British Liver Trust

Budd-Chiari syndrome (BCS) is a disorder affecting the liver and blood vessels, where blood flowing into the liver has difficulty in being able to flow out, leading to serious complications.

After blood has passed through the liver, it flows out through the hepatic veins and into the inferior vena cava, a large blood vessel that carries blood back to the heart. In BCS, this flow is partially blocked, sometimes referred to as ‘an obstruction of hepatic venous outflow’.

The immediate result is a build-up (congestion) of blood in the liver, as there is more flowing in than flowing out. The liver becomes swollen, tender to the touch and a source of discomfort. The congestion causes fluid to leak from the liver into the abdominal cavity. The resulting build-up of fluid in the abdominal area is called ascites, one of the commonest symptoms of Budd-Chiari syndrome. It may be visible as a bulge in your tummy area and can be uncomfortable and make it hard to breathe and eat normally.

If the blockage is extensive and also blocks the large blood vessel which returns blood to the heart from the lower half of the body (the inferior vena cava), there may be other places that become swollen. This is particularly likely in the ankles and legs and is called peripheral oedema.

Another effect is less visible but is also serious. This is called portal hypertension, where the blood pressure in the portal vein is increased, because of the blockage in the blood coming out of the liver. As happens elsewhere in the body, blocked veins cause other veins to enlarge (varicose veins) to provide an escape route for the obstructed blood. In portal hypertension, blood may return to the heart by using extra veins lining your oesophagus and stomach where they are known as varices. These veins have fragile walls, which cannot easily handle the increased blood flow and may burst. This leads to internal bleeding and is referred to as ‘bleeding oesophageal varices’ which is a medical emergency.

There are a range of treatment options available for BCS, and it is essential that it is diagnosed early before it causes liver damage or even liver failure.

However, the disorder is very rare. Its prevalence is not exactly known but one person in 100,000 is the most common estimate. BCS occurs in people from all ethnic backgrounds and affects both sexes equally.

BCS takes it name from George Budd, the English doctor who described the fi rst cases in 1845, and Hans Chiari who is credited with providing the first description of how it affects the body in 1899.