What Is Budd Chiari Syndrome?

Overview 

Budd-Chiari syndrome is a rare but serious condition that occurs when the blood flow through the liver is blocked. The blockage can occur in any of the three major veins that carry blood from the liver: the hepatic vein, the portal vein, or the inferior vena cava. ⁽¹⁾  As the flow is partially blocked, Budd-Chiari Syndrome (BCS) is sometimes also referred to as "an obstructive hepatic venous outflow".⁽¹⁾  

The liver becomes tender to touch, swells, and causes discomfort. Because of the congestion, fluid leaks from the liver into the abdominal cavity. One of the most prevalent symptoms of Budd-Chiari syndrome is ascites, which are the result of a buildup of fluid in the abdomen area. It may seem like a bulge in your tummy and can be uncomfortable, making it difficult to eat normally and breathe. ⁽²⁾ 

Causes of budd chiari syndrome

Budd-Chiari syndrome can be caused by a variety of factors that lead to obstruction of the blood flow through the liver. These include:²,³

1. Blood clots: The most common cause of Budd-Chiari syndrome is a blood clot that forms in one of the veins that carries blood from the liver. This can happen due to a number of factors, including genetic conditions that cause the blood to clot more quickly, pregnancy, the use of birth control pills, and certain medical conditions such as polycythemia vera
2. Tumors: In rare cases, tumors in or around the liver can compress the veins and cause obstruction of the blood flow
3. Infections: Certain infections such as hepatic tuberculosis or fungal infections can cause inflammation and scarring of the liver tissue, leading to obstruction of the veins
4. Autoimmune diseases: Autoimmune diseases such as lupus or rheumatoid arthritis can cause inflammation and scarring of the liver tissue, leading to obstruction of the veins
5. Congenital abnormalities: Some people may be born with abnormalities of the veins in the liver that can lead to Budd-Chiari syndrome
6. Medications: Certain medications such as anabolic steroids or chemotherapy drugs can increase the risk of blood clots and lead to Budd-Chiari syndrome

It's important to note that in many cases, the exact cause of Budd-Chiari syndrome is unknown.²

Signs and symptoms of budd chiari syndrome

The signs and symptoms of Budd-Chiari syndrome can vary depending on the severity of the obstruction and how quickly it develops. The BCS can present  with the following:⁽¹⁾⁽²⁾⁽³⁾ 

1. Abdominal pain: This is the most common symptom of Budd-Chiari syndrome, and it is often located in the upper right quadrant of the abdomen
2. Enlarged liver: An enlarged liver (hepatomegaly) is a common finding in people with Budd-Chiari syndrome
3. Ascites: This is the accumulation of fluid in the abdomen, which can cause swelling and discomfort. 
4. Jaundice
5. Fatigue: Many people with Budd-Chiari syndrome experience fatigue, which can be caused by a variety of factors, including decreased liver function and anemia
6. Nausea and vomiting: These symptoms can occur due to the buildup of toxins in the body as a result of decreased liver function
7. Hepatic encephalopathy: In severe cases, Budd-Chiari syndrome can lead to the buildup of toxins in the brain, which can cause confusion, lethargy, and coma
8. Swelling can sometimes be seen in the legs due to the unusual fluid accumulation  within the tissues

It's important to note that not all people with Budd-Chiari syndrome will experience all of these symptoms, and some may have no symptoms at all.

Management and treatment for budd-chiari syndrome

The aim of the treatment is to keep your liver function stable by maintaining blood flow. Mostly, the treatment of Budd-Chiari Syndrome depends on the underlying cause of the obstruction and the severity of the conditions. Treatment options may include:

1. Anticoagulant (blood-thinning) medications may be used to prevent blood clots and improve the blood flow through the liver¹
2. Diuretics may be used to reduce the buildup of fluids in the body¹
3. Minimally invasive procedures may be used to remove the blockage in the veins. These procedures may include angioplasty, stent placement, or balloon dilation³
4. In rare cases, surgery may be necessary to remove the blockage or create a new pathway for blood flow called transjugular intrahepatic portal systematic stunting (TIPS)³,⁴
5. In severe cases of Budd-Chiari syndrome that lead to liver failure or irreversible liver damage, liver transplantation is necessary³

Diagnosis of budd chiari syndrome

Diagnosis of Chiari syndrome includes:

1. Imaging tests: Imaging tests such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) are used to visualize the liver and veins and look for signs of blockage²
2. Liver function tests are done¹
3. Liver biopsy¹,³
4. Venography: This is a specialized imaging test that involves injecting contrast dye into the veins and using X-rays to visualize blood flow and detect any blockages³

Risk factors

There are several risk factors that may increase the likelihood of developing Budd-Chiari syndrome. These include:

1. Blood disorders: Certain blood disorders, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria (PNH), can increase the risk of blood clots, which can lead to Budd-Chiari syndrome¹
2. Inherited clotting disorders: Some inherited clotting disorders, such as the factor V Leiden mutation, can increase the risk of blood clots and lead to Budd-Chiari syndrome¹
3. The use of hormonal contraceptives, particularly those that contain estrogen, can increase the risk of blood clots and may contribute to the development of Budd-Chiari syndrome¹
4. Pregnancy¹
5. Liver disease: Certain liver diseases, such as cirrhosis or hepatitis, can lead to scarring and blockage of the liver veins, increasing the risk of Budd-Chiari syndrome¹
6. Tumours, particularly liver cancer, most commonly hepatocellular carcinoma(HCC)¹
7. Chronic inflammatory diseases like Behcet's disease, inflammatory bowel disease (IBD), or Sjogren syndrome can lead to BCS¹

Complications

Budd-Chiari syndrome is a rare condition that affects the liver and is characterized by obstruction of the hepatic veins or inferior vena cava. This obstruction can lead to the following complications:²,³

1. Ascites: This is the accumulation of fluid in the abdominal cavity. It occurs due to increased pressure in the liver blood vessels, causing fluid to leak into the abdominal cavity
2. Portal hypertension: The obstruction in the hepatic veins leads to increased pressure in the portal vein, which supplies blood to the liver. This can lead to portal hypertension, a condition that causes the veins in the esophagus and stomach to enlarge and bleed
3. Liver failure
4. Hepatic encephalopathy: This is a condition in which the liver is not able to remove toxins from the blood, leading to a buildup of toxins in the brain. It has the potential to cause confusion, coma, and even death
5. Increases the risk of liver cancer
6. Variceal hemorrhage 

FAQs

How can I prevent budd chiari syndrome?

There is nothing in particular that can be done to prevent Budd-Chiari syndrome, as it can be genetic as well. But by knowing the underlying cause, a proper treatment plan can be made.

How common is budd chiari syndrome?

BCS is a sporadic disease, occurring in 1/100,000 in the general population.

When should I see a doctor?

If you have the following symptoms:

1. Abdominal pain 
2. Swelling of the abdomen
3. Yellowing of the skin and eyes (jaundice)
4. Fatigue
5. Nausea and vomiting
6. Enlarged liver
7. Ascites (accumulation of fluid in the abdomen)
8. Dark urine

In some cases, Budd-Chiari syndrome may not cause any symptoms until it has progressed to an advanced stage. Therefore, if you have any risk factors for Budd-Chiari syndrome, such as a history of blood clots or liver disease, it is important to see a doctor for regular checkups and to discuss any concerns you may have.

Summary

Budd-Chiari syndrome is a rare condition that affects the liver, characterized by the obstruction of the hepatic veins or inferior vena cava. This obstruction can lead to liver damage, liver failure, and other serious complications. Symptoms of Budd-Chiari syndrome may include ascites, jaundice, fatigue, nausea and vomiting, and an enlarged liver. Risk factors for Budd-Chiari syndrome include a history of blood clots or liver disease. Diagnosis is made through physical exams, blood tests, imaging tests, and a liver biopsy. Treatment options include medication, angioplasty or stenting, or liver transplantation in severe cases.