Abstract
Familial adenomatous polyposis (FAP) is an autosomal dominant disorder affecting patients
with germline mutations of the adenomatous polyposis coli (APC) tumor suppressor gene.
The surgical treatment of colorectal disease in FAP, which has the goal of colorectal
cancer prevention, varies based on both patient and disease factors but can include
the following: total colectomy with ileorectal anastomosis, proctocolectomy with stapled
or hand-sewn ileal pouch-anal anastomosis, or total proctocolectomy with end ileostomy.
The operative options and extent of resection, as well as the use of endoscopy and
chemoprevention for the management of polyposis, will be discussed in detail in this
article. In addition, commonly debated management decisions related to the treatment
of patients with FAP, including the timing of prophylactic colorectal resections for
patients with FAP and management of the polyp burden in the rectum, will be discussed.
Finally, genotype considerations and the impact of desmoid disease on operative decisions
in the setting of FAP will also be reviewed.
Keywords
familial adenomatous polyposis - proctocolectomy - adenomatous polyposis coli - desmoid
