title: Surgical treatment and long-term outcome of patients with familial adenomatous polyposis: 16 years experience at the Tel Aviv Sourasky Medical Center.
abstract: BACKGROUND
  Restorative proctocolectomy eliminates the risk of colorectal cancer in patients with familial adenomatous polyposis. Complications and extra-intestinal manifestations are inherent to the procedure.
  
  
  OBJECTIVE
  To evaluate operative procedures, complications, early and late results and long-term functional outcome in FAP patients operated in our department.
  
  
  METHODS
  The study group included all patients with FAP who were operated between 1988 and 2003. Demographic data, length of follow-up, complications, colorectal cancer, pouch function and extracolonic manifestations were recorded.
  
  
  RESULTS
  Median age at surgery was 33 years (range 13-61 years). The final operative breakdown was: 48 proctocolectomies, 41 ileal pouch-anal anastomoses, 2 Kock's pouch, 5 permanent ileostomies, and 2 colectomies with ileorectal anastomosis. There was no perioperative mortality. Early and late complications occurred in 20 and 9 patients, respectively. Twelve patients required reoperation. Colorectal carcinoma was diagnosed in eight patients, three of whom were in an advanced stage. The mean follow-up was 74 months (range 3-288 months). Four patients were lost to follow-up. Extracolonic manifestations developed in 38 patients, including desmoid tumors (in 12), duodenal adenomas (in 9), pouch adenomas (in 5), and rectal stump adenomas (in 3). Two patients died (4%) because of desmoid tumor and malignant fibrous histiocytoma. At last follow-up, 37 IPAA patients have (median) six bowel movements/24 hours and good fecal control.
  
  
  CONCLUSIONS
  Restorative proctocolectomy can be performed with low mortality, acceptable morbidity, and good functional results. Patients should be closely followed after surgery for development of other manifestations of the syndrome. Relatives of the affected patients should be referred to a specialist multidisciplinary clinic.
authors.affiliation: Colorectal Unit, Division of Surgery B, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel. hagitt@tasmc.health.gov.il
authors.forename: Hagit
authors.initials: H
authors.lastname: Tulchinsky
authors.forename: Andrei
authors.initials: A
authors.lastname: Keidar
authors.forename: Gideon
authors.initials: G
authors.lastname: Goldman
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Klausner
authors.forename: Micha
authors.initials: M
authors.lastname: Rabau
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000473:pathology / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D003108:Colonic Diseases / Q000209:etiology / Q000517:prevention & control; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D016737:Proctocolectomy, Restorative; D012086:Reoperation; D016896:Treatment Outcome