Abstract

Familial adenomatous polyposis (FAP) and hereditary nonpolyposis colorectal cancer (HNPCC) are the two most common forms of hereditary colorectal cancer (CRCA), representing approximately 5% of all CRCA. Endoscopic surveillance of at-risk colorectal mucosa is one of the key issues in the management of each of these diseases. In patients with FAP who are treated with total abdominal colectomy and ileorectal anastomosis, the risk of a metachronous rectal cancer is 4% to 10% at 10 years and increases with the duration of follow-up. After proctocolectomy with ileal pouch anal anastomosis, both the ileal pouch and the anal transition zone are at risk for adenomas and invasive adenocarcinoma. Patients with FAP need yearly proctoscopy or pouchoscopy, performed with a flexible endoscope. Increasing adenoma numbers, sizes, and dysplasia are indications to consider proctectomy or chemoprevention (in patients with a pouch). In patients with HNPCC who undergo segmental or subtotal colectomy, the risk for a second cancer developing in the remaining colon or rectum is in the range of 4% to 15%. These patients need yearly colonoscopy or proctoscopy (in patients with an IRA). In this chapter, details of technique and the management of metachronous neoplasia are discussed.

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