Curr Urol Rep
DOI 10.1007/s11934-013-0332-y
PEDIATRIC UROLOGY (M CASTELLAN AND R GOSALBEZ, SECTION EDITORS)
Modern Management of Bladder Exstrophy Repair
Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo &
Bhavik B. Shah & John P. Gearhart
# Springer Science+Business Media New York 2013
Abstract The exstrophy–epispadias complex is a rare con-
genital malformation of the genitourinary system, abdominal
wall musculature, and pelvic bones. Historically, surgical out-
comes in patients with classic bladder exstrophy, the most
common presentation of the exstrophy–epispadias complex,
were poor. However, modern techniques have increased the
success of achieving urinary continence, satisfactory
cosmesis, and improved quality of life. Still, recent studies
recognize complications that may occur during management
of these patients. This review provides readers with an over-
view of the exstrophy–epispadias complex, the modern man-
agement of bladder exstrophy, and potential surgical
complications.
Keywords Bladder exstrophy repair . Management .
Cosmesis . Exstrophy–epispadias complex . Surgical
complications
Abbreviations
EEC Exstrophy–epispadias complex
CBE Classic bladder exstrophy
OEIS Omphalocele, exstrophy, imperforate anus, spinal
abnormalities
VUR Vesicoureteral reflux
MRI Magnetic resonance imaging
CIC Clean intermittent catheterization
MSRE Modern staged repair of exstrophy
CPRE Complete primary repair of exstrophy
BNR Bladder neck reconstruction
CUD Continent urinary diversion
B. M. Inouye : E. Z. Massanyi : H. Di Carlo : B. B. Shah :
J. P. Gearhart (*)
James Buchanan Brady Urological Institute,
Division of Pediatric Urology, Charlotte Bloomberg
Children’s Hospital, The Johns Hopkins University School
of Medicine, 1800 Orleans St. Suite 7304,
Baltimore, MD 21287, USA
e-mail: jgearha2@jhmi.edu
Introduction
The exstrophy–epispadias complex (EEC) is a rare spectrum
of multisystem birth defects involving the genitourinary and
gastrointestinal tracts, musculoskeletal system, pelvic floor,
and bony pelvis. In classic bladder exstrophy (CBE), there is
a lower abdominal wall defect exposing an open bladder and
urethra, a wide diastasis of the pubic symphysis, and an
epispadic urethral opening. Cloacal exstrophy presents sim-
ilarly but with a bilobed bladder separated by a portion of
cecum between two hemibladders. Being a more severe
birth defect, cloacal exstrophy also presents with various
other anomalies, such as malformations of the gastrointesti-
nal, musculoskeletal, and central nervous systems, also
known as the OEIS (omphalocele, exstrophy, imperforate
anus, spinal abnormalities) complex. Complete epispadias is
the least severe form of EEC and presents with a closed
bladder, dorsally open urethral meatus, and mild pubic
diastasis.
Patients with EEC will undergo multiple reconstructive
surgical procedures beginning with closure of the urethra,
bladder and anterior abdominal wall, along with approxima-
tion of the pubic rami. Oftentimes, children with CBE must
undergo pelvic osteotomy and lower extremity immobiliza-
tion. While current techniques achieve reasonable success in
preservation of renal function, continence, and cosmesis,
there are also many recognized complications associated
with reconstruction.
Overview and Epidemiology of the Exstrophy–epispadias
Complex
CBE is the most common presentation of EEC, occurring in
approximately one per 10,000 to 50,000 births [1] and
affecting males approximately twice as often as females
[2]. Additionally, Caucasian infants are more likely to pres-
ent with the complex compared to non-Caucasian infants
[3]. Other risk factors include young maternal age and

multiparity. Furthermore, there is an increased risk of EEC
among children conceived with assisted-reproductive tech-
nologies such as in vitro fertilization [4].
Embryology
While the cause of ECC has not been completely elucidated,
it is thought to be due to a disorder of cloacal membrane
development. During the fourth gestational week, the cloa-
cal membrane may over develop and prevent the mesen-
chyme from migrating between the ectoderm and endoderm.
It is thought that this malformation not only inhibits the
normal development of the lower abdominal musculature
and pelvic bones, but also makes the cloacal membrane
unstable and prone to early rupture. The timing and location
of rupture of the cloacal membrane dictates the patient’s
presentation along the exstrophy–epispadias spectrum [5,
6]. CBE results if the rupture occurs after the urorectal
septum divides the gastrointestinal from the genitourinary
tracts while cloacal exstrophy results if the rupture occurs
before this separation [7].
Functional Anatomy of the Exstrophy–epispadias
Complex
Bladder
The bladder and urethra are exposed anteriorly in both
CBE and cloacal exstrophy. The bladder template may
be small (<3 cm) [8], covered with polyps, or inelastic,
thus requiring delayed closure for a successful outcome
[9]. If the patient’s bladder capacity does not grow
following successful closure, the patient may ultimately
need a bladder augmentation [10]. While there are sig-
nificantly fewer myelinated nerves per histologic field in
exstrophic bladder compared to controls, this follows
the hypothesis that these bladders are immature and
have potential for normal development after a successful
initial closure [11].
The ureters in patients with CBE enter the bladder at
an abnormal angle resulting in vesicoureteral reflux
(VUR) in all patients following bladder closure [12,
13]. These ureters are usually reimplanted into the blad-
der at the time of bladder neck reconstruction or aug-
mentation cystoplasty.
Pelvic Bones
Patients with EEC may have diastasis of their pubic
rami with divergent distal rectus abdominis muscles.
Compared to controls, CBE patients have external
Curr Urol Rep
rotation of both the anterior and posterior segments of
the pelvis, 30 % shortening of the anterior pelvis, in-
creased distance between the triradiate cartilage, and
retroversion of the acetabulum [14]. These patients also
have wider sacroiliac joint angles, a more inferiorly
rotated pelvis, and a larger sacrum when compared to
non-exstrophy patients [15]. Pelvic deformities may
cause the child to have a waddling gait.
Abdominal Wall
In CBE, the bladder and urethra are exposed through a
triangular defect in the lower abdominal wall. The opening
is limited superiorly by the umbilicus and inferiorly by the
intrasymphyseal band. This band connects the posterior
urethra and bladder neck to the pubic ramus. Any umbilical
hernia is usually insignificant and can be repaired with the
primary exstrophy repair. Indirect inguinal hernias are com-
mon in CBE patients due to a persistent processus vaginalis,
large inguinal rings, and the relatively straight direction of
the inguinal canal [16]. While an omphalocele is uncommon
with CBE, it usually occurs with cloacal exstrophy. In
cloacal exstrophy, patients may have two bladder plates that
can be separated in the midline by a portion of duplicated
cecum or hindgut [17].
Pelvic Floor Musculature
Magnetic resonance imaging (MRI) studies have shown that
pelvic floor musculature is significantly different in preop-
erative EEC patients compared to controls or postoperative
patients. In CBE, the levator ani is located more posteriorly
and is externally rotated and flattened resulting in a “box-
like, open book” pelvis with an anteriorly positioned blad-
der [18••]. These abnormalities contribute to incontinence in
these patients and predispose females to uterine prolapse
[19].
Anorectal Abnormalities
Patients with CBE have an anteriorly displaced anus
and anal sphincter. Along with the differences in the
pelvic floor musculature, the sphincter misalignment
predisposes these patients to fecal incontinence. Other
structural anomalies in CBE patients can include imper-
forate anus, rectal stenosis, and rectal prolapse [20•]. An
imperforate anus is especially common in cloacal
exstrophy. Compared to CBE, patients with cloacal
exstrophy also have a higher incidence of other associ-
ated gastrointestinal abnormalities including a rudimen-
tary hindgut, malrotation of the bowel, and short gut
syndrome (which can be worsened with the myriad of
bowel surgeries these children may need) [17, 21].
Curr Urol Rep
Genitalia
In males, the urethral meatus is located on the dorsal surface
of the phallus between the peno-pubic angle and the prox-
imal glans. The glans is open along the dorsal surface and
shaped like a spade. The phallus is short and broad with a
dorsal chordee. Contributing to the shorter appearing phal-
lus is the lateral displacement of the corporal bodies under
the pubic bones [22]. In cloacal exstrophy, the male or
female phallus is usually halved with each half often of
unequal size [17].
In females, the clitoris is bifid with superiorly divergent
labia. The distal dorsal aspect of the urethra is open with a
patulous bladder neck. They may also have a short vagina or
stenotic vaginal orifice [23•].
Spinal Abnormalities
Patients with CBE have a 6.7 % chance of presenting with a
spinal abnormality such as spina bifida occulta, scoliosis,
and hemivertebrae. Most of the abnormalities are uncompli-
cated, but spinal dysraphism may cause neurologic dysfunc-
tion [24]. Nearly all cloacal exstrophy patients present with
a significant neurological abnormality, including neural tube
defects, vertebral anomalies, and tethered cord [25]. These
associated anomalies necessitate prompt neurological eval-
uation and can further exacerbate urinary and bowel
incontinence.
Diagnosis of Classic Bladder Exstrophy
Bladder exstrophy can be diagnosed prenatally with
fetal transabdominal ultrasound between the 15th and
32nd weeks of pregnancy [26]. Absence of bladder
filling, a low-set umbilicus, widened pubic rami, small
genitalia, and a lower abdominal mass that increases
throughout the duration of pregnancy are all character-
istics of CBE on antenatal imaging [27, 28]. Since only
25 % of patients with EEC were diagnosed prenatally
[29••], it is important that the remaining children be
diagnosed quickly so they can be appropriately managed
at a specialized center. Studies have shown that
exstrophy patients treated at specialty center have lower
rates of postoperative morbidity and mortality [30].
Management of Classic Bladder Exstrophy
Abdominal and Bladder Closure
Historically, patients with exstrophy often required
cystectomy and died from complications of renal failure.
Current treatments preserve the bladder in nearly every patient
and allow for continence through the urethra [31, 32]. How-
ever, if the primary closure fails or if the patient’s bladder
remains small or noncompliant it may require an augmenta-
tion cystoplasty or bladder neck transection with continent
urinary diversion to achieve dryness [33–35]. These patients
will need to perform clean intermittent catheterization (CIC)
to empty their bladders/pouches.
Repair of bladder exstrophy typically includes closure
of the bladder and abdominal wall, repair of the
epispadias, ureteral reimplantation, and bladder neck
reconstruction (BNR). Children can also undergo pelvic
osteotomies (often performed with primary bladder clo-
sure) to deepen their flattened pelvis, close the pubic
diastasis, and release tension on the abdominal wall.
Following osteotomy, appropriate pain management and
4–6 weeks of postoperative pelvic and lower extremity
immobilization are required. Epispadias repair is gener-
ally performed at 6 months to 1 year of age and BNR
after 4–5 years of age, when the child wants to be
continent. Successful primary closure is of utmost im-
portance since it is associated with decreased overall
costs, decreased inflammation and fibrosis of the blad-
der, improved bladder growth, and decreased need for
urinary diversion [8, 36, 37, 38•, 39, 40].
The timing of the primary closure is still in debate.
Proponents of early bladder closure (closure during the
first 72 hours of life) argue that prompt closure allows
for sooner bladder cycling and improved bladder expan-
sion. In addition, closure while the pelvis is still mal-
leable may allow adequate reduction of the pelvic
diastasis without the need for osteotomy. Recent studies
have suggested that bladders that remain exstrophic for
long periods of time are more likely to undergo precan-
cerous changes [41•]. Other studies refute exstrophied
bladders and the intestinal metaplastic changes they may
undergo as not being precursors for cancer [42]. As
previously stated, delaying bladder closure may be help-
ful in patients born with small bladder templates. In
these patients, allowing the bladder template to develop
prior to closure is believed to increase the likelihood of
postclosure bladder growth. Additionally, delaying blad-
der closure may allow epispadias repair to be performed
concomitantly rather than as an additional procedure.
Bladder Augmentation
Patients with failed CBE repair have only a 60 % chance of
achieving adequate bladder capacity for a bladder neck
reconstruction [43]. Even if the bladder closure is success-
ful, a bladder that is noncompliant or of insufficient capacity
may undergo augmentation cystoplasty [44]. Common tech-
niques utilize segments of bowel or redundant ureter to

expand the bladder wall. During the postoperative period,
the bladder must be drained and irrigated continuously to
ensure proper healing and removal of bowel mucus.
Continent Urinary Diversion
Continent urinary diversion (CUD) is typically required
when a patient undergoes augmentation cystoplasty. A seg-
ment of colon or ileum may be utilized to connect the
bladder to the skin and provide a continent stoma for inter-
mittent catheterization [45].
Modern Staged Repair of Bladder Exstrophy (MSRE)
Seventy percent of patients who undergo modern staged
repair bladder exstrophy repair achieve dryness with
minimal complications [32]. The first stage focuses on
closing the abdominal wall and bladder in the newborn
child [46]. However, bladder closure may be delayed if
it is too small or covered with polyps [47]. If the
closure is delayed after the first 72 hours of life or
the pelvis has poor mobility, bilateral transverse anterior
innominate and vertical posterior iliac osteotomies are
performed.
The second stage is to close the urethra at 6 to
12 months of age. Male patients undergo a Modified
Cantwell–Ransley repair of the epispadias once their
urethral groove is of adequate length. Following
epispadias repair, the patient’s bladder capacity is mea-
sured annually with gravity cystogram under anesthesia.
Once the patient achieves a bladder with adequate
capacity and desires continence (usually between 5 to
9 years of age), he or she will undergo a continence
procedure, such as the Young–Dees–Leadbetter BNR and
ureteral reimplantation. Children who are not candidates for
BNR or who fail to achieve urinary continence after the
procedure may require bladder neck transection, augmenta-
tion cystoplasty, continent urinary diversion, and continent
catheterizable stoma.
Complete Primary Repair of Exstrophy (CPRE)
Unlike MSRE, the complete primary repair combines
primary abdominal wall and bladder closure with
epispadias repair and partial tightening of the bladder
neck [48]. Proponents argue that this technique may
decrease costs, decrease the morbidity associated with
multiple operations, and stimulate early bladder growth.
The epispadias is repaired by fully dissecting the ure-
thral plate from the corporal bodies, a procedure known
as “penile disassembly” to better facilitate urethral clo-
sure and posterior positioning of the bladder neck into
the pelvis [49]. While CPRE reduces the number of
Curr Urol Rep
surgeries for CBE, many children still require surgery
for resulting hypospadias, persistent vesicoureteral re-
flux, incontinence, or failed primary closure [48, 50].
Moreover, CPRE have also been associated with com-
pilations such as wound dehiscence, bladder prolapse,
vesicocutaneous fistula, and loss of penile tissue
[51–53]. However, long-term urinary continence with
CPRE may be similar to MSRE [54, 55].
Pelvic Osteotomies and Immobilization
Pelvic osteotomies are recommended in patients who no
longer have a malleable pelvis, which usually occurs after
72 hours of age. Osteotomies may increase surgery time and
risk for postoperative complications. However, the use of
osteotomies during closure is associated with improved
success of primary closure by providing a tension-free ap-
proximation of the pubic symphysis and abdominal wall that
also results in deeper placement of the bladder into the
pelvis [56].
A combination of bilateral anterior transverse innom-
inate and vertical posterior iliac osteotomies has been
shown to decrease the rate of abdominal dehiscence and
bladder prolapse as compared to other osteotomies [57].
At the time of osteotomy, fixator pins and external
fixation devices can be placed and left postoperatively
for 4 to 6 weeks. The fixators and pins can be removed
when good callous formation is seen on pelvic radiog-
raphy (usually 6 to 8 weeks postoperatively).
The pelvis is also immobilized in traction for approx-
imately 4 to 6 weeks postoperatively to provide pelvic
stabilization and decrease the risk of failed closure.
Modified Buck’s traction exerts pull longitudinally on
the lower extremities and is used after osteotomy. Mod-
ified Bryant’s traction, where the hips are placed into 90 ° of
flexion, may be used if there is no osteotomy. Spica
casts also immobilize the pelvis without the need for
external fixators or traction [58]. However, these casts,
along with the technique of “mummy wrapping” the
child’s legs, have been called into question after a
retrospective study found both to have lower overall
success rates and higher rates of skin breakdown com-
pared to patients who were placed in modified Buck’s
or Bryant’s traction [56].
Postoperative Management
The single most important predictor of long-term blad-
der growth and continence is successful primary bladder
closure [33, 38•]. A failed CBE closure is defined by
wound dehiscence, bladder prolapse, bladder outlet ob-
struction, or formation of a vesicocutaneous fistula [53,
Curr Urol Rep
59]. Pelvic osteotomy and postoperative immobilization
in carefully selected patients have been shown to de-
crease the rate of failure [56].
Following primary closure, patients should be
maintained on prophylactic antibiotics to prevent poten-
tial infections of the urine, bone or wound. Furthermore,
the bladder and kidneys must be drained with a
suprapubic catheter and ureteral stents for the duration
of immobilization to prevent bladder distention, urinary
obstruction, or leakage into the wound. Removal of the
suprapubic tube occurs after testing for urethral patency.
Annual cystoscopy and cystography after the procedure
is performed to evaluate the bladder capacity the grade
of VUR.
Potential Complications
Both MSRE and CPRE have been shown to be suitable
procedures to repair CBE. Complications such as wound
dehiscence, bladder prolapse, bladder outlet obstruction,
and vesicocutaneous fistula formation have been
reported with both techniques. Additional intestinal
complications such as ileus, volvulus, and small bowel
obstruction can occur. CPRE specifically can also lead
to the significant complication of penile loss following
penile disassembly.
Osteotomies increase the risk of transient nerve and
muscle palsies (which typically resolve by 12 weeks
post operation), delayed ileal union, superficial infec-
tion, and inflammation at pin sites. Furthermore,
osteotomies may fail, leading to a recurrent pubic
diastasis, if the pelvis partially rotates or if there is
continued delayed of growth of the ischiopubic bone
[60].
Augmentation cystoplasty and vesicocutaneous diver-
sion also present unique potential complications such as
bladder calculi, chronic bacterial colonization, epithelial
polyps, and mucus production [43]. Bladder augmenta-
tion may also lead to metabolic acidosis and carcinoma
[44] while continent urinary diversion has been associ-
ated with stomal stenosis [45].
Conclusion
In the past, CBE was a devastating multisystem birth defect
affecting a patient’s bladder function and quality of life. Howev-
er, current management with bladder closure, pelvic osteotomies,
and traction and immobilization have changed EEC to a man-
ageable diagnosis that can leave a child continent and with a high
quality of life. These advancements also come with uncommon,
yet unique complications that surgeons should be aware of.
Conflict of Interest Dr. Brian M. Inouye reported no potential con-
flicts of interest relevant to this article.
Dr. Eric Z. Massanyi reported no potential conflicts of interest
relevant to this article.
Dr. Heather Di Carlo reported no potential conflicts of interest
relevant to this article.
Dr. Bhavik B. Shah reported no potential conflicts of interest
relevant to this article.
Dr. John P. Gearhart reported no potential conflicts of interest
relevant to this article.
Human and Animal Rights and Informed Consent This article
does not contain any studies with human or animal subjects performed
by any of the authors.
References
Papers of particular interest, published recently, have been
highlighted as:
• Of importance
•• Of major importance
1. Lattimer JK, Smith MJ. Exstrophy closure: a followup on 70 cases.
J Urol. 1966;95(3):356–9.
2. Gambhir L et al. Epidemiological survey of 214 families with
bladder exstrophy–epispadias complex. J Urol. 2008;179(4):1539–
43.
3. Nelson CP, Dunn RL, Wei JT. Contemporary epidemiology of
bladder exstrophy in the United States. J Urol. 2005;173(5):1728–
31.
4. Wood HM, Babineau D, Gearhart JP. In vitro fertilization and the
cloacal/bladder exstrophy–epispadias complex: a continuing asso-
ciation. J Pediatr Urol. 2007;3(4):305–10.
5. Muecke EC. The Role of the Cloacal Membrane in Exstrophy: The
First Successful Experimental Study. J Urol. 1964;92:659–67.
6. Martinez-Frias ML et al. Exstrophy of the cloaca and exstrophy of
the bladder: two different expressions of a primary developmental
field defect. Am J Med Genet. 2001;99(4):261–9.
7. Ambrose SS, O'Brien III DP. Surgical embryology of the exstrophy–
epispadias complex. Surg Clin North Am. 1974;54(6):1379–90.
8. Dodson JL et al. The newborn exstrophy bladder inadequate for
primary closure: evaluation, management and outcome. J Urol.
2001;165(5):1656–9.
9. Baka-Jakubiak M. Combined bladder neck, urethral and penile
reconstruction in boys with the exstrophy–epispadias complex.
BJU Int. 2000;86(4):513–8.
10. Diamond DA et al. Normal urodynamics in patients with bladder
exstrophy: are they achievable? J Urol. 1999;162(3 Pt 1):841–4.
discussion 844-5.
11. Mathews R et al. Neural innervation of the newborn exstrophic
bladder: an immunohistochemical study. J Urol. 1999;162(2):506–
8.
12. Canning DA et al. The cephalotrigonal reimplant in bladder neck
reconstruction for patients with exstrophy or epispadias. J Urol.
1993;150(1):156–8.
13. Mathews R, Hubbard JS, Gearhart JP. Ureteral reimplantation
before bladder neck plasty in the reconstruction of bladder
exstrophy: indications and outcomes. Urology. 2003;61(4):820–4.
14. Sponseller PD et al. The anatomy of the pelvis in the exstrophy
complex. J Bone Joint Surg Am. 1995;77(2):177–89.

15. Stec AA et al. Evaluation of the bony pelvis in classic
bladder exstrophy by using 3D-CT: further insights. Urology.
2001;58(6):1030–5.
16. Connolly JA et al. Prevalence and repair of inguinal hernias
in children with bladder exstrophy. J Urol. 1995;154(5):1900–
1.
17. Diamond DA, Jeffs RD. Cloacal exstrophy: a 22-year experience. J
Urol. 1985;133(5):779–82.
18. •• Stec AA et al. Evaluation of pelvic floor muscular redistribution
after primary closure of classic bladder exstrophy by 3-
dimensional magnetic resonance imaging. J Urol. 2012;188(4
Suppl):1535–42. Utilizing safe and modern technology, 3D MRI
imaging provides a new frontier for evaluating exstrophy–
epispadias patients. Not only can specific dimensions of the soft
tissue be associated with specific outcomes, but one may also use
these dimensions to help guide current procedures and techniques.
19. Woodhouse CR, Hinsch R. The anatomy and reconstruction of the
adult female genitalia in classical exstrophy. Br J Urol.
1997;79(4):618–22.
20. • Stec AA et al. Colorectal anomalies in patients with classic
bladder exstrophy. J Pediatr Surg. 2011;46(9):1790–3. Colorectal
anomalies occur in 1.6 % of CBE patients, a 72-fold increase
compared to the general population. Physicians confronted with
CBE should be aware of possible colorectal malformations and
promptly evaluate and treat them.
21. Hurwitz RS et al. Cloacal exstrophy: a report of 34 cases. J Urol.
1987;138(4 Pt 2):1060–4.
22. Silver RI et al. Penile length in adulthood after exstrophy recon-
struction. J Urol. 1997;157(3):999–1003.
23. • Ansari MS et al. The application of pelvic osteotomy in
adult female patients with exstrophy: applications and out-
comes. BJU Int. 2011;108(6):908–12. The six exstrophy–
epispadias patients who had previously undergone bladder
closure presented with uterine prolapse or concerns about
the appearance of their genitalia or abdominal wall. This
study provides details about the possible genitalia presenta-
tions in the exstrophy–epispadias complex that physicians
should be acknowledge.
24. Cadeddu JA et al. Spinal abnormalities in classic bladder
exstrophy. Br J Urol. 1997;79(6):975–8.
25. McLaughlin KP et al. Cloacal exstrophy: the neurological impli-
cations. J Urol. 1995;154(2 Pt 2):782–4.
26. Ebert AK et al. The exstrophy–epispadias complex. Orphanet J
Rare Dis. 2009;4:23.
27. Gearhart JP et al. Criteria for the prenatal diagnosis of classic
bladder exstrophy. Obstet Gynecol. 1995;85(6):961–4.
28. Mirk P, Calisti A, Fileni A. Prenatal sonographic diagnosis of
bladder extrophy. J Ultrasound Med. 1986;5(5):291–3.
29. •• Goyal A et al. Antenatal diagnosis of bladder/cloacal exstrophy:
challenges and possible solutions. J Pediatr Urol. 2012;8(2):140–4.
The prospective study demonstrates that even with classic ultra-
sound characteristics of exstrophy, only 25 % of the newborns
were diagnosed prenatally. Radiologists should be aware of these
characteristics so that more children are diagnosed prenatally
since 5 of 16 pregnancies with suspicion of exstrophy were termi-
nated. MRI and karyotyping are other prenatal diagnostic modal-
ities that can be explored.
30. Nelson CP et al. Bladder exstrophy in the newborn: a
s n a p s h o t of contemporary practice patterns. Urology.
2005;66(2):411–5.
31. Ebert AK et al. Long-term follow-up of male patients after recon-
struction of the bladder-exstrophy–epispadias complex: psychoso-
cial status, continence, renal and genital function. J Pediatr Urol.
2010;6(1):6–10.
32. Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder
exstrophy: a contemporary series. J Pediatr Urol. 2007;3(4):311–5.
Curr Urol Rep
33. Novak TE et al. Failed exstrophy closure: management and out-
come. J Pediatr Urol. 2010;6(4):381–4.
34. Mathews R. Achieving urinary continence in cloacal exstrophy.
Semin Pediatr Surg. 2011;20(2):126–9.
35. Chan DY, Jeffs RD, Gearhart JP. Determinants of continence in the
bladder exstrophy population: predictors of success? Urology.
2001;57(4):774–7.
36. Nelson CP et al. Economic impact of failed or delayed primary
repair of bladder exstrophy: differences in cost of hospitalization. J
Urol. 2008;179(2):680–3.
37. Gearhart JP et al. The multiple reoperative bladder exstrophy
closure: what affects the potential of the bladder? Urology.
1996;47(2):240–3.
38. • Baradaran N et al. Effect of failed initial closure on bladder
growth in children with bladder exstrophy. J Urol.
2011;186(4):1450–4. The authors determined that patients
with failed bladder closures have significantly smaller
cystographic capacities and slower bladder growth compared
to children with successful primary closures. This data high-
lights the importance of succeeding in the closing the defect
for the first time.
39. Oesterling JE, Jeffs RD. The importance of a successful initial
bladder closure in the surgical management of classical blad-
der exstrophy: analysis of 144 patients treated at the Johns
Hopkins Hospital between 1975 and 1985. J Urol. 1987;137(2):258–
62.
40. McMahon DR et al. Vesical neck reconstruction in patients
with the exstrophy–epispadias complex. J Urol. 1996;155(4):1411–
3.
41. • Rubenwolf PC et al. Persistent histological changes in the
exstrophic bladder after primary closure-a cause for concern? J
Urol. 2013;189(2):671–7. Analyzing bladder biopsies, the authors
found inflammatory, proliferative, and metaplastic in CBE patients
undergoing primary bladder closure, secondary reconstructive
procedures, and cystectomy for failed reconstruction. While dys-
plasia or neoplasia were not observes, patients may be at risk for
bladder metaplasia and abnormal urothelial differentiation with a
prolonged exstrophied bladder. Furthemore, these changes may
persist after closure indicating that these patient may be at an
increased risk for dysplasia or neoplasia.
42. Corica FA et al. Intestinal metaplasia is not a strong risk factor for
bladder cancer: study of 53 cases with long-term follow-up. Urol-
ogy. 1997;50(3):427–31.
43. Chalmers D, Ferrer F. Continent urinary diversion in the
epispadias-exstrophy complex. Semin Pediatr Surg. 2011;20(2):102–
8.
44. Woodhouse CR, North AC, Gearhart JP. Standing the test of time:
long-term outcome of reconstruction of the exstrophy bladder.
World J Urol. 2006;24(3):244–9.
45. Surer I et al. Continent urinary diversion and the exstrophy–
epispadias complex. J Urol. 2003;169(3):1102–5.
46. Jeffs RD. Functional closure of bladder exstrophy. Birth Defects
Orig Artic Ser. 1977;13(5):171–3.
47. Novak TE et al. Polyps in the exstrophic bladder. A cause for
concern? J Urol. 2005;174(4 Pt 2):1522–6. discussion 1526.
48. Grady RW, Mitchell ME. Complete primary repair of exstrophy. J
Urol. 1999;162(4):1415–20.
49. Mitchell ME, Bagli DJ. Complete penile disassembly
for epispadias repair: the Mitchell technique. J Urol.
1996;155(1):300–4.
50. El-Sherbiny MT, Hafez AT. Complete repair of bladder
exstrophy in boys: can hypospadias be avoided? Eur Urol.
2005;47(5):691–4.
51. Schaeffer AJ et al. Complete primary repair of bladder
exstrophy: a single institution referral experience. J Urol.
2011;186(3):1041–6.
Curr Urol Rep
52. Husmann DA, Gearhart JP. Loss of the penile glans and/or corpora
following primary repair of bladder exstrophy using the complete
penile disassembly technique. J Urol. 2004;172(4 Pt 2):1696–700.
discussion 1700-1.
53. Alpert SA et al. Bladder neck fistula after the complete primary
repair of exstrophy: a multi-institutional experience. J Urol.
2005;174(4 Pt 2):1687–9. discussion 1689-90.
54. Shnorhavorian M et al. Long-term followup of complete primary
repair of exstrophy: the Seattle experience. J Urol. 2008;180(4
Suppl):1615–9. discussion 1619-20.
55. Hammouda HM, Kotb H. Complete primary repair of bladder
exstrophy: initial experience with 33 cases. J Urol. 2004;172(4 Pt
1):1441–4. discussion 1444.
56. Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immo-
bilization after bladder exstrophy closure: complications and im-
pact on success. Urology. 2003;62(6):1109–13.
57. Gearhart JP et al. A combined vertical and horizontal pelvic
osteotomy approach for primary and secondary repair of bladder
exstrophy. J Urol. 1996;155(2):689–93.
58. Arlen AM et al. Safety and efficacy of spica casts for immobiliza-
tion following initial bladder closure in classic bladder exstrophy. J
Pediatr Urol. 2011;7(4):456–9.
59. Novak TE. Failed exstrophy closure. Semin Pediatr Surg.
2011;20(2):97–101.
60. Sponseller PD et al. Anterior innominate osteotomy in repair of
bladder exstrophy. J Bone Joint Surg Am. 2001;83-A(2):184–93.