Alternative management of bladder exstrophy

Recent long-time outcome studies of patients with bladder exstrophy treated with primary urinary diversions or primary reconstruction force pediatric urologists to reassess the place of alternative management options in the armamentarium of bladder exstrophy treatment.

After classic ureterosigmoidostomy (USS) or Mainz II pouch, continence rates are very high even in the long-term follow-up with a mean of 50 years. In contrast, multiple procedures including bladder neck repair (BNR) were needed in the majority of patients after complete primary repair of bladder exstrophy (CPRE) to achieve normal voiding and to provide cosmetically acceptable and functional genitalia. After multiple staged repair of bladder exstrophy (MSRE), the requirements of multiple procedures including bladder augmentation to achieve urinary continence, not normal voiding, is well known. The augmentation rates published in the literature range form 0 to 82%. There is increasing concern that the risk for tumor formation might be the same for any type of inclusion of intestinal tissue in the urinary tract even without the direct exposure to the fecal stream.

The hope of providing normal voiding with a single operation (CPRE) might not be fulfilled even in the most skilled hands. This means that existing concerns after reconstructive techniques in bladder exstrophy regarding risk of tumor formation and BNR not standing the test of time remain important. Long-term psychosocial and sexual outcomes, including fertility of patients after reconstruction or primary diversion, will need to be taken into consideration.