Female exstrophy: failure of initial reconstruction and its implications for continence.

PURPOSE

Bladder exstrophy is a rare malformation affecting only 1 female out of every 5 patients. In the female initial closure is combined with reconstruction of the outer genitalia, and urinary continence can be achieved by some girls without the need for later bladder neck reconstruction. We evaluated the management and outcome of failed initial closures in the female exstrophy population.

MATERIALS AND METHODS

We performed a retrospective database review of patients with the exstrophy complex. Females with classic bladder exstrophy with failure of initial closure were identified. Age at initial closure, use of osteotomies, reasons for failure and number of closures, as well as definitive treatment and long-term outcomes were evaluated.

RESULTS

Of 71 females with classic bladder exstrophy 14 had failure of initial closure. Of these patients 1 had undergone initial closure at our institution and 13 were referred for reclosure. Mean followup was 6.5 years (range 3 to 12) and mean age was 10 years (4 to 14). The patients underwent a maximum of 3 closures (mean 2.4). Initial osteotomies were performed in 4 patients, no osteotomy in 8 and status was unknown in 2. Reason for initial failure was dehiscence in 11 patients and prolapse in 3. Five patients underwent a second closure elsewhere. On referral reclosure was successful using osteotomies in all patients. Bladder neck reconstruction was done in 5 patients (3 are daytime continent) and continent diversion in 4 (all are dry). The other patients are awaiting final treatment.

CONCLUSIONS

The single most important step to achieve urinary continence is successful initial bladder and posterior urethral closure. Pelvic osteotomies ensure a tension-free closure and enhance bladder outlet resistance. Radical mobilization of the vesicourethral complex allows placement of the bladder deep within the pelvis. Failure of the initial closure in the female exstrophy population has a severe impact on long-term outcome and quality of life.