Focusing on the fetus as the patient, this new edition also brings together a new team of editors whose perspectives on tissue engineering & molecular biology will bring this well into the 21st century To purchase this book please visit www.asia.elsevierhealth.com

1. HISTORICAL PERSPECTIVES
The earliest account of bladder exstrophy can be found on As-
syrian tablets, dating back to 2000 BC. Von Grafenberg first de-
scribed the medical condition in 1597, and Mowat is credited
with providing a complete description of bladder exstrophy in
1748. It was not until 1780, however, that Chaussier first
coined the term “exstrophie.”1 Early management of bladder
exstrophy included the application of an external urinary re-
ceptacle to the surface of the exposed bladder,2 ureterosigmoi-
dostomy,3 transplantation of the bladder trigone into the
rectum,4 and coverage of the exposed bladder with lateral skin
flaps.5 These methods were fraught with continued urinary
incontinence and/or urosepsis. Contributions by Coffey,
Nesbitt, Leadbetter, and Clarke improved the technique of
ureterosigmoidostomy; however, associated morbidities in-
cluded infection, electrolyte abnormalities, and malignancy.6
Complete urinary diversion into the colon or alternate conduit
was preferentially used to provide continence and minimize
infection, but the problems of anatomic reconstruction and
sexual function persisted.
Paralleling efforts to develop improved methods of urinary
diversion were attempts at successful bladder closure. Trende-
CHAPTER 120 lenburg described bilateral sacroiliac osteotomies and the ap-
plication of a pelvic sling to support bladder and abdominal
wall closure in 1892.7 The first case of successful closure
and continence in a female patient with bladder exstrophy
Bladder and Cloacal was not reported until 1942 by Young.8 Michon subsequently
reported successful reconstruction in a male patient 6 years
later.9 Despite these accounts of functional closure, a 1970 re-
Exstrophy view of 329 cases by Marshall and Muecke concluded that
only 19% of patients undergoing total reconstruction had
fair-to-satisfactory results.10 These unfavorable outcomes
Lynn L. Woo, John C. Thomas, and John W. Brock III were reported by others, spurring efforts to improve methods
of surgical repair.11,12
EPIDEMIOLOGY
The authors would like to acknowledge Romano T. DeMarco
and James A. O’Neill, Jr. for their previous contributions to The incidence of bladder exstrophy is estimated at between 1
this chapter. in 10,000 and 1 in 50,000 live births13 with a higher male-
to-female ratio of between 2.3:1 and 4:1.14 Familial recur-
rence is approximately 1 in 100.15 On the basis of a survey
of 2500 indexed cases, familiar occurrence was found to be
Bladder Exstrophy 1 in 275.14 Multiple reports of bladder exstrophy among
------------------------------------------------------------------------------------------------------------------------------------------------
identical twins have demonstrated variability in involvement
Bladder exstrophy is a rare midline defect and exists as part of of one or both twins. Subsequent siblings and the offspring
a larger spectrum of abdominal-pelvic fusion abnormalities, of individuals with bladder exstrophy may be at increased risk
known collectively as the exstrophy-epispadias complex of being affected.14,15 However, no clear pattern of inheritance
(EEC). Presentation of EEC can range from isolated glanular has been characterized and no specific genetic or environmen-
epispadias to cloacal exstrophy, in which several other organ tal factor that predisposes to bladder exstrophy has yet been
systems may be affected. In the case of bladder exstrophy, the identified.
open bladder can be seen everting through a lower abdominal
wall defect. This is accompanied by epispadias, a widened pu-
EMBRYOLOGY
bic diastasis, and an anus that is anteriorly displaced. Over the
past 2 decades, continued improvements in the methods of The underlying embryologic defect shared by bladder exstro-
functional bladder closure have dramatically increased recon- phy and other variants of the EEC is due to abnormal devel-
structive success rates; however, achieving the ultimate goals opment of the cloacal membrane, a bilaminar structure
of adequate bladder capacity, urinary continence, and a good composed of endoderm and ectoderm that overlies the cloacal
cosmetic outcome remain challenging. cavity at the caudal end of the germinal disk.16 In normal
1515

2. 1516 PART VIII GENITOURINARY DISORDERS
Mesonephric
duct
Allantois
Primitive Bladder
urogenital
Phallus
sinus
Mesonephric Perineal
Cloacal duct body
membrane Ureteric bud
Urorectal
Hindgut Anorectal
Urorectal septum Ureter
A septum B canal C
FIGURE 120-1 Division of the cloaca in the urogenital sinus and rectum. A, At the end of the fifth week. B, At 7 weeks. C, At 8 weeks. (Modified from Sadler
TW: Langman’s Medical Embryology, 8th ed. Philadelphia, Lippincott Williams and Wilkins, 2000, p 316.)
development, lateral ingrowth of mesoderm occurs between submucosal tunnel in the bladder wall.22 With continued ex-
the two layers of the cloacal membrane during the fourth posure and chronic inflammation, the exstrophied bladder
and fifth weeks of gestation. This results in formation of the becomes thickened and polypoid (Fig. 120-3). Long-term
lower abdominal wall and pelvis. Subsequent caudal growth exposure may eventually result in a fibrotic, rigid bladder
of the urorectal septum results in its fusion with the cloacal plate that is ultimately unsuitable for closure.
membrane, thus fully separating the cloaca into the bladder
anteriorly and the rectum posteriorly (Fig. 120-1). The paired GENITAL DEFECTS—MALE
genital tubercles, which will give rise to the phallus, migrate
medially to fuse in the midline. Normal perforation of the clo- In the male infant, the open and everted urethral plate can be
acal membrane occurs after fusion with the urorectal septum, seen joining the exposed bladder. The penis is characteristi-
at approximately the sixth week, resulting in formation of cally short with a flattened, everted glans. The prepuce is lo-
separate urogenital and anal openings.17 cated on the penile ventrum (Fig. 120-4). The ejaculatory
Migratory failure of the lateral mesodermal folds and ab- ducts are typically normal and exit at the exposed verumon-
normal overdevelopment of the cloacal membrane have both tanum in the posterior urethra. The base of the penis and scro-
been proposed as potential causes of the prevention of normal tum are widely separated, with lateral displacement of the
mesodermal ingrowth to the cloacal membrane.16,18 The lack corporal bodies and neurovascular bundles. Historically it
of adequate mesodermal reinforcement is thought to result in was believed that the individual corpora were of normal cal-
premature rupture of the cloacal membrane, the timing of iber and appeared shortened because of their attachment to
which determines the extent of the abdominal wall defect the widened pubic diastasis and associated dorsal chordee.
and degree/severity of urogenital tract involvement.19 Rupture
of the cloacal membrane after fusion with the urorectal sep-
tum results in bladder exstrophy, whereas rupture before fu-
sion gives rise to the more severe presentation of cloacal
exstrophy (see later discussion).
CLINICAL PRESENTATION
In general infants with bladder exstrophy are born full term,
without coexisting anatomic anomalies. At birth, an everted
posterior bladder plate of varying size is seen in the midline
of the lower abdomen. The mucosa of the exposed bladder
in the newborn is typically smooth and pink. The umbilical
cord exits from the superior-most border of the bladder plate,
and a small umbilical hernia may be present (Fig. 120-2). In
addition, there is significant widening of the pubic symphysis
and the anus is anteriorly displaced. The levator ani complex
is also divergent, leading to an inherent weakness in the pelvic
floor and a tendency toward rectal prolapse and varying de-
grees of fecal incontinence. Associated inguinal hernias are
common and have been reported in 82% of boys and 10%
of girls.20 The upper urinary tract is usually normal, though
renal anomalies including ectopic, horseshoe, hypoplastic,
dysplastic kidneys, and megaureters may be observed.21 Vesi- FIGURE 120-2 Typical findings of classic bladder exstrophy in a new-
born male. The bladder plate is small, and a small hernia is evident at its
coureteral reflux occurs in the vast majority of children after superior border. The penis is foreshortened, with widely splayed corporal
bladder closure, secondary to an exaggerated lateral course bodies and glanular separation. The urethral plate is short and located on
of the ureters within the pelvis and lack of adequate the anterior surface of the split phallus.

3. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1517
GENITAL DEFECTS—FEMALE
The clitoris is bifid, with divergence of the mons pubis, labia,
and clitoral halves (Fig. 120-6). The urethra and vagina are
shortened, and the introitus is anteriorly displaced. The vag-
inal orifice is often stenotic. The uterus and adnexa are typi-
cally normal, though vaginal and uterine duplication have
been reported.24,25 Uterine prolapse occurs commonly in
female patients, secondary to the inherent weakness in pelvic
floor support.
PELVIC DEFECTS
Some degree of widening of the pubic symphysis is present in
all cases of bladder exstrophy and contributes to outward ro-
tation and eversion of the pubic rami at their junctions with
the ischial and iliac bones (Fig. 120-7). Using computed to-
mography (CT), Sponseller and colleagues further character-
ized the pelvic anatomy of a large group of exstrophy
patients, noting a significantly increased distance between
the triradiate cartilages (31%), external rotation of the anterior
pelvis (18%), and 30% shortening of the pubic rami.26 On the
FIGURE 120-3 When the bladder has been exposed for at least 1 week basis of three-dimensional models generated by CT, Stec and
after birth and the mucosa is subjected to continued exposure and inflam- colleagues observed that among children with exstrophy, the
mation, polypoid excrescences typically appear, as in this female infant. levator ani muscles were more posteriorly positioned and out-
wardly rotated. Furthermore, the puborectal sling had a more
More recently, an MRI-based study by Silver and colleagues of flattened configuration and supported twice the body cavity
adult men with exstrophy and age-matched controls found area in exstrophy patients.27 As mentioned previously, these
that although the length of the posterior corporal bodies pelvic floor defects predispose to pelvic organ and rectal
was the same between groups, anterior corporal length in prolapse in this patient population.
men with exstrophy was nearly 50% shorter than that of con-
trols.23 Therefore the penis appears shortened not only second-
PRENATAL DIAGNOSIS
ary to corporal divergence, dorsal chordee, and abnormal
crural attachments to the corpora cavernosa but also because The use of prenatal ultrasound (US) and MRI has improved
of an inherent deficiency of corporal tissue (Fig. 120-5). The the antenatal diagnosis of bladder exstrophy, allowing for ap-
testes may appear to be undescended, but in most cases they propriate parental counseling and planning of postnatal man-
are actually retractile and will eventually reside in the scrotum agement. The prenatal diagnosis of bladder exstrophy may be
without the need for formal orchiopexy. Should it be required, suggested on US by failure to visualize the bladder in the pres-
orchiopexy is performed in conjunction with inguinal hernia ence of normal kidneys and amniotic fluid.28–30 In a review of
repair. prenatal US studies from 25 women who delivered infants
1
2
3
4
5
6
7
FIGURE 120-4 Classic exstrophy in the male. The penis is pulled downward to expose the dorsal aspect, revealing the urethral plate leading to the
exposed bladder. 1, umbilical cord; 2, bladder mucosa; 3, paraexstrophy tissues; 4, left ureteric orifice; 5, verumontanum; 6, urethral plate; 7, glans penis.

4. 1518 PART VIII GENITOURINARY DISORDERS
2
1
2
11
9
3
FIGURE 120-5 Penile configura-
tion in classic bladder exstrophy. 4
Normal male perineum (A) and with 7
bladder exstrophy (B). Note the loss
of the normal triangular shape of 7 8
8
the perineum and widening of the 6
pubic symphysis. In the setting of
exstrophy, the corpora cavernosa
are widely separated and are intrin- 6
sically shorter. 1, corpus caverno- 9
sum of the penis; 2, glans penis;
3, corpus spongiosum; 4, bul-
bospongiosus muscle; 5, ischium; 5 5
6, ischiopubic ramus; 7, pubis; 8,
ischio-cavernosus muscle and crus 10 10
of penis; 9, urogenital diaphragm;
10, anus and external anal sphincter. A B
with exstrophy, Gearhart and colleagues observed the follow- be accomplished in a multi- or single-stage (complete) repair.
ing features: absent bladder (71%), lower abdominal bulge Multiple contemporary approaches including the modern
(47%) and anteriorly displaced scrotum with small phallus staged reconstruction of exstrophy (MSRE) and complete
in male fetuses (57%), low-set umbilical cord (29%), and primary reconstruction of exstrophy (CPRE) along with War-
abnormal widening of the iliac crest (18%).31 saw,32 Erlangen,24 Mainz,33 and Kelly34 techniques have been
published; however, for the purposes of this review, only the
major principles of MRSE and CPRE are discussed.
SURGICAL RECONSTRUCTION
Surgical management of classic bladder exstrophy consists of
functional closure of the native bladder, closure of the epispa-
dic urethra and genitalia, and creation of a continence mech-
anism to allow for proper urine storage. Reconstruction may
FIGURE 120-6 Typical appearance of classic bladder exstrophy in a fe- FIGURE 120-7 Plain radiograph of a neonate with bladder exstrophy
male. Note the widely divergent labia and anterior displacement of the demonstrates the soft tissue mass effect of the exposed bladder, the wide
vaginal introitus and anus. diastasis of the symphysis pubis, and the posterior rotation of the
acetabula.

5. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1519
INITIAL MANAGEMENT
At birth, the umbilical cord should be ligated with a silk suture
to avoid irritation of the bladder surface from the traditional
plastic clip. The exposed bladder mucosa should be moistened
with saline and protected with a nonadherent sheet of plastic
wrap (e.g., Saran Wrap). A complete physical examination is
performed to rule out associated anomalies and to assess the
size of the bladder plate and extent of the genital defect.
Renal US may be obtained to exclude hydronephrosis and/or
other upper tract abnormalities. Prophylactic antibiotics
should be administered.
MODERN STAGED RECONSTRUCTION
OF BLADDER EXSTROPHY
A three-stage approach for the treatment of bladder exstrophy
was first pioneered by Jeffs and Cendron in the 1970s,35,36
and continued improvements in technique have contributed
to increased success of the procedure.37–39 Stage 1 is per-
formed at birth to protect the upper urinary tracts and assist
later continent reconstruction. It consists of early closure of FIGURE 120-8 Typical findings of classic bladder exstrophy in a female
the bladder, posterior urethra, and abdominal wall with or and the incision template used for repair.
without osteotomy. The primary objective of functional clo-
sure is to convert the bladder exstrophy into a complete epis-
padias.40 Stage 2 is performed in later infancy and involves distally to the verumontanum on both sides of the prostatic
repair of the epispadias, with the goal of optimizing genital urethra, leaving a wide strip of bladder neck and urethral
function and appearance, as well as increasing outlet resis- plate.
tance to promote bladder growth. Stage 3 is undertaken before A major objective of primary closure in the male patient is
school age and consists of bladder neck repair for continence to place the bladder and prostate deep within the pelvis to
and ureteral reimplantation for vesicoureteral reflux. achieve a more normal anatomic position.44,45 In some boys,
Primary functional closure is generally undertaken in the this maneuver results in inadequate length of the urethral
neonatal period, which offers several potential advantages. plate for subsequent penile reconstruction. Duckett therefore
The pliability of the pelvic ring, in infants younger than proposed transection of the urethral plate, distal to the veru-
72 hours old, may obviate the need for osteotomy; early montanum, with the development of lateral paraexstrophic
closure prevents further exposure and scarring of the bladder skin flaps, which could be then rotated medially to bridge
plate; there is theoretically less opportunity for bacterial the gap between the transected edges of the urethra.46 Al-
colonization of the plate with decreased risk of postoperative though this technique allows for better mobilization of the
infection. Alternatively, delayed closure in combination with bladder, Gearhart and colleagues41,47 reported a 40% compli-
pelvic osteotomy may be performed, allowing for patient cation rate associated with the use of paraexstrophy flaps. Ure-
growth and a period of time out of the hospital before thral stricture is the most common complication and may be
reconstruction. secondary to local tissue ischemia. The routine use of such
flaps appears to be decreasing, though their application re-
Stage 1: Functional Closure
mains a viable option when a short urethral plate prohibits
At the time of surgery, the patient should be prepped widely adequate bladder mobilization.48
including the entire body anteriorly and posteriorly below the If the urethral plate is left intact, it should be mobilized to
nipple line so that intraoperative turning is easier. Cardiopul- the level of the prostate to create as much urethral length as
monary monitoring and adequate intravenous access are crit- possible. Following complete mobilization of the bladder,
ical. Intraoperative and postoperative analgesia may be the corpora cavernosa are dissected off the inferior pubic rami
afforded by means of an epidural catheter. The most common bilaterally, taking care to preserve the neurovascular bundles
technique of functional closure is based on descriptions by and avoid penile devascularization. This maneuver aids in pe-
Jeffs and colleagues36,41,42 and Duckett and Caldamone.43 nile lengthening, primarily through release of dorsal chor-
Figure 120-8 depicts the initial incisional template for bladder dee.23 After placement of a Malecot suprapubic tube and
closure in the female infant, and Figure 120-9 details the com- exteriorization of the ureteral catheters, the bladder is closed
plete sequence of stage 1 closure in the male. Traction sutures anteriorly in the midline and the urethra tubularized over a
are placed into the glans penis, and ureteral catheters are se- 10- to 12-Fr sound. The first-stage repair thus results in an
cured in each ureteral orifice. An incision is made around the isolated penopubic epispadias, which is generally incontinent.
periphery of the exstrophic bladder plate, and a plane of dis- Closure of the pelvic ring is required to assist in abdominal
section is established between the rectus fascia and bladder. wall closure. Pubic approximation without ancillary osteot-
The umbilical cord is excised, and umbilicoplasty may be per- omy may be possible in the immediate newborn period, when
formed during or after the initial procedure. Dissection is con- the bones are still malleable; however, osteotomy is generally
tinued toward the pubis, and the incision is then extended required after 3 days of age. Although multiple techniques are

6. 1520 PART VIII GENITOURINARY DISORDERS
A B C D
E F G H
I J K
FIGURE 120-9 Sequence of repair of bladder exstrophy in a male. A, Completion of the dissection around the periphery of the bladder and the urethral
plate. B, Inversion of the bladder plate and approximation of the corpora as a first stage in epispadias repair. Also note the inferior paraexstrophy incisions.
C, Further closure of the skin over the corpora and their partial freeing from the pubis. D, Placement of a suprapubic drainage tube. E, Further closure of the
skin inferiorly, with approximation to the urethral plate. Creation of the paraexstrophy flaps is now evident. F, The urethral plate is prepared for tubular-
ization over a catheter. G, The urethral plate is now tubularized, and ureteral catheters are placed bilaterally and brought out on each side of the bladder.
The bladder is also in the process of being tubularized. H, Completion of tubularization of the bladder and urethra, and location of the various drainage
tubes. I, After two-layer closure of the bladder and urethral plate, the bladder is reduced into the pelvis and fixed with sutures. J, Sutures are placed to
encourage approximation of the pubic halves. K, Drainage tubes are brought out superiorly, and fascia, subcutaneous tissue, and skin are approximated.
Approximation of the pubis helps protect the bladder closure and the abdominal wall closure.

7. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1521
described, combined bilateral anterior innominate and verti- The technique for initial closure in a female patient is sim-
cal iliac osteotomy is most frequently used to assist symphy- ilar to that described previously. The traction suture is initially
seal approximation and medial rotation of the pelvic bones.49 placed anterior to the vagina, which is fully mobilized, as the
Fixator pins are then placed into the iliac wings and lower neourethra is tubularized. The vagina is then repositioned to
osteotomized segments. Our group generally used bilateral create a more caudal angle of entry.
anterior iliac osteotomies (Fig. 120-10). Closure of the pelvic Postoperatively, the patient is maintained on antibiotic pro-
ring is performed using a large-sized, monofilament suture, phylaxis. Parenteral nutrition may be used initially in order to
taking care to place the knot anteriorly to avoid erosion into avoid abdominal distension. Close attention must be given to
the soft tissue below. The newly closed bladder and urethra patient positioning and fixator pin sites to minimize the risk of
can now be covered by reapproximation of the rectus fascia skin ulceration and nerve injury.
and skin, with externalization of tubes and drains. External
Stage 2: Epispadias Repair
fixators are applied to the pins to hold the pelvis in the correct
configuration. Lower extremity traction is applied to keep the The second stage of repair is generally undertaken between
legs still and prevent destabilization of the pelvis (Fig. 120-11). 6 and 12 months of age. It centers on reconstruction of the
The external fixator remains in place for 4 to 6 weeks after phallus, with repair of epispadias and urethroplasty. This
surgery, allowing for callus formation at the osteotomy sites. may further optimize bladder capacity, through an increase
As an alternative to external fixation, immobilization may also in outlet resistance.50 Although many techniques have been
be accomplished through the application of a spica cast, which used, the method described by Cantwell and later modified
envelops the hips and lower extremities. The cast remains in by Ransley has been shown effective in accomplishing urethral
place for 4 to 6 weeks. relocation to the penile ventrum, correction of chordee, and a
A
FIGURE 120-10 A, If the pubis
cannot be approximated in a new-
born or if the procedure is under-
taken later, when the pelvis is less
flexible, pelvic osteotomy is re-
quired. The location of an anterior il-
iac osteotomy is shown. B, External
fixator pins are used to hold the
B pelvis, and the pubic halves are
brought together in the midline.

8. 1522 PART VIII GENITOURINARY DISORDERS
incised transversely at the point of maximum dorsal curvature,
and the corporocavernostomy defects sutured together, thus
covering neourethra. This maneuver places the urethra ven-
trally between the corporal bodies, causes downward deflec-
tion of the penis, and also provides extra length. If chordee is
correctable by simple corporal rotation, bilateral corporo-
tomies are not required. The glansplasty is completed in
two layers, resulting in a ventrally placed neourethra.54 Fur-
ther modifications to the technique of epispadias repair in-
clude the use of full penile disassembly described by
Mitchell and Bagli (Fig. 120-13).55
Stage 3: Bladder Neck Reconstruction
and Ureteroneocystostomy
The final stage of exstrophy repair involves the construction of
a urinary continence mechanism and is generally undertaken
around 4 years of age.56 During this interval, the patient is
monitored periodically with renal US to evaluate the adequacy
of upper tract drainage. Bladder capacity is also assessed be-
fore bladder neck surgery. Jeffs and colleagues57 reported that
a bladder capacity of greater than 60 mL typically allows for
adequate functional storage capacity without the need for
concomitant bladder augmentation, although others have
FIGURE 120-11 An infant in Bryant’s traction. The patient must remain reported median capacities of greater than 85 mL to be more
immobilized for 4 to 6 weeks postoperatively.
predictive of achieving continence.58 Regardless, it is generally
agreed that continence is highly dependent on the size of the
low fistula rate (Fig. 120-12).51,52 This technique involves full original bladder plate, successful initial bladder closure, and
mobilization of the corpora and dorsal urethral plate, which is an adequate preoperative bladder capacity.
dissected free and tubularized. Correction of dorsal chordee is The Young-Dees-Leadbetter technique of bladder neck re-
achieved by incising each corpora transversely, creating pair remains the most common approach to bladder neck
diamond-shaped defects and anastomosing their dorsal me- reconstruction (Fig. 120-14). After opening the bladder, the
dial aspects over the tubularized urethra. A ventral meatotomy ureters are first mobilized and reimplanted into a more ceph-
is then performed at the tip of the glans to produce a more alad position by either cross-trigonal or cephalotrigonal ure-
anatomically normal meatal position.53 Other described teroneocystotomy.22,59 This procedure not only corrects
modifications of the Cantwell-Ransley technique include full vesicoureteral reflux, which occurs in virtually all cases of
detachment of the urethral plate from the corporal bodies, closed bladder exstrophy, but also allows for creation of pos-
leaving only the distal-most 1 cm of urethra attached to the terior bladder plate flaps for the bladder neck reconstruction.
glans tip. The urethra is then tubularized, the corpora are A recent report by Braga and colleagues also describes
A B C
FIGURE 120-12 Steps in the Cantwell-Ransley epispadias repair. A, The urethral plate is dissected from the corpora and is tubularized, taking care to
preserve the lateral blood supply of the urethra and the neurovascular bundles. B, Corporotomies are created at the midphallus, and the urethra is trans-
posed to the ventral surface. C, The corpora cavernosa are rotated medially and reapproximated at the corporotomy sites, pulling the corporal bodies
inward and providing coverage of the neourethra. This procedure permits further urethral lengthening, approximation of the corpora with preservation
of the blood supply, and full coverage of the urethra.

9. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1523
bladder, bladder neck, and urethra. As described by Grady
and Mitchell,60 CPRE begins with intubation of each ureteral
orifice with ureteral catheters. Traction sutures are placed into
each hemiglans, the bladder plate is circumscribed, and dis-
section is continued inferiorly along the ventral aspect of
the penis. The urethral plate is mobilized off the penis, which
is fully disassembled into separate right and left corporal bod-
ies and the spongiosum-containing urethra. The intersymphy-
seal band is incised (Fig 120-15), which allows the bladder
unit to be positioned deep within the pelvis. A suprapubic
tube is left in place, the ureteral catheters are externalized,
and the bladder is closed. Similar to the staged approach, pel-
vic osteotomy may be required for abdominal closure. The pu-
B bic symphysis is approximated using PDS sutures, and the
abdominal wall is closed. The urethral plate is tubularized
and transposed to the penile ventrum, and the corporal bodies
A are rotated medially and reapproximated (Fig. 120-16).
Because of the new posterior positioning of the bladder unit,
urethral length is often inadequate to reach the glans, and
a hypospadiac meatus is left for future reconstruction
(Fig. 120-17). Penile shaft coverage is achieved through the
use of ventral rotational penile skin flaps. Postoperatively,
the patient remains immobilized as previously described in
the technique of staged closure. Additional procedures to cor-
rect for residual hypospadias, vesicoureteral reflux, and
incontinence may be required as the child grows.
URINARY DIVERSION
Urinary diversion, in the form of a bowel conduit or reservoir,
may ultimately be required for patients with insufficient blad-
C D der plate or after reconstructive efforts have been unsuccessful
and is discussed in Chapter 118.
FIGURE 120-13 Complete penile disassembly technique. A, The corpo-
ral bodies and the hemiglans are separated. B, The urethra is tubularized
and moved ventrally. C, The corpora are reapproximated dorsally. D, Glans OUTCOMES AND COMPLICATIONS
closure is performed distally to complete the repair. (Modified from
Mitchell ME, Bagli DK: Complete penile disassembly for epispadias repair: The most devastating complication of bladder closure is dehis-
The Mitchell technique. J Urol 1996;155:300.) cence. Major contributing factors include wound infection,
abdominal distension, bladder prolapse, and loss of ureteral
successful bilateral ureteral reimplantation at the time of pri- and/or suprapubic catheters within 6 days of closure.61 Uri-
mary bladder closure.33 nary diversion, reclosure of the bladder as a urethral tube
A strip of posterior bladder plate 2 cm  4 cm is marked for later augmentation, or delayed repair of the bladder may
off, and the triangles of bladder laterally are demucosalized. be performed. If not performed in the initial setting, pelvic
The strip of bladder is tubularized, and the triangles of de- osteotomy is frequently necessary for successful reclosure.
nuded muscle are mobilized laterally to provide coverage of Urinary incontinence remains a significant problem for up
the neourethra. If prior urodynamic evaluation has demon- to 30% of bladder exstrophy patients. In the case of bladder
strated inadequate bladder capacity, augmentation cystoplasty neck incompetence, injectable bulking agents, bladder neck
with a bowel segment may be performed at this setting. sling or artificial urinary sphincter have all been applied. Blad-
der neck reconstruction or formal closure of the bladder neck,
with the creation of a catheterizable channel, can also be per-
SINGLE-STAGE RECONSTRUCTION: formed. In cases where incontinence is secondary to insuffi-
COMPLETE PRIMARY REPAIR OF EXSTROPHY cient bladder capacity, augmentation cystoplasty remains the
most viable treatment option.
Recently, Mitchell and Grady minimized the number of re- Following epispadias repair, the most common complica-
quired operations by combining bladder closure with epispa- tion is urethrocutaneous fistula, which ranges from 2% to
dias repair at birth in a technique known as complete primary 26% in modern series.51,54,62
repair.60 Major potential benefits of this approach include the The incidence of adenocarcinoma of the bladder in adults
earlier creation of bladder outlet resistance, theoretically lead- with bladder exstrophy has been estimated to be 250 times that
ing to normal cycling and improved bladder capacity and of the normal population and is likely due to chronic inflam-
functionality as the patient grows. Major principles of CPRE mation, infection, and metaplasia of an exposed bladder
include total penile disassembly and division of the intersym- plate.63 A series by Woodhouse and colleagues, however, re-
physeal band, which enables posterior positioning of the cently documented an 800-fold risk in the incidence of bladder

10. 1524 PART VIII GENITOURINARY DISORDERS
A
Bladder
Suspensory sutures
Urethra
Lateral view
FIGURE 120-14 Young-Dees-
Leadbetter procedure for bladder
exstrophy repair. A, The ureters are
appropriately reimplanted to avoid
reflux. Triangular areas at the blad-
der base are then denuded, and
the remaining muscle is tubularized
over a catheter. This serves to
lengthen the urethra and provides
sufficient pressure to encourage
the development of improved blad-
der capacity without causing ure-
thral obstruction. B, The bladder is
reclosed, and the bladder neck is
further supported by sutures, which B
secure it to the pubis anteriorly.
malignancy among those with a history of bladder exstrophy by Bladder augmentation is preferred in patients without ade-
age 40.64 The development of adenocarcinoma and transitional quate bladder capacity, and bladder neck closure with creation
cell carcinoma of the bladder is also a potential risk in those of a continent catheterizable stoma may be performed when
patients who have undergone augmentation cystoplasty.65,66 other continence procedures have failed.
Fertility in patients with bladder exstrophy and epispadias
was studied by Shapiro and colleagues,14 who surveyed 2500
patients. Among these, 38 men had successfully fathered chil- Cloacal Exstrophy
dren and 131 women had given birth. Diminished fertility ------------------------------------------------------------------------------------------------------------------------------------------------
rates among males may be secondary to retrograde ejaculation, Cloacal exstrophy is a rare condition occurring in 1 of 200,000
though libido and erectile function appear to be normal to 400,000 live births68 and comprises the most severe defor-
according to a report by Woodhouse and colleagues.67 Female mation along the EEC spectrum, which includes both epispa-
patients face a significant risk of uterine prolapse. dias and classic bladder exstrophy. Cloacal exstrophy is also
referred to as the OEIS complex (omphalocele, exstrophy, im-
CONCLUSION perforate anus, and spinal defect) when other malformations
of the urogenital, gastrointestinal, skeletal, and neurospinal
Contemporary reconstructive techniques for the repair of axis are present.
bladder exstrophy have resulted in acceptable function and Although first described by Littre in 1709, historic survival
cosmesis for the majority of patients with classic bladder rates were dismal secondary to sepsis or fluid, electrolyte, and
exstrophy. Overall continence rates range from 70% to 80%. nutritional derangements from short gut syndrome or

11. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1525
Cross section
Correction of Exstrophy
Plane of Cross section
Incision through
perineal membrane
Bladder
Symphysis
Prostate
Urethra
Corpus spongiosum
Perineal
membrane
Sup. Transverse
perineal muscle
Anus
FIGURE 120-15 Pelvic view of male exstrophy repair as described by Grady and Mitchell. Aggressive dissection along each side of the urethra and division
of the intersymphyseal band allow posterior positioning of the bladder in the pelvis. (Modified from Grady RW, Mitchell ME: Complete primary repair of
exstrophy. J Urol 1999;162:1416.)
intestinal obstruction.69 The first successful repair was given no embryologic stage similar to cloacal exstrophy exists
reported in 1960 by Rickham, who recommended staged sur- in normal development.76 Disruption of the cloacal membrane,
geries for reconstruction.70 Advances in neonatal care and sur- as the principle underlying abnormality, has been supported by
gical technique have resulted in present-day survival rates that surgically induced exstrophy in animal models.19,77 The pre-
exceed 90%, and principle goals of treatment are now directed vailing developmental theories are further clouded by several
toward improving quality of life in these patients.68,71–75 recent reports documenting rupture as late as 26 weeks.78,79
Rupture at 5 weeks gestation, as traditionally postulated, would
cause anterior herniation of the bladder and small bowel, which
EMBRYOLOGY AND GENETICS
would prevent normal midline fusion of the hindgut, bladder
The underlying defect in cloacal exstrophy is thought to be re- ¨
plate, genital tubercles, and mullerian ducts, thus resulting in
lated to abnormal development and premature rupture of the the typical anatomic presentation of two open bladder halves
cloacal membrane, as described earlier in the bladder exstro- separated by a strip of exstrophied cecum, hemiphallic halves
phy section. In the setting of cloacal exstrophy, it has been pos- with a widely separated pubic diastasis, an underdeveloped
tulated that membrane rupture occurs within the first 8 weeks and blind-ending distal hindgut with imperforate anus, and
of gestation. Confirmation of this theory is difficult, however, an omphalocele of varying size (Fig. 120-18).
A B
FIGURE 120-16 A, Closure of the urethral plate and bladder as a continuous unit. B, Placement of the urethra ventral to the corporal bodies by posi-
tioning the bladder, bladder neck, and urethra posteriorly in the pelvis. (Modified from Grady RW, Mitchell ME: Complete primary repair of exstrophy. J Urol
1999;162:1417.)

12. 1526 PART VIII GENITOURINARY DISORDERS
ASSOCIATED ANOMALIES
Unlike classic bladder exstrophy, cloacal exstrophy is typically
associated with a variety of other anatomic defects (Table 120-1).
GASTROINTESTINAL
Ileocecal exstrophy with an associated omphalocele, hindgut
remnant, and imperforate anus is the most common clinical
presentation.54 Omphaloceles are present in 88% to 100%
of infants and generally contain portions of small bowel
and/or liver.54,89 Other findings include intestinal duplication
anomalies, gastroschisis, ectopic anus, colonic exstrophy, and
malrotation.90,91 Short gut syndrome may be a significant
source of morbidity among patients with cloacal exstrophy
and is observed in 25% of cases.71 The risk of short gut
syndrome is markedly increased in patients subjected to
ileostomy placement as the initial intestinal diversion proce-
FIGURE 120-17 At completion of complete primary closure, placement dure.92,93 Furthermore, the phenomenon may occur even
of the bladder and urethral units deeper within the pelvis along with ven-
tral transposition of the urethra may result in a hypospadiac meatus, which
in the presence of normal bowel length, implicating an inher-
can later be reconstructed with formal urethroplasty. ent absorptive abnormality of the intestine.71,72,90
No single environmental exposure or consistent genetic de-
GENITOURINARY
fect in the etiology of cloacal exstrophy has yet been identified.
Thauvin-Robinet and colleagues recently identified an unbal- Abnormalities of the upper urinary tract have been reported in
anced translocation between chromosomes 9q and Yq, and other 41% to 66% of cases.72,89 Unilateral renal agenesis, pelvic kid-
studies have implicated mutations in homeobox genes such ney, and/or hydronephrosis were observed in up to 33% in
as HLXB9 and HOX, which are involved in the development Diamond’s 1990 series.89 Less commonly reported findings
of embryonic mesoderm.80–82 Although there have been multi- include horseshoe kidney, fusion anomalies, and ureteral ab-
ple reports of cloacal exstrophy among members of the same normalities.72,89,90 Complete separation or even absence of
family, these are generally anecdotal and have involved multigen- the phallic/clitoral halves may be observed, and the scro-
erational relatives or nontwin siblings.83–85 Multiple instances of tum/labia are widely separated. Male infants frequently have
affected monozygotic twins have been reported, however, undescended testes with associated bilateral inguinal hernias,
which lends support to an underlying genetic cause.76,86–88 ¨
whereas failure of mullerian duct fusion in females results in
varying degrees of uterine and vaginal duplication anomalies
in the majority of patients.72,89
TABLE 120-1
Anomalies Associated with Cloacal Exstrophy
Gastrointestinal
Omphalocele
Imperforate anus, anal atresia/stenosis
Short gut syndrome
Intestinal malrotation
Intestinal duplication
Genitourinary
Unilateral renal agenesis
Pelvic kidney
Ureteral duplication
Hydronephrosis
Bilateral cryptorchidism, inguinal hernias
Uterine duplication
Vaginal duplication
Central Nervous System
Spinal dysraphism
Skeletal
Vertebral (absent, extra, hemi)
Club foot
FIGURE 120-18 Typical presentation of cloacal exstrophy in a male in- Other lower limb (absence, shortening)
fant. O, omphalocele; hB, hemibladder; Ce, exstrophied cecum; hP, hemi- Hip subluxation
phallus; hS, hemiscrotum; I, prolapsed ileal segment.

13. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1527
CENTRAL NERVOUS SYSTEM
SURGICAL REPAIR
Some form of spinal dysraphism including tethered cord,
Immediate Postnatal Management
myelomeningocele, or lipomyelomeningocele is present in
nearly all patients, with recent reports ranging from 64% to After delivery and stabilization of the newborn, exposed or-
100%.74,84,91,94 Neurologic impairment is variable and may gans and mucosal surfaces including the omphalocele, blad-
affect bladder function, urinary continence, lower extremity der, intestine, and myelomeningocele should be protected
movement, and erectile function. Detailed postmortem micro- by enclosing the infant’s lower torso in a bowel bag or by first
dissection studies have demonstrated both aberrant origin and moistening surfaces with saline and covering with sterile plas-
vascular supply of the pelvic autonomic nerves,95 and these tic wrapping.90 Urologic examination should attempt to note
nerves are at additional risk of iatrogenic injury during oper- genetic sex and size of hemibladder plates. Baseline renal
ative repair. Other reported abnormalities include periventri- function, electrolyte, and hematologic status should be deter-
cular leukomalacia, hydrocephalus, hypoplastic cerebellum, mined. Karyotyping can be performed if gender has not been
and Chiari malformation.84 previously determined or is not obvious on examination. Ini-
tial imaging should include plain films of the chest and spine
along with head, abdominal, renal, and spinal US. In the ab-
sence of obvious spinal dysraphism, magnetic resonance im-
aging (MRI) may be advisable for detection of occult lesions.
SKELETAL
Consultation should also be made to general surgery, neuro-
Abnormalities of the spine, pelvis, and limbs have all been ob- surgery, and orthopedics for operative planning. Once the ini-
served in the setting of cloacal exstrophy. Spinal anomalies, ex- tial evaluation has been completed, discussion may be had
cluding myelodysplasia, have been reported in 22% to 60% with the parents regarding gender assignment, surgical recon-
and consist mainly of absent or extra vertebrae, scoliosis, struction, potential functional deficits, and overall expected
and kyphosis.90,96,97 The pelvic deformity is similar to that quality of life.
of classic bladder exstrophy but typically more severe with sig-
nificant widening of the pubic diastasis, external angling of the
posterior and anterior segments, and external rotation and ab- Principles of Repair
duction of the iliac wings.70 A review by Jain and Weaver The surgical management of cloacal exstrophy is typically un-
found a 17% to 26% incidence of associated lower limb abnor- dertaken in the newborn period (48 to 72 hours) as a com-
malities.98 Certain limb malformations like club foot and equi- bined effort between pediatric surgery and urology. In the
novarus deformities can be seen in association with setting of associated spinal dysraphism, neurosurgical consul-
myelomeningocele, which often accompanies cloacal exstro- tation and closure should be undertaken as soon as the infant
phy; however, a variety of true limb malformations including is medically stable. Early operation minimizes bacterial colo-
hypoplasia, absence, split foot, and ectopic or additional digits nization of exposed viscera and may decrease the need for pel-
have also been observed.98 vic osteotomy.68,104 The traditional approach of staged repair
has been thoroughly described by Gearhart and Jeffs.40,94
Complete primary repair has also been reported by Howell
and colleagues,68 Zderic and colleagues,105 Hendren,106
PRENATAL DIAGNOSIS and most recently by Mitchell and Plaire.75 It is generally
agreed that an individualized approach toward reconstruc-
Early prenatal diagnosis allows time for thorough parental tion, whether in a single-staged or multistaged procedure, re-
counseling and allows for consideration of pregnancy termi- sults in the best long-term outcomes.107 The main goals of
nation. Prenatal diagnosis was first reported by Meizner and reconstruction include secure abdominal wall and bladder
Bar-Ziv in 1985,99 and since then, several authors have pro- closure, preservation of renal function, prevention of short
posed criteria for the prenatal diagnosis of cloacal exstrophy. gut syndrome, creation of functionally and cosmetically ac-
Principle findings include failure to visualize the urinary ceptable genitalia, and attainment of urinary and fecal
bladder along with a large midline anterior abdominal wall continence.73,94
defect and/or lumbosacral myelomeningocele.87,100–102 The Although various operative algorithms have been published,
prolapsed ileal segment, which may appear as an “elephant all approaches begin with initial separation of the intervening
trunk–like” mass on US, has also been reported as a path- cecal plate from the two bladder halves, closure of the ompha-
ognomic finding.103 From a review of 22 cases, Austin and locele, and hindgut preservation (Fig. 120-19).68,71,72,75,107
colleagues102 developed a list of major and minor criteria In the past, the bowel was initially diverted through the creation
for prenatal US diagnosis on the basis of the frequency with of loop or end-ileostomies, and the hindgut segment was uni-
which abnormalities were observed. Major criteria were those formly discarded. This practice has since fallen out of favor in
seen in greater than 50% of cases and included nonvisualiza- order to maximize the absorptive capabilities of the intestinal
tion of the bladder (91%); a large, midline, infraumbilical tract.72,74 Currently, after tubularization of the exstrophied ce-
anterior abdominal wall defect or cystic anterior abdominal cum, it is recommended that the hindgut segment be brought
wall structure (82%); omphalocele (77%); and myelomenin- out as an end-colostomy.92 In the rare instance when the hind-
gocele (68%). Minor criteria were observed in less than 50% gut remnant is not used, it may be left as a mucous fistula for
and consisted of lower extremity defects (23%), renal anom- use in future urologic or vaginal reconstruction.90
alies (23%), ascites (41%), widened pubic arches (18%), nar- The omphalocele is reduced to assist abdominal wall clo-
row thorax (9%), hydrocephalus (9%), and single umbilical sure; however, in cases of large omphaloceles, complete initial
artery (9%).102 reduction may not be possible. In this setting, a silo device

14. 1528 PART VIII GENITOURINARY DISORDERS
Ileum
Colon
A B C
FIGURE 120-19 Repair of cloacal exstrophy. A and B, The bowel and bladder mucosa are separated, and the ileocecal junction is tubularized and brought
out as an end-colostomy. The bladder halves are turned in, as in a complete exstrophy repair, with approximation of the pubic rami. C, Alternatively, the
bladder halves are approximated in the midline and left open for staged repair if tubularization is not possible. The omphalocele is also closed.
may be used or the omphalocele may be allowed to re- inherent preference toward male behaviors and sexual identi-
epithelialize, converting it to a ventral hernia, which may be ties in these patients.75,105,109 It remains a topic of continued
repaired at a later time (Fig. 120-20).94 study and debate. Gender reassignment has since been largely
The hemibladders are dissected and then reapproximated abandoned in the current management of cloacal exstrophy,
in the midline. In infants with few other associated malforma- though functional and aesthetic phallic reconstruction re-
tions and who are medically stable, complete closure of the ab- mains challenging. Vaginal reconstruction is necessary in fe-
dominal wall, bladder, and phallic halves may be undertaken at males and in gender-reassigned males and is accomplished
this point in a single-stage procedure with or without pelvic through the use of bowel or skin grafts.
osteotomy. If this is not possible at the initial setting, the two Gastrointestinal reconstruction, in the form of a pull-
bladder halves can first be joined in the midline, recapitulating through procedure, may be performed in select patients, some
the appearance of classic bladder exstrophy, which can then be time after initial diversion and abdominal closure. The deci-
repaired in a staged fashion as described in the previous section. sion is based on the potential for fecal continence and may
Genital reconstruction consists of bringing the phallic be influenced by colonic length, ability to form solid stool,
halves together to create an appearance congruent with the and the presence of anal stenosis versus imperforate anus.
assigned gender. In the male infant with cloacal exstrophy, Like those with classic bladder exstrophy, these patients
the phallic halves are characteristically diminutive, widely will also require the creation of antireflux and urinary conti-
separated, and asymmetric. Historically, genetically male in- nence mechanisms. The presence of myelodysplasia in these
fants were routinely assigned to female gender at the time of patients usually necessitates augmentation cystoplasty with
initial closure, undergoing orchiectomy and feminizing geni- a bowel segment and intermittent catheterization in order to
toplasty.108 Recent data regarding gender identity outcomes in achieve continence. Continence procedures include creation
gender-reassigned cloacal exstrophy patients has suggested an of a neourethra, construction of a catheterizable abdominal
stoma with concomitant bladder augmentation, and/or blad-
der neck closure, the selection of which is influenced by the
presence of short gut syndrome, manual dexterity, degree of
mobility, and patient motivation.94
POSTOPERATIVE CONSIDERATIONS
Given the high incidence of short gut syndrome, fluid and nu-
tritional status must be carefully monitored and the initial use
of total parenteral nutrition (TPN) is advocated.110 The keys to
postoperative success are similar to those for repair of classic
bladder exstrophy. Patients are immobilized in some type of
traction device. In the setting of pelvic osteotomy, an external
fixator is left for 4 to 6 weeks postoperatively. Broad-spectrum
antibiotics are administered to minimize risk of wound in-
fection and urosepsis. In contrast to patients with classic
bladder exstrophy, the presence of associated myelodysplasia
in cloacal exstrophy generally precludes use of an epidural
catheter. Pain control in cloacal exstrophy patients can be
FIGURE 120-20 If initial closure of the omphalocele is not possible, it
challenging, and the involvement of the pediatric pain service
may be allowed to epithelialize, converting the omphalocele to a ventral is recommended. Finally, the importance of limiting abdomi-
hernia, which can be repaired at a later time. nal distension to ensure successful abdominal closure and

15. CHAPTER 120 BLADDER AND CLOACAL EXSTROPHY 1529
adequate drainage of both ureteral and bladder catheters medical and surgical management continue to improve func-
cannot be understated.61,111,112 Following repair, close mon- tional and quality of life outcomes in these patients, but it is
itoring of the upper tracts by US is mandatory to observe for important that these individuals remain under the care of a
adequate renal growth and to detect evidence of obstruction multidisciplinary team of providers who can offer medical
or VUR, which has been reported in 50% to 60% of cloacal care, psychologic support, and lifelong follow-up.
exstrophy patients after staged or complete primary
repair.60,107 The complete reference list is available online at www.
expertconsult.com.
CONCLUSION
For the past 20 years, survival among patients with cloacal
SUGGESTED READINGS
exstrophy has exceeded 90%.68,72,74,105 Death is typically re-
lated to complications related to extreme prematurity, renal ¨
Ebert AK, Reutter H, Ludwig M, Rosch WH. The exstrophy-epispadias com-
agenesis, or other complex malformations that are incompat- plex. Orphanet J Rare Dis 2009;4:23.
Gearhart JP, Mathews R. The Exstrophy-Epispadias Complex: Research Con-
ible with life. It is interesting to note that cardiovascular anom- cepts and Clinical Applications. New York: Kluwer Academic Publishers;
alies are rarely observed in the setting of cloacal exstrophy. The 1999.
various complications related to the management of patients Hernandez DJ, Purves T, Gearhart JP. Complications of surgical reconstruction
with cloacal exstrophy are similar to those of patients with of the exstrophy-epispadias complex. J Pediatr Urol 2008;4:460–466.
Husmann DA. Surgery insight: Advantages and pitfalls of surgical techniques
classic bladder exstrophy, as described in the previous section.
for the correction of bladder exstrophy. Nat Clin Pract Urol 2006;3:
Compared with those with classic exstrophy, however, cloacal 95–100.
exstrophy patients face additional challenges of achieving Ludwig M, Ching B, Reutter H, Boyadjiev SA. Bladder exstrophy-epispadias
bowel and bladder continence secondary to the need for anal complex. Birth Defects Res A Clin Mol Teratol 2009;85:509–522.
reconstruction and the associated defect of spinal dysraphism. 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:
It must be stressed that multiple operations are the rule, and 244–249.
these patients will likely face significant medical, psychologic, Woo LL, Thomas JC, Brock JW. Cloacal exstrophy: A comprehensive review of
and social challenges throughout their lives. Advancements in an uncommon problem. J Pediatr Urol 2009 Oct; (Epub ahead of print).