Once colorectal polyps are found, the only effective management is total proctocolectomy in FAP. Because colorectal cancer is diagnosed in 5% of FAP patients by 20 years of age, surgery should be recommended at the time of diagnosis. Surgical options for colonic disease include total colectomy with ileorectal anastomosis (IRA), proctocolectomy with ileal pouch-anal anastomosis (IPAA), total proctocolectomy with Brook ileostomy, and total proctocolectomy and continent ileostomy (Koch). It should be noted that for patients with AFAP, IRA is an appropriate therapy.
Duodenal and periampullary disease poses a much more challenging therapeutic dilemma. Endoscopic ablation via snare, argon plasma coagulation therapy, and laser photodynamic therapy are being attempted for mild to moderate disease; however, they also carry significant opportunity for morbidity and are often difficult to tolerate. As only a small proportion of these patients develop carcinoma, it becomes difficult to justify prophylactic resection. This decision is further complicated by the associated significant morbidity of pancreaticoduodenec-tomy with questionable improvement on prognosis. Consequently, these options are reserved for diagnosed cancer or severe dysplasia.
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