Familial adenomatous polyposis (FAP) is a genetic disorder transmitted
in an autosomal dominant pattern. One-half of members of an affected
family will carry the gene, and all carriers will succumb to colon can
cer or extracolonic manifestations if not detected and treated early.
When the diagnosis is made, surgery is indicated. Surgical options inc
lude total proctocolectomy with ileostomy, continent ileostomy, total
colectomy with ileorectal anastomosis, and total proctocolectomy with
deal pouch anal anastomosis. Many diverse factors, such as extent of r
ectal disease, the presence and extent of carcinoma, sphincter functio
n, and extracolonic disease, influence which surgical procedure is mos
t appropriate for the individual patient with FAP. This article review
s the surgical options for treating FAP, with emphasis on specific ind
ications, contraindications, and anticipated outcomes. (C) 1995 Wiley-
Liss, Inc.