Autosomal Recessive Polycystic Kidney: Liver

Liver abnormalities in autosomal recessive polycystic kidney disease (ARPKD): Lobular architecture of the liver is preserved. The portal areas are expanded and show dilated, tortuous and anastomosing bile ducts at the periphery and blood vessels in the center. There is increased portal fibrosis. In older individuals, there is congenital hepatic fibrosis with resultant portal hypertension and hepatosplenomegaly.