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Bartosz Wnęk, Marcin Strugała, Aleksandra Łożyńska-Nelke, Robert Burdyński and Piotr Krokowicz

Abstract

Familial adenomatous polyposis (FAP) is a colorectal cancer syndrome caused by a germline mutation inherited in an autosomal-dominant pattern with a 100% penetrance.

Our detailed case report presents a history of a 55-year-old FAP female patient who had been under constant clinical observation for 30 years. The disease was diagnosed at the age of 22. The patient underwent restorative proctocolectomy with ileal pouch-anal anastomosis (PRC-IPAA). During our follow-up extra-colonic manifestations occurred such as a desmoidtumour, fundic gland polyps in the stomach and duodenal polyps also in the periampullary region. Apart from disease-related symptoms the patient manifested other complications such as small bowel adhesive obstruction, benign breast tumours, uterine myomas, cholelithiasisand thyroid nodules.

Our analysis of the above case presents advantages of a long-term medical observation of a FAP patient carried out by a specialist surgical medical centre

Open access

Robert Burdyński, Tomasz Banasiewicz, Ryszard Marciniak, Maciej Biczysko, Jacek Szmeja, Jacek Paszkowski, Marcin Grochowalski, Jakub Maik, Przemysław Majewski, Piotr Krokowicz and Michał Drews

Intestinal Pouch Complications in Patients Who Underwent Restorative Proctocolectomy for Ulcerative Colitis and Familial Adenomatous Polyposis in 1985-2008

Restorative proctocolectomy is considered a surgical treatment of choice in ulcerative colitis (UC) and familial adenomatous polyposis (FAP).

The aim of the study was to evaluate postoperative complications in patients who underwent surgery for familial adenomatous polyposis and ulcerative colitis, on the basis of a retrospective data analysis.

Material and methods. Data of 138 patients after restorative proctocolectomy performed between 1985 and 2008 were collected at routine follow-up visits in 2004-2008. We evaluated the presence of pouchitis, the degree of ileal pouch mucosa atrophy, the presence of ileal pouch mucosal metaplasia, the presence of ileal pouch malignancies, the necessity for diverting ileostomy, the necessity for pouch resection, and severe faecal incontinence.

Results. Complications were observed in 45 (32.4%) patients. Thirty-seven patients developed pouchitis (26.6%). Low-degree dysplasia, severe dysplasia or malignancies were observed in total in 20 patients (14.4%). Six (4.3%) operated patients developed other analysed complications.

Conclusions. The most common complications of restorative proctocolectomy were dysplasia and pouchitis. The most common complication in patients operated for UC was pouchitis. The low observed incidence of intestinal pouchitis may be attributed to the implemented prophylaxis of inflammation. Dysplasia was the most common complication in patients undergoing proctocolectomy for FAP. Due to an increased risk of dysplastic lesions as compared with UC patients, careful endoscopic follow-up examinations are obligatory in this patient group. Other analysed complications were uncommon and were mostly a consequence of chronic pouchitis. Clinical symptoms of pouch-related problems were similar in both analysed groups.