Abdominal cocoon syndrome, a case report of a rare disease entity causing intestinal obstruction

Abstract

Introduction and importance: Abdominal cocoon syndrome is a rare condition characterized by small bowel encapsulation by a fibrous membrane or a cocoon-like sac. It is an uncommon cause of intestinal obstruction. Less than 300 cases have been reported from all over the world. This is the first case of such a disease entity to be reported from Sudan.

Case presentation: A young female patient, presented with features of intestinal obstruction that was managed conservatively. Failure of the conservative management has warranted a laparotomy.

Clinical findings and investigations: Her features were suggestive of intestinal obstruction that was confirmed radiologically.

Interventions and outcome: Laparotomy revealed a membrane-like fibrous material and extensive multiple loops adhesions, findings consistent with primary sclerosing encapsulating peritonitis (PSEP), also known as abdominal cocoon's disease. The membrane was excised and adhesiolysis was done. Intestinal obstruction was relieved after surgery and the patient showed good outcome.

Conclusions: Abdominal cocoon syndrome is a rare cause of intestinal obstruction.

Relevance and impact: The takeaway lesson from this case would be that the PSEP should be sought in any patient with no clear cause for obstruction can be identified. A contrast-enhanced CT scan is the diagnostic modality of choice. Finally, we think that the disease is underreported from Africa and more efforts should be carried out to increase patients' access to healthcare especially in rural areas with no access to hospitals in order to bring more cases to light. This case report has been reported in line with the SCARE Criteria (Agha et al., 2020 [17]).

Keywords: Case report; Cocoon; Encapsulating; Peritonitis; Sclerosing; Sudan.