15. Budd-Chiari Syndrome
Definition
Budd-Chiari syndrome is an uncommon condition of obstruction of intrahepatic veins. It is induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow, leading to congestive hepatopathy. The hepatopathy results from large and small vein obstruction of venous outflow, producing hepatocellular injury from microvascular ischemia. It eventually results in portal hypertension and then liver insufficiency and failure.
Incidence
Exact incidence of Budd-Chiari syndrome is not known; it is classified as a rare entity both in the United States and internationally.
Etiology
Budd-Chiari syndrome frequently occurs in patients who already have some manner of thrombotic diathesis, including myeloproliferative disorders, pregnancy, tumors, chronic inflammatory diseases, clotting disorders, and infections.
Causes of Budd-Chiari Syndrome
Chronic Infections
• Amoebic abscess
• Aspergillosis
• Hydatid cysts
• Syphilis
• Tuberculosis
Chronic Inflammatory Diseases
Hematologic Disorders
• Antiphospholipid antibody syndrome
• Essential thrombocytosis
• Paroxysmal nocturnal hemoglobinuria
• Polycythemia rubra vera
• Unspecified myeloproliferative disorder
Inherited Thrombotic Diathesis
• Antithrombin III deficiency
• Factor V Leiden deficiency