Encapsulating Peritoneal Sclerosis – A 5 Year Experience

TITLE: Encapsulating peritoneal sclerosis – A 5 year experience AIM: Encapsulating peritoneal sclerosis (EPS) is a rare, life-threatening condition, characterised by a progressive, intra-abdominal inflammatory process resulting in fibrotic visceral constriction. We report the aetiology, management, and outcome of EPS in Belfast. METHOD: All patients diagnosed with EPS in Belfast over the past 5 years are included. Presentation, aetiology, imaging, pathology, and outcome are retrospectively analysed and reported. RESULTS: 7 patients (4 males) were identified with EPS with a mean age 54 years (range 33–69). Aetiology included peritoneal dialysis (3), radiation enteritis (1), peritoneal dialysis and radiation enteritis (1), tuberculosis, cirrhosis, and beta-blocker use (1), infected aorto-bifemoral graft (1). Of the 7 patients, 5 underwent definitive surgery. Bowel conserving surgery (laparotomy, division of adhesions, excision of membrane) was performed in 4 patients. One patient required an ileocaecal resection for radiation enteritis. Median pre-operative and post-operative hospital stay were 25 and 62 days respectively. Three patients required total parenteral nutrition (TPN) pre-operatively, 3 patients post-operatively; with 4 of the 7 patients discharged on TPN. 5 out of 7 patients are alive at median follow-up of 24 months. There was no 30-day in-hospital mortality. CONCLUSIONS: Patients with EPS often require parenteral nutrition before and after surgery. Peritoneal dialysis is a major risk factor for the development of EPS but other aetiologies should be considered. These patients have multiple co-morbidities, and operations for EPS are challenging with a high risk of peri-operative complications. Therefore these patients are best managed in a specialised unit with experience in intestinal failure surgery and access to a multi-disciplinary nutrition support team.