[Familial adenomatous polyposis: long term follow-up of patients after surgical treatment. Prognostic factors].
The purposes of this study are: 1) to evaluate if recent progresses (knowledge of natural history, genetic diagnosis and surgical treatment) have an impact upon the long term follow up of familial adenomatous polyposis (FAP); 2) to assess the prognosis factors that are relative to recent progresses in diagnosis and treatment procedures. A retrospective study of 51 cases was carried out in July 2002 to analyse the following characteristics: phenotype, treatment, operative mortality and morbidity, late complications, especially rectal stump cancer after ileo-rectal anastomosis, duodenal adenomatosis and desmoid tumors. Twenty seven men and 24 women underwent surgery: 11 colo-rectal cancers were present at first step. Initial surgical procedures included 39 total colectomies with ileo-rectal anastomosis (IRA), 6 coloproctectomies with ileo-anal anastomosis (IAA) and 6 coloproctectomies with permanent ileostomy. Operative mortality was nil. Operative morbidity affected 11 patients. The rectum had to be secondary removed in 11 patients with convert in IAA. Duodenal adenomatosis required surgery in 10 patients: 5 surgical local excisions, 4 duodenopancreatectomies and 1 palliative by-pass. Six desmoid tumors were noted during the follow-up. On the whole 3 patients were lost of sight. Nine patients died (19.1%), 4 deaths were in relation with the disease: 1 rectal cancer, 2 duodenal cancers, 1 desmoid tumor necrosis. At the end of the follow up (mean duration: 17 years) 26 IRA and 17 IAA are present with good functional results. This study, according to already published data, suggests that today the risk of death related to colorectal cancer is becoming lower than the risk of death from duodenal cancer and desmoid tumor evolution, particularly since the introduction of the restorative proctocolectomy. The genetic diagnosis is useful in order to determine the choice of surgical procedures.
Malafosse
Michel
M
Hôpitaux de Paris. mmalaf@club-internet.fr
D011125:Adenomatous Polyposis Coli / Q000235:genetics / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D000368:Aged; D003082:Colectomy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007081:Ileostomy; D008297:Male; D008875:Middle Aged; D016737:Proctocolectomy, Restorative; D011379:Prognosis; D012189:Retrospective Studies; D013997:Time Factors