Surgery for Familial Adenomatous Polyposis

Abstract

Patients with familial adenomatous polyposis are at high risk of developing colorectal cancer. Successful prevention of cancer depends on timely and appropriate surgery. Decisions regarding the timing and choice of surgery are driven primarily by the severity of the polyposis and the social situation of the patient. Immediate surgery is recommended for patients presenting with cancer, while symptomatic patients and those with profuse polyposis should be operated without delay. Asymptomatic patients and those with mild or attenuated polyposis may be operated electively, and surgery can be delayed for years as long as regular colonoscopy shows no increase in cancer risk. A secondary aim of prophylactic colectomy in familial adenomatous polyposis is preservation of bowel function and quality of life. To this end, patients with mild polyposis are recommended to have a colectomy and ileorectal anastomosis, often performed with minimally invasive technique. Careful selection results in a low incidence of rectal cancer and proctectomy. Severely affected patients have proctocolectomy and ileal pouch-anal anastomosis, and most have acceptable functional outcomes. Lifetime surveillance of residual gastrointestinal tract is mandatory for all patients. Desmoid disease occurs in 30% of familial adenomatous polyposis patients, and is the second most common cause of death. Patients at high risk of desmoid disease should have the surgical strategy re-evaluated with a view to minimizing the incidence and impact of desmoids.