Congenital hepatic fibrosis. A case report with study of three dimensional reconstruction of serial sections of the liver.

A patient who developed pancytopenia and hepatosplenomegaly with portal hypertension is described. Liver wedge biopsy at the time of operation showed typical histology of congenital hepatic fibrosis. Renal surgical biopsy revealed tubular ectasia. A continuous anastomosing network of the biliary tree was observed by reconstruction of serial sections of the liver. Some parts of the network were dilated and no isolated bile ducts were present.