Pediatric

Laksbmana Fuicher, MD Das

Case
Narla, MD

of the
#{149}

Day1
Doherty, MD
#{149} Elizabeth

R. Donald

A. Hingsbergen,

MD

#{149} Ann

S.

Figures 1, 2. (1) Supine abdominal radiograph urethrograms show urachal diverticulum (arrows vere reflux into markedly dilated ureters (arrows

bulging in a), scaphoid in C).

shows

abdominal flanks. (2) Voiding cystomegalourethra (arrows in b), and se-

. HISTORY A i-day-old male infant ultrasound examination

Index terms: Abdomen,
1998;

in whom

an

antenatal

had

shown

dilated

ure-

ters and a distended bladder underwent tion. Abdominal radiography and voiding urethrography were performed.

evaluacysto-

diseases,
18:1318-1322

80.1444

#{149}

Children,

genitourinary

system,

80.1444

#{149}

Prune-belly

syndrome,

80.1444

RadIoGiaphlcs From Richmond, the

Department VA 23298-0615.

of Radiology, From the 27. Address

Medical 1997 requests

College RSNA

of Virginia assembly.

Hospitals, Received

Virginia March

Commonwealth 30, 1998;

University, revision requested

Room May

3-415, 5 and

401

N 12th

St, May

scientific to L.D.N.

received

26; accepted
#{176}RSNA,1998

May

reprint

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Figure
a prostatic

4.

Voiding
utricle

cystourethrogram
(arrows).

demonstrates

Figure
a widely

3.

Voiding

urethra
urethra.

patent (arrows),

cystourethrogram demonstrates bladder neck, a dilated posterior and tapering at the membranous

less

pronounced prevalence

in females of the

than

in males. has

An

increased

syndrome

been

reported

in Nigeria

as well

as in Sasof prune-belly
Three possible outlet ob-

katchewan, Canada (5). The cause and embryogenesis

FINDINGS Supine abdominal radiography showed bulging abdominal flanks secondary to markedly dilated and tortuous ureters and absence of abdorninal musculature (Fig 1). Voiding cystourethrography demonstrated urachal diverticulurn, scaphoid rnegalourethra, and severe reflux into the markedly dilated ureters (Fig 2). DIAGNOSIS: Barrett syndrome, Prune-belly
triad

syndrome causes have

remain been

controversial. proposed:

bladder

struction, mesodermal arrest, and dysgenesis of the yolk sac. In 1903, Strumme proposed in utero bladder outlet obstruction as the cause of prune-belly
syndrome and postulated that the abdominal

syndrome
syndrome).

(Eagle-

DISCUSSION Prune-belly syndrome is classically defmed as the triad of partial or complete absence or hypoplasia of the abdominal musculature, bilatera! cryptorchidism, and a variety of urinary tract abnormalities. Abdominal wall tumidity was noted by Frohlich in 1839 (1), who also noted the associated urinary tract abnorrnalities. Osler (2) is credited with giving the syndrome the name prune-belly. Eagle and Barrett (3) were the first to describe the triad

wall abnormalities were secondary to pressure effects of the distended bladder on the developing myotornes of the abdominal wall (6). Subsequent clinical reports noted an obstructing lesion in only iO%-20% of cases compared with up to 80% of cases reported in earlier Series (7,8). The cause of the obstruction may be

areas of stenosis, atresia, posterior valves, or a pinpoint diaphragm at of the posterior and membranous A second theory postulates the prune-belly syndrome as mesodermal curing between the 6th and 10th
gestation. In patients with prune-belly

urethral the junction urethra. cause of arrest ocweeks of

syn-

drome,
sparsely

an abundance
placed smooth

of fibrous
muscle

tissue
throughout

with
the

as a syndrome.

Prune-belly 29,000-40,000
cryptorchidism

syndrome occurs live births. The

effectively

in one criterion
the

of every of
synprunein fe-

eliminates

drome
belly

in female
is also

patients,

but
of the

the

term

urinary tract is more indicative of an inherent problem with mesoderm differentiation than of obstruction. This theory also provides an explanation for the frequent association of the syndrome with megalourethra (9).

descriptive

disease

males, ported

who constitute about 3%-5% of recases (4). Urinary tract involvement

is

September-October

1998

Das

Narla

et al

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#{149} 1319

The yolk sac theory proposes dysgenesis of the embryonic yolk sac and allantois as the cause of prune-belly syndrome and may explain some of the changes seen in the bladder and prostatic urethra. However, this theory does not provide an explanation for the abnormalities of the upper urinary tract or testes (10).
Autopsy studies have shown that the mus-

plains

the

common

association

with

vesico-

ureteral reflux. The bladder neck is terior urethra is dilated there is tapering at the (Fig 3). The verurnontanurn True obstructive lesions prostatic and mernbranous 20% of infants, usually
sis. Lack of development

widely
and

patent,
elongated,

the
and

pos-

rnembranous urethra is small or absent. at the junction of the urethra are seen in those with poor prognoof the epithelial por-

des most severely affected are located yentrally and laterally. The epaxial and hypaxial trunk muscles develop normally. Affected muscles in decreasing order of severity include the transverse muscle of the abdomen, the rectus abdominis muscle below the umbilicus, the internal and external oblique muscles of the abdomen, and the rectus abdominis muscle above the umbilicus. The renal dysmorphism in prune-belly syndrome is due to a combination of a ureteric bud and a metanephric defect. The Berdon
classification of genitourinary involvement in-

cludes Potter syndrome (oligohydramnios) (group 1), severe neonatal and infantile involvernent (group 2), and mild involvement (group 3) (1 1). The degree of hydronephrosis may not correlate with the abdominal wall deficiency. The renal parenchyma is often well
preserved.

tion of the prostate gland is characteristic of the syndrome, and prostatic hypoplasia is one of the causes of infertility (14). The prostatic utricle is a small, epitheliumlined diverticulum of the prostatic urethra (Fig 4). It is a normal anatomic variant representing the remnant of the fused caudal ends of the m#{252}llerian ducts and is homologous to the female vagina and cervix. There is an increased prevalence of a large utricle in high-type imperforate anus, Down syndrome, hypospadias, posterior urethral valves, and prune-belly syndrorne. Abnormalities of the anterior urethra in
prune-belly syndrome range from urethral atre-

sia to fusiform rnegalourethra. Bulbous urethral dilation is evident in nearly 70% of patients. A transient obstruction in the glandular and penile urethra during fetal development has been
suggested as the cause for the classic radio-

The hallmark of prune-belly syndrome is the elongated, tortuous, dilated appearance of the ureters at radiography (i2). The lower onethird of the ureter is more profoundly affected than the proximal portion. Vesicoureteral reflux is seen in over 70% of patients. Histologic analysis shows a diffuse increase in connective tissue with replacement of the smooth musdes. A marked decrease in the number of
nerves and degeneration of nonmyelinated

graphic appearance of the ureters as well as for the known association of prune-belly syndrome with rnegalourethra. Both scaphoid and fusiform megalourethra are associated with the syndrome (Fig 5) (15). Fusiform rnegalourethra, accompanied by deficient corpora cavernosa, is a more severe defect frequently associ-

ated
alies. by

with

renal

dysplasia
rnegalourethra of the and

and
corpus

other

lethal

anornized with

Scaphoid a deficiency glans

is characterspongiosurn fossa.

Schwann fibers have been reported (13). This may explain the poor ureteral peristalsis even after corrective surgery. The bladder is thick-walled, enlarged, and free of trabeculations in prune-belly syndrome. The increased wall thickness is largely due to the presence of fibrocytes and collagen. The bladder capacity ranges from 600 to 800 mL with a voiding pressure within normal range for age. A patent urachus may be present. The ureteric orifices are located laterally, which ex-

a normal

navicular

Bilateral cryptorchidism is an essential cornponent of prune-belly syndrome. The intraabdorninal position of the testes predisposes
these patients to an increased risk for malig-

nant than
testes.

degeneration that in patients

To our has been

that is 30-50 with normal

no reported

times greater descended

with this a child.

knowledge,

patient to father

syndrome

Early orchidopexy is advocated to optimize tential spermatogenesis and improve evaluation for possible malignancy.

po-

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infection involving with prune-belly

tions include vesicostomy,

the urinary tract in children syndrome. These intervenurethrotomy, cystoplasty, cutaneous and total

internal reduction

urinary reconstruction (reduction cystoplasty, bilateral ureteral tapering, and reirnplantation with resection of distal ureter). Patients undergo orchidopexy by 2 years of age. Progressive renal from renal dysplasia, recurrent pyelonephritis
transplantation. nor fully, months kidneys with to of clean have age 21 years. intermittent Both been

insufficiency resulting reflux nephropathy, may necessitate

cadaveric transplanted Bilateral and living successranging nephroureterec-

and renal
dofrom 8

at transplantation

tomy
use

is performed

prior

to transplantation.
catheterization to

The

empty the decompensated traindication for renal antibiotic prophylaxis

transplant recipients.

bladder is not a contransplantation. Ongoing should be given to all

Figure 5. Illustration shows fusiform (arrows A) and scaphoid (arrowheads in B) megalourethra associated with prune-belly syndrome.

in

The treatment of prune-belly syndrome has become refined as a result of significant improvernents in antibiotic therapy, diagnostic assessment of the urinary tract, and understand-

Respiratory

manifestations

in prune-belly

ing of the aggressive adenopulis advocated

natural history of the disease. treatment of the ureters and

in the majority of patients

Less bladder
with

syndrome
rnatoid monary

include
malformation. problems

hypoplasia
Clinically are noted anomalies

and
in 55% are

cystic

significant of patients. seen

normal renal plasty

months

renal

function.

Infants

with

adequate abdorninoat 12-24

require

Gastrointestinal

in 30%

function should and orchidopexy

of age. All

benefit from performed

of these patients

of patients
stenosis, torsion, sis.

and
volvulus,

include

malrotation,
anus, and disease,

atresia,
splenic gastroschi-

irnperforate

Hirschsprung

careful lifelong urologic surveillance. Although it is generally reported that approximately 20% of patients with prune-belly
syndrome die within 1 month and another 30%

Cardiovascular anomalies have been reported in up to 10% of patients and include ventricular septal defect, patent ductus artenosus, and tetralogy of Fallot. of patients had

die within 2 years, surviving patients have varying degrees of renal abnormalities that are often compatible with extended life spans. Patients with prune-belly syndrome who live

In one series, 50% more musculoskeletal

scoliosis, pectus

anomalies hemimelia,

one including dislocation

or

deformity,

arthrogryposis,

more than 70 years have been reported. The causes of death in patients with of is
belly urosepsis. syndrome Now are that chronic patients renal are failure living

pruneand longer,

clubfoot, the
not

valgus

foot,

hip, Fetal

sacral agenesis, and polydactyly (16). intervention in prune-belly syndrome

except in rare instances of dys-

respiratory
cause

failure clinical

has

become in this

a contributing case is not

warranted

of death.

tocia may

secondary

to fetal
at the time

bladder
of labor

distention.
and delivery

De-

compression

The known.

course

be necessary. A variety of surgical performed to correct

interventions poor drainage,

have stasis,

been and

September-October

1998

Das

Narla

et al

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#{149} 1321

U REFERENCES 1 . Burke EC, Shin MH, Kelalis PP. Prune-belly syndrome: clinical fmdings and survival. Am J Dis
2. Child 1969; 117:668-671. Osler W. Congenital absence musculature with distended of abdominal and hypertro-

8.

9.

Tank ES, McCoy G. Limited surgical intervention in the prune-belly syndrome. J Pediatr Surg 1983; 18:688-691. Kroovand RL, Al-Ansari RM, Perlmutter AD.

Urethral
belly

and genital
syndrome. J Urol

malformations
1982;

in prune

127:94-96.

pined
3.

urinary

bladder.

Bull Johns

Hopkins

10.

Hosp 1901; 12:331-333. Eagle JF, Barrett GS. Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: syndrome report of nine

Stephens congenital

FD. Triad (prune belly syndrome) in malformation of the urinary tract.

485-511.

New York, NY: Praeger, 1983; 1 1 . Berdon WR, Baker DH, Wigger The radiologic and pathologic

cases. 4.

Pediatrics

1950;

6:721-736.
JF. Prune belly syn12.

prune

belly

syndrome.

Radiol

HJ, Blanc WA. spectrum of the Clin North Am

belly syndrome. JT, Howard 55,

Rabinowitz

R, Schillinger

5.

6.

drome in the female subject. J Urol 1977; 118: 454-456. Adeyokunu AA, Familus JB. Prune-belly syndrome in two siblings and a first cousin: possible genetic implications. Am J Dis Child 1982; 136:23-25. Greskovich FJ, Nyberg LM. The prune-belly syndrome: a review of its etiology, defects, treatment and prognosis. J Urol 1988; 140:
707-712.

1977; 15:83-92. Snow BS, Duckett JW. Prune In: Gillenwater JY, Grayhack

Duckett
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JW. Adult

and pediatric

radiology

1977. Year Book Medical, 1977; 1709-1725. Ehrlich RM, Brown WJ. Ultrastructural ana-

14. 15.

tomic observations of the ureter in the prunebelly syndrome. Birth Def 1977; 13:101-103. Hinman F. Alternatives to orchiopexy. J Urol
1980; Shorom 123:548-551. SH, Cromie WJ, Duckett JW. Mega-

7. Lattimer

JK. Congenital deficiency of abdominal musculature and associated genitourinary anomalies. J Urol 1958; 79:343-352.

16.

lourethra. Urology Loder RT, Guiboux RN. Musculoskeletal

syndrome: Dis Child

1981; 17:152-156. JP, Bloom DA, Hensinger aspects of prune-belly

pathogenesis. Am J

description and 1992; 1224-1229.

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