An adult case of primary biliary cholangitis accompanied by a patent ductus venosus showing type-I congenital absence of the portal vein

Abstract

A 40-year-old Japanese man with a history of kidney transplantation was admitted to our hospital for the detailed evaluation of elevated serum hyperammonia level, hepatic encephalopathy and vascular abnormality detected on computed tomography images. Ultrasonography revealed a patent ductus venosus, which was confirmed by venous catheterization. Balloon occlusion of the ductus venosus resulted in the congestion of extrahepatic portal flow with no demonstration of intrahepatic portal vein branches. He was diagnosed as congenital absence of the portal vein (type I), and percutaneous liver biopsy provided histological proof of primary biliary cholangitis (Scheuer’s classification, stage 2; Nakamuma’s classification, stage 3). This is a very rare adult case of congenital portosystemic shunt, and liver transplantation may be recommended in the future.