대한대장항문학회

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