive at a specific diagnosis or explana-
tion. Instead, excluding illnesses that might be harmful to the
child, keeping the child comfortable, and reassuring the patient
and family that you have done so may be appropriate immediate
goals.

PHONE CALL
Questions
1. How long has the patient had the rash?
2. Is urticaria (hives) present?
3. Is the child wheezing or in any respiratory distress?
4. What are the vital signs?
5. What medications has the child received in the past 12 hours?
6. Does the child have any known allergies?
7. What is the child’s age and admitting diagnosis?

Orders
If the rash is associated with signs of anaphylaxis (wheezing, stri-
dor, dysphagia, shortness of breath, periorbital edema, lip swelling,
or hypotension), order the following without delay:
1. Place an intravenous (IV) line immediately and start a normal
saline bolus.
2. Stop any current infusions (antibiotics, blood products, etc.)

279
280 Patient-Related Problems

3. Have airway support materials at the bedside, including suction,

a laryngoscopy tray, oxygen, and an Ambu bag.
4. Epinephrine, 0.01 mg/kg (0.1 mL/kg of a 1:10,000 solution for
IV administration or 0.01 mL/kg of a 1:1,000 solution for sub-
cutaneous administration). Do not confuse these doses and
routes of administration!
5. IV diphenhydramine (Benadryl), 1 mg/kg and/or IV ranitidine
1 mg/kg
6. IV methylprednisolone, 1 mg/kg
If there are no signs of an acute allergic reaction, instruct the
nurse to avoid applying anything to the rash until you have exam-
ined the patient.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.” Evidence of
anaphylaxis or acute allergy requires immediate evaluation. A new
onset of purpura or petechiae, particularly in a child with fever, also
requires immediate evaluation.

ELEVATOR THOUGHTS
Many medications can cause skin eruptions, and such rashes are
among the most common that you will be asked to evaluate. The
lesions may be urticarial, macular, papular, erythematous, vesicu-
lar, bullous, petechial, or purpuric. Most drug rashes are widely dis-
tributed over the body. Remember that rashes can evolve, and
changes may occur with time. What the nurse observed may not
be what you see 2 or 3 hours later. The more common rashes
and some of their causes are listed here.

Urticaria (Rare but Potentially Life Threatening)

Histamine-mediated allergic reactions: IV contrast material, anti-
biotics, opiates, anesthetic agents, vasoactive agents
Drug reactions with an unknown mechanism: aspirin, nonsteroidal
antiinflammatory drugs
Food allergies: shellfish, nuts, tomatoes
Physical agents: detergents, perfumes, cold, heat, pressure
Idiopathic

Erythematous, Maculopapular (Morbilliform) Rashes

Infections
Measles (rubeola)
Rubella
Roseola
Kawasaki disease
 Rashes 281

Systemic-onset juvenile idiopathic arthritis

Drug reactions. (Because some drug rashes have a late onset, the
medication history should include all medications taken in
the last 4 weeks.)
Antibiotics
Antihistamines
Antidepressants
Diuretics
Sedatives
Vesicobullous Rashes
Varicella-zoster (primary, chicken pox; or secondary, herpes zoster)
Erythema multiforme (many viruses and drugs)
Stevens-Johnson syndrome (this is a particularly life-threatening
variant of erythema multiforme characterized by involvement
of at least two mucous membranes, especially the oral mucosa
and eye).
Toxic epidermal necrolysis: Lyell syndrome (sulfonamide, allopurinol)
Antibiotics (sulfonamides, dapsone)
Antiinflammatory agents (penicillamine)
Sedatives (barbiturates)
Petechiae or Purpura
Vasculitis (palpable purpura)
Sepsis or disseminated intravascular coagulopathy (DIC; meningo-
coccemia, Haemophilus influenzae sepsis, cytomegalovirus)
Antibiotics (sulfonamides, chloramphenicol)
Diuretics
Antiinflammatory agents (salicylates, indomethacin, phenylbutazone)
Thrombocytopenia

Exfoliative Dermatitis (Erythroderma)

Scarlet fever
Toxic shock syndrome
Antibiotics (streptomycin)
Antiinflammatory agents (gold, phenylbutazone)
Antiepileptics (carbamazepine, phenytoin)

Fixed Drug Reaction

Antibiotics (sulfonamides, metronidazole)
Antiinflammatory drugs (phenylbutazone)
Analgesics (phenacetin)
Sedatives (barbiturates, chlordiazepoxide)
Laxatives (phenolphthalein)
Certain drugs may produce a skin lesion in a specific area.
Repeated administration of the drug reproduces the skin lesion
282 Patient-Related Problems

in the same location. The lesions are usually dusky red or viola-
ceous patches over the trunk and limbs.

MAJOR THREAT TO LIFE

• Anaphylactic shock
• Septic shock with DIC
• Stevens-Johnson syndrome
Urticarial eruptions indicate histamine release and may be a
prodrome to systemic histamine effects, including hypotension
and shock. In hospitalized children, drugs and IV contrast mate-
rials are the most common causes of anaphylactic reactions.
Sepsis and septic shock can evolve rapidly. The rashes associated
with certain conditions, such as meningococcemia and toxic shock
syndrome, may develop during the early part of the hospitalization.
Stevens-Johnson syndrome presents a major risk of significant
dehydration as a result of oral mucosal involvement, as well as a
major threat of permanent visual impairment from uveitis or cor-
neal scarring.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical?
A patient in impending anaphylaxis appears anxious and
hyperalert, usually with progressive respiratory distress.

Airway and Vital Signs

What is the blood pressure?
Hypotension is an ominous sign and requires immediate and
aggressive intervention (see Chapter 25).
What is the temperature?
Almost all skin rashes become more apparent when the child is
febrile because there is greater perfusion of the skin.
Selective Physical Examination
Is there evidence of impending anaphylaxis, sepsis or DIC, or
Stevens-Johnson syndrome?
HEENT Pharyngeal, periorbital, or facial edema;
conjunctivitis or uveitis; oral mucosal lesions
Respiratory Stridor, wheezing
Skin Urticarial rash, erythema multiforme rash, purpura,
petechiae
HEENT, Head, Eyes, Ears, Nose, Throat.
 Rashes 283

What is the location of the rash? Is the rash generalized, acral

(hands and feet), or localized?
Remember that to evaluate a child for a rash, you must examine
the entire child, including the buttocks (a common site for drug
eruptions) and the genital region, as well as the scalp.
What is the color of the rash?
Rashes may be erythematous, pale, brown, purple, or pink
Describe the primary lesions (Fig. 27.1):
Macules: flat, with or without a distinct margin (noticeable from
the surrounding skin because of the color difference)
Patch: a large macule
Papule: solid, elevated, less than 1 cm
Plaque: solid, elevated, greater than 1 cm
Vesicle: fluid filled, elevated, well circumscribed, less than 1 cm
Pustule: vesicle containing purulent fluid
Bulla: fluid filled, elevated, well circumscribed, greater than 1 cm

FIGURE 27.1 Primary skin lesions. (From Marshall SA, Ruedy J:

On Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 291.)
284 Patient-Related Problems

Nodule: deep-seated mass, indistinct borders, size less than

0.5 cm in both width and depth
Cyst: nodules filled with expressible fluid or semisolid material
Wheal (hives): urticaria; pruritic, well-circumscribed, flat-
topped, firm elevation (papule, plaque, or dermal edema) with
or without central pallor and with irregular borders
Petechiae: red or purple, nonblanching macules less than 3 mm
Purpura: red or purple, nonblanching macule or papule greater
than 3 mm
Describe the secondary lesions (Fig. 27.2):
Scales: dry, thin plates of thickened keratin layers (white color
differentiates scales from crusts)
Crust: dried yellow exudate of plasma (results from broken ves-
icles, bullae, or pustules)

FIGURE 27.2 Secondary skin lesions. (From Marshall SA, Ruedy J:

On Call: Principles and Protocols, 4th ed. Philadelphia, Elsevier,
2004, p 292.)
 Rashes 285

Lichenification: dry, leathery thickening; shiny surface with

accentuation of skin markings
Fissure: linear, epidermal tear
Erosion: wide, epidermal fissure; moist and well circumscribed
Ulcer: erosion into the dermis
Scar: flat, raised (keloid), or depressed area of fibrosis
Atrophy: depression secondary to thinning of the skin
What is the configuration of the rash?
Annular: circular, well circumscribed
Linear: in lines
Grouped: clusters (e.g., vesicular lesions of herpes zoster or her-
pes simplex)

Selective History and Chart Review

How long has the rash been present? Is it changing or evolving?
Is it pruritic?
How has it been treated?
Is it a new or recurrent problem?
Which medications was the child receiving before onset of
the rash?
Was there previously anything overlying the location of the rash?
(i.e., electrocardiogram [ECG] leads, Tegaderm, tape)
Management
1. If the rash is suspected to be a manifestation of an allergy of any
kind, it is usually pruritic and should respond to diphenhydra-
mine (Benadryl).
2. If the rash is associated with urticaria and is secondary to a drug
reaction, administration of the drug should be withheld until
the diagnosis can be confirmed in the morning.
3. If the rash is nonurticarial and thought to be secondary to a
drug reaction and the drug is essential to treatment of the child’s
underlying illness, administration of the drug can be continued
with close monitoring of the patient’s condition, as long as there
is no sign of respiratory compromise or abnormalities in blood
pressure and other vital signs.
4. When the rash is not a drug reaction and the diagnosis is clear,
the standard recommended treatment of that disorder should
be instituted.
5. If the diagnosis of the rash is unclear, describe the lesions thor-
oughly in your note. If the rash does not cause undue discom-
fort, no therapy is necessary until the rash evolves or other
symptoms or signs develop and a specific diagnosis can be
made. Exceptions include the following:
Petechial rash, which can indicate disorders of platelet number
or function.
286 Patient-Related Problems

Purpuric rash, which can indicate DIC and sepsis and requires
checking a blood culture, prothrombin time, activated par-
tial thromboplastin time, and platelet count, as well as
prompt administration of antibiotics.
Vesicular rash secondary to varicella or herpes zoster, which
requires immediate isolation of the patient from any poten-
tially immunocompromised children and pregnant women.
If the child is immunocompromised or an infant, urgent
evaluation and treatment with IV acyclovir is indicated to
prevent dissemination of the infection to the central nervous
system.

REMEMBER
Your ability to describe the rash is critical to establishing its cause
and significance. This will help you to think carefully about the
possible explanations and appropriate management when you have
been called to make a “rash decision.”
 C HA P TE R

28
Respiratory Distress
Brian Carroll, MD

Respiratory distress is one of the most common complaints that an

on-call pediatric house officer is asked to assess. To evaluate a child
in respiratory distress, it is necessary to consider the respiratory
rate in the context of the age of the child. Neonates typically breathe
35 to 50 times per minute, older infants and toddlers 30 to 40 times
per minute, elementary school–aged children 20 to 30 times per
minute, and preadolescents and adolescents 12 to 20 times per
minute.
In addition to the rate, it is important to observe the quality of
the breathing, including depth, use of accessory muscles (e.g., sub-
costal, intercostal, or supraclavicular retractions), grunting, tra-
cheal tugging, and nasal flaring. Next, you should listen for the
presence of adventitious lung sounds such as wheezing, rales, rhon-
chi, stridor, stertor, or muffled/absent breath sounds. The clinical
meaning of these sounds is discussed later in the Physical
Examination section. Lastly, you should identify in which phase
of the respiratory cycle (inspiratory, expiratory, or both) that these
sounds reside because this will help you to identify the location of
the pathology. The specific characteristics of the breathing pattern
combined with the physical findings and selective laboratory and
radiographic tests (when necessary) will allow you to determine
the probable cause of the respiratory distress.

PHONE CALL
Questions
1. How old is the patient?
2. Why is the patient in the hospital?
3. How long has the patient been in respiratory distress?
4. Was the onset sudden or gradual?

287
288 Patient-Related Problems

5. What are the vital signs?

6. Does the child appear cyanotic?
7. Is the child retracting, flaring, wheezing, or coughing?
8. Are oxygen and a pulse oximeter present in the room?

Orders
1. Have the nurse provide oxygen by nasal cannula or mask and
obtain a pulse oximeter measurement immediately. Start with 1
to 2 L/min by cannula or 5 to 10 L/min by mask.
2. Set up materials to obtain an arterial blood gas measurement or
call the lab to draw blood for arterial blood gas studies.
3. If the child has been admitted for reactive airway disease and/or
asthma, have the nurse set up an appropriate dose of a nebu-
lized bronchodilator.
4. Inform the nurse that you are on your way. Respiratory distress
deserves immediate evaluation!

ELEVATOR THOUGHTS
Respiratory distress may be a manifestation of several very different
pathologic processes. Distress can include depressed respirations,
as well as tachypnea.
Pulmonary Pneumonia, bronchospasm, bronchiolitis,
processes pulmonary hemorrhage, or interstitial lung
disease
Airway processes Croup, foreign body aspiration,
retropharyngeal abscess, laryngeal edema or
spasm, epiglottitis, tracheitis,
laryngotracheal malacia, vascular ring, or
esophageal masses
Cardiac Congestive heart failure (left-to-right shunt,
processes left ventricular failure), cardiac tamponade,
or pulmonary embolism
Space- Pleural effusion, empyema, pneumothorax,
occupying diaphragmatic hernia, massive ascites, or
lesions severe scoliosis. Abdominal distention can
cause respiratory compromise, especially in
infants, who rely on diaphragmatic
breathing, or in children with restrictive
lung disease (i.e., severe scoliosis).
Neurologic Opiate overdose, increased intracranial
processes pressure, anxiety, or chest wall and/or
diaphragmatic weakness. Tachypnea
secondary to pain or discomfort.
 Respiratory Distress 289

MAJOR THREAT TO LIFE

Hypoxia resulting in inadequate tissue oxygenation is the most
worrisome consequence of any process that results in respiratory
distress. In addition, respiratory failure is the most common pre-
cipitant of cardiac arrest in children and should be addressed
quickly.

BEDSIDE
Quick-Look Test
The first step is to determine if the child appears (1) well (comfort-
able), (2) sick (uncomfortable or distressed), or (3) critical (about
to die).
A child in distress should be placed on a cardiorespiratory mon-
itor immediately and pulse oximetry applied. Oxygen should be
administered and airway support supplies, including suctioning
and intubation equipment, brought to the bedside. If a child
appears critically ill, you should activate the appropriate response
team as early as possible to assist you in management.

Airway and Vital Signs

Is the upper airway (including nares) clear, and can the patient pro-
tect his or her airway (for example, coughing appropriately and able
to swallow secretions)?
An obtunded patient in respiratory distress requires intubation.
Upper airway obstruction may make intubation difficult or impos-
sible, such as in a patient with oral or facial trauma, foreign body
aspiration, or severe epiglottitis. A surgical airway may thus be nec-
essary via emergency cricothyroidotomy.
What is the respiratory rate and pattern?
Rates less than 20 breaths per minute in most young children
reflect central respiratory depression, such as with opiates, barbi-
turates, or alcohol. Tachypnea suggests hypoxemia, hypercapnia,
acidemia, pain, and/or anxiety. Retractions and nasal flaring indi-
cate the use of accessory muscles of respiration because of inade-
quate tidal volume or airway obstruction. Thoracoabdominal
dissociation is a worrisome finding. The chest and abdomen should
rise and fall together and not paradoxically.
What is the heart rate?
Increased sympathetic tone secondary to respiratory distress
results in sinus tachycardia. Hypercapnia causing acidosis will also
cause tachycardia. Bradycardia may herald impending cardiorespi-
ratory collapse, and a normal heart rate may be inappropriate
for the level of respiratory distress and a worrisome finding.
290 Patient-Related Problems

Supraventricular tachycardia or nonsinus bradycardia may result

in congestive heart failure and subsequent respiratory distress.
What is the temperature?
Fever is accompanied by tachypnea. Obviously, fever suggests
infection, and respiratory distress may be due to airway, pleural,
or parenchymal lung infection.
What is the blood pressure?
Hypotension in the setting of respiratory distress suggests
shock, acidosis, and possible cardiac compromise as a result of ten-
sion pneumothorax. In children, pulsus paradoxus, or when inspi-
ration causes a drop in systolic blood pressure of more than the
usual 4 to 10 mm Hg, is rarely noted, but it may occur in the setting
of respiratory distress and hypotension (pericardial effusion), as
well as with obstructive airway disease as a reflection of the degree
of airflow obstruction.
Hypertension can occur as a result of increased sympathetic tone
from respiratory distress or significant hypercapnia and acidosis.

Selective Physical Examination

Is the patient cyanotic? If yes, see Chapter 13 on Cyanosis.
Vital signs Repeat now, including pulse oximetry
HEENT Nasal flaring, cyanotic mucous membranes,
oropharyngeal foreign body
Neck Midline trachea, stridor or stertor
Respiratory Symmetry of air entry. wheezing, rales, rhonchi,
stridor and stertor, decreased breath sounds,
dullness to percussion, ability to phonate,
retractions, grunting
Neurologic Mental status, ability to defend the airway (gag reflex)

HEENT, Head, Ears, Eyes, Nose, Throat.

Stridor is a harsh noise usually caused by decreased caliber of a

large airway, usually representing obstruction (such as croup) or
foreign body. Stertor is a low-pitched “snoring” sound usually
representing soft tissue collapse at the level of the larynx or soft pal-
ate. Wheezing is a high-pitched “whistling” sound, usually repre-
senting small airway obstruction (bronchospasm in asthma).
Rhonchi are low-pitched “rattling” noises usually representing
secretions in large to medium airways. Rales are a fine “crackling”
noise usually representing the popping open of small airways and
alveoli that were collapsed due to fluid/exudate collection (pneu-
monia) or lack of aeration (atelectasis).
After you have identified any adventitious sounds, you should
then localize the lesions. A quick rule of thumb is the I/E and E/I
 Respiratory Distress 291

rule. If a sound is heard only during Inspiration, then the lesion is

usually Extrathoracic (above the vocal cords). If heard only in expi-
ration, then the lesion is likely Intrathoracic. If a noise is biphasic
(heard during both phases), then there is a fixed obstruction such
as a foreign body, severe airway narrowing (severe croup), or mass
effect. This principle is due to the relative pressure changes during
respiration where intraluminal pressure of the upper airway (above
the vocal cords) is lower than extraluminal pressure (allowing col-
lapse of the airway) during inspiration but not expiration. Thus, if
there is a pathologic narrowing of the upper airway (like croup),
that narrowing is worsening during inspiration and the adventitious
noise is louder. The opposite is true of the intrathoracic airways,
where the intraluminal pressure is higher during inspiration but
lower in expiration. Thus airway collapse during expiration will
make a pathologic narrowing (like bronchospasm in asthma) worse.

Management
What immediate measures need to be taken to correct hypoxemia?
Administer adequate oxygen. How much oxygen and by what
route depend on the age of the child and the amount of distress.
Infants may require an oxygen hood because they do not keep a
cannula or mask in place easily. Older children may do very well
with either a nasal cannula or mask. The amount of oxygen should
be just enough to normalize the oxygen saturation (92% to 97%)
and/or PO2. Remember, pulse oximetry does not give any informa-
tion about the effectiveness of ventilation, such as PCO2, pH, base
excess or deficit, or the alveolar-arterial (A-a) O2 gradient.
What harm can your treatment cause?
Giving 100% oxygen for a prolonged time can lead to atelectasis
because the inert gases that are not absorbed help keep the alveoli
inflated. With 100% oxygen, these inert gases are washed out of the
alveoli, and collapse can occur as the oxygen is absorbed. Oxygen
also has direct toxic effects on the lungs, especially in neonates and
infants.
With chronic carbon dioxide retention, the drive for respiration
becomes hypoxia, not hypercapnia. Therefore, in patients with
chronic, poorly controlled asthma and in patients with cystic fibro-
sis, it is advisable to not exceed 30% oxygen without checking arte-
rial blood gases carefully.
Is the child dyspneic with effective air movement or with poor air
movement?
Children with alveolar disease have distress and hypoxia despite
good air movement. This includes children with pneumonia and
congestive heart failure. Children with airway disease have dimin-
ished air exchange for a variety of reasons.
292 Patient-Related Problems

Management depends on the underlying cause of the respira-

tory distress. Management of four general categories of respiratory
distress is discussed here: pulmonary processes, airway processes,
cardiac processes, and space-occupying processes. Management
of asthma and respiratory failure is also included.

Pulmonary Processes
Selective History
Is there a history of fever, cough, or upper respiratory infection?
Is the child immunocompromised?
Does the child have a history of pneumonia, aspiration, or
bronchospasm?

Selective Physical Examination

Are the breath sounds heard equally in all areas of the chest?
Can areas of consolidation be identified by auscultation or percussion?
Are rhonchi, rales, or wheezing heard (see Physical Examination
section earlier)?
In older children, is egophony or whisper pectoriloquy appreciated?

Chest Radiographic Findings

Pulmonary processes vary in their radiographic appearance and
include lobar consolidation (bacterial pneumonia), streaky intersti-
tial markings (bronchiolitis), patchy bilateral alveolar infiltrates
(mycoplasma), and pleural effusion. Trust your physical examina-
tion. Remember that a volume-depleted child with pneumonia may
not manifest a full-blown infiltrate until rehydrated. Early in the
course of pneumonia, the chest radiograph may also be unimpres-
sive. A two-view chest radiograph (anteroposterior [AP] and lat-
eral) is superior to a single view for evaluating retrocardiac
consolidations and pleural effusions but only obtain if the child
is able to be safely transported to the radiology department. Oth-
erwise a bedside single-view radiograph is sufficient for most clin-
ical decision-making.

Laboratory Evaluation
Many instances of respiratory distress do not require laboratory
evaluation. However, if a patient is ill-appearing or does not
respond to treatment as expected, you may obtain laboratory stud-
ies. If concerned for infection, obtain a complete blood count and a
blood culture before initiating antibiotics. You should obtain a
blood gas, preferably arterial sample, to help determine indexes
of ventilation not found on bedside monitoring. Sampling sputum
is impractical in small children, but in rare cases, you can send
sputum for Gram stain and culture. In an immunocompromised
 Respiratory Distress 293

child, one must consider Pneumocystis carinii pneumonia and

other opportunistic pathogens. In infants with bronchiolitis, naso-
pharyngeal swabs can be obtained for assay for common respira-
tory viruses such as syncytial virus, influenza, parainfluenza, and
adenovirus. However, send only viral swabs if it may change your
current management.

Treatment
GENERAL MEASURES
• Oxygen

SPECIFIC MEASURES
Antibiotics. The choice of antibiotic therapy in neonates includes
ampicillin and either gentamicin or third-generation cephalospo-
rin to cover both gram-positive (Streptococcus pneumoniae) and
gram-negative (Escherichia coli, Haemophilus influenzae) organ-
isms, as well as Listeria monocytogenes. In older children,
second- and third-generation cephalosporins are commonly used
as monotherapy. Azythromycin is necessary for Mycoplasma pneu-
moniae. If aspiration is suspected, gram-negative and anaerobic
coverage is very important so consider adding clindamycin or
ampicillin-sulbactam. Hospital-acquired pneumonia may be
caused by Pseudomonas, Enterobacter, or Acinetobacter species,
as well as Serratia, especially in the cystic fibrosis population.
P. carinii requires intravenous pentamidine or trimethoprim-
sulfamethoxazole (Bactrim).
In the setting of significant influenza in the community, an anti-
viral agent can be started if within the window of efficacy, usually
within 4 days of onset of symptoms. However, in the case of signif-
icant disease, initiation of therapy is warranted and can be
reevaluated.
Bronchodilators. A mainstay of therapy for reactive airway
disease, bronchodilators may have some utility in treating lobar
bacterial pneumonia but are controversial in bronchiolitis. Nebu-
lizer treatments combined with bronchial hygiene therapy may
help loosen inspissated secretions and mucous plugs. Remember
that bronchodilators are β2-agonists but have β1 activity and result
in tachycardia, jitteriness, and sometimes agitation. Albuterol in
particular also affects extracellular potassium transport.
Pulmonary Hygiene. Infants are obligate nose breathers and as
such are more affected by obstruction of the nasopharynx. Consider
nasal or nasopharyngeal suctioning to relieve secretions in a patient
with bronchiolitis. If concerned for atelectasis on physical exam or
radiograph, consider asking a respiratory therapist to perform chest
physiotherapy or positive expiratory pressure (PEP) therapy.
294 Patient-Related Problems

Steroids. Another mainstay in the treatment of reactive airway

disease, steroids have limited application in patients with pneumo-
nia. Children with cystic fibrosis or immunocompromised children
suspected of having Pneumocystis pneumonia may be considered
for steroids.
Antituberculosis Regimens. Tuberculosis (TB) must be trea-
ted with at least a two-drug regimen and often requires three- or
four-drug combinations. Rifampin, isoniazid, ethambutol, pyrazi-
namide, and streptomycin are recommended in various combina-
tions for extended periods.

Airway Processes
Selective History
Is there any history of a sudden choking or coughing spell preced-
ing the respiratory distress?
Has the child’s voice changed?
Can the child phonate?
Is there dysphagia or drooling?
Was the onset of respiratory distress sudden?
Was it associated with sudden high fever, chemical or noxious gas
inhalation, or neck trauma?
Selective Physical Examination
Can the child swallow?
Is the child drooling?
Does the child hold his or her head in a particular position or
assume a “tripod position”?
Is there evidence of a foreign body in the oropharynx?
Is the neck swollen or mobile, and does the child have any cervical
adenopathy?
What is the appearance of the pharynx? (Caution: If acute suppu-
rative epiglottitis is suspected, examination of the pharynx
should occur in the operating room with an anesthesiologist
and otolaryngologist present.)
Is the trachea in the midline?
Can the child phonate?

Airway Films
A lateral neck film helps to evaluate the integrity of the airway from
the nasopharynx to the midtrachea. Significant tonsillar or adenoid
enlargement, retropharyngeal abscess or cellulitis, epiglottitis, tra-
cheal pseudomembrane, and foreign bodies may be seen. An ante-
roposterior airway film may show subglottic steepling in
parainfluenza (croup), deviation of the trachea, foreign bodies,
or external compression of the trachea.
 Respiratory Distress 295

Laboratory Evaluation
Febrile infants and children must be handled carefully if epiglottitis
is suspected. Laboratory studies should include a complete blood
count, blood culture, and possibly an arterial blood gas determina-
tion but should be postponed until the airway has been visualized
and secured. Because not all foreign bodies are radiopaque, otolar-
yngology and/or general surgical consultation should be obtained
for possible rigid bronchoscopy.

Treatment
Disturb a child with suspected epiglottitis as little as possible, and
expedite transfer to the operating room for visualization. If con-
cerned for croup, give humidified oxygen and consider nebulized
epinephrine with or without steroids for subglottic edema. For sup-
purative paratracheal processes, broad-spectrum antibiotics should
be initiated promptly. Abscesses of the tonsils and retropharynx
should be surgically drained as well. In a child with upper airway
redundant soft tissue may have positional stertor. Consider repo-
sitioning these patients.

Cardiac Processes
Congestive heart failure in infants and children is most often the
result of left-to-right shunts as a consequence of congenital heart
disease. Because of pulmonary overcirculation, pulmonary edema
develops and respiratory distress gradually ensues.
Selective History
Is there a known cardiac defect?
In infants, what is the child’s feeding pattern?
How has the child been growing?
Does the child become dyspneic, diaphoretic, and tired with
feedings?
What medications does the child take?
Was the onset of symptoms abrupt and associated with pleuritic
chest pain?
Selective Physical Examination
General Assess the child’s volume status. Is there fluid
overload?
HEENT Dysmorphic features (high association with
congenital heart disease)
Neck Jugular venous distention (rarely seen in infants
and young children)
Chest Symmetry, precordial activity
Respiratory Rales, crackles at the bases, pleuritic pain,
effusion
296 Patient-Related Problems

Cardiovascular Location of the point of maximal intensity;

abnormal impulses (right ventricular heave,
thrills); tachycardia; S1; S2, including splitting;
S3; murmurs (systolic and diastolic); clicks,
rubs, or gallops; brachial and femoral pulses
Abdomen Hepatosplenomegaly, hepatojugular reflex,
ascites
Extremities Peripheral edema, thrombophlebitis
The most common congenital defect is a ventricular septal
defect, which usually results in a left-to-right shunt and pulmonary
overcirculation. Other lesions can cause pulmonary edema, includ-
ing patent ductus arteriosus, any of the left-sided obstructive
lesions (coarctation, aortic stenosis, mitral stenosis), cardiomyop-
athies (dilated, hypertrophic, or restrictive), and some
dysrhythmias.
Chest Radiographic Findings
Cardiomegaly (Fig. 28.1)
Increased pulmonary vascular markings
Right-sided aortic arch

Pulmonary vascular redistribution

Perihilar congestion
(“batwing” appearance)

Kerley B lines

Interstitial or
alveolar edema
Cardiomegaly
(C:T ratio >0.5)
Pleural effusion
FIGURE 28.1 Chest radiographic features of congestive heart
failure. C:T ratio, cardiac diameter to thoracic diameter. (From
Marshall SA, Ruedy J: On Call: Principles and Protocols, 4th ed.
Philadelphia, Elsevier, 2004, p 270.)
 Respiratory Distress 297

Area of oligemia

Raised hemidiaphragm
with atelectasis
Unilateral wedge-shaped infiltrate

Unilateral pleural effusion

FIGURE 28.2 Variable chest radiographic features of pulmonary
embolism. (From Marshall SA, Ruedy J: On Call: Principles and
Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 274.)

Kerley B lines
Pleural effusion
Pulmonary embolism findings (Fig. 28.2)

Laboratory Evaluation
A 12- to 15-lead electrocardiogram should be obtained. If the child
is desaturated, a hyperoxia test should be performed (see
Chapter 13). Definitive diagnosis may require cardiology consulta-
tion and an echocardiogram. Serum electrolytes, blood urea nitro-
gen, and creatinine should be checked to assess hydration status
and renal function. If pulmonary embolism is suspected, an arterial
blood gas determination is essential. A ventilation-perfusion (V/Q)
scan or high-resolution computed tomography (CT) scan is
necessary.

Treatment
GENERAL MEASURES
• Oxygen
• Elevate the head of the bed 30 degrees
298 Patient-Related Problems

SPECIFIC MEASURES
Frequently, the first response to a child with apparent cardiogenic
respiratory distress is to give intravenous or intramuscular furose-
mide (Lasix). Although this may be indicated in infants and chil-
dren with left-to-right shunt lesions, it could be disastrous in a
child with cardiomyopathy who depends on a high end-diastolic
volume or atrial filling pressure to maximize ventricular volume
and maintain cardiac output. Therefore it is imperative to define
the child’s physiologic features and obtain the cardiac diagnosis
underlying the respiratory compromise before empirically treating
with a diuretic.
If the child has known cardiac disease and is receiving diuretic
therapy, an intravenous dose can be administered to augment
diuresis. If a dilated cardiomyopathy is the problem, inotropic sup-
port is necessary, and diuretics may initially be contraindicated.
Mitral regurgitation may be helped by reduction of systemic
afterload.
Pericardial tamponade is a life-threatening emergency that is
frequently manifested as respiratory distress with left chest and
shoulder pain and coughing. Orthopnea is marked. Drainage of
the pericardium, ideally via a catheter placed under echocardio-
graphic guidance, is necessary.
In a crisis, one may acutely decompress the pericardium as fol-
lows. First, the area of the xyphoid is prepared in sterile fashion.
The skin and subcutaneous tissues should be anesthetized with
1% lidocaine. A No. 18 or 20 angiocatheter can then be placed
on a three-way stopcock and attached to a 20-mL syringe. The
angiocatheter is inserted lateral to the xyphoid, aiming for the left
shoulder and aspirating as it is inserted. Watch the electrocardio-
graphic monitor for signs of ectopy. If straw-colored fluid or thin
bloody fluid is obtained, the catheter should be threaded fully and
the needle withdrawn. The stopcock can then be attached directly
to the catheter and the fluid aspirated. If blood is obtained again,
check for ventricular ectopy and for pulsatile flow. If you suspect
that you have entered the right ventricle, remove the apparatus
and be prepared to give volume replacement. Obviously, placement
of sharp objects very close to a beating heart is to be done carefully.
If pulmonary embolism is suspected, anticoagulation therapy
must be started immediately. Be certain that the child has no con-
traindications to anticoagulant therapy, such as a history of a coa-
gulopathy, previous stroke, peptic ulcer disease, or bleeding
disorder. A baseline complete blood count, activated partial throm-
boplastin time (aPTT), prothrombin time, and platelet count must
be obtained. Heparin is the mainstay of initial therapy and should
be started with a 100-U/kg bolus, followed by an infusion of 15 to
 Respiratory Distress 299

25 U/kg per hour. The aPTT must be monitored closely and the
heparin infusion adjusted to maintain the aPTT at 1.5 to 2 times
baseline. Thrombolytic therapy is much higher risk and requires
transfer to the Pediatric Intensive Care Unit (PICU) and consulta-
tion with both cardiology and cardiovascular surgery specialists.
Space-Occupying Processes
Selective History
Does the patient complain of pleuritic pain?
Was the onset gradual or sudden?
Is the patient febrile?
Has the patient ever had symptoms like this before?
In a newborn, could this be a congenital diaphragmatic hernia?
Selective Physical Examination
Is the trachea in the midline?
Do both sides of the chest move together?
Is there obvious splinting?
Is there evidence of ascites or other abdominal processes that are
limiting diaphragmatic excursion?
Is there a pleural rub?
Are the breath sounds equal right and left?
Is the abdomen scaphoid?

Chest Radiographic Findings

Pleural effusion
Empyema
Pneumothorax
Intrathoracic masses, including the mediastinum
Severe scoliosis
Massive ascites
Diaphragmatic hernia
Laboratory Evaluation
Thoracentesis may be indicated for diagnosis, as well as therapy.
Arterial blood gases should be monitored in children with severe
distress. Pleural and/or mediastinal masses require chest CT scan-
ning and/or magnetic resonance imaging after the child’s airway
and breathing are secure.
Special Section on Asthma
Asthma is one of the most common admitting diagnoses in pedi-
atrics. As a result, there is often a tendency to become complacent
regarding the risk for decompensation in these children. Remem-
ber, asthma can cause respiratory distress very quickly.
300 Patient-Related Problems

Selective History
Did the child’s condition suddenly become worse?
Has the child ever required intubation?
Are there any obvious precipitating triggers?
Is this an anaphylactic reaction?
What are the child’s current medications?
Selective Physical Examination
Is there evidence of acute airway obstruction?
Vital signs Pulsus paradoxus
HEENT Cyanosis
Neck Midline trachea, jugular venous distention
Respiratory Retractions, flaring, prolonged expiration, abnormal
inspiratory-expiratory ratio, hyperinflation,
wheezing, aeration, consolidation

Chest Radiographic Findings

Hyperinflation
Pneumothorax or pneumomediastinum
Flattened diaphragm
Atelectasis, infiltrates
Laboratory Evaluation
Arterial blood gases are very important in the assessment of an
asthmatic patient who has deteriorated acutely. Pulse oximetry
studies may be falsely reassuring when the patient’s PCO2 has begun
to rise, which is indicative of impending respiratory failure.
Treatment
GENERAL MEASURES
• Oxygen
• Intravenous hydration
SPECIFIC MEASURES
The initial response should be nebulizer treatments with β-agonists
(albuterol, 2.5 to 10 mg) as often as necessary or even continuously.
The anticholinergic agent ipratropium bromide, 0.5 mg, may also be
given via nebulizer in conjunction with albuterol. Side effects from
either are obviously greater with an increased frequency of treatments.
Intravenous steroids should be given immediately (2 mg/kg
methylprednisolone). Steroids should be continued every 4 hours
initially at a dose of 1 mg/kg methylprednisolone.
In patients who remain in significant distress, xanthines, such
as aminophylline or theophylline, can be used. An initial bolus
 Respiratory Distress 301

of 6 mg/kg should be followed by a continuous infusion of 1.0 mg/

kg per hour. Serum levels should be monitored closely until a
steady state is achieved. Higher levels may cause nausea, vomiting,
tachycardia, chest pains, headache, and irritability. Be careful when
also giving erythromycin, cimetidine, β-blockers, allopurinol, and
other drugs that may potentiate xanthine drug effects or affect
serum levels.
Additional treatment may include intubation and mechanical
ventilation, intravenous ketamine, and magnesium sulfate. Consul-
tation with the critical care staff and transfer to the PICU will be
necessary at the point that these measures are considered.

Warning Signs in Asthma

1. Sudden acute deterioration may signal the development of
pneumothorax.
2. A rising PCO2 in the face of maximal therapy portends respira-
tory failure. Arterial blood gases must be monitored closely.
3. The disappearance of wheezing is not always a good sign. Lack
of wheezing may reflect lack of air exchange and indicate respi-
ratory failure. Similarly, not all respiratory noises in asthma are
wheezing. Asthmatic children also can get upper airway
obstruction and inhaled foreign bodies like other children.
4. A sleepy patient with asthma is a worrisome patient. Because
sedatives are contraindicated and both β-agonists and xan-
thines are stimulants, most patients will be hyperalert or
agitated.
5. Rarely, there is a triad of asthma, nasal polyps, and aspirin
hypersensitivity. Avoid aspirin and nonsteroidal antiinflamma-
tory drugs whenever possible in patients with asthma because
fatal anaphylactoid reactions have been described.

Respiratory Failure
Any of the aforementioned conditions may lead to respiratory fail-
ure. Bradypnea (<20 breaths per minute), thoracoabdominal dis-
sociation, CO2 retention, profound hypoxemia, and profound
respiratory acidosis all imply respiratory failure.
1. Ensure that the patient has not received or is not receiving any
respiratory depressant, especially narcotics, barbiturates, and
benzodiazepines. Do not hesitate to give naloxone hydrochlo-
ride (Narcan), 0.2 to 2.0 mg intravenously, if opiates are
suspected.
2. Notify the PICU early of a patient in distress. Direct therapy to
the underlying causes of the respiratory problem, and assist
ventilation and oxygenation as indicated. Acute respiratory aci-
dosis frequently requires mechanical ventilatory support until
the underlying cause is addressed.
302 Patient-Related Problems

REMEMBER
1. Abdominal problems may cause significant respiratory distress
and compromise.
2. Do not be worried about your inexperience with endotracheal
intubation. Unless there is severe upper airway obstruction,
most patients can be effectively ventilated for an extended time
with a bag-valve mask unit until help and more hands arrive.
3. Even though respiratory distress is a very common cause of calls
in the middle of the night, it is essential to monitor patients fre-
quently to make sure that they are responding appropriately to
your treatment so that you can make changes in treatment as
indicated.
 C HA P TE R

29
Seizures
Purabi Sonowal, MD

When a seizure unexpectedly develops, the sudden and often dra-

matic nature of the event has a tendency to create a sense of crisis
among parents, other family members, nurses, and house officers.
Everyone will feel a need to “do something” and to do it quickly to
stop the seizure. The first order of business when you are called is to
remain calm and recognize that, although a seizure needs to be
addressed immediately, the urgency to “do something” should
not lead you to act reflexively or irrationally. There is time to orga-
nize your thoughts and develop a plan for further evaluation and
treatment that is best for the patient. Remember, almost all seizures
are paroxysmal events with abrupt onset, are variable in length but
usually brief (minutes), and are generally self-limited. Careful
attention to the airway, breathing, and circulation (ABCs) is often
all that is initially necessary because this will maintain cerebral
blood flow and oxygenation while you consider the need for further
treatment or diagnostic tests.

PHONE CALL
Questions
1. Is the child still seizing?
2. What was witnessed? Ask the nurse to describe what happened.
Was it generalized or focal, tonic-clonic, or just tonic? (Was the
event actually a seizure and not merely a startle response or
myoclonus?)
3. What was the patient’s level of consciousness?
4. Was the event associated with apnea, cyanosis, or loss of blad-
der or bowel control?
5. What is the child’s admitting diagnosis?
6. Is the child febrile?

303
304 Patient-Related Problems

Orders
1. Ask the nurse to see that the child is positioned on his or
her side.
2. Ask the nurse to maintain seizure precautions, including suc-
tioning and oxygen supplies at the bedside, padded bedrails,
a properly sized oral airway at the bedside, and intravenous
(IV) lorazepam readily available.
3. If the child does not have an IV line in place, ask the
nurse to have the supplies at the bedside and to have an
IV placed.
4. Ask the nurse to also keep ready either nasal midazolam or rec-
tal diazepam because if the child does not have an IV access,
then it might be difficult to get an IV access if the child is still
seizing and in that case you might need either of these to control
the seizure.
5. Ask the nurse to obtain a full set of vital signs immediately and
put the patient on continuous pulse oximetry monitoring.
6. Check a point of care blood glucose.

Inform Registered Nurse

Tell the RN, “I will arrive at the bedside in … minutes.”
Seizures require immediate evaluation.

ELEVATOR THOUGHTS
Did the child have a seizure?
Remember that several conditions can mimic seizures, includ-
ing breath-holding spells, syncope, chorea, narcolepsy, benign
myoclonus, night terrors, and pseudoseizures. Your first task will
be to determine the likelihood that the child experienced a seizure.
You should be able to do this after your telephone discussion with
the nurse.
What causes seizures?
There are several types of seizures (Table 29.1) and an even lon-
ger list of potential causes (Box 29.1).

MAJOR THREAT TO LIFE

• Aspiration
• Hypoxemia
The majority of seizures will have stopped by the time you
arrive at the child’s bedside. Advise the nurse to try to position
the child on his or her side to discourage airway obstruction or
aspiration during the postictal state. Patients are rarely apneic
 Seizures 305

TABLE 29.1 Classification of Epileptic Seizures and Some

Epileptic Syndromes

Clinical Seizure Type Epileptic Syndrome

Partial Seizures
Simple partial (consciousness not impaired) Benign focal epilepsy
Motor signs Juvenile myoclonic epilepsy
Special sensory (visual, auditory, olfactory, West syndrome
gustatory, vertiginous, or Lennox-Gastaut syndrome
somatosensory)
Autonomic Acquired epileptic aphasia
Psychic (dejà vu, fear, and others) Benign neonatal convulsions
Complex partial (consciousness impaired)
Impaired consciousness at onset
Development of impaired consciousness
Generalized Seizures
Absence
Typical
Atypical
Tonic-clonic
Atonic
Myoclonic
Tonic
Clonic
Unclassified
Neonatal
From Behrman RE, Kliegman R (eds): Nelson Essentials of Pediatrics, 2nd ed. Philadelphia,
WB Saunders, 1994, p 681.

BOX 29.1 Etiology of Seizures

Perinatal Conditions
Cerebral malformation
Intrauterine infection
Hypoxia-ischemia*
Trauma
Hemorrhage*
Infections
Encephalitis*
Meningitis*
Brain abscess
Metabolic Conditions
Hypoglycemia*

Continued
306 Patient-Related Problems

BOX 29.1 Etiology of Seizures—cont’d

Hypocalcemia
Hypomagnesemia
Hyponatremia
Hypernatremia
Storage diseases
Reye syndrome
Degenerative disorders
Porphyria
Pyridoxine dependency (deficiency)
Poisoning
Lead
Drugs
Drug withdrawal
Neurocutaneous Syndromes
Tuberous sclerosis
Neurofibromatosis
Sturge-Weber syndrome
Klippel-Trenaunay-Weber syndrome
Linear sebaceous nevus
Incontinentia pigmenti
Systemic Disorders
Vasculitis (central nervous system or systemic)
Systemic lupus erythematosus
Hypertensive encephalopathy
Renal failure
Hepatic encephalopathy
Other
Trauma*
Tumor
Febrile*
Idiopathic*
Familial
*Common.
From Behrman RE, Kliegman R (eds): Nelson Essentials of Pediatrics, 2nd ed. Philadelphia,
WB Saunders, 1994, p 681.

during a seizure. Children can usually withstand status epilepticus

for up to 30 minutes with no subsequent neurologic damage.
The procedures to follow if the seizure has stopped are discussed
subsequently, as are those for status epilepticus.

BEDSIDE
If the Seizure Has Stopped
 Seizures 307

Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable or
distressed), or critical (about to die)?
Most children have a period of postictal unresponsiveness after
a generalized tonic-clonic seizure. Prolonged depression of mental
status is ominous and requires prompt evaluation with head com-
puted tomography (CT) or magnetic resonance imaging (MRI).
A child in shock must be stabilized while addressing the seizure.

Airway and Vital Signs

In what position is the child lying?
The patient should be positioned in the left lateral decubitus posi-
tion to prevent aspiration of vomited gastric contents (Fig. 29.1). Do
an immediate oral suctioning if there are secretions in the mouth.

FIGURE 29.1 Positioning of the patient to prevent aspiration of

gastric contents. (From Marshall SA, Ruedy J: On Call: Principles
and Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 254.)
308 Patient-Related Problems

If the child is unresponsive but adequately ventilating, it is prudent to

insert an oral airway. (An awake child does not tolerate an airway, so
be prepared to remove it as the child awakens.) Oxygen should be
given via nasal prongs or face mask. Have the nurse obtain a repeat
set of vital signs, again with a pulse oximeter saturation.
What is the blood glucose result?
Hypoglycemia may be rapidly treated, and raising the serum
glucose level may prevent further hypoglycemic seizures.

Management I
After you have established that the event was likely to be a seizure,
establish IV access, and draw blood for the following studies: elec-
trolytes, glucose, magnesium, calcium, blood urea nitrogen, creat-
inine, and serum levels of any anticonvulsant medications that the
child may be taking. A toxicology screen and a serum lead level
should also be considered. A complete blood count should be
obtained, as well as a blood culture if the child is younger than
4 years and has a significant fever. A venous pH determination
should be made after any prolonged seizure, and an arterial blood
gas determination should be considered and obtained if any respi-
ratory compromise is evident. If encephalitis or meningitis is a con-
sideration because of the presence of fever or other signs and
symptoms, a lumbar puncture with cerebrospinal fluid analysis
and culture will be necessary.

Selective Physical Examination I

Mental Assess the response to verbal, tactile, and painful
status stimuli. Altered level of consciousness is discussed
in Chapter 7, Altered Mental Status
Airway Check body position, airway patency, and quality of
breath sounds

Selective History and Chart Review

Was the event witnessed? Ask witnesses about the characteristics
and duration of the seizure.
Was it generalized tonic-clonic or focal?
Did the seizure start focally or was it generalized?
Did the child suffer any injury as a result of the seizure
(head trauma, tongue or lip trauma, bruises or lacerations on the
extremities)?
Is the child normally receiving anticonvulsants or any other
medications that might lower the child’s seizure threshold?
What were the child’s most recent laboratory results? Quickly
review the child’s chart before a more complete physical
examination.
 Seizures 309

Selective Physical Examination II

Mental status Assess whether the child has lost consciousness.
Does the child respond to verbal, tactile, or
painful stimuli? Is the child in a postictal state?
HEENT Test the cranial nerves; again assure yourself that
the child can defend his or her airway (gag
reflex), and check the airway position
(Fig. 29.2). Look for a potential source of
infection in febrile patients (e.g., otitis,
sinusitis). Any children who have had a seizure
must have their fundi examined thoroughly,
HEENT, Head, Ears, Eyes, Nose, Throat.

FIGURE 29.2 Airway management: correct positioning of the

head, correct suctioning, and correct insertion of an oral airway.
A, Neck flexion closes the airway. B, Neck extension to the sniffing
position opens the airway. C, Suctioning. D, Placement of the air-
way. (From Marshall SA, Ruedy J: On Call: Principles and Proto-
cols, 4th ed. Philadelphia, Elsevier, 2004, p 255.)
310 Patient-Related Problems

with dilation of the pupils if necessary,

especially in infants (to look for retinal
hemorrhages, as well as evidence of
papilledema)
Neck Nuchal rigidity
Lungs Signs of aspiration (crackles, decreased breath
sounds)
Neurologic Complete neurologic examination within the
limits of the child’s level of consciousness,
including reflexes, motor and sensory function,
cerebellar function, visual fields, and short- and
long-term memory
Miscellaneous Check for oral and/or scalp lacerations, passive
range of joint mobility, bruising, and other
signs of injury incurred during the seizure

Management II
Given the history, one should establish a preliminary or provisional
differential diagnosis. Remember that seizures are a symptom, not
a diagnosis, and therefore there must be an underlying condition
that is manifested by the seizure. Your job is to find and treat
the underlying condition. In children, even hospitalized children,
fever is a common underlying cause of seizures. It is thought that
the rapidity with which the temperature rises precipitates the sei-
zure activity. It may be difficult, however, to determine whether a
febrile child with a seizure has simply had a “febrile seizure” or has
had a seizure secondary to meningitis, encephalitis, or brain
abscess. When this distinction cannot be made, it is necessary to
perform a lumbar puncture to rule out meningitis. CT or MRI
may be necessary before lumbar puncture to rule out cerebral
edema and increased intracranial pressure.
Any child with an abnormal neurologic examination after a sei-
zure should also have an imaging procedure (CT or MRI) per-
formed to rule out tumor, edema, or other space-occupying
lesions (arteriovenous malformation, abscess) as a cause of the
seizure.
Remember also that there can be complications secondary to
seizures, such as aspiration and trauma, especially to the head. Sei-
zure precautions should be ordered immediately whenever an
inpatient suffers a seizure.
In a hospitalized child who has a first-time seizure, it is usually
prudent to maintain IV access but to withhold anticonvulsant ther-
apy if the seizure is not prolonged and some readily identifiable
underlying cause is apparent. Exceptions include a patient with a
 Seizures 311

known seizure disorder who has a subtherapeutic serum anticon-

vulsant level. This, in fact, is the most common cause of in-hospital
seizures in children. Other patients who warrant anticonvulsant
therapy are victims of head injuries, children with central nervous
system tumors, and those with known cerebrovascular accidents.
Remember that benzodiazepines, such as diazepam (Valium)
and lorazepam (Ativan), are useful in stopping status epilepticus
but have no role in preventing recurrent events. The choice of anti-
convulsant depends in part on the type of seizure and the age of the
child. Anticonvulsant therapy should be discussed with a child
neurologist (see Box 29.1).
If the Child Is Still Seizing
Don’t panic! Most seizures resolve spontaneously and last no
more than several minutes. (This, of course, is an eternity to the
child’s parents and often the nurses.) Therefore busy yourself with
evaluating the child’s ABCs. The child needs the evaluation, and it
calms the parents to see that “something” is being done.
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
A generalized tonic-clonic seizure is a disconcerting event to
witness. However, one should be reassured that a child who is seiz-
ing has both a heart rate and a blood pressure. Still, remember your
limitations (a seizure is a medical emergency), and have a nurse
page your senior resident immediately. You should not deal with
a seizure alone if you do not absolutely have to do so. If there is
clonic activity of the extremities, gently hold the extremity to see
whether you can suppress the activity. If so, it is not seizure activity.
Airway and Vital Signs
In what position is the patient?
If at all possible, the child should be positioned in the lateral
decubitus position with suction readily available to prevent aspira-
tion of gastric contents. Be prepared to restrain the child gently but
firmly to prevent traumatic injury. Patency of the airway should be
the first concern, followed by adequacy of ventilation. Apply oxy-
gen by mask or nasal prongs.
What are the child’s vital signs?
Tachycardia is expected with a seizure. It is virtually impossible
to obtain an accurate cuff blood pressure reading during a tonic-
clonic seizure. The child’s perfusion tells as much as a blood pres-
sure reading fraught with inaccuracy. A point of care blood glucose
assessment is indicated because hypoglycemia commonly results in
seizures (and is easily corrected).
312 Patient-Related Problems

Management I
How long has the child been seizing?
See earlier if the seizure has already stopped. If the seizure has
lasted more than 3 minutes, first check the child’s ABCs. Closely
observe the seizure. Be sure that IV supplies are at hand if the child
does not have a working IV line. Do not try to obtain IV access in a
seizing patient unless it is absolutely necessary. Remember, the sei-
zure is likely to be over in 2 to 3 minutes. Also remember that the
antecubital fossa becomes a less attractive site for IV access in a
patient who involuntarily flexes at the elbow. A hand, forearm,
or saphenous site will be a better choice.

Medications
Status epilepticus is defined as general or partial seizures lasting
longer than 30 minutes without the patient regaining conscious-
ness. Before administering any medication, remember that all anti-
convulsants can depress the child’s level of consciousness and
respiratory drive. It is best to have airway support equipment at
hand, including suctioning supplies, Ambu bag, appropriately
sized masks, and a laryngoscopy tray. If the child has been seizing
for 5 minutes, begin to prepare airway support equipment, check a
point of care (finger stick) glucose and administer an IV glucose
bolus (5 mL/kg of 10% dextrose in water [D10W]) if hypoglycemia
is present. Change the IV solution to normal saline, and have anti-
convulsant doses readied. Often, this process requires enough time
for the seizure to stop spontaneously. In any case, most clinicians
agree that treatment should probably be initiated after 10 to
15 minutes of continuous seizure activity. Do not let the presence
of a frantic parent or nurse force you into treating the child phar-
macologically before you are ready and comfortable doing so. The
treatment is not without complications, and the risks of treatment
should be considered along with the benefits.
The most important principle of anticonvulsant therapy is
to choose a drug and use enough. Small doses of multiple drugs may
be ineffective. Use full loading doses and do not administer additional
drugs until you have reached the maximum recommended dose.
Diazepam and lorazepam are effective immediately in most
children for tonic-clonic seizures. Diazepam has a short half-life,
and seizures will tend to recur unless a longer-acting anticonvul-
sant is also administered. The starting dose of diazepam (see
Table 29-2 for dosing). This may be repeated every 10 to 15 minutes
to a maximum of three doses. Lorazepam has the benefit of longer
duration of action and is less likely than diazepam to result in hypo-
tension and respiratory depression, (see Table 29-2 for dosing).
 Seizures 313

If an IV line cannot be established, both diazepam and loraze-

pam can be given rectally. A rectal diazepam gel in standard doses
of 2.5, 5, and 10 mg is now available. The rectal and IV doses of
lorazepam are identical. Midazolam may be given intranasally if
no IV is present. The dose is 0.2 mg/kg to a maximum dose of
10 mg. It is preferable to give via a nasal insuflator, but it can be
rapidly given with a syringe as well.
Phenytoin should be used next and is generally begun at a load-
ing dose of 15 to 20 mg/kg at an infusion rate no faster than 1 mg/
kg/min. All patients should be on a cardiorespiratory monitor
when receiving phenytoin because of the risk for dysrhythmias.
If bradycardia or hypotension results, the infusion must be slowed.
The prodrug fosphenytoin has been used increasingly because it is
more water soluble and less irritating when administered intrave-
nously. The loading dose of fosphenytoin is usually 15 to 20 phe-
nytoin equivalents (PE)/kg. If seizure activity persists, another dose
of phenytoin may be given, up to a 25-mg/kg total loading dose.
Remember that phenytoin forms a precipitate with glucose solu-
tions and must therefore be administered in saline solution.
Phenobarbital is often the second-line drug for status epilepti-
cus. The loading dose is 5 to 20 mg/kg infused no faster than 1 mg/
kg/min. Monitor vital signs, especially respirations and pulse oxim-
etry values. After phenobarbital loading, the child may remain
sedated for a period of hours.
Levetiracetam has also been used for status epilepticus with doses
of 20 to 60 mg/kg with 50 mg/kg being a common starting point.
Table 29.2 has a listing of the drugs used for status epilepticus.
Persistent status seizure activity despite the administration of
three anticonvulsants warrants transfer to the intensive care unit
for the administration of a continuous infusion of diazepam, pro-
pofol, or pentobarbital. Correct any electrolyte abnormalities.
After the child is no longer in status epilepticus, maintenance
therapy depends on the type of seizure. Consultation with a pedi-
atric neurologist is recommended, especially to help educate the
family about seizure disorders, prognosis, and medication manage-
ment. Further evaluation, including lumbar puncture, MRI or CT,
or electroencephalography, may need to be pursued.

SUMMARY
Seizures are upsetting for all who witness them. The specific char-
acteristics of the episode will help you to determine whether the
patient has in fact had a seizure and, if so, what type of seizure
occurred. Because most seizures last less than 5 minutes, the most
important emergency intervention is to prevent secondary injury,
aspiration, and respiratory compromise. Remember to address the
314 Patient-Related Problems

TABLE 29.2 Doses of Commonly Used Antiepileptic Drugs

in Status Epilepticus

Druga Route Dosage

Lorazepam Intravenous 0.1 mg/kg up to 4 mg total, may repeat in
5–10 min
Intranasal 0.1 mg/kg
Midazolam Intravenous 0.2 mg/kg up to 10 mg total dose, may
repeat in 5–10 min
0.08–0.23 mg/kg/h maintenance
Intramuscular 0.2 mg/kg
Intranasal 0.2 mg/kg
Buccal 0.5 mg/kg
Diazepam Intravenous 0.15 mg/kg up to a max total dose of
10 mg, may repeat in 5–10 min
Rectal 2–5 years: 0.5 mg/kg
6–11 years: 0.3 mg/kg
12 years: 0.2 mg/kg
Fosphenytoin Intravenous 20 mg/kg phenytoin equivalents (PE), then
3–6 mg/kg/24 h, loading rate up to
50 mg PE per min
Phenobarbitalb Intravenous 5–20 mg/kg
Pentobarbital Intravenous 13.0 mg/kg, then 1–5 mg/kg/hr
comab
Propofolb Intravenous 1 mg/kg (bolus), then 1–15 mg/kg/h
(infusion)
Thiopentalb Intravenous 5 mg/kg/1st h, then 1–2 mg/kg/h
Valproateb Intravenous Loading: 25 mg/kg, then 30–60 mg/kg/
24 h
Lacosamideb Intravenous Loading: 4 mg/kg then 4–12 mg/kg/24 h
Levetiracetam Intravenous 20–60 mg/kg
Topiramate Enterally 5–10 mg/kg/24 h (loading dose) then
same or lower for maintenance
a
Reflects current trends in use which may not be FDA approved.
b
May cause PR prolongation.
From Behrman RE: Nelson Textbook of Pediatrics, 20th ed. Philadelphia, Elsevier, 2016,
Table 593-18.

ABCs before proceeding to pharmacologic seizure management. In

most cases the seizure will end before medications are adminis-
tered. Status epilepticus is continuous seizure activity without
regaining consciousness for 30 minutes. Treatment of status epilep-
ticus should include the following, in order: address the ABCs;
administer glucose intravenously; administer diazepam or loraze-
pam; administer phenytoin or fosphenytoin; administer phenobar-
bital; and transfer to the pediatric intensive care unit for respiratory
support and further management, such as continuous diazepam
infusion, pentobarbital, or paraldehyde.
 C HA P TE R

30
Urine Output
Abnormalities
Lea Steffes, MD

Urine output abnormalities can be a challenging problem. There

are a wide range of factors that influence urine output including
hydration status, cardiac output, intrinsic renal function, and uro-
logic patency to name a few. Urine output is closely measured by
the nurses in all pediatric hospitalized patients as a way to monitor
the function of multiple organ systems. Therefore, it is common for
a pediatric resident to be called with questions regarding too little
or too much urine output.

PHONE CALL
Questions
1. How old is the patient?
2. How much does the child weigh?
3. Why is the patient in the hospital?
4. How much urine has the patient produced in the last 24 hours?
5. How much fluid has the child taken in or been given over the
last 24 hours?
6. What are the vital signs?
7. What is the child’s admitting diagnosis?
8. When did the child last have an electrolyte panel and kidney
function checked?
Orders
1. If the child has an indwelling Foley catheter and decreased urine
output, ask the nurse to check the catheter for patency and flush
the catheter with 10 to 20 mL of normal saline solution if nec-
essary (see Chapter 26).

315
316 Patient-Related Problems

2. Consider ordering serum electrolyte, blood urea nitrogen

(BUN), and creatinine and urinalysis (pH and specific
gravity).
3. If the child does not have an intravenous (IV) line, place order
to insert peripheral IV and order appropriate fluids based on
findings of the electrolyte panel and kidney function.
4. If the child is receiving IV fluids and has decreased urine output
with signs of kidney dysfunction on lab results, any potassium
in the IV fluid should be removed or at least reduced. The rate
of the IV fluids should be adjusted carefully in response to the
urine output.

Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.”
Decreased urine output deserves fairly prompt evaluation,
because it can be a sign of decreased cardiac output, dehydration,
or renal failure. Increased urine output also demands evaluation
promptly, especially in neonates, infants, and small children. Keep
in mind that decreased urine output in neonates may result from
congenital anomalies of the genitourinary tract.

ELEVATOR THOUGHTS
Decreased Urine Output
What are the causes?
Reduced cardiac output Heart failure
(prerenal) Cardiomyopathy
Congenital heart disease
Pericardial effusion
Volume depletion
Vomiting
Diarrhea
Blood loss
Renal causes Tubulointerstitial problems
(acute tubulonecrosis,
nephrotoxic drugs)
Hemolytic-uremic syndrome
Hemoglobinuria, myoglobinuria
Acute crystalline nephropathy
(oxalosis, hyperuricemia)
Glomerulonephritis
Renal artery thrombosis
Renal artery embolization
 Urine Output Abnormalities 317

Postrenal causes Ureteropelvic junction (UPJ)

obstruction
Nephrolithiasis
Bilateral ureteral obstruction
Bladder outlet obstruction
(blocked Foley catheter,
urethral trauma, posterior
urethral valves)
Neurogenic bladder
Syndrome of inappropriate
antidiuretic hormone (SIADH)
production

Increased Urine Output

What are the causes?
Urinary tract infection
Central diabetes insipidus (DI)
Diuretic use
Distal tubular dysfunction
Nephrogenic DI
High-output phase of acute tubular necrosis
Proximal tubular dysfunction
Aminoaciduria (cystinuria, Hartnup disease)
Familial hypophosphatemic rickets (vitamin D–refractory
rickets)
Diabetes mellitus
Psychogenic polydipsia

MAJOR THREAT TO LIFE

• Renal failure
• Hyperkalemia
• Sepsis
Decreased urine output for any cause can become a self-
perpetuating situation, with progressive renal insufficiency leading
to renal failure. Hyperkalemia is the most serious and life-
threatening complication of renal insufficiency because of its high
association with cardiac dysrhythmias.

BEDSIDE
Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable or
distressed), or critical (about to die)?
318 Patient-Related Problems

Critically ill appearing children usually have decreased

perfusion with subsequent renal failure. An uncomfortable child
may have a distended bladder, flank pain, and/or cramps. Children
with serious renal problems frequently appear deceptively well.

Airway and Vital Signs

Check for postural changes and signs of dehydration. A postural
rise in heart rate greater than 15 beats per minute, a fall in systolic
blood pressure greater than 15 mm Hg, or any decrease in dia-
stolic pressure suggests significant hypovolemia. Baseline tachy-
cardia is frequently a nonspecific indicator of volume depletion
and/or stress. Fever suggests an infectious cause. Hypertension
is suggestive of a renal artery problem or glomerulonephritis.
Hypotension with tachycardia is concerning for hypoperfusion
of end organs.

Selective Physical Examination

Approach the physical examination with prerenal, renal, and post-
renal causes of decreased renal output in mind. Tachycardia,
delayed capillary refill, and dry mucous membranes suggest intra-
vascular depletion and thus a prerenal cause. Edema and hyperten-
sion suggest a renal cause. A tender, distended bladder suggests a
postrenal cause of decreased urine output.

HEENT Icterus (hepatorenal syndrome), facial purpura

and macroglossia (amyloidosis), periorbital
edema (nephrotic syndrome), mucous
membranes (dry or moist?)
Respiratory Crackles, rales, dullness to percussion (fluid
overload)
Cardiovascular Pulse rate and quality, capillary refill
Abdomen Enlarged kidneys (horseshoe kidney, UPJ
obstruction, polycystic kidney), bladder
fullness (bladder outlet obstruction), bladder
tenderness, flank, or costovertebral angle
tenderness
Rectal Enlarged prostate (rare in children)
Genitourinary Hypospadias
Pelvic (if Cervical and/or adnexal masses (UPJ
indicated) obstruction)
Skin Morbilliform rash, purpura, bruising, turgor,
jaundice
Extremities Peripheral edema (nephrotic syndrome, renal
failure)
HEENT, Head, Eyes, Ears, Nose, Throat.
 Urine Output Abnormalities 319

Selective Chart Review

Does the child have a past history of urinary tract infection, vesicour-
eteral reflux, instrumentation, or trauma to the genitourinary tract?
Are there other congenital anomalies?
Does the child have any history of renal failure?
What is the admitting diagnosis?
What medications is the child receiving?
Does the child have a condition that could lead to SIADH?
Has the child had laboratory studies recently that could indicate
a prerenal, renal, or postrenal cause of decreased urine output?
What has been the child’s fluid intake for the past 48 hours?
What was the urine output trend for the past 48 hours—was the
decrease in urine output sudden or gradual?
A BUN-creatinine ratio greater than 20 suggests a prerenal cause,
as does a urine specific gravity greater than 1.020 or a urine sodium
level less than 20 mmol/L. Table 30.1 illustrates laboratory differ-
ences in children with prerenal, renal, and postrenal insufficiency.
If urine output is excessive, DI must be considered. Central DI
may result from traumatic head injury, hypoxic-ischemic brain
injury, congenital abnormalities. Nephrogenic DI may be primary
(a rare X-linked recessive condition) or secondary to acute or
chronic renal failure with loss of tubular concentrating ability or
insensitivity to antidiuretic hormone at the tubules.

Management I: Decreased Urine Output

Prerenal
In hospitalized children, prerenal causes of decreased urine output
are relatively common, and you should be able to conclude whether
the cause is prerenal based on the history, the chart review, your
examination, and the urinalysis. Euvolemia is the goal. Fluid-
resuscitate a dehydrated child and diurese a child in congestive
heart failure.
Fluid boluses should always consist of isotonic solutions such as
0.9% saline. Children in acute or chronic renal failure are unable to
excrete potassium and are at risk for life-threatening hyperkalemia.

Postrenal
Lower urinary tract obstruction is usually easily managed by place-
ment of a Foley catheter in the bladder.
1. Bladder outlet obstruction in a newborn boy can be secondary
to posterior urethral valves and requires urologic surgical inter-
vention. Postobstructive diuresis is often observed once the
bladder is decompressed.
2. Obstruction of an indwelling Foley catheter can be relieved by
flushing the catheter with 10 to 20 mL of normal saline solution
 320
TABLE 30.1 Laboratory Differential Diagnosis of Renal Insufficiency

Patient-Related Problems
Prerenal Renal
Child Neonate Child Neonate Postrenal
Urine Na+ (mEq/L) <20 <20 to 30 >40 >40 Variable, may be >40
FENa (%)a <1 <2 to 5 >2 >2 to 5 Variable, may be >2
Urine osmolality (mOsm/L) >500 >300 to 500
300
300 Variable, may be <300
RFI (%)b <1 <2 to 5 >2 >2 to 5 Variable
Serum BUN-creatinine ratio >20 10
10 >10 Variable, may be >20
Response to volume Diuresis No change No change
Response to furosemide Diuresis No change No change or diuresis
Urinalysis Normal RBC, WBC, casts, proteinuria Variable or normal
Comments Hx: diarrhea, vomiting, hemorrhage, Hx: hypotension, anoxia, exposure to Hx: poor urine stream or output
diuretics nephrotoxins Px: flank mass, distended bladder
Px: volume depletion Px: hypertension, edema
a
FENa,¼ fractional excretion of sodium (%) ¼ (urine sodium/plasma sodium Π urine creatinine/plasma creatinine)  100.
b
RFI ¼ renal failure index ¼ (urine sodium/urine creatinine/plasma creatinine)  100.
BUN, Blood urea nitrogen; Hx, history; Px, physical signs; RBC, red blood cell; WBC, white blood cell.
From Behrman RE, Kliegman R (eds): Nelson Essentials of Pediatrics, 2nd ed. Philadelphia, WB Saunders, 1994, p 602.
 Urine Output Abnormalities 321

to displace the catheter from the bladder wall or to dislodge

bladder sediment.
3. Successful catheterization of the bladder can help localize the
level of obstruction (bladder outlet obstruction and lower uri-
nary tract obstruction). Upper urinary tract obstruction sec-
ondary to congenital anomalies or nephrolithiasis is best
diagnosed by ultrasonography or computed tomography (CT).
Renal
If prerenal and postrenal factors are not causing the patient’s poor
urine output, it is likely secondary to a renal or glomerular
condition.
Are any of the five potentially life-threatening complications or
consequences of renal failure present?
Hyperkalemia
Congestive heart failure
Severe metabolic acidosis (pH <7.2)
Uremic encephalopathy
Uremic pericarditis
Hyperkalemia is the most immediately threatening conse-
quence of low urine output. A serum potassium level should be
checked and an electrocardiogram should be obtained to check
for peaked T waves. Further indications that the serum potassium
level is dangerously high are conduction abnormalities, such as PR
and QRS prolongation and ST-T wave depression. Potassium-
containing intravenous fluids, including parenteral nutrition,
should be stopped.
Congestive heart failure is suggested by the presence of jugular
venous distention, tachypnea and rales, dependent edema, and an
S3 gallop. Treatment of congestive heart failure usually includes
fluid restriction, inotropic support as needed, diuretics, and respi-
ratory support as needed.
Tachypnea may represent pulmonary congestion and/or meta-
bolic acidosis as the child attempts to compensate by reducing
PCO2.
Uremic encephalopathy is generally manifested as a gradual
onset of confusion, stupor, or seizures and is almost always an indi-
cation for emergency dialysis.
Uremic pericarditis also requires dialysis and is usually mani-
fested as pleuritic chest pain radiating to the shoulder, pericardial
friction rub, distant or muffled heart sounds, and diffuse ST-
segment elevation on the electrocardiogram.
Is the patient taking any drug that may complicate renal
insufficiency?
Potassium supplements
Potassium-sparing diuretics (aldactone, triamterene, amiloride)
322 Patient-Related Problems

Nephrotoxic drugs (nonsteroidal antiinflammatory drugs

[NSAIDs], aminoglycosides)
Review the indications for each carefully and consider alterna-
tive medications if possible. If aminoglycosides are necessary,
serum peak and trough levels should be monitored closely.
Is the child in oliguric renal failure?
If a child produces less than 1 to 2 mL/kg per hour of urine, the
child has oliguric renal failure. The first goal of therapy is to convert
the patient to nonoliguric renal failure, which has a far better
prognosis:
1. Correct prerenal (fluid resuscitation) and postrenal factors
(bypass obstruction).
2. Give diuretics to increase urine output. Furosemide, 1 mg/kg per
dose, may be given intravenously. If there is no response within 1
to 2 hours, a double dose should be administered. (Larger doses
should be administered slowly to avoid ototoxicity.)
Does the child need dialysis?
If the child does not respond to diuretics, the indications for
urgent dialysis are any of the five complications of renal failure:
hyperkalemia, congestive heart failure with pulmonary edema,
metabolic acidosis, uremia, and complications of uremia, including
pericarditis and encephalopathy. A nephrology consultation is nec-
essary, and until dialysis can be arranged, it may be necessary to
treat the child for the aforementioned conditions with measures
that do not involve dialysis.
Hyperkalemia. Glucose with insulin infusion, NaHCO3, and
sodium polystyrene sulfonate temporarily reduce the serum potas-
sium level by driving K+ into intracellular fluid and binding K+
within the gastrointestinal tract for excretion. Furosemide can also
be given to increase potassium excretion in the urine. Calcium glu-
conate stabilizes the myocardium to help prevent dysrhythmias
secondary to hyperkalemia.
Congestive heart failure. Inotropy, afterload reduction, and
respiratory support should be provided as needed.
Metabolic acidosis. NaHCO3 provides correction of pH, but it is
only temporary. Fluid resuscitation with 0.9% normal saline if con-
cern for hypovolemia/hypoperfusion without congestive heart
failure.
Uremia. Uremic pericarditis rarely causes a sizable effusion that
requires pericardiocentesis. Conservative measures are recom-
mended, such as the use of NSAIDs, but they need to be used care-
fully with adequate hydration and close monitoring of renal
function. Aspirin and indomethacin are contraindicated, because
they may worsen acidosis.
Specific therapy for the renal etiology that caused renal failure,
such as glomerulonephritis, frequently depends on the results of
 Urine Output Abnormalities 323

renal biopsy. Routine urinalysis, renal ultrasound studies, and 24-

hour urine sampling for protein and creatinine should be ordered.
The urinalysis should be studied for the following:
Urine dipstick. Hematuria and proteinuria suggest glomerulo-
nephritis. Remember, a positive dipstick result for blood can mean
red blood cells, free hemoglobin, or myoglobin. Suspect rhabdomy-
olysis if the result is positive with few red blood cells by microscopic
examination. (In this case, check the creatinine phosphokinase, cal-
cium, phosphate, and urine myoglobin levels.) A positive urine
protein test result should prompt investigation of serum albumin,
and a 24-hour urine collection for protein and creatinine clearance
should be started. When the urine sample is concentrated (specific
gravity >1.015), the dipstick may be positive for protein in an oth-
erwise normal child. In this case, a spot urine protein-creatinine
ratio may be helpful (a ratio of 0.2 or less suggests normal renal
function).
Urine microscopy. Red blood cell casts are diagnostic of glo-
merulonephritis. Oval fat bodies are suggestive of nephrotic syn-
drome. White blood cells are characteristic of pyelonephritis and
can be seen with nephrolithiasis. Eosinophils are suggestive of
acute interstitial nephritis.
Management II: Increased Urine Output
The major threat of any form of DI is dehydration. It is important
to match urine output with adequate replacement fluid while search-
ing for the cause. Administration of desmopressin acetate (DDAVP)
intravenously or intranasally is both diagnostic (decreased urine
output in central DI; no change in urine output in nephrogenic, anti-
diuretic hormone–insensitive DI) and therapeutic for central DI.
If central DI is suspected, an endocrinology consultation is advisable
to investigate hypothalamic or pituitary function.
Urinalysis and urine culture should be ordered if there is any
suspicion of urinary tract infection or diabetes mellitus resulting
in polyuria. Urine and serum electrolytes, creatinine, and osmolar-
ity should also be determined. These tests can help differentiate
causes of increased urine output.
Glucose in urine increases suspicion for diabetes mellitus.
If glucosuria is present, you should check the serum glucose
level, venous pH, and urine for ketones while remembering that
diabetes mellitus in childhood is often manifested as diabetic
ketoacidosis.

REMEMBER
Many medications are excreted by the kidneys and have effects on
kidney function. All medications in an oliguric or anuric child must
324 Patient-Related Problems

be scrutinized and discontinued if they are nephrotoxic. Similarly,

drugs that require renal metabolism (digoxin, aminoglycosides)
must have their doses and dosing schedules modified and levels
closely monitored. If this is overlooked, drug levels could reach
toxic levels and result in significant problems for the patient.
 C HA P TE R

31
Vomiting
Alina G. Burek, MD

In hospitalized children, vomiting is often a nonspecific symptom

accompanying any illness. A single episode of vomiting or a few
instances of intermittent vomiting without additional significant
gastrointestinal or neurologic symptoms or signs is unlikely to
be indicative of a life-threatening problem. However, as with other
problems that arise while on call, it is critical that pediatric house
officers consider the potential for life-threatening causes of vomit-
ing and at least assure themselves that such potential causes have
been either excluded or evaluated and managed appropriately. It is
also important to have an understanding of the complications of
vomiting that may arise when vomiting has been excessive.

PHONE CALL
Questions
1. What is the child’s age?
2. Has the child been vomiting previously, or is this a new
symptom?
3. What is the child’s admitting diagnosis? Past medical history?
4. Is fever or diarrhea associated with the vomiting?
5. Is there blood or bile in the vomitus?
6. Does the child have an intravenous (IV) line in place?
7. Does the child appear to be in pain or complaining of pain?
8. Does the child have a headache or other neurologic symptoms?
9. What are patient’s most recent vital signs?
10. Any concerning changes on RN’s assessment?
Orders
1. The nurse should obtain a new full set of vital signs.
2. If the RN report is concerning, make the patient NPO until you
assess (see list of concerning symptoms/signs later).

325
326 Patient-Related Problems

3. Ask the nurse to hold on to the vomitus for direct observation

(this will provide you with great information!)
Inform RN
Tell the RN, “I will be at the bedside in … minutes.”
Vomiting in a neonate or very young infant can frequently be a
sign of a surgical problem and deserves evaluation promptly. In
older children, one may adjust the urgency of evaluation according
to the presence or absence of concerning signs/symptoms such as:
Bilious vomiting
Projectile vomiting
Hematemesis
Hematochezia
Neurologic symptoms (headache, altered consciousness, sei-
zures, etc.)
Severe hypertension
Reported physical examination findings such as abdominal dis-
tention, bulging fontanelle, etc.

ELEVATOR THOUGHTS
A brief review of the common causes of vomiting is best organized
by age.
Neonatal Anatomic
vomiting Gastroesophageal reflux
Esophageal duplication cyst
Duodenal atresia or stenosis
Ileal atresia
Ladd bands
Hirschsprung disease
Tracheoesophageal fistula (esophageal
atresia)
Pyloric stenosis
Annular pancreas
Malrotation
Meconium ileus
Anal atresia or imperforate anus
Metabolic
Inborn errors of metabolism
Adrenogenital syndrome
Toxic exposure
Perinatal drug exposure
Therapeutic drug overdose/side effects)
Intracranial (increased intracranial pressure
[ICP])
 Vomiting 327

Hydrocephalus
Subdural or subarachnoid hemorrhage
(e.g., nonaccidental trauma)
Infectious
Urinary tract infection
Necrotizing enterocolitis
TORCH (toxoplasmosis, other agents, rubella,
cytomegalovirus, herpes simplex) infection
Others
Dietary protein intolerance or allergy (e.g.,
milk-protein intolerance)
Infant and Anatomic
childhood Congenital anomalies of the gastrointestinal
vomiting tract
Hirschsprung disease
Intussusception
Malrotation
Swallowed foreign body (bezoar)
Intracranial
Brain tumor
Subdural hematoma
Hydrocephalus
Brain abscess
Metabolic
Inborn errors of metabolism
Uremia
Adrenogenital syndrome
Toxic ingestion
Diabetic ketoacidosis
Infectious
Gastroenteritis (viral vs. parasitic)
Hepatitis
Appendicitis
Bacterial colitis
Mesenteric adenitis
Urinary tract infection
Pancreatitis
Pneumonia
Acute otitis media
Streptococcal pharyngitis
Meningitis
Others
Lactose intolerance
Gluten intolerance
Postconcussive
328 Patient-Related Problems

Preadolescent Anatomic
and adolescent Malrotation
vomiting Incarcerated hernia
Adhesions
Intracranial
Brain tumor
Cerebrovascular accident
Subdural hemorrhage
Metabolic
Uremia
Toxic ingestion (including toxic effect of
therapeutic medication)
Diabetic ketoacidosis
Adrenal crisis
Infectious
Gastroenteritis (viral, parasitic)
Bacterial colitis
Hepatitis
Pancreatitis
Pneumonia
Urinary tract infection
Meningitis
Psychogenic
Bulimia
School avoidance
Anxiety
Others
Pregnancy
Gastroparesis
Peptic ulcer
Postconcussive
Cyclic vomiting

MAJOR THREAT TO LIFE

• Increased ICP
• Surgical abdominal emergencies (intussusception, bowel
obstruction, necrotizing enterocolitis)
• Diabetic ketoacidosis
• Adrenal crisis
The differential diagnosis of vomiting is best approached ini-
tially by the age of the child. In newborns and neonates, congenital
malformations of the gastrointestinal tract must be considered,
including duodenal or ileal atresia (associated with Down syn-
drome), pyloric stenosis, malrotation and midgut volvulus,
 Vomiting 329

tracheoesophageal fistula, annular pancreas, meconium ileus, and

Hirschsprung disease. In a preterm infant, especially one younger
than 32 weeks’ gestation and/or under 1500 g, vomiting may be a
sign of necrotizing enterocolitis, which can progress rapidly to a
perforated viscus, peritonitis, septic shock, and death. Nongas-
trointestinal diseases of the very young, including urinary tract
infection, inborn errors of metabolism, adrenal crisis, and
increased ICP (hydrocephalus or subdural hematoma), can pro-
duce significant vomiting.
In older infants, infectious gastroenteritis becomes more com-
mon, including gastroenteritis secondary to rotavirus and influenza
A. In toddlers and older children, toxic ingestion, bacterial food
poisoning, hepatitis, and inflammatory bowel diseases are added
to viral gastroenteritis. Nongastrointestinal disorders include uri-
nary tract infection, as well as brain tumors, other causes of
increased ICP, and postconcussive vomiting. Diabetic ketoacidosis
should always be considered in a child with vomiting even if no
history of diabetes mellitus, because this could be the first
presentation.

BEDSIDE
Quick-Look Test
Does the child appear well (comfortable), sick (uncomfortable or dis-
tressed), or critical (about to die)?
All children appear acutely uncomfortable when they are
actively vomiting. They are anxious, tachycardic, and frequently
diaphoretic. In infectious processes, nausea and vomiting tend to
come in waves, with periods of relative calm and comfort in
between, and almost invariably occur with fever. Acute surgical
vomiting is usually accompanied by abdominal pain, which may
or may not be well localized (see Chapter 6, Abdominal Pain).
Vomiting associated with intracranial processes often appears early
in the day and lessens as the day progresses, often with lack of
nausea.

Airway and Vital Signs

What are the temperature, pulse, and blood pressure?
Hypotension associated with vomiting is a late and ominous
sign of hypovolemia and shock. Fever implies infectious or inflam-
matory processes and can worsen dehydration. Hypertension and
bradycardia imply severe increased ICP. Remember the Cushing
triad: irregular respirations, elevated systolic blood pressure with
large pulse pressure, and bradycardia!
330 Patient-Related Problems

Selective History and Chart Review

Is abdominal pain associated with the vomiting?
This is difficult to ascertain in infants but is quite helpful in
older children. Although pain can occur with infectious gastroen-
teritis, it is unusual.
When did the vomiting start?
Vomiting can result from some medications, such as chemo-
therapeutic agents, as well as overdoses of a variety of other med-
ications. Vomiting after closed head injury can indicate a
concussion or more serious complication, such as subarachnoid
or subdural hemorrhage. Persistent or recurrent vomiting can rep-
resent cyclic vomiting, bulimia, or metabolic-endocrine disorders.
Is the vomiting forceful or effortless?
All babies spit up. All babies have some gastroesophageal reflux.
Reflux is normal and is a medical problem only if (1) the volume of
reflux is such that the child does not gain weight or (2) the child
aspirates. Otherwise, all reflux does is create dirty laundry (the
child’s as well as the parents’). Reflux should be distinguished from
vomiting. Reflux is effortless regurgitation in small infants and can
occur immediately after feeding or 2 to 3 hours later. The child is
not distressed; in fact, the child may be quite happy and content.
Vomiting is forceful and uncomfortable. An infant with pyloric ste-
nosis is often described as having “explosive or projectile” vomiting
(“across the room”). Projectile vomiting may also be seen with
increased intracranial pressure and it is usually in the absence of
nausea or retching.
What is the nature of the emesis?
Bilious, brown, or feculent emesis is pathognomonic of bowel
obstruction, either paralytic or mechanical. Frank blood implies
upper gastrointestinal bleeding, especially a Mallory-Weiss tear,
variceal bleeding, vascular anomalies, vasculitis, esophagitis, or
gastric ulcer disease, except in a newborn, in whom it may reflect
swallowed maternal blood. Vomiting food after fasting is consistent
with gastric outlet obstruction and/or delayed gastric emptying.
Is there associated diarrhea?
Viral and/or bacterial enterocolitis is very common in children.
Viral gastroenteritis tends to be seasonal, with specific causes com-
mon to summer (enteroviruses) and winter (rotavirus, influenza).
Food poisoning with Staphylococcus or Salmonella can cause a par-
ticularly sudden onset of acute vomiting that tends to be followed
by diarrhea. The absence of diarrhea and fever should always lead
you to consider intracranial causes for the vomiting.
What medications is the child taking?
Emesis is a well-described side effect of certain cancer chemo-
therapeutic medications, including cyclophosphamide, doxorubicin,
 Vomiting 331

and vincristine. Vomiting is also well known with toxic levels of

other numerous drugs.
Selective Physical Examination
HEENT Mucous membranes, dilated pupils, ketotic
breath, nystagmus, and cranial nerve deficits
may imply a nongastrointestinal cause of
vomiting. In infants the fontanelles should be
checked
Neck Nuchal rigidity
Respiratory Left lower lobe pneumonia and/or empyema may
cause vomiting
Abdomen Quality and activity of bowel sounds, distention,
localized or diffuse tenderness, masses,
hepatosplenomegaly, costovertebral angle
tenderness, rebound tenderness, rigidity.
Olive-like mass may indicate pyloric stenosis.
Rectal Small rectum (Hirschsprung disease), bleeding
(occult blood testing positive)
Genitourinary Hernia, scrotal masses, scrotal pain
Neurologic Mental status, focal findings, visual fields,
funduscopic examination, Romberg sign
HEENT, Head, Eye, Ear, Nose, Throat.

Management
Management of vomiting depends on the underlying cause
(Fig. 31.1). In many cases the vomiting is mild, self-limited, and
not a sign of serious life-threatening problems, such as increased
ICP or a surgical abdomen. After you have excluded these possibil-
ities, you may not need to do any more initially than ensure that the
child remains hydrated and frequently reevaluate the child via
serial abdominal examinations. Remember that intra-abdominal
processes may begin with isolated vomiting and lead to additional
signs and symptoms over time. If there are signs of bowel obstruc-
tion, rapid gastrointestinal bleeding, or peritonitis, surgical consul-
tation will be necessary. If there are signs of increased ICP,
neuroimaging will be necessary, and transfer to the pediatric inten-
sive care unit with institution of measures to decrease ICP may be
required (see Chapter 7, Altered Mental Status).
Vomiting in a newborn should be distinguished from reflux.
Forceful or persistent vomiting deserves diagnostic evaluation
and may require intervention. Flat and upright abdominal films
may confirm the presence of intestinal obstruction with a double
bubble (duodenal atresia), air-fluid levels (small bowel obstruc-
tion), or megacolon (meconium ileus or Hirschsprung disease).
 332
Patient-Related Problems
hypoglycemia)

FIGURE 31.1 Flowchart for management of vomiting.

 Vomiting 333

Plain films may also confirm the presence of a radiopaque foreign

body. (Remember, many of the things that children swallow are not
radiopaque.) Ultrasound studies are useful in neonates with hypo-
chloremic, hypokalemic metabolic acidosis caused by hypertrophic
pyloric stenosis and in older children with suspected appendicitis.
Prompt surgical consultation should be obtained if there is a sug-
gestion of bowel obstruction. Bowel obstruction in older children
can result from an incarcerated hernia, volvulus, intussusception,
or adhesions from previous abdominal surgery.
Dehydration should be addressed as discussed in Chapter 15,
Diarrhea and Dehydration. Obviously, the usefulness of oral rehy-
dration may be limited by severe vomiting. For this reason, IV
access is very important and should be made a priority.
If the child has been vomiting excessively, keep in mind that
electrolyte abnormalities may be developing, particularly hypoka-
lemia. Checking the electrolytes and correcting abnormalities may
be necessary (see Chapter 34, Electrolyte Abnormalities).
The use of antiemetic medications is controversial. When the
cause of vomiting is clear, such as after chemotherapy, antiemetic
medications may and should be used for symptomatic relief. How-
ever, in patients with vomiting of unknown cause, antiemetics must
be used cautiously. Vomiting may become persistent in children
with hepatitis, pancreatitis, and gastroenteritis, especially when
there is an element of dehydration. The use of promethazine (Phe-
nergan), chlorpromazine (Thorazine), prochlorperazine (Compa-
zine), or trimethobenzamide (Tigan) may be accompanied by
significant extrapyramidal side effects. Ondansetron and granise-
tron, serotonin antagonists, are effective treatment of a variety of
causes of refractory vomiting, including the vomiting associated
with chemotherapy.

REMEMBER
The most critical elements when evaluating a hospitalized child
with vomiting are to rule out the surgical causes unique to each
age group, consider the possibility of intracranial causes of the
vomiting, and support the child’s hydration status and electrolyte
balance while keeping the child comfortable. Oral rehydration can
be accomplished in most cases by giving small amounts frequently,
such as ice chips. Consider the need for antiemetics very cautiously,
especially in children with infectious causes of vomiting. Do not
forget that all forms of infectious vomiting are highly contagious.
Do yourself and your next patient a big favor and wash your hands
very well before and after evaluating every patient. Consider plac-
ing patient on contact isolation.
 CHAPTER

32
Acidosis and Alkalosis
Corinne Swearingen, MD

Multiple clinical conditions can affect a child’s acid-base status.

For example, profuse diarrhea may result in a metabolic acidosis
because of loss of bicarbonate-rich intestinal fluid in stool. Likewise,
vomiting may lead to a metabolic alkalosis from loss of hydrogen
ions in gastric fluid. Chronic derangements of a child’s acid-base
status can interfere with normal growth and development, whereas
acute derangements can be potentially fatal. Therefore it is critical
for the body to be able to regulate its acid-base status within a
certain pH. This is made possible through interactions between
the lungs, kidneys, and intracellular and extracellular buffers.

NORMAL ACID-BASE BALANCE

A normal pH is between 7.35 and 7.45. In a healthy individual, nor-
mal metabolism produces acids, which must then be buffered. The
main buffering system of the body is the bicarbonate buffer system;
other effective buffers include proteins (e.g., albumin, hemoglo-
bin), phosphate, and bone.
The bicarbonate buffer system is based on the relationship
between CO2 and bicarbonate:
H + + HCO

3 $ H2 CO3 $ CO2 + H2 O

Acids react with extracellular HCO
3 , which is then converted

to CO2 and eliminated through the lungs. PCO2 is sensed centrally,
and ventilation is adjusted (e.g., increased or decreased) to main-
tain normal values. These mechanisms maintain the concentration
of hydrogen ions ([H+]) within a narrow range, thereby maintain-
ing the arterial pH within a “normal” range.
The Henderson-Hasselbalch equation expresses the relation-
ship between pH, pK, and the concentrations of an acid and its

336
 Acidosis and Alkalosis 337

conjugate base. From the Henderson-Hasselbalch  equation,

 the
following relationship among [H+], PCO2, and HCO
3 can be
derived:
 

pH ¼ pKa + log HCO
3 =½CO2 
 
½H +  ¼ 24  PCO2 = HCO
3

This equation shows that the hydrogen ion concentration,

 and

therefore the pH, is determined by the ratio of PCO2 and HCO
3 .
The kidneys are responsible for regulating the serum bicarbon-
ate concentration; this is accomplished by renal reabsorption of
filtered bicarbonate and tubular secretion of hydrogen ions. Bicar-
bonate is mainly reabsorbed in the proximal tubule, whereas the
collecting duct is the main location for hydrogen ion secretion.
Bicarbonate reabsorption is increased in the setting of volume
depletion (“contraction alkalosis”), hypokalemia, and high PCO2;
it is decreased in the setting of low PCO2, elevated parathyroid hor-
mone (PTH), certain medications (e.g., acetazolamide), and prox-
imal tubular dysfunction (e.g., renal tubular acidosis [RTA]).
Acid excretion occurs through production of acid (H2PO
4,
NH+4 ) in the urine, which allows for net reabsorption of newly syn-
thesized HCO
3 . Acid excretion is regulated mainly by extracellular
pH. Low pH causes the secretion of aldosterone, which stimulates
acid excretion in the collecting duct.

CLINICAL ASSESSMENT OF ACID-BASE

DISORDERS
Acidemia is defined as an arterial pH < 7.35, whereas alkalemia
is an arterial pH > 7.45. Acidosis and alkalosis refer to the path-
ologic processes that cause an increase or decrease in [H+],
respectively. For example, a child may have a mild metabolic aci-
dosis with a concurrent severe respiratory alkalosis, resulting in
net alkalemia.
A simple acid-base disorder refers to a single primary distur-
bance. The simple acid-base disorders are: (1) metabolic acidosis,
(2) metabolic alkalosis, (3) respiratory acidosis, and (4) respiratory
alkalosis (Table 32.1). With
 a metabolic
 acidosis, the primary dis-
turbance is a low serum HCO
3 , whereas with a metabolic
 alka-
losis, the primary disturbance is a high serum HCO
3 . With a
respiratory acidosis, the primary disturbance is a high PCO2, and
with a respiratory alkalosis, the primary disturbance is a low PCO2.
At the onset of a simple acid-base disorder, the body begins to
predictably compensate (see Table 32.1).
 338
Laboratory-Related Problems
TABLE 32.1 Simple Acid-Base Disorders and Expected Compensation

Simple Acid-Base Primary

Disorder pH Disturbance Compensation Predicted Compensatory Response
   
Metabolic acidosis <7.35 # HCO
# PCO2 ("RR) PCO2 ¼ 1.5  HCO
3 +82
 3   
Metabolic alkalosis >7.45 " HCO
3 " P CO2 (#RR)
 P CO2 increases
 by 0.7 mm Hg for every 1 mEq/L increase in HCO

3
Respiratory acidosis <7.35 " PCO2 " HCO


3  HCO3
 increases by 0.1 mEq/L for each 1 mm Hg increase in PCO2
Acute <7.35 " PCO2 " HCO
3 HCO3 increases by 0.35 mEq/L for each 1 mm Hg increase in PCO2
Chronic (>24 hr)    
Respiratory alkalosis >7.45 # PCO2 # HCO


3 

HCO3
 falls by 0.2 mEq/L for each 1 mm Hg decrease in PCO2
Acute >7.45 # PCO2 # HCO3 HCO3 falls by 0.4 mEq/L for each 1 mm Hg decrease in PCO2
Chronic
RR, Respiratory rate.
 Acidosis and Alkalosis 339

Respiratory compensation is rapid and accomplished by

increasing PCO2 (e.g., alkalemia) or decreasing PCO2 (e.g., acidemia)
by changes in ventilation. Respiratory compensation cannot “over-
compensate” for or normalize the pH. Expected metabolic com-
pensation is highly dependent on whether the respiratory
process is acute or chronic. It is accomplished through regulation
of bicarbonate reabsorption and hydrogen ion excretion. In the
acute setting, metabolic compensation can occur within minutes.
In the chronic (>24 hours) setting, metabolic compensation at
the level of the kidney is much slower, beginning 12 to 24 hours
after the onset and continuing for several days.
A mixed acid-base disorder occurs when there is more than 1 pri-
mary acid-base disturbance. For example, a child with severe pneu-
monia and sepsis may have a respiratory acidosis due to respiratory
failure as well as a concurrent metabolic acidosis from elevated lactic
acid. A mixed disorder is suspected when compensation appears
to be greater or less than what is expected or deemed appropriate.
Any acid-base disturbance should be evaluated as a three-step
process: (1) determine if acidemia or alkalemia is present; (2) deter-
mine a cause of the acidemia or alkalemia; (3) determine whether a
mixed disorder is present. This evaluation should be interpreted in
the context of the clinical situation (e.g., reported symptoms; acute
vs. chronic course). Of note, for step 1, there are two instances in
which pH may be normal but an underlying acid-base disturbance
is present—a mixed disorder whereby the two processes have
opposite effects, and simple chronic respiratory alkalosis with
appropriate metabolic compensation.

PRIMARY ACID-BASE DISORDERS

Metabolic Acidosis
 
The primary disturbance in metabolic acidosis is a low HCO
3
secondary to depletion as a buffer or gastrointestinal/renal losses.
The most common cause of metabolic acidosis is diarrhea. The pre-
dictable compensatory response to metabolic acidosis is hyperventi-
lation, leading to a decrease in PCO2. A mixed acid-base disturbance is
present if the respiratory compensation is not appropriate based on
the Winter formula (see Table 32.1). If the PCO2 is greater than pre-
dicted, a concurrent respiratory acidosis is suspected. If the PCO2 is less
than expected, a concurrent respiratory alkalosis is suspected.
After a metabolic acidosis has been identified, the anion gap
should be determined.
 

Anion gap ¼ ½Na + 
½Cl
 + HCO
3
Normal range ¼ 8 to 16 mEq=L
340 Laboratory-Related Problems

The anion gap is the difference between the unmeasured cations

(potassium, magnesium, calcium) and unmeasured anions (albumin,
phosphate, urate, sulfate). As bicarbonate is depleted, the concentra-
tion of other anions is increased to maintain electroneutrality. The
added anion can be Cl
or an unmeasured anion (phosphate, lactate,
formate, β-hydroxybutyrate). A normal anion gap occurs if the con-
centration of Cl
is increased; in contrast, an increased anion gap
occurs when there is an increase in the unmeasured anions.
Causes
The causes of metabolic acidosis can be divided into those that pro-
duce a normal anion gap and those that produce an increased
anion gap (Table 32.2).  
If the anion gap is normal, either loss of HCO
3 has occurred
through the gut or kidneys or there has been rapid dilution of extra-
cellular volume. If the anion gap is increased, acids have been
added either endogenously (e.g., lactic acidosis or diabetic ketoaci-
dosis) or exogenously (e.g., ingestion). If the osmolal gap is
increased in the setting of anion gap metabolic acidosis, ingestion
should be suspected.

TABLE 32.2 Causes of Metabolic Acidosis

Normal Anion Gap Increased Anion Gap

(Hyperchloremic) (Normochloremic)
• Diarrhea • Poisoning: ethylene glycol, methanol,
• Bowel, biliary, or pancreatic salicylate, toluene, paraldehyde
tube or fistula drainage • Kidney failure (uremia)
• Renal tubular acidosis: distal • Ketoacidosis: diabetic ketoacidosis,
(type I), proximal (type II), starvation ketoacidosis, alcoholic
hyperkalemic (type IV) ketoacidosis
• Urinary tract diversions • Lactic acidosis: shock, hypoxemia,
(through intestinal segments) severe anemia, acute heart failure
• Posthypocapnia • Liver failure
• Ammonium chloride intake • Inborn errors of metabolism (through
• Adrenal insufficiency excessive production of ketoacids,
lactic acid, and/or other organic
anions)
• Malignancy
• Intestinal bacterial overgrowth
• Medications: nucleoside reverse
transcriptase inhibitors, metformin,
propofol
 
Normal serum HCO
3 is 24 mEq/L (range, 20-28 mEq/L) and arterial blood gas PCO2 is 40 mm
Hg (range, 35-45 mm Hg).
From Kliegman R, et al.: Nelson Textbook of Pediatrics, ed 20. Philadelphia, Elsevier, 2016.
 Acidosis and Alkalosis 341

Osmolal gap ¼ Measured serum osmolality

Calculated serum osmolality
Calculated serum osmolality ¼ 2½Na +  + ðSerum BUN=2:8Þ
+ ðSerum glucose=18Þ

Manifestations
The clinical manifestations of metabolic acidosis are related to the
degree of acidemia and underlying disorder. Hyperventilation may
be present as the child attempts to eliminate CO2; it is typically sub-
tle, with worsening respiratory distress potentially indicative of
worsening acidemia. Ketotic breath or other odors may be clues
to a metabolic cause of the acidosis, such as diabetic ketoacidosis
or an inborn error of metabolism. If the acidosis is severe, altered
mental status, decreased cardiac contractility, and shock may
all occur.
Management
As with other acid-base disorders, treating the underlying cause is
essential. For most children, maintaining adequate hydration,
maximizing cardiac output and perfusion, correcting other electro-
lyte abnormalities, removing potential toxins, and providing sup-
portive care prevent progression of the acidemia. Many causes of
metabolic acidosis do require specific therapy (e.g., administration
of insulin in diabetic ketoacidosis, administration of glucocorticoid
and mineralocorticoid in adrenal insufficiency, hemodialysis for
renal failure).
Depending upon the cause and chronicity, oral or intravenous
bicarbonate therapy may be indicated (e.g., RTA, chronic renal fail-
ure, salicylate poisoning). There are inherent risks to bicarbonate
administration, including hypernatremia, volume overload, and
subsequent alkalemia with impaired cell function. If not directly
indicated as treatment for a specific cause, bicarbonate administra-
tion is typically reserved for severe acute lactic acidosis and severe
diabetic ketoacidosis. In these cases, it is not necessary to return the
pH to normal; the goal should be to attain a pH of approximately
7.25. When a rapid response is needed, intravenous sodium bicar-
bonate may be given.
When administering bicarbonate, careful attention must be
paid to the serum potassium concentration. If an acidemic child
is normokalemic or hypokalemic, life-threatening hypokalemia
may be precipitated by the administration of bicarbonate due to
shift of potassium intracellularly. In this situation, bicarbonate
should be diluted and administered slowly; rapid infusion may
result in dysrhythmias.
342 Laboratory-Related Problems

Metabolic Alkalosis
 
The primary disturbance in metabolic alkalosis is a high HCO
3 .
In children the most common causes are vomiting and diuretic use.
Generation and maintenance of a metabolic alkalosis occurs in the
setting of two processes: (1) addition of base to the body and
(2) impairment in the kidney’s ability to excrete the base. The pre-
dictable compensatory response to a metabolic alkalosis is hypo-
ventilation, leading to an increase in PCO2. A mixed acid-base
disturbance is present if the respiratory compensation is not appro-
priate (see Table 32.1). If the PCO2 is greater than predicted, a con-
current respiratory acidosis is suspected. If the PCO2 is less than
expected, a concurrent respiratory alkalosis is suspected. In general,
appropriate respiratory compensation never exceeds a PCO2 of 55
to 60 mm Hg.
Causes
The causes of metabolic alkalosis are divided into two groups:
(1) chloride responsive and (2) chloride resistant (Table 32.3).
The division is based on urinary chloride level. Alkalosis in chil-
dren with low urinary chloride level is due to volume depletion
(with losses of sodium, potassium, and chloride). These children
require chloride and fluid repletion to correct their volume deple-
tion and metabolic alkalosis; in other words, they are “chloride

TABLE 32.3 Causes of Metabolic Alkalosis

Chloride-Responsive
(Urinary Chloride Chloride-Resistant
<15 mEq/L) (Urinary Chloride >20 mEq/L)
• Gastric losses (emesis, High blood pressure
nasogastric suction) • Hyperaldosteronism (adrenal adenoma or
• Diuretics (loop or hyperplasia)
thiazide) • Elevated renin (renovascular disease,
• Chloride-losing diarrhea renin-secreting tumor)
• Chloride-deficient • 17β-Hydroxylase deficiency
formula • 11β-Hydroxylase deficiency
• Cystic fibrosis • Cushing syndrome
• Posthypercapnia • Licorice ingestion
• Liddle syndrome
Normal blood pressure
• Gitelman syndrome
• Bartter syndrome
• Autosomal dominant hypoparathyroidism
• Base administration (citrate, lactate,
acetate)
 Acidosis and Alkalosis 343

responsive.” In comparison, alkalosis in a child with an elevated

urinary chloride concentration does not respond to volume reple-
tion and is therefore termed “chloride resistant.”
The main causes of chloride-responsive metabolic alkalosis
include vomiting and diuretic use. Vomiting causes alkalemia
due to loss of H+ in gastric fluid. With net loss of acid, there is
net gain of bicarbonate. In the setting of persistent vomiting, vol-
ume depletion also occurs, worsening the alkalemia due to
increased reabsorption of bicarbonate and hydrogen ion secretion.
Diuretics lead to alkalemia through volume depletion, which
increases angiotensin II, aldosterone, and adrenergic stimulation
of the kidney; contraction alkalosis also occurs, limiting urinary
bicarbonate losses. Typically, urinary chloride levels are high
(>20 mEq/L) immediately following diuretic administration but
later become low with appropriate renal chloride retention in a
volume-depleted state.
The chloride-resistant causes are divided based on blood pres-
sure. Hypertensive causes are related to elevated aldosterone, which
causes retention of sodium and renal excretion of hydrogen and
potassium.
Manifestations
The symptoms are typically related to the underlying disease pro-
cess, associated electrolyte disturbances, and fluid status. In gen-
eral, alkalemia increases the risk of arrhythmias and can cause
hypoxia and altered mental status.

Management
Metabolic alkalosis associated with volume depletion (low urinary
chloride) responds to the administration of normal saline solution.
Once the fluid and chloride deficits are repleted, renal excretion of
bicarbonate allows for resolution of the alkalemia. Remember that
an associated hypokalemia must also be corrected before normal
saline solution is effective. As with other acid-base disorders, cor-
recting the underlying cause is usually sufficient.
Respiratory Acidosis
The primary disturbance in respiratory acidosis is high PCO2 due
to impaired removal by the lungs. It is often due to severe pulmo-
nary disease, respiratory muscle fatigue, or central nervous system
(CNS) depression. The predictable compensatory response
depends on if the process is acute or chronic (see Table 32.1). In
acute respiratory acidosis, metabolic compensation can occur
within minutes due to cellular buffering mechanisms. With chronic
respiratory acidosis (>24 hours), more significant renal compensa-
 
tion is needed and occurs over 3 to 4 days. However, HCO
3
344 Laboratory-Related Problems

usually does not rise above 38 mEq/L. A mixed acid-base distur-

bance is present
 if the metabolic compensation is not appropriate.
If the HCO
3 is greater than
 predicted,
 a concurrent metabolic
alkalosis is suspected. If the HCO
3 is less than expected, a con-
current metabolic acidosis is suspected.
Causes
1. Pulmonary disease: pneumonia, pneumothorax, asthma, bron-
chiolitis, bronchopulmonary dysplasia, acute respiratory dis-
tress syndrome, cystic fibrosis, pulmonary edema, pulmonary
hemorrhage, hypoplastic lungs
2. CNS depression: encephalitis, hypoxic brain damage, head
trauma, brain tumor, primary pulmonary hypoventilation
(Ondine curse), medications (e.g., narcotics, barbiturates, anes-
thesia, benzodiazepines)
3. Respiratory muscle weakness: muscular dystrophy, mal-
nutrition, hypothyroidism, medications (succinylcholine,
corticosteroids)
4. Disorders of spinal cord, peripheral nerves, or neuromuscular
junction: diaphragmatic paralysis, Guillain-Barre syndrome,
poliomyelitis, spinal muscular atrophies, myasthenia gravis,
botulism, multiple sclerosis, spinal cord injury, medications
(e.g., vecuronium, aminoglycosides, organophosphates)
5. Upper airway disease: aspiration, laryngospasm, extrinsic
tumor, obstructive sleep apnea, angioedema
Manifestations
The clinical manifestations of respiratory acidosis depend on the
severity, duration, underlying disease process, and presence or
absence of hypoxemia. A rapid increase in PCO2 can lead to anxiety,
confusion, and hallucinations and may ultimately end in coma. In
chronic hypercapnia, sleep disturbances, loss of memory, and day-
time somnolence are possible; in addition, coordination and motor
disturbances (e.g., tremor, asterixis, myoclonic jerks) may be pre-
sent. Most importantly, high PCO2 can lead to CNS symptoms, such
as headache, papilledema, abnormal reflexes, and focal muscle
weakness, due to cerebral vasodilation.

Management
The treatment of respiratory acidosis depends on its severity and
rate of onset (acute vs. chronic). In general, management is directed
at the underlying cause. Intubation, mechanical ventilation, and
transfer to the intensive care unit may all be necessary for a child
with moderate to severe respiratory acidosis. Any child with respi-
ratory acidosis requires frequent monitoring with serial arterial
blood gas determinations while you are identifying and treating
 Acidosis and Alkalosis 345

the underlying cause. Tachypnea, labored breathing, and the use of

accessory muscles may initially prevent the development of acido-
sis, but eventual fatigue may lead to rapid decompensation.
Monitoring oxygen saturation alone with a pulse oximeter is
inadequate and may be falsely reassuring because preservation of
oxygen saturation may continue despite a decline in ventilation.
In addition, patients with chronic respiratory acidosis may actually
experience progression of their respiratory acidosis when given
oxygen, because ventilation in them is driven more by oxygen pres-
sure versus the normal parameters of PCO2 and pH. In general, cor-
rection of chronic respiratory acidosis should be performed
gradually, with the aim of restoring PCO2 to baseline levels.

Respiratory Alkalosis
The primary disturbance in respiratory alkalosis is low PCO2,
which is usually due to hyperventilation. Other causes include
extracorporeal membrane oxygenation (ECMO) or hemodialysis
where CO2 is lost directly into the circuit. The predictable compen-
satory response depends on if the process is acute or chronic (see
Table 32.1). In acute respiratory alkalosis, metabolic compensation
can occur within minutes due to cellular buffering mechanisms.
With chronic respiratory acidosis (>24 hours), more significant
renal compensation is needed and occurs over 2 to 3 days. Of note,
a chronic respiratory alkalosis is the only acid-base disturbance
where compensation can normalize the pH. A mixed acid-base dis-
turbance is present
 ifthe metabolic compensation is not appropri-
ate. If the HCO
3 is greater than predicted,
  a concurrent
metabolic alkalosis is suspected. If the HCO
3 is less than
expected, a concurrent metabolic acidosis is suspected.
Causes
1. Hypoxemia or tissue hypoxia: pneumonia, pulmonary edema,
congestive heart failure, asthma, severe anemia, aspiration, car-
bon monoxide poisoning, pulmonary embolism, interstitial
lung disease, ECMO
2. Lung receptor stimulation: pneumonia, pulmonary edema,
asthma, pulmonary embolism, respiratory distress syndrome,
pneumothorax
3. Central stimulation: fever, pain, panic attack, liver failure, sep-
sis, mechanical ventilation, CNS disease (e.g., subarachnoid
hemorrhage, encephalitis, trauma, brain tumor, stroke), medi-
cations (e.g., salicylate intoxication, theophylline, exogenous
catecholamines, caffeine)
Many different stimuli can increase ventilatory drive, resulting
in a respiratory alkalosis. For example, arterial hypoxemia or tissue
hypoxia can stimulate peripheral chemoreceptors, which signal to
346 Laboratory-Related Problems

the central respiratory center to increase ventilation. The lung also

contains chemoreceptors and mechanoreceptors that respond to
irritants and stretching and, when activated, will also signal to
the respiratory center to increase ventilation. Finally, direct stimu-
lation of the central respiratory center can occur, such as in the set-
ting of CNS disease (e.g., meningitis, hemorrhage, trauma), or by
situations that cause pain, stress, or anxiety.
Manifestations
Acute respiratory alkalosis may cause chest tightness, palpitations,
light-headedness, circumoral numbness, or paresthesias; less com-
monly, it may cause tetany, seizures, muscle cramps, or syncope.
The paresthesias, tetany, and seizures may be related to decreased
serum ionized calcium due to increased binding of calcium to albu-
min. Light-headedness and syncope may be related to cerebral
hypoperfusion secondary to low PCO2. In contrast, chronic respira-
tory acidosis is usually asymptomatic because of metabolic
compensation.
Management
Most instances of respiratory alkalosis are mild and short-lived.
Management of respiratory alkalosis depends on relieving the
underlying cause. In cases of hyperventilation secondary to anxiety,
rebreathing into a paper bag may be beneficial.
 C HA P TE R

33
Anemia,
Thrombocytopenia,
and Coagulation
Abnormalities
Shela Sridhar, MD

Anemia is a common problem in the pediatric age group and is

often discovered during hospitalization for an acute illness. Many
systemic illnesses are accompanied by mild to moderate anemia
and do not require extensive evaluation or specific treatment.
Similarly, anemia may be expected to be present with many chronic
illnesses, and appropriate management of the underlying illness
generally prevents progressive worsening of the anemia. When
anemia is severe or a systemic illness has not been identified, fur-
ther evaluation of the anemia may be required to determine the
cause and to initiate appropriate treatment. Treatment of anemia
based solely on a specific hemoglobin or hematocrit (Hct) value
is not always necessary; however, when the anemia results in symp-
toms or signs of hemodynamic compromise, or when cardiac or
pulmonary disease is present, treating the anemia will likely be
beneficial.
Thrombocytopenia and abnormalities in coagulation are much
less common than anemia in the pediatric population. These
abnormalities require further evaluation and often specific treat-
ment. A low platelet count or abnormal clotting study result gen-
erally reflects significant illness; therefore, when notified of such
abnormalities, your response should be prompt.

347
348 Laboratory-Related Problems

ANEMIA
Causes
The potential causes of anemia can be narrowed by considering the
patient’s clinical signs and symptoms and by the initial laboratory
evaluation. An evaluation of anemia should include hemoglobin,
Hct, reticulocyte count, red blood cell (RBC) indices (mean corpus-
cular volume [MCV], mean corpuscular hemoglobin [MCH], mean
corpuscular hemoglobin concentration [MCHC]), and RBC mor-
phology. Further evaluation may be necessary, depending on the
results of these initial tests. It is important to remember that normal
values for some of these measurements vary with age (Table 33.1).
Anemias can be divided into two large categories by analyzing
the reticulocyte count:
1. Low reticulocyte count: Inability of bone marrow to produce
RBCs
2. High reticulocyte count: Retained ability of bone marrow to
produce RBCs
Low Reticulocyte Count: Inadequate production
This category can be further categorized based on the MCV.
Microcytic Anemias (Low Mean Corpuscular Volume)
1. Iron deficiency
2. Thalassemias
3. Lead toxicity
4. Sideroblastic anemia
5. Chronic disease (infection, inflammation, renal disease)

Normocytic Anemias (Normal Mean Corpuscular

Volume)
1. Transient erythroblastopenia of childhood
2. Aplastic anemia (congenital or acquired, e.g., drugs)
3. Pure RBC aplasia
4. Bone marrow suppression or replacement (leukemia, tumors,
storage diseases, infections, hemophagocytosis)
5. Chronic disease
Macrocytic Anemias (High Mean Corpuscular Volume)
1. Vitamin B12 deficiency
2. Folate deficiency
3. Aplastic anemia
4. Pure RBC aplasia (Diamond-Blackfan syndrome)
5. Hypothyroidism
 Anemia, Thrombocytopenia, and Coagulation Abnormalities
TABLE 33.1 Estimated Normal Mean Values and Lower Limits of Normal (95% Range) for Hemoglobin, Hematocrit, Mean
Corpuscular Volume, and Mean Corpuscular Hemoglobin

Hemoglobin (g/L) Packed Cell Volume (%) MCV (fL) MCH (pg)
Age (Years) Mean Lower Limit Mean Lower Limit Mean Lower Limit Mean Lower Limit
0.5-4 125 110 36 32 80 72 28 24
5-10 130 115 38 33 83 75 29 25
11-14, F 135 120 39 34 85 77 29 26
11-14, M 140 120 41 35 85 77 29 26
15-19, F 135 120 40 34 88 79 30 27
15-19, M 150 130 43 37 88 79 30 27
20-44, F 135 120 40 35 90 80 31 27
20-44, M 155 135 45 39 90 80 31 27
MCH, Mean corpuscular hemoglobin; MCV, mean corpuscular volume.

349
350 Laboratory-Related Problems

High Reticulocyte Count: Increased Loss or Red Blood

Cell Destruction
RBC Losses: Bleeding
1. Trauma
2. Gastrointestinal (GI) bleeding
3. Splenic sequestration
4. Pulmonary hemorrhage
5. Ruptured aneurysm
6. Ruptured ectopic pregnancy
7. Intraventricular hemorrhage (in premature infants)

RBC Destruction: Hemolysis

1. Hemoglobinopathies
Sickle cell disease
Hemoglobin SC
2. RBC membrane defects
Spherocytosis
Elliptocytosis
Paroxysmal nocturnal hemoglobinuria
3. Enzymopathies
Pyruvate kinase deficiency
Glucose-6-phosphate dehydrogenase
4. Extracellular defects
Isoimmune hemolysis (Coombs positive)
Fragmentation (disseminated intravascular coagulopathy
[DIC], hemolytic-uremic syndrome [HUS], thrombotic
thrombocytopenic purpura [TTP])
Splenomegaly
Keep in mind that in children with chronic anemia, exacerba-
tions may develop as a result of another cause (e.g., splenic
sequestration or aplastic crisis in those with sickle cell
disease) and that a single cause may lead to other causes
(e.g., chronic GI blood loss leading to iron deficiency).

CLINICAL MANIFESTATIONS
The manifestations of anemia are those of the underlying cause.
Pallor is often the initial clue that an anemia is present. The palpe-
bral conjunctivae, mucous membranes, and nail beds are the most
obvious sites to look for pallor. Specific manifestations of the ane-
mia depend on whether the anemia is acute or chronic. Acute and
rapid onset of anemia secondary to hemorrhage results in signs and
symptoms of hypovolemia and/or shock:
1. Tachycardia, hypotension
2. Cool, clammy extremities
 Anemia, Thrombocytopenia, and Coagulation Abnormalities 351

3. Delayed capillary refill

4. Diaphoresis, tachypnea
Acute hemolysis may result in tachycardia, tachypnea, and
pallor but does not usually produce the same degree of hypovole-
mia that is seen with hemorrhage. Jaundice secondary to hyperbi-
lirubinemia, dark urine (hemoglobinuria), and/or splenomegaly
may be clues to hemolysis.
Chronic anemia results in less obvious signs and symptoms:
1. Pallor
2. Fatigue, lethargy
3. Dyspnea with exertion
4. Mild tachycardia, tachypnea

Management
Determine the severity. The clinical status of the patient, not the
laboratory value, should be used to determine the need for treat-
ment. For example, acute hemorrhage may initially result in a mild
decrease or even no decrease in the hemoglobin level. If the patient
is dehydrated, hemoconcentration may falsely increase the hemo-
globin and Hct despite a significant reduction in RBCs. Conversely,
many patients are asymptomatic despite profound anemia, espe-
cially if the anemia has developed over time.
A patient who is hypovolemic or in shock due to severe anemia
needs prompt intervention with a transfusion of packed RBCs
(PRBCs) or whole blood. A patient with normal vital signs and a
physical examination that does not indicate hypovolemia may need
intervention if (1) the hemoglobin level is very depressed and a
further drop is anticipated or (2) the hemoglobin level is very
depressed and other factors (heart or lung disease) mandate inter-
vention to improve oxygen-carrying capacity. A child with no
symptoms and mild to moderate anemia may need further evalu-
ation but probably does not need immediate intervention.
Hypovolemia
The same principles that apply in other conditions of hypovolemia
and shock apply in a patient with hypovolemia and anemia (see
Chapter 15, Diarrhea and Dehydration, and Chapter 25, Hypoten-
sion and Shock). Rapid expansion of the intravascular space is the
initial goal.
1. Make sure that the child has at least one (ideally two) large-bore
intravenous (IV) lines placed. “Large bore” is defined as the
largest that you can place, usually a 16-gauge line in an older
child and an 18-gauge line in a younger child or infant.
2. Send blood to the blood bank for an immediate cross match.
Always err on the side of requesting more units. The blood is
not wasted if you decide later not to use it.
352 Laboratory-Related Problems

3. Expand the intravascular space. The ideal fluid in this situation,

when acute anemia and hypovolemia coincide, presumably
because of massive blood loss (hemorrhage or hemolysis), is
cross-matched whole blood. If the situation is critical (ongoing
rapid blood loss in a patient in shock), O-negative blood should
be given. If blood is not yet available, normal saline or lactated
Ringer’s solution should be infused, starting at 20 mL/kg and
additional boluses administered according to the blood pressure
response, heart rate, and clinical signs (pulse, capillary refill). Once
blood is available, it should be used in place of crystalloid. After
volume has been restored and the child is normotensive, the
hemoglobin level and Hct should be determined, with additional
infusion of blood or PRBCs dependent on the degree of anemia.
4. Determine the cause of the blood loss. This should be done coin-
cidentally with fluid resuscitation. Search for obvious sites of hem-
orrhage, as well as occult sources. Periumbilical (Cullen sign) or
flank (Grey Turner sign) ecchymoses may indicate abdominal
hemorrhage. A careful abdominal and rectal examination with
Hemoccult determination is mandatory. A chest radiograph
should be obtained if pulmonary hemorrhage is a consideration.
Review the child’s medication list for anticoagulants and the chart
for potential coagulopathies or recent surgery.
5. Surgical consultation may be necessary if intra-abdominal
bleeding is suspected.

Euvolemia
A euvolemic child does not require immediate intervention but
may need a transfusion if symptomatic or the degree of anemia
is profound.
1. Determine the cause of the anemia. As noted earlier, a complete
blood count with RBC indices, reticulocyte count, and review of
the peripheral smear (Fig. 33.1) should allow you to narrow the
possibilities or make a definitive diagnosis. Remember that even
though hypovolemia is not present, blood loss may have
occurred or may be ongoing and the potential exists for rapid
decompensation if the patient is hemorrhaging. As in hypovo-
lemic patients, your physical examination should be directed
toward signs of obvious or occult bleeding.
2. If a transfusion is necessary, the amount of cross-matched
PRBCs to be transfused can be calculated by using the following
formula:
Volume of PRBCsðmLÞ ¼ Estimated blood volumeðmLÞ
 ðDesired Hct  Observed HctÞ
=Hct of PRBCs
 Anemia, Thrombocytopenia, and Coagulation Abnormalities 353

FIGURE 33.1 Examples of blood smears demonstrating helpful

diagnostic features associated with specific anemias. RBC, Red
blood cell. (From Marshall SA, Ruedy J: On Call: Principles and
Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 325.)

where the Hct of PRBCs can be estimated to be 65 and blood

volume is estimated as follows:
Premature infants 100 mL/kg
Neonates 85 mL/kg
Infants (>1 month) 75 mL/kg
Children 70 mL/kg
Adolescents 65 mL/kg
Alternatively, a transfusion of 10 to 15 mL/kg of PRBCs can be
administered. With either approach, rechecking the hemoglobin
concentration and Hct in 4 to 6 hours allows you to determine
the adequacy of the replacement.

THROMBOCYTOPENIA
Causes
The cause of a low platelet count can be divided into two categories:
1. Inadequate production (bone marrow infiltration or
suppression)
2. Increased destruction or sequestration
354 Laboratory-Related Problems

Inadequate Malignancies (e.g., leukemia, neuroblastoma)

production Storage diseases (e.g., Gaucher disease)
Wiskott-Aldrich syndrome
Thrombocytopenia absent radius syndrome
Infections
Drugs (resulting in marrow suppression)
Hemophagocytic syndromes
(hemophagocytic lymphohistiocytosis
[HLH], macrophage activation syndrome
[MAS])
Increased Idiopathic thrombocytopenic purpura
destruction Systemic lupus erythematosus (SLE)

CLINICAL MANIFESTATIONS
Thrombocytopenia may be asymptomatic, result in petechiae only,
or be associated with significant bleeding. The underlying disorder,
rather than the specific platelet count, determines the severity of
the clinical manifestations. Hemorrhage is generally rare unless
the platelet count is less than 20,000/mm3.
Bleeding secondary to mild or moderate thrombocytopenia
tends to involve the skin and mucous membranes rather than dee-
per internal organs. Petechiae, purpura, bruising, and bleeding
from gums or IV sites are seen most often. This is in contrast to
bleeding that results from an abnormality in clotting factors, in
which deeper bleeding (e.g., hemarthroses) is more common. How-
ever, if thrombocytopenia is severe enough, children may be at
increased risk for intracranial or other internal bleeding. Purpura
secondary to thrombocytopenia is nonpalpable and should be dis-
tinguished from palpable purpura, which is more suggestive of
vasculitis.
Additional clinical findings and laboratory studies should help
you narrow the list of potential causes of a low platelet count. If
anemia and/or leukopenia is also present, marrow suppression
or SLE should be further considered. Isolated thrombocytopenia
is more suggestive of a problem causing increased destruction of
platelets (e.g., idiopathic thrombocytopenic purpura [ITP]). The
presence of an associated hemolytic anemia increases the likeli-
hood of an autoimmune (SLE, Evans syndrome) or microangio-
pathic (DIC, HUS, TTP) cause. Reviewing the peripheral smear
may suggest a microangiopathy if schistocytes, helmet cells, and
other fragmented cells are evident. In such a context, an elevated
 Anemia, Thrombocytopenia, and Coagulation Abnormalities 355

blood urea nitrogen and/or creatinine level with other signs of renal
failure should increase your suspicion for HUS; the additional
presence of neurologic symptoms should alert you to the possibility
of the much less common diagnosis of TTP. An associated prolon-
gation of the prothrombin time (PT) and partial thromboplastin
time (PTT) should raise your suspicion for DIC.

Management
Treatment of thrombocytopenia varies, depending on the underly-
ing cause. In general, platelet transfusions are only beneficial in
conditions in which thrombocytopenia is a result of inadequate
production. In conditions involving increased destruction, platelet
transfusions may temporarily increase the platelet count, but as
long as the pathogenic mechanisms leading to increased destruc-
tion continue, the platelet count eventually falls again. Nonetheless,
if life-threatening hemorrhage is present, platelets should be trans-
fused in these situations, even if it is only a temporary measure.
If a platelet transfusion is necessary, it is customary to transfuse
1 unit of platelets per 10 kg of patient weight. This can be expected
to increase the platelet count by approximately 50,000/mm3.
An estimate of the expected increase can also be obtained with
the following formula:


Platelet count increase per mm3
¼ 30, 000  Units transfused=Total blood volume ðLÞ
The platelet count should be checked 1 hour after transfusion to
evaluate the response.

Idiopathic Thrombocytopenic Purpura

Treatment is not required when the patient is asymptomatic and
the platelet count is greater than 35,000 mm3. Treatment options
include IV immunoglobulin, anti-Rh D therapy (in patients who
are Rh D positive), and corticosteroids. Decisions regarding treat-
ment of ITP should be made in consultation with a pediatric
hematologist.

Thrombotic Thrombocytopenic Purpura

Children with TTP are usually quite ill and generally require man-
agement in the intensive care setting. Plasmapheresis is usually
indicated and other treatments including corticosteroids may be
considered. Treatment plans should be undertaken in consultation
with a pediatric hematologist.
356 Laboratory-Related Problems

FIGURE 33.2 The coagulation cascade. aPTT, Activated partial

thromboplastin time. (From Marshall SA, Ruedy J: On Call: Princi-
ples and Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 337.)

COAGULATION ABNORMALITIES: PROLONGED

PROTHROMBIN TIME AND PARTIAL
THROMBOPLASTIN TIME
Causes
The PT tests the extrinsic pathway of the clotting cascade
(Fig. 33.2). The PTT tests the intrinsic pathway. The extrinsic path-
way is most affected by deficiencies in factors I (fibrinogen), II
(prothrombin), V, VII, and X, whereas the intrinsic pathway is
most affected by deficiencies in factors VIII, IX, XI, and XII.
Disorders prolonging the Clotting factor deficiencies
prothrombin time (factors I, II, V, VII, X)
Oral anticoagulants (warfarin
sodium [Coumadin])
 Anemia, Thrombocytopenia, and Coagulation Abnormalities 357

Vitamin K deficiency (e.g.,

hemorrhagic disease of the
newborn)
Liver disease
Disseminated intravascular
coagulopathy
Heparin (sometimes)
Disorders prolonging the Clotting factor deficiencies
partial thromboplastin (factors VIII, IX, XI, XII)
time Anticoagulants (heparin;
sometimes oral anticoagulants)
Circulating endogenous
anticoagulant (e.g., lupus
anticoagulant)
Disseminated intravascular
coagulopathy
von Willebrand disease
(sometimes)
Factor deficiencies can be suspected or excluded by the results
of mixing studies, in which normal plasma is mixed 1:1 with the
child’s plasma. The PT or PTT should “correct” if a deficient factor
has been replaced by the addition of normal plasma. Failure to cor-
rect is consistent with an anticoagulant.

CLINICAL MANIFESTATIONS
With the exception of lupus anticoagulant, bleeding is the obvious
manifestation. Bleeding associated with a prolongation of the PT
and/or PTT tends to involve deeper parts of the body, including
visceral organs and joints.
Lupus anticoagulant is not associated with bleeding but rather
predisposes one to thrombosis. The prolongation of the PTT that is
usually (but not always) discovered is the result of in vitro phenom-
ena in which the presence of lupus anticoagulant interferes with the
test itself.
Management
Factor Deficiencies
Fresh frozen plasma (10 to 15 mL/kg) or specific factor concen-
trates can be infused to replace the deficient factor, once identified.
Cryoprecipitate (0.2 bag/kg) contains high concentrations of
fibrinogen and factor VIII and can also be used when these
factors are deficient. If the bleeding is minor, IV or intranasal
desmopressin (DDAVP) may be administered to increase plasma
factor VIII levels. In children with severe bleeding, the use of
358 Laboratory-Related Problems

products such as coagulation factor VIIa (recombinant) should be

considered. However, these products should not be administered
without consulting a hematologist.
von Willebrand Disease
In most cases, bleeding is minor and may be treated with DDAVP,
which releases von Willebrand factor (vWF). Less commonly, von
Willebrand disease causes major bleeding that may be treated with
Humate-P, a factor VIII concentrate that contains high levels of
vWF. Cryoprecipitate or fresh frozen plasma is also effective.
Use of these products should always be undertaken in consultation
with a pediatric hematologist.

Vitamin K Deficiency
Hemorrhagic disease of the newborn occurs during the first week
of life when an exaggeration of the normally mild decrease in vita-
min K–dependent factors occurs. Prophylactic administration of
intramuscular (IM) vitamin K at the time of birth usually prevents
hemorrhagic disease but is less effective in premature infants. If
bleeding ensues, SC or IM vitamin K, 1 to 2 mg, is effective, and
a response should be seen within a few hours.
Intestinal malabsorption and prolonged antibiotic treatment
may result in vitamin K deficiency beyond the neonatal period.
Vitamin K should be administered orally, subcutaneously, or intra-
venously (1 mg for infants, 2 to 3 mg for children, 10 mg for ado-
lescents and adults). If vitamin K is ineffective or rapid correction is
required, fresh frozen plasma should be administered.
Liver Disease
Liver disease may result in decreased synthesis of clotting factors
and subsequent factor deficiencies. For mild bleeding, administra-
tion of vitamin K, as outlined previously, may be all that is neces-
sary. If bleeding is severe, fresh frozen plasma (10 to 15 mL/kg)
corrects all clotting factor deficiencies except fibrinogen deficiency.
Cryoprecipitate (0.2 bag/kg) can be infused to correct the fibrino-
gen deficiency.
Disseminated Intravascular Coagulopathy
While the underlying cause is being treated, supportive care should
include infusions of fresh frozen plasma, cryoprecipitate, and plate-
lets when bleeding, thrombocytopenia, and PT and PTT abnormal-
ities are severe.
 C HA P TE R

34
Electrolyte
Abnormalities
Eric Velazquez, MD

HYPERNATREMIA (SERUM SODIUM >150 MEQ/L)

Causes
Sodium excess
Improperly mixed formula
Excessive sodium bicarbonate administration for acidosis
Ingestion of ocean water
Hyperaldosteronism
Water deficit
Inadequate intake
Renal losses
Diabetes insipidus (central or nephrogenic)
Diabetes mellitus
Osmotic diuresis
Obstructive uropathy
Renal dysplasia
Extrarenal losses
Diarrhea
Excessive sweating
Excessive insensible losses (burns, phototherapy)
Manifestations
Hypernatremia is usually the result of abnormal water loss in
excess of sodium loss rather than an increase in total body sodium.
In children, diarrhea is the most common cause and results in
dehydration. With hypernatremic dehydration, the shift of water
into the extracellular space tends to preserve intravascular volume;
therefore urine output may remain close to normal and such

359
360 Laboratory-Related Problems

children may initially be less symptomatic than those with other

forms of dehydration. Polyuria should suggest diabetes insipidus
or diabetes mellitus. In an infant who does not appear dehydrated
and has normal urine output, you should carefully determine how
the infant is being fed and how the formula has been mixed. Errors
in mixing are a common cause of hypernatremia in this age group.
The clinical manifestations of hypernatremia are those that
result from the osmotic shift of water from the intracellular com-
partment to the extracellular compartment. Pulmonary edema
may ensue, and its effects on brain cells may result in lethargy,
irritability, coma, seizures, hypertonicity, and muscle spasms.
Brain hemorrhage is the most serious potential consequence of
hypernatremia.
Management
Determine the hydration status of the child and the severity of the
hypernatremia. Most infants and children with hypernatremia are
dehydrated. Management of a dehydrated patient with hypernatre-
mia is discussed in Chapter 15.
If the patient is normovolemic (or volume overloaded) and hyper-
natremic, as in instances in which sodium intake has been excessive,
diuresis may be useful. Furosemide, 1 mg/kg intravenously (IV) ini-
tially with repeated doses at 2- to 4-hour intervals, promotes urinary
sodium loss. If a large volume must be diuresed to return the sodium
level to normal, urinary losses may be measured and replaced with 5%
dextrose in water (D5W). Frequent monitoring of the patient’s hydra-
tion status and serum sodium level is mandatory. Remember that
serum sodium levels must be corrected slowly (10 to 15 mEq/L per
day) because cerebral edema may develop if free water shifts rapidly
intracellularly as the extracellular sodium concentration falls.
Severe hypernatremia (>200 mEq/L) may require peritoneal
dialysis or hemodialysis and consultation with a pediatric nephrol-
ogist.

HYPONATREMIA (SERUM SODIUM <130 MEQ /L)

Causes
The volume status of the patient should guide your differential of
potential causes of hyponatremia. An estimate of volume status
and the urinary sodium concentration allows you to limit the list
of possibilities (Fig. 34.1). A hypovolemic patient is losing sodium
(and free water) either through urine or from extrarenal sites. The
urinary sodium concentration should allow you to distinguish
renal from extrarenal losses. A euvolemic patient may also have
urinary sodium losses, but they are lower than in a hypovolemic
 Electrolyte Abnormalities
361
FIGURE 34.1 Classification, diagnosis, and treatment of hyponatremic states. ADH, Antidiuretic hormone; IV, intravenous; RTA,
renal tubular acidosis; SIADH, syndrome of inappropriate antidiuretic hormone. (From Berry PL, Belsha CW: Hyponatremia.
Pediatr Clin North Am 37:354, 1990, with permission)
362 Laboratory-Related Problems

patient, and water loss is minimal or absent. In some cases (e.g.,

syndrome of inappropriate antidiuretic hormone [SIADH]), there
may be an increase in free water. The urine sodium level in those
with euvolemic hyponatremia is concentrated at greater than
20 mEq/L. A hypervolemic patient has edema from heart, liver,
or renal disease or overt renal failure. The urinary sodium concen-
tration is usually very high in those with renal failure.
Keep in mind that pseudohyponatremia or factitious hyponatre-
mia may also occur. Pseudohyponatremia is a laboratory measure-
ment error that occurs when excessive protein or lipid is present in
plasma. Hyperproteinemia or hyperlipidemia increases plasma vol-
ume by decreasing the percentage of plasma that is free water. Some
laboratory machines measure and report the sodium concentration
in terms of the volume of total plasma rather than plasma water, and
therefore the sodium concentration is artificially low. A clue to the
presence of pseudohyponatremia is normal plasma osmolality
despite a low serum sodium concentration. Factitious hyponatremia
is redistribution of water from the intracellular to the extracellular
compartment because of excessive extracellular osmolality. Hyper-
glycemia or the administration of mannitol increases plasma osmo-
lality, thereby resulting in a shift of free water and a fall in the sodium
concentration. In general,
Decrease in sodium ðmEq=LÞ ¼ 1:6 mEq=L∞Increase in blood
glucose=100 mg=dL

Manifestations
The clinical manifestations depend in part on the volume status of
the child and the underlying cause of the hyponatremia. When
hyponatremia develops rapidly (<24 hours) and when it is severe
(<120 mEq/L), the following may occur as a result of the intracel-
lular shift of water:
1. Altered mental status
2. Seizures
3. Nausea and vomiting
4. Muscle cramps and weakness
5. Coma

Management
Determine the hydration status of the child and the severity of the
hyponatremia.

Hypovolemic Patients
Most children with hyponatremia are dehydrated and hypovole-
mic, without symptoms such as altered mental status or seizures.
 Electrolyte Abnormalities 363

These children can be managed as outlined in the Diarrhea and

Dehydration chapter, with gradual replacement of the sodium
and water deficit over a 24-hour period.
If altered mental status or seizures are present, more rapid
correction of the decreased concentration may be necessary. In this sit-
uation, hypertonic saline solution (either 3% or 5%, containing 513
and 855 mEq/L of sodium, respectively) should be administered, with
the goal of raising the serum sodium concentration to 125 mEq/L. The
following formula can be used to calculate the number of
milliequivalents of sodium necessary to achieve this concentration:
Sodium ðmEqÞ required ¼ ð125  Current serum NaÞ
∞ 0:6∞ Weight ðkgÞ
The total required should be infused over a period of approx-
imately 4 hours or at a rate of 5 mEq/kg/hr. Once the serum
sodium concentration reaches approximately 125 mEq/L, further
corrections of the sodium deficit can proceed as discussed in
Chapter 15.

Euvolemic Patients
Euvolemic patients or those with slight increases in extracellular
volume usually require water restriction. SIADH production (most
often associated with meningitis) or water intoxication are the most
likely causes. Restriction to two-thirds of the maintenance fluid
requirement is the usual initial treatment; adjustments may be nec-
essary, with frequent monitoring of the serum sodium level. If the
child is symptomatic, with seizures or altered mental status, the
serum sodium concentration may be increased by combining
hypertonic saline solution and diuresis with IV furosemide. In this
way, excessive free water is diuresed because a more hypertonic
solution is being infused, thereby preventing a further increase
in extracellular volume. The same formula discussed for hypovole-
mic patients may be used to calculate the required amount of
sodium to be administered. Serum electrolytes (including potas-
sium) should be measured frequently during such treatment
because hypokalemia may ensue during diuresis.

Hypervolemic Patients
Salt and water restriction is required for those with edema-forming
states or renal failure. Diuresis with furosemide also helps to
decrease extracellular volume. As with hypovolemia and euvole-
mia, a child with severe, symptomatic hyponatremia may require
hypertonic saline solution, which should be combined with
diuretics. Dialysis may be necessary for those in renal failure.
364 Laboratory-Related Problems

HYPERKALEMIA (SERUM POTASSIUM

>5.5 MEQ/L)
Causes
Decreased excretion
Renal failure (acute or chronic)
Potassium-sparing diuretics (amiloride, triamterene, spironolactone)
Adrenal insufficiency
Distal tubular dysfunction (type IV renal tubular acidosis)
Impaired extrarenal regulation
Diabetes mellitus
Drugs (β-blockers, succinylcholine, angiotensin-converting
enzyme inhibitors)
Shift from intracellular to extracellular fluid
Acidosis
Tissue destruction (trauma, hemolysis, burns, tumor lysis,
rhabdomyolysis)
Hyperkalemic periodic paralysis
Increased intake
Potassium supplements (IV or orally)
Blood transfusions
Salt substitutes
Factitious
Difficulty drawing blood (hemolysis, as with a heel puncture)
Thrombocytosis
Manifestations
The effects of hyperkalemia on the cardiac conduction system are
the most significant and may be fatal. The progressive changes that
can be seen on an electrocardiogram (ECG) as the serum potas-
sium concentration rises are, in order,
1. Peaked T waves (serum potassium, 6 to 7 mEq/L)
2. Depressed ST segments
3. Decreased R wave amplitude
4. Prolonged PR interval (serum potassium, 7 to 8 mEq/L)
5. Small or absent P waves
6. Wide QRS complexes (serum potassium, 8 to 9 mEq/L)
7. Sine wave pattern
8. Asystole or dysrhythmias
In addition, hyperkalemia may cause paresthesias, muscle
weakness, and decreased tendon reflexes as a result of depolariza-
tion of muscle cells.

Management
Determine the severity of the hyperkalemia. All patients with
hyperkalemia should have an ECG performed immediately to
 Electrolyte Abnormalities 365

search for the signs just listed. Continuous ECG monitoring is also
necessary until the problem is corrected.

Severe Hyperkalemia
If the serum potassium level is greater than 8 mEq/L or ECG
changes other than peaked T waves are seen, you should proceed
as follows:
1. Notify your supervisor.
2. Remove any potassium from the IV fluids
3. Administer 10% calcium gluconate, 0.5 mL/kg IV over a 2- to
5-minute period. This does not change the serum potassium
level but protects the heart from the effects of hyperkalemia.
The onset of action is immediate, and effects last approximately
1 hour.
4. Administer sodium bicarbonate, 1 to 2 mEq/kg IV over a 3-
to 5-minute period. Make sure to flush the calcium gluconate
from the line before giving bicarbonate because the two may
be incompatible. The sodium bicarbonate shifts potassium
intracellularly; its effect is immediate and lasts for 1 to
2 hours.
5. Administer glucose, 0.5 g/kg, with 0.3 unit of insulin per gram
of glucose over a period of 2 hours.
6. Nebulized albuterol will move potassium intracellularly by
stimulating β1-adrenergic receptors.
7. Sodium polystyrene sulfonate (Kayexalate), 1 to 2 g/kg, with
3 mL of sorbitol per gram of resin divided every 6 hours orally
or 5 mL of sorbitol per gram of resin as an enema over a period
of 4 to 6 hours. This is the only drug treatment that removes
potassium from the body. It is estimated that 1 g/kg decreases
the serum potassium concentration by 1 mEq/L.
8. If these measures are unsuccessful, hemodialysis is necessary.
9. The serum potassium concentration should be determined
every hour until it is less than 6.5 mEq/L.
Moderate Hyperkalemia
If the serum potassium level is between 6.5 and 8 mEq/L and the
ECG reveals only peaked T waves, you should proceed as follows:
1. Notify your supervisor.
2. Remove any potassium from the IV fluids.
3. Administer sodium bicarbonate, glucose and insulin, and
Kayexalate in the doses outlined earlier.
4. Monitor the serum potassium level every hour until it is less
than 6.5 mEq/L.
Mild Hyperkalemia
If the serum potassium level is less than 6.5 mEq/L and the ECG is nor-
mal or has peaked T waves only, you should consider correcting other
366 Laboratory-Related Problems

contributing factors (e.g., acidosis), as well as administering Kayexalate

as previously outlined. Potassium should again be removed from
any IV fluids. If the cause is identified and is not progressive, the
serum potassium measurement can be repeated in 4 hours.

HYPOKALEMIA (SERUM POTASSIUM <3.5 MEQ/L)

Causes
Excessive renal losses
Diuretics
Antibiotics (penicillins, amphotericin, aminoglycosides)
Glucocorticoid excess
Renal tubular acidosis type I
Hyperaldosteronism
Vomiting, nasogastric suctioning leading to alkalosis
Extrarenal losses
Vomiting
Diarrhea
Laxative abuse
Shift from extracellular to intracellular space
Alkalosis
Insulin
β-Catecholamines
Lithium
Inadequate intake

Manifestations
As with hyperkalemia, the cardiac effects are the most significant
and include the following:
1. Premature atrial contractions
2. Premature ventricular contractions (PVCs)
3. Flattened T waves
4. Appearance of U waves
5. ST-segment depression
In addition, neuromuscular symptoms and signs may appear,
such as weakness, paresthesias, ileus, and depressed tendon reflexes.

Management
Determine the severity of the hypokalemia. All patients should
have an ECG performed and undergo continuous ECG monitoring
until the problem is corrected.
Severe Hypokalemia
If the serum potassium level is less than 2.5 mEq/L and there are
PVCs, U waves, or ST-segment changes, IV supplementation of
 Electrolyte Abnormalities 367

potassium should be considered. Potassium chloride, 0.5 to 1

mEq/kg, up to 10 mEq maximum, can be given IV over a 1-hour
period while the patient is continually monitored with cardiac
monitor. The serum potassium level should be measured again
in 1 hour. Further supplementation can proceed more slowly by
adding up to 40 mEq/L of potassium to an IV solution and infusing
at the standard maintenance rate.
Moderate or Mild Hypokalemia
In those with a serum potassium level greater than 2.5 mEq/L and
no ECG changes, hypokalemia may often respond to correction of
the underlying cause, with no need for supplementation. If supple-
mentation is necessary, oral supplements should be sufficient. The
serum potassium level should be measured again in 4 to 6 hours to
ensure that it does not continue to fall.

HYPERCALCEMIA
Causes
Increased intake
Vitamin D or A intoxication
Excessive calcium supplementation
Milk-alkali syndrome (antacid ingestion)
Increased production or mobilization from bone
Hyperparathyroidism (primary or tertiary)
Hyperthyroidism
Immobilization
Malignancies (bone metastases, tumor lysis syndrome)
Sarcoidosis
Decreased excretion
Thiazide diuretics
Familial hypocalciuric hypercalcemia
Miscellaneous
Williams syndrome
Pheochromocytoma
Adrenal insufficiency

Manifestations
“Stones, bones, groans, and psychic moans” refer to some of the man-
ifestations of hypercalcemia. Renal stones may develop, and polyuria
and polydipsia are also common because hypercalcemia reduces the
ability to concentrate urine. Bone pain (“bones”) and abdominal
symptoms (“groans”), such as pain, nausea, constipation, vomiting,
and pancreatitis, may also occur. “Psychic moans” may be manifested
as delirium, dementia, psychosis, lethargy, and even coma.
368 Laboratory-Related Problems

The ECG may reveal a short QT interval and a prolonged PR

interval. Dysrhythmias may develop if the hypercalcemia is severe
enough.
Management
Determine the severity of the hypercalcemia. Approximately half
the total serum calcium is bound to albumin, and the other half
is present in a free or ionized form. The clinical effect of hypercal-
cemia depends on the amount that is unbound or the ionized
calcium. Most laboratories routinely report total calcium, which
reflects both ionized calcium and calcium that is bound to albumin.
Thus, in hypoalbuminemic states, there may be an increase in ion-
ized calcium despite normal serum total calcium. A useful assump-
tion that can allow you to estimate the ionized calcium is that each
1-g/dL decrease in serum albumin decreases bound calcium (and
total calcium) by approximately 0.8 mg/dL.
Severe Hypercalcemia
A serum calcium level greater than 14 mg/dL or the presence of
symptoms requires immediate treatment. The serum calcium con-
centration can be reduced by rapid expansion of intravascular
volume. Infusing a bolus of 20 mL/kg of normal saline solution
results in a reduction in serum calcium concentration as a result
of hemodilution and the increase in urinary calcium excretion that
accompanies the excess sodium excreted in urine. Urinary calcium
excretion can also be promoted with IV furosemide, 1 mg/kg every
2 to 4 hours. While you are monitoring the child’s volume status
closely, the normal saline boluses and furosemide may be repeated
and should result in a fall in the serum calcium concentration. If it
does not begin to decrease soon after volume expansion and diure-
sis, hemodialysis should be considered. Hemodialysis should be
considered initially if the serum calcium concentration is greater
than 15 mg/dL or the child has severe symptoms (e.g., coma).
Mild or Moderate Hypercalcemia
If the serum calcium concentration is less than 14 mg/dL, you may
proceed at a less urgent pace. As with severe hypercalcemia, volume
expansion and diuresis help to increase urinary excretion of
calcium. Increasing the IV rate to slightly expand intravascular
volume after an initial 20-mL/kg bolus of normal saline solution
may be sufficient. Likewise, IV furosemide, 1 mg/kg every 3 to
4 hours, should result in a gradual fall in the serum calcium con-
centration. Prednisone, 1 mg/kg/day, may be helpful because it
decreases intestinal absorption of calcium by blocking the effect
of 1,25-dihydroxyvitamin D. The effect of prednisone should be
apparent within 2 to 3 days. Bisphosphonates and calcitonin are
 Electrolyte Abnormalities 369

additional potential therapies because they inhibit bone resorption,

but they should be used only after consultation with a pediatric
endocrinologist.

HYPOCALCEMIA
Causes
Decreased intake
Vitamin D deficiency (malabsorption, nutritional deficiency,
abnormal vitamin D metabolism, lack of sunlight)
Short bowel syndrome
Decreased production or mobilization
Hypoparathyroidism
Pseudohypoparathyroidism
Vitamin D deficiency
Hyperphosphatemia
Magnesium deficiency
Pancreatitis
Rhabdomyolysis
Alkalosis
Increased excretion
Chronic renal failure
Drugs (loop diuretics, aminoglycosides)
Exchange transfusion in neonates
Manifestations
Papilledema, abdominal pain, mental status changes, laryngo-
spasm (stridor), carpopedal spasm, seizures, and paresthesias may
all occur. The ECG may reveal a prolonged QT interval. Chvostek
sign and Trousseau sign may be present (Figs. 34.2 and 34.3).
Management
Determine the severity of the hypocalcemia by the presence and
type of symptoms. An asymptomatic patient does not require
urgent correction of the calcium concentration. IV calcium admin-
istration entails some special risks (see later); therefore it should be
reserved for those who cannot take oral calcium or whose symp-
toms demand immediate correction.
As with hypercalcemia, first correct for the serum albumin con-
centration. Hypoalbuminemia is common in hospitalized children,
and the ionized calcium concentration may be normal despite a sig-
nificant reduction in the total serum calcium concentration.
Remember also to check the serum phosphate concentration. If
it is markedly elevated, you need to consider correcting the phos-
phate concentration before administering calcium. Metastatic
370 Laboratory-Related Problems

FIGURE 34.2 Chvostek sign: facial muscle spasm elicited by

tapping the facial nerve anterior to the earlobe and below the
zygomatic arch. (From Marshall SA, Ruedy J: On Call: Principles
and Protocols, 4th ed. Philadelphia, Elsevier, 2004, p 331.)

FIGURE 34.3 Trousseau sign: carpal spasm elicited by occluding

arterial blood flow to the forearm for 3 to 5 minutes. (From
Marshall SA, Ruedy J: On Call: Principles and Protocols, 4th ed.
Philadelphia, Elsevier, 2004, p 332.)
 Electrolyte Abnormalities 371

calcification may occur if the serum phosphate concentration

remains high as calcium is administered.
A symptomatic child should be treated with 10% IV calcium
gluconate, 100 to 200 mg/kg over a period of 5 to 10 minutes. Addi-
tional doses can be given if necessary. If possible, peripheral infu-
sion of calcium should be avoided. Extravasation of calcium may
result in significant tissue necrosis. Scalp veins and other small
peripheral veins should be avoided when infusing calcium
solutions. Hypotension and bradycardia are also potential effects
of calcium infusion; therefore all patients should be monitored
while receiving an infusion. Once the symptoms of hypocalcemia
have resolved, oral calcium supplementation can be started. Ele-
mental calcium can be given in a dosage of 50 mg/kg/day divided
into three to four doses.
An asymptomatic child with hypocalcemia may be treated with
oral elemental calcium in the same doses as just listed.
There are several circumstances in which caution is necessary in
the treatment of hypocalcemia. In children with abnormal calcium
mobilization, such as those with pancreatitis or rhabdomyolysis,
care should be taken when correcting the serum calcium concen-
tration because hypercalcemia may eventually result from the
release of complexed calcium when the pancreatitis or rhabdomy-
olysis resolves. In a child with acidosis, the ionized calcium concen-
tration will be increased because of displacement of calcium from
albumin. Correction of the acidosis may cause the ionized calcium
level to fall even further. Finally, in those with hypomagnesemia,
parathyroid hormone release is impaired and the tissue response
to parathyroid hormone is also diminished. The magnesium con-
centration will need to be corrected to treat the hypocalcemia
effectively.

REMEMBER
The most important element of the management of any electrolyte
problem is close monitoring with repeated measurement of electro-
lytes to gauge the effect of your treatment plan. The approaches
outlined in this chapter allow you to begin thinking about the prob-
lem and to initiate a treatment plan, but repeated reevaluation of
the patient’s clinical status and laboratory studies is essential.
 CHAPTER

35
Glucose Disorders
Alison Coren, MD

Glucose is the preferred substrate for most cellular processes. It is

particularly important for cerebral metabolism because the brain
cannot use free fatty acids and is only partially supported by
ketones.

Glucose Homeostasis
Glucose is absorbed in the small intestine and, transported through
the bloodstream and into the liver and pancreas via insulin-
independent GLUT-2 transporters. Insulin is released by beta cells
in the pancreatic islets of Langerhans in response to rising glucose.
It stimulates peripheral glucose uptake and glycogen synthesis and
inhibits endogenous glucose production and free fatty acid oxida-
tion. Counterregulatory processes maintain euglycemia by sup-
pressing insulin secretion and stimulating glycogenolysis,
gluconeogenesis, and lipolysis, most importantly through gluca-
gon, epinephrine, cortisol, and growth hormone.

Hypoglycemia
Symptoms of hypoglycemia stem from the brain’s response to
glucose deprivation. Neurogenic symptoms (e.g., palpitations,
tremor, anxiety, sweating, hunger, parethesias) precede neurogly-
copenic symptoms (e.g., confusion, coma, seizures). Symptoms typ-
ically occur when the plasma glucose decreases to 50 to 70 mg/dL,
although repeated episodes of hypoglycemia blunt awareness and
impair hepatic glucose release, perpetuating hypoglycemia. Common
causes of hypoglycemia are listed in Table 35.1.
Transient hypoglycemia is expected in the first 24 to 48 hours of
life as neonates transition from intrauterine to extrauterine life.
Critical labs should be obtained prior to treatment in all neonates
>48 hours and children with a blood glucose <60 mg/dL. All point-
of-care glucose values should be confirmed using a clinical labora-
tory method. Bicarbonate, β-hydroxybutarate, lactate, and free

372
 Glucose Disorders 373

TABLE 35.1 Causes of Hypoglycemia

Inborn errors of metabolism Glycogen storage diseases

Gluconeogenesis defects
Fatty acid oxidation defects
Hyperinsulinism Infant of a diabetic mother
Congenital hyperinsulinism
Insulinoma
Exogenous administration of insulin
Hormone deficiencies Hypopituitarism
Cortisol deficiency
Growth hormone deficiency
Drugs Oral hypoglycemics
Alcohol
Salicylates
β-Blockers
Other Starvation
Postsurgical dumping syndrome
Ketotic hypoglycemia

fatty acids are useful for narrowing the differential diagnosis

(Fig. 35.1). Consider checking additional labs if a particular diag-
nosis is suspected.
Treatment should not be delayed if the blood sample cannot
be collected quickly. If the child is able to eat, then 15 to 20 g of
simple carbohydrate is given. If not, then 2 mL/kg of 10% dextrose
is given as an intravenous bolus, followed by 10% dextrose at
maintenance with the goal to keep glucose >70 mg/dL. Long-term
management depends on the underlying etiology and may include
adherence to a particular diet, medication, surgery, and/or con-
sultation with a subspecialist.

Hyperglycemia
Hyperglycemia may be a sign of diabetes mellitus but is also seen in
response to stress and some medications (Table 35.2). Symptoms of
hypoglycemia may not appear until the concentration of serum
glucose exceeds the capacity of the proximal renal tubule to reab-
sorb glucose, which occurs at approximately 180 mg/dL. Gluco-
suria drives the classic symptoms of polyuria, polydipsia,
polyphagia, and weight loss. The diagnosis of diabetes mellitus is
made if the child meets any of the following criteria: random
plasma glucose 200 mg/dL, plasma glucose 126 mg/dL after
an eight hour fast on two separate occasions, plasma glucose
200 mg/dL two hours after a 75 gram glucose load, or hemoglo-
bin A1C >6.5%. If the diagnosis is unclear, repeat testing should be
performed.
 Metabolic Clues to Hypoglycemia Diagnosis

374
Hypoglycemia

Laboratory-Related Problems
HCO3, BOHB, Lactate, FFA

No Acidemia
Acidemia

BOHB ↓ BOHB ↓ Lactate ↑ BOHB ↑

FFA ↓ FFA ↑

Genetic Hyperinsulinism Fatty Acid Oxidation Gluconeogenesis Ketotic Hypoglycemia

Hypopituitarism in newborns Defects Defects Glycogenoses
Transitional Neonatal Hypoglycemia GH deficiency
Perinatal Stress Hyperinsulinism Cortisol deficiency
FIGURE 35.1 Metabolic clues to hypoglycemia diagnosis. HCO3, Bicarbonate; BOHB, beta hydroxybutarate; FFA, free fatty
acids; GH, growth hormone. From Thornton PS, Stanley CA, DeLeon DD, Harris D, Haymond MW, Hussain K, et al. Recommen-
dations from the Pediatric Endocrine Society for Evaluation and Management of Persistent Hypoglycemia in neonates, infants,
and children. J Pediatr 167: 238-245, 2015.
 Glucose Disorders 375

TABLE 35.2 Causes of Hyperglycemia

Stress Acute illness/sepsis

Trauma
Surgery
Burns
Drugs Parenteral administration of dextrose
Atypical antipsychotics
β-Blockers
Corticosteroids
Calcineurin inhibitors
Protease inhibitors
Thiazide and thiazide-like diuretics
Diabetes mellitus Type 1 diabetes mellitus
Type 2 diabetes mellitus
Neonatal diabetes
Maturity-onset diabetes of the young
Cystic fibrosis–related diabetes

Type 1 Diabetes Mellitus

Type 1 diabetes mellitus (T1DM) is caused by the autoimmune
destruction of the insulin-producing pancreatic beta cells. Autoan-
tibodies may be detected in the blood and distinguish T1DM from
other types of diabetes mellitus.
Treatment requires insulin replacement. Children with long-
standing T1DM typically require between 0.7 and 1 unit/kg/day
of insulin. Children with new-onset T1DM may present in “hon-
eymoon,” a phase in which they have residual endogenous insulin
secretion from remaining beta cells, and require less exogenous
insulin. Pubescent children may experience a period of insulin
resistance and require more exogenous insulin. Many different
insulin analogues and regimens are available (Table 35.3 and
Table 35.4). About 1/3 of the total daily insulin dose is given as
the basal dose and 2/3 is given as bolus doses with meals.
Approximately 25% of children with T1DM present in diabetic
ketoacidosis (DKA). Children with known diabetes may also
develop DKA when they deliberately or inadvertently miss insulin
or experience illness or trauma that overwhelm homeostatic mech-
anisms. Signs and symptoms include dehydration, tachycardia,
tachypnea, Kussmal respirations, nausea, vomiting, abdominal
pain, confusion, drowsiness, and progressive loss of consciousness.
Laboratory findings include hyperglycemia, metabolic acidosis (pH
<7.30, bicarbonate <15 mmol/L), and ketonemia.
Treatment of DKA starts with fluid resuscitation. A 10 to
20 ml/kg normal saline bolus is given over 1-2 hours and repeated
376 Laboratory-Related Problems

TABLE 35.3 Types of Insulin

Rapid acting Lispro (Humalog)

Aspart (Novolog)
Glulisine (Apidra)
Short acting Regular insulin
Intermediate Neutral Protamine Hagedorn
acting (Novolin N, Humalin N)
Long acting Glargine (Lantus)
Detemir (Levemir)

TABLE 35.4 Insulin Regimens

Method Description
Basal-bolus Long-acting insulin is given once daily; rapid-acting insulin is
given with meals and snacks
Mixed-split Intermediate-acting and short-acting insulin are given twice
daily with breakfast and dinner
Insulin Continuous subcutaneous rapid acting insulin infusion;
pump boluses are given with meals and snacks

until peripheral perfusion is restored. Subsequent fluid manage-

ment includes intravenous fluids with a tonicity 0.45% saline
with added potassium, typically 20 mmol/L potassium phosphate
and 20 mmol/L potassium chloride or acetate. The goal is to
replace the estimated 5% to 10% fluid deficit plus daily mainte-
nance fluid requirements over 48 hours. Usually hyperglycemia
resolves before ketoacidosis, so dextrose is added when the plasma
glucose drops below 250 to 300 or sooner if the drop is precipitous.
An insulin drip infusing at 0.05 to 0.1 unit/kg/hr is started after the
initial fluid bolus and continued until the ketoacidosis resolves.
Children must be closely monitored with hourly vitals, neurolog-
ical exams, and point of care glucose checks. Electrolytes are
checked every 2-4 hours. Blood urea nitrogen, calcium, magne-
sium, and phosphorus are often abnormal due to dehydration
and urinary losses secondary to DKA. The abnormalities typically
resolve with treatment and are followed if abnormal.
Cerebral edema occurs in 0.5% to 1% of children who present in
DKA and is associated with a high rate of morbidity and mortality.
Risk factors include young age, new onset diabetes, severe DKA,
and aggressive initial fluid resuscitation. It usually occurs 4
to 12 hours after starting treatment. Signs and symptoms include
headache, vomiting, altered mental status, incontinence, and
Cushing triad. Prompt administration of mannitol 1 g/kg or
 Glucose Disorders 377

3% hypertonic saline 5 mL/kg is given over 10 to 15 minutes and

repeated if there is no response in 30 minutes.
Type 2 Diabetes Mellitus
Type 2 diabetes mellitus (T2DM) results from insulin resistance
and relative insulin deficiency. Some factors that help to distin-
guish T1DM from T2DM include insidious onset; onset after
puberty; association with obesity and other features of metabolic
syndrome; presence of acanthosis nigricans; absence of autoanti-
bodies; family history of T2DM; as well as and nonwhite European
descent. Many children with T2DM have signs of microvascular
and macrovascular complications at the time of diagnosis, includ-
ing nephropathy, retinopathy, and neuropathy.
All children are encouraged to make lifestyle modifications to
promote a healthy diet and exercise. Metformin monotherapy is
started in children with a hemoglobin A1C <9. Metformin may
cause gastrointestinal upset, so the dose is started at 500 mg daily
and increased by 500 mg per week to a maximum dose of 1000 mg
twice daily. Insulin is added in children with a hemoglobin A1C 9
and those who have not achieved a hemoglobin A1C <6.5 after 6
months of Metformin monotherapy. Other pharmacologic agents
may be used but are not U.S. Food and Drug Administration
(FDA) approved in children.
Hyperglycemic hyperosmolar state (HHS) is a rare but life-
threatening complication of T2DM. Children may present with
altered mental status or seizures. Laboratory findings include
marked hyperglycemia (plasma glucose >600 mg/dL), hyperos-
molality (serum osmolality >330 mOsm/kg), severe dehydration,
and little to no ketonuria.
There are no prospective data to guide management of children
and adolescents with HHS; however, guidelines have been extrap-
olated from adult literature. The mainstay of treatment is to replace
the fluid deficit, which is estimated to be at least 12% to 15% of
body weight. Normal saline boluses are given to restore peripheral
perfusion, followed by intravenous fluids with 0.45 to 0.75% NaCl
at a rate to replace the fluid deficit over 24 to 48 hours. The goal is
to reduce the sodium by 0.5 mmol/L/hr and glucose by 75 to
100 mg/dL/hr. Serum glucose typically drops rapidly with fluid
resuscitation. Once the rate of decline is <50 mg/dL/hr, an insulin
drip may be added at 0.025 to 0.05 unit/kg/hr to achieve a decrease
in serum glucose of 50 to 75 mg/dL/hr.

Monogenic Diabetes
Monogenetic diabetes occurs in 1% to 4% of children with diabetes.
Monogenetic forms of diabetes may present similarly to T1DM and
T2DM. Neonatal diabetes mellitus should be considered in infants
378 Laboratory-Related Problems

diagnosed with diabetes mellitus at less than 6 months old.

Maturity-onset diabetes of the young should be considered in chil-
dren and adolescents who have a family history of diabetes in mul-
tiple successive generations and an absence of autoantibodies and
risk factors for T2DM. Genetic testing confirms the diagnosis.
Depending on the variant, some monogenic forms of diabetes
can be treated with oral hyperglycemic medications, such as
sulfonylureas.

Cystic Fibrosis–Related Diabetes

Cystic fibrosis (CF) is caused by a mutation in the CF transmem-
brane conductance regulator, which is present in the beta cell. Cys-
tic fibrosis–related diabetes (CFRD) primarily results from damage
to the islet cells and subsequent insulin insufficiency. It also results
from insulin resistance, particularly during acute illnesses and sec-
ondary to medications such as glucocorticoids. Children may expe-
rience an insidious decline in clinical status in the years leading up
to diagnosis. Screening with an oral glucose tolerance test should
start at age 10. Insulin replacement is the only recommended med-
ical treatment. Most patients require between 0.5 and 0.8 unit/kg/
day when they are at their baseline states of health but may require
much higher levels when sick.
Discussion
Physicians should have a high index of suspicion for glucose dis-
orders and be prepared to quickly manage patients with hypogly-
cemia and hyperglycemia.
 C HA P TE R

36
Hyperbilirubinemia
Keli Coleman, MD

Hyperbilirubinemia is characterized by excess bilirubin in the

blood. An accumulation of bilirubin, a breakdown product of
heme, leads to a yellowing of the skin, mucous membranes, and
sclerae known as jaundice. Jaundice is not a disease but is a clinical
sign of an underlying medical condition associated with hyperbi-
lirubinemia that needs to be investigated further. In adults, jaun-
dice is most often associated with hepatic disorders. In children,
although similar potentially severe hepatic illnesses may also be
associated with jaundice, most cases of jaundice are transient
and without significant consequences. The pediatrician must com-
plete an evaluation to determine the etiology of hyperbilirubinemia
and then plan the appropriate intervention. Jaundice in a hospital-
ized child rarely has an acute onset. The pediatrician on call may be
the first to be notified of hyperbilirubinemia, and it is most com-
monly identified in the newborn nursery.

PHONE CALL
Questions
1. What is the child’s age?
2. What are the child’s vital signs?
3. Does the child appear ill?
4. What is the child’s admitting diagnosis?
5. Has the child ever been jaundiced before?
6. Is the child receiving any medications?
Orders
Obtain the following laboratory tests as soon as possible:
1. Total and direct serum bilirubin level
2. Alkaline phosphatase (ALP), alanine transaminase (ALT),
aspartate transaminase (AST), γ-glutamyltransferase (GGT),
and lactate dehydrogenase (LDH)

379
380 Laboratory-Related Problems

3. Complete blood count, reticulocyte count, and peripheral blood

smear
4. In a newborn the mother’s and infant’s blood types and a
Coombs’ test will also be helpful
Inform RN
Tell the RN, “I will arrive at the bedside in … minutes.”
Hyperbilirubinemia is rarely life threatening, but in younger
children it deserves timely evaluation. In neonates the evaluation
should be immediate because in some instances the rise in bilirubin
may be rapid and severe and accompanied by life-threatening ane-
mia or place the newborn at risk for kernicterus.

ELEVATOR THOUGHTS
The differential diagnosis of jaundice is extensive. Jaundice in a
neonate usually has different potential causes than jaundice in
an older child. Direct (conjugated) hyperbilirubinemia and indirect
(unconjugated) hyperbilirubinemia are also associated with differ-
ent causes (Fig. 36.1 and Tables 36.1 and 36.2).

MAJOR THREAT TO LIFE

• Hemolytic anemia–mediated shock, heart failure, or nephropathy
• Kernicterus
• Liver Failure
Hyperbilirubinemia is generally most threatening when its
onset is rapid and the bilirubin is unconjugated. In the relatively
immature central nervous system of a neonate, especially if prema-
ture, unconjugated bilirubin may be deposited in the basal ganglia,
hippocampus, and subthalamic nuclei of the brain and can result in
severe brain damage and hearing loss.

BEDSIDE
Quick-Look Test
Does the patient appear well (comfortable), sick (uncomfortable or
distressed), or critical?
A general rule to follow in estimating the serum bilirubin level in a
neonate is that the sclerae become icteric at a bilirubin level of 2 to
4 mg/dL, the face and mucous membrane at a level of 4 to 7, the chest
and abdomen at a level of 8 to 10, the legs at levels greater than 12, and
the soles of the feet at levels greater than 15. Remember, this is a very
rough estimate, and a serum level must be obtained to accurately
assess the magnitude of the jaundice. Does the patient exhibit any
signs of hemodynamic instability or neurologic deficits?
 Hyperbilirubinemia
FIGURE 36.1 Schematic approach to the diagnosis of neonatal jaundice. G6PD, Glucose-6-phosphate dehydrogenase;

381
PK, pyruvate kinase. (From Oski FA: Differential diagnosis of jaundice. In Taeusch HW, Ballard RA, Avery MA (eds): Schaffer
and Avery’s Diseases of the Newborn, 6th ed. Philadelphia, WB Saunders, 1991, p 774.)
 382
TABLE 36.1 Diagnostic Features of the Various Types of Neonatal Jaundice

Peak Bilirubin

Laboratory-Related Problems
Nature of van Jaundice Concentration Bilirubin Rate of
den Bergh Age Accumulation
Diagnosis Reaction Appears Disappears mg/dL (days) (mg/dL/day) Remarks
“Physiologic Usually related to degree of
jaundice” maturity
Full-term Indirect 2-3 days 4-5 days 10-12 2-3 <5
Preterm Indirect 3-4 days 7-9 days 15 6-8 <5
Hyperbilirubinemia Metabolic factors: hypoxia,
as a result of respiratory distress, lack
metabolic factors of carbohydrate
Full-term Indirect 2-3 days Variable <2 1st wk <5 Thyroid
Premature Indirect 3-4 days Variable <15 1st wk <5 Genetic factors Drugs
Hemolytic states Indirect May appear Variable Unlimited Variable Usually >5 Drugs
and hematoma in 1st
24 hr
Mixed hemolytic Indirect and May appear Variable Unlimited Variable Usually >5 Infection
and hepatotoxic direct in 1st
factors 24 hr
Hepatocellular Indirect and Usually 2-3 Variable Unlimited Variable Variable, can be
damage direct days >5
From Brown AK: Neonatal jaundice. Pediatr Clin North Am 9:589, 1962.
 TABLE 36.2 Differential Diagnosis of Jaundice in Childhood

Unconjugated Hyperbilirubinemia Conjugated Hyperbilirubinemia

Hemolysis and Reticulocytosis
Positive Coombs’ Negative
Test Coombs’ Test No Hemolysis Obstructive Infectious Metabolic Toxic Idiopathic Autoimmune
ABO and Rh RBC enzyme Gilbert Biliary atresia Hepatitis A, B, C, D, E Wilson disease Total Idiopathic neonatal Autoimmune
incompatibility defect (G6PD syndrome Choledochal Cytomegalovirus α1-Antitrypsin parenteral hepatitis chronic
Autoimmune, deficiency) Physiologic cyst Herpes simplex 1, 2, 6 deficiency nutrition Alagille syndrome hepatitis
systemic lupus Hemoglo- jaundice of Cholelithiasis Epstein-Barr virus Galactosemia Acetaminophen Nonsyndromic paucity Sclerosing
erythematosus binopathy the Tumor/ Coxsackievirus Tyrosinemia Ethanol of intrahepatic bile cholangitis
Drug-induced and (sickle cell newborn neoplasia Echovirus Fructosemia Salicylates ducts Graft-versus-
idiopathic anemia) Breast milk Bile duct Measles Niemann-Pick Iron Progressive familial host
acquired RBC membrane jaundice stenosis Varicella disease Halothane intrahepatic disease
hemolytic anemia defect Crigler-Najjar Spontaneous Syncytial giant cell Gaucher disease Isoniazid cholestasis
(hereditary syndrome bile duct (paramyxovirus) Zellweger Valproic acid Familial benign recurrent
spherocytosis) Hypothyroidism perforation Toxoplasmosis syndrome Venoocclusive cholestasis
Hemolytic-uremic Pyloric stenosis Bile-mucous Syphilis Wolman disease disease Cholestasis with

Hyperbilirubinemia
syndrome Internal plug Leptospirosis Cystic fibrosis lymphedema
Wilson disease hemorrhage Bacterial sepsis/urinary Neonatal iron (Aagenaes
tract infection storage disease syndrome)
(especially gram Indian childhood Cholestasis with
negative) cirrhosis hypopituitarism
Cholecystitis Trihydroxypro stanic Familial erythrophago-
Fitz-Hugh–Curtis acidemia cytic lymphohistio-
syndrome cytosis

383
G6PD, Glucose-6-phosphate dehydrogenase; RBC, red blood cell.
From Behrman RE, Kliegman RM: Nelson Essentials of Pediatrics, 2nd ed. Philadelphia, WB Saunders, 1994.
384 Laboratory-Related Problems

Airway and Vital Signs

Tachycardia, pallor, respiratory distress, and poor perfusion can be
seen with severe hemolytic processes that can cause heart failure.
Fever may represent systemic infection such as sepsis. Children
with severe hepatic dysfunction usually appear quite ill.

Selective Physical Examination

HEENT The sclerae and mucous membranes (especially
under the tongue) are very good places to
assess jaundice, particularly in darker
pigmented children; cephalohematoma or
excessive bruising
Cardiovascular Heart rate, pulse volume, blood pressure,
perfusion, jugular venous distention
Abdomen Distention, hepatosplenomegaly, masses,
tenderness, ascites (fluid wave), caput medusa,
incision scars
Neurologic Mental status, tone (axial as well as segmental in
infants), cranial nerves, quality of cry,
primitive reflexes (Moro, grasp, suck, tonic-
neck), motor function

HEENT, Head, Eyes, Ears, Nose, Throat.

Acute cardiac failure can occur with severe hemolytic processes.

A change in color of the urine would also be expected with severe
hemolysis.
Management
Diagnostic investigation depends largely on the age of the child and
the presence of associated findings besides jaundice. The two most
common scenarios are discussed in this chapter: (1) a neonate in
the nursery or on the inpatient floor whose laboratory results indi-
cate a high bilirubin level is brought to the attention of the on-call
pediatrician and (2) a child in whom jaundice develops acutely dur-
ing a hospitalization.

Neonatal Hyperbilirubinemia
Neonatal hyperbilirubinemia is defined as a total serum or plasma
bilirubin greater than 95th percentile on the hour-specific nomo-
gram in a 35-week gestational age or older infant or a total serum
concentration of bilirubin greater than 5 mg/dL (86 μmol/L)
(Fig. 36.2). Infants at risk for developing neonatal hyperbilirubine-
mia include premature babies, and infants admitted to rule out
sepsis immediately after birth due to prolonged rupture of mem-
branes, maternal chorioamnionitis, or respiratory distress.
 Hyperbilirubinemia 385

25 428

20 342
95th percentile
High Risk Zone
Serum Bilirubin (mg/dl)

e
Zon
15 Risk 257
diate
rme ne
Inte Zo
High Risk

μmol/L
diate
rme
Inte
10 Low 171

Low Risk Zone

5 85

0 0
0 12 24 36 48 60 72 84 96 108 120 132 144
Postnatal Age (hours)

FIGURE 36.2 Nomogram to determine the risk of specific eleva-

tions in serum bilirubin. (From American Academy of Pediatrics
Subcommittee on Hyperbilirubinemia: Management of hyperbi-
lirubinemia in the newborn infant 35 or more weeks of gestation.
Pediatrics 114:297-316, 2004.)

Hyperbilirubinemia within the first 24 hours of birth is worri-

some. A rapid rise in unconjugated bilirubin in the first 24 hours is
common in hemolytic processes involving ABO incompatibility
between the infant and the mother, concealed hematoma or hem-
orrhage, cytomegalovirus infection, sepsis, congenital rubella, or
congenital toxoplasmosis. Laboratory evaluation should be initi-
ated immediately and include a complete blood count and periph-
eral smear, reticulocyte count, ABO type and screen, direct and
indirect Coombs’ tests, total and direct bilirubin, and serum albu-
min. Acute hemolytic anemia in a newborn may require not only
simple transfusion but also partial or complete exchange transfu-
sion. A cord hemoglobin level of 10 g/dL, a bilirubin level of
5 mg/dL or greater on the first day of life, and a reticulocyte count
of 15% or more suggest severe hemolytic anemia and may require
at least partial exchange transfusion.
Exchange transfusions require experience and advanced plan-
ning to coordinate safely. It is a treatment option reserved for
symptomatic infants with severe hyperbilirubinemia that does
not decrease adequately with phototherapy. It is prudent to contact
an experienced provider to assist with this procedure. Blood for
exchange transfusion should be as fresh as possible and be
completely cross matched if possible. In acute settings, type
O-negative blood may be used. The blood should be warmed to
37 degrees C and should be continuously mixed or gently agitated
during the transfusion. The exchange requires large intravenous
lines, which in a newborn generally means umbilical venous
386 Laboratory-Related Problems

catheterization (see Chapter 26, Lines, Tubes, and Drains, for the
details of umbilical catheterization). When proper positioning of
the umbilical line or lines has been confirmed by radiograph,
10- to 20-mL aliquots of blood are withdrawn, alternating with
equal volumes of donor blood. Calculation of the total volume of
the exchange is based on an estimated total circulating blood vol-
ume of 85 mL/kg body weight.
Phototherapy is the treatment of choice for mild indirect hyper-
bilirubinemia. The infant is placed in a diaper only, or a surgical
mask may be used to create a “bikini diaper” so that the maximal
amount of skin surface area can be bathed in blue (420- to 470-nm
wavelength) light. Bilirubin in the skin absorbs this wavelength,
and photoisomerization converts toxic 4Z, 15Z-bilirubin into the
unconjugated, configurational isomer 4Z, 15E-bilirubin, which
can be excreted without the need for conjugation. One must be
conscientious about shielding the eyes of newborn infants from this
intense light exposure and monitoring the infant’s temperature,
regardless of their gestational age. Remember that phototherapy
increases the infant’s insensible water loss by as much as 20%,
and therefore the maintenance fluid requirements of the baby must
be adjusted to maintain adequate hydration and ensure good urine
output to adequately excrete bilirubin.
The risk to the infant of specific serum bilirubin levels relative to
age can be determined by using a nomogram (see Fig. 36.2). Risk
factors for the development of severe hyperbilirubinemia are
shown in Box 36.1.
In an infant who is 2 to 3 days of age, hyperbilirubinemia may be
physiologic and require no intervention if the level remains reason-
able. Table 36.1 details several types of jaundice and their onset,
along with ranges of bilirubin levels and some of their causes. Levels
of 15 mg/dL or less total bilirubin in a full-term infant rarely require
intervention. So-called physiologic jaundice, or icterus neonatorum,
may be influenced by maternal diabetes, polycythemia, race, male
sex, trisomy 21, bruising or cephalohematoma, delayed stooling,
oxytocin induction, breastfeeding, and other nonspecific factors.
The diagnosis of physiologic jaundice in term or preterm
infants can be established by excluding known causes of neonatal
jaundice by history and clinical and laboratory findings, as in
Table 36.2. The cause of jaundice should be pursued if (1) hyper-
bilirubinemia occurs within 24 hours of birth, (2) the level exceeds
12 mg/dL in the absence of risk factors, (3) the level rises at a rate
greater than 5 mg/dL/24 hr, or (4) the jaundice persists for longer
than 2 weeks.
If the percentage of direct to total bilirubin rises, one must sus-
pect a cholestatic process, hepatocellular damage, or a metabolic
disorder (galactosemia, tyrosinemia, α1-antitrypsin deficiency).
 Hyperbilirubinemia 387

BOX 36.1 Risk Factors for the Development of Severe

Hyperbilirubinemia in Infants of 35 or More Weeks’
Gestation (in Approximate Order of Importance)

Major Risk Factors

Predischarge TSB or TcB level in the high-risk zone (see Fig. 36.2)
Jaundice observed in the first 24 hr
Blood group incompatibility with a positive direct antiglobulin test, other
known hemolytic disease (e.g., G6PD deficiency)
Gestational age of 35-36 weeks
Previous sibling received phototherapy
Cephalohematoma or significant bruising
Exclusive breastfeeding, particularly if nursing is not going well and weight
loss is excessive
East Asian race
Minor Risk Factors
Predischarge TSB or TcB level in the high intermediate-risk zone (see Fig. 36.2)
Gestational age of 37-38 weeks
Jaundice observed before discharge
Previous sibling with jaundice
Macrosomic infant of a diabetic mother
Maternal age
25 years
Male gender
Decreased Riska
TSB or TcB level in the low-risk zone (see Fig. 36.2)
Gestational age
41 weeks
Exclusive bottle feeding
Black raceb
Discharge from hospital after 72 hr
From American Academy of Pediatrics Subcommittee on Hyperbilirubinemia: Management
of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics
114:297-316, 2004, with permission.
a
Factors associated with a decreased risk for significant jaundice, listed in order of decreasing
importance.
b
Race as defined by mother’s description.
G6PD, Glucose-6-phosphate dehydrogenase; TcB, transcutaneous bilirubin; TSB, total serum
bilirubin.

Direct hyperbilirubinemia is always considered pathologic. Further

laboratory testing is necessary, including a prothrombin time (PT)
and partial thromboplastin time (PTT) to assess hepatic synthetic
function, as well as measurement of the transaminases AST (also
known as serum glutamic-oxaloacetic transaminase [SGOT]),
ALT (also known as serum glutamate pyruvate transaminase
[SGPT]), LDH, ALP, and GGT to assess hepatocellular damage.
Testing for cystic fibrosis is indicated, as are abdominal ultrasound
388 Laboratory-Related Problems

studies to determine the integrity of the biliary tree (screening for

biliary atresia or a choledochal cyst). Diagnoses such as the latter
are also suggested by persistent jaundice in the face of acholic stools
and poor weight gain.
Figs. 36.3 and 36.4 present nomograms for the treatment of
indirect hyperbilirubinemia in healthy and at-risk term infants
with phototherapy and exchange transfusion, respectively.
Acute Jaundice in Children
Jaundice beyond the neonatal period is much less common and is
often indicative of an acute, chronic, or critical condition. In this
age group, it is important to distinguish between mild, self-limiting
conditions and serious liver or hematologic diseases. Acute jaun-
dice in children, especially conjugated hyperbilirubinemia, is
almost always a manifestation of severe hepatocellular damage
from a hypoxic-ischemic insult, accumulation of a hepatotoxin
(acetaminophen ingestion), or acute hepatitis. Because conjugated
hyperbilirubinemia is usually a consequence of liver and biliary dis-
ease, the initial differential will focus on conditions affecting the
liver and biliary tree. These children will benefit from early consul-
tation with a pediatric hepatologist to assist in evaluation and man-
agement. As in neonates, accumulation of unconjugated bilirubin
in children is usually due to hemolysis of red blood cells with
release of bilirubin into the blood. Hemoglobinopathies, erythro-
cyte enzyme defects, and erythrocyte membrane defects may all
cause hemolysis. Conditions that impair the hepatic uptake of bil-
irubin or conjugation (i.e., Gilbert syndrome and Crigler-Najjar
syndrome types I and II) can also increase the level of unconjugated
bilirubin in children. Diagnostic evaluation in children is much the
same as for neonates. Synthetic hepatic function, as well as hepa-
tocellular injury, must be investigated, as should cholestasis. Acute
hepatocellular injury is manifested by an elevation in aminotrans-
ferases (AST, ALT), LDH, and GGT regardless of the cause. The
most marked transaminase elevations are seen with acute viral hep-
atitis, hypoxic-ischemic injury, hepatotoxin exposure, and Reye
syndrome. A differential rise in ALT or AST can suggest a variety
of processes, but usually the two are elevated similarly. Elevation of
ALP, 50 -nucleotidases, cholesterol, and conjugated bilirubin sug-
gests obstruction and/or inflammation of the hepatobiliary tract.
Assessment of the synthetic function of the liver is important.
The PT and PTT are important functional assays for the various
serum globulins manufactured in the liver, particularly vitamin
K–dependent clotting factors (II, VII, IX, X). Information regard-
ing the patient’s recent travel, dietary history, and family history
may provide clues to the underlying cause of potential hemolysis
or liver disease. For the on-call physician, little in the way of
 25 428

Total Serum Bilirubin (mg/dL)

20 342

15 257

μmol/L
10 171

5 85
Infants at lower risk (≥38 wk and well)
Infants at medium risk (≥38 wk + risk factors or 35-37 6/7 wk. and well)
Infants at higher risk (35-37 6/7 wlk. + risk factors)
0 0
Birth 24 h 48 h 72 h 96 h 5 Days 6 Days 7 Days

Hyperbilirubinemia
Age

Use total bilirubin. Do not subtract direct reacting or conjugated bilirubin.

Risk factors = isoimmune hemolytic disease, G6PD deficiency, asphyxia, significant lethargy, temperature instability,
sepsis, acidosis, or albumin < 3.0g/dL (if measured)
For well infants 35-37 6/7 wk can adjust TSB levels for intervention around the medium-risk line. It is an option to
intervene at lower TSB levels for infants closer to 35 wks and at higher TSB levels for those closer to 37 6/7 wk.
It is an option to provide conventional phototherapy in hospital or at home at TSB levels 2-3 mg/dL (35-50mmol/L)
below those shown but home phototherapy should not be used in any infant with risk factors.

FIGURE 36.3 Nomogram for phototherapy in hospitalized infants of 35 or more weeks’ gestation. G6PD, Glucose-6-phosphate dehy-

389
drogenase; TSB, total serum bilirubin. (From American Academy of Pediatrics Subcommittee on Hyperbilirubinemia: Management of
hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 114:297-316, 2004.)
 30 513
Infants at lower risk (≥38 wk and well)

390
Infants at medium risk (≥38 wk + risk factors or 35-37 6/7 wk. and well)

Total Serum Bilirubin (mg/dL)

Infants at higher risk (35-37 6/7 wlk. + risk factors)
25 428

Laboratory-Related Problems
μmol/L
20 342

15 257

10 171
Birth 24 h 48 h 72 h 96 h 5 Days 6 Days 7 Days
Age

The dashed lines for the first 24 hours indicate uncertainty due to a wide range of clinical circumstances
and a range of responses to phototherapy.
Immediate exchange transfusion is recommended if infant shows signs of acute bilirubin encephalopathy
(hypertonia, arching, retrocollis, opisthotonos, fever, high-pitched cry) or if TSB is ≥ 5 mg/dL (85 μmol/L)
above these lines.
Risk factors - isoimmune hemolytic disease, G6PD deficiency, asphyxia, significant lethargy, temperature
instability, sepsis, acidosis.
Measure serum albumin and calculate B/A ratio (see legend).
Use total bilirubin. Do not substract direct reacting or conjugated bilirubin
If infant is well and 35-37 6/7 wk (median risk) can individualize TSB levels for exchange based on actual
gestational age.

FIGURE 36.4 Nomogram for exchange transfusion in infants of 35 or more weeks’ gestation. G6PD, Glucose-6-phosphate dehydro-
genase; TSB, total serum bilirubin. (From American Academy of Pediatrics Subcommittee on Hyperbilirubinemia: Management of
hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 114:297-316, 2004.)
 Hyperbilirubinemia 391

intervention can be done in an older child with acute jaundice other

than supportive care and initiation of the diagnostic evaluation.
Children who have suffered hepatocellular damage are subject to
shock and disseminated intravascular coagulation, which may need
to be aggressively supported early in their disease while you are
completing the diagnostic evaluation.
Summary
Hyperbilirubinemia in a newborn may be physiologic, but a wide
variety of pathologic conditions must be considered and ruled out
by history, physical examination, and laboratory evaluation.
Although rarely life threatening, hyperbilirubinemia may have seri-
ous consequences. Management goals include initiating prompt,
appropriate therapy (i.e., phototherapy or exchange transfusion)
when indicated. A systematic evaluation of the risks of developing
severe hyperbilirubinemia is based on the total bilirubin level, ges-
tational age, presence or absence of risk factors, and health of the
infant and is facilitated by using an hour-specific bilirubin nomo-
gram. In older children, hepatocellular injury from infection,
toxins, or a hypoxic-ischemic event must be ruled out and aggres-
sive supportive treatment instituted at once to allow time to make
the diagnosis and initiate more definitive therapy.
 AP P EN D I X

A
Pediatric Procedures
James J. Nocton, MD and Rainer G. Gedeit, MD

Procedures are a great source of anxiety for the house officer or

medical student. As a pediatric house officer, you may have limited
opportunities to perform some procedures; consequently, it may be
difficult to feel a sense of competence when you find yourself in the
position of needing to obtain intravenous (IV) access or intubate a
child. However, if you take advantage of every opportunity to try
procedures, you will increase your chances of becoming proficient
at such techniques as peripheral IV line placement, venipuncture,
arterial blood gas sampling, lumbar puncture, intubation, umbilical
line placement, joint aspiration, interosseous line placement,
femoral line placement, and peripheral arterial line placement.
The key to success with any procedure involving a sharp object
(e.g., a needle) is comfort of the person holding the sharp object. Set
up for every procedure in the same way so that the routine is com-
fortable and automatic. Know the supplies that you will need and
prepare them in advance, whether they are blood tubes, culture
bottles, slides, or swabs.
For almost every procedure involving needles, there is a choice
regarding the use of local anesthesia. Obviously, this is not a con-
sideration in a dire emergency, but that is the exception, not the
rule. Small children are fearful and move and resist, thereby reduc-
ing the likelihood of success. If properly anesthetized, the child
resists and moves less, and you will be a hero when the IV goes
in on the first try!
Topical anesthetics can be used for any procedure in which
numbing of the skin will be helpful. Many different types of topical
anesthetics are available, from creams to aerosols. Learn what is
available to you and learn the indications and contraindications
for each. Topical anesthetics are ideal for lumbar puncture or joint
aspiration, especially in older children. Remember to consider the
child’s comfort during every procedure; the child’s comfort may
increase your own.

394
 Pediatric Procedures 395

INTRAVENOUS ACCESS
IV access is an important ingredient in intervention and stabiliza-
tion of any patient in the hospital. The following is a brief overview
of the options available, some helpful technical tips, and a general
approach to this procedure in pediatrics.
The legendary house officers who can get an IV line into anyone
always have a routine that they follow with every patient, regardless
of age. First, try to perform all procedures in a treatment or proce-
dure room. All the supplies are there, and it maintains the patient’s
room as a sanctuary where the child is free from harm. Second, make
sure that all your favorite supplies are set up before bringing the
patient into the room. Third, get help to hold the child during the
procedure. Do not ever ask a parent to hold a child for a procedure.
First, they might faint, whereupon you will have two patients. Sec-
ond, the parents should “rescue” their child from you afterward, not
assist you. Regarding whether parents should observe procedures, if
you are not comfortable having the parents in the room, tell them
that honestly and explain that you are more likely to be successful
if they are not present. Most parents respond favorably if you express
your desire to make the procedure easier for their child.
The person holding the patient is as important to this process as
the person holding the needle! Give the assistant clear instructions
about how to restrain the child and assist you best. This is true in
both a dire emergency and routine replacement of a peripheral IV
line. Apply a rubber tourniquet above the potential site tight
enough to occlude the veins but not so tight that you cannot palpate
a pulse distally. Look and feel for the veins in the same places that
you have veins, starting at the most distal point. Veins feel hollow,
like a straw. Tendons are tense and cordlike. Arteries are firm and
deep and should be pulsatile. Look at several different sites before
making an attempt. It pays to shop around a little. Remove the
tourniquet until you are ready to make your attempt.
The sites that should be explored in any patient include the dor-
sal hand veins, the radial vein of the wrist, the anterior ulnar vein of
the forearm, the median cephalic vein in the lateral antecubital
fossa, the median basilic vein in the medial antecubital fossa, the
superficial veins of the dorsum of the foot, and the saphenous vein
anterior and superior to the medial malleolus of the ankle and
along its proximal length on the medial aspect of the foreleg. Next,
the external jugular should be considered. Remember, neck lines in
any child and scalp IV lines in infants are very distressing to par-
ents and should be choices of last resort. IV line placement is not a
benign intervention. Children suffer more complications from IV
lines than from any other medical intervention in the hospital.
396 Appendices

Selecting the catheter for a peripheral IV line is very important,

not only to the success of your attempt but also to the longevity of the
line that you place. Whatever size you originally think of, choose one
size larger. Larger IV catheters will go in easier and last longer. In
most full-term infants, regardless of hydration status, a 22-gauge
catheter can be placed in any vein. Frequently in infants, a 20-gauge
catheter or even an 18-gauge catheter can be placed in the antecu-
bital, distal saphenous, or external jugular veins. Save the 24-gauge
catheters for preterm infants. Smaller IV catheters do not advance
into veins easily, cannot handle high flow rates, and cause a jetlike
stream within the vessel that damages endothelium and causes infil-
tration into the surrounding tissues. The bigger needle may appear
to hurt more, but it also goes in better and lasts longer, which means
fewer IV attempts and less discomfort for your patient.
After you have selected the site, put on your gloves and prepare
the site with povidone-iodine scrub and alcohol. Apply the tourni-
quet again and confirm that this is a good site. Then ask your assis-
tant to gently but firmly hold the child. The IV starter should hold
the extremity to ensure its position. Make the attempt boldly to get
through the skin in one quick stab. Anticipate the withdrawal reac-
tion and then advance into the vessel.
An IV catheter is a needle within a plastic tube. When you see
blood return in the needle, you know that the lumen of the needle
is within the lumen of the vessel; it does not necessarily mean that the
catheter is within the lumen of the vessel. Because the needle tip
extends 2 to 3 mm beyond the catheter, you must advance the needle
and catheter a bit more before the catheter can be advanced into the
vessel as you withdraw the needle. The blood return in the catheter
should remind you to release the tourniquet and place the needle in a
safe place away from the field and the child. Instruct your assistant to
maintain control over the child until the IV line is safely and securely
taped into place. Be meticulous about how you tape the IV line, and
do it the same way every time. This ensures that the nurse will not be
calling you again in 30 minutes to replace the same IV line.
In conclusion, do not approach placement of an IV line timidly.
Confidence begets success. Be systematic, consistent, methodical,
and caring, and pick a larger catheter. In addition, know your
limitations. If you are not successful, call someone else to attempt
the line placement. Rather than leave once help arrives, stay and
observe “the master” at work. You may learn more than you expect.
Remember that you want to help the patient to be healed and be
comfortable. “Getting” a difficult IV line is a big confidence boost,
but it should not come at the expense of causing more discomfort
to the patient than is acceptable.
If a peripheral IV line cannot be established, a deep or central
line may be required. Again, know your limitations. Central lines
 Pediatric Procedures 397

require experience, time, sterile technique, and sedation and are

not to be pursued casually or without supervision. In an extreme
emergency, an intraosseous line can be life saving and should be
considered within 90 seconds, even in a hospitalized patient. There
are many options for insertion of an intraosseous needle. Become
familiar with what is available at each institution. In children youn-
ger than 3 years, the anterior tibial plateau is prepared with
povidone-iodine and alcohol, chlorhexidine, or alcohol. The needle
is directed 1 to 3 cm below the tibial tuberosity at a 30-degree angle
caudally to avoid the epiphyseal growth plate. Insertion requires
firm, steady pressure with a twisting motion through the bone. Less
resistance is felt as the marrow cavity is entered. Other options are
available for intraosseous placement, including needles that screw
into the bone with or without an accompanying “drill.” After nee-
dle placement, attempt to aspirate, but frequently, no marrow is
aspirated. Infusion with a syringe should be free of resistance or soft
tissue swelling. In older children the distal end of the femur can be
used, with the needle angled 30 degrees cephalad. Once in place, the
needle must be secured and the extremity restrained. Again, med-
ical students or house officers must know their own limitations, be
willing to learn, and be concerned for the welfare and comfort of the
patient.
Lumbar Puncture
A lumbar puncture, or spinal tap, is likely to be the most common
procedure performed by a pediatric house officer. Obtaining cere-
brospinal fluid (CSF) for culture, microscopy, and biochemical
analysis is an essential part of every work-up for sepsis.
For a right-handed person performing a lumbar puncture, the
patient’s head should be positioned to the left, with the supply kit
to the right. This allows you to position your left hand so that your
fingers are on the iliac crest and your thumb is on the desired inter-
space. The right hand is then free to insert the needle and fill the tubes.
The person holding the child is equally important. This person
must also monitor the child’s condition, especially the respiratory pat-
tern in infants. Infants can be held quite effectively in the left lateral
decubitus position or in the upright sitting position (Fig. A.1). Older
children, if uncooperative, may require a second holder. Remember,
never attempt a lumbar puncture without adequate help.
After you have set up the supply tray and have given your
holder or holders their instructions, put on gloves in sterile fashion.
With the child in the desired position, prepare the lower part of the
back vigorously with povidone-iodine or chlorhexidine. Place the
cover drape, and find the anatomic landmarks by positioning your
left fingers on the iliac crest and feeling for the posterior iliac spines
with your left thumb. The level of the crest should be L2-3, and the
398 Appendices

FIGURE A.1 Sitting position for restraining an infant for lumbar

puncture. Lateral view.

level of the posterior spine should be L5. Feel for the L3-4 inter-
space, and keep your left thumb at that level. Using a prefilled 3-
mL syringe, inject a small amount of 1% lidocaine subcutaneously.
Be prepared for the child to move, and make sure that your holder
is also prepared. Once the child settles, advance the needle, aspirate,
and inject approximately 0.5 mL of lidocaine. There is no good rea-
son to not use local anesthesia for a lumbar puncture. It will help
immensely in avoiding a traumatic or bloody tap because of move-
ment of the child. Return the lidocaine syringe to your sterile tray
and insert the spinal needle at a 15- to 20-degree angle cephalad to
avoid the posterior vertebral spines (Fig. A.2). If resistance is met,
withdraw and angle more cephalad. In a term infant, the needle will
advance 1 to 1.5 cm, and then resistance will be felt before the
“pop” as the needle penetrates the dura (Fig. A.3). Withdraw the
stylet, and watch for CSF backflow. If an opening pressure is
 Pediatric Procedures 399

FIGURE A.2 The needle should be inserted in a slightly cephalad

direction to avoid the vertebral bodies.

Ligamentum flavum
Cauda equina Dura
Epidural space
FIGURE A.3 Once the needle has penetrated the dura, the stylet is
withdrawn to allow spinal fluid to flow freely.

desired, attach the manometer via the three-way stopcock and

determine the CSF level in centimeters. Then proceed to collect
approximately 5 mL of CSF in sterile test tubes. Replace the stylet,
withdraw the needle, and apply pressure on the site with the left
thumb. The holder should allow the child to straighten and relax.
A small bandage should be placed and the date written on it.
As with every procedure, a succinct procedural note should be
written immediately to document the indications for the lumbar
puncture, the technique, the results, and any complications.
 AP P EN D I X

B
Resuscitation
Calculations
James J. Nocton, MD and Rainer G. Gedeit, MD

Intubation Equipment
Endotracheal
Age/Weight Tube Size Laryngoscope Blade
Newborn/3-5 kg 2.5-3.5 mm 0-1 straight
Infant/6-9 kg 3.5 mm uncuffed 1 straight
Toddler/10-11 kg 4.0 mm uncuffed 1 straight
Small child/12-14 kg 4.5 mm uncuffed 2 straight
Child/15-18 kg 5.0 mm uncuffed 2 straight or curved
Child/19-22 kg 5.5 mm uncuffed 2 straight or curved
Large child/24-28 kg 6.0 mm cuffed 2-3 straight or curved
Adult/30 kg 6.5 mm cuffed 3 straight or curved

Resuscitation Medications
Drug Dosage
Epinephrine First dose
IV/Intraosseous (IO): 0.01 mg/kg
(1:10,000, 0.1 mL/kg)
Endotracheal tube (ETT): 0.1 mg/kg
(1:1000, 0.1 mL/kg)
Subsequent doses
Repeat every 3-5 minutes during cardiopulmonary
resuscitation (CPR)
Atropine 0.02 mg/kg/dose; minimum dose: 0.1 mg;
Maximum dose 1 mg
Glucose IV/IO: 0.5-1 g/kg (1-2 mL/kg of 50% solution)

400
 AP P EN D I X

C
Calculation of
Creatinine Clearance
James J. Nocton, MD

CrCl ðmL= min Þ ¼ UCr ðmg=mLÞ  V ðmL= min Þ= PCr ðmg=mLÞ

CrCl ¼ creatinine clearance

UCr ¼ urinary concentration of creatinine
V ¼ urinary flow rate
PCr ¼ plasma concentration of creatinine
To correct clearance for body surface area (BSA):


Corrected CrCl ¼ CrCl ðmL= minÞ  1:73=BSA m2

401
 AP P EN D I X

D
Calculation of
Alveolar-Arterial
Oxygen Gradient
James J. Nocton, MD

The alveolar-arterial oxygen gradient, or P(A–a)O2, can be cal-

culated easily from the arterial blood gas (ABG) results. It is useful
for confirming the presence of a shunt.

PðA
aÞO2 ¼ PAO2
PaO2

• PAO2 ¼ alveolar oxygen tension calculated as shown subsequently

• PaO2 ¼ arterial oxygen tension measured by ABG determination
PAO2 can be calculated by the following formula:

PAO2 ¼ ðPB
PH2 OÞðFIO2 Þ
PaCO2 =R

• PB ¼ barometric pressure (760 mm Hg at sea level)

• PH2O ¼ 47 mm Hg
• FIO2 ¼ fraction of O2 in inspired gas
• PaCO2 ¼ arterial CO2 tension measured by ABG determination
• R ¼ respiratory quotient (0.8)
Normal P(A–a)O2 ranges from 12 mm Hg or less for infants,
children, and adolescents.
In pure ventilatory failure, P(A–a)O2 will remain 12 to 20 mm
Hg. In oxygenation failure, it will increase.
Adapted from Marshall SA, Ruedy J: On Call: Principles and
Protocols, 4th ed. Philadelphia, WB Saunders, 2004, p 425.

402