Abdominal cocoon: An enigmatic entity

Abstract

Abdominal cocoon, variously described as encapsulating peritoneal sclerosis (EPS) or sclerosing encapsulating peritonitis or peritonitis chronica fibrosa incapsulata, represents a syndrome of clinical symptoms related to formation of a fibro-collagenous peritoneal membrane that involves the small intestinal loops. It is the outcome of several different etiologic processes. The major factors associated with development of an abdominal cocoon include continuous ambulatory peritoneal dialysis, tuberculosis, post-renal or liver transplant, use of beta-blockers although a proportion are of idiopathic origin. It is believed that the pathogenesis is driven by epithelial-to-mesenchymal transition of the mesothelial cells. The clinical presentation is related to development of altered gut motility resulting in abdominal pain and features of intestinal obstruction. Surgery is usually required in cases of unremitting intestinal obstruction. Although computed tomography may provide a pre-operative diagnosis, many cases are diagnosed at the time of surgery. Role of conservative therapy using drugs like steroids, tamoxifen, and mechanistic target of rapamycin (mTOR) inhibitors, or antitubercular therapy (in patients with tuberculosis) is uncertain. Early management aimed at the underlying etiological factor may ameliorate the symptoms and halt progression to formation of frank cocoon. Occasionally, conditions like peritoneal carcinomatosis or peritoneal encapsulation may mimic EPS.Â