Total Colectomy with Mucosectomy and Ileal Pouch-Anal Anastomosis in Patients with Familial Adenomatous Polyposis

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.
Key words: Familial adenomatous polyposis; ileal pouch-anal
anastomosis;adenomas; colorectal cancer; desmoid tumours