Ileo-caecal doughnut bladder augmentation in children with exstrophy- epispadias complex- a study of 100 cases.

AIM: The small bladder in failed exstrophy needs to be augmented and also accommodate bladder neck surgery, ureteric re-implantations and a catheterizable channel. We present a novel method of ileocecal bladder augmentation providing a non-implanted, un-detached appendicular channel, rendered non refluxing by an ileal "fundoplication". The colonic segment could receive ureteric reimplantation when bladder plate is unsuitable. MATERIALS AND METHODS: Operative technique: Bilateral cephalo-trigonal reimplantation and bladder neck surgery is done. The ileocecal segment is isolated and arranged as a ring (doughnut) around the un-detached appendix which is made to pass behind the terminal ileum, through the ileal mesentery and then in front of the ileum as it exits as a catheterizable channel. The bowel segment is opened on the antimesenteric aspect except for the retro appendiceal ileal segment. The opened ileo-caecum is reconfigured as a de-tubularised augmentation pouch while the retro appendiceal ileal segment is wrapped around the appendix like a fundoplication. The colon segment may receive the ureteric reimplantation if required. CASE MATERIAL: 100 children (mean age 6.3 years) with failed exstrophy underwent doughnut augmentation (3 neobladders) in 2006-2021. Ninety eight were incontinent, with 30 open/dehisced bladder plate, 48 units were hydronephrotic (HUN), 105 ureters were refluxing (VUR) and eGFR was subnormal in 20 children. 79 children underwent YDL bladder neck plasty and 23 underwent bladder neck closure (primarily in 18). Three infants with HUN did not undergo bladder neck surgery. 126 ureters were reimplanted into the bladder, 44 into the colon segment while 30 were not reimplanted. RESULTS: 95 children are continent on CIC, which was gradually introduced starting with once or twice daily change of indwelling Mitrofanoff catheter leading to regular 3-4 hourly CIC as the child became confident. The appendicular channel was easily catheterizable without any leak in 95, had minor issues in 4 while one necrosed and replaced by a gastric tube. HUN recovered (25/48) or stabilized with a significant reduction in the grade of hydronephrosis (p = 0.001). VUR resolved in 90% of bladder reimplants, 85% of colonic reimplants and 70% of non-reimplanted ureters. In the 20 children with compromised renal function eGFR improved (71-106 ml/m2/min) significantly (p = 0.036) at follow up (mean 5 years). Urodynamics study (N = 33) showed a mean capacity of 95% of expected bladder capacity (EBC) with end filling pressure less than 25 cm of water (Mean end filling cystometric pressure = 15 cm of water). Complications included adhesive intestinal obstruction (N = 16) and bladder calculi (N = 12). CONCLUSION: The doughnut ileocecal augmentation provides a easily catheterizable, non-refluxing, appendicular channel, not requiring isolation of the appendix nor its reimplantation into the bladder. The colonic segment can provide a bed for ureteric reimplantation. Dryness with a good capacity bladder and preserved upper tract is achieved.

Skin cover in epispadias repair by dorsal transposition of a ventral island flap: a modification of the Pippi Salle technique.

PURPOSE: To describe a technique for a reliable and cosmetic skin cover for epispadias repair. This is achieved using a ventral flap of prepuce and the penile skin. This is a modification of the technique described by Pippi Salle, who wrapped the penile shaft with the same flap, from one side with a resultant suture line on to the contralateral side of the penis. METHODS: Thirty-five children (18 with pure epispadias and 17 with exstrophy epispadias complex) who underwent epispadias repair by the described technique in our hospital were reviewed. In our technique, a ventral flap of prepuce and penile skin, as described by Pippi Salle, is developed and a button hole is made in the dartos fascia. This button hole is utilized to deliver the shaft ventrally which effectively brings the flap onto the dorsal aspect of the penis. The skin flap is then wrapped in toto around to provide a complete cover on the penile shaft with suture line on the ventral aspect. RESULTS: All the 35 children had satisfactory skin cover with a scarless dorsum of penis. Two children had immediate post-operative ventral suture line breakdown, one requiring resuturing and the other healed spontaneously. CONCLUSION: The 'button hole' technique with dorsal transposition of the prepuce and skin flap effectively prevents the dorsal scar contracture by completely avoiding a longitudinal suture line dorsally. On the other hand, the healed ventral suture line is continuous with the scrotal midline raphe, giving a natural look. The overall result is a sturdy repair with a cosmetic appearance of the penis.

Cervicovaginal atresia with hematometra: restoring menstrual and sexual function by utero-coloneovaginoplasty.

BACKGROUND: Cervicovaginal atresia is a rare Mullerian anomaly. The management of cervicovaginal atresia has evolved from historical recommendations of hysterectomy to various reconstructive procedures more recently. The latter carries a risk of significant morbidity and unknown fertility. We present our experience in the management of this complex anomaly. METHODS: Twenty patients with cervicovaginal atresia were operated in our hospital from January 2004 through December 2013. The details of their anatomical variations and functional outcomes were analyzed. RESULTS: Eighteen out of twenty patients had cervical agenesis. Two patients had cervical hypoplasia. All patients underwent utero-coloneovaginoplasty. Post operatively, all patients have regular menstrual cycles. One patient is married, sexually active and has satisfactory coital function. One patient had a bowel anastomotic leak that required a diversion ileostomy. Two patients developed mild stenosis. One patient has mild neovaginal mucosal prolapse. No patient has developed pyometra. CONCLUSION: Patients with cervicovaginal atresia need to be counselled about the various reconstructive options available and the potential risks. Social and economic factor play a significant role in determining the plan of management. For patients from conservative societies, utero-coloneovaginoplasty provides a safe conduit for the passage of menstrual flow and coitus, at the cost of permanent infertility.

Rectovestibular fistula with vaginal atresia: our experience and a proposed course of management.

BACKGROUND: Rectovestibular fistula with coexisting vaginal atresia poses a surgical dilemma with regard to the timing and type of reconstruction. We present our experience and suggest an appropriate course of management. METHODS: Seven patients with rectovestibular fistula and coexisting vestibular atresia were operated in our hospital during January 2004 through December 2013. The details of their bowel, menstrual and sexual functions were recorded. RESULTS: Five of the seven patients who underwent anoplasty in childhood presented to us in their teens with primary amenorrhea and cyclical abdominal pain. All five had sigmoid colon neovaginoplasty. Four of these had the uterus or its remnants anastomosed to the neovagina. All four have regular menstrual cycles. One patient is sexually active and has satisfactory sexual function. The bowel function in all the five patients is good. The remaining two patients presented in their infancy and had the anorectovestibular fistula left as the neovagina. The recto-sigmoid was pulled down to form the neoanus. Both these patients have bowel incontinence. CONCLUSION: We recommend the rectovestibular fistula be used as the neoanus and not as the neovagina. Delayed bowel vaginal replacement has excellent results and allows for optimal assessment of functioning uterine body or remnants.