Encapsulating Peritoneal Sclerosis: A Rare Devastating Peritoneal Disease: Case Series and Literature Review

Abstract

Background: Encapsulating peritoneal sclerosis is a rare but life-threatening condition in peritoneal dialysis. It is associated with extensive thickening and fibrosis of the peritoneum resulting in recurrent small bowel obstructions and malnutrition. Herein we present case series of encapsulating peritoneal sclerosis diagnosed and treated in a large center of peritoneal dialysis in the State of Qatar followed by literature review to increase the awareness of physicians to it. Case Presentation: The data were collected retrospectively from 180 peritoneal dialysis patients at Hamad General Hospital, Doha, Qatar, between 2008 and 2016. The diagnosis of encapsulating peritoneal sclerosis was based on clinical and radiological features. Seven patients (3.8%) were diagnosed with encapsulating peritoneal sclerosis. The mean age of the patients was 49 years (range, 23 to 65 years). The mean duration of peritoneal dialysis was 6 years (range, 3 to 7 years). The mean peritonitis episodes were 1.8 (range 1 to 4 episodes). Five patients were diagnosed with encapsulating peritoneal sclerosis after stopping peritoneal dialysis, and two patients diagnosed while they were on peritoneal dialysis treatment. Five patients are still alive and were treated with oral prednisolone and tamoxifen. Two patients underwent adhesiolysis surgery, one of them died due to recurrent sepsis and malnutrition. Another patient died because of cardiac arrest during hemodialysis. The overall mortality rate was 28.5% in our series. Conclusions: Encapsulating peritoneal sclerosis is a rare and life-threatening peritoneal disease in chronic peritoneal dialysis patients. It requires high index of suspicion for early diagnosis and treatment.