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Budd-Chiari Syndrome

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Lisa Craine
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Budd-Chiari Syndrome is disorder in which veins carrying blood out of the liver become narrow and/or blocked due to blood clots. In a healthy person, blood normally flows from the intestines to the liver through the hepatic portal vein and then out of the liver through the hepatic veins and into the inferior vena cava, the large vein that flows back to the heart. When the blood cannot flow out of the liver in a normal manner, it backs up within the liver. When Budd-Chiari Syndrome occurs, the backed-up blood creates high blood pressure back to the portal vein. This condition is known as portal hypertension.

Additional complications can occur among people with Budd-Chiari Syndrome, including severe scarring of the liver (cirrhosis) and fluid buildup in the belly (ascites). It can also lead to varicose veins (varices) in the esophagus that can rupture and bleed.

There are certain conditions that appear to put people at increased risk for Budd-Chiari Syndrome. These include those that cause the body to make too many red blood cells or conditions in which the blood clots too easily.

Budd-Chiari Syndrome may also be known by other names, including:

The onset of Budd-Chiari Syndrome may happen over an extended time, or it may occur very suddenly. Some people with this syndrome have more veins that are affected, or the affected veins are in more difficult areas of the liver. If that is the case, those individuals would likely have a more serious form of the syndrome that those with fewer veins impacted in the liver.

Facts at-a-Glance

  • Budd-Chiari Syndrome affects approximately one in 100,000 people, thereby making it a rare disorder.
  • Some disorders affect males or females in disproportionately high numbers, but Budd-Chiari Syndrome appears to affect males and females in an even distribution.
  • The age group of people with Budd-Chiari Syndrome is generally within the 20- to 40-year-old range.
  • Budd-Chiari Syndrome is usually a chronic (long-term) condition, but it can in some cases be sudden (acute).
  • Budd-Chiari Syndrome can lead to cirrhosis and the need for a liver transplant.

Information for the Newly Diagnosed

There may  not be a typical set of symptoms for all people suffering from Budd-Chiari Syndrome because symptoms depend on how many veins of the liver are impacted by clots and where those clots are located. People may have pain in the upper right area of their abdomen, where the liver is located. They may also have jaundice, a condition in which their skin and the whites of the eyes turn yellow.

More common symptom for those with severe, chronic forms of Budd-Chiari Syndrome include ascites, portal hypertension, and an enlarged spleen (splenomegaly).

Researchers have not been able to pinpoint the exact cause for approximately three-quarters of all people diagnosed with Budd-Chiari Syndrome. A small percentage of people with the syndrome (about 10%) have a type of blood cancer called polycythemia vera.

Other disorders’ symptoms and characteristics resemble those of Budd-Chiari Syndrome, so doctors must rule out those illnesses before determining that a patient has Budd-Chiari Syndrome. Diagnostic tests that are performed may include:

  • Angiography, a special X-ray in which doctors can examine blood vessels by using a dye that is put into the body for contrast (to make the vessels stand out more to the eye)
  • Imaging tests such as MRI and ultrasounds to view soft tissues and organs in the body
  • Biopsy, in which a small sample of liver tissue is removed and examined under a microscope

If Budd-Chiari Syndrome is identified early, treatment may involve the use of clot-dissolving medications. Not everyone is diagnosed early, however. Long-term Budd-Chiari Syndrome may be treated with drugs called anticoagulants. These drugs help prevent blood clots from forming. One common anticoagulant is heparin.

Sometimes surgeons can treat patients by widening the affected veins in a procedure called angioplasty. This can help prevent the backing up of blood caused by the clots and limit pressure from building up in the blood vessels.

A third treatment may involve unblocking a vein and placing a stent into the vein. A stent is a thin tube that can be placed inside the vein so that blood can flow properly without obstruction.

Finally, if a person has suffered advanced liver damage due to Budd-Chiari Syndrome, liver transplantation may be discussed as a treatment option. Liver transplantation involves removing the unhealthy liver and replacing it with a donated liver from an organ donor.

It is important to note that researchers are continuously searching for answers about all diseases. There may be clinical trials (research studies about treatments or procedures) for Budd-Chiari Syndrome for which people may qualify. Patients can speak to their doctors about clinical trials if they wish to pursue this option.

Healthy and Sick Livers

The Healthy Liver

A healthy liver has the amazing ability to grow back, or regenerate when damaged.

Fibrosis

When treated successfully at this stage, there’s a chance your liver can heal itself.

Liver Cancer

Cirrhosis and hepatitis B are leading risk factors for primary liver cancer.

Liver Transplant

Removal of unhealthy liver and replace with a whole or portion of a healthy liver.

There are many different types of liver disease. But no matter what type you have, the damage to your liver is likely to progress in a similar way.

Whether your liver is infected with a virus, injured by chemicals, or under attack from your own immune system, the basic danger is the same – that your liver will become so damaged that it can no longer work to keep you alive.

Cirrhosis, liver cancer, and liver failure are serious conditions that can threaten your life. Once you have reached these stages of liver disease, your treatment options may be very limited.

That’s why it’s important to catch liver disease early, in the inflammation and fibrosis stages. If you are treated successfully at these stages, your liver may have a chance to heal itself and recover.

Talk to your doctor about liver disease. Find out if you are at risk or if you should undergo any tests or vaccinations.

Clinical trials are research studies that test how well new medical approaches work in people. Before an experimental treatment can be tested on human subjects in a clinical trial, it must have shown benefit in laboratory testing or animal research studies. The most promising treatments are then moved into clinical trials, with the goal of identifying new ways to safely and effectively prevent, screen for, diagnose, or treat a disease.

Speak with your doctor about the ongoing progress and results of these trials to get the most up-to-date information on new treatments. Participating in a clinical trial is a great way to contribute to curing, preventing and treating liver disease and its complications.

Start your search here to find clinical trials that need people like you.

Questions to Ask Your Doctor

  • What tests am I going to need to find out if I have Budd-Chiari Syndrome?
  • Will I be awake for these tests, will I need sedation, or will I be put under general anesthesia?
  • What type of Budd-Chiari Syndrome do I have?
  • How much damage has this syndrome done to my liver?
  • Do I have any complications right now?
  • Do you expect that I will develop complications?
  • Will medication be enough to treat my condition, or will I need to have any surgical procedures?
  • What do you expect will be the outcome of treatment?
  • Do you know about any clinical trials for Budd-Chiari Syndrome that I can investigate and if so, who can I contact?

Additional Resources

Sources of Information
  • National Organization for Rare Disorders
  • Cleveland Clinic
  • National Institutes of Health
  • Merck Manuals

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