213 Budd-Chiari Syndrome

Hepatic vein occlusion may result from various clinical conditions such as polycythemia, leukemia, neoplasms (e.g., hepatoma and hypernephroma), systemic lupus erythematosus, infections, membranous obstruction of the inferior vena cava, and trauma. However, no definite cause can be found even at autopsy in a majority of cases. The obstruction to the hepatic vein may also be caused either by compression from outside or by invasion by tumor or thrombi. Other recognized causes of BCS include thrombophilic conditions, chronic inflammatory disease like Behcets syndrome, inflammatory bowel disease, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinemia, venoocclusive disease, and hypereosinophilic syndrome. Other inherited and hypercoagulable statesmay also predispose to thrombosis (protein C or S deficiency, antithrombin III deficiency, etc.). A similar picture of BCS has been observed in association with primary pulmonary hypertension, constrictive pericarditis, and right atrial myxoma. Hepatic vein thrombosis has also been reported in neonates with gastroschisis or large omphalocele after surgical repair and in older children following some herbal ingestion. Enlargement of the caudate lobe is a recognized potential cause of obstruction to the inferior vena cava or hepatic veins.