EARLY DIAGNOSIS: The Budd-Chiari syndrome results from an obstruction of the suprahepatic venous drainage. The condition spontaneously evolves towards liver fibrosis and death. Early diagnosis is thus of prime importance to initiate adapted treatment promptly.
Explorations: Color-coded and pulsed Doppler coupled with ultrasonography is the key to positive diagnosis. Magnetic resonance imaging may provide further precision. DECONGESTION OF THE LIVER: As the hepatic lesions are reversible, satisfactory drainage must be achieved as rapidly as possible, either by percutaneous puncture or surgery. The problem is to control the underlying hematology disease to prevent recurrent venous thrombosis, generally the cause of treatment failure. PREVENTIVE ANTICOAGULATION: Effective anticoagulation using low-molecular-weight heparin, which appears to be more adapted than standard heparin, must be achieved prior to any decongestion procedure. Long-term management requires anti-vitamin K therapy if the risk of thrombosis persists.