Abdominal Cocoon Syndrome (ACS) or Idiopathic Sclerosing Peritonitis: An Extremely Rare Cause of Intestinal Obstruction

Abstract

Introduction: Abdominal cocoon syndrome is an extremely rare cause of intestinal obstruction in which loops of small bowel are encapsulated by a fibro-collagenous membrane. This condition is also known in the literature as sclerosing peritonitis, and in the majority of cases, it has no known cause. The majority of patients exhibit long-standing signs and symptoms of partial intestinal obstruction. Presentation of case: A 15-year-old female patient presented with complete intestinal obstruction with no known co-morbidity and no history of prior laparotomy. Intraoperatively, the entire small bowel was found to be encapsulated by a dense fibrous sac. The peritoneal sac was excised, followed by lysis of the inter-loop adhesions with appendicectomy done at the same time. Discussion: Most patients with abdominal cocoon syndrome present with features of recurrent acute or chronic small bowel obstruction secondary to kinking and/or compression of the intestines within the constricting cocoon. An abdominal mass may also be present due to an encapsulated cluster of dilated small bowel loops. Conclusions: Abdominal cocoon is a rare condition causing intestinal obstruction, and diagnosis requires a high index of suspicion because of the non-specific clinical picture. Contrast-enhanced CT-scan (CT) of the abdomen is a useful radiological tool to aid in preoperative diagnosis. Peritoneal sac excision and adhesiolysis is the treatment, and the outcome is usually satisfactory.