Primary Sclerosing Encapsulating Peritonitis Presenting asRecurrent Intestinal Obstruction.

Abstract

A 21-year-old woman was admitted to our hospital with complaints of abdominal pain and vomiting for 10 days. The pain was colicky in nature, and was located on the left-hand side of the center of the abdomen. She had multiple episodes of similar pain during the past 1 year that had resolved spontaneously. Her abdominal examination showed a vague mobile lump in the left side of her abdomen. All of her laboratory blood test results were normal. An abdomen contrast-enhanced computed tomography scan showed clumping of the small-bowel loops with a membrane surrounding it (Figure A). The duodenum was dilated and colonic loops were collapsed (Figure B). Laparotomy showed a thick fibrotic peritoneal wrapping of the small bowel, suggestive of an abdominal cocoon (Figure C). Excision of the fibrous capsule and release of adhesions between the loops were performed. The patient had an uneventful postoperative period, and she remained well during the early follow-up evaluation. Pathologic examination of the membrane showed a dense fibrocollagenous tissue with no neoplastic or granulomatous change (Figure D). Based on clinical, radiologic, surgical, and histopathologic findings a diagnosis of primary sclerosing encapsulating peritonitis (PSEP) was made. PSEP is an extremely rare idiopathic condition presenting as an intestinal obstruction. It typically is characterized by total or partial encasement of the small bowel by a thick fibrotic membrane.1Xu P. Chen L.H. Li Y.M. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon): a report of 5 cases.World J Gastroenterol. 2007; 13: 3649-3651Google Scholar PSEP classically presents in young adolescent girls in tropical and subtropical countries such as China, India, Turkey, Nigeria, Malaysia, Singapore, Pakistan, Kenya, and Saudi Arabia, although cases in temperate zones also have been reported.2Akbulut S. Accurate definition and management of idiopathic sclerosing encapsulating peritonitis.World J Gastroenterol. 2015; 21: 675-687Google Scholar The cause of PSEP still is unknown. The more common secondary form of sclerosing encapsulating peritonitis has been reported in association with peritoneal dialysis, prior abdominal surgery, abdominal tuberculosis, drug (practolol) use, peritoneovenous shunting, intraperitoneal chemotherapy, gastrointestinal malignancy, and endometriosis.3Trigka K. Dousdampanis P. Chu M. et al.Encapsulating peritoneal sclerosis: a single-center experience and review of the literature.Int Urol Nephrol. 2011; 43 (539): 519-526Google Scholar PSEP usually presents with recurrent abdominal pain, nausea, vomiting, weight loss, and recurrent episodes of partial or complete small-bowel obstruction.2Akbulut S. Accurate definition and management of idiopathic sclerosing encapsulating peritonitis.World J Gastroenterol. 2015; 21: 675-687Google Scholar Although many cases are diagnosed incidentally at surgery, a better awareness of this condition and radiologic imaging may assist in a preoperative diagnosis. The computed tomography scan of the abdomen usually shows congregation of small-bowel loops to the center of the abdomen encased by a thick membrane.4Krestin G.P. Kacl G. Hauser M. et al.Imaging diagnosis of sclerosing peritonitis and relation of radiologic signs to the extent of the disease.Abdom Imaging. 1995; 20: 414-420Google Scholar A close differential diagnosis of this condition is an internal hernia, which, however, does not show a membrane-like sac. Surgical treatment is required in most cases. A simple surgical release of the encased bowel via removal of the fibrotic capsule is all that is required. In general, prognosis after surgery is good; however, there is some risk of recurrence (∼20%) and the need for repeat surgery on follow-up evaluation.1Xu P. Chen L.H. Li Y.M. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon): a report of 5 cases.World J Gastroenterol. 2007; 13: 3649-3651Google Scholar Although corticosteroids, tamoxifen, and immunosuppressive agents have been used in patients with secondary SEP, with the aim of reducing inflammation and peritoneal fibrosis, data on their use in patients with PSEP are limited.