Abstract

INTRODUCTION

Familial adenomatous polyposis (FAP) is treated by abdominal colectomy with ileorectal anastomosis (IRA), restorative proctocolectomy with ileal J-pouch anal anastomosis (IPAA), and total proctocolectomy with ileostomy. This study investigated question is what type of surgery is better.

METHODS

Twenty FAP patients, who underwent surgery in a single hospital between 2004 and 2014, were included. Patients were classified in 3 groups according to the operation procedures. Demographic, surgical, pathological, and outcome data were analyzed from hospital records retrospectively.

RESULTS

Overall 20 patients were included, 15 index patients, and 5 relatives diagnosed by screening. Median age of patients with or without colorectal cancer was 37 and 20 years respectively. Four patients were treated by IPAA, 4 by IRA, and 12 by proctocolectomy. All patients presented with 100 or more colonic polyps. The median follow-up time was 38 months. During the follow-up period, 8 (40.0%) patients developed recurrent or metastatic disease. There were; distant metastasis in 4 patients, rectal cancer in three (who underwent IRA), desmoid tumor in one. Adenoma developed in one rectal remnant, ileal pouch adenoma or adenoma in ileostomy was not seen in any patient. Four patients died 3 of them because of metastatic disease and the other one died because of a huge desmoid that caused renal failure.

DISCUSSION AND CONCLUSION

Abdominal colectomy with IRA was not a good option for classic FAP patients who presented with colorectal cancer preoperatively, but it should be noticed that this study consists of small number of patients.