PORTAL CAVERNOMA IN CONGENITAL HEPATIC-FIBROSIS - ANGIOGRAPHIC REPORTS OF 10 PEDIATRIC CASES

A retrospective pediatric study of 30 angiograms in congenital hepatic fibrosis (CHF) was undertaken to analyze the different aspects of the portal venous system in this disease. Besides the classical angiograp hic signs (hepatofugal veins and intrahepatic duplications), a hepatop etal venous network was present in ten children, contrasting with the patency of the portal venous system. Analysis of the medical charts fo r these ten patients showed that the clinical signs and outcome were s imilar to classical CHF. Liver biopsies were available in six cases an d, in addition to the diagnostic features of CHF, demonstrated paucity or absence of portal venules. This unusual association of hepatopetal and hepatofugal collateral veins in an intrahepatic block could be ex plained by hypoplasia or thrombosis of portal venules. Thus, the assoc iation between hepatomegaly and hepatopetal collateral veins without p ortal obstruction should be suggestive of CHF.