The plication and splinting procedure for idiopathic sclerosing encapsulating peritonitis

Idiopathic sclerosing encapsulating peritonitis (ISEP) is a rare cause of small intestinal obstruction. Histologically, ISEP is characterized by a thick fibrotic membrane encasing the small bowel without any apparent pathophysiological factors. While ISEP is predominantly present congenitally in female adolescents from subtropical region, it has been identified throughout the world. Evidence-based effective diagnostics and treatments are pitifully thin. We experienced six cases, four males and two females, which exhibited symptoms in their later thirties and forties. Five patients presented with acute and subacute intestinal obstruction, and one patient with cryptorchidism and seminoma was referred. Due to the limitation in distention and motility of bowel loops of ISEP, imaging exams may not be very reliable for accurate diagnosis and estimation of obstruction progress. They were successfully treated with the mesenteric plication and intraluminal splinting procedures. Two cases had an uneventful postoperative period, and the returns of normal bowel function were delayed in the other four patients. Two recurrences of small bowel obstructions were noted over a mean follow-up period of 33 months with mild symptoms. This article reviews the patterns of clinical presentations, diagnostic clues, and theories of potential risk factors of ISEP as well as its controversial surgical managements.