Localized Encapsulating Peritoneal Sclerosis Constricting the Terminal Ileum—an Unusual Appearance Requiring Surgical Intervention

Abstract

♦ Background: Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD). It is characterized by encapsulation of the bowel, causing symptoms of intestinal obstruction. Exclusive involvement of parts of the bowel may occur and may be more common than previously thought. Our main objective was to investigate and report on patients with localized EPS. ♦ Methods: Between July 2002 and December 2011, 9 of 17 EPS patients were referred to our department of surgery for a diagnostic laparotomy. Three of the 9 cases showed localized encapsulation of the small bowel and were selected for the purpose of this study. ♦ Results: All 3 patients presented with an acute inflammatory state and symptoms of bowel obstruction. In 2 patients, EPS became clinically overt after kidney transplantation; the third patient was diagnosed while on hemodialysis. All shared a history of PD ranging from 31 to 101 months. In none of the patients was radiologic examination conclusive, although 2 showed peritoneal thickening and ascites. Each patient underwent laparotomy, confirming EPS. In all cases, a thickened peritoneal membrane became apparent, predominantly covering the ileocecal region of the intestine. In addition, a constrictive membrane at the level of the terminal ileum was noted. In 2 cases, the patients underwent enterolysis and dissection of the constricting fibrotic peritoneal membrane (peritonectomy) without bowel resection. The 3rd patient was managed with parenteral nutrition and tamoxifen. The postoperative course in 1 patient was complicated by infected ascites that resolved with antibiotic treatment. Eventually, all patients were doing well, with adequate oral intake and without the need for repeat surgery. ♦ Conclusions: Localized EPS may be more common than previously thought. It has a predilection for the level of the terminal ileum. We believe that an elective diagnostic laparotomy should be considered early, because this procedure offers both diagnostic opportunities and therapeutic options. Localized EPS cases may benefit most from enterolysis and peritonectomy.

Case report: acute bowel obstruction with an isolated transition point in peritoneal dialysis patients; a presentation of encapsulating peritoneal sclerosis?

“…Similarly Habib et al reported 3 cases of “localized” EPS, where intra-operatively, an obvious sclerotic and thickened peritoneal membrane was observed, predominantly covering and constricting the terminal ileum [ 15 ].…”

Classification of Encapsulating Peritoneal Sclerosis is Important, but Must Encapsulate the Entire Spectrum of the Disease

“…Latus et al (6) propose categorizing EPS based on three different physical appearances; however, those appearances do not cover the full range of presentations, others of which are described by Habib et al (7). The appearance of the peritoneal cavity in EPS that is encountered at surgery can be described in terms of each of the following elements: The large bowel may be affected by sclerosis and tanning, but is rarely obstructed.…”

“…In this edition of Peritoneal Dialysis International , two papers describe the varying manifestations of EPS at the time of surgery (6,7). One describes a localized form of the disease; the other suggests classifying EPS into three patterns based on its appearance, but interestingly does not distinguish between localized and generalized disease.…”

Post-transplantation encapsulating peritoneal sclerosis without inflammation or radiological abnormalities

“…Additionally, the CT scan showed a small amount of localized ascites in the abdomen but no other diagnostic signs of EPS like bowel tethering, calcifications, or peritoneal thickening. Despite the absence of typical findings, but the severity of the progressive symptoms we considered the possibility of localized EPS, a condition in which the peritoneum shows encapsulating sclerosis predominantly at the level of the terminal ileum [ 13 , 14 ]. Surgical exploration was considered as a final diagnostic procedure.…”