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EEC
The document discusses various surgical techniques for repairing exstrophy of the bladder, including: 1. Early complete primary repair of exstrophy aims to close the bladder and abdominal wall simultaneously with epispadias repair and bladder neck reconstruction in one stage. 2. Staged repair involves initial bladder closure followed by later epispadias repair and bladder neck reconstruction once the bladder has grown adequately. 3. Bladder neck reconstruction aims to increase outlet resistance but has varying success rates, and patients may later require additional surgeries like Y-V plasty if incontinence develops. 4. Augmentation cystoplasty is an option for patients with small non-compliant bladders or
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- 1. “We try toclose 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 andtiming of surgery Dr. Faheem Ul Hassan Fellow Pediatric Urology Dr. Vinay Jadhav Assoc. Prof. Pediatric Surgery and Pediatric Urology IGICH Banglore
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- 4. MODERN STAGED REPAIROF 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
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- 6. Timeline Early repair Advantages Disadvantages Lessmucosal 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 AdvantagesDisadvantages Less anaesthetic complications Intestinal metaplasia & fibrosis Less metabolic complications Osteotomy is required Normal bladder cycling and growth Mother child bonding
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- 10. EXSTROPHY Early or delayed CPRE<72 h CPRE>72h 0% Retention 38% retention 33% required BNR (FUP 9 years) 0 Required BNR ( FUP 2 years)
- 11. MODERN STAGED REPAIROF EXSTROPHY Functional bladder closure
- 12. MODERN STAGED REPAIROF EXSTROPHY Functional bladder closure
- 13. MODERN STAGED REPAIROF EXSTROPHY • Inversion of the bladder plate and approximation of the corpora . Also note the inferior paraexstrophy incisions. Functional bladder closure
- 14. MODERN STAGED REPAIROF EXSTROPHY • Closure of the skin over the corpora and their partial freeing from the pubis Functional bladder closure
- 15. MODERN STAGED REPAIROF EXSTROPHY • The urethral plate is prepared for tubularization over a catheter. Functional bladder closure
- 16. MODERN STAGED REPAIROF EXSTROPHY • The urethral plate is now tubularized, • ureteral catheters are placed bilaterally • Bladder being closed Functional bladder closure
- 17. MODERN STAGED REPAIROF 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 REPAIROF EXSTROPHY • Sutures are placed to for approximation of the pubic halves
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- 21. CANTWELL-RANSLEY EPISPADIAS REPAIR. •IPGAM Epispadias repair
- 22. CANTWELL-RANSLEY EPISPADIAS REPAIR. •The urethral plate is dissected from the corpora Epispadias repair
- 23. CANTWELL-RANSLEY EPISPADIAS REPAIR. •Urethral reconstruction (urethroplasty) Epispadias repair
- 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
- 26. BLADDER NECK RECONSTRUCTION 4-5years of age Bladder capacity should be 65 -80 ml Timing
- 27. BLADDER NECK RECONSTRUCTION Usethe 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 REPAIROF 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 INCREASETHE 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
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- 31. BN SUSPENSION BYRECTUS FASCIAL SLING
- 32. MODERN STAGED REPAIROF EXSTROPHY Bladder Neck Wraps and Slings • does not consistently maintain long-term urinary continence, especially in male patients Management of failed BNR
- 33. SURGICAL RECONFIGURATION OF THE BLADDEROUTLET • YDL • Mollard • Koff • Mitchell • Koyle • Arap • Tanagho • Kropp • Pippi Salle • BNC
- 34. YDL • First theureters are reimplanted ( trigonocepahic migration of ureters) Bladder Neck Reconstruction YDLB
- 35. YDL • a stripof bladder mucosa ~ 1.5–1.8 cm wide and 3–4 cm long is generated Bladder Neck Reconstruction YDLB
- 36. YDL • A tubeis formed over 8 French catheter Bladder Neck Reconstruction YDLB
- 37. MODERN STAGED REPAIROF 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 REPAIROF 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 REPAIROF 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 REPAIROF 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
- 41. COMPLETE PRIMARY REPAIROF EXSTROPHY
- 42. COMPLETE PRIMARY REPAIROF 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 theentire length of UP and bladder • Make a circumcoronal incision Technique
- 45. CPRE • Dissection isinitiated along the lateral aspect of UP • Dissection is above Bucks • NVB is under Bucks Technique
- 46. CPRE • Ventral dissection •CS is left attached to the UP ( common blood supply) Technique
- 47. CPRE • Dissection onmedial aspect is on TAB • UP and CS are lifted as a unit Technique
- 48. CPRE • UP andCS lifted together Technique
- 49. CPRE • Complete disassemblyof CC upto intersymphyseal band • ISB is then divided on both sides Technique
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- 51. CPRE • Division ofthe intersymphyseal band Technique
- 52. CPRE • The prostaticUrethra, bladder and bladder neck should be placed deep in the pelvis Technique
- 53. CPRE • The CCare medially rotated and sutured • Urethra is brought underneath Technique
- 54. CPRE • CCs arebrought together by suturing edges of Bucks Fascia Technique
- 55. CPRE • The urethrais now on the ventrum • Sometimes meatus may be hypospadiatic Technique
- 56. CPRE • Completion ofthe 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)
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- 59. CPRE • Increased ratesof blood transfusion. • Hypospadias 50% to 82%. • Bladder neck fistulas 41% of CPRE repairs • Partial or complete hemiglans and/or corporal loss • bladder rupture has been reported • Reccurent operations for Hypo, VUR, VCF, failed primary repair, bladder prolapse and bladder dehiscence. complications
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- 62. CR VS MITCHELL complications CRMitchell High fistula rate Hypo difficult to treat Dorsal Chordee Penile loss Short penile length Loss of glans Bifid glans Fistula
- 63. CR VS MITCHELL Shorteningof 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 describeda 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 reducedBC 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 whoare 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 forwhom 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
- 68. ACP Types • Intestinal cystoplasty •Ileo cystoplasty • Sigmoid cystoplasty • Ileocecal cystoplasty • Gastro cystoplasty • Autoaugmentation cystoplasty • Uretero cystoplasty
- 69. ACP Types • Intestinal cystoplasty •Ileo cystoplasty • Sigmoid cystoplasty • Ileocecal cystoplasty • Gastro cystoplasty • Autoaugmentation cystoplasty • Uretero cystoplasty
- 70. ACP Complications • Urolithiasis andmucous production • Perforation • Metabolic derrangements • hypercontractility and incontinence • UTIs • Hematuria Dysuria syndrome • Malignancy • Diarrhoea & Vit B12 defficiency
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