The newborn exstrophy bladder inadequate for primary closure: Evaluation, management and outcome

Purpose: The surgical approach to the small newborn exstrophy bladder inadequate for primary closure remains undetermined. Various methods for long-term management have been implemented. We evaluated our experience with late primary closure of the small exstrophied bladder template. Materials and Methods: Our institutional database of patients treated and followed for the exstrophy-epispadias complex was reviewed. Of these patients 19 had a bladder template that was too small to close in the newborn period. The treatment and outcome of these 19 patients were reviewed. Results: Of the 19 children who had delayed closure due to a small bladder template 14 were males and 5 were females. Follow-up from birth ranged from 2 to 36 years (mean 18 years). Primary closure was performed at a mean patient age of 13 months (range 6 months to 2 years). Pelvic osteotomy was performed in 16 patients. Of the 19 patients 9 achieved continence after gaining a bladder capacity sufficient for bladder neck reconstruction, 4 required enterocystoplasty to augment bladder volume and perform clean intermittent catheterization (2 per stoma and 2 per urethra), 1 required a colon conduit for an extremely small bladder, and 1 underwent cystectomy and ureterosigmoidostomy for rhabdomyosarcoma. Four patients are currently incontinent, including 3 who are awaiting bladder neck reconstruction and 1 who has frequent nighttime incontinence that is medically managed. Conclusions: Delayed primary closure of the small bladder exstrophy template can allow the native bladder tissue adequate time to grow to a size feasible for successful closure. Epispadias repair can usually be performed at the same time and is facilitated by prior testosterone administration. Bladder neck reconstructive techniques have achieved continence without the need for augmentation or bladder replacement in 47% of the patients in our series. For patients who do not achieve adequate capacity for bladder neck reconstruction, preservation of the native bladder template facilitates future augmentation and ureteral reimplantation, thus requiring use of less bowel in the growing child.