Issue: 2008 > July-August > case report

Liver transplantation in a patient with encapsulating peritoneal sclerosis

W.H. de Vos tot Nederveen Cappel, J. Dubbeld, S.M. Willems, J. Ringers, B. van Hoek


Encapsulating peritoneal sclerosis (EPS) is a poorly
understood condition in which excess fibrosis results in
an encasement of the small bowel, which can clinically
result in obstruction. The condition is thought to be related to the persistent expression of transforming growth factor beta on mesothelial cells causing proliferation of subserosal fibroblasts, massive production of extracellular matrix and loss of mesothelial cells. We report a patient with liver cirrhosis in whom the diagnosis of EPS was made. During laparotomy for liver transplantation the complete peritoneum was found to be thickened, consisting of white sheets; liver transplantation was deferred. Histological examination showed peritoneal sclerosing fibrosis. Immunosuppressive medication was started and a difficult but successful liver transplantation followed. If EPS is diagnosed during laparotomy for
organ transplantation, adjusted immunosuppression is
preferred as calcineurin inhibitors such as cyclosporin and tacrolimus may accelerate EPS while prednisone and some other drugs may stop progression.