Cloacal exstrophy: a unified management plan

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.

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.

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.

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.