The Budd-Chiari Syndrome - the occlusion of the liver vein

What is Budd-Chiari Syndrome?

The Budd-Chiari Syndrome is named after George Bush and Hans Chiari, who first described it.
This is a rare liver disease in which a clot (thrombosis) in the hepatic veins causes a drainage disorder in the liver. This thrombosis described is often caused by blood and coagulation diseases. If left untreated, Budd-Chiari syndrome ends within a few years as a result of liver failure leading to death.

Recognizing Budd-Chiari Syndrome

Symptoms of Budd Chiari Syndrome

The symptoms of the Budd-Chiari Syndrome are relatively unspecific. It depends on whether the occlusion 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 extreme fatigue
In general, there is a drainage disorder in the liver in both cases. This causes the blood to build up in the liver, causing the liver to enlarge (hepatomegaly). The spleen can also be affected (splenomegaly).

The tissue of the liver is damaged by the increased pressure in the liver. There may be a connective tissue remodeling of the liver (liver fibrosis) with a deterioration in liver function. In the course of the disease, cirrhosis of the liver - a liver with damaged tissue - can develop.
Affected people often develop an accumulation of ascites as the size of the abdomen increases. This is also called ascites.

You might also be interested in: Liver Fibrosis - How Dangerous Is It? or Symptoms of cirrhosis of the liver

Diagnosis of Budd-Chiari Syndrome

The diagnosis of Budd-Chiari Syndrome can be made by simple procedures.

• The patient should first be interviewed (= anamnesis) to identify the symptoms. The clinic differs depending on whether the Budd-Chiari syndrome develops quickly or slowly. In the acute form, there is usually a strong feeling of pressure.
• The physical examination may show an accumulation of ascites and a palpable enlargement of the liver due to the venous outflow disorder.
  Read more on the subject at: Enlarged liver - what's behind it?
• If the Budd-Chiari syndrome is suspected, the Doppler examination using the ultrasound can help. The occlusion of the hepatic vein and the increased blood flow from the bypass vessels can be shown here.
• The liver function can be recorded via a blood test by recording the transaminases.

This is how Budd-Chiari Syndrome is treated

If the Budd-Chiari syndrome is recognized, treatment is necessary in every case. Because without treatment, liver damage and death can result as a result of liver damage.
Treatment is aimed at restoring blood flow to the liver. Depending on the extent of the drainage disorder, different therapies can be considered.
If the hepatic vein is blocked by a blood clot (thrombosis), the blood can be diluted with medication, which is intended to loosen the thrombus. If the Budd-Chiari syndrome is chronic, the person concerned should take blood thinners on a permanent basis to prevent further blood clots from forming.

If the drug treatment does not provide sufficient blood flow to the liver, the occluded hepatic vein can be recanalized. Another therapy option is the creation of a TIPS (transjugular intrahepatic portosystemic shunt). This creates a small connection between the hepatic vein and the portal vein that carries the blood to the liver. This should help that the blood does not accumulate so much in the liver, but can be carried on to the heart via a created short circuit.

If this is unsuccessful, and surgical procedures do not help, a liver transplant must be considered as the last therapeutic option.

Read more on the topic: Liver transplant

Treatment of water retention in Budd-Chiari syndrome

Treatment may differ depending on how much ascites a patient with Budd-Chiari syndrome has. When taking drugs from the group of diuretics, only small amounts of ascites can be excreted from the body. For example, drug therapy to relieve the stomach is usually not sufficient.
As a further therapy option, the ascites can be punctured. This is a relatively minor procedure, but it is somewhat invasive (body-breaking). If new ascites keeps forming, several ascites punctures are necessary.

Further information on this topic can be found at:

• Ascites
• Puncture water in the abdomen

Course of the disease in a Budd-Chiari syndrome

In Budd-Chiari syndrome, the drainage disorder leads to an increasing deterioration in liver function. This causes abdominal fluid to build up and the waist circumference increases.
Depending on when the Budd-Chiari syndrome is treated and whether the treatment ensures successful blood flow to the liver, the symptoms can improve.

If the Budd-Chiari syndrome is not treated, the increased pressure in the liver leads to progressive damage to the liver and to connective tissue remodeling of the liver (liver fibrosis). This can lead to liver failure.

Also read:

• Symptoms of deteriorated liver function
• Connective tissue of the liver (liver fibrosis)
• Liver failure

Duration of the Budd-Chiari Syndrome

The duration of the treatment of a Budd-Chiari syndrome depends on the underlying cause, the timing and the success of the therapy. It aims to restore blood flow to the liver.
Chronic Budd-Chiari Syndrome may require lifelong therapy with blood thinners.

Life expectancy with Budd-Chiari syndrome

The prognosis of the Budd-Chiari syndrome, on the other hand, depends on the beginning and success of the therapy.
If Budd-Chiari Syndrome is not treated, survival time due to liver failure is limited to less than three years in 90% of cases.
On the other hand, if therapy is started early, up to 10% of patients die within the first five years.

Causes of Budd-Chiari Syndrome

The Budd-Chiari syndrome is triggered by a complete or incomplete occlusion of the liver vessels. In most cases, it affects the great hepatic vein, which carries blood from the liver to the inferior vena cava to the heart. The majority of the occlusion is due to a thrombosis, i.e. a blood clot. In this case, the person concerned should be examined for (malignant) blood disorders that are associated with impaired coagulation.
This also includes the myeloproliferative syndrome, which can be understood as a precursor to malignant blood cancer (leukemia). It is one of the most common causes of the development of the Budd-Chiari syndrome. Other blood diseases that increase the risk of the Budd-Chiari syndrome are factor V disease and thrombophilia.

Read more on this topic at:

• Bleeding disorder
• Curiosity about thrombosis (thrombophilia)
• Factor V Leiden

However, there are also cases in which the clinical picture of the Budd-Chiari syndrome is not caused by a thrombosis, but by a tumor that presses on the hepatic vein from outside. One example of this is Wilms' tumor in children.

For more information on this topic, we recommend our page on: Wilms tumor - what is the prognosis?

Nevertheless, the exact causes for the development of Budd-Chiari syndrome are not entirely clear.

Further questions

Is Budd-Chiari Syndrome Contagious?

In most cases, Budd-Chiari syndrome is caused by the formation of a blood clot that blocks the hepatic vein. This is not contagious. The risk factors for Budd-Chiari syndrome (bleeding disorders), however, may have been inherited.