Case 7 -

Congenital Hepatic Fibrosis Kay Washington, M.D., Ph.D. Pathology, C-3321 MCN Vanderbilt University Medical Center Nashville, TN 37232-2561

Clinical History This 50 year old woman was in good health until 1994, when she developed esophageal variceal bleeding. An arteriogram showed pre-sinusoidal portal hypertension. Serum liver tests were within normal limits. Further work-up showed medullary sponge kidney. A mesocaval shunt was performed; this liver biopsy was obtained during surgery. Morphologic Findings The portal tracts are enlarged by fibrous tissue and contain numerous aberrant biliary channels. While some of the biliary structures are embedded within the portal tract, others are preferentially located at the periphery of the portal area. Many of the biliary channels are dilated, and some are angular and distorted. Portal vein branches are abnormally small. There is no portal inflammation or piecemeal necrosis. The lobular architecture is largely preserved and there is no canalicular cholestasis. No cysts are identified. Click on each slide thumbnail image for an enlarged view: Slide 17 Congenital Hepatic Fibrosis The hepatic parenchyma is distorted by fibrous expansion of portal tracts containing numerous abnormal biliary channels. Slide 18 Congenital Hepatic Fibrosis Dysmorphic anastomosing biliary channels are arranged around the perimeter of the enlarged portal tracts. Diagnosis: Congenital Hepatic Fibrosis