Clinical characteristics of familial adenomatous polyposis and management of duodenal adenomas

Abstract

The aim of this study was to evaluate the clinical characteristics of patients with familial adenomatous polyposis (FAP) undergoing surgical treatment over a 10-year period and specifically to evaluate the incidence and clinical outcome of patients treated for duodenal adenomas. Patients with FAP who underwent surgical treatment for colonic polyposis at the University of Louisville from January 1992 to July 2002 were investigated. Surgical treatment included colectomy and ileal J-pouch-anal anastomosis (IPAA) or completion proctectomy with or without IPAA in those who had previously undergone subtotal colectomy elsewhere. All patients underwent screening gastroduodenoscopy at 3-year intervals beginning at the time of diagnosis or referral. Postoperative morbidity, mortality, and functional outcome were evaluated, as well as the occurrence of extracolonic manifestations and results of treatment for duodenal adenomas when required. Fifty-four patients were included in the study (mean age 28 ± 2 years). Twentyseven of them (50%) underwent colectomy and IPAA as the initial operation. Twenty-seven patients had previously undergone subtotal colectomy. Eight of these 27 patients had cancer in the rectum, of which three were T4 and one was T2N1 cancer. Twenty-two patients underwent a completion proctectomy and three required abdominoperineal resection. Twenty of the 54 patients developed duodenal adenomas. The mean age of diagnosis of duodenal disease was not significantly different from that of patients who were still free of duodenal polyps (40 ± 11 vs. 34 ± 12 years). Seven of these 20 patients underwent local excision of duodenal polyps (either endocopically or transduodenally); four of these patients developed recurrent disease. Six patients underwent pancreaticoduodenectomy for duodenal adenomas with severe dyplasia. These patients experienced an increased number of bowel movements, from five per day (range 4 to 8) to 10 per day (range 6 to 15). One patient required pouch excision and end ileostomy to control diarrhea. Our data demonstrate the following: (1) patients with FAP who have undergone prior subtotal colectomy and ileorectal anastomosis have a high risk of developing advanced cancer in the rectal stump; (2) duodenal adenomas are common in patients with FAP and may occur at an early age; (3) screening duodenoscopy should be initiated at the time of diagnosis of FAP; (4) local excision of duodenal adenomas is associated with a high risk of local recurrence; and (5) even though pancreaticoduodenectomy is the treatment of choice for advanced duodenal adenomas, this procedure may adversely affect pouch function in some patients.