Pediatr Surg Int (2012) 28:219228

DOI 10.1007/s00383-011-3046-6

REVIEW ARTICLE

Prune belly syndrome

S. Hassett G. H. H. Smith A. J. A. Holland

Accepted: 14 December 2011 / Published online: 25 December 2011

Springer-Verlag 2011

Abstract The majority of paediatric surgeons will

encounter a patient with prune belly syndrome (PBS) only
a few times in their clinical practice. There have been many
opposing views in the literature regarding the pathogenesis
and management of this complex condition. A detailed
review was conducted using PubMed to identify key publications involving PBS. This article discusses the evolution of our understanding of the pathogenesis and diagnosis
of PBS, including its typical characteristics. We describe
the management options available for bilateral intraabdominal testes, the deficient abdominal wall, the dilated
urinary system and examine the evidence base used to
support the current approaches employed.
Keywords Prune belly syndrome
Cryptorchidism

Abdominal wall
Hypotonia
Ectasia

Introduction
Prune belly syndrome (PBS) has been characterised by
deficient or absent abdominal wall musculature, hypotonia,
ectasia (that is pathophysiological dilatation or distension)
of the urinary system and bilateral intra-abdominal testes.

S. Hassett
A. J. A. Holland (&)

Douglas Cohen Department of Paediatric Surgery,
The Childrens Hospital at Westmead, Sydney Medical School,
The University of Sydney, Sydney, NSW, Australia
e-mail: andrew.holland@health.nsw.gov.au
G. H. H. Smith
Department of Urology, The Childrens Hospital at Westmead,
Sydney Medical School, The University of Sydney, Sydney,
NSW, Australia

It was initially reported by Frolich in 1839, although the

term prune belly syndrome was coined by Osler [1]. In
1950, nine cases were reported by Eagle and Barrett [2]
who described the condition as Eagle-Barrett Syndrome.
Other synonyms used in the literature include triad syndrome and abdominal musculature deficiency syndrome
[3]. Whilst PBS was originally described in male infants
with bilateral cryptorchidism as a diagnostic feature,
identical abdominal wall and urinary tract abnormalities
have been, albeit rarely, described in females [4].
While the triad of signs described remain a constant
feature in PBS, many other non genito-urinary associations
have been reported (Table 1). Recognition of these additional elements led Smolkin et al. [5] to suggest the term
prune belly association.
The purpose of this review article was to examine the
current evidence on the pathogenesis, diagnosis and management of PBS, together with the level and quality of the
evidence utilised to support the approach employed in the
treatment of patients.

Methods
A literature search was conducted using PubMed. The term
prune belly syndrome was used and limited to humans.
A total of 642 publications were identified. Studies which
used a heterogeneous population of patients including PBS
were excluded. Case reports, apart from those describing
genetic associations, were also omitted. Seventy-two relevant articles were identified where the study population
consisted solely of PBS patients. Levels of evidence were
assessed the Scottish Intercollegiate Guidelines Network
(SIGN) grading system for recommendations in evidencebased guidelines [6].

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Table 1 Prune belly syndrome associations

Pulmonary (58%)
Pulmonary hypoplasia

Cardiovascular (25%)

Gastrointestinal (24%)

Musculoskeletal (23%)

PDA

Malrotation (40%)

Scoliosis

Tetralogy of fallot

Atresia of small bowel and colon

Congenital hip dislocation

ASD

Splenic torsion

Club feet

VSD

Imperforate anus

Pectus carinatum

Valvular anomalies

Gastroschisis

Polydactylism

Omphalocele

Arthrogryposis

Cloacal anomaly

Hemivertebrae
Bilateral cervical ribs

Epidemiology
A recent review of the epidemiology of male infants with
PBS by Routh et al. [7] cited an incidence of 3.76 cases per
100,000 live births. An earlier study by Druschel et al. [8]
quoted an incidence in females of 1.1 per 100,000. Data
from the United States (US) indicates a significant increase
in incidence in the Afro-Caribbean population, with a
corresponding lower incidence in those of Hispanic origin
in comparison to the Caucasian population. There also
appears to be an increased incidence of PBS in children of
younger mothers [8].
There are reports of PBS occurring in association with
Trisomy 13, 18 and 21, but these cases are too few to
indicate a formal association with these genes [911].
There is some evidence to suggest a genetic influence in
PBS. There are 12 reported cases of familial PBS. Analysis
of these cases has implicated a sex hormone influenced
autosomal recessive mode of inheritance due to the male
predominance of the reported cases [12].
PBS is commoner in both monozygotic and dizygotic
twin pregnancies. Interestingly, in cases of monozygotic
twins, both discordance and concordance for PBS has been
reported, implying that inherited genetic mutations alone
cannot explain the pathogenesis of PBS [8, 13, 14].
An abnormality of hepatocyte nuclear factor 1b
(HNF1b) has been demonstrated in patients with sporadic
PBS. This transcription factor, expressed on chromosome
17, is found on numerous tissues in the body including
mesonephric duct derivatives, renal tubules of the metanephros and the developing prostate [15, 16].
Pathogenesis
Pathogenic theories of PBS include urethral obstruction
and a mesodermal developmental defect.
The theory of urethral obstruction was based mainly on
observations of postmortem specimens and the anatomy of
infants with PBS [17]. Urethral obstruction causes bladder
distension and urinary tract dilatation in the developing

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foetus. This distension prevents normal abdominal musculature development and testicular descent. Gonzalez
et al. [18] demonstrated PBS in sheep that had undergone
complete urethral obstruction between 43 and 45 days
gestation.
This theory fits neatly with cases of PBS, which feature
urethral atresia but does not explain PBS when the urethra
is patent, or the failure to develop the PBS phenotype in
cases of posterior urethral valves. Delayed canalization of
the urethra during the 11th16th weeks of gestation has
been proposed as a possible mechanism [19]. Transient
urethral obstruction at a critical time in the development of
the urinary tract may be responsible. One hypothesis is the
presence of a hypoplastic prostate, which occurs due to a
focal insult to the developing urogenital sinus mesoderm.
The resultant hypoplastic prostate may cause the unsupported membranous urethra to twist, or to create a flap
valve mechanism resulting in transient urethral obstruction
[20]. This is supported by the presence of generalized
prostatic hypoplasia found in PBS patients [21].
The other main theory in PBS is a failure of mesodermal
development [22]. As the urinary tract, abdominal wall
musculature, kidneys and the prostate gland, all affected in
PBS, share a mesodermal origin, this appears attractive. An
insult to the mesodermal layer during the early stages of
gestation should impact on other organ systems, however,
which is not the case in PBS. Perhaps more importantly,
the cause of the insult to the mesodermal layer has not been
identified.
Diagnosis
Antenatal diagnosis in the second trimester is now the most
common presentation of PBS [23]. Cases of trans-abdominal ultrasound diagnosis between 11 and 14 weeks of
gestation have been reported [2325]. Key elements in the
sonographic diagnosis of PBS include bilateral hydroureter
and hydronephrosis, a distended, thin-walled bladder and
oligohydramnios [26]. Oligohydramnios implies reduced
urine output, poor renal function and subsequent lung

Pediatr Surg Int (2012) 28:219228

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hypoplasia. The presence of severe renal dysfunction and

pulmonary hypoplasia in PBS is a predictor of nearly 100%
mortality within the first few days of postnatal life [27].
The differential diagnosis includes posterior urethral
valves (PUV) and megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS). In PUV, there is isolated
dilatation of the posterior urethra in association with a
thick-walled bladder leading to a keyhole sign. This can
also occur in PBS if there are associated urethral abnormalities making it difficult to differentiate between PBS and
isolated PUV on antenatal ultrasound [28]. In MMIHS, seen
much more frequently in females, a thin-walled bladder is
noted in association with normal liquor volume [29].
The issue of antenatal intervention in PBS is a part of
the wider question of the antenatal intervention in a foetus
showing signs of lower urinary tract obstruction (LUTO).
Decompression of the urinary system in utero can be
achieved via insertion of a vesico-amniotic shunt. Case
reports of vesico-amniotic shunting between 17 and
24 weeks gestation in PBS, in a foetus with a normal
karyotype and the absence of other severe congenital
abnormalities, have been reported [3032]. In all cases
oligohydramnios improved with survival of the foetus
through the last trimester and into postnatal life, although it
remains difficult to ascertain whether this was a reflection
of the natural history of the condition or as a result of
antenatal intervention. A meta-analysis performed by
Clarke et al. [33] indicated that prenatal bladder drainage
may improve perinatal survival in fetal LUTO. An attempt
to definitively answer this question is currently being
undertaken by the percutaneous shunting of lower urinary
tract obstruction (PLUTO) trial, the first randomized control trial of intervention versus conservative management
in foetuses with evidence of lower urinary tract obstruction
[34]. The results of this trial will hopefully provide guidance regarding antenatal intervention in the PBS patient.

Postnatal presentation
Classification of PBS, proposed by Woodard [35], was
based on antenatal and anatomical features (Table 2), with

20% of infants born with PBS classified as category 1. The

presence of severe renal dysfunction and pulmonary
hypoplasia resulted in near 100% mortality within the first
few days of postnatal life.
Category 2 patients have the classic features of PBS
with their prognosis dictated by the degree of renal dysplasia present. There appears wide variation in the degree
of renal dysplasia present in these patients with severe
forms requiring early dialysis. Routh et al. [7] reported an
incidence of 15% of newborns with PBS exhibiting some
degree of renal dysplasia with 4% presenting in acute renal
failure. Category 3 patients have normal renal function and
mild phenotypic features of PBS [36].
The initial postnatal course of PBS infants is dictated by
their co-morbidities: 75% of infants born with PBS have
co-existing morbidities [37, 38]. The most common is
prematurity with 43% of infants born preterm. Cardiovascular and pulmonary problems are also common (Table 1)
[7, 39, 40].
Perinatal mortality rates for PBS are quoted between 10
and 25%. This is primarily related to the degree of pulmonary hypoplasia, co-morbid conditions and prematurity.

Management issues in PBS

Urinary tract
The hallmark of PBS is a low-pressure dilated urinary
system, evident from the renal pelvis to the urethra.
Elongated, tortuous ureters occur as normal smooth muscle
is replaced by collagen and fibrous tissue. This does not
occur in a homogeneous fashion but rather in segments
along the ureter with the distal ureter most affected. Vesico-ureteric reflux (VUR) is found in 75% of PBS children
(Fig. 1) [41]. Like the ureter, the bladder is enlarged and
distorted due to replacement of bladder muscle with collagen and fibrous tissue. While the bladder may bulge in
different directions when intra-vesical pressure is
increased, trabeculations, and muscular hypertrophy are
uncommon. A urachal remnant appears common while
persistence of a patent urachus can be found in the presence

Table 2 Woodard classification of prune belly syndrome

Category 1 (20%)

Category 2 (40%)

Category 3 (40%)

Pulmonary hypoplasia and/or pneumothorax

Typical external features

External features mild or incomplete

Oligohydramnios

Hydroureteronephrosis

Normal renal function

Renal dysplasia

Uropathy

Mild degree of uropathy

Urethral obstruction patent urachus

Renal dysplasia

Club feet

Risk of urosepsis
Risk of azotemia

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Pediatr Surg Int (2012) 28:219228

Fig. 1 MCUG demonstrating irregular shaped floppy bladder with

gross VUR bilaterally

of urethral atresia, which allows decompression of the

urinary system in utero. Urodynamic profiles of PBS
bladders show that they are efficient at urine storage but
demonstrate significant residuals due to the high incidence
of VUR and poor detrusor contraction [42].
The prostatic urethra is wide and elongated and tapers as
it becomes the membranous urethra when it passes through
the urogenital diaphragm. This can give the impression of
mechanical obstruction, however, the tapering seen is
mainly due to redundant folds of urethral mucosa (Fig. 2).
Abnormalities of the anterior urethra have all been
reported in association with PBS. Both fusiform and scaphoid megalourethra have been found in association with
PBS but are rare. Conversely over 50% of cases of megalourethra are associated with PBS [43]. A mildly dilated
anterior urethra is the most common abnormality present in
approximately 70% of PBS patients [44].
The primary aim in management of the urinary tract is
preservation of renal function and prevention of urinary
infection. Woodward and colleagues felt that this was best
achieved using an approach of early surgery. Proximal
urinary drainage with cutaneous ureterostomies followed
by later definitive reconstruction and remodelling of the
urinary tract was their procedure of choice in PBS patients.
They reported stable renal function and a reduced rate of
urinary tract infection in PBS patients treated using this
approach (Level 3) [45, 46]. The authors acknowledged
that the success of this approach relied on maintenance of
adequate bladder emptying which may or may not be
achieved with reduction cystoplasty (Level 3) [46].

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Fig. 2 MCUG depicting large hypotonic bladder, wide open bladder

neck and dilated prostatic urethra

The rationale of ensuring adequate bladder emptying is

the basis of the conservative approach to the urinary tract in
PBS patients. Implementation of prophylactic antibiotics,
double voiding and timed voiding in toilet-trained children
coupled with regular functional renal assessments appears
as effective, with fewer complications, as early total urinary reconstruction in the setting of stable renal function
(Level 3) [4749]. If adequate bladder emptying cannot be
achieved by double voiding and timed voiding, regular
catheterisation can be implemented to aid urinary drainage.
If this proves problematic or painful, due to urethral
abnormalities, an appendicovesicostomy remains a useful
procedure to aid bladder emptying. Ectasia and drainage
patterns also improve over time making the conservative
approach a more attractive option in these complex
patients.
Long-term follow-up of PBS patients also provides
important insights into the early surgical versus conservative approach. Fallat et al. reported on 15 patients who had
undergone early urinary reconstruction, consisting of ureteric tapering, reimplantation and reduction cystoplasty. In
all patients, there were no changes in pre- and postoperative serum creatinine levels (Level 3) [39]. Bukowski et al.
examined the reduction cystoplasty element of their urinary
reconstructions. In their cohort of 11 patients, there was
some improvement in voiding and reduction in number and
severity of urinary tract infections, but long-term review

Pediatr Surg Int (2012) 28:219228

(mean 7.7 years) demonstrated no reduction in bladder

capacity or improvement in voiding dynamics (Level 3)
[50]. Kinahan et al. [42] also demonstrated no difference in
urodynamic profiles between reconstructed and nonreconstructed PBS patients (Level 3).
There is a high complication rate associated with surgery in PBS patients [39, 51]. This is likely due to the poor
peristalsis and inherent muscular deficiencies present in the
PBS ureter and bladder. Vesico-ureteric junction (VUJ)
obstruction requiring re-operation in 40% of patients and
failure of resolution of VUR post-reimplant in 30% of
patients was reported in one series [39].
It appears as if early aggressive surgery on the basis of
urinary tract dilatation alone without evidence of compromised renal function in PBS appears unwarranted given
the reported success of the conservative approach coupled
with the high complication rate of surgery associated with
PBS patients (Level 3) [39, 4749, 51].
Anecdotal reports recommend circumcision in the setting of recurrent urinary tract infection in PBS patients,
although no series exploring this question has been published [52]. It is likely that the practice has evolved following observations in the PUV and high-grade reflux
populations of a reduction in urinary tract infections following circumcision [53].
One definite indication for early surgery is where
obstruction is present secondary to urethral atresia. These
cases tend to have a patent urachus or bladder fistula that
has enabled urinary tract decompression in utero. Formation of a vesicostomy or short-term placement of an
indwelling urachal catheter provides adequate decompression until more definitive surgery is performed in the future
(Level 3) [46, 54].
If there is a steady decline in renal function or consistent
failure to prevent or eradicate infection using conservative
measures then surgery must be considered to provide more
effective drainage of the urinary system (Level 3) [39, 51].
Pyleoplasty, ureteric tapering and reimplantation and
cystoplasty may improve drainage and prevent infections
in some cases, but carry complication rates of up to 40%
with revisional surgery often required (Level 3) [39, 51].
The complexity of PBS patients means that each case must
be approached on an individual basis with no general
consensus as to which surgical procedure is most effective
in aiding drainage and preventing infections.
Abdominal wall
As with other anatomical manifestations of PBS, there is a
wide phenotypic variation in the abdominal wall of PBS. It
classically consists of poorly organized central abdominal
musculature interspersed with dense collagen (Fig. 3). All
muscle layers are commonly affected particularly below

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Fig. 3 Newborn male with PBS, demonstrating classic abdominal

wall appearance

the umbilicus where muscle may be entirely replaced by

fibrous tissue. In contrast, the peripheral abdominal wall
shows normal or nearly normal muscle and fascial layers
[55, 56].
The basis of abdominal wall reconstruction is the
advancement of these well-innervated, well-vascularised
peripheral muscle layers with or without excision of the
deficient central muscle and fascial portions. The rationale
is that improved abdominal tone, due to the better quality
tissue, may lead to improved sensation of bladder distension and an enhanced efficiency of the Valsalva manoeuvre
in bladder emptying (Level 4) [57].
A number of techniques have been described in abdominal wall reconstruction in PBS. In 1981, Randolph [58]
reported his technique utilising a U-shaped incision between
the tips of the 12th ribs with full thickness excision of the
lower abdominal layers and advancement of the upper
abdominal layers towards the groin and pubis (Fig. 4).
Erhlich [59] described advancing one fascial flap medially to
the opposite side via a midline vertical incision creating a
double-breasted fascial plate (Fig. 5). Whilst similar, the
Monfort technique, described in 1991, involves extensive
dissection of both lateral fascial plates prior to a doublebreasted midline closure. This procedure was performed on
nine patients (age range 110 years old) with reports of a
satisfactory result in all cases (Fig. 6, Level 3) [60].
The Firlit technique, reported in 1998, has the advantage
of an extra-peritoneal approach. Skin flaps are raised laterally to expose the fascial layer via a vertical midline

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Fig. 4 Randolph technique: this technique utilises a U-shaped

incision between the tips of the 12th ribs with full thickness excision
of the lower abdominal layers and advancement of the upper
abdominal layers towards the groin and pubis

Fig. 5 Erhlich technique: this technique advances one fascial flap

medially to the opposite side via a midline vertical incision creating a
double-breasted fascial plate

incision (Fig. 7). This lateral fascial layer is plicated in a

double-breasted fashion in the midline without lateral fascial dissection or incision of the midline fascial plate. This

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Pediatr Surg Int (2012) 28:219228

Fig. 6 Monfort technique: this technique involves extensive dissection of both lateral fascial plates prior to a double-breasted midline
closure

Fig. 7 Firlit technique: this technique plicates the lateral fascial

layers in a double-breasted fashion in the midline without lateral
fascial dissection or incision of the midline fascial plate

technique has been described in 13 boys (age range

9 months11 years) with a follow-up period of
17207 months. Three patients required further surgery at
14 months, 1 and 5 years post-initial surgery (Level 3)
[61]. A laparoscopic-assisted technique was described in

Pediatr Surg Int (2012) 28:219228

2005 to visualise suture placement and prevent inadvertent

suturing of underlying bowel [62].
Smith et al. reported on improved bladder emptying in
their series of 12 patients following Monfort abdominal
reconstruction. Subjective improvements in continence,
sensation and urinary flow were noted in the majority of
patients. Improved postvoid residuals from 40% down to
13% were reported. Five out of 10 patients noticed a
reduced defecation time with increased quantities of stool
evacuated, while two out of 12 patients with poor bowel
control reported a reduced laxative requirement (Level 3)
[57]. The authors speculated that a contributory factor to
these improvements was as a result of the increased pressure of the Valsalva manoeuvre in accordance with Pascals
principle (pressure = force/area), although no formal
measurements were performed.
Crompton et al. [63] demonstrated that air-trapping
secondary to poor respiratory effort from deficient
abdominal wall musculature was the main feature of spirometry testing in patients with PBS (Level 4). No investigation of postoperative spirometry has been published in
PBS patients, but anecdotal evidence suggests an improved
clearance of respiratory secretions post-abdominal reconstruction 64].
While these studies are informative, there is insufficient
evidence currently to justify abdominal wall reconstruction
as anything other than a measure to improve the appearance of the abdominal wall. Advocates argue that the
improvement in a patients self-esteem post-reconstruction
warrants an aggressive approach at an early age although
this is based on anecdotal reports rather than any evidence
base. It is worth noting that good cosmetic outcomes have
also being achieved with improvement in abdominal wall
laxity over time using abdominal corsets for support,
although once again the evidence for corset use remains
weak [58, 65].
Cryptorchidism
Bilateral intra-abdominal testes remain one of the major
features of PBS. Mechanical obstruction secondary to the
enlarged bladder and urinary system, coupled with the
absence of central abdominal wall musculature likely
impedes normal descent of the testes. The gubernaculum,
important in testicular descent, has been noted to be atretic
or abnormally attached at the pubic tubercule [66]. The
presence of developmental abnormalities in the PBS testis
leading to failure of descent has also been suggested
although the evidence is contradictory.
Orvis et al. [67] studied the testes from fetal specimens
and noted reduced spermatogonia and Leydig cell hyperplasia. Conversely Nunn and Stephens [66] described
normal testicular histology for age in autopsy specimens

225

from newborns and prepubertal children. Massad et al. [68]

described atypical germ cells in testicular biopsy specimens from three boys aged 5.5, 6 and 7 months old. Adult
males with PBS are azoospermic with little or no germ cell
maturation [69]. It is unclear whether this occurs due to
developmental arrest, failure of descent, late orchidopexy
or a combination of all three factors.
There are three treatment options available to treat intraabdominal testes in PBS: single stage bilateral orchidopexy
with preservation of the vascular pedicle, single stage
bilateral Fowler-Stephens orchidopexy or a two stage
Fowler-Stephens bilateral orchidopexy. These can be performed via either an open or a laparoscopic technique.
Success rates of 80 and 85% are reported for single stage
and two stage Fowler-Stephens orchidopexy, respectively
[70]. A standard single stage orchidopexy for intraabdominal testes has a reported success rate of [90% [71,
72]. The laparoscopic approach would seem the procedure
of choice for the management of intra-abdominal testes with
comparable rates of testicular survival reported [73, 74].
Literature relating specifically to orchidopexies in PBS
demonstrates higher rates of testicular loss. Denes et al.
[51] reported on single stage bilateral orchidopexy at the
time of urinary reconstruction. In 32 patients, an 8%
atrophy rate was noted and 8% of testes were positioned in
the inguinal region. The mean age of surgery was
23 months with a mean follow-up period of 60 months
(Level 3) [51]. Fallat et al. demonstrated that children
[2 years showed a higher atrophy rate (30%) compared
with only 4% of those \2 years following orchidopexy
performed at the time of urological reconstruction. Of
these, 82% were single stage orchidopexy, 14% the Fowler-Stephens technique and 4% a staged Fowler-Stephens
technique [39]. The largest series was reported in 2004 by
Patil et al.: in the 31 boys who underwent a single stage
orchidopexy, at a mean age 8 years, there was a testicular
atrophy rate of 16% with 24% showing evidence of a
smaller testis. In the two stage orchidopexy group
(n = 30), where the mean age of surgery was 3.2 years, the
testicular atrophy rate was 3% with smaller testes noted in
13% of boys. Of those patients who had a single stage
bilateral orchidopexy, 10% required hormonal supplementation at puberty while all children who had a two stage
orchidopexy underwent spontaneous puberty (Level 3)
[75].
There are only three reports in the literature of laparoscopic orchidopexy in PBS and these primarily relate to
technical issues rather than testicular survival. Problems
with air leak were reported due to the absent abdominal
musculature. Recommendations from these reports suggest
careful port fixation, higher CO2 flow rates and the use of
longer instruments due to the lax abdominal wall (Level 4)
[7678].

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The higher rates of testicular loss reported in PBS

patients might reflect older age at orchidopexy rather than
PBS as an independent risk factor for increased testicular
loss following orchidopexy. Orchidopexy was often timed
when urinary reconstruction and abdominal wall reconstruction were performed which was beyond the first year
of life. Current guidelines recommend early orchidopexy
(\1 year) to maximise testicular function and reduce the
risk of malignancy [79]. These guidelines should also
apply to PBS patients. Where urinary or abdominal wall
reconstruction is considered in the first year of life, a
single stage abdominal orchidopexy should be performed
at the same time. At this early age, it has been noted that
it is easier to achieve mobilisation to allow for successful
scrotal placement of the testis (Level 4) [39]. If no
abdominal surgery is planned then a laparoscopic
approach should be utilised with a single stage procedure
used where possible to allow maximum potential for the
testes to survive and develop.
Renal transplantation
Nearly a third of PBS patients outside the postnatal period
will progress to renal failure requiring transplantation.
Early identifiable risk factors are renal cystic disease and
dysplasia reported in approximately 50% of infants with
PBS. The most accurate marker of significant renal dysplasia is nadir creatinine; that is the lowest consistent
creatinine level in the first 12 months of life. Canning et al.
reported that a creatinine level of [0.7 mg/dl was a strong
predictor of progression to renal failure in later life.
Interestingly, degree of dilatation of the urinary system was
not a significant prognostic factor for renal function,
lending credence to the non-obstructive nature of PBS
(Level 3) [80]. Renal failure may also occur from chronic
pyelonephritis related to urinary stasis and incomplete
bladder emptying with repeated infections and obstructive
nephropathy.
The commonest indications for a renal transplant in PBS
patients are renal failure secondary to chronic pyelonephritis and renal dysplasia [8185]. While there remains no
specific data available on end stage renal failure (ESRF)
rates in PBS patients, the mean age at transplantation has
been reported to be between 7.5 and 13.5 years [82, 84,
85]. Early progression to ESRF is attributed to significant
renal dysplasia while later progression is thought to occur
due to renal damage from repeated infections and high
pressures generated from obstruction (Level 3) [81].
The literature has emphasized the importance of
achieving and maintaining adequate bladder emptying
prior to transplantation. Poor bladder emptying leads to
high detrusor pressures, stasis and infection, associated
with higher rates of graft deterioration post-transplant.

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Improvement in bladder emptying may be achieved by

timed voiding and intermittent catheterisation. If low
postvoid residuals are not achieved using these conservative methods, then more aggressive treatment by internal
urethrotomy or vesicostomy has been reported (Level 4)
[83, 84]. Acute graft torsion secondary to the abdominal
wall laxity is a specific problem in PBS patients, with
abdominoplasty or transplant nephropexy advocated to
prevent occurrence (Level 4) [83, 84].
PBS patients have similar outcomes to age-matched
controls following renal transplantation. This is likely a
reflection of the preoperative preparation to ensure adequate bladder emptying coupled with vigilant post-transplant follow-up in these patients.

Conclusion
This review highlights the complexities of patients with
PBS and the difficult management decisions that are faced.
The literature, while informative, has been predominately
retrospective reviews from single institutions reporting
treatment options and outcomes. A more collaborative
approach is now required in the form of multicentre prospective randomized studies assessing specific treatment
outcomes. Without this, we will be none the wiser over the
coming decades into the correct management of these
complex set of patients.

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