Abdominal cocoon syndrome: an obstructive adhesiolytic metamorphosis

Abstract

Abdominal cocoon syndrome (ACS), also known as idiopathic sclerosing peritonitis and primary sclerosing peritonitis, is a rare condition causing small bowel obstruction first described in 1978 by Foo et al. It is characterised by total or partial encasement of the small bowel in a fibrocollagenous cocoon-like sac accompanied by extensive intrinsic small bowel adhesions. While the aetiology of this condition remains largely unknown, ACS can be divided into two subtypes: primary or idiopathic, which is often accompanied by cryptorchidism, and secondary to another cause such as congenital dysplasia or medications. Definitive diagnosis can only be achieved following laparotomy with extensive lysis of adhesions to alleviate the obstruction. However, preoperative diagnosis is possible if clinicians are aware of the condition and its radiologic signs.

Abdominal Cocoon Syndrome in a Liver Transplant Patient: A Case Report and Review of the Literature

“…The primary is considered an idiopathic condition where the exact cause is still unknown but can be associated with genetic factors, whereas the secondary was attributed to multiple aetiologies such as recurrent episodes of peritonitis, peritoneal dialysis, the use of α adrenergic blockers such as practolol, beta-blockers, previous abdominal surgeries, autoimmune diseases (systemic lupus erythematosus), tuberculosis, indwelling peritoneal catheter and liver cirrhosis and liver transplantation which is the case discussed in this report. [6,7].…”

An Unusual Case of Extranodal Marginal Zone Lymphoma Mimicking Abdominal Cocoon Syndrome in an Adolescent Patient

“…Abdominal cocoon syndrome is rare and characterized by total or partial encapsulation of abdominal organs by dense fibrous adhesions. 13,14 This syndrome most often involves the intestinal tract, presenting with signs and symptoms of obstruction. The etiology is poorly understood, but secondary forms have been described in the setting of intrabdominal inflammation, autoimmune disease, and following abdominal surgery.…”

Coexisting of small bowel perforation and abdominal cocoon syndrome: A case report

“…Medical treatments such as steroids, immunosuppressive agents, and colchicine have been tried, but surgery is the main treatment modality (11). Although various comments have been made for the type and limits of the surgery to be performed in the literature, such as removal of the membrane totally by wide aggressive adhesiolysis, it is likely to encounter intense adhesions on the intestinal segments (12). Therefore, it should be kept in mind that extensive adhesions and total removal of the membrane may result in intestinal injuries or fistula development (13).…”