title: Long-term outcome of familial adenomatous polyposis patients after restorative coloproctectomy.
abstract: BACKGROUND
  Restorative proctocolectomy (RPC) eliminates the risk of colorectal adenocarcinoma in familial adenomatous polyposis (FAP) patients, but desmoid tumors, duodenal, and ileal adenomas can still develop. Our aim was to assess the long-term outcome of FAP patients after RPC.
  
  
  METHODS
  FAP patients who had RPC between 1983 and 1990 were contacted for interview and upper gastrointestinal (GI) and ileal pouch endoscopy.
  
  
  RESULTS
  Sixty-two males and 48 females had undergone hand-sewn RPC during this period. One patient died postoperatively (0.9%). Among 96 patients available for a minimal follow-up of 11 years, 7 patients died: 3 from causes unrelated to FAP, 2 from metastatic colorectal cancer, and 2 from mesenteric desmoid tumor (MDT). Thirteen patients had a symptomatic MDT (13.5%). Of 73 patients who had an upper GI endoscopy, 52 developed duodenal and/or ampullary adenomas. Four patients required surgical treatment of their duodenal lesions. Among 54 patients who underwent ileal pouch endoscopy, pouch adenomas were noted in 29. No invasive duodenal or ileal pouch carcinoma were detected. Functional results of RPC were significantly worse in MDT patients.
  
  
  CONCLUSIONS
  RPC eliminates the risk of colorectal cancer, and close upper GI surveillance may help prevent duodenal malignancy. MDTs are the principal cause of death, once colorectal cancer has been prevented, and the main reason for worsening functional results.
authors.affiliation: Department of Digestive Surgery, Hôpital Saint-Antoine AP-HP, University Pierre et Marie Curie, Paris, France. yann.parc@sat.ap-hop-paris.fr
authors.forename: Yann
authors.initials: Y
authors.lastname: Parc
authors.forename: Arnaud
authors.initials: A
authors.lastname: Piquard
authors.forename: Roger R
authors.initials: RR
authors.lastname: Dozois
authors.forename: Rolland
authors.initials: R
authors.lastname: Parc
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Tiret
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D018222:Desmoid Tumors / Q000453:epidemiology; D005767:Gastrointestinal Diseases / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000453:epidemiology; D016737:Proctocolectomy, Restorative / Q000009:adverse effects; D013997:Time Factors; D017211:Treatment Failure