Surgical Treatment for Patients with Familial Adenomatous Polyposis

Abstract

Purpose: We evaluated the surgical treatment and long-term outcome of patients with familial adenomatous polyposis（FAP）who underwent restorative proctocolectomy. The timing of surgery, and selection of surgical procedure, must be carefully decided, considering the lifetime cancer risk of each patient while maintaining quality of life（QOL）at the highest level possible. Materials and Methods: Between 1970 and 2005, we encountered 73 patients with FAP in 16 families. Thirty patients were treated by total colectomy with ileo-rectal anastomosis（IRA）for patients with sparse rectal polyposis without cancer. Twenty-two patients were treated by proctocolectomy with ileal Jpouch anal canal anastomosis （IACA）, and 8 patients were treated by proctocolectomy with ileal J-pouch anal anastomosis（IAA）. Results: Mean follow-up after IRA was 21.7 years. Nine of the 30 （30%） treated by IRA developed cancer in the residual rectum. Five of 9 developed advanced cancer, and 5 died of cancer in the residual rectum. However, none of patients treated by IACA or IAA developed rectal cancer. The mean age at the development of colorectal cancer was 42.3 years in patients with system A（codon 2–479）or system B （codon 348–785） of APC gene mutation. The mean age at the development of colorectal cancer in patients with system C（codon 658–1283）and system D（codon 1099–1700）was 28.8 years. Conclusions: These cases suggest that we should perform restorative proctocolectomy by IACA for patients with system A–B, and by IAA for patients with system C–D.