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Exstrophy Epispadias Complex MSRE & CPRE
This document discusses modern techniques for repairing exstrophy of the bladder, including: 1. Modern staged repair of exstrophy (MSRE) involves closure of the bladder and urethra at birth, epispadias repair at 6-12 months, and bladder neck reconstruction at 4-5 years when bladder capacity is adequate. 2. Complete primary repair of exstrophy (CPRE) simultaneously closes the bladder, abdominal wall, and repairs epispadias to decrease costs and morbidity from multiple surgeries. 3. Techniques are described for MSRE bladder closure, epispadias repair using the Cantwell-Ransley technique, and CPRE involving complete dis
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Exstrophy Epispadias Complex MSRE & CPRE
- 1. MSRE and CPRE DrFaheem Ul Hassan Andrabi Fellow Pediatric Urology Prof. Narendrababu Prof. Pediatric Surgery and Pediatric Urology IGICH Banglore
- 2. Modern Staged Repairof Exstrophy • Stage I: bladder and posterior urethral closure soon thereafter birth • Stage II: epispadias repair at 6–12 months of life • Stage III: BNR when the bladder capacity is adequate (usually at 4–5 years of life)
- 3. Modern Staged Repairof Exstrophy • The umbilicus is ligated and trimmed • Incisions are made from the midline above the umbilicus, extending around the margins of the bladder, and forward onto the root of the penis upto the distal limit of the veru montanum.
- 4. Modern Staged Repairof Exstrophy • Bladder Mobilisation • extraperitoneal
- 5. Modern Staged Repairof Exstrophy • Completion of the dissection around the periphery of the bladder and the urethral plate.
- 6. Modern Staged Repairof Exstrophy • Inversion of the bladder plate and approximation of the corpora . Also note the inferior paraexstrophy incisions.
- 7. Modern Staged Repairof Exstrophy • Closure of the skin over the corpora and their partial freeing from the pubis
- 8. Modern Staged Repairof Exstrophy • Placement of a suprapubic drainage tube.
- 9. Modern Staged Repairof Exstrophy • Further closure of the skin inferiorly, • approximation to the UP • Creation of the paraexstrophy flaps
- 10. Modern Staged Repairof Exstrophy • The urethral plate is prepared for tubularization over a catheter.
- 11. Modern Staged Repairof Exstrophy • The urethral plate is now tubularized, • ureteral catheters are placed bilaterally • Bladder being closed
- 12. Modern Staged Repairof Exstrophy • Completion of tubularization of the bladder and urethra, and location of the various drainage tubes
- 13. 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.
- 14. Modern Staged Repairof Exstrophy • Sutures are placed to for approximation of the pubic halves
- 15. Modern Staged Repairof Exstrophy • Drainage tubes are brought out superiorly, • Approximation of the pubis helps in successful closure of bladder and abdominal wall
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- 17. Cantwell-Ransley epispadias repair. •The urethral plate is dissected from the corpora and is tubularized
- 18. Cantwell-Ransley epispadias repair. •Corporotomies are created at the midphallus, and the urethra is transposed to the ventral surface.
- 19. 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.
- 20. Modern Staged Repairof Exstrophy • The distal urethral plate at the tip of the glans is incised longitudinally and the incision is closed transversely with fine, absorbable sutures. This ‘IPGAM’ maneuver brings the urethral meatus more ventrally on the glans.
- 21. Modern Staged Repairof Exstrophy • Following epispadias repair, the patient’s bladder capacity is measured annually with gravity cystogram under anesthesia.
- 22. MSRE Stage 1+ 2: Combined Exstrophy and Epispadias Closures faied repair (30), and delayed repair (5) Results were comparable with conventional MSRE repair However Osteotomy in this approach is necessary
- 23. Modern Staged Repairof Exstrophy • Cephalotrigonal ureteric reimplantation is done • a strip of bladder mucosa ~ 1.5–1.8 cm wide and 3–4 cm long is generated • A tube is formed over 8 French catheter Bladder Neck Reconstruction YDLB
- 24. 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
- 25. 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 • 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
- 26. Modern Staged Repairof Exstrophy • Use of osteotomies and patient immobilization through spica casting or Bryant’s traction increases the success of bladder closure and subsequent continence. • Delayed bladder closure increases the likelihood of bladder augmentation due to inadequate bladder capacity that, in turn, reduces the chance for voluntary voiding. Results of BNR
- 27. Modern Staged Repairof Exstrophy Results of BNR • Bryant’s traction after exstrophy closure without osteotomies.
- 28. Modern Staged Repairof Exstrophy Results of BNR • Patient seen with an external fixator and in modified Buck’s traction.
- 29. 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. less invasive alternatives to the Young-Dees Leadbetter BNR should be sought
- 30. 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
- 31. Modern Staged Repairof Exstrophy Artificial Urinary Sphincter – Plagued by multiple operative revisions – Used only in older children – AUS erosions into bladder and bowel may occur Bladder Neck Wraps and Slings – does not consistently maintain long-term urinary continence, especially in male patients Management of failed BNR
- 32. Modern Staged Repairof Exstrophy • Bladder Neck Bulking Agents • Bulking agents can be used as an adjunctive procedure for those patients who demonstrate slight leakage due to stress incontinence after BNR • 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
- 33. Modern Staged Repairof Exstrophy • Jeffs and colleagues noted normal upper urinary tracts in 87% • series from Johns Hopkins also achieved low rates of renal damage (17%), • continence rates of 60%–88% are achieved with MSRE Results of MSRE early exstrophy closure in conjunction with pelvic ring approximation, results in a higher rate of continence
- 34. Modern Staged Repairof Exstrophy Results of MSRE
- 35. Modern Staged Repairof Exstrophy Failed Initial Closure – complete bladder dehiscence, – bladder prolapse with obstruction, and – stricture of the posterior urethra. Results of MSRE Failure of the initial closure significantly decreases the chance of continence
- 36. Modern Staged Repairof Exstrophy Dehiscence • Can be prevented by – approximation of the pelvic ring, – adequate pelvic immobilization, – prevention of wound infection, – Prevention of abdominal distention, – Prventing urinary tube malfunction. Results of MSRE The child should be given a 6-month recovery period prior to a second attempt at closure.
- 37. Modern Staged Repairof Exstrophy Bladder Prolapse – Prolapse represents dehiscence of the lower end of the closure. – Occurs due to incomplete pelvic ring closure Results of MSRE
- 38. Modern Staged Repairof Exstrophy Bladder outlet obstruction • is characterized by incresed PVRU. • It can increases the risk of – renal damage by raising storage pressures to dangerous levels – urinary tract infection. Results of MSRE The bladder outlet may be assessed with sounds or bougies 4–6 weeks after closure USG of the bladder and upper tracts should be performed 3 months after closure Treatment is UD and CIC
- 39. Modern Staged Repairof Exstrophy Neourethral stricture • it is due to – paraexstrophy skin flaps in the posterior urethra – suture reaction – urethral stents • RBUS shows features of BOO • Management options include – DU – DVIU – EPA – CUD Results of MSRE
- 40. Complete Primary Repairof Exstrophy
- 41. Complete Primary Repairof Exstrophy In the late 1980s, Mitchell initiated simultaneous bladder and abdominal wall closure with epispadias repair and bladder neck remodeling
- 42. 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
- 43. CPRE • Mark theentire length of UP and bladder • Make a circumcoronal incision Technique
- 44. CPRE • Dissection isinitiated along the lateral aspect of UP • Dissection is above Bucks • NVB is under Bucks Technique
- 45. CPRE • Ventral dissection •CS is left attached to the UP ( common blood supply) Technique
- 46. CPRE • Dissection onmedial aspect is on TAB • UP and CS are lifted as a unit Technique
- 47. CPRE • UP andCS lifted together Technique
- 48. CPRE • Complete disassemblyof CC upto intersymphyseal band • ISB is then divided on both sides Technique
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- 50. CPRE • Division ofthe intersymphyseal band Technique
- 51. CPRE • The prostaticUrethra, bladder and bladder neck should be placed deep in the pelvis Technique
- 52. CPRE • The CCare medially rotated and sutured • Urethra is brought underneath Technique
- 53. CPRE • CCs arebrought together by suturing edges of Bucks Fascia Technique
- 54. CPRE • The urethrais now on the ventrum • Sometimes meatus may be hypospadiatic Technique
- 55. CPRE • Completion ofthe procedure • Umbilicus is made at a point between two iliac crests Technique
- 56. CPRE • Patient iskept on oral antibiotics while the tubes are in place. • If the patient had osteotomies, he is placed in a spika cast for 4 weeks. • If no osteotomies are performed, no special restrictions of activity are given. • The ureteric/urethral stents are removed one at a time starting 5 days after surgery. • The suprapubic tube is not removed until the patient is voiding to completion. • If the patient cannot empty completely, clean intermittent catheterization with a 6 F catheter is started Post-Op care
- 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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- 60. CPRE Outcome However Better bladderstability and compliance was noted
- 61. CPRE • Increased ratesof blood transfusion. • Hypospadias 50% to 82%. • Bladder neck fistulas 41% of CPRE repairs • Partial or complete hemiglans and/or corporal loss has been reported by several authors • bladder rupture has been reported complications
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- 67. CPRE Cosmetic Outcome • Cosmeticoutcome is not very encouraging • significantly small phallus can lead to low self-esteem, psychosocial and psychosexual dysfunction, and suicidal ideation • Thus, neophallic reconstruction has been performed, although this procedure is done in a select few centers and with significant risk for complications
- 68. CPRE Osteotomy • Candidates forosteotomy include those • >72 hours old, • newborns with a wide pubic diastasis of >4 cm, • newborns with CE
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