Encapsulating peritoneal sclerosis: The abdominal cocoon

Rizna Abdul CADER, Rozita MOHD, Syahrizan SAMSUDDIN, Halim GAFOR, Ema JULIATY, Norella CT KONG 

Nephrology Unit, Department of Internal Medicine, Universiti Kebangsaan Malaysia Medical Centre, Malaysia

ABSTRACT

Encapsulating Peritoneal Sclerosis (EPS) is a rare but serious complication of long-term peritoneal dialysis (PD) that is associated with a high morbidity and mortality. EPS has been previously referred to as sclerosing peritonitis, sclerosing encapsulating peritonitis or the ‘abdominal cocoon’. EPS is characterised by diffuse thickening and sclerosis of the peritoneal membrane which leads to a decrease in ultrafiltration, ascites and ultimately partial or complete small bowel obstruction. The aetiology of EPS is unknown but it has been reported to be closely associated with the duration on PD, recurrent peritonitis, high transporter status and use of high concentration of glucose dialysis solutions. Stopping or switching from PD seems to be another trigger factor. Several treatment options including oestrogen and immunosuppressive therapy have been tried, but the mortality remains high at approximately 50%. In some cases, surgery has been attempted but this is associated with high morbidity and mortality. Supportive therapy includes nutritional support with total parenteral nutrition. EPS needs to be diagnosed early and one has to have a high index of suspicion. We report three cases of EPS in patients on PD with different presentation of EPS, their treatment and a brief literature review.  

Keywords: Encapsulating peritoneal sclerosis, high transporters, membrane failure, peritoneal dialysis, sclerosing peritonitis

Correspondence author: Rizna ABDUL CADER, Nephrology Unit, Department of Internal Medicine, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, Cheras, Kuala Lumpur 56000, Malaysia.

Tel: +603 91456097, E mail: Rizna_c@hotmail.com

Brunei Int Med J. 2013; 9 (1): 56-62