Cloacal exstrophy: a unified management plan.

BACKGROUND/PURPOSE: The belief that patients with cloacal exstrophy have a short and therefore useless colon is all too common. Frequently, the colon is used for urinary or vaginal reconstruction, and the possibility of a pull-through is lost. In the authors' experience, the use of a unified management plan allowed most patients to undergo pull-through and avoid a permanent stoma.

METHODS: Twenty-five patients were treated for cloacal exstrophy in the authors' institution from 1985 through 1999. In all patients, bladder closure, omphalocele repair, and creation of a colostomy were performed at birth. All available colon, no matter how small, was incorporated into the fecal stream. After at least 1 year, patients were assessed for the ability to form solid stool through their stoma. Normal colonic length, capacity to form solid stool, or success with a bowel management regimen through the stoma were considered indications for pull-through. Genitourinary reconstruction was contingent on the colorectal plan.

RESULTS: Colonic length ranged from normal in 12 patients, 40 to 70 cm in 3 patients, 10 to 30 cm in 4 patients, and less than 10 cm in 2 patients. All 25 patients underwent pull-through. Three are totally continent, 4 are continent with occasional soiling, 11 remain clean with a bowel management regimen, and 4 are too young to assess. One patient was clean, but now refuses bowel management. Two early patients, both with less than 10 cm of colon, now have ileostomies.

CONCLUSIONS: During neonatal repair, a colostomy should be formed incorporating all pieces of colon, no matter how small. With time, most patients will be able to form solid stool, and a pull-through should be undertaken if that ability exists. Decisions regarding genitourinary reconstruction should be made only after the gastrointestinal plan is established to achieve the optimal use of available bowel.