Management

Up to now, surgery is the only effective therapy for removing all the polyps and preventing the inevitable progression to cancer. The general recommendation is that prophylactic colectomy is advisable by the late teens in patients with a sure diagnosis of FAP [5]. There are currently three surgical options including: (1) restorative proctocolectomy with ileal pouch anal anastomosis (RPC/IPAA) with mucosectomy, (2)

Table 1. FAP: associated benign and malign lesions (modified from [5])

Neoplastic lesions Non-neoplastic lesions

Duodenal polyps/tumour (5-11%) Osteomas

Pancreatic tumour (2%) Desmoid tumours

Thyroid cancer (2%) Duodenal, jejunal, ileal polyps

Brain, medulloblastoma (< 1%) Gastric adenomas

Hepatoblastoma (0.7% of children <5 years) Ocular fundic lesions

Epidermoid cysts Radiopaque jaw lesions

RPC/IPAA using the double staple technique and (3) colectomy with ileo-rectal anastomosis (IRA). A fourth option, a proctocolectomy with permanent ileostomy, can only be considered in the few cases in which an RPC/IPAA or an IRA is contraindicated.

An RPC/IPAA with mucosectomy has the advantage of removing the entire colon, that means all the polyps, and taking away the anal mucosa with minimal remaining risk of developing colorectal cancer. In spite of the obvious technical problems, restorative proctocolectomy with IPAA and mucosectomy has been the preferred approach at the Mayo Clinic with a low reported post-operative complication rate and satisfactory functional results [23, 24].

As a critical comment on these results, some authors argue that islands of rectal mucosa might be retained even with mucosectomy, in other words the risk of developing new polyps is reduced, but not eliminated [25, 26]. The alterative double-stapling technique performed at the Cleveland Clinic offers a better functional outcome, but also presents a 28% increase in the development of adenomas in the transitional zone [27].

A total colectomy with ileo-rectal anastomosis could offer better compliance, but needs a very careful surveillance program. It is usually offered to young patients with partial rectal sparing and less than 20 rectal polyps. As in other critical patient populations, like the IBD patients, surgical management should be tailored to the different patients.

A review at Mount Sinai Medical Center documented the rise of rectal cancer at a mean follow-up of 13 years in 25% of patients with ileo-rectal anastomosis. They concluded that proctocolectomy with ileo-anal pouch should be the preferred surgical option, which is in agreement with other authors [28, 29].

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