Protocolo diagnóstico y tratamiento del síndrome de Budd-Chiari primario

Vascular disorders of the liver represent a heterogeneous group of diseases characterized by the frequent presence of a prothrombotic condition. Primary Budd-Chiari syndrome is characterized by the obstruction of the hepatic vein drainage at any topographical level. Clinical presentation is very heterogeneous ranging from the complete absence of symptoms to fulminant liver failure, depending on thrombosis extension, velocity of the appearance and on the development of compensatory mechanisms like formation of collateral vessels. Portal vein thrombosis is usually the consequence of a combination of local and systemic risk factors, and its manifestations differ in acute or chronic thrombosis. In both cases diagnosis is based on the demonstration of the venous obstruction usually by imaging like abdominal ultrasound or CT scan. The main goal of therapy is to provide vessel recanalization and/or decompression of the affected vascular bed.