대한대장항문학회

Familial adenomatous polyposis(FAP) is a rare genetic disease characterized by the<br>development of 100 to several thousands colorectal adenomas of which one or more will<br>degenerate into carcinoma unless prophylactic colectomy is performed. It is inherited by<br>a Mendelian autosomal dominant fashion with a high penetrance. The principle of<br>treatment is complete removal of the diseased colon. Because of technical difficulty and<br>operative morbidity, some alternative operative procedures are proposed and used.<br>Though treatment of the already diseased patient is important, it is more important and<br>mandatory that early detection and treatment of FAP in family members are performed<br>prior to the development of colorectal cancer.<br>We experienced three cases of familial polyposis, all of which had retinal lesions, and<br>reported with a brief review of literatures.<br>