The document discusses the exstrophy-epispadias complex, which results from abnormal cloacal development. It is caused by failure of the cloacal membrane to be reinforced by mesodermal ingrowth. The complex includes classic bladder exstrophy and other variants. Reconstruction involves bladder, abdominal wall, and urethral closure in newborns. Osteotomies may be required to approximate the pubic bones. Epispadias repair is usually done later, along with bladder neck reconstruction and antireflux procedures to achieve urinary control.

1. Ex0strophy –epispadias
complex
Dr Anchal
dnb resident
MMHRC

2.  The exstrophy-epispadias complex of
genitourinary malformations is heterogenous
group of clinical entity resulting from abnormal
cloacal development.
 The cause of this is thought to be the failure of
the cloacal membrane to be reinforced by
ingrowth of mesodermis .

3.  The first account of bladder exstrophy was
ascribed to Assyro-Babylonian sources dating
from the first and second millennia
 The first recorded case of epispadias is
attributed to the Byzantine Emperor
Heraclius (AD 610-641) and the first
description of bladder exstrophy to Schenck in
1595

4. Incidence and Inheritance
 The incidence :1 in 10,000 to 1 in 50,000
(Lattimer and Smith,1966) live births
 The male-to-female ratio is 5 : 1 to 6 : 1 .

5. Risk factors
 The risk of bladder exstrophy in the
offspring of individuals with bladder
exstrophy and epispadias is 1 in 70 live
births.
 Bladder exstrophy tends to occur in infants of
younger mothers,

6.  a 10-fold increase in exstrophy births to mothers
who had received large doses of progesterone in
the early part of the first trimester
 Assisted reproductive techniques is a risk factor.

7. Embryology
 Bladder exstrophy, cloacal exstrophy, and
epispadias are variants of the exstrophy-epispadias
complex .
 The theory of embryonic maldevelopment
in exstrophy held by Marshall and Muecke
(1968) is that the basic defect is an
abnormal overdevelopment of the cloacal
membrane, which prevents medial
migration of the mesenchymal tissue and
proper lower abdominal wall development.

8.  The timing of rupture of this defective membrane
determines the variant of the exstrophy-epispadias
complex that results
 Classic exstrophy accounts for more than 50% of
the patients born with this complex

10. OTHER THEORIES
 failure of one or both of the lateral body wall
folds to move far enough ventrally to meet its
counterpart in the midline (Sadler and
Feldkamp, 2008).
 Abnormal development of the genital hillocks
caudal to the normal position.

11. There may be involvement of the allantois in the
development of cloacal exstrophy (Zarabi and
Rupani, 1985).
 lack of “rotation” of the pelvic ring primordium
prevents structures attached to the pelvic ring
from joining in the midline, allowing herniation
of the bladder to occur

12. ENTITIES COMPRISING EXSTROPHY
–EPISPADIAS COMPLX

15. CLASSIC BLADDER EXSTROPHY

16. Skeletal Defects
 classic bladder exstrophy have a mean
external rotation of the posterior aspect of
the pelvis of 12 degrees on each side,
retroversion of the acetabulum, and a mean
18 degrees of external rotation of the
anterior pelvis, along with 30% shortening
of the pubic rami, and diastasis of the
symphysis pubis .

18.  The sacrum in exstrophy has a 42.6% larger
volume and 23.5% more surface area than in
controls
 These rotational deformities of the pelvic skeletal
structures contribute to the short, pendular penis
increased distance among the hips,waddling gait,
and outward rotation of the lower limbs

19. Pelvic Floor Defects
 The levator ani group is positioned more
posteriorly in exstrophy patients, with 68%
located posterior to the rectum and 32% anterior
(vs. 52% posterior and 48% anterior in healthy
controls)
 The levators are also rotated outward 15.5
degrees, and in the coronal aspect the levators
are 31.7 degrees more flattened than normal.
 This deviation from normal makes the exstrophy
puborectal sling more flattened than its normal
conical shape.

21. Abdominal Wall Defects
 The triangular defect caused by the premature
rupture of the abnormal cloacal membrane is
occupied by the exstrophy bladder & posterior
urethra. The fascial defect is limited inferiorly by
the intrasymphyseal band.
 an umbilical hernia is usually present, it is
usually of insignificant size.

22. Anorectal Defects
 The perineum is short and broad, and the
anus corresponds to the posterior limit of
the triangular fascial defect
 The divergent levator ani and puborectalis
muscles and the distorted anatomy of the
external sphincter contribute to varying degrees
of anal incontinence and rectal prolapse

23. Male Genital Defect
 The male genital defect is severe and is probably
the most troublesome aspect of the surgical
reconstruction
 The anterior corporal length of male
patients with bladder exstrophy is almost
50% shorter than that of normal controls
[ Silver and colleagues]

25.  The volume, weight, and maximum cross-sectional
area of the prostate appeared
normal compared with published control
values
 The vas deferens and ejaculatory ducts are
normal in the exstrophy patient fertility is not
impaired by testicular dysfunction.

27. Female Genital Defects
 The vagina is shorter than normal, hardly
greater than 6 cm in depth, but of normal
caliber
 The vaginal orifice is frequently stenotic
and displaced anteriorly, the clitoris is
bifid, and the labia, mons pubis, and clitoris
are divergent.
 The fallopian tubes and ovaries are normal.

29. Urinary Defects
 Varying degrees of polyps, von Brunn nests,
cystitis cystica, and cystitis glandularis can be
found.Cystitis glandularis was noted in a higher
percentage of secondary closures.
 Because of the potential risk of adenocarcinoma
associated with cystitis glandularis, future
surveillance of these patients with urine cytology
and cystoscopy as they enter adulthood is
recommended

30.  Most of patients have compliant and stable
bladders before bladder neck reconstruction.
 Horseshoe kidney, pelvic kidney, hypoplastic
kidney, solitary kidney, and dysplasia with
megaureter are all encountered in these patients

31. Prenatal Diagnosis
 Absence of bladder filling,
 a low-set umbilicus
 widening pubis
 diminutive genitalia,
 a lower abdominal mass that increases in size as
the pregnancy progresses and as the
intraabdominal viscera increases in size

32. 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 the
bladder surface
 The bladder can then be covered with a
nonadherent film of plastic wrap to prevent
sticking of the bladder mucosa to clothing or
diapers.

34. Selection of Patients for Immediate
Closure
 The exstrophied bladder that is estimated at the
time of birth to have a capacity of 5 mL or more
and demonstrates elasticity and contractility can
be expected to develop useful size and capacity
after successful bladder, posterior urethral, and
abdominal wall closure with early epispadias
repair

35.  penoscrotal duplication, ectopic bowel within the
extruded bladder , a hypoplastic bladder, and
significant bilateral hydronephrosis preclude
primary repair
 waiting for the bladder template to grow for
6 to 12 months in the child with a small
bladder can be done
 excision of the bladder and a nonrefluxing colon
conduit or ureterosigmoidostomy can be done for
totally unfit bladder.

36. Modern Reconstruction
of Bladder Exstrophy
 The bladder closure, abdominal wall
closure, and posterior urethral closure well
onto the penis in the newborn period with
bilateral innominate and vertical iliac
osteotomy,if indicated should be done
 epispadias repair at 6 months to 1 year of
age; bladder neck reconstruction along with
antireflux procedure at age 4 to 5 years,
when the child has achieved an adequate
bladder capacity

37. Osteotomy
 The most frequently used osteotomy today is the
bilateral anterior innominate and vertical iliac
osteotomy
 If the patient is younger than 72 hours old
and examination under anesthesia reveals
that the pubic bones are malleable and able
to be brought together easily in the midline
by medial rotation of the greater
trochanters, the patient can undergo
closure without osteotomy

38. ADVANTAGES
 Easy approximation of the symphysis with
diminished tension on the abdominal wall
closure and elimination of the need for
fascial flaps;
 placement of the posterior vesicourethral
unit deep within the pelvic ring, enhancing
bladder outlet resistance; and
 bringing the large pelvic floor muscles near
the midline, where they can support the
bladder neck and aid in eventual urinary
control

40.  At the end of the procedure, the pelvis is closed
with a suture between the two pubic rami. The
external fixators are then applied between the
pins to hold the pelvis in a correct position
 The external fixator is kept on for 4 to 6 weeks,
until adequate callus is seen at the site of the
osteotomy

42. Bladder, Urethral, and
Abdominal Wall Closure

44.  Before removal of the suprapubic tube, 4
weeks after surgery, the bladder outlet is
calibrated by a urethral catheter or a
urethral sound to ensure free drainage.
 Cystoscopy and cystography at yearly
intervals are used to evaluate the degree of
reflux and to provide an estimate of bladder
capacity .

45. Epispadias Repair
 In a group of patients with a small bladder
capacity after initial closure, there was a mean
increase of 55 mL in males in only 22 months
after epispadias repair.
 Because most boys with exstrophy have a
somewhat small penis and a shortage of available
penile skin, all patients undergo testosterone
stimulation before urethroplasty and penile
reconstruction

46.  correction of dorsal chordee,
 urethral reconstruction,
 glanular reconstruction, and
 penile skin closure.

47. Modified Cantwell-Ransley Repair

52. Penile Disassembly Epispadias
Repair
 This technique of epispadias repair was
developed by Mitchell and Bagli (1996).
 It has now been incorporated in the CPRE
exstrophy repair for primary closure in the
newborn

54. Continence and Antireflux
Procedure
 some modern exstrophy repairs claim to establish
suitable continence without formal bladder neck
repair.
 EACH CHILD should undergo gravity cystogram
under anesthesia yearly after newborn closure to
assess bladder growth
 Continence and antireflux procedures performed
at the age of 4 or 5 .

55. Modified Young-Dees-Leadbetter bladder
neck reconstruction

58.  At the end of 3 weeks the suprapubic tube is
clamped, and the patient is allowed to
attempt to void.
 Initially, the tube should not be clamped for
more than 1 hour. If voiding does not occur,
the child is given an anesthetic and an 8-Fr
Foley catheter is placed.
 This is left in place for 5 days and removed,
and then another voiding trial is begun.

59. CLOACAL EXSTROPHY
 Cloacal exstrophy includes a spectrum of
abnormalities but is primarily an anterior
abdominal wall defect
 A reported incidence of 1 : 200,000 to 1 : 400,000
makes this one of the rarer urologic
abnormalities
 Most cases are sporadic, and isolated incidences
of unbalanced translocations have been reported

60.  cloacal exstrophy includes exstrophy of the
bladder, complete phallic separation, wide pubic
diastasis, exstrophy of the terminal ileum
between the two halves of the bladder, a
rudimentary hindgut, imperforate anus, and the
presence of an omphalocele.
 Abnormalities of the spinal cord or vertebral
column, or both,have been noted in 85% to 100%
of children

62. ABNORMALITIES
 The pelvic defects that are seen with classic
bladder exstrophy are noted with greater severity
in the patient with cloacal exstrophy.
 The interpubic distance (diastasis) in children
with cloacal exstrophy was noted to be almost
twice that of children with classic bladder
exstrophy.
 Skeletal and limb anomalies were also
reported by Diamond (1990) in 12% to 65% of
cases

63. Intestinal Tract Abnormalities
 the incidence of omphalocele is around 88%
 malrotation, duplication anomalies,and
anatomically short bowel occur with varying
frequencies.
 A hindgut remnant of varying size is also noted
in most patients

64. Genitourinary Abnormalities
 The most commonly reported müllerian
anomaly was uterine duplication, seen in
95% of patients
 Upper urinary tract anomalies occurred in
41% to 60% of patients
 Genital anomalies in the male have typically
included complete separation of the two phallic
halves and accompanied separation of the scrotal
halves.

65.  Testes may be noted in the scrotum but are
frequently noted to be undescended, and
associated inguinal hernias are a common
finding.
 Girls typically have widely divergent clitoral
halves
 two exstrophied hemibladders flanking the
exstrophied intestinal segment.Each bladder half
usually drains the ipsilateral ureter

66. Prenatal Diagnosis
 The three main criteria used to identify the
diagnosis are a large midline infraumbilical
anterior abdominal wall defect, lumbosacral
myelomeningocele, and failure to visualize the
urinary bladder
 early diagnosis may permit appropriate prenatal
counseling for parents and expedite postnatal
care.

67. Gender Assignment
 Because of the significant separation of the
corpora of the penis and scrotum and the
reduction in corporal size noted in boys with
cloacal exstrophy, early reports had
recommended universal gender reassignment of
boys (46,XY) with cloacal exstrophy to functional
females.
 Currently, however, most authors recommend
assigning gender that is consistent with
karyotypic makeup of the individual if at all
possible .

68. Modern Functional Reconstruction of
Cloacal Exstrophy
 Immediate Neonatal Assessment
 Evaluate associated anomalies
 Decide whether to proceed with reparative surgery
 Functional Bladder Closure (Soon after Neonatal
Assessment)
 ONE-STAGE REPAIR (FEW ASSOCIATED ANOMALIES)
 Excision of omphalocele
 Separation of cecal plate from bladder halves
 Joining and closure of bladder halves and urethroplasty
 Bilateral anterior innominate and vertical iliac osteotomy
 Gonadectomy in males with unreconstructible phallus
 Terminal ielostomy/colostomy
 Genital revision if needed

69. TWO-STAGE REPAIR
 First stage (newborn period)
 Excision of omphalocele
 Separation of cecal plate from bladder halves
 Joining of bladder halves
 Gonadectomy in male with unreconstructible
phallus
 Terminal ileostomy/colostomy

70. Second stage
 Closure of joined bladder halves and
urethroplasty
 Bilateral anterior innominate and vertical iliac
osteotomy
 Genital revision if necessary

71.  Anti-Incontinence/Reflux Procedure (age 4-
5 yr)
 Bladder capacity ≥ 85 mL (small select group of
patients)
 Young-Dees-Leadbetter bladder neck
reconstruction
 Bilateral Cohen ureteral reimplantations
 Bowel and/or stomach segment used to augment
bladder Or Continent diversion with
abdominal/perineal stoma
 Vaginal Reconstruction
 Vagina constructed or augmented using colon,
ileum, or fullthickness skin graft

72. LONG-TERM ISSUES IN
CLOACAL EXSTROPHY
 Bowel and continence problems
 Fertility appears to be universally compromised
in boys, but girls have normal fertility and
pregnancy has been reported.
 Girls have higher degrees of cervical prolapse
when compared with their counterparts with
bladder exstrophy
 Despite the extensive malformations noted,
many patients have gone on to live fruitful
lives.

73. EPISPADIAS
 Epispadias varies from a mild glanular defect in
a covered penis to the penopubic variety with
complete incontinence in males or females.
 Isolated male epispadias is a rare anomaly,
with a reported incidence of 1 in 117,000
males
 Most male epispadias patients (about 70%)
have complete epispadias with
incontinence

74. MALE EPISPADIAS FEMALE EPISPADIAS

75. Associated Anomalies
 Diastasis of the pubic symphysis, and deficiency
of the urinary continence mechanism
 The only renal anomaly observed in 11 cases of
epispadias was agenesis of the left kidney
 The ureterovesical junction is inherently
deficient in complete epispadias, and the
incidence of reflux has been reported in a
number of series to be between 30% and 40%

76. Surgical Management
 In patients with complete epispadias and good
bladder capacity, epispadias and bladder neck
reconstruction can be performed in a single-stage
operation.
 A firm intrasymphyseal band typically bridges
the divergent symphysis, and an osteotomy is not
usually performed
 Epispadias reconstruction ca be done by
Modified Cantwell-Ransley Repair, Penile
Disassembly Epispadias Repair.

77.  The Young-Dees-Leadbetter bladder neck plasty,
Marshall-Marchetti- Krantz suspension, and
ureteral reimplantation are performed when the
bladder capacity reaches approximately 80 to 85
mL, which usually occurs between 4 and 5 years
of age.
 Clinically, these bladders are more supple,
easier to mobilize, and more amenable to
bladder neck reconstruction.

78. SEXUAL FUNCTION AND FERTILITY
IN THE EXSTROPHY PATIENT

79. Male Patient
 Sporadic instances of pregnancy or the
initiation of pregnancy by males with
bladder exstrophy have been reported.
 Male patients with genital reconstruction and
closure of the urethra demonstrated high risk of
infertility.
 newer techniques such as gamete
intrafallopian transfer (GIFT) or
intracytoplasmic sperm injection (ICSI) can
be used to assist these patients in their goal
of pregnancyachievement.
 Sexual function and libido in exstrophy
patients are normal

80. Female Patient
 Vulvoplasty is sometimes indicated in
patients before they become sexually active
or startusing tampons.
most patients will require vaginoplasty
before intercourse could take place
 vaginal prolapse and uterine prolapse were noted
commonly and even quite early in life (mean age
16 years).
 Review of the literature reveals 45 women
with bladder exstrophy who successfully
delivered 49 normal offspring.

81. Thanks !!
!