Three Cases of Familial Adenomatous Polyposis

Kim, Jin Ah; Seo, Geom Seog; You, Keyoung Hoon; Hwang, Ho Geun

Abstract

Familial polyposis is a rare genetic disorder which the large intestine is diffusely carpeted by numerous adenomatous polyps. According to the recent studies, familial polyposis is a disease which produces polypoid lesions not only in the large intestine but also in the stomach, duodenum, ileum and jejunum. The natural course of extracolonic lesions is variable, but the colonic polyps are associated with high incidence of cancer. The risk of malignant change is virtually 100% if untreated. Thus when adenomatosis is noted, familial members at risk for familial adenomatous polyposis must be screened and prophylactic surgery performed to prevent inevitable colon cancer. Controversy exists about the most appropriate prophylactic treatment. Because of many disadvantages of ileostomy, total colectomy with ileorectal anastomosis (ileoproctostomy) or total colectomy with rectal mucosa stripping and ileoanal anastomosis has become a more preferred approach. After ileorectal anastomosis, polyps in the retained rectum must be removed by endoscopic polypectomy and rigorously followed by sigmoidoscopy every few months for prevention of polyp reccurence or malignant transformation. We experienced three cases of familial adenomatous polyposis and 2 of them were treated successfully by preoperative colonoscopic polypectomy, total colectomy and ileoproctostomy.