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• W2149059248 abstract "SUMMARY Purpose: The aim of this study was to present our experience in the treatment of patients with familial adenomatous polyposis (FAP) and their families. Materials and methods: The material comprises 36 patients with FAP who had undergone prophylactic colorectal surgery, including those operated on because of colorectal cancer (CRC). Anal continence preserving surgery was performed on 34 patients: 30 had ileal-pouch anal anastomosis as primary surgery, 4 had ileal-pouch anal anastomosis as secondary operation after ileorectal anastomosis. Total proctocolectomy was performed on 2 patients with FAP and rectal cancer. Two patients with desmoid tumours were detected. Surgical outcome was assessed on the basis of hospital records. A questionnaire was used to evaluate the postoperative functional outcome. Finally, most family members had blood samples taken for detection of mutation of the APC gene. Results: The histology of the specimen retrieved from these patients showed in four a malignant tumour which had not been suspected preoperatively: two rectal adenocarcinomas (one in the ileorectal group), and two carcinomas in situ. One of these patients accepted the option for pouch excision and permanent ileostomy but the other refused. Surgical outcome was very good, without any major early or late postoperative complications. Fuctional results after ilealpouch anal anastomosis are satisfactory. All pouches are in place and functional. Conclusions: Preventive surgery is indicated in patients with FAP. Total colectomy, anal mucosectomy and ileal-pouch anal anastomosis, when possible, is preferred over ileorectal anastomosis or total colectomy with permanent ileostomy. The coexixtence of cancer, the age of the patient, the development of desmoid tumours or extracolonic neoplastic tumours are factors that influence the choice of the operation and the outcome." @default.
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• W2149059248 date "2003-01-01" @default.
• W2149059248 modified "2023-09-24" @default.
• W2149059248 title "Total Colectomy with Mucosectomy and Ileal Pouch-Anal Anastomosis in Patients with Familial Adenomatous Polyposis" @default.
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