Encapsulating peritoneal sclerosis

Abstract

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.

Keywords: Abdominal cocoon, Sclerosing encapsulating peritonitis, Peritoneal sclerosis, Peritonectomy, Enterolysis, Peritoneal dialysis, Tamoxifen, Corticosteroids

Core tip: Encapsulating peritoneal sclerosis (EPS) is a rare, but potentially devastating disorder. Most literature is derived from the nephrology literature surrounding peritoneal dialysis, however, the gastroenterologist is likely to encounter EPS from a variety of etiologies. We present a comprehensive review of EPS from all etiologies and a summary of treatments from a gastroenterologist’s perspective including the role of nutrition, surgery, immunosuppression, anti-fibrotic agents, and the novel use of μ-opioid antagonists and guanylate cyclase C agonists in the management of patients with EPS.