Enterocutaneous fistulae in familial adenomatous polyposis patients with abdominal desmoid disease.

Aim: Enterocutaneous fistula is a severe complication of intra-abdominal desmoid disease. It is hard to repair because of the presence of the desmoid itself, the possibility of distal obstruction and the complexity of multiple laparotomies. Here we report the outcome of a series of patients presenting with abdominal desmoid disease and associated enterocutaneous fistula.

Method: This is a retrospective, descriptive study of patients presenting to a hereditary colorectal cancer registry with familial adenomatous polyposis-related intra-abdominal desmoid disease and associated enterocutaneous fistulae. Patients were identified through the registry database and aspects of their treatment and outcome were abstracted.

Results: Sixteen patients (11 women, five men) were treated. The mean age at index surgery was 25.2 years and mean time to first fistula was 115.6 (± 92.7 standard deviation) months from index surgery. Index surgery included restorative proctocolectomy with ileal pouch (n = 9), colectomy with ileorectal anastomosis (n = 4) and proctocolectomy with end ileostomy (n = 1). One patient had only a small bowel bypass and another did not have any index surgery. Ten patients underwent laparotomy for the enterocutaneous fistula; eight had a repair ± resection, one had a diversion and one a bypass. All eight patients who had a repair healed, and the bypassed fistula was successfully palliated. Three fistulae recurred and two were successfully repaired at a second procedure. One patient was explored but nothing could be done for the fistula. Two surgery patients died of causes unrelated to the fistula. Six patients received medical treatment, four of whom died.

Conclusion: Selected desmoid-related enterocutaneous fistulae can be repaired successfully.