Budd–Chiari syndrome

As Budd -Chiari syndrome refers to a rare disease of the liver, where it comes to a complete or incomplete closure of the efferent blood vessels of the liver. The closure of the hepatic veins at the classic relates Budd -Chiari syndrome preferably the large hepatic vein and it is still separated from diseases relating to the clogging of small hepatic veins.

The disease was named after her Erstbeschreibern George Budd (1808-1882) and Hans Chiari ( 1851-1916 ).

Symptoms

Acute Budd -Chiari syndrome is characterized by an onset usually within a few hours feeling of pressure or pain in the right upper abdomen. In addition, one often comes ascites. Laboratory tests partly strong increase in transaminases (GOT / ASAT often higher than GPT / ALT). Liver and spleen usually appear enlarged ( hepatosplenomegaly )

Causes

The causes of Budd -Chiari syndrome are often not known precisely. In the very great majority of cases there is an occlusion of the hepatic vein through the formation of blood clots (thrombosis ). However, there are few cases in which, for example, a space-occupying tumor by external compression of the hepatic vein leads to a Budd -Chiari syndrome. In children, a Wilms' tumor thrombosis of the inferior vena cava and right atrium lead.

Diseases that lead to increased blood clotting ( thrombophilia ), represent a risk factor for the occurrence of a Budd -Chiari syndrome dar. Thus increases a factor V Leiden mutation, the relative risk by a factor of 11 A protein C deficiency or a mutation of the prothrombin gene also lead to an increased risk of BCS.

In about 25% of patients several possible causes are. Most often, the Budd -Chiari syndrome is associated with a malignant disease of cells of the hematopoietic system on ( myeloproliferative disorder ).

Diagnostics

The diagnosis is made by detection of occluded branches of the hepatic vein by means of Doppler ultrasonography and the diagnosis of the underlying disease ( eg, bone marrow puncture).

The exact extent of vascular occlusion is determined by a hepatic venography. In an unclear diagnosis, a liver biopsy provide further evidence.

Therapy

If left untreated, the Budd -Chiari syndrome lead to severe liver damage ( necrosis ) to the liver failure, since a sufficient blood flow to the liver is no longer guaranteed by the closure of the hepatic veins. A chronic Budd -Chiari syndrome can lead to increased blood pressure in the liver circulation ( portal hypertension ) and in consequence to liver fibrosis.

In order to produce sufficient blood flow again, a thrombolytic drug to the blood clot can be performed. There is also the possibility for creating a TIPS ( transjugular intrahepatic portosystemic shunt). In addition, there are several surgical procedures for the restoration operation of the blood outflow.

Failure to permanently ensure adequate hepatic blood flow, a liver transplant may be necessary.

In chronic Budd -Chiari syndrome occurs a permanent anticoagulation, such as phenprocoumon, with the aim of preventing the formation of new blood clots.