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Given the 100% risk of developing colorectal cancer, prophylactic surgery is the standard of care in familial adenomatous polyposis (FAP) when it is diagnosed in the asymptomatic stage. The optimal procedure is total proctocolectomy with conventional ileostomy or ileal pouch-anal anastomosis. The ileal pouch needs to be monitored for the development of adenomas.

Some tertiary care centers specializing in the management of FAP patients advocate less radical, rectum-sparing procedures such as subtotal colectomy with ileo-rectal anastomosis. The rectum remains at risk for carcinoma and these patients have to comply with rigorous surveillance protocols. About 25% of these patients end up needing total proctectomy subsequently due to the development of rectal cancers. They also need to be monitored for adenomas elsewhere in the GI tract such as ampulla of Vater and stomach.

The image show a small microscopic adenoma consisting of several dysplastic crypts from a colon with FAP.

Image 72 of 113