Budd-Chiari syndrome (BCS) is a rare liver disease that can occur in adults and children.
In this condition liver (hepatic) veins are narrowed or blocked. This stops the normal flow of blood out of the liver and back to the heart.
The blockage in the liver can happen slowly over time or suddenly. It may happen because of a blood clot. Budd-Chiari syndrome can cause minor to serious liver damage.
Hepatic vein thrombosis is another name for this syndrome.
Budd-Chiari types in Adults
In adults, Budd-Chiari syndrome may present as different types depending on how fast it’s causing symptoms or how much liver damage has occurred. These types include:
- Chronic Budd-Chiari. This is the most common type of Budd-Chiari. Symptoms occur slowly over time. Almost 50 percent of people with chronic Budd-Chiari also have kidney problems.
- Acute Budd-Chiari. Acute Budd-Chiari happens suddenly. People with this type get symptoms like stomach pain and swelling very quickly.
- Fulminant Budd-Chiari. This rare type happens even faster than acute Budd-Chiari. Symptoms present unusually quickly and may lead to liver failure.
Budd-Chiari syndrome is even rarer in children, and there aren’t any unique types in children.
According to a 2017 medical study conducted in London, two-thirds of children with this syndrome have an underlying condition that causes blood clots.
Children with Budd-Chiari usually have slowly developing chronic symptoms. Liver damage doesn’t happen suddenly. It’s more common in boys and can occur in children as young as 9 months.
Signs and symptoms of Budd-Chiari syndrome depend on how severe the condition is. They can be minor or very serious. About 20 percent of people with Budd-Chiari have no symptoms at all.
Signs and symptoms include:
- pain in the upper right abdomen
- nausea and vomiting
- weight loss
- liver damage
- yellowing of the skin and eyes (jaundice)
- an enlarged liver (hepatomegaly)
- stomach swelling or bloating (ascites)
- high blood pressure in the liver (portal hypertension)
- body or leg swelling (edema)
- blood in vomit (rare symptom)
Budd-Chiari syndrome is rare. It usually occurs along with a blood disorder.
There are several causes of Budd-Chiari syndrome. In many cases, the exact cause is not known. Sometimes other liver conditions like cirrhosis can trigger Budd-Chiari syndrome.
Most people with this syndrome have an underlying health condition that causes too much blood clotting.
Blood disorders that can lead to Budd-Chiari syndrome include:
- sickle cell disease (blood cells are shaped like crescents instead of being round)
- polycythemia vera (too many red blood cells)
- thrombophilia (too much clotting)
- myelodysplastic syndrome (bone marrow disorder)
Adult women are at higher risk for Budd-Chiari if they use birth control pills. In some cases pregnancy can lead to this syndrome, which may happen after delivery.
Other causes include:
- inflammatory disorders
- immunosuppressant drugs
- liver cancer and other cancers
- liver trauma or injury
- blockages or webbing in other large veins (like the inferior vena cava)
- vein inflammation (phlebitis)
- infections (tuberculosis, syphilis, aspergillosis)
- Behcet diease (autoimmune disorder)
- vitamin C deficiency
- protein S deficiency (affects blood clotting)
Budd-Chiari can lead to several liver complications and problems with other body organs and systems.
- liver scarring (fibrosis)
- low liver function
- high blood pressure (hypertension)
- gallbladder problems
- digestive problems
- kidney problems
In serious cases, Budd-Chiari syndrome can lead to liver disease or liver failure.
When to see a doctor
- See your healthcare provider if you have any symptoms or signs of liver damage, such as stomach or right side pain, yellowing of skin and eyes, bloating or swelling in the stomach, legs or anywhere in the body.
- If you have any medical history of a blood condition, or if a blood condition runs in your family, ask your healthcare provider for a complete checkup.
- If you have a blood condition, ask your healthcare provider about the best way to manage it. Take all your medications exactly as prescribed.
Budd-Chiari syndrome is mainly diagnosed after a physical exam. Your healthcare provider finds that the liver is bigger than normal, or there’s unusual swelling in the body.
Your healthcare provider will look at your liver with scans to check its size and to check for any blockages in the liver veins.
Scans and tests that may be used include:
A procedure called venography may be performed if imaging tests have conflicting results and to help your healthcare provider determine the best way to plan your treatment.
During this procedure, a tiny tube or catheter is passed through the veins into the liver. The catheter measures blood pressure inside the liver.
If confirming the diagnosis is difficult, a liver biopsy may be performed. However, due to an increased risk for bleeding, biopsies are not preformed routinely.
During a liver biopsy, the area will be numbed or you’ll be asleep for hte procedure.
A hollow needle is used to remove a tiny piece of the liver. The liver sample is examined in a lab to look for signs of Budd-Chiari syndrome. However, it’s important to note that a biopsy is generally not required for diagnosis.
Budd-Chiari syndrome may be treated with medications to dissolve and prevent blood clots in the liver.
Treatment for Budd-Chiari typically begins with your healthcare provider prescribing medications called anticoagulants. These drugs are used to help stop too much blood clotting.
Other medications called fibrinolytic drugs can be prescribed to help dissolve clots in the veins in the liver.
If there’s an underlying blood condition, treating it may help resolve Budd- Chiari syndrome.
In some cases, the syndrome can be managed with medications alone.
In other cases, a person may need a stent or tube put in through the vein to unblock it. A specialist may use scans of the liver to help guide the tubing into the vein.
You’ll need regular checkups and blood tests even if the clots in the liver are fixed.
In more serious cases of Budd-Chiari syndrome, medications and treatment may not work because the liver is too damaged. In these cases, other surgical procedures or a liver transplant may be needed.
What you can do at home
If you’re prescribed medications to help prevent blood clots, you may need to avoid certain foods that prevent anti-clotting drugs from working well. Ask your healthcare provider about the best diet for you.
You may need to avoid or limit some foods that are high in vitamin K, which is a nutrient that helps the body form clots.
Avoid eating or drinking large amounts of:
- Brussel sprouts
- green tea
Check vitamins and supplements for vitamin K.
Also, avoid drinking alcohol and cranberry juice. They can interact with some blood-thinning drugs and raise the risk for bleeding.
Budd-Chiari is a rare liver condition that can be life threatening. Without treatment, this condition can lead to liver failure in some cases.
However, with treatment, the condition can be managed.
Medical studies in Europe show that almost 70 percent of people with Budd- Chiari were successfully treated with stents and other procedures to open up the liver veins.