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EEC
1. “We try to close the neck of bladder in
exstrophy incontinence but it
recanalizes and doesn’t close & when we
try to open it in strictured urethra it
closes and doesn’t open. That makes
the Urethra the craziest thing to deal
with”
Dr. Vinay Jadha
2. Exstrophy: types and timing of surgery
Dr. Faheem Ul Hassan
Fellow Pediatric Urology
Dr. Vinay Jadhav
Assoc. Prof. Pediatric Surgery and
Pediatric Urology IGICH Banglore
4. MODERN STAGED REPAIR OF EXSTROPHY
• Stage I: bladder and posterior urethral closure soon thereafter birth
(within 72 hours)
• Stage II: epispadias repair at 6–12 months of life (phalloplasty and
urethroplasty)
• Stage III: BNR when the bladder capacity is adequate (usually at 4–5
years of life) (BC 80 ml)
Timeline
Gearhart
6. Timeline
Early repair
Advantages Disadvantages
Less mucosal changes,
inflammation, fibrosis &
metaplasia
Anaesthetic complications
Doesn’t require osteotomy
More perioperative complications
Parental reaasurance
Hypoxia, acid base abnormalities
and decreased GFR
7. Timeline
Delayed repair repair
Advantages Disadvantages
Less anaesthetic complications Intestinal metaplasia & fibrosis
Less metabolic complications Osteotomy is required
Normal bladder cycling and
growth
Mother child bonding
13. MODERN STAGED REPAIR OF EXSTROPHY
• Inversion of the bladder plate and approximation of the
corpora . Also note the inferior paraexstrophy incisions.
Functional bladder closure
14. MODERN STAGED REPAIR OF EXSTROPHY
• Closure of the skin over the corpora and their partial
freeing from the pubis
Functional bladder closure
15. MODERN STAGED REPAIR OF EXSTROPHY
• The urethral plate is prepared for tubularization over
a catheter.
Functional bladder closure
16. MODERN STAGED REPAIR OF EXSTROPHY
• The urethral plate is now tubularized,
• ureteral catheters are placed bilaterally
• Bladder being closed
Functional bladder closure
17. MODERN STAGED REPAIR OF EXSTROPHY
• After two-layered closure of the bladder and urethral plate, the
bladder is reduced into the pelvis and fixed with sutures.
Functional bladder closure
18. MODERN STAGED REPAIR OF EXSTROPHY
• Sutures are placed to for approximation of the pubic halves
24. CANTWELL-RANSLEY EPISPADIAS REPAIR.
• Corporotomies are created at the midphallus, and the urethra is transposed to
the ventral surface.
Epispadias repair
25. CANTWELL-RANSLEY EPISPADIAS REPAIR.
• The corpora cavernosa are rotated medially and reapproximated at the corporotomy sites, pulling
the corporal bodies inward and providing coverage of the neourethra.
Epispadias repair
27. BLADDER NECK RECONSTRUCTION
Use the patient’s own tissue
Use a minimal amount of bladder tissue
Durable and efficacious
Allow easy access to the bladder through
catheterization
Ideal BNR
28. MODERN STAGED REPAIR OF EXSTROPHY
(1) the urethra is stricture-free and capable of
catheterization
(2) under anesthesia, the bladder capacity is 60–85 cc;
and
(3) the child is mature enough to participate in the
postoperative voiding program (4-5 years)
Pre-requisites of BNR
29. PROCEDURES TO INCREASE THE
BLADDER OUTLET RESISTANCE
Suspend the bladder
outlet
• Marshall –Marchetti-Krantz
• Burch ‘s procedure
• Raz sling suspension
• McGuire procedure
Compress the bladder
outlet
• AUS
• Bladder outlet wraps
• Injection therapy
32. MODERN STAGED REPAIR OF EXSTROPHY
Bladder Neck Wraps and Slings
• does not consistently maintain long-term urinary
continence, especially in male patients
Management of failed BNR
34. YDL
• First the ureters are reimplanted ( trigonocepahic migration of ureters)
Bladder Neck Reconstruction YDLB
35. YDL
• a strip of bladder mucosa ~ 1.5–1.8 cm wide and 3–4 cm long is
generated
Bladder Neck Reconstruction YDLB
36. YDL
• A tube is formed over 8 French catheter
Bladder Neck Reconstruction YDLB
37. MODERN STAGED REPAIR OF EXSTROPHY
• Surgical success is defined as a dry interval of >2–3 hours
and spontaneous voiding without catheterization.
• YDL-BNR has a urinary continence rates of 30%–80+% for
patients (Gearhart)
• Many factors influence the outcome of surgery.
• initial failed bladder closure or
• prior failed BNR
• A preoperative bladder capacity of <85 ccs
Results of BNR
38. MODERN STAGED REPAIR OF EXSTROPHY
Results of BNR
Approximately 80% of patients had compliant and stable bladders before BNR
19% maintained normal filling and voiding dynamics after reconstruction.
39. MODERN STAGED REPAIR OF EXSTROPHY
Results of BNR
8 of 13 patients with initially successful BNR required further surgery in
second decade of life because of poor compliance and incontinence
40. MODERN STAGED REPAIR OF EXSTROPHY
• Bladder Neck Closure with CUD
• BNC with Mitrofanoff is an option in those patients who have
failed multiple attempts at BNR.
• This option eliminates the chance to void per urethra
Management of failed BNR
42. COMPLETE PRIMARY REPAIR OF EXSTROPHY
In the late 1980s, Mitchell initiated simultaneous bladder and abdominal
wall closure with epispadias repair and bladder neck remodeling
43. CPRE
this technique may
• decrease costs,
• decrease the morbidity associated with multiple
operations
• stimulate early bladder cycling and growth.
• Complete disassembly allows placement of PU and
bladder necks in the pelvis
• Fosters improved bonding between the parents and
infant.
Proponents argue
44. CPRE
• Mark the entire length of UP and bladder
• Make a circumcoronal incision
Technique
45. CPRE
• Dissection is initiated along the lateral aspect of UP
• Dissection is above Bucks
• NVB is under Bucks
Technique
52. CPRE
• The prostatic Urethra, bladder and bladder neck should
be placed deep in the pelvis
Technique
53. CPRE
• The CC are medially rotated and sutured
• Urethra is brought underneath
Technique
54. CPRE
• CCs are brought together by suturing edges of Bucks
Fascia
Technique
55. CPRE
• The urethra is now on the ventrum
• Sometimes meatus may be hypospadiatic
Technique
56. CPRE
• Completion of the procedure
• Umbilicus is made at a point between two iliac crests
Technique
57. CPRE
Results
20% boys 43%, girls have achieved primary urinary continence without the need for BNR
additional 18% of boys and girls achieved continence with only bladder neck injection
Rest were treated with BNR (mitchell)
62. CR VS MITCHELL
complications
CR Mitchell
High fistula rate Hypo difficult to treat
Dorsal Chordee Penile loss
Short penile length Loss of glans
Bifid glans
Fistula
63. CR VS MITCHELL
Shortening of urethral plate 97%
Narrowing of urethral plate 30-50%
satisfying cosmetic results 80%
Postoperative complications encountered in our patients were dominated by
fistulas and dehiscence, particularly in patients who had EEC
64. CR VS MITCHELL
described a modification of the penile disassembly
technique preserving the connection between the distal
urethral wedge and the corporal bodies to decrease the
incidence of hypospadias
65. Indications
• Markedly reduced BC or
• Poor compliance
• Incontinence becomes bothersome impairs a patient’s lifestyle
despite medical treatment
• when high-pressure urinary storage places the upper urinary
tracts at risk.
• Storage Pressures >40cm Of H20
ACP
66. • Patients who are unable or unwilling to perform life-long
intermittent CIC
• Patients with inflammatory bowel disease (especially Crohn
disease),
• short or irradiated bowel,
• bladder tumors,
• severe radiation cystitis.
ACP
contraIndications
67. ACP
In patients for whom a Mitrofanoff is planned, some surgeons
routinely perform BNC
however, if the outlet is intact and continent, it should be maintained
to provide an alternative route for catheterization
