Deceased donor liver transplantation for congenital hepatic fibrosis associated with autosomal recessive polycystic kidney disease: a case report

Abstract

We report a 20-year-old man with congenital hepatic fibrosis (CHF) involved with autosomal recessive polycystic kidney disease (ARPKD). He was diagnosed as having ARPKD on the basis of renal dysfunction at the age of three. When he was 16, computed tomography (CT) revealed hepatomegaly and intrahepatic bile duct dilatation consistent with CHF. As his liver function gradually worsened, he was introduced to our liver transplant unit at 19 years old. He was registered for a transplant candidate on the national waiting list of deceased-donor liver transplantation (DDLT). Gradual exacerbations in liver and renal functions were then observed. When 20 years old, he received DDLT. The technique of the venous `jump-graft' from the superior mesenteric vein was applied because of a severely narrowed portal vein. Moreover, a veno-venous bypass was used to avoid renal venous congestion during the anhepatic phase. We successfully managed to preserve the renal function during the peri-transplant period by combination therapy with rapamycin and tacrolimus minimization. Although some reports of living-donor liver transplantation for ARPKD had been made, to our knowledge this is the first case of DDLT for an adult ARPKD in Japan.