Urology for the Pediatrician, An Issue of Pediatric Clinics

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 Urology for the Pediatrician

Contents

Preface: What Pediatricians Need to Know about Urology xv

Pasquale Casale and Walid A. Farhat

Prenatal Ultrasound and Urological Anomalies 739

Douglass B. Clayton and John W. Brock III
Prenatal ultrasound is an integral part of caring for pregnant women in the
United States. Although surprisingly few data exist to support the clinical
benefit of screening ultrasound during pregnancy, its use continues to
rise. Urologic anomalies are among the most commonly identified, with
overall detection sensitivity approaching 90%. Prenatal hydronephrosis
is the most frequently identified finding and predicting postnatal pathology
based on its presence can be difficult. As the degree of fetal hydronephro-
sis increases so does the risk of true urinary tract pathology. Diagnoses
that require more urgent care include causes of lower urinary tract ob-
struction and bladder and cloacal exstrophy.

The Current Management of the Neurogenic Bladder in Children with Spina Bifida 757
Dominic Frimberger, Earl Cheng, and Bradley P. Kropp
The urological care of the neurogenic bladder consists of 2 components:
medical management with preservation of renal function and quality-of-
life issues with achieving dryness and independence of bladder and bowel
management. Both components are equally important for patients to live
a healthy and fulfilled life. This report explores the diagnosis of the neuro-
genic bladder; quality-of-life issues that caregivers and patients should ex-
pect; the importance of primary care knowledge of the neurogenic bladder
and treatment; surgical options; the transition of pediatric patients to adult
care; and the importance of caregiver and patient understanding of their
disease, treatment options, and responsibilities.

Inguinal and Genital Anomalies 769

Laura Stansell Merriman, Lindsey Herrel, and Andrew J. Kirsch
Problems of the groin and genitalia are a common presenting complaint in
both pediatrician’s offices and emergency departments. The authors en-
deavor to provide a comprehensive review of the most common inguinal
and genital anomalies encountered by the pediatrician, with a special
focus on examination and management.

Functional Bladder Problems in Children: Pathophysiology, Diagnosis, and

Treatment 783
Israel Franco
Functional lower urinary tract problems, bladder and bowel problems, or
dysfunctional elimination syndrome are all terms that describe the com-
mon array of symptoms that include overactive bladder syndrome, voiding
x Contents

postponement, stress incontinence, giggle incontinence, and dysfunc-

tional voiding in children. This article discusses the nomenclature and
looks at the pathophysiology of functional bladder disorders from a differ-
ent perspective than has been the norm in the past. Some standard med-
ical treatments as well as some newer forms of treatment are outlined.
Treatment algorithms for urinary frequency and urinary incontinence
have been created to help the practitioner manage the patient.

Current Options in the Management of Primary Vesicoureteral Reflux in Children 819

Fernando F. Fonseca, Fabio Y. Tanno, and Hiep T. Nguyen
The management of vesicoureteral reflux (VUR) is varied and remains con-
troversial, which is likely because children with VUR have different risks for
urinary tract infection or renal injury. Consequently, the treatment of VUR
needs to be individualized based on the patient’s characteristics. In this
article, the authors review the medical and surgical management options
for VUR in the pediatric population. The authors hope to provide a system-
atic approach to determine which treatment is optimal for a specific patient.

Management of Primary Vesicoureteral Reflux in Children: Editorial Commentary 835

Ron Keren
Decisions are needed about the clinical management of children with
vesicoureteral reflux (VUR) and a standardized risk-specific treatment
approach is needed. Clinicians and researchers must begin to think crea-
tively about VUR. Clinicians are beginning to understand that there are
a variety of abnormalities in specific host defenses that might predispose
some children to recurrent urinary tract infection, and that knowledge of
these deficiencies may lead to therapies designed to compensate for
them. There is also much to be learned about host inflammatory response
to kidney infection.

Hydronephrosis: A View from the Inside 839

Hrair-George O. Mesrobian and Shama P. Mirza
Unilateral ureteropelvic junction obstruction (UPJO) is the most common
prenatally detected disease leading to hydronephrosis. The obstructive
anatomic lesion leads to varying degrees of hydronephrosis, ranging
from no apparent effect on renal function to atrophy. Furthermore, the
natural course of hydronephrosis varies from spontaneous resolution to
progressive deterioration and may take upwards of 3 years for a kidney
to declare itself. The objectives of this article are to update our knowledge
regarding the evaluation and management of UPJO in depth and to dis-
cuss the emerging value of urinary proteome analysis to the clinical arena.

Update on the Management of Disorders of Sex Development 853

Rodrigo L.P. Romao, Joao L. Pippi Salle, and Diane K. Wherrett
A number of factors have contributed to a sharp increase in the number of
publications related to disorders of sex development (DSD) in the past 5
years, namely: the establishment of a consensus in 2006 about nomencla-
ture, investigations and the need to treat these patients in a multidisciplinary
 Contents xi

setting; increase of the knowledge base about genetic mechanisms of nor-

mal and abnormal sex development; critical appraisal about the timing and
nature of genital surgery in patients with DSD. Herein, the authors present
a comprehensive review with up-to-date data about the approach to the
newborn with ambiguous genitalia as well as the diagnosis and manage-
ment of the most common DSD.

Management of Disorders of Sex Development: Editorial Commentary 871

David E. Sandberg
In 2005, the Lawson Wilkins Pediatric Endocrine Society and the European
Society for Pediatric Endocrinology convened a conference on intersex to
review clinical management practices and data from long-term health-
related and gender-related outcomes research and to identify key areas
for future research. Romao and colleagues provide an overview of the
evolving changes after publication of this guidance, informed by experi-
ences in their multidisciplinary clinic. This commentary highlights and
expands on several of the topics explored, with a special emphasis on
the psychosocial aspects of care for persons affected by disorders of
sex development and their families.

Urolithiasis in Children: Medical Approach 881

Lawrence Copelovitch
Childhood urolithiasis is an evolving condition with an increasing incidence
and prevalence over the last 2 decades. Over that time the underlying
cause has shifted from predominantly infectious to metabolic in nature.
This review describes the pathophysiology, underlying metabolic abnor-
malities, clinical presentation, evaluation, and management of childhood
urolithiasis. A comprehensive metabolic evaluation is essential for all
children with renal calculi, given the high rate of recurrence and the impor-
tance of excluding inherited progressive conditions.

Urolithiasis in Children: Surgical Approach 897

Candace F. Granberg and Linda A. Baker
Over the past 3 decades, minimally invasive stone surgery has completely
overtaken open surgical approaches to upper tract pediatric urolithiasis.
Progressing from least to most minimally invasive, extracorporeal shock
wave lithotripsy, ureteroscopy, and percutaneous nephrolithotomy are
the surgical methods of today for kidney and ureteral stones. The choice
of treatment modality is individualized in children, considering patient
age, stone size, number, location, and anatomic and clinical contributing
factors. The purpose of this article is to review these techniques for pediat-
ric upper urinary tract stones and summarize outcomes and complications.

Issues in Febrile Urinary Tract Infection Management 909

Martin A. Koyle and Donald Shifrin
Urinary tract infections are common occurrences in the pediatric age group
and are a cause of significant morbidity and expense. The understanding of
the consequences and sequelae of febrile urinary tract infections led to
xii Contents

revision of standard protocols initiated by the American Academy of Pedi-

atrics (AAP) in 1999. A less invasive protocol of radiologic evaluation has
been the major outcome of the revised AAP guidelines. Emphasis on pre-
vention of recurrent febrile urinary tract infections has also led to therapeu-
tic programs that are centered less around the use of prophylactic
antibiotics than has previously been the practice.

Evidence Basis for Individualized Evaluation and Less Imaging in Febrile Urinary
Tract Infection: An Editorial Commentary 923
Thomas B. Newman
The past decade has seen a remarkable retreat from previous dogma re-
garding urinary tract infections (UTIs). Less aggressive imaging is now rec-
ommended because although vesicoureteral reflux (VUR) is frequently
found in children with a history of febrile UTIs, most VUR resolves spontane-
ously and we do not have evidence that treatment of the rest improves out-
come. Available evidence suggests urine testing for UTI can be less
aggressive as well, focusing on those with the most risk factors for UTI, those
with the most severe illness, and those at highest risk of complications.

Advances in the Surgical Pediatric Urologic Armamentarium 927

Robert M. Turner II, Janelle A. Fox, and Michael C. Ost
The surgical armamentarium of the pediatric urologist has changed greatly
in the past 2 decades on account of new technology and careful adaptation
of minimally invasive techniques in children. Conventional laparoscopy,
robotic-assisted laparoscopy, laparoendoscopic single-site surgery, and
endourologic surgery have, to varying degrees, provided new approaches
to urologic surgery in the pediatric population. This article reviews the tech-
nology and adaptations behind these recent advances as well as their
current applications in management of urologic disease in children.

Is Bladder Dysfunction in Children Science Fiction of Science Fact: Editorial

Comment 943
Darius J. Bägli
Functional bladder problems in children are often insidious and are fre-
quently ignored by the child, by parents, and by many caregivers. Consid-
eration of both the urinary and bowel outlets, and more recently, of the
corticospinal tracts and brain reveal great complexity in this condition. In
this article, the author addresses many of these issues in depth with
a familiar personal experience derived from many years of dedicated con-
sideration of these problems. Bladder dysfunction in the child is in many
ways the pediatric urologist’s hypertension diagnosis. Like antihyperten-
sive therapy, bladder retraining strategies must be adhered to for life.

Pediatric Urologic Oncology 947

Gwen M. Grimsby and Michael L. Ritchey
This article reviews common pediatric urologic cancers involving the gen-
itourinary system. Rhabdomyosarcoma may occur in the bladder, pros-
tate, paratesticular regions, vagina, or uterus. Some of these locations,
 Contents xiii

such as the paratesticular region, have a more favorable outcome. Benign

neoplasms account for the majority of pediatric testicular tumors and most
are managed with testis-sparing surgery. Most genitourinary malignancies
are expected to have a good outcome. One focus of treatment is organ
preservation but not at the expense of a good oncologic outcome. Late
sequelae of anticancer therapy are a concern and every attempt is made
to decrease the intensity of tumor treatment.

Geintourinary Malignancies in Children: Editorial Comment 961

Armando J. Lorenzo
The modern management of pediatric genitourinary malignancies has
resulted in survival rates that are dramatically better than figures from
just a few decades ago. This is largely due to advances in multimodal
treatment, collaborative efforts, and multidisciplinary management. Never-
theless, issues related to long-term side effects, treatment-related morbid-
ity, and progression or recurrences remain important and pressing in terms
of research directions and areas for improvement. In this Editorial Com-
ment the author attempts to employ the current state of the art, masterfully
summarized in the accompanying review by Drs Grimsby and Ritchey, to
provide a view of trends that are likely to become increasingly important in
the future, highlighting common patterns in treatment philosophy seen in
other areas of oncology: more selective or patient-tailored treatment strat-
egies, refined protocols and —whenever possible— tissue sparing and
minimally invasive surgical interventions.

Pediatric Urological Emergencies 965

Sarah M. Lambert
Although few children are severely ill when evaluated in the pediatric office,
developing the skills to recognize an infant or child who requires hospital-
ization is critical. Some children will require treatment in an emergency de-
partment or direct admission to an inpatient facility, whereas other children
can be managed as outpatients. Determining when an infant requires an in-
patient admission is particularly important because the metabolic reserve is
less abundant in the newborn. Patients with hemodynamic instability must
be emergently addressed. This article outlines the most common urgent
and emergent pediatric urological conditions with the goal to direct initial
evaluation and treatment.

Circumcision Controversies 977

Kirk Pinto
Despite its long history and common practice, circumcision remains a contro-
versial procedure. This article reviews the history of this operation, examines
the controversy that surrounds it, and emphasizes the performing practi-
tioner’s responsibility to the patient and his family in guiding them through
the complicated decision making surrounding newborn circumcision.

Index 987
 Urology for the Pediatrician

Preface: What Pediatricians

Need to Know about Urology

Pasquale Casale, MD Walid A. Farhat, MD, FRCSC

Guest Editors

Over the past two decades, the pediatric urology subspecialty has been revolutionized
with new diagnostic and therapeutics advances. We are honored to be the editors of
this particular issue focusing on pediatric urology for the Pediatrics Clinics of North
America. Although this issue examines some of the important advances that have
been made in the field of pediatric urology, we opted to concentrate more on evolving
pediatric urology problems. In order to provide additional insight into these topics, we
invited some authors to further cover these topics through editorial comments.
Common general pediatric urology pathologies (undescended testicles and hernias
with hypospadias) are covered by Kirsch et al, while Dr Pinto from Texas revisits the histor-
ical topic of circumcision with more current data and evidence-based recommendations.
The routine use of prenatal ultrasound may have had the most significant impact
on pediatric urology practice. Therefore Brock et al from Vanderbilt provides the
pediatricians with an insightful run of all the possible diagnoses, approaches, and
possible dispositions when urological anomalies are suspected. Since hydronephrosis
is one of the most common prenatal ultrasound findings, we dedicated an article by
Dr Mesrobian from Wisconsin to providing an update on how to best manage patients
with an obstruction, highlighted with the most recent advances on the role of noninva-
sive diagnostic studies, such as urinary proteomics.
With the newly published AAP guidelines for the management of infants and chil-
dren with urinary tract infection, it was compulsory to dedicate an article to addressing
this topic from a pediatric urologist’s point of view, presented by Drs Koyle from
Toronto and Shifrin from Seattle, accompanied with an overarching editorial comment
by Tom Newman from San Francisco. The treatment of VUR is better standardized
today and the role of surgery is better defined. Subsequently, Nguyen et al from Boston
provide an evidence-based approach for the management of VUR, while Dr Keren
elaborates further by addressing some of the points that general pediatricians need
to know, focusing on this evolving subject.
Frimberger et al from Oklahoma and Chicago present an update on the manage-
ment of children with neurogenic bladder as well as an update on recent advances

Pediatr Clin N Am 59 (2012) xv–xvi

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0031-3955/12/$ – see front matter Ó 2012 Elsevier Inc. All rights reserved.
xvi Preface

in this area. Israel Franco from New York updates us on the pathophysiology of voiding
dysfunction, coupled with an interesting commentary by Dr Bagli from Toronto.
Urolithiasis in children is on the rise and Dr Copelovitch from Philadelphia wrote
a state-of-the-art article on the medical approach to children with urolithiasis, while
Baker et al delineated the surgical approaches to children with stones, hence im-
proving on the pediatrician’s knowledge to counsel and tailor treatment options for
those children. Dr Ritchey et al from Phoenix provided a review of common and un-
common pediatric urogenital tumors with the current treatment options, while
Dr Lorenzo from Toronto added insights into future surgical options that minimize
morbidity while maintaining and even enhancing excellent survival.
Finally, a topic of intense controversy and uncertainty is the intersex topic, which
was covered by an endocrinologist and a pediatric urologist from Toronto. Romao
et al touched on all the necessary information that pediatricians may need to know
to adequately manage a patient with ambiguous genitalia with a thorough literature
review of advances in the field, while Dr Sandberg from Michigan shed further light
on the behavioral issues that surround patients with intersex.
Although pediatric urologists currently spend more time in the office treating
nonsurgical conditions such as enuresis, voiding dysfunction, reflux, and prenatally
diagnosed hydronephrosis, there have been major leaps in surgical techniques. The
incorporation of new technologies, such laparoscopic approaches and robotic
surgery, has changed the landscape of this subspecialty, hence markedly improving
morbidity while maintaining the excellent outcomes. Ost et al give an overview of all
the surgical innovations and advanced technologies used in the field of pediatric
urology such as minimally invasive and robotic surgery.
We sincerely thank all of the contributors for their hard work and hope that this
issue will help generate interest in this wonderful subspecialty.

Pasquale Casale, MD
Division of Urology
Columbia University
Columbia University Medical Center
Morgan Stanley Children’s Hospital
3959 Broadway, 11th Floor
New York, NY 10032, USA
Walid A. Farhat, MD, FRCSC
University of Toronto
Division of Urology
The Hospital for Sick Children
Toronto, Ontario, Canada M5G 1X8
E-mail addresses:
[email protected] (P. Casale)
[email protected] (W.A. Farhat)
P re n a t a l U l t r a s o u n d a n d
U ro l o g i c a l A n o m a l i e s
Douglass B. Clayton, MD*, John W. Brock III, MD

KEYWORDS
 Ultrasonography  Congenital anomalies  Hydronephrosis
 Urinary bladder neck obstruction  Urethral obstruction

KEY POINTS
 Fetal ultrasound is a routine part of prenatal care in the United States despite limited
evidence of clinical benefit.
 Prenatal ultrasound use is rising in North America and urologic anomalies are among the
most commonly detected findings.
 Hydronephrosis is the most frequently identified fetal urologic abnormality but the severity
and clinical implications of prenatal hydronephrosis can vary greatly. As the severity of hy-
dronephrosis increases so does the risk for clinically significant urinary tract pathology.
 The majority of fetuses with a urologic anomaly can be managed expectantly and only
a small minority of fetuses will require urgent attention.
 Fetuses with suspected lower urinary tract obstruction comprise the group that may need
urgent pediatric urology consultation and may even require fetal intervention.

INTRODUCTION

The performance of ultrasonography during pregnancy in the United States has

become commonplace in the obstetric care of women. In reality, the practice of prenatal
ultrasound exists largely outside the control of pediatricians or pediatric urologists.
Despite the embracement of prenatal screening for organ system anomalies as routine
by the medical community as a whole, the long-term clinical impact of identifying
congenital anomalies prenatally remains undefined for many diagnoses. For some
anomalies, inconsistent correlation between prenatal ultrasound appearance and post-
natal clinical outcome leads to uncertainty in the aggressiveness with which postnatal
evaluation should be pursued. By contrast, select anomalies may benefit from prenatal

Disclosures: Neither author has any financial or commercial relationships to disclose.

Division of Pediatric Urology, Vanderbilt University, 4102 Doctor’s Office Tower, 2200 Children’s
Way, Nashville, TN 37232, USA
* Corresponding author.
E-mail address: [email protected]

Pediatr Clin N Am 59 (2012) 739–756

doi:10.1016/j.pcl.2012.05.003 pediatric.theclinics.com
0031-3955/12/$ – see front matter Ó 2012 Elsevier Inc. All rights reserved.
740 Clayton & Brock

diagnosis by allowing for prompt and immediate tertiary care after birth or by providing
the opportunity for fetal intervention before delivery.
For practicing pediatricians, a common clinical scenario likely exists. A newborn
with a prenatally diagnosed urologic finding is now under the care of a pediatrician
in the newborn nursery. Several questions likely come to mind. How much information
was obtained about this anomaly before delivery? Should more or less information
have been acquired before birth? Did the parents receive counseling from a pediatric
urologist during gestation? How should this anomaly classified? Is it mild or is it
severe? What are the next steps in the care of the neonate?
The severity of congenital urologic anomalies can be highly variable. In some chil-
dren, the correct diagnosis and subsequent course of action is clear from the start,
yet in other patients, such decisions may be less obvious. This review hopes to provide
a clear reference for pediatricians as they see newborn babies with prenatally diag-
nosed urologic issues in their practice. The complexities of the postnatal evaluation
in neonates with a urologic anomaly, specifically PNH, are beyond the scope of this
article and are not addressed.

ULTRASOUND USE IN NORTH AMERICA

Few medical technologies have had such rapid incorporation into the care of patients as
has prenatal ultrasonography. Although some parents incorrectly view the early second-
trimester ultrasound as an opportunity to diagnose fetal gender, its purpose is to screen
for organ system anomalies. Prenatal ultrasound in obstetric care has its temporal roots
in England, where, in the late 1950s, it was used for detecting abdominal masses in
women.1 In the early 1960s, investigators in Glasgow began measuring fetal cephalic
growth in gravid women.2 A 1970 report describing the prenatal diagnosis of polycystic
kidneys was a seminal event in the prenatal identification of organ anomalies.3 The main-
stream incorporation of sonographic fetal anomaly screening in obstetrics occurred as
a result of several clinical trials conducted over the past 30 years.4–7 The ability to prove
the clinical benefit of routine screening for organ anomalies remains elusive. A randomized
trial from Europe compared prenatal ultrasound screening with expectant management
and reported improved fetal survival after prenatal diagnosis of organ anomalies, but this
survival improvement was highly influenced by pregnancy terminations that occurred after
severe anomalies were detected.5 To date, the only randomized trial in the United States
evaluating routine ultrasound screening during pregnancy failed to conclusively demon-
strate that the use of prenatal ultrasound and subsequent prenatal anomaly diagnosis
has a positive impact on clinical outcome.4 Furthermore, 2 separate meta-analyses were
also unable to demonstrate that routine use of ultrasound in low-risk or unselected pregnant
women leads to a reduction in adverse outcomes or provides clinical benefit.8,9
Routine prenatal ultrasound use is on the rise. From 1995 to 2006 in the United States,
the mean number of prenatal sonograms performed per pregnancy reportedly
increased from 1.5 to 2.7. By the year 2006, women with high-risk pregnancies under-
went twice as many studies, having an average of 4.2 ultrasounds per pregnancy.10
Similar US reports document an approximate doubling of prenatal ultrasounds per-
formed over a 7-year period, from 1998 to 2005.11 Likewise, in Canada, a 55% increase
in prenatal ultrasound use was recognized between 1996 and 2006.12

SCREENING SENSITIVITY

The combination of routine prenatal ultrasound and improving ultrasound technology

ensures that more organ anomalies are detected before birth. Perhaps the best avail-
able data regarding the frequency of organ system anomalies come from 2 separate
 Prenatal Ultrasound and Urological Anomalies 741

large-scale ultrasound screening studies, the Eurofetus study and the EuroScan
study, performed in obstetric centers throughout Europe in the 1990s.13,14 In both
studies, approximately 2% of all pregnancies were affected by a congenital anomaly.
The frequency of detecting anomalies on prenatal ultrasound is heavily contingent on
the organ system studied and the experience of the center performing the study.15 In
the Eurofetus study, a second-trimester screening ultrasound detected 61% of post-
natally confirmed anomalies before birth, with 44% detected before 24 weeks.13 By
contrast, in the only randomized trial of prenatal ultrasound screening ever performed
in the United States, the Routine Antenatal Diagnostic Imaging with Ultrasound trial,
the sensitivity for detecting organ system anomalies before 24 weeks of gestation
was only 16% and was 35% irrespective of gestational age.4 The sensitivity for detect-
ing urologic anomalies before birth seems universally high.16 Table 1 shows the rela-
tive distribution of anomalies by organ system identified at birth in the Eurofetus
study.17 Of the 954 urogenital anomalies detected in Eurofetus, 88.5% were identified
prenatally. By contrast, heart and great vessel anomalies were identified only 27% of
the time.17

NORMAL FETAL URINARY TRACT APPEARANCE

Reviewing the normal appearance of the fetal urinary tract is a prerequisite to discus-
sing congenital anomalies of genitourinary system. Fig. 1 depicts the normal ultra-
sound appearance of the fetal kidney and bladder on prenatal ultrasound. The
healthy fetal kidney is typically not visualized on a transabdominal ultrasound until
at least week 15 of gestation.18 Fetal renal length varies during development (see
Fig. 1).19 The anterior posterior diameter (APD) of the fetal renal pelvis is a measure-
ment that has been increasingly used to categorize fetal renal dilatation as normal or
abnormal. Fetal renal APD measurements less than 4 mm in the second trimester and
less than 7 mm in the third trimester are considered physiologic levels of fetal renal
dilatation.20 In the normal fetus, the ureters are not visible on prenatal ultrasound
whereas normal ureteral diameter in neonates is reported to be 5 mm or less.21 The
fetal bladder is visible on transvaginal ultrasound in 87% of cases by 12 weeks of
gestation and care should be taken to ensure the bladder is identified during the
second-trimester screening ultrasound.22 If the bladder is not visualized, repeat
imaging later in the study or on a subsequent repeat ultrasound should confirm its
presence or absence. Bladder enlargement, also termed megacystis may be noted
on prenatal ultrasound and can suggest urinary tract obstruction. Measurements
that define a normal fetal bladder size have not been concretely defined. From weeks
10 to 14, suggested normal parameters for bladder size include either a longitudinal

Table 1
Most common congenital organ anomalies by system

Total Postnatal Prenatal Ultrasound

System Number Frequency (%) Sensitivity (%)
Musculoskeletal anomalies 1043 23 36
Heart and great vessels 953 21 27
Urinary tract abnormalities 954 21 88
Central nervous system 738 16 88

Data from Grandjean H, Larroque D, Levi S. Sensitivity of routine ultrasound screening of pregnan-
cies in the Eurofetus database. The Eurofetus Team. Ann N Y Acad Sci 1998;847:118–24.
742 Clayton & Brock

Fig. 1. Normal fetal urinary tract appearance and normal fetal renal length (see chart). (A)
Appearance of healthy right kidney on a 25-week ultrasound. Renal length measures 3.24
cm. (B) Appearance of the normal fetal bladder in a healthy 21-week male fetus. (Data
from Cohen HL, Cooper J, Eisenberg P, et al. Normal length of fetal kidneys: sonographic
study in 397 obstetric patients. AJR Am J Roentgenol 1991;157(3):545–8.)

bladder diameter of less than 6 mm or a diameter measuring less than 10% of crown-
rump length.23,24 Normal bladder size in the second and third trimesters remains
undefined. A normal bladder in the second trimester has been characterized subjec-
tively as one of small size that empties during a 45-minute time frame.25 Amniotic fluid
levels can be a surrogate marker for fetal urinary tract function. Although fetal urine
production begins by 8 to 10 weeks of gestation, it is only beyond 16 weeks of devel-
opment that the amniotic fluid is primarily composed of fetal urine.18,26 Thus, abnor-
malities in the amniotic fluid levels in the second and third trimesters may be
harbingers of urinary tract problems.

PRENATAL UROLOGIC ANOMALIES

A variety of urologic diagnoses may be detected before birth. The certainty of prenatal
suspicion, however, can only be confirmed with accurate postnatal evaluation and
diagnosis. A follow-up report from the EuroScan study detailed the diagnoses in
1130 patients with urologic anomalies diagnosed from a population of 709,030
births.27 Table 2 lists the most common diagnoses in the 609 patients with isolated
urologic anomalies and the percentage of each diagnosis that were detected
prenatally.27
 Prenatal Ultrasound and Urological Anomalies 743

Table 2
Most common congenital urologic anomalies (n 5 609)

Anomaly Percentage of Total Percentage Detected Prenatally

All anomalies (n 5 609) — 82
Hydronephrosis 51 84
Multicystic dysplastic kidney 17 97
Unilateral renal agenesis 10 62
Duplicated kidney 6 95
Renal ectopia 4 56
Posterior urethral valves 4 70
Solitary renal cyst 4 76
Bladder exstrophy 3 53

Data from Wiesel A, Queisser-Luft A, Clementi M, et al. Prenatal detection of congenital renal
malformations by fetal ultrasonographic examination: an analysis of 709,030 births in 12 European
countries. Eur J Med Genet 2005;48(2):131–44.

The gestational age at which urologic anomalies are first identified is of variable
importance. Almost universally, the presence of oligohydramnios in the second
trimester is a poor prognostic sign for fetal survival.28–31 Fetal megacystis may be
seen as early as the first trimester, and spontaneous resolution is reported common
in fetuses with longitudinal diameters less than 12 mm.23 In the second and third
trimesters, megacystis alone has little predictive value. Rather, the clinical picture of
the fetus, from a urologic perspective, should be derived from the ultrasound appear-
ance of the entire urinary tract and the amniotic fluid level, not simply the size of the
bladder. The importance of timing in the initial identification of PNH (ie, second-
trimester detection vs third-trimester detection) and its correlation with true urinary
tract pathology is uncertain. With the majority of screening ultrasounds occurring
during the second trimester, women with a normal second-trimester ultrasound are
unlikely to undergo a repeat ultrasound in the third trimester. A few studies, however,
have shown that PNH severity on a third trimester scan may be more predictive of clin-
ical outcome than the appearance on a second-trimester study.20,32
This discussion of specific prenatal urologic diagnoses begins with hydronephrosis.
The remainder of the review focuses on those diagnoses that can be managed expec-
tantly and those that require more urgent attention in the fetal and neonatal period.

Hydronephrosis
PNH is the most commonly identified prenatal urinary tract abnormality. Unfortunately,
hydronephrosis is not actually a diagnosis but rather a sign of some other underlying
problem in the urinary tract. A recent meta-analysis of 17 studies identified 1678
fetuses with PNH of a total screened population of 104, 572 (1.6% prevalence of
PNH). Of these 1678, 36% had identifiable urinary tract pathology on postnatal eval-
uation.33 Several factors make the interpretation of PNH controversial, including the
lack of diagnostic specificity, the variable methods for classifying its severity, and
the inconsistent postnatal clinical outcomes with varying degrees of dilatation, partic-
ularly in children with mild or moderate PNH.

Hydronephrosis Grading
Accurately classifying the degree of upper urinary tract dilatation in the fetus and
neonate can be difficult. Ideally, PNH would be graded using one objective scale
744 Clayton & Brock

that could then accurately predict the risk of true postnatal pathology. Fetuses would
be stratified into prognosis groups accordingly, which help guide the postnatal evalu-
ation of the fetus. Unfortunately, no one system to date conclusively allows for such
determinations. In general, as the degree of PNH increases so does the risk for persis-
tent postnatal pathology.33
As discussed previously, the APD of the renal pelvis is a commonly used method for
defining PNH. For proper APD calculations, measurements should be obtained from
a transverse axial image of the renal pelvis at approximately the level of the renal
hilum.20 Renal APD measurements can vary depending the gestational age of the
fetus, and the thresholds for concern must change as well. An early report
from George Washington University used renal APD threshold values of 4 mm before
33 weeks and 7 mm after 33 weeks to define hydronephrosis. The study found both of
these gestational age–based APD thresholds nearly 100% sensitive for detecting PNH
but poorly specific for predicting both the postnatal persistence of hydronephrosis
and the need for postnatal surgery.20 Repeat analysis of the data in a separate report
identified APD measurements greater than 15 mm at any time during gestation as
a crucial indicator of severe PNH and correlated with a real risk for postnatal
obstructive pathology.34 The importance of 15 mm of APD has been affirmed by other
series and is a measurement that can be used in practice to identify children with
severe PNH that should certainly have prompt follow-up with a urologist soon after
birth.35,36 Fig. 2 depicts the appropriate measurement of fetal renal APD and a subse-
quent classification scheme proposed in a recent consensus statement on PNH pub-
lished by the Society for Fetal Urology (SFU). The classification system is based on
renal APD measurements in the second and third trimesters.36
Both prenatally and postnatally, the severity of hydronephrosis is often character-
ized using subjective descriptors that include terms, such as pelviectasis, caliectasis,
pelvocaliectasis, mild hydronephrosis, moderate hydronephrosis, and severe hydro-
nephrosis. A more objective method for postnatal grading of hydronephrosis was
published by the SFU in 1993.37 The goal of the SFU 5-point classification scheme

Fig. 2. Fetal renal APD and hydronephrosis grading. (A) Measurement of the renal APD in
the transverse axial plane in a 38-week fetus with bilateral hydronephrosis. Measurement
(A) in the right kidney demonstrates a renal APD of 15 mm and measurement (B) in the left
kidney is 9.4 mm. The table represents a classification scheme for grading hydronephrosis.
(Data from Nguyen HT, Herndon CD, Cooper C, et al. The Society for Fetal Urology consensus
statement on the evaluation and management of antenatal hydronephrosis. J Pediatr Urol
2010;6(3):212–31.)