Hepatoportal sclerosis in childhood.

The clinical, angiographic, and pathologic features of hepatoportal sclerosis in five children are presented. Hepatosplenomegaly with preservation of liver function and esophageal varices were demonstrated. Histologically, portal alterations were consistent and prominent and included subintimal sclerosis of the intrahepatic venous branches and telangiectases. Angiographic patterns were variable, but all exhibited intrahepatic portal venous obstruction. In three patients, there was an association with extrahepatic portal vein obstruction at different sites. Our data suggest that intrahepatic portal vein thrombosis could be the primary disorder in hepatoportal sclerosis, which may vary in extent and site, involving the intrahepatic branches of the portal vein with or without the involvement of the portal trunk. Combined clinical, angiographic, and pathologic data can contribute to a better understanding of hepatoportal sclerosis in children.