Cloacal exstrophy--pull-through or permanent stoma? A review of 53 patients

Patients with cloacal exstrophy have complex anomalies of the genitourinary and gastrointestinal tract with a spectrum of colonic length. Often, colon is lost during the initial management by use of ileostomies and for urologic and genital reconstruction. It is a common belief that these patients require permanent stomas, which we hypothesized is inaccurate, and therefore reviewed our experience with exstrophy, focusing specifically on a patient's potential to undergo a colonic pull-through.

All patients with exstrophy or exstrophy variant treated by the authors were retrospectively reviewed. Their ability to form solid stool was assessed via bowel management involving a constipating diet, antidiarrheals, bulking agents, and a daily enema through the stoma. Patients who underwent successful bowel management through the stoma were offered a pull-through.

Fifty-three patients were treated over a 26-year period, including typical cloacal exstrophy (27), or a covered variant (16), and complex anorectal malformations with short colon (10). Newborn operations (48 done at other institutions, 5 by us) involved ileostomy in 11 or end colostomy in 42. Eight patients with ileostomies suffered acidosis and failure to thrive and underwent "rescue" operations to incorporate all defunctionalized colon into the fecal stream. Four had colon used for their urologic reconstruction and 6 for their genital reconstruction, leaving them borderline or unable to form solid stool. Twenty-three are undergoing bowel management or being observed for growth of the colonic pouch to determine if they are pull-through candidates. Of the others, 90% (27/30) underwent colonic pull-through. Ten percent (3/30) had a permanent stoma. Of 20 available for follow-up after pull-through, 17 are clean with bowel management (85%), 2 (10%) have voluntary bowel movements with occasional soiling, and 1 is incontinent but noncompliant.

Indication for pull-through depends on successful bowel management through the stoma, which depends on the ability to form solid stool. To maximize this potential, it is crucial to use all available hindgut for the initial colostomy and avoid use of colon for urologic or genital reconstruction. Most patients have poor prognosis for bowel control but can remain clean with bowel management. Our experience indicates that a permanent stoma is not required for the most of these patients and that bowel management can keep them clean, which we believe provides them with a better quality of life. Using these criteria, most exstrophy patients, contrary to popular belief, are candidates for a pull-through.