Budd-Chiari Syndrome Mimicking Chronic Biliary Disease

Abstract

Venous outflow obstruction in the liver is histologically characterized by sinusoidal dilatation, congestion, and red blood cell extravasation into the space of Disse. Necrosis can occur in acute or subacute cases, while fibrosis develops with chronicity. These changes can occur due to impairment of venous outflow at the level of large hepatic veins or the inferior vena cava (Budd-Chiari syndrome), sinusoids or small hepatic veins (sinusoidal obstruction syndrome) or right-sided heart disease. In addition, similar histologic changes can be seen in other vascular conditions like portal vein thrombosis, systemic inflammatory disorders like rheumatoid arthritis and Castleman disease and neoplasms like renal cell carcinoma and Hodgkin disease. Although centrizonal changes are well known in venous outflow obstruction, the portal changes are not well recognized. Bile ductular reaction with or without bile duct damage and periportal fibrosis can occur in venous outflow obstruction. These changes can be mistaken for biliary disease. Imaging studies can demonstrate the normal biliary tree, and these changes are likely to be due to low grade ischemic changes rather than primary biliary disease. Large regenerative nodules can occur in Budd-Chiari syndrome and can mimic cirrhosis or hepatic neoplasms on imaging. These can histologically resemble focal nodular hyperplasia or hepatic adenoma.