Disorders of the Intra- and Extrahepatic Bile Ducts
Case 7 -
Congenital Hepatic Fibrosis
Kay Washington, M.D., Ph.D.
Pathology, C-3321 MCN
Vanderbilt University Medical Center
Nashville, TN 37232-2561
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.
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.