Budd-Chiari Syndrome: Pathology

Abstract

Budd-Chiari syndrome (BCS) is a rare condition resulting from obstruction to the outflow of blood from the liver. It is characterized clinically by hepatomegaly, splenomegaly, right upper quadrant (RUQ) abdominal pain, and ascites.1–16 Most patients have an acute onset, but occasionally the disorder appears insidiously. BCS is caused by many different conditions and diseases, including membranous webs, veno-occlusive disease (VOD), and hepatic vein thrombosis. Almost one third of cases of hepatic vein thrombosis are idiopathic.1, 5 Well-recognized underlying conditions associated with hepatic vein thrombosis include hematologic disorders, such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria, lupus anticoagulant,6 antithrombosis 3 deficiency,7 and myeloproliferative disorders, as well as the use of oral contraceptives.8 Worldwide, the most common cause of hepatic outflow obstruction is membranous obstruction of the inferior vena cava.9 Cases related to oral contraceptives are discussed by Lewis et al.10 and Maddrey11. In a case-control study from France, the relative risk of hepatic vein thrombosis among recent oral contraceptive users was 2.37, compared with nonusers.12 The duration of oral contraception prior to development of the syndrome is variable, with a mean of approximately 30 months.10 The etiopathogenesis of hepatic vein thrombosis in women on oral contraceptives remains undetermined. It has been suggested that oral contraceptives may increase the risk of thrombosis associated with primary myeloproliferative disorders, which was found to be a major cause of hepatic vein thrombosis in young women in one study.13