it is a presentation on epidemiology, embryology, clinical presentation, diagnostics and management options of bladder exstrophy and epispadias.

1. BLADDER EXSTROPHY AND
EPISPADIAS
PREPARED BY:
RUHAMA YOSEPH
(SR-IV)

2. Outline
 Introduction
 Epidemiology
 Embryology
 Pathophysiology
 Clinical presentation and Diagnosis
 Surgical management
 Outcomes of management
Oct 9,2014 2

3. Introduction
 Exstrophy and epispadias are part of a
spectrum of anomalies characterized
by exposure of part or all of the
mucosa of the lower urinary tract to
the external environment through a
defect in the anterior abdominal wall
Oct 9,2014 3

4. Introduction
 Depictions on Assyrian tablets as early
(2000 BC)
 The first historical description is done
by von Grafenberg’s in1597
 The term ‘exstrophie’ coined by
Chaussier on 1780
Oct 9,2014 4

5. Introduction
 In Lay terms:
◦ Deformity resulting when as in one blade
of a pair of scissors is passed through the
urethra of a normal person…..
Oct 9,2014 5

6. Introduction
 Types:
◦ Classic bladder exstrophy
◦ Epispadias
◦ Cloacal exstophy
◦ Other variants
Oct 9,2014 6

7. Introduction
 Classic bladder exstrophy
◦ Characterized by exposure of the bladder
and posterior urethral mucosa through a
defect in the anterior abdominal wall. It is
associated with a complete epispadias in
boys and bifid clitoris in girls.
Oct 9,2014 7

8. Introduction
 Classic bladder exstrophy in a male
neonate
Oct 9,2014 8

9. Introduction
 Classic bladder exstrophy in a female
child
Oct 9,2014 9

10. Introduction
 Cloacal exstrophy:
◦ Characterised by a defect in the anterior
abdominal wall, exposing the mucosa of
the caecum with bladder on both sides of
it, and resulting in maldevelopment of the
hindgut
◦ Commonly associated with other
anomalies
Oct 9,2014 10

11. Introduction
 Newborn with cloacal exstrophy &
Omphalocele
Oct 9,2014 11

12. Introduction
 Epispadias
◦ The urethra is a partial or complete open
plate on the dorsal surface of the phallus
Oct 9,2014 12

13. Introduction
 Epispadias:
◦ Position of the meatus on the dorsal
surface of the penis varies
 When at peno-pubic area the whole urethral
mucosa is open dorsally, and associated with
dorsal chordee, may or may not be associated
with incompetent sphincter
Oct 9,2014 13

14. Introduction
 Epispadias…
◦ In female, the urethra may be in a normal
position ( but lax and patulous) or be
found anywhere from the bladder neck to
the normal position
◦ An associated VUR and incompetent
sphinicter may be there
◦ Usualy diastasis of the pubic symphysis
◦ Clitoris is usualy bifid
Oct 9,2014 14

15. Introduction
 External view in female patient with
epispadias. Labia are separated
anteriorly
Oct 9,2014 15

16. Introduction
 Superior vessical fissure variant of
exstrophy
Oct 9,2014 16

17. Introduction
 Female with covered exstrophy. The umbilicus is
very low, the pubic bones are widely separated,
and the rectus muscles are divergent. The bladder
is intact, but the patient has undergone bladder
neck reconstruction to achieve urinary continence.
The external genitalia are normal.
Oct 9,2014 17

18. Introduction
 Eventhough these conditions may not
be lethal, patients may have
significant morbidities and impaired
quality of life
Oct 9,2014 18

19. Epidemiology
 The incidence of bladder exstrophy in
Africa is not known, but various
studies report it to be 3.3 per 100,000,
with a male-to-female ratio of 2.3:1.
 The Western Countries have
incidence of 1 in 10,000 to 50,000 with
sex ratio of 3 to 6:1
Oct 9,2014 19

20. Epidemiology
 The incidence of epispadias is
estimated to be 1 in 117,000 in males,
with a male-to-female ratio of 3–4:1.
 The incidence of cloacal exstrophy is
estimated to be 1 in 200,000 to 1 in
400,000.
 Prevalence of classic bladder
exstrophy is 3.3 per 100,000 births
Oct 9,2014 20

21. Epidemiology
 The risk of transmission from a patient
with exstrophy to a child is about 1 in
70.
 The risk of recurrence in a particular
family is estimated to be about 1 in
100.
Oct 9,2014 21

22. Embryology
 Different theories postulated to
describe the defect during
embryogenesis
 Cloacal membrane rupture is one of
these theories
Oct 9,2014 22

23. Embryology
 Separation of the primitive cloaca into
the urogenital sinus and hindgut
occurs during the first trimester at
approximately the same time as
maturation of the anterior abdominal
wall
Oct 9,2014 23

24. Embryology
 Failure of mesenchyme to migrate
between the ectodermal and
endodermal layers of the lower
abdominal wall leads to instability of
the cloacal membrane and its rupture
Oct 9,2014 24

25. Embryology
 The timing of rupture of cloacal
membrane due to failure of
incorporation of mesoderm matters
◦ If very early rupture then results cloacal
exstrophy (before the urogenital septum
has devided cloaca)
◦ If rupture of the cloacal membrane after
complete separation of the genitourinary
and GI tracts results in classic bladder
exstrophy
Oct 9,2014 25

26. Embryology
 Epispadias is a variant that displays
normal bladder formation but
incomplete urethral tubularization from
the bladder neck down
Oct 9,2014 26

27. Pathophysiology
 Boney abnormalities:
◦ Pubic bones are widely separated
◦ Wider angle between the sacroiliac bones
and the sagittal plane….
◦ Waddling gait
Oct 9,2014 27

28. Pathophysiology
 Typical appearance of pelvis in patient with
exstrophy-epispadias complex with wide
separation of pubic symphysis
Oct 9,2014 28

29. Pathophysiology
 Muscular abnormalities:
◦ Rectus sheath is attached to the
separated pubic bones
◦ Pelvic diaphragm also inserts into the
widely separated pubic bones and forms
an intersymphyseal fibrotic band
◦ This leads to incontinence of urine, smt
incontinence of stool, prolapse of uterus,
smt prolapse of rectum
Oct 9,2014 29

30. Pathophysiology
 Anomalies of the urinary system:
◦ Bladder plate at birth may be large which
can be easily closed while others have
small fibrotic plate that is difficult
◦ Persistent trauma leads to inflammation,
fibrosis, metaplasia, and possible
carcinoma later in life
◦ UVJ is often incompetent resulting in
reflux in nearly all patients
Oct 9,2014 30

31. Pathophysiology
 Anomalies of the genitalia:
◦ In classic bladder exsrophy penis is
completely open dorsally as a complete
epispadias
◦ Wide attachment of corpora cavernosa to the
separated pubis, short corpora, dorsal
chordee of the penis
◦ Anatomic variations include bifid penis
◦ Testes and vas deferens normal, but there
may be associated inguinal hernias
◦ Prostate gland does not surround the urethra
Oct 9,2014 31

32. Pathophysiology
 Bladder exstrophy, two hemiscrota,
and abmormal perineum and anus
Oct 9,2014 32

33. Pathophysiology
 In a female with bladder exstrophy, a bifid
clitoris, and a wide-open urethral plate. The
labia minora are small and the vaginal
opening lies between them, more anteriorly
Oct 9,2014 33

34. Pathophysiology
 Anomalies of the anus:
◦ Functions normally, tends to be placed
more anteriorly
Oct 9,2014 34

35. Pathophysiology
 Anomalies associated with cloacal
exstrophy:
◦ Omphalocele
◦ Myelodysplasia
◦ Abnormal location of kidneys, agenesis
◦ Cryptorchidism
◦ Uterus didelphys, vaginal agenesis
◦ Anorectal and intestinal anomalies
◦ Skeletal anomalies
Oct 9,2014 35

36. Pathophysiology
 46,XY newborn with cloacal exstrophy. A very large
omphalocele is present, and the hemibladders
flank the exstrophic cecal plate.
Oct 9,2014 36

37. Pathophysiology
 Lipomeningocele in the same newborn
Oct 9,2014 37

38. Clinical presentation
 Antenatal ultrasonography findings suggestive of
exstrophy-epispadias complex:
◦ Repeated failure to visualize the bladder
on U/S
◦ Lower abdominal wall mass
◦ Low-set umbilical cord
◦ Abnormal genitalia
◦ Increased pelvic diameter
Oct 9,2014 38

39. Clinical presentation
 Additional antenatal ultrasound
findings suggestive of cloacal
exstrophy include omphalocele, limb
abnormalities, myelomeningocele, and
trunk sign from prolapsed intestine.
Oct 9,2014 39

40. Clinical presentation
 Most variants are identifiable at birth in
the delivery room
 Unrecognized female epispadias may
present as persistent childhood
incontinence
 Unrecognized split-symphysis variants
of exstrophy may be identified in
childhood only because of persistent
incontinence or a waddling gait
Oct 9,2014 40

41. MANAGEMENT OF BLADDER
EXSTROPHY AND EPISPADIAS

42. Management
 Team of experts required to manage
this complex anomaly
 Initial management includes:
◦ Counseling of parents
◦ Gender assignment if possible
◦ Protection of bladder mucosa
◦ Parenteral nutrition for neonates with
cloacal exstrophy
◦ Antibiotic therapy for those prepared for
surgery
Oct 9,2014 42

43. Management
 Use of plastic wrap to protect the
delicate bladder mucosa in a newborn
with bladder exstrophy.
Oct 9,2014 43

44. Management
 Surgical options:
◦ Staged reconstruction (MSRE)
◦ Complete primary reconstruction (CPRE)
◦ Urinary diversion
Oct 9,2014 44

45. Management
 Components of procedures for
reconstruction :
◦ Bladder closure
◦ Posterior urethra and abdominal wall
repair
◦ Bilateral osteotomies
◦ Epispadias repair
◦ Bladder neck reconstruction
◦ Antireflux procedure
Oct 9,2014 45

46. Management
 Modern Staged Repair for
Exstrophy(MSRE)
◦ Soon after birth
 Bladder closure, posterior urethra and
abdominal wall repair, +/- Bilateral osteotomies
◦ 6 mo to 1 year
 Epispadias repair
◦ When bladder capacity is at least 85 ml
 Bladder Neck Reconstruction (BNR)
Oct 9,2014 46

47. Management
 Complete Primary Repair for Epispadias
(CPRE)
◦ Bladder closure, posterior urethra and
epispadias repair, bilateral osteotomies
done at any age in one stage
◦ Bladder neck repair with a anti-reflux
surgery done when child has appropriate
bladder capacity
Oct 9,2014 47

48. CPRE
 Best done at neonatal period
 Advantages:
◦ Allows normal bladder cycling and
physiological development after
reconstruction
◦ If done early, it may be technically simpler
and avoids need of osteotomies
◦ Attains a more normal appearance
Oct 9,2014 48

49. CPRE
 The bladder neck, and urethra are
moved posteriorly within the pelvis.
◦ This movement maximizes the effect of
the pelvic muscles
 Total penile disassembly
◦ Reduces anterior tension on the urethra
◦ It prevents bladder dehiscence and dorsal
chordee
Oct 9,2014 49

50. CPRE
 Surgical Technique: Boys
Transversely oriented traction sutures are
placed into each of the hemiglans of the
penis
Lines of dissection marked
Catheters placed into both ureters
Bladder polyps are removed before
beginning the dissection
Oct 9,2014 50

51. CPRE
Oct 9,2014 51

52. CPRE
Periumbilical skin incised
circumferentially and dissection
proceeds from superior to inferior
between the bladder and skin& fascia
Oct 9,2014 52

53. CPRE
 Penile/Urethral Dissection
 Dissection begins along the ventral
aspect of the penis as a circumcising
incision
Oct 9,2014 53

54. CPRE
Shallow incisions are made laterally
along the dorsal aspect of the urethra
to begin the dissection. Sharp
dissection is required to develop the
plane between the urethral wedge and
the corporeal bodies
Oct 9,2014 54

55. CPRE
 The lateral dissection on the penis
should be superficial to Buck’s fascia
to avoid the hit of neurovascular
bundles
Oct 9,2014 55

56. CPRE
Complete Penile Disassembly and
Deep Dissection
 After development of a plane, the
penis may be disassembled into three
components:
1. The right corporeal body
2. The left corporeal body (with their
respective hemiglans )
3. The urethral wedge (urothelium with
underlying corpora spongiosa)
Oct 9,2014 56

57. CPRE
Separation of corporeal bodies and
urethra
 If urethra is long enough and the bladder mobile
enough, the 3 components may not need separation
Oct 9,2014 57

58. CPRE
Deep pelvic dissection
◦ Proximal dissection of the urethra to the
bladder neck exposes the pelvic
diaphragm
Incision of the intersymphyseal band
(the condensation of anterior pelvic
fascia and ligaments) posterior and
lateral to each side of the urethral
wedge is
oThis carried until pelvic musculature
becomes visible
Oct 9,2014 58

59. CPRE
Oct 9,2014 59

60. CPRE
 Tubularization of the neourethra over the
urethral catheter
Oct 9,2014 60

61. CPRE
Primary Closure
 bladder closure and urethral
tubularization done
• The urethra is tubularized using a two
layer running closure with monofilament
and braided absorbable suture
• The bladder closed using a three-layer
closure with monofilament absorbable
suture
Oct 9,2014 61

62. CPRE
The pubic symphysis is
reapproximated using two No. 1
interrupted sutures placed in a figure-
of-eight fashion
 Knots are left anteriorly to prevent
suture erosion into the bladder neck
Oct 9,2014 62

63. CPRE
Rectus fascia is reapproximated using
an interrupted or running suture
 Optionally sutures can be placed
along the dorsal aspect of the
corporeal bodies to reapproximate
them
Oct 9,2014 63

64. CPRE
Penile skin coverage is provided using
either a primary dorsal closure or flaps
if needed
Skin covering the abdominal wall is
reapproximated using a two-layer
closure of absorbable monofilament
suture
Oct 9,2014 64

65. CPRE
If there is adequate urethral length,
the urethra may be brought up to each
hemiglans ventrally to create an
orthotopic meatus.
If urethra is too short to reach the
glans (in about half the cases), it is
matured along the ventral aspect of
the penis to create a hypospadias
Oct 9,2014 65

66. CPRE
 Hypospadias created in presence of
short urethra
Oct 9,2014 66

67. CPRE
 Surgical Technique: Girls
The planned lines of incision marked with
the bladder neck, urethra, and vagina
mobilized as a unit
Traction sutures are placed into each of
the clitoris
Catheters placed into both ureters
Oct 9,2014 67

68. CPRE
 The appropriate plane of dissection is found
anteriorly along the medial aspect of the glans
clitoris and proceeds posteriorly along the lateral
aspect of the vaginal vault
Oct 9,2014 68

69. CPRE
 The vagina is mobilized with the urethra
and bladder neck
Oct 9,2014 69

70. CPRE
The dissection is carried down dividing
the intersymphyseal band
Oct 9,2014 70

71. CPRE
 The bladder and urethra are closed in multiple
layers, and the suprapubic tube is brought out
superiorly
Oct 9,2014 71

72. CPRE
 Pelvic positioning of the bladder and vagina
and closure of the pubic symphysis with
figure-of-eight sutures
Oct 9,2014 72

73. CPRE
 Repair of the abdominal wall and vaginal
introitus
Oct 9,2014 73

74. MSRE Stage I
 Initial Early Bladder Exstrophy
Closure
 Surgical objectives include
◦ closure and repositioning of the bladder
and urethra inside the pelvic ring
 posterior urethra in males and entire urethra in
females
◦ Approximation of the pelvic ring with
closure of the abdominal wall
 This converts the exstrophic bladder
into continent epispadia
Oct 9,2014 74

75. MSRE Stage I
 Male MSRE Bladder and Posterior
Urethral Closure Technique
Ureteral catheters are inserted and
secured
A traction suture is placed in the glans
penis
The posterior urethra is developed by
incising a 2-cm wide strip of mucosa
from the distal trigone to below the distal
verumontanum
Oct 9,2014 75

76. MSRE Stage I
Oct 9,2014 76

77. MSRE Stage I
Initial dissection begins at the umbilicus
and is continued circumferentially,
incising around the bladder plate
A plane developed between the rectus
sheath and the bladder wall
The peritoneum is dissected from the
dome of the bladder
 Extraperitoneal dissection continues
until an area lateral to the trigone is
reached exposing the urogenital
diaphragm
Oct 9,2014 77

78. MSRE Stage I
Oct 9,2014 78

79. MSRE Stage I
The urogenital diaphragm fibers and
the intersymphyseal band sharply
taken down from the pubic
subperiosteum bilaterally
Oct 9,2014 79

80. MSRE Stage I
 Lack of urethral plate length can be
managed by dividing the prostatic
plate distal to the verumontanum in a
“V”-shaped fashion
Oct 9,2014 80

81. MSRE Stage I
Oct 9,2014 81

82. MSRE Stage I
 In patients with questionable urethral length para-
exstrophic flaps are created
 The flaps permit bladder mobilization deep into the
pelvis after division of the bladder plate from the
penis
Oct 9,2014 82

83. MSRE Stage I
The corpora cavernosa are dissected
off the inferior pubic rami and then
reapproximated in the midline
The para-exstrophy flaps are
mobilized and approximated in the
midline and to the base of bladder
Oct 9,2014 83

84. MSRE Stage I
Oct 9,2014 84

85. MSRE Stage I
 The bladder and neourethra are tubularized after
exteriorization of the ureteral stents and placement
of malecot suprapubic tube
Oct 9,2014 85

86. MSRE Stage I
The bladder mucosa and posterior
urethra are then closed in two layers
by using absorbable sutures
 The final urethral orifice must be able
to admit a 12 Fr to 14 Fr stent to allow
adequate resistance for bladder
growth while preventing outlet
obstruction
Oct 9,2014 86

87. MSRE Stage I
The pubis approximated anteriorly
Approximation of rectus fascia and
skin done
Oct 9,2014 87

88. MSRE Stage I
 If pelvic approximation not possible,
external fixator or Gallows’s traction
must placed for 4-6 wks
Oct 9,2014 88

89. MSRE Stage I
 Female MSRE Bladder and
Posterior Urethral Closure
Technique
 The surgical technique of bladder, pelvic
ring and abdominal wall closure are
identical to that described for the male
 The difference is:
◦ The female urethra is completely reconstructed
at stage 1
◦ No need of para-exstrophy flaps
Oct 9,2014 89

90. MSRE Stage I
 The urethral plate mucosal incision is 2 cm
wide, traversing from the distal trigone to
the vaginal orifice in the female
Oct 9,2014 90

91. MSRE Stage I
The medial aspect of each hemiclitoris
is de-epithelialized to permit
approximation of the two glans clitori
and reconstruction of the mons
The bladder and female urethra are
closed in a two-layers…
Oct 9,2014 91

92. MSRE Stage I
 Post-operative care:
◦ Gallows traction or external fixation for 4-6
wks
◦ Adequate urethral caliber and minimal
postvoid residuals are assessed before
suprapubic tube removal at 4 weeks
◦ Ureteral stents left for 10-14 days
Oct 9,2014 92

93. MSRE Stage I
 Post-operative care…
◦ U/S assessment of the upper urinary tract
at time of discharge, after 3 mo and every
6 mo for one year
◦ Antibiotic prophylaxis for vesicoureteral
reflux is used
◦ Yearly cystograms under anesthesia at
age 1-3 years to assess bladder
growth/capacity
Oct 9,2014 93

94. MSRE Stage II
 The goals of epispadias repair include:
◦ A straight penis and urethra
◦ Easy urethral catheterization
◦ Normal erectile function
◦ A cosmetically satisfactory phallus
Oct 9,2014 94

95. MSRE Stage II
 The Modified Cantwell-Ransley
Technique
◦ Stay suture placed onto glans penis
◦ Ventral penile skin taken down
Oct 9,2014 95

96. MSRE Stage II
 The Modified Cantwell-Ransley
Technique…
◦ A reverse meatal advancement and
glanuloplasty (MAGPI) used to advance
urethral meatus onto the glans
Oct 9,2014 96

97. MSRE Stage II
 The Modified Cantwell-Ransley
Technique…
◦ Lateral edges of urethral plate and
epispadial meatus incised and it is
mobilized free from corporeal bodies
◦ Glans wings developed and corporeal
bodies separated from each other
Oct 9,2014 97

98. MSRE Stage II
 The Modified Cantwell-Ransley
Technique…
Oct 9,2014 98

99. MSRE Stage II
 The Modified Cantwell-Ransley …
◦ Urethra tabularized over 6 or 8 Fr stent
with 6-0 absorbable sutures
◦ Corporeal bodies rotated medially and
approximated at midline
Oct 9,2014 99

100. MSRE Stage II
 The Modified Cantwell-Ransley …
◦ Caverno-cavernostomy may be done to
facilitate approximation
Oct 9,2014 100

101. MSRE Stage II
 The Modified Cantwell-Ransley …
◦ The glans wings approximated with 5-0
absorbable sutures
Oct 9,2014 101

102. MSRE Stage II
 The Modified Cantwell-Ransley …
◦ Penile skin approximated , or Z-plasty
used if too tight
Oct 9,2014 102

103. MSRE Stage II
 Post-op care:
◦ Bladder anti-spasmodics
◦ Removal of urethral catheter after 2 wks
◦ Broad-spectrum antibiotics
Oct 9,2014 103

104. Bladder Neck Reconstruction
 After initial surgery, a continence
procedure is indicated when
◦ The urethra is stricture free and capable of
catheterization
◦ Under anesthesia, the bladder capacity has
achieved a minimum volume of 60 to 85 mL
◦ The child is mature enough to participate in the
postoperative voiding program (around age 4 to
5 years)
Oct 9,2014 104

105. BNR
 Ureteroneocystostomy may be
required at the time of BNR to correct
VUR and to move the ureters from the
lower bladder where BNR will occur
Oct 9,2014 105

106. BNR
 Modified Young-Dees-Leadbetter
BNR
The bladder neck is extensively
dissected and a vertical cystotomy is
made
Oct 9,2014 106

107. BNR
 Modified Young-Dees-Leadbetter
BNR
Transtrigonal/cephalotrigonal bilateral
ureteral reimplantation
Oct 9,2014 107

108. BNR
 Modified Young-Dees-Leadbetter
BNR
 Strip of bladder mucosa 1.5 to 1.8 cm wide and 3.0
to 4.0 cm long is generated and the lateral bladder
triangles are demucosalized
Oct 9,2014 108

109. BNR
 Modified Young-Dees-Leadbetter
BNR
 The neourethra is tubularized over an
8-Fr stent using interrupted or running
sutures
Oct 9,2014 109

110. BNR
 Modified Young-Dees-Leadbetter
BNR
The two triangular regions of
demucosalized detrusor muscle are
then closed over the mucosal tube in a
two-layer
Oct 9,2014 110

111. BNR
 Modified YDL BNR
◦ Urine drains via ureteral catheters and
suprapubic drainage tube
◦ Ureteral catheters removed at 2-3wks and
voiding trials will be started
Oct 9,2014 111

112. BNR
 Results:
◦ Surgical success is defined as a dry
interval of more than 2 to 3 hours and
spontaneous voiding without
catheterization
Oct 9,2014 112

113. Complications
 Of Primary bladder closure
◦ Partial or complete wound dehisence
◦ Bladder prolapse
◦ Bladder outlet stenosis, pyelonephritis
◦ VUR
Oct 9,2014 113

114. Complications
 Of epispadias repair
◦ Urethrocutaneuos fistulas, 5.5% to 42%
◦ Atrophy of the corpora cavernosa and
urethra
◦ Short penis
 Of BNR
◦ Persistent Incontinence
Oct 9,2014 114

115. Complications
 Subtotal prolapse of bladder wall through patulous
bladder neck after bladder closure in female with
cloacal exstrophy.
Oct 9,2014 115

116. Complications
 Bilateral vesicoureteral reflux and small-capacity
bladder after initial exstrophy closure. Contrast
escapes readily through the incompetent bladder
neck
Oct 9,2014 116

117. Management of Cloacal Exstrophy
 Treatment of myelodysplasia and GI
anomalies has priority over
management of urinary and genital
anomalies.
 Closure can be staged or performed in
one stage. A large omphalocele may
limit the success of closing the
abdomen and the bladder in one stage
Oct 9,2014 117

118. Management of Cloacal Exstrophy
 First stage involves:
◦ separation of the GI and genitourinary
tracts
◦ closure of the colon
◦ creation of a colostomy
◦ closure of the omphalocele
◦ Bringing the bladder plates together in the
midline
Oct 9,2014 118

119. Management of Cloacal Exstrophy
 The hindgut incorporated into the GI
tract to maximize absorptive surface
area
 A decision for rectal pull-through
versus permanent colostomy is based
on surgeon preference and projected
potential for social fecal continence.
Oct 9,2014 119

120. Management of Cloacal Exstrophy
 Subsequently
◦ bladder closure can be attained using
principles of complete primary repair
◦ Or Consideration may be given to
continent diversion as the second stage
◦ Because of more severe pubic diastasis,
pelvic osteotomies are required
Oct 9,2014 120

121. Management of Cloacal Exstrophy
 Historically, all males with cloacal
exstrophy underwent early gender
conversion
 Testicular histology is normal despite
frequent cryptochidism
 Cloacal exstrophy is now included as a
subset of disorders of sex
development.[10] Multidisciplinary
evaluation and both early and long-term
counseling should be offered.
Oct 9,2014 121

122. Urinary Diversions
 Indications:
◦ Inadequate bladder plates
◦ Failure of attempts of reconstruction
 Includes:
◦ Internal diversions
 Ureterosigmoidostomy
◦ Incontinent urinary diversion
 Ileal, colonic conduits
◦ Continent urinary reservior (external
diversion)
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123. Urinary Diversions
 Ureterosigmoidostomy
◦ Was accepted because there is no need
of stoma
◦ Complications are:
 Intermittent Pyelonephritis
 Ureteral obstruction
 Hyperchloremic metabolic acidosis
 Rectal incontinence
 Adenocarcinoma at the site of anastomosis
(250-300 fold increase)
Oct 9,2014 123

124. Fertility
 Although there has been no formal
study, it seems likely that females with
exstrophy have normal fertility unless
surgery has caused tubal obstruction
or some other genital complication
Oct 9,2014 124

125. Fertility
 Males are probably born with normal
testes and so with normal fertility
potential.
 A combination of surgery, recurrent
infection, and the erectile deformities
leads to a high rate of infertility
 fertility was better in boys who had
had an early diversion rather than
reconstruction
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126. Pregnancy and delivery
 With modern obstetric care,
pregnancy and delivery should be
uncomplicated except for the risk of
prolapse
Oct 9,2014 126

127. THANKYOU!
Oct 9,2014 127

128. REFERENCES
 Ashcraft’s Pediatric Surgery, 5th Ed
 Grosfeld, Pediatric Surgery, 6th Ed
 Paediatric Surgery:A Comprehensive
Text for Africa
 Stringer, Pediatric Surgery and
Urology
 Springer Surgery Atlas Series,
Pediatric Surgery
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