Bladder exstrophy is a rare and devastating congenital anomaly. These patients are usually seen immediately after birth where correction can be attempted. Presentation in adulthood where bony fusion has occurred and bladder capacity is very low presents a major surgical challenge. In developing countries, we do find patients with bladder exstrophy-epispadias complex in our clinics, primarily presenting in adulthood with continuous incontinence. Most of them are neglected, socially isolated and emotionally wrecked. Successful surgical correction of this rare anomaly provides gratifying results in functional and cosmetic outcomes and overall quality of life. We present our experience of managing five adult patients with bladder exstrophy who underwent successful repair with continent cutaneous diversion and conduit formation from​ 2016-2018.

Two adult males and three females with ages ranging from 18 to 46 years with complete bladder exstrophy- epispadias complex were managed in past 2 years. None of them had any previous attempts at surgical correction. All patients were subjected for preoperative biopsy from urinary bladder plate. Patients underwent evaluation for upper tracts and thorough systemic evaluation to rule out the possibility of coexisting congenital anomalies. All patients were counseled for self-catheterization​ except one patient who opted for ileal conduit. In all cases, only bladder mucosectomy was performed and vesical plate muscularis was utilized to support anterior abdominal wall reconstruction. All patients underwent urinary diversion with continent cutaneous pouch (CCP) (Penn pouch in 1, Modified Koch’s pouch in 1 and Abol-Enein Double T pouch in 2) and ileal conduit in one patient. None of the patients required osteotomy. Four patients underwent primary abdominal wall closure while one patient underwent abdominal wall reconstruction with anterolateral thigh flap. Both male patients underwent epispadias repair in one stage.

All patients had an uneventful intra- and postoperative course. They are followed up with USG KUB, Renal function tests every 6 monthly. All patients with CCP are continent by day and night and successfully carrying out self-catheterization. None of the patients during the follow-up period developed significant nutritional deficiencies. All patients showed improvement in their self-esteem​ and social acceptance following surgery.

Excellent results in functional and social outcomes still possible at this age of presentation​

Surgical correction with various described surgical techniques for exstrophy repair provide excellent results in functional and social outcomes.