Last update:
21-Jan-2019

Thrombosis of the portal vein and the hepatic veins are the most widely known vascular diseases of the liver. Portal vein thrombosis is the predominant vascular liver disorder. It is usually a complication of local (cirrhosis, neoplasm, surgery) or systemic prothrombotic conditions, mainly myeloproliferative diseases. The clinical presentation varies widely, depending on the time of occurrence, the extent of the thrombosis and the presence and nature of underlying chronic liver disease. It may thus range from an asymptomatic finding to a life-threatening condition. Diagnosis is based primarily on imaging techniques, and in particular, Doppler ultrasonography. Early onset of anticoagulation treatment improves the outcome, achieving recanalization in approximately 40% of cases. Obstruction of venous drainage due to thrombosis of the hepatic veins or the inferior vena cava is known as the Budd-Chiari syndrome. It is the result of the combined effect of prothrombotic agents, especially myeloproliferative diseases, factor V Leiden's mutations and the use of oral contraceptives. In most cases, the disease follows a chronic course, characterized by subsequent development of complications of portal hypertension, although it may occasionally be manifest as acute liver failure. Imaging techniques are usually sufficient to establish diagnosis. Treatment is based on progressive escalation to more aggressive intervention, starting with anticoagulant treatment, often progressing to transjugular intrahepatic portosystemic shunt, and ending up with liver transplantation.

Key words: Budd-Chiari syndrome, Portal hypertension, Portal vein thrombosis, Transjugular intrahepatic portosystemic shunt.