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 Fooet 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.