Tag Archives: Keywords: Familial adenomatous polyposis

Purpose The main operative method in familial adenomatous polyposis (FAP) patients

Purpose The main operative method in familial adenomatous polyposis (FAP) patients is restorative proctocolectomy with J-shaped pouch and temporary loop ileostomy. in 7% of cases, high-grade dysplasia 7?years later in around 10% of patients and neoplasia 14?years after surgery in 15% of cases. Conclusions In conclusion, the Polyposis Registry encompassing whole country is the best way of controlling FAP patients. The regular lifelong endoscopic monitoring gives the opportunity of the early detection of the dysplasia and can protect against neoplasia. Keywords: Familial adenomatous polyposis, Restorative proctocolectomy, J-pouch, Pouchitis, Dysplasia Introduction Familial adenomatous polyposis (FAP) is usually a rare genetic disorder characterized by multiple colorectal polyps undergoing malignant transformation. buy Morusin FAP incidence in European Union countries estimated by European Medicines Agency in 2009 2009 is usually three to ten new cases per 100,000 that means 11,300C37,600 FAP cases EU-wide [1]. Because of the genetic background of the disease, there is no causative treatment. Surgical treatment is usually devoted to avoid further neoplasia. The main operative method in FAP patients is usually restorative proctocolectomy with J-shaped pouch and temporary loop ileostomy. This is a very extensive and radical procedure [2], not yet fully protecting against malignant transformation in the rectal remnant and anastomosis [3]. Another widely accepted surgical procedure is usually colectomy buy Morusin with ileo-anal anastomosis performed when there are few polyps in the rectum. Some studies suggest that there should not be more than ten rectal polyps [4], others that no more than five [5]. A great advantage of this method is the preservation of the rectal innervations, subsequent better quality of life [6] and fewer problems with erection and ejaculation. In closely selected cases, especially with fewer polyps than in FAP, namely in attenuated familial adenomatous polyposis and also in patients disagreeing to surgery, regular endoscopic polypectomies are necessary. This is not a standard procedure and is not recommended as the treatment of choice in polyposes [7]. Another significant issue in patients after an ileal pouch-anal anastomosis or ileorectal anastomosis is usually a recurrence of adenomas and malignancy in the rectal remnant, anastomosis or pouch. Frequency and dynamics of pouch dysplasia is being still discussed. There is a high discrepancy between the studies reporting the incidence of dysplasia in 8C74% of patients undergoing proctocolectomy due to FAP [5, 8]. Patients and methods A group of 165 FAP patients subjected to restorative proctocolectomy in the years 1985C2009 was retrospectively analysed. A group of 86 females and 79 males with the mean age Neurod1 of 22.49 (12) was carefully selected from the total number of admitted patients to the Department of General, Gastroenterological and Endocrynological Surgery and Department of General and Colorectal Surgery, Poznan University of Medical Sciences, Poland. Follow-up investigations were done in both departments and, in some cases, in four other clinical centers, easy accessible for the patients. Clinical data coming from follow-up visits in the years 2004C2009 as well as medical documentation from these centers were also evaluated. Operative technique The first group of 13 patients was collected to the proctocolectomy with J-pouch with manual ileorectal anastomosis and mucosectomy. The remaining 152 patients underwent the same but stapled procedures: 3 of them had restorative proctocolectomy with J-pouch without temporary ileostomy, 142 had two-stage procedure with ileostomy and 7 had three-stage surgery (colectomy with end-ileostomy, J-pouch construction with temporary ileostomy and closure of ileostomy). Another 13 patients demonstrated acute buy Morusin medical procedures indications, such as mechanical bowel obstruction and bleeding and were operated immediately: nine buy Morusin of them underwent three-stage procedures and four had two-stage surgery. Endoscopic examination It is a standard since the onset of restorative proctocolectomy performed at.