3.2 Budd-Chiari syndrome

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Presents as a sudden onset of painful enlarged liver and formation of ascites.

In these conditions there is a disturbed venous drainage of the liver due to compression thrombosis in the large hepatic vein.

The syndrome is usually caused by a thrombosis in the hepatic veins. In 80% of cases the cause is a consequence of prothrombotic abnormalities (acquired or congenital deficiencies, the same risk factors as for portal vein thrombosis). In 50% there are several factors that play a role simultaneously. Very rarely the venous drainage is obstructed by a web in the vena cava.

Budd-Chiari syndrome usually presents acutely, with sudden, severe and painful hepatomegaly and the development of ascites. In this situation, the three hepatic veins are usually thrombosed.

In 15% of cases, there is also a portal vein thrombosis.

The diagnosis is made by means of an ultrasound, a CT or an MRI scan, where the hepatic veins are no longer demonstrable, together with perfusion disorders in the liver. There is usually a hypertrophy of the lobus caudatus, because it has a separate blood drainage, which is less likely to thrombose. The swollen liver gives compression on the vena cava. The ascitic fluid shows a high protein and albumin content.

The patient must be anticoagulated for life, even in the presence of esophageal varices and also after liver transplantation. If a hepatic vein is still open, venous drainage can be improved by angioplasty or transjugular intrahepatic portosystemic shunt (TIPS). In some cases, urgent liver transplantation is necessary.

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