The "rescue operation" for patients with cloacal exstrophy and its variants.

Abstract

A common error in the initial operative management of patients with cloacal exstrophy is to create an ileostomy leaving the colon defunctionalized and connected to the urinary tract. These patients benefit from a "rescue operation" to give them the best opportunity to be future pull-through candidates. Nineteen patients were identified who underwent an inadequate diversion during the newborn period, leaving a distal defunctionalized colon, and required a "rescue operation". A retrospective review of the medical records of these patients was performed. A piece of colon was disconnected from the urinary tract, rescued from the pelvis, and incorporated into the fecal stream. The original stoma was closed, and an end colostomy was created. Fifteen patients were females and four were males. The length of rescued colon ranged from 5.5 to 20 cm. Symptoms present before the operation included: hyperchloremic acidosis (6), urinary tract infections (6), failure to thrive (5), sepsis (1), dehydration (1), and TPN dependent (1). There was resolution of these symptoms post-operatively. On follow up, 10 patients still have their colostomies as we are waiting for continued colonic growth, 6 patients had a pull-through after responding to our bowel management program through the stoma, 2 patients have a permanent stoma, and one patient expired. When patients with cloacal exstrophy, or its variants, receive an ileostomy or proximal colostomy at birth, a rescue operation should be attempted.