Encapsulating Peritoneal Sclerosis

Abstract

Encapsulating peritoneal sclerosis (EPS) is an uncommon but extremely serious complication of long-term peritoneal dialysis (PD). It is diagnosed by a combination of clinical symptoms including bowel obstruction and radiological findings of features of encapsulating peritoneal fibrosis. The leading causes of death are mainly complications related to bowel obstruction. There are two major risk factors: peritoneal deterioration due to long-term PD and inflammation due to severe or recurrent peritonitis. Although many patients experience clinical symptoms following ultrafiltration (UF) failure, differentiating peritoneal sclerosis from EPS on the basis of UF failure alone is difficult from a pathological perspective.

A computed tomography (CT) scan is recommended as the first-line modality in diagnosis due to its reproducibility. Treatment with medications such as corticosteroids, immunosuppressants, or tamoxifen has been reported, but there is yet little evidence in its support. In the advanced stages, surgical intervention is the treatment of last resort, to which experienced surgeons may have recourse. The mortality rate among adults with EPS is high. For pediatric EPS, the mortality rate is lower than that for adults, but the reasons remain unclear.