index of suspicion in the nursery
Unusual Cause of Feeding Intolerance
The reader is encouraged to write
possible diagnoses for each case
before turning to the discussion.
We invite readers to contribute
case presentations and discussions.
Please inquire first by contacting
Dr. Philip at aphilip@stanford.edu.
Author Disclosure
Dr Abdelhamid has disclosed no
financial relationships relevant to this
article. This commentary does contain
a discussion of an unapproved/
investigative use of a commercial
product/device.
e732 NeoReviews Vol.13 No.12 December 2012
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in a Term Infant
Case Presentation
A 39-5/7 weeks gestation infant boy
weighing 3.670 kg is born by emer-
gency caesarian section for signicant
fetal distress. The mother is a 37-year-
old primigravida whose pregnancy is
uneventful except for oligohydramnios
due to leaky membranes.
Liquor is scanty, meconium stained
and has an offensive smell. The infant
is born in poor condition, needs re-
suscitation, and moves to the neonatal
unit ventilated and passively cooled.
Apgar scores are 1, 4, and 6 at 1, 5,
and 10 minutes, respectively. Cord
blood pH is 6.8, and base excess is
18 mmol/L. On admission, tem-
perature is 38.3C, and the infant,
covered with thick meconium, is hy-
pertonic, tachycardiac, breathing with
difculty, not synchronizing with ven-
tilator, and having a bilaterally dis-
tended chest, diminished air entry to
both lung elds, mufed heart sounds,
and a pre- and postductal O2 satura-
tion difference of 10% to 15%.
After two boluses of normal saline,
and initiating active whole-body cool-
ing, sedation, muscle relaxation, intra-
venous antibiotics, maintenance uids,
inhaled nitric oxide, and cerebral
function monitoring, umbilical lines
are placed. Blood tests conrm both
sepsis and severe hypoxic-ischemic
injury.
Chest radiograph reveals abnor-
mal bell-shaped chest, small lung vol-
ume, and bilateral pneumothoraces,
more on the right hemithorax, neces-
sitating chest drain insertion.
A urinary catheter is placed. Urine
output (UOP), noticed to be low
during the rst 36 hours, starts to re-
cover. He is rewarmed after 72 hours
of cooling, the urinary catheter is
pulled out, and antibiotics are stopped
by day 5.
Ventilation remains difcult to wean,
and pneumothoraces keep reaccumulat-
ing. Ultimately, he is extubated to air,
and chest drains are removed, by day 14.
Once the urinary catheter is re-
moved, the infants UOP signi-
cantly drops, which is thought to
be related to urinary bladder atony
complicating the hypoxic-ischemic
injury. Another urinary catheter is
placed, and UOP starts to normalize
between 3 and 5 mL/kg per hour.
Feeding starts by day 4 but is not
tolerated with increasingly bile-stained
gastric aspirate and vomiting.
Case Discussion
Diagnosis and Course
Repeat investigations reveal a nor-
mal complete blood cell count, a se-
rum sodium of 131 mEq/L (131
mmol/L); potassium of 5.3 mEq/L
(5.3 mmol/L); calcium of 8.12 mg/
dL (2.03 mmol/L); phosphorus of
8.70 mg/dL (3.00 mmol/L); blood
urea nitrogen of 33 mg/dL (11.8
mmol/L); and creatinine of 1.4 mg/
dL (123.9 mmol/L).
The urinary catheter is pulled out
again by day 7, but UOP starts to
drop, and bladder starts to distend.
Abdominal examination reveals a dis-
tended bladder and a para-midline
right-sided mass. A plain abdominal
radiograph reveals gas paucity on the
right side and absence of rectal gas.
The surgical team diagnoses mid-
gut malrotation and arranges for a
contrast study that reveals no bowel
distension and relative absence of in-
testinal gases on the right side as well
as distended bladder (Fig 1).
Abdominal ultrasound scan rules
out malrotation but detects a mass dis-
placing and compressing the rst and
second parts of the duodenum (Fig 2).
 index of suspicion in the nursery

valves are followed by an ultrasound

that reveals resolution of the previ-
ously reported right-sided urinoma
but persistent pelvic dilatation. Renal
dimercaptosuccinic acid (DMSA) scan
(Fig 4) reveals asymmetric renal size
and function.

Figure 1. Contrast study: no bowel distension, but an inferior soft tissue mass
consistent with a distended bladder and a relative absence of intestinal gases in the
right side of the abdomen.
A micturating cystourethrogram re-
veals a distended bladder that has
a smooth outline, with reux into the
right tortuous dilated ureter, in addition
to pelvicalyceal blunting, contrast ex-
travasation into the mass, consistent
with an urinoma, and a markedly dis-
tended posterior urethra with narrowed
anterior urethra consistent with poste-
rior urethral valves (PUVs) (Fig 3).
Initial resection, repeat cystoscopy,
and resection of small remnants of
Condition
PUV is a signicant cause of renal mor-
bidity, with an incidence of 1 in 8,000
to 25,000 live births. The congura-
tion of the obstructive membrane
within the urethra is responsible for
the varied severity and degree of ob-
struction associated with the anomaly.
Type I, the most common type,
represents a ridge lying on the oor
of the urethra that takes an anterior
course and divides into two forklike
processes in the region of the bulbo-
membranous junction. Type II valve
arises from the verumontanum (an
elevation in the oor of the pros-
tatic portion of the urethra where
the seminal ducts enter) and extends
along the posterior urethral wall to-
ward the bladder neck. Type II

Figure 2. Abdomino-pelvic ultrasound scan: the duodenal cap and the duodeno-jejunal flexure are at different levels, with the
flexure lying well to the left of the midline; appearances probably due to a large right-side mass, most probably urinoma, displacing
and compressing the first and second parts of the duodenum rather than being due to malrotation.

NeoReviews Vol.13 No.12 December 2012 e733

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index of suspicion in the nursery

Figure 3. Micturating cystourethrogram: markedly distended posterior urethra with narrowed anterior urethra consistent with
posterior urethral valves.

(no longer referred to as valves)
represents nonobstructive folds prob-
ably resulting from hypertrophy of
muscles of the supercial trigone
and prostatic urethra in response
to high voiding pressure from distal
obstruction.
The less common Type III repre-
sents a membrane lying transversely
across the urethra with a small perfo-
ration near its center. The membrane
is distal to the verumontanum and
sometimes is elongated, reaching
the bulbous urethra.
PUV is usually seen as a multifac-
tor gene-mediated embryopathy in-
volving several genes and inheritance
patterns. The timing of valve develop-
ment appears to be during or after the
8th week of gestation where the pros-
tatic urethra has developed from the
urogenital sinus. The precise anatomy
and embryology of PUV remain
undened.
Pathophysiology
Defective urination in utero leads to
oligohydramnios that results in pul-
monary hypoplasia.
Proximal urethra, prostate, blad-
der neck, bladder, ureters, and kid-
neys are all affected and undergo
various forms and degrees of damage.
Renal dysplasia, found in approxi-
mately 60% of prenatally diagnosed in-
fants with PUV, usually leaves
permanent renal damage, in contrast
to that resulting from obstructive
uropathy, which is usually reversible
and improves with initial treatment.
Progressive tubular injury leading
sometimes to nephrogenic diabetes
insipidus may also occur. Bladder
problems of poor sensation, hyper-
contractility, low compliance, and
eventual myogenic failure all may
contribute to incontinence and poor
emptying. Ureteric problem due to
poor contractility and inability to
co-apt and transport urine may im-
prove initially, but most patients
end having chronic hydronephrosis.
Diagnosis
ANTENATAL. Typical prenatal nd-
ings comprise bilateral hydrouretero-
nephrosis, distended bladder, and
dilated prostatic urethra. Discrete fo-
cal cysts in renal parenchyma are di-
agnostic of renal dysplasia.
Fetal urinary biochemical assess-
ment of renal function, electrolytes,
and b2-microglobulin, provides the

Figure 4. Renal dimercaptosuccinic acid scan revealing asymmetric renal size and function, normal left renal outline with no focal
parenchymal defects, and smaller right kidney with a smooth outline. Divided renal function is left 72% and right 28%.

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most accurate results. Elevated fetal
urine electrolytes and b2-microglobulin
levels are indications of irreversible
renal dysfunction.
CLINICAL PRESENTATION. Neo-
nates with PUV may present with re-
spiratory distress at birth due to
pulmonary hypoplasia. Other nd-
ings include delayed voiding or poor
urinary stream, abdominal mass or
abdominal distension (urinary asci-
tes), failure to thrive, poor feeding,
lethargy, urosepsis, palpable bladder,
Potter facies, and limb deformities
and indentation of the knees and el-
bows due to in-utero compression.
RENAL AND BLADDER ULTRASO-
NOGRAPHY. These may reveal
a thickened bladder wall with dilated
and elongated posterior urethra, vary-
ing degrees of unilateral or bilateral hy-
dronephrosis, perirenal collection of
urine, echogenic kidneys, and subcor-
tical cyst. Perineal ultrasongraphy may
conrm posterior-uretheral dilation and
visualize the valve leaets.
VOIDING CYSTOURETHROGRAPHY.
Diagnosing PUV is founded on
thickened trabeculated bladder, with
dilatation and elongation of the pos-
terior urethra; circumferential lling
defect at the level of the pelvic oor;
and prominent bladder neck and
vesicoureteric reux.
RENAL SCINTIGRAPHY. Functional
imaging of the upper urinary tract is
usually postponed to 4 or 6 weeks af-
ter birth to allow some maturation of
the developing kidneys.
Radionuclide studies are useful in
cases of thin or abnormal paren-
chyma of either kidney, and in those
with a postoperative ultrasound scan
revealing no improvement to help
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differentiate between persistent ob-
struction and cystic dysplasia.
A dynamic study with a tubular
tracer that is taken up by the kidney
and excreted into the urine is in
use. The parameters that can be esti-
mated include differential renal func-
tion and drainage, which can also be
assessed by static scans such as the
DMSA scan.
URODYNAMIC STUDIES. These of-
fer information about bladder storage
and emptying ability, directing the
type of bladder management espe-
cially after valve ablation.
LABORATORY INVESTIGATIONS.
Analyses include electrolytes, urea, cre-
atinine, and urine for microscopy, cul-
ture, and sensitivity.
Surgical Treatment
ANTENATAL. Earlier intervention
would theoretically improve postnatal
renal function. However, being associ-
ated with signicant morbidity,
identifying infants who may benet
from early intervention is not currently
possible. Three procedures are used:
vesicoamniotic shunting, vesicostomy,
and fetal endoscopic valve ablation.
POSTNATAL. PUV requires active
management to avoid progressive re-
nal dysfunction. The bladder behav-
ior and its subsequent management
following valve ablation may inu-
ence the long-term renal outcome
in these patients. Procedures cur-
rently in use to treat PUV include
bladder drainage, endoscopic valve
ablation, and cutaneous vesicostomy.
Lessons for the Clinician
Posterior urethral valve (PUV)
may present in neonates with
pulmonary hypoplasia, abdominal
index of suspicion in the nursery
distension, urinary ascites, difcult
voiding, or poor urinary stream.
Despite greater antenatal recogni-
tion, PUV may not be detected
until after the infant is born.
Antenatal treatment of PUV is dif-
cult to justify; identifying infants
who may benet from intervention
remains elusive.
(Adel Abdelhamid, MB BCh, MSc,
Neonatal Unit, Rosie Hospital, Cam-
bridge University, Cambridge, UK)
American Board of Pediatrics
Neonatal-Perinatal Content
Specifications
Recognize the
clinical
manifestations of
anatomic
abnormalities of
the kidneys and
urinary tract in infants.
Know how to diagnose specific
anatomic abnormalities of the
kidneys and urinary tract in infants.
Suggested Readings
Carr MC, Kim SS. Prenatal management of
urogenital disorders. Urol Clin North
Am. 2010;37(2):149158
Casale AJ. Posterior Urethral Valves. In:
McDouglas WS, Wein AJ, Kavoussi LR,
et al, eds. Campbell-Walsh Urology, 10th ed.
St. Louis, MO: Elsevier; 2011:33893410
Cortes-Osorio B, Concheiro-Guisan A,
Fernandez-Eire P, Vazquez-Castelo
JL. Neonatal ascites and oligohy-
dramnios: the role of kidney. J Matern
Fetal Neonatal Med. 2012;25(9):
18251826
Lee RS, Borer GJ. Perinatal Urology. In:
McDouglas WS, Wein AJ, Kavoussi LR,
et al, eds. Campbell-Walsh Urology, 10th ed.
St. Louis, MO: Elsevier; 2011:30483066
Nasir AA, Ameh EA, Abdur-Rahman LO,
Adeniran JO, Abraham MK. Posterior
urethral valve. World J Pediatr. 2011;7(3):
205-216
NeoReviews Vol.13 No.12 December 2012 e735
Index of Suspicion in the Nursery: Unusual Cause of Feeding Intolerance in a
Term Infant
Adel Abdelhamid
NeoReviews 2012;13;e732
DOI: 10.1542/neo.13-12-e732

Updated Information & including high resolution figures, can be found at:
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Index of Suspicion in the Nursery: Unusual Cause of Feeding Intolerance in a
Term Infant
Adel Abdelhamid
NeoReviews 2012;13;e732
DOI: 10.1542/neo.13-12-e732

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://neoreviews.aappublications.org/content/13/12/e732

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