Long-term outcome of familial adenomatous polyposis patients after restorative coloproctectomy.
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.
Parc
Yann
Y
Department of Digestive Surgery, Hôpital Saint-Antoine AP-HP, University Pierre et Marie Curie, Paris, France. yann.parc@sat.ap-hop-paris.fr
Piquard
Arnaud
A
Dozois
Roger R
RR
Parc
Rolland
R
Tiret
Emmanuel
E
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