Subtotal colectomy for familial polyposis. A clinical series and review of the literature.

Familial polyposis is an inherited syndrome in which untreated persons have virtually a 100% incidence of developing colon cancer. Much controversy exists over whether subtotal colectomy with ileoproctostomy is the appropriate procedure in these patients owing to the risk of subsequent cancer in the retained portion of the rectum. At Duke University Medical Center, Durham, NC, a group of 25 patients chose to undergo the subtotal colectomy and ileoproctostomy instead of the definitive total proctocolectomy. Of the 25 patients in this series, invasive adenocarcinoma has developed in the rectal segment in only 1 patient. This patient, the oldest in our series, had carcinoma in situ in her initial operative specimen and has done well following an abdominal perineal resection and 13 years of follow-up. Six other patients have subsequently undergone definitive resections of the rectum because of intractable benign polyps. These results compare favorably with those reported in the literature. We conclude that subtotal colectomy with ileoproctostomy is still a useful and successful mode of treatment for select patients with familial polyposis if they are followed up frequently and aggressively and if the surgeon maintains a low threshold for recommending completion proctectomy.