Surgical options for familial adenomatous polyposis

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 cancer or extracolonic manifestations if not detected and treated early. When the diagnosis is made, surgery is indicated. Surgical options include total proctocolectomy with ileostomy, continent ileostomy, total colectomy with ileorectal anastomosis, and total proctocolectomy with ileal pouch anal anastomosis. Many diverse factors, such as extent of rectal disease, the presence and extend of carcinoma, sphincter function, and extracolonic disease, influence which surgical procedure is most appropriate for the individual patient with FAP. This article reviews the surgical options for treating FAP, with emphasis on specific indications, contraindications, and anticipated outcomes.