Journal of Pediatric Urology (2010) 6, 91e93

CASE REPORT

A large extra-abdominal prevesical pseudo-cyst in

a newborn with posterior urethral valves
Mathijs Binkhorst a,*, Robert P.E. de Gier b

a
Department of Neonatology, Radboud University Nijmegen Medical Centre, P.O. Box 1901, 6500 HB,
Nijmegen, The Netherlands
b
Pediatric Urology Centre, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands

Received 29 October 2008; accepted 23 April 2009

Available online 28 May 2009

KEYWORDS Abstract A male newborn is described, in whom a large extra-abdominal prevesical pseudo-
Prevesical cyst; cyst as well as prune-belly features were present, both of which were supposedly secondary to
Posterior urethral posterior urethral valves. It is postulated that the subvesical obstruction caused pressure
valves; build-up in the urinary tract, followed by fetal bladder rupture. The resultant urinoma
Prune-belly syndrome; migrated extra-abdominally. Distension of the urinary tract, bilateral cryptorchidism and
Fetal bladder rupture; abdominal wall laxity contributed to a prune-belly phenotype.
Urinoma ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Case report These prenatal findings were interpreted as most likely

A 32-year-old healthy primigravida was referred to our
centre because of fetal urological malformations on
a routine prenatal ultrasound. The pregnancy had thitherto
been uneventful. Ultrasonographic examination at 37
weeks of gestation revealed bilateral hydronephrosis with
megaureters and a distended, thick-walled bladder in
a male fetus. A large extra-abdominal, subumbilical cystic
structure, possibly connected to the fetal bladder, was
discerned. In addition, oligohydramnios and a compara-
tively large fetal abdominal circumference (95th percen-
tile) were noted. No other fetal anomalies were observed.
* Corresponding author. Tel.: þ31 (0) 24 361 39 36; fax: þ31 (0) 24
361 90 52.
E-mail addresses: m.binkhorst@cukz.umcn.nl (M. Binkhorst), r.
degier@uro.umcn.nl (R.P.E. de Gier).
representing a urachal cyst in the presence of PUV.
At 38þ5 weeks of gestation labor was induced and a male
newborn was vaginally delivered. Apgar scores were 6, 8
and 9 at 1, 5 and 10 min, respectively. Birth weight was
2.765 kg (25th percentile). The ventral abdominal wall
appeared conspicuously tumid and wrinkled with marked
diastasis. On top of this, a large transparent cystic mass was
located just inferior to the normally implanted umbilical
cord (Fig. 1). A small triangular patch of skin anterior to the
pubic symphysis was eroded. The penis was unremarkable
with a normally positioned external urethral orifice.
Neither testis had descended to a palpable position. The
right kidney appeared enlarged on palpation. The
remainder of the physical examination did not yield any
additional peculiarities.
Spontaneous urination occurred directly postnatally,
though with a weak stream. Umbilical discharge of urine

1477-5131/$36 ª 2009 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.jpurol.2009.04.008
92 M. Binkhorst, R.P.E. de Gier

Figure 1 Lax abdominal wall with a large subumbilical cyst in

the midline. The umbilical cord is normally implanted. Small
skin erosion just cranial to the penis (arrow).

Figure 2 VCUG (oblique view). The elongated bladder is fil-

was not observed. Because PUV and VUR were suspected, led with contrast, with passage of contrast medium through
a transurethral catheter was inserted and antibiotic a fistulous tract (narrow arrow) to the extra-abdominal cyst
prophylaxis administered immediately after birth. Upon (wide arrow).
catheter insertion, diuresis increased to the extent that the
patient became volume depleted and hyponatriemic, for right kidney, respectively. Drainage from both kidneys was
which replacement with 0.9% NaCl was instituted. Oxy- drastically delayed as a result of hydronephrosis.
butynine was administered to prevent bladder spasms. In
the following days, serum creatinine decreased from 102 to
42 mmol/l.
Discussion
A renal ultrasound demonstrated severe bilateral
hydronephrosis with tortuous megaureters, most We have described the unprecedented occurrence of
pronounced on the right side. A VCUG was indicative of a large extra-abdominal prevesical pseudo-cyst in a male
PUV, though it did not show VUR on either side. Interest- newborn with considerable hydronephrosis secondary to
ingly, the contrast medium clearly passed through a fistu- PUV. This newborn additionally featured major character-
lous tract from the elongated bladder to the prevesical cyst istics of the prune-belly syndrome, including abdominal
(Fig. 2). wall laxity, bilateral cryptorchidism and marked dilation of
Using a pediatric resectoscope, the valves in the pros- the supravesical urinary tract.
tatic urethra were ablated. The orifice of the left ureter Several anatomical and histological aspects of the pre-
was found to be lateralized, whereas its right counterpart vesical pseudo-cyst refute the prenatal postulation of it
was orthotopically positioned. The apex of the bladder representing a urachal cyst. The urachus is the fibrous
appeared edematous. During subsequent resection of the
prevesical cyst (Fig. 3), it became evident that the tubular
tract connecting the bladder with the cyst departed from
the anterior bladder wall and ran through the transversalis
fascia and rectus sheath. The cyst and tubular structure
were excised, the bladder was closed and the rectus sheath
and overlying skin were approximated (Fig. 4).
Microscopic analysis of the excised specimen revealed
a cyst surrounded by fibrovascular stroma showing an
inflammatory reaction. Urothelial cells were merely focally
present in the cyst capsule. The specimen stained negative
for alpha-smooth muscle actin, thereby refuting the pres-
ence of detrusor tissue in the cyst wall.
The baby boy recovered well from his operation.
Following catheter removal and spontaneous urination, he
was discharged. Serum creatinine remained normal and
urethrocystoscopy did not reveal residual urethral valves. A
MAG3 scan performed at the age of two months showed Figure 3 Cyst resection. The tweezers point to the opening
normal time activity curves for both kidneys. However, the of the fistulous connection between the cyst on this end and
filtration capacity emerged as 33 and 67% for the left and the bladder on the other.
Prevesical pseudo-cyst
Figure 4 Abdominal wall after cyst resection. Triangular skin
erosion just cranial to the base of the penis. The scrotum
appears empty. Transurethral catheter inserted.
remnant of the obliterated allantois. It is located within the
adipose tissue of Retzius’ space between the peritoneum
and transversalis fascia, and it extends from the bladder
apex to the umbilicus [1]. Urachal cysts develop when the
central portion of the allantois remains patent, while both
ends obliterate [2]. The urachus, and thus the capsule of
a urachal cyst, is normally composed of an internal layer of
urothelium, an intermediate layer of fibroconnective tissue
and an outer layer of smooth muscle (detrusor) tissue [1].
Inasmuch as the prevesical cyst in our case is situated
outside the transversalis fascia, it is connected via
a tubular structure to the anterior bladder wall instead of
to the bladder apex, and the cyst capsule does not contain
a definite urothelial layer or detrusor tissue, so it cannot
represent a urachal cyst.
We postulate the following sequence of events in the
origination of the pseudo-cyst. Due to PUV the pressure in
the urinary tract was retrogradely increased, especially
after closure of the urachus, which serves as a urinary
diversion in the first few weeks of embryonic life. Conse-
quently, a fetal bladder rupture might have occurred as
a ‘pop-off’ mechanism to decompress the urinary system.
Such a pressure vent supposedly prevents severe hydro-
nephrosis and renal parenchymal destruction and preserves
bladder function [3,4]. The extravasated urine has puta-
tively been encapsulated to form a prevesical urinoma,
which somehow came to lie outside the transversalis fascia
and maintained a connection to the bladder. The above-
mentioned histological findings substantiate this hypoth-
esis. The fact that the capsule of the pseudo-cyst consisted
of fibrovascular tissue can be explained by the urine-
induced inflammatory and fibrotic reactions in the pre-
vesical fat [5]. The presence of dispersed urothelial cells in
the cyst capsule supports the idea of ‘seeding’ following
93
the bladder ‘blow-out’. However, the mechanism by which
the pseudo-cyst traversed the transversalis fascia remains
elusive.
Our patient displayed features of the prune-belly
syndrome. Although an alternative pathogenetic pathway,
involving mesenchymal dysplasia, has been proposed to
account for the abdominal wall laxity and bilateral crypt-
orchidism in this syndrome, the obstructive theory may
serve as an explanation in our case [6]. That is due to
marked distension of the urinary system the blood supply to
the abdominal wall muscles is thwarted, resulting in
muscular atrophy. If the condition is aggravated by
a rupture somewhere along the urinary tract with conse-
quent urinary ascites, direct pressure atrophy of the
abdominal wall musculature may ensue. Testicular mal-
descent may be caused by the enlarged bladder or
decreased intra-abdominal pressure resulting from
abdominal wall laxity.
Up to this moment, our patient has thrived with an
adequate renal function. Orchidopexy will be performed
at the approximate age of 1 year. Whether or not
abdominoplasty will be necessary remains to be
determined.
Conflict of interest
None.
Funding
None.
Ethical approval
Not required.
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