Exstrophy-Epispadias

Complex :
current management

J O U R N A L R EA D I NG
OLEH :
Abstract
Bladder exstrophy is a rare malformation
Incomplete closure of the bladder with mucosa continuous with the
abdominal wall, epispadias, and alterations in the pelvic bones and
muscles.
More common in boys
Embryology
The most popular theory explaining the exstrophy–epispadias defect
describes an overgrowth of the cloacal membrane that prevents medial
migration of the mesenchymal tissue.
Anatomical defects
Children with bladder exstrophy have an everted bladder,epispadias,
wide diastasis of the pubic symphysis, and pelvic muscular defects that
result in anterior displacement of the anus and occasionally rectal
prolapse.
 The defects result in an open book configuration of the
pelvis in which the pubic bone is 30% deficient and the pelvic
bones are externally rotated, resulting in an average diastasis
of the pubic symphysis of 4 cm (Fig. 4)
Exsotrophy Complex and
Variant
Three main presentation :
- Classic Bladder Exstrophy
- Cloacal exstrophy
- Epispadias
Prenatal Diagnosis
In a review of 25 prenatal ultrasound examinations with the subsequent
birth of newborn with CBE (Classic Bladder Exsotrophy), Gearhart
(1995) made several observation :
 Absence of bladder filling
 A low-set umbilicus
 Widening pubis ramus
 Diminutive genitalia
 A lower abdominal mass that increased in size as the pregnancy
progressed and as the intra-abdominal viscera increased in size
 A lower abdominal mass
that increased in size as
the pregnancy progressed
and as the intra-
abdominal viscera
increased in size
Surgical reconstruction of
bladder exstrophy
 Sweetser (1952) initially described a staged surgical approach for
bladder exsotrophy.
 The initial staged approach to fuctional bladder closure included
three separated stages :
1. Bladder and abdominal wall closure
2. Bladder neck reconstruction and antireflux procedure
3. Epispadias repair
Evaluation and Management at Birth
• In the delivery room the umbilical cord should be tied with 2-0 silk
close to the abdominal wall so that the umbilical clamp does not
traumatize the delicate mucosa and cause excoriation of bladder
surface.
• the bladder can then covered with non-adherent film or plastic wrap
Cont…
 Cardiopulmonary and general physician assessment measures can be
carried out in the first few hours of life.
 Well-done ultrasound studies are usually sufficient to provide evidence
of renal structure, function, and drainage.
Evaluation and Management at Birth
• Selection of patient for immediate closure
• The size and the functionaly capacity of the detrusor muscle are important
• Correlation between apparent bladder size and the potential bladder
capacity must not be confused

• Small Exsotrophy Bladder unsuitable for newborn closure

• A small, fibrotic bladder patch that is stretched between the edges of the
small triangular fascia defect without elasticity or contractility cannot be
selected for the usual closure procedure (Gearhart and Jeff, 1998)
Evaluation and Management at Birth
•Osteotomy
Pelvic osteotomy performed at the time of initial closure confers several
advantages, including :
1. Easy approximation of the symphysis
2. Placement of the posterior vesicourethral unit deep within the
pelvic ring, enhancing bladder outlet resistance
3. Bringing the large pelvic floor muscle near the midline
 Osteotomy
A : Eight month old patient with classic bladder exstrophy closed at birth without osteotomy
B : Patient after having undergone anterior innominate and vertical iliac osteotomy and placement of external
fixator and pins
C : Same patient 4 months after removal of external fixator and pins
Osteotomy
Complications of Osteotomy and
Immobilization Techniques
• Failure of the closure
• Bladder prolapse
• Loss of suprapubic tubes
• Ureteral stents
 Surgical Option in Newborn
with Classic Bladder Exstrophy
• Warsaw Approach (Baka-Jubiak, 2000)
• Involves closing the bladder, posterior urethra, pubis, and abdominal wall at the time of
primary closure with or without osteotomy but always with appropriate immobilization
• Kelly Repair (Kelly, 1980)
• Multistage repair that includes : (1) closure of the bladder dome and hernia repair at birth,
followed at age 3 to 6 month by (2) reconstruction of the proximal urethra, and (3) repair of
the resulting penoscrotal hypospadias at 3 years of age
• Complete Repair (Grady & Mitchell, 1999)
• Combines the standard bladder closure with the “penile disassembly” technique for
epispadias repair
• Mainz Repair (Hohenfelner, 1964)
• Using ureterosigmoidostomy for both failed exstrophy patients and for patients with small
bladder templates
• Erlangen Approach (Schrott, 1984)
• (1) radical mobilization of posterior vesicouretral unit from surrounding tissue, (2) combined
closure with epispadias repair in only carefully patient (3) tension free closure of abdominal
wall and pelvic bones, (4) definition of strict criteria for selection of newborns to undergo
closure
Modern Reconstruction of Bladder Exstrophy
The primary objective in functional closure is to convert the bladder
exstrophy into a complete epispadias with urethra well up onto the
proximal shaft or midshaft of the penis with combined complete
epispadias repair only in highly selected patients.
Initial closure of bladder and posterior urethra
Initial closure of bladder and posterior urethra
Management after primary closure
o Before the removal of suprapubic tube, 4 weeks after surgery the
bladder outlet is calibrated by urethral cateter to ensure free drainage
o A complete ultrasound exam is performed to ascertain the status of
the renal pelves and ureters
o Appropriate urinary antibiotics are administered because all patient
have reflux after closure
o Cystoscopy and cystography at yearly intervals are used to evaluate the
degree of relux to provide an estimate of bladder capacity
o Should bladder outlet resistance be such that urine is retained within
the bladder and reflux and ureteral dilatation develop with infected
urine, It may be necessary to dilate urethra or to begin intermitten
catheterization.
Penile and urethral closure
in extrophy
Kelly repair
A : The penis has been degloved and pulled down over scrotum
B : Initial incision made into pubis to create a flake of bone, taking care to go down only to the level of the
emergence of the dorsal nerve of the penis.
C : Incision made through the levator medial to the white line
D : once the vessel and bone flake are totally free, the tissue of the penis, bladder neck and urethra are
reconstructed
Mitchell Repair
A : Corporal separation with dissection of urethral plate to complete penile disassembly
B : Tailoring bladder neck in complete primary repair of exstrophy procedure
C : Urethral plate tubularization done with interrupted sutures
D : Reaproximation of pubic symphysis with medial rotation of the corpora and placement of
tubularized urethra underneath corpora
Modified Cantwell-Ransley epispadias repair
A : Initial incision line extending around the urethral plate and the coronal sulcus
B-D : performance of the reversed meatal advanced and granuloplasty procedure to bring the
urethral meatus to the tip of the glans
E : Dissection of the foreskin on the ventral aspect of the penis
F : The corpora are dissected off the urethral plate, and parallel incisons are made into the glans
to create glans wings
Modified Cantwell-Ransley epispadias repair
G : the urethra is tubularized using a continuous running suture
H : Approximation of the corpora using the incision in the corpora if indicated
I : Corpora approximated above the urethra to provide an anatomically ventral location of the
urethra
J : Glans is reconstructed in two layers
 Modified Cantwell-Ransley epispadias repair
K : preparation of skin closure
L : Suture is placed at the base of the penis to locate the foreskin on the shaft of the penis as well as to provide
an area of distinction between the penis and the scrotum
M : Foreskin is sewn to the coronal sulcus
N : Completion of the repair with resurfacing of the penis and use of a proximal Z-plasty incision to provide
downward penile deflection
 Modified Young-Dees-Leadbetter bladder neck
reconstruction
A : Vertical bladder incision with distal transverse extension where the bladder and posterior urethra are under the
pubic bar.
B and C : Ureters are identified, mobilized, and reimplanted in a cephalotrigonal position
D and E : Segments of mucosa are excised from either side of a median strip (1.5 x 3 cm) that will form the neourethra.
Short incisions in the muscle will permit extension of the bladder neck. F, Urethra is tubularized using a continuous
running suture
 Modified Young-Dees-Leadbetter bladder neck
reconstruction
A : Vertical bladder incision with distal transverse extension where the bladder and posterior urethra are under the
pubic bar.
B and C : Ureters are identified, mobilized, and reimplanted in a cephalotrigonal position
D and E : Segments of mucosa are excised from either side of a median strip (1.5 x 3 cm) that will form the neourethra.
Short incisions in the muscle will permit extension of the bladder neck. F, Urethra is tubularized using a continuous
running suture
 Modified Young-Dees-Leadbetter bladder neck
reconstruction
G : Posterior urethra and bladder neck are reconstructed
H and I : Bladder muscle is brought together in a double-breasted fashion over the neourethra
J : Bladder closure is completed and the distal suspensory sutures are tied over the abdominal wall (see inset)
Female Exstrophy
The principles of repair of female and male exstrophy are similar.
However, in the female patient we recommend the following :
1. Radical dissection of the bladder and urethra from surrounding
structures while connection to the vagina is maintained
2. Dissection of the lateral aspects of the posterior vesicourethral and
vaginal unit from their attachments to the pelvic floor
3. Tension-free closure of the abdomen, bladder-urethra, and external
genitalia
4. Judicious use of osteotomy and proper immobilization of the pelvis
and extremities
MODERN INITIAL REPAIR OF BLADDER
EXSTROPHY:
OUTCOMES AND RESULTS

Husmann et al, 1989a; Mergurian et al, 1991; Perlmutter et al,

1991; Franco et al, 1994; McMahon et al, 1996; Chan et al, 2001;
Cole et al, 2003
MODERN INITIAL REPAIR OF BLADDER
EXSTROPHY:
OUTCOMES AND RESULTS

“The most important long-term factor gleaned from a review of all these
series is the fact that bladder capacity at the time of bladder neck
reconstruction is the most important determinant of eventual success”
EXSTROPHY RECONSTRUCTION FAILURES
AND COMPLICATION
 Failed Closure
 failures can manifest as complete bladder dehiscence,
bladder prolapse, neourethral stricture and obstruction, soft-
tissue loss, and vesicocutaneous fistula.
 Failed Bladder Neck Repair
 inadequate outlet resistance, a small bladder capacity (lack of
growth after bladder neck reconstruction), decreased
compliance or a combination of these factors
 Failed Genitourethral Reconstruction
 Common complications of modern epispadias repair include
urethrocutaneous fistula formation
EXSTROPHY RECONSTRUCTION FAILURES
AND COMPLICATION
A1 : Failed female newborn closure.
A2 : Major bladder prolapse after complete primary repair of exstrophy (CPRE) without
osteotomy.
B : Complete dehiscence and loss of right glans and partial loss of corpora after CPRE.
C : Loss of right glans and urethra after penile disassembly in newborn (under testosterone
stimulation before placement of full-thickness (skin graft).
ADOLESCENTS AND ADULTS WITH EXSTROPHY
EPISPADIAS COMPLEX
A S C H I L D R E N W I T H E X S T R O P H Y G R O W I N TO A D U LT H O O D , T H E R E I S A N
INCREASING NEED TO ADDRESS LONG -TERM FUNCTIONAL AND
P S YC H O L O G I C A L A S P E C T S O F D E A L I N G W I T H A M U LT I O R G A N B I R T H
D E F E C T. S O M E A S P E C T S S U C H A S S E X U A L I T Y A N D C O N T I N E N C E A R E
C O M M O N T O A L L F O R M S O F T H E E X S T R O P H Y E P I S PA D I A S C O M P L E X
CONTINENCE
 Early surgical management of patients with bladder exstrophy included
initial cystectomy and replacement with an ileal conduit or
ureterosigmoldostorny. The use of ureterosigrnoidostomy has fallen
into disfavor owing to an increased identification of lethal colonic
cancer (Cobet et al, 2009)
 Renal function, however, was preserved in the majority of patients with
ureterosigmoidostomy, Some institutions have been using continent
catheterizable pouches for bladder management (Nerli et al, 2008)
SEXUALITY
 Male Concerns
 The most often expressed male concerns relate to the length, appearance,
and the axis deviation (chordee) of the penis noted with exstrophy.
 Functional penile length can be gained by degloving the penis and resecting
all of the residual scar tissue and releasing any remnants of the suspensory
ligaments. Skin coverage can be a significant problem after penile
lengthening procedures.

 Female Concerns
 Appearance of the external genitalia, adequacy of the vaginal opening, and
uterine prolapse.
 Although initial correction of the female external genitalia defect is
performed at birth, sometimes "touch-up" surgery needs to be performed at
puberty.
FERTILITY
 Male
 Reconstruction of the male genitalia and preservation of fertility were not primary
objectives in early surgical management of bladder exstrophy
 Regardless of the method of reconstruction of the external genitalia and bladder
neck, newer techniques such as gamete intrafallopian transfer (GIFT) or
intracytoplasmic sperm injection (ICSI) can be used to assist these patients in their
goal of achieving pregnancy
 Female
 The main complication after pregnancy was cervical and uterine prolapse
 Deans and colleagues (2012) stress that pregnancy in women with bladder
exstrophy remains high risk for both the mother and the fetus and stress the need
for referral to a tertiary care center for successful outcomes. In the majority of
cases, planned cesarean section appears to be the safest mode for delivery.
Quality of Life
 The preliminary results of the QUALEX (Quality of Life of Bladder
Exstrophy) study (Jochault-Ritz et al, 2010) suggest that after
reconstruction patients had reduced QoL
 Adolescents reported a superior QoL score as compared with adults
and children.
 Schaeffer and colleagues (2013) confirmed that adolescents with
bladder exstrophy appear to have good QoL scores when compared
with norms; although incontinent patients had a tendency to have
lower QoL scores, the sample size evaluated was inadequate to
demonstrate statistical significance.
LONG-TERM ADJUSTMENT ISSUES
 Children with exstrophy undergo multiple reconstructive surgeries and
have potential problems with respect to urinary incontinence and
sexual dysfunction.
 Incontinence may have a negative impact on social function and self-
esteem.
 Multiple hospitalizations may interfere with the ability to be like other
children

Being born with exstrophy per se does not result in childhood

psychopathology. Children with exstrophy exhibit some tendency
toward increased problems with acting out or lack of attainment of age-
appropriate adaptive behavior (Montagnino et al, 1998; Reiner, 1999)
Key-Points : BLADDER EXSTROPHY PRIORITIES
IN MANAGEMENT
 Size and quality of bladder template
 Extent of pubic diastasis and malleability of the pelvis
 Need for osteotomy
 Length and width of urethral plate
 Penile size
 Associated anomalies
EPISPADIAS
E P I S PA D I A S VA R I ES F ROM A MI L D G L A N U L A R D E F EC T I N A
COV E RE D PE N I S TO T H E P E N OP U B I C VA R I E TY WI TH
CO M P L E T E I N CO N T I N E N C E I N M A L ES O R F E MA L ES . I T I S
M O S T CO MM O N LY N OT E D A S A CO M P O N E N T O F B L A D D ER
A N D C LOA C A L E XS T RO P H Y.
Male Epispadias
• Incidence of 1 in 117,000 males
• Consists of a defect in the dorsal wall
of the urethra
• The displaced meatus may be found
on the glans, on the penile shaft, or
in the penopubic region
Female Epispadias
• Occurs in 1 of every 484,000 female
patients
• Characterized by a bifid clitoris
• The mons is depressed in shape and
coated by a smooth, glabrous area of
skin
• The labia minora are usually poorly
developed and terminate anteriorly at
the corresponding half of the bifid
clitoris, where there may be a rudiment
of a preputial fold
Classification
A : Normal female anatomy
of Davis (1928)
B : Vesicular epispadias-urethra mainly normal but relation to clitoris altered
C : Subsymphyseal epispadias-defect in anterior wall of urethra for about one half its length.
D : Retrosymphyseal epispadias-defect in anterior wall of entire urethra. Sphincter is usually involved.
SYM, symphysis.
Operative technique
A : Typical appearance of female epispadias with initial incisions outlined.
B : Excision of the glabrous skin of the mons
C : Tapering of the urethra with a dorsal resection of a wedge of tissue.
D : Reconstruction of the urethra over a catheter with running suture.
E and F : Medial aspect of the labia minora and clitoris.
G : Initial layer of mons closure.
Operative technique
H : Approximation of the labia minora over the urethral reconstruction.
I : Second layer of mons closure.
J : Creation of clitoral hood.
K : Completion of mons closure.
Surgical Result
Cloacal Exstrophy
 Anatomic Considerations
 Neurospinal abnormalities
 Abnormalities of the spinal cord or vertebral column or both have been noted in 85°/o to
100°/o of children with cloacal exstrophy
 The embryologic basis for the neurospinal defects associated with cloacal exstrophy has
been postulated to be secondary to disruption of the tissue of the dorsal mesenchyme
rather than failure of neural tube closure (McLaughlin et al, 1995)
 Skeletal system abnormalities
 The pubic diastasis noted in the exstrophy epispadias complex are seen at their most
extreme with doacal exstrophy (Sponseller and associates,1995)
 Intestinal tract abnormalities
 Gastrointestinal tract anomalies occur in virtually all patients with cloacal exstrophy. the
incidence of omphalocele was 88 %, and most other series report an incidence of 95 % or
greater
 Genitourinary abnormalities
 Mullerian anomalies have been frequently noted in conjunction with cloacal exstrophy
 Upper urinary tract anomalies occurred in 41 % to 60 %
 Additional system abnormalities
 Life-threatening cardiovascular and pulmonary anomalies are rarely seen in cloacal
exstrophy
Internal view of a patient with cloacal exstrophy.
Pudendal vessels, nerves, and other vessels and autonomic innervations of the corporeal
bodies are demonstrated. Internal structures of the pelvis along with duplication of the vena
cava in this dissected specimen are also shown.
Coding grid used to describe cloacal exstrophy and its variants
B1e : everted bowel
0 : omphalocele
HBLe : hemibladder,
HG : hindgut
HP : hemiphallus.
Prenatal Diagnosis
 Ultrasound diagnosis These authors indicated that the three main
criteria used to identify the diagnosis were a large midline
infraurnbilical anterior abdominal wall defect, lumbosacral
myelomeningocele, and failure to visualize the urinary bladder.
(Meizner and Bar-Ziv, 1985)
 MRI has been used successfully for the prenatal evaluation of cloacal
exstrophy (Chen et al, 2008)
 MRI was able to definitively identify the omphalocele, gender
ambiguity, and spinal defect that were not easily defined with
ultrasonography ( Goto et al, 2012)
Surgical reconstruction
of cloacal exstrophy
Evaluation and
Management at Birth
• Immediate management is directed to
the medical stabilization of the infant.
•Complete physical examination and
determination of the various anatomic
defects present allow short- and long-
term management strategies to be
created (Box 139-1 ).
Gender assignment
Because of the significant separation of the corpora of the penis and scrotum and
the reduction in corporeal size noted in boys with cloacal exstrophy, early reports
had recommended universal gender reassignment of boys ( 46,XY) with cloacal
exstrophy to functional females (Tank and Lindenaur, 1970)

A 46,XY child with cloacal exstrophy with a dominant right hemiphallus.

This child underwent reconstruction and was reared as male.
Immediate Surgical Reconstruction
 Cloacal exstrophy patients should undergo carefully planned and
individualized reconstructions (Ricketts et al, 1991; Lund and Hendren,
1993; Mathews et al, 1998)
 Neonatal omphaloccle closure is recommended to prevent untimely
rupture and is typically combined with intestinal diversion.
 At the initial stage of omphalocele closure, if it is determined that
bladder and abdominal wall closure may not be accomplished, the
bladder halves are approximated in the midline without further
dissection and the defect is converted to a bladder exstrophy (Ricketts
et al, 1991; Mathews et al, 1998)
Urinary Reconstruction
 Modern Staged Reconstruction
The staged management of the urinary tract follows that used for the
management of bladder exstrophy
Reconstruction of the external genitalia in the immediate post natal period is
performed to make the infant's appearance more congruent with the gender
assigned

 Role of Osteotomy
Osteotomy is indicated in all children with cloaca} exstrophy at the time of
bladder closure because of the wide diastasis that is invariably present
(Mathews et al, 1998).

 Single-Stage Reconstruction
 Crady and Mitchell ( 1999) have reported using a single-stage reconstruction
in cloacal exstrophy, a procedure similar to that done for bladder exstrophy
KEY POINTS: CLOACAL EXSTROPHY PRIORITIES
IN MANAGEMENT
• Medical stabilization
• Gender assignment
• Colonic functionalization
• Separation of bladder from the gastrointestinal tract
• Functional genital reconstruction
 Summary
Evolution in management of cloacal exstrophy has permitted near
universal survival with significant improvement in cosmetic and
functional outcomes. Debate continues regarding the issue of
gender reassignment, and long-term data are still accruing on the
best strategy for management. Continence can be achieved with
appropriate reconstruction and the use of intermittent
catheterization.Despite the extensive malformations noted, many
patientshave gone on to live fruitful lives