What is a Budd- Chiari syndrome?
The Budd- Chiari Syndrome is named after the first descriptors George Bush and Hans Chiari. This is a rare liver disease in which a clot (thrombosis) in the liver veins leads to an outflow disorder in the liver. This thrombosis is often caused by blood and coagulation disorders. If left untreated, Budd- Chiari Syndrome ends in death within a few years due to liver failure.
Detection of a Budd- Chiari Syndrome
The symptoms of the Budd- Chiari syndrome are relatively unspecific. It depends on whether the closure of the hepatic vein occurs quickly or slowly. An acute Budd- Chiari syndrome can lead to a strong feeling of pressure in the right upper abdomen with severe fatigue.
The blood accumulates in the liver, causing the liver to enlarge (hepatomegaly). The spleen may also be affected (splenomegaly). The increased pressure in the liver causes damage to the liver tissue.
This can lead to connective tissue remodelling of the liver (liver fibrosis) with a deterioration in liver function. In the course of this, liver cirrhosis – i.e. a liver with damaged tissue – can develop. Those affected often develop an accumulation of abdominal fluid with an increase in the circumference of the abdomen.
This is also called ascites. The diagnosis of the Budd- Chiari syndrome can be made by simple procedures. – First of all, the patient should be interviewed (= anamnesis) to determine the symptoms.
Depending on whether the Budd- Chiari Syndrome develops quickly or slowly, the clinic differs. In the acute form there is usually a strong feeling of pressure in most cases. – During the physical examination, an accumulation of abdominal fluid (ascites) as well as a palpable enlargement of the liver due to the venous outflow disorder may be noticed.
- In case of suspicion of the Budd- Chiari syndrome, the Doppler examination via ultrasound is helpful. Here the occlusion of the hepatic vein and the increased blood flow from the bypass vessels can be shown. – The liver function can be determined by means of a blood test by recording the transaminases.
How to treat Budd- Chiari Syndrome
If the Budd- Chiari Syndrome is detected, treatment is necessary in any case. Because without treatment, liver failure and death can result from the liver damage. The treatment aims at restoring the blood supply to the liver.
Depending on the extent of the outflow disorder, different therapies can be considered. If the hepatic vein is closed by a blood clot (thrombosis), the blood can be thinned with medication to loosen the thrombus. If the Budd- Chiari Syndrome is chronic, the patient should take blood thinners permanently to prevent further blood clot formation.
If the drug treatment does not provide sufficient blood supply to the liver, the blocked liver vein can be recanalized. Another therapeutic option is the creation of a TIPS (transjugular intrahepatic portosystemic shunt). This creates a small connection between the hepatic vein and the portal vein, which carries the blood to the liver.
This is intended to help prevent the blood from accumulating in the liver, but to allow it to continue to the heart via a created short circuit. Even if this is unsuccessful and surgical procedures do not help, a liver transplant must be considered as the last therapeutic option. Depending on how much abdominal fluid the patient with Budd- Chiari Syndrome has, the treatment can differ.
Only small amounts of abdominal fluid can be excreted from the body by taking medication from the group of diuretics. So the drug therapy to relieve the abdomen is usually not sufficient. As a further therapy option, the abdominal fluid can be punctured. This is a relatively minor procedure, but is somewhat invasive (intervening in the body). If new abdominal fluid is constantly forming, several abdominal fluid punctures are necessary.
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