Cocoon Syndrome as a Cause of Intestinal Failure and Indication for Combined Liver-Intestine-Kidney Transplantation

Abstract

Background: Cocoon Syndrome, or sclerosing encapsulating peritonitis, is a rare but severe complication of long-term peritoneal dialysis. It can lead to progressive fibrotic encapsulation of the intestines, causing intestinal obstruction due to a fibrous membrane encasing the abdominal organs.

Case report: We present a complex case of a 29-year-old male with history of nephronophthisis and four years on peritoneal dialysis who developed Cocoon Syndrome, leading to intestinal perforation and total enterectomy, resulting in irreversible intestinal failure. After prolonged dependence on parenteral nutrition, he developed intestinal failure-associated liver disease, ultimately becoming a candidate for combined liver-intestine and kidney transplantation.

Conclusion: This case illustrates how advanced Cocoon Syndrome can lead to severe and progressive complications that may ultimately require combined liver-intestine and kidney transplantation. It underscores the importance of early recognition and appropriate multidisciplinary management to improve patient outcomes.

Keywords: Cocoon syndrome; Combined liver-intestinal transplant; Intestinal transplant; Kidney transplant.

Fig. 1

Computed tomography (CT) axial views. On the left, the CT image corresponds to the patient described in the present case, showing extensive peritoneal thickening and signs of peritoneal encapsulation consistent with early sclerosing encapsulating peritonitis (SEP). On the right, an illustrative image from a different patient is shown to depict the typical CT findings associated with advanced-stage SEP, including marked peritoneal calcifications, encapsulated bowel loops, and a cocoon-like appearance. This comparison highlights the spectrum of radiological manifestations of SEP.