Encapsulating peritoneal sclerosis.
Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents (i.e. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPS-specific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is time-consuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease.
['Danford|Christopher J|CJ|', 'Lin|Steven C|SC|', 'Smith|Martin P|MP|', 'Wolf|Jacqueline L|JL|']
D005938:Glucocorticoids D006801:Humans D007415:Intestinal Obstruction D007421:Intestine, Small D010289:Parenteral Nutrition, Total D010530:Peritoneal Dialysis D056627:Peritoneal Fibrosis D010537:Peritoneum D010538:Peritonitis D012008:Recurrence D012598:Sclerosis D013629:Tamoxifen D014057:Tomography, X-Ray Computed D016896:Treatment Outcome
Peritoneal sclerosis Peritoneal dialysis Corticosteroids Tamoxifen Peritonectomy Enterolysis Abdominal cocoon Sclerosing encapsulating peritonitis