Continent urinary diversions in the exstrophy complex: why do they fail?

Urinary continence is one of the primary goals in the surgical treatment of the patient with exstrophy. Children with inadequate bladder capacity, noncompliant or neuropathic bladder, or failed bladder neck reconstruction may require creation of a continent urinary reservoir to achieve continence. While initial success rates are excellent, some patients suffer persistent urinary incontinence, severely affecting their quality of life. The reasons for such failure and subsequent management were evaluated.

A retrospective database review was performed in 748 patients with the exstrophy complex. Patients with a history of failed continent urinary diversion with incontinence from the stoma or urethra were identified. Initial diagnosis, number of previous operations, reason for and time interval after surgery when failure occurred, and management strategies were evaluated.

Of 92 patients undergoing continent diversion procedures 19 (21%) had failure initially (15 with classic and 4 with cloacal exstrophy). Four of the 19 patients underwent primary surgery at our institution and 15 were referred. The procedure initially performed was an intussuscepted nipple valve in 8 patients, an intussuscepted ileocecal valve in 1 and an ileal or sigmoid reservoir with appendiceal flap valve (Mitrofanoff) in 10. Main reasons for failure included de-intussusception and bladder neck incompetence. Mean followup after continence was achieved was 2.9 years (range 3 months to 10 years). These patients underwent up to 8 operations to achieve continence (mean 5). Most patients (79%) were successfully treated with either 1 (47%) or 2 revisions (32%), while 1 required 3 reoperations. Overall, 89 of 92 patients (97%) are currently continent, including those requiring medications and further surgical procedures, while 3 are still wet.

Most patients with exstrophy with failure of continent urinary diversion have a long complicated surgical history. In this series incontinence from an intussuscepted nipple stoma was mostly due to de-intussusception. In patients with an initial Mitrofanoff stoma all incontinence occurred within the first few months postoperatively. Continence was achieved by either recreation of a Mitrofanoff stoma or bladder neck transection. Patients with a failed continent urinary diversion benefit from careful preoperative evaluation, meticulous surgical execution and persistent attempts to achieve continence.