First stage approximation of the exstrophic bladder in patients with cloacal exstrophy--should this be the initial surgical approach in all patients?

PURPOSE

Cloacal exstrophy is rare and it represents a reconstructive challenge. Options for managing the urinary tract include primary closure or approximation of the bladder halves in the midline with later closure. We present our observations and evolving thoughts concerning optimal treatment in these patients.

MATERIALS AND METHODS

We retrospectively reviewed the records of patients with cloacal exstrophy seen in the last 5 years. Initial management was examined, including complete primary closure vs a staged approach. We noted midline defects, spinal cord abnormalities or other anatomical reasons that precluded primary closure.

RESULTS

Seven patients, including 5 females and 2 males, were identified. An omphalocele noted in all 7 patients was closed in 5 at initial operation. All underwent preservation of the hindgut in the fecal stream. Spinal cord tethering was noted in 7 of 7 cases. Complete primary bladder closure was performed in 3 of the 7 patients, while the size of the bladder plates or a large abdominal wall defect precluded closure in the remainder. Continence was not achieved in the 3 cases closed primarily. All patients achieving urinary continence underwent bladder neck closure and augmentation cystoplasty with a continent catheterizable channel.

CONCLUSIONS

Patients with cloacal exstrophy have anatomical issues that can prevent complete primary bladder closure or preclude the achievement of urinary continence. The high incidence of tethered cord places these patients at risk for upper tract changes and bladder decompensation during followup. Despite successful primary closure in 3 of 7 patients all have a tiny bladder and require secondary procedures to become continent. Extensive dissection during the first operation can contribute to more difficult dissection with potential increased morbidity during subsequent surgeries. Therefore, the best initial approach for the typical patient may be closure of the abdominal wall and approximation of the exstrophied bladder halves in the midline. Secondary closure with continent diversion and reconstruction of the external genitalia can be performed at ages 18 to 24 months.