Pediatric urology:Epispadias cloacal exstrophy

Epispadias cloacal exstrophy

1. EPISPADIAS
Dept of Urology
Govt Royapettah Hospital and Kilpauk Medical College
Chennai

1

2. Professors:
Prof. Dr. G. Sivasankar, M.S., M.Ch.,
Prof. Dr. A. Senthilvel, M.S., M.Ch.,

Asst Professors:
Dr. J. Sivabalan, M.S., M.Ch.,
Dr. R. Bhargavi, M.S., M.Ch.,
Dr. S. Raju, M.S., M.Ch.,
Dr. K. Muthurathinam, M.S., M.Ch.,
Dr. D. Tamilselvan, M.S., M.Ch.,
Dr. K. Senthilkumar, M.S., M.Ch.
Dept of Urology, GRH and KMC,
Chennai. 2

3.  A defect in the dorsal wall of the urethra.

 A broad, mucosal strip lining the dorsum of the
penis extending toward the bladder, with potential
incompetence of the sphincter mechanism.

 The displaced meatus is free of deformity, and
occurrence of urinary incontinence is related to the
location of the dorsally displaced urethral meatus.
3
Dept of Urology, GRH and KMC,
Chennai.

4. May be found on the glans, on the penile shaft,or
in the penopubic region.

Associated with varying degrees of dorsal chordee.

In penopubic or subsymphyseal epispadias, the
entire penile urethra is opened and the bladder
outlet may be large enough to admit the examining
finger, indicating obvious gross incontinence.

4
Dept of Urology, GRH and KMC,
Chennai.

5. Isolated male epispadias incidence of 1 in
117,000 males.

Most male epispadias patients (about 70%)
have complete epispadias with incontinence.


most commonly noted as a component of
bladder and cloacal extrophy.
5
Dept of Urology, GRH and KMC,
Chennai.

6. Patients have a characteristic widening of the
symphysis pubis caused by outward rotation of the
innominate bones.
 This separation of the pubis causes divergent
penopubic attachments that contribute to the
short, pendular penis with dorsal chordee.

 Male-to-female ratio 3 : 1 and 5 :1.
6
Dept of Urology, GRH and KMC,
Chennai.

7. Female epispadias
◦ occurs in 1 of every 484,000 female patients.
Davis classification:
 Least severe degree the urethral orifice simply
appears patulous.
Intermediate epispadias,the urethra is dorsally
split along most of the urethra.
 In the most severe degree of epispadias, the
urethral cleft involves the entire length of the
urethra and sphincteric mechanism and the
patient is rendered incontinent.
7
Dept of Urology, GRH and KMC,
Chennai.

8. The genital defect is characterized by
A bifid clitoris
The mons is depressed in shape
Coated by a smooth, glabrous area of skin.
The labia minora are usually poorly developed ,
terminate anteriorly at the corresponding half of
the bifid clitoris, where there may be a rudiment of
a preputial fold.

8
Dept of Urology, GRH and KMC,
Chennai.

9. The symphysis pubis is usually closed ,represented
by a narrow fibrous band.


The vagina and internal genitalia are usually normal.

9
Dept of Urology, GRH and KMC,
Chennai.

10. MALE EPISPADIAS
10
Dept of Urology, GRH and KMC,
Chennai.

11. 11
Dept of Urology, GRH and KMC,
Chennai.

12. Deformities of the external genitalia.
 Diastasis of the pubic symphysis.

Deficiency of the urinary continence mechanism.

The only renal anomaly observed was agenesis of
the left kidney.

12
Dept of Urology, GRH and KMC,
Chennai.

13. The ureterovesical junction is inherently deficient
in complete epispadias, and the incidence of reflux
is between 30% and 40% in males.


Incidence of reflux in females is reported to be 30%
to 75%.


13
Dept of Urology, GRH and KMC,
Chennai.

14. 

 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
14
Dept of Urology, GRH and KMC,
Chennai.

15.  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.
15
Dept of Urology, GRH and KMC,
Chennai.

16.  Classic exstrophy accounts for more than 50% of
the patients born with this complex
16
Dept of Urology, GRH and KMC,
Chennai.

17. 17
Dept of Urology, GRH and KMC,
Chennai.

18. 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.
18
Dept of Urology, GRH and KMC,
Chennai.

19.  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
19
Dept of Urology, GRH and KMC,
Chennai.

20. 20
Dept of Urology, GRH and KMC,
Chennai.

21. 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
21
Dept of Urology, GRH and KMC,
Chennai.

22. Objectives :

 Acheivement of urinary continence with
preservation of upper urinary tracts

Reconstruction of cosmetically acceptable
genitalia


22
Dept of Urology, GRH and KMC,
Chennai.

23. Patients of complete epispadias with good
bladder capacity:
 Epispadias and bladder neck
reconstruction can be performed in a single
stage.
A small incontinent bladder with reflux : Ideal
for bladder neck reconstruction and ureteral
reimplantation.
In epispadias, bladder capacity is the
predominant indicator of continence.

23
Dept of Urology, GRH and KMC,
Chennai.

24.  Most boys with exstrophy have small penis and a
shortage of available penile skin, all patients
undergo testosterone stimulation before
urethroplasty and penile reconstruction
24
Dept of Urology, GRH and KMC,
Chennai.

25. Correction of dorsal chordee
Lengthen dorsomedial aspect of corpora by
incision and anastomosis of corpora themselves.
Placement of dermal graft
Urethral reconstruction
Tubularization of urethral plate ,moving the
urethral plate under corporal bodies
Glanular reconstruction
Penile skin closure.
25
Dept of Urology, GRH and KMC,
Chennai.

26. 26
Dept of Urology, GRH and KMC,
Chennai.

27. 27
Dept of Urology, GRH and KMC,
Chennai.

28. 28
Dept of Urology, GRH and KMC,
Chennai.

29. 29
Dept of Urology, GRH and KMC,
Chennai.

30. 30
Dept of Urology, GRH and KMC,
Chennai.

31.  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
31
Dept of Urology, GRH and KMC,
Chennai.

32. 32
Dept of Urology, GRH and KMC,
Chennai.

33. PRINCIPLES:
Radical dissection of the bladder and urethra
from sorrounding structures while connection
to vagina is maintained.
Dissection of lateral aspects of posterior
vesicourethral and vaginal unit from their
attachments to pelvic floor.
Tension free closure of the abdomen bladder
urethra and external genitalia.
Judicious use of osteotomy and proper
immobilization of the pelvis and extremities.
33
Dept of Urology, GRH and KMC,
Chennai.

34.  Excision of the glabrous skin of the mons.
 Tapering of the urethra with a dorsal resection
of a wedge of tissue.
 Reconstruction of the urethra over a catheter
with running suture.
Medial aspect of the labia minora and clitoris
denuded.Initial layer of mons closure.
Approximation of the labia minora over the
urethral reconstruction.
 Second layer of mons closure.
Creation of clitoral hood.

34
Dept of Urology, GRH and KMC,
Chennai.

35. 35
Dept of Urology, GRH and KMC,
Chennai.

36. Postoperative pain and bladder spasm

Controlling bladder spasms is paramount because
they are associated with urinary extravasation and
fistula formation.

36
Dept of Urology, GRH and KMC,
Chennai.

37. All of patients have a caudal epidural catheter
placed at the time of surgery .
Oxybutynin is started immediately after surgery to
decrease the incidenceof bladder spasms .

At the time of discharge, the plastic dressing on the
penis is left intact .

37
Dept of Urology, GRH and KMC,
Chennai.

38.  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
38
Dept of Urology, GRH and KMC,
Chennai.

39. Recent reports have indicated a greater incidence
of cloacal exstrophy associated with maternal
exposure to cigarette smoke .
It is interesting to note that mothers of infants with
cloacal exstrophy were more compliant with
preconceptional folate use.
When neurospinal defects and omphalocele coexist
withcloacal exstrophy, the term
OEIS complex
(omphalocele, exstrophy,
imperforate anus, spinal
defects)
39
Dept of Urology, GRH and KMC,
Chennai.

40. 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
40
Dept of Urology, GRH and KMC,
Chennai.

41. 41
Dept of Urology, GRH and KMC,
Chennai.

42. Secondary to disruption of the tissue of the
dorsal mesenchyme rather than failure of
neural tube closure.
 The defects that lead to the formation of
cloacal exstrophy may lead to the
developing spinal cord and vertebrae being
pulled apart .
Clinically significant neurologic anomaly
was found to negatively affect the
development of continence.
42
Dept of Urology, GRH and KMC,
Chennai.

43. The autonomic bladder innervation being
derived from a more medial location.

This potentially puts the nerve supply in
jeopardy during initial bladder dissection and
reconstruction and can potentially leave the
bladder neuropathic after reconstruction.
43
Dept of Urology, GRH and KMC,
Chennai.

44. 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
44
Dept of Urology, GRH and KMC,
Chennai.

45. Club foot malformations
Absence of feet
Severe tibial /fibular deformities
Congenital hip dislocations

45
Dept of Urology, GRH and KMC,
Chennai.

46. Incidence of omphalocele is around 88%.

Omphaloceles do vary in size and usually
contain small bowel or liver or both.


Immediate closure of the omphalocele defect in
the newborn period is advised to prevent
subsequent rupture.
46
Dept of Urology, GRH and KMC,
Chennai.

47.  Malrotation, duplication anomalies,and
anatomically short bowel occur with
varying frequencies.

 A hindgut remnant of varying size is also
noted in most patients
47
Dept of Urology, GRH and KMC,
Chennai.

48. The most commonly reported müllerian
anomaly was uterine duplication, seen in 95%
of patients.

Partial uterine duplication,predominantly a
bicornate uterus.

Vaginal duplication 65% .
vaginal agenesis 25% to 50% .
48
Dept of Urology, GRH and KMC,
Chennai.

49. Upper urinary tract anomalies 41% to 60% of
patients.
The most common anomalies were pelvic
kidney and renal agenesis.
Hydronephrosis and hydroureter.
Multicystic dysplastic kidneys and fusion
anomalies .
Ectopic ureters draining to the vasa in the male
and into the uterus, vagina, or fallopiann tubes
in the female .



49
Dept of Urology, GRH and KMC,
Chennai.

50.  Genital anomalies in the male have
typically included complete separation of
the two phallic halves and accompanied
separation of the scrotal halves.
Testes may be noted in the scrotum but are
frequently noted to be undescended, and
associated inguinal hernias are a common
finding.


50
Dept of Urology, GRH and KMC,
Chennai.

51. Girls typically have widely divergent clitoral
halves.
The lower urinary tract is composed of two
exstrophied hemibladders flanking the
exstrophied intestinal segment.
Each bladder half usually drains the
ipsilateral ureter.

Each bladder half usually drains the ipsilateral
ureter and is closely related to the ipsilateral
phallic segment.
51
Dept of Urology, GRH and KMC,
Chennai.

52. Cyanotic heart disease
Aortic duplication
A bilobed lung
An atretic right upper lung

Caval duplication
52
Dept of Urology, GRH and KMC,
Chennai.

53. 53
Dept of Urology, GRH and KMC,
Chennai.

54. Major criteria
Nonvisualisation of urinary bladder
A large midline infraumbilical anterior
abdominal wall defect/a cystic anterior wall
structure
Omphalocele
Lumbosacral Myelomeningocele


Early diagnosis may permit appropriate prenatal
counseling for parents and expedite postnatal
care.
54
Dept of Urology, GRH and KMC,
Chennai.

55. Minor criteria
Lower extremity defects
Renal anomalies
Widened pubic arches
Ascites
Hydrocephalus
A single umbilical artery
Narrow thorax
55
Dept of Urology, GRH and KMC,
Chennai.

56.  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 .
56
Dept of Urology, GRH and KMC,
Chennai.

57.  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 ileostomy/colostomy
 Genital revision if needed
57
Dept of Urology, GRH and KMC,
Chennai.

58.  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
58
Dept of Urology, GRH and KMC,
Chennai.

59. Second stage
 Closure of joined bladder halves
and urethroplasty
 Bilateral anterior innominate and vertical iliac
osteotomy
 Genital revision if necessary
59
Dept of Urology, GRH and KMC,
Chennai.

60.  Anti-Incontinence/Reflux Procedure (age 4-
5 yr)
 Bladder capacity ≥100 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
60
Dept of Urology, GRH and KMC,
Chennai.

61. 61
Dept of Urology, GRH and KMC,
Chennai.

62. 62
Dept of Urology, GRH and KMC,
Chennai.

63. Role of osteotomy
Indicated in all children with cloacal
exstrophy at the time of blaader closure
because of wide diastasis that is invariably
present.
Currently combined B/L anterior innominate
and vertical iliac osteotomies routinely used.
63
Dept of Urology, GRH and KMC,
Chennai.

64.  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.
64
Dept of Urology, GRH and KMC,
Chennai.

65.  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.
65
Dept of Urology, GRH and KMC,
Chennai.

66.  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.
66
Dept of Urology, GRH and KMC,
Chennai.

67. 67
Dept of Urology, GRH and KMC,
Chennai.

68.  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
pregnancy achievement.
 Sexual function and libido in exstrophy
patients are normal.
68
Dept of Urology, GRH and KMC,
Chennai.

69.  Vulvoplasty is indicated in patients before
they become sexually active or start using
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.
69
Dept of Urology, GRH and KMC,
Chennai.

70. 70
Dept of Urology, GRH and KMC,
Chennai.