Management of Bladder

Exstrophy
Bladder Exstrophy
• Rare and severe congenital anomaly
• Characterized by the closure defect of lower abdominal wall and
bladder
• The bladder, bladder mucosa, ureteral orificios, posterior bladder
neck, and urethra are everted through the ventral wall of the
abdomen, between the umbilicus and the symphisis pubis.
• Commonly associated with structural anomalies of the pubic bones.
Exstrophy-Epispadias Complex
A.Cloacal exstrophy
B.Superior vesical fissure
C.Classic bladder extrophy
D.Male epispadia
E. Female epispadias

(2016). Campbell-Walsh Urology, Eleventh Edition. Philadelphia, Elsevier.

Epidemiology
• The prevalence of bladder extrophy was 2.07 per 100,000 births
• Bladder extrophy is twice as common in males than females
• Prevalence increased among older maternal age
Embriology
• The primary defect is dearrangement in midline development that
presents in various forms.
• In epispadias, the fusion of dorsal urethra is not succeed.
• Bladder exstrophy is caused by the failure of the abdominal muscles,
pelvic ring, and pelvic floor musculature to fuse in midline.
• Cloacal extrophy includes the exstrophy of hindgut tube.
 Bladder Exthrophy Goals
Prenatal Diagnosis and Management
• Ultrasound evaluation of the fetus by means of high-resolution real-time units
allows a survey of fetal anatomy even during routine obstetric ultrasonography
• Observations :
• Absence of a fluid-filled bladder
• Low-set umbilicus
• Widening of the pubic rami
• Diminutive genitalia
• Lower abdominal mass that increased in size as the pregnancy progressed and as the intra-
abdominal viscera increased in size

Purves, J. T., & Gearhart, J. P. (2010). The Bladder Exstrophy-epispadias-cloacal Exstrophy Complex. In Pediatric Urology (pp. 386-415). Elsevier Inc.
 Bladder Exsthrophy Goals
• Quality of ultrasound imaging is enhanced by the use of surface rendering to
produce three-dimensional images
• In equivocal cases, MRI has been used to overcome obstacles such as maternal
body habitus, fetal position, and oligohydramnios.
• Prenatal diagnosis of bladder exstrophy  optimal prenatal management (a
pediatric center prepared to handle this complex malformation and appropriate
prenatal counseling of the parents about the nature of the congenital
malformation)

Purves, J. T., & Gearhart, J. P. (2010). The Bladder Exstrophy-epispadias-cloacal Exstrophy Complex. In Pediatric Urology (pp. 386-415). Elsevier Inc.
 Bladder Exthrophy Goals
• Historically, exstrophy patients were treated with cystectomy and
often died at a young age secondary to complications of renal failure
• Repair of bladder exstrophy begins with closure of the bladder and
abdominal wall by either the Modern Staged Repair of Exstrophy
(MSRE) or Classic Primary Repair Exstrophy (CPRE)
• However, if the primary closure fails or if the patient’s bladder
remains small or noncompliant it may require AC with or without a
Continent Urinary Diversion (CUD) to achieve dryness

Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo & Bhavik B. Shah & John P. Gearhart.” Modern Management of
Bladder Exstrophy Repair”. Curr Urol Rep
DOI 10.1007/s11934-013-0332-
Management of Classic Bladder
Exstrophy
Abdominal
and Bladder
Closure

Bladder
Augmentation

Continent
Urinary
Diversion
Modern Staged Repair of Bladder
Exstrophy
Modern Staged Repair of Exstrophy (MSRE)
• Bladder, posterior urethra, and abdominal wall closure in the newborn period with bilateral innominate
and vertical iliac osteotomy,

If indicated :
• Epispadias repair at 6 to 12 months of age; and bladder neck reconstruction with an antireflux procedure at
4 to 5 years old.

When the child has achieved adequate bladder capacity and is motivated to
participate in a postoperative voiding program

Purves, J. T., & Gearhart, J. P. (2010). The Bladder Exstrophy-epispadias-cloacal Exstrophy Complex. In Pediatric Urology (pp. 386-415). Elsevier Inc.
Modern Staged Repair of Bladder
Exstrophy Bladder Exthrophy
Grady and Mitchell Schrott and colleagues Stein and coworkers

• Combining bladder • Bladder closure, ureteral • Ureterosigmoidostomy

exstrophy closure with reimplantation, in the newborn period
epispadias repair in the epispadias repair, and with abdominal wall and
newborn period bladder neck bladder closure.
• With combined bladder reconstruction in the
neck reconstruction and newborn period
epispadias repair when
the child reaches a
satisfactory age for
participation in a voiding
program

Purves, J. T., & Gearhart, J. P. (2010). The Bladder Exstrophy-epispadias-cloacal Exstrophy Complex.
In Pediatric Urology (pp. 386-415). Elsevier Inc.
Complete Primary Repair of Exstrophy
(CPRE).
• CPRE combines primary abdominal wall and
bladder closure with epispadias repair and
partial tightening of the bladder neck.
• The epispadias repair is done by “penile
disassembly,” where the urethral plate is fully
dissected from the corporal bodies
• Potential advantages of CPRE over MSRE may
require longer follow-up with additional
cases, although the rarity of EEC makes this a
significant challenge

Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo & Bhavik B. Shah & John P. Gearhart.”
Modern Management of Bladder Exstrophy Repair”. Curr Urol Rep
DOI 10.1007/s11934-013-0332-
Pelvic Osteotomies and
Immobilization
• Osteotomies may increase surgery time and
risk for postoperative complications
• The use of osteotomies during closure is
associated with improved success of primary
closure by providing a tension-free
approximation of the pubic symphysis and
abdominal wall.
• A combination of bilateral anterior transverse
innominate and vertical posterior iliac
osteotomies has been shown to decrease the
rate of abdominal dehiscence and bladder
prolapse.

Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo & Bhavik B. Shah & John P. Gearhart.”
Modern Management of Bladder Exstrophy Repair”. Curr Urol Rep
DOI 10.1007/s11934-013-0332-
 Bladder Augmentation
• A bladder that is noncompliant or of
insufficient capacity may
undergo Augmentation Cystoplasty
(AC).
• Common techniques utilize segments
of bowel, stomach, or redundant
ureter to expand the bladder wall.

Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo & Bhavik B. Shah & John P. Gearhart.”
Modern Management of Bladder Exstrophy Repair”. Curr Urol Rep
DOI 10.1007/s11934-013-0332-
 Continent Urinary Diversion
• Continent Urinary Diversion (CUD) is typically required when a
patient undergoes Augmentation Cystoplasty (AC).
• A segment of appendix or ileum may be utilized to connect the
bladder to the skin and provide a continent stoma through which to
perform CIC.

Brian M. Inouye & Eric Z. Massanyi & Heather Di Carlo & Bhavik B. Shah & John P. Gearhart.”
Modern Management of Bladder Exstrophy Repair”. Curr Urol Rep
DOI 10.1007/s11934-013-0332-
Concept of Repair

Secure abdominal wall closure

Urinary continence (volitional voiding) with preservation

of renal function

Functionally and cosmetically accepted external genitalia

Concept, Single-stage or Staged ?
Single staged Staged

• Primary defect is anterior abdominal • Proper functional bladder closure is

wall herniation, thus bladder and the cornerstone for successful repair
urethra must be treated as single unit • Maturational delay
• Early reconstruction would allow for • Sensitive for outlet obstruction
mechanical cycling of the bladder,
that enhance restoration of • Multifactorial defect
continence
• Complete repair can be achieved in
single stage
Continence after Exstrophy repair

Continence Renal Damage

Single- Stage Surgery 88% 13-20%

Staged Surgery 65-80% 0

Exstrophy Repair Techniques : Single-
Stage
• Seattle approach by Mitchells (CPRE)
• Best done in newborn period
• Bladder closure, epispadias repair (without BN repair)
• Bladder moved posteriorly within the pelvis
• Epispadias fixed using complete penile disassembly
technique

Docimo S.G., The Kelalis-King-Belman Textbook of Clinical Pediatric Urology, Informa, 2007
Inouye, B.M.,Gearhart, J.P., Modern Management of Exstrophy-Epispadias Complex, Hindawi, 2014
Exstrophy Repair Techniques : Staged
• John Hopkins Approach by Gearhart (MSRE)
• Stage 1 :
• Closure and repositioning of bladder and urethra inside pelvic ring
• Approximation of the pelvic ring with closure of the abdominal wall
• Stage 2: Epispadias repair 12-18 mo, via modified Cantwell-Ransley
technique
• Stage 3: Bladder neck repair 4-5 yrs, via Young-Dees-Leadbetter
bladder neck reconstruction

Docimo S.G., The Kelalis-King-Belman Textbook of Clinical Pediatric Urology, Informa, 2007
Inouye, B.M.,Gearhart, J.P., Modern Management of Exstrophy-Epispadias Complex, Hindawi, 2014
Osteotomy / Symphysiotomy
• Advantages
• Diminishes tension on abdominal wall closure  minimal
dehiscence
• Placement of posterior urethra and bladder neck deep
within pelvic ring  continence
• Bringing of pelvic floor musculature near the midline to
support bladder neck  continence
Osteotomy, with or without?
• Time of surgical reconstruction
• Extent of pubic diastasis
• Malleability of the pelvis
• Size of bladder template
Types of Osteotomy
• Posterior iliac osteotomy, for patients with wider diastasis,
poor mobility, failed prior closure or cloacal exstrophy
• Anterior innominate osteotomy, for primary or failed closure
with good result
• Combined anterior
• Others

Purves, J.T., Gearhart, J.P., Pelvic Osteotomy in The Modern Treatment of th Exstrophy-Epispadias Complex, EAU-EBU Update, 2007
Docimo S.G., The Kelalis-King-Belman Textbook of Clinical Pediatric Urology, Informa, 2007
Young-Dees-Leadbetter bladder neck
reconstruction
• YDL bladder neck plasty and transtrigonal/cephalotrigonal
bilateral ureteral reimplantation  MSRE stage 3
• Modified YDL procedure  bladder neck extensively
dissected + vertical cystostomy

Docimo S.G., The Kelalis-King-Belman Textbook of Clinical Pediatric Urology, Informa, 2007