Budd Chiari Syndrome: Radiology, Causes, Treatment, Diet

Budd-Chiari Syndrome is a rare condition that affects approximately 1 in every 100,000 people in the global population. The veins that carry blood out of the liver become blocked, causing a backup of blood flow. 

Some patients may experience no symptoms at all. However, most of them commonly experience abdominal pain, weakness, nausea, and jaundice.

If not managed on time, Budd Chiari syndrome can result in life-threatening complications. Read below to know more about Budd-Chiari Syndrome, its definition, symptoms, causes, types, diagnosis, prevention, and treatment.

Budd-Chiari Syndrome is a rare liver condition that occurs when the blood flow from the liver is blocked or restricted. This blockage typically happens due to the narrowing or clotting of the veins that carry blood away from the liver. 

The liver plays a crucial role in filtering and processing blood, so when the veins are blocked, blood backs up and accumulates in the liver. It can lead to liver damage, scarring, and impaired liver function. 

As a result, blood builds up in the liver, causing swelling, congestion, and potential damage to the organ.

Budd-Chiari Syndrome can be classified into two types based on the origin of the obstruction. The classification depends on whether the obstruction originates from within the veins or is caused by factors outside the veins.

1. Primary Budd-Chiari Syndrome: When the blockage occurs within the veins themselves, typically due to the presence of a blood clot, it is referred to as primary Budd-Chiari Syndrome.
2. Secondary Budd-Chiari Syndrome: On the other hand, if the obstruction is caused by external factors like compression from surrounding structures or invasion by a tumor, it is called Secondary Budd-Chiari Syndrome. 

Budd-Chiari Syndrome can be categorized into different forms based on their severity and how they develop over time:

1. Subacute Form: It is the most common type. This form lasts months to years and shows rapid development of symptoms such as abdominal pain, ascites, enlarged liver, jaundice, and possible kidney failure.
2. Acute Form: This form typically develops within six months and presents with similar symptoms as the fulminant form but with a less severe progression.
3. Chronic Form: This form develops slowly over months to years and is characterized by progressive fluid buildup in the abdomen (ascites).
   
   Jaundice may not be present, but about half of the patients may also have kidney problems. Laboratory findings are not specific when ascites develop, but there is a decrease in serum albumin levels.
4. Fulminant Form: It is rarest type. This form has a sudden onset of severe liver failure.
   
   Symptoms include fluid buildup in the abdomen (ascites), a tender and enlarged liver, yellowing of the skin and eyes (jaundice), and kidney failure. Blood tests show high levels of liver enzymes.

Most individuals diagnosed with Budd-Chiari Syndrome commonly experience three main symptoms. These are abdominal pain, ascites (fluid buildup in the abdomen), and hepatomegaly (enlarged liver), which are commonly are Budd-Chiari Syndrome Triad. The primary symptoms are as follows:

1. Abdominal Pain: Many individuals with Budd-Chiari Syndrome experience persistent or recurrent abdominal pain, ranging from mild to severe.
   
   The pain is typically located in the upper right side of the abdomen and may be accompanied by a feeling of fullness or discomfort.
2. Ascites: Liver congestion and increased pressure in the veins can lead to the development of ascites (fluid buildup). This can cause abdominal swelling, tightness, and discomfort.
3. Hepatomegaly: The liver is enlarged in this condition, which is a common finding in Budd-Chiari Syndrome. The liver may be tender to touch and can extend beyond its normal size. This enlargement occurs due to the obstruction of blood flow in the liver.
4. Jaundice: It is characterized by yellowing of the skin and eyes. In Budd-Chiari Syndrome, the impaired blood flow through the liver can lead to the accumulation of bilirubin, resulting in jaundice.
5. Fatigue and Weakness: Many individuals experience fatigue and weakness, which can significantly impact their daily activities. These symptoms can be attributed to compromised liver function and decreased production of essential proteins and nutrients.

Budd-Chiari Syndrome can also present with additional symptoms. These symptoms can vary depending on the severity and progression of the disease.

1. Nausea
2. Vomiting 
3. Loss of appetite 
4. Weight loss
5. Change in urine colour (dark colour)

Budd Chiari Syndrome can occur due to a variety of causes. In some cases, the cause can also be unknown. The common causes are:

1. Blood Clots (Thrombosis): Budd-Chiari Syndrome can occur when blood clots form within the veins.
   
   These veins carry blood from the liver (hepatic veins) or the large vein that brings blood from the lower body to the liver (inferior vena cava).
   
   These clots can obstruct or partially block the blood flow, leading to the development of the syndrome.
2. Myeloproliferative Disorders: Polycythemia vera, thrombocythemia, or primary myelofibrosis are conditions where the bone marrow produces excessive amounts of blood cells.
   
   This abnormal blood cell production can increase the risk of blood clot formation and contribute to the development of Budd-Chiari Syndrome.
3. Liver Diseases: Certain liver diseases, such as cirrhosis or liver fibrosis, can cause scarring and narrowing of the blood vessels within the liver.
4. Infections: In rare cases, infections such as tuberculosis or syphilis can lead to inflammation and scarring of the blood vessels in the liver, obstructing blood flow and developing Budd-Chiari Syndrome.
5. Autoimmune Disorders:  Autoimmune hepatitis or systemic lupus erythematosus can cause inflammation and damage the liver and blood vessels.
   
   This inflammation and damage can increase the risk of blood clot formation.
6. Other Factors: Factors that may contribute to the development of Budd-Chiari Syndrome include certain medications, genetic or inherited conditions affecting blood clotting.
   
   External factors include such as trauma or compression of the blood vessels.

Taking preventive measures can help reduce the risk of developing Budd-Chiari Syndrome and promote a healthier liver. Few measures are:

1. Maintain a Healthy Lifestyle: Adopting a healthy lifestyle that includes regular exercise, a balanced diet, and maintaining a healthy weight can help reduce the risk of Budd-Chiari Syndrome.
2. Stay Hydrated: Drinking adequate water and staying hydrated can help promote healthy blood flow and reduce the risk of blood clot formation.
3. Manage Underlying Conditions: If you have underlying conditions such as myeloproliferative disorders or autoimmune disorders, treating these conditions can help reduce the risk.
4. Regular Medical Check-ups: These can help detect and manage any underlying conditions or risk factors that may contribute to Budd-Chiari Syndrome at an early stage.

It is crucial to consider the possibility of Budd-Chiari Syndrome in individuals who have symptoms or a history of liver disease, whether it is acute or chronic.

Regardless of the presence of symptoms, being aware of this condition is essential as it allows for timely diagnosis and appropriate management. The diagnosis includes:

1. Blood or Biochemical Abnormalities: Blood tests often show mild elevations in liver enzymes (ALT and AST) and alkaline phosphatase levels in a subset of patients.
2. Imaging Tests: Doppler ultrasonography is typically the initial test of choice, providing valuable information to confirm the diagnosis. Computed tomography (CT) or magnetic resonance imaging (MRI) may also be used.
   
   In cases where other tests are inconclusive but suspicion remains high, venography, an invasive procedure, can be considered.
3. Diagnostic Paracentesis: Analyzing the fluid accumulated in the abdomen (ascitic fluid) can provide important diagnostic clues.
   
   Elevated protein levels and a low white blood cell count are commonly observed in the chronic form of the syndrome.
4. Liver Biopsy: While not always necessary, a liver biopsy may be performed to gather additional information about the extent of liver damage and to rule out other liver diseases.

How to prepare for the doctor’s consultation?

You can always prepare yourself prior to a doctor’s appointment. Some of the things questions to consider asking are:

1. How can one prevent developing Budd Chiari Syndrome symptoms?
2. Can it be treated without medications?
3. What are the side effects of medications?
4. How long will I have to take these medications?
5. Can it be treated without surgery?
6. What would be the cost of liver transplantation?
7. Is there any other alternative, such as Ayurveda or homoeopathy?

The main goal in treating Budd-Chiari syndrome is to relieve the blockage, prevent the clot from getting worse, protect the liver from further damage, and address any complications that may arise.

Non-Surgical Treatment

These non-surgical procedures can help improve blood flow and alleviate symptoms in Budd-Chiari syndrome. 

1. Medications: Prescribed medications, such as anticoagulants or thrombolytics, help to prevent blood clots or dissolve existing clots.
2. Diuretics: Diuretic medications help to reduce fluid retention and manage symptoms like ascites.
3. Percutaneous Transluminal Angioplasty: A procedure where a catheter with a balloon is inserted into a blood vessel to widen the blocked vein, and a stent may be placed to keep it open.
4. Transjugular Intrahepatic Portosystemic Shunt: TIPS is a procedure in which a small artificial channel is created within the liver to redirect blood flow and reduce portal hypertension.

Surgical Treatment

In some cases of Budd-Chiari Syndrome, surgical intervention may be required to address the underlying causes and alleviate symptoms.

1. Liver Transplantation: In severe cases, when the liver is extensively damaged, a surgical procedure where a healthy liver from a donor is transplanted into the patient, replacing the diseased liver.

Cost of Budd-Chiari Syndrome Surgery

The cost of Budd-Chiari Syndrome surgery in India can vary depending on several factors, including the type of surgery, the hospital or clinic where the procedure is performed, and the location.

The following table shows the cost of different types of Budd-Chiari Syndrome surgery:

You can find more details on the following:

1. Hepatic Vein Stenting Cost in India
2. TIPS Cost in India

Budd Chiari Syndrome is a severe condition that, if not appropriately managed, can lead to life-threatening complications and even death. Complications of Budd-Chiari Syndrome:

1. Portal Hypertension: Budd-Chiari Syndrome can cause high blood pressure in the portal vein, which is responsible for carrying blood to the liver.
   
   This can lead to various complications, such as enlargement of blood vessels in the esophagus and stomach, increased risk of bleeding, and fluid accumulation in the abdomen.
2. Hepatocellular Carcinoma (HCC): Although rare, individuals with Budd-Chiari Syndrome have a small risk of developing liver cancer known as hepatocellular carcinoma.
   
   Regular monitoring and surveillance are important to detect and manage HCC if it occurs.
3. Worsening of Underlying Diseases: In approximately 10% of cases, the underlying diseases that contribute to Budd-Chiari Syndrome.
   
   Myeloproliferative disorders (MPD), can progress and have a negative impact on the prognosis and management of Budd-Chiari Syndrome.
4. Bleeding: Budd-Chiari Syndrome can increase the risk of bleeding, both within the liver and in other organs. Changes in blood flow and clotting can lead to bleeding complications that require medical attention.
5. Thrombosis: In addition to the liver, blood clots can form in other organs, potentially causing blockages and impairing organ function.

When to See a Doctor?

See a doctor if there are any signs that indicate the worsening of the disease. Some of the alarming signs when already diagnosed with Budd Chiari syndrome or have pre-existing coagulation disorders include: 

1. Vomiting blood
2. Severe abdominal pain due to spleen enlargement
3. Swelling of the veins in legs and feet
4. Altered mental status, such as poor concentration, drowsiness, etc.

A balanced diet plays a crucial role in managing Budd-Chiari Syndrome and promoting liver health. Here are some dietary guidelines to consider:

1. Fruits and Vegetables: Include a variety of colourful fruits and vegetables rich in antioxidants, such as berries, citrus fruits, leafy greens, and cruciferous vegetables like broccoli and cauliflower.
2. Whole Grains: Opt for whole grain sources like brown rice, quinoa, whole wheat bread, and oats to provide essential nutrients and fibre.
3. Lean Proteins: Choose lean protein sources like skinless poultry, fish, tofu, legumes, and low-fat dairy products to meet your protein needs without excessive saturated fats.
4. Healthy Fats: Incorporate sources of healthy fats, such as avocados, nuts, seeds, and olive oil, to support liver function and reduce inflammation.
5. Low-Sodium Options: Limit your intake of high-sodium foods like processed snacks, canned soups, and fast food, and opt for low-sodium alternatives to help manage fluid retention.

Budd-Chiari syndrome (BCS) is a rare and serious condition caused by a blockage in the veins that drain blood from the liver. The primary focus in treating BCS is to restore normal blood flow through the liver, regardless of the location of the blockage.

You can get in touch with HexaHealth to know the best treatment options available for Budd-Chiari Syndrome. We can help you know about the condition and be there till the post-surgical care stage.