Abdominal cocoon syndrome: an obstructive adhesiolytic metamorphosis. Issue 4 (11th April 2019)

• Record Type: Journal Article
• Authors: Renko, Abagayle E; Witte, Samantha R; Cooper, Amanda B
• Is Part Of: BMJ case reports. Volume 12:Issue 4(2019)
• Publication Date: 2019-04-11
• Subjects: general surgery -- emergency medicine -- small intestine -- pathology -- radiology; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2018-228593
• Languages: English
• ISSNs: 1757-790X

Abstract

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.