Abstract
Purpose: We evaluated postoperative surveillance and long-term outcome for patients with familial adenomatous polyposis (FAP) who underwent total colectomy with ileorectal anastomosis (IRA). Materials and Methods: During the 30 years from 1970 to 2002, 72 patients with FAP underwent surgery, of whom 29 were treated by total colectomy with IRA for patients with rectal sparse polyposis without cancer. Results: The mean age of the 29 cases was 28.9 years, involving 16 men and 13 women. Mean follow-up after IRA was 19.7 years (range; 1.3-30 years). Eight of the 29 (27.6%) treated by IRA developed cancer in the residual rectum, i.e., 3 men and 5 women. All cases were diagnosed as rectal sparse polyposis, but 4 were profuse colon polyposis. Seven had an APC gene mutation (codon 348-785: 1 case, codon 658-1283:3 cases, codon 1099-1700:3 cases). Mean follow-up between initial and second surgery was 15.0 years (range; 1.3-30). Five of 7 developed advanced cancer, and 2 died of cancer of the residual rectum, but all received regular follow-up at least once a year. Conclusions: These cases suggest that we conduct the restorative proctocolectomy with ileal J-pouch anal canal anastomosis (IACA) or ileal J-pouch anal anastomosis (IAA) for FAP patients, and should be careful to undertake lifelong surveillance.
