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

TL;DR

Cocoon Syndrome can lead to severe intestinal failure and require combined liver-intestine-kidney transplantation.

Contribution

This paper presents a rare case highlighting the need for combined organ transplantation due to advanced Cocoon Syndrome.

Findings

Cocoon Syndrome can progress to intestinal perforation and irreversible intestinal failure.

Prolonged parenteral nutrition can lead to intestinal failure-associated liver disease.

Combined liver-intestine and kidney transplantation may be necessary in advanced cases.

Abstract

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.

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.

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.

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