What are the other Names for this Condition? (Also known as/Synonyms)

• Hepatic Veno-Occlusive Disease
• Hepatic Venous Outflow Obstruction
• HVT (Hepatic Vein Thrombosis) 

What is Budd-Chiari Syndrome? (Definition/Background Information)

• Budd-Chiari Syndrome is a rare liver disorder, usually seen at an incident rate of 1 in a million. It is also known as Hepatic Vein Thrombosis (HVT)
• In this condition, there is an obstruction to blood flowing out of the liver in the hepatic veins. This results in the congestion of the liver, leading to multiple signs and symptoms
• Budd-Chiari Syndrome typically occurs in individuals, who are predisposed to blood clots
• Common clinical features include abdominal pain, jaundice, abdominal distention, enlarged liver, elevated liver enzymes, and change in metal status
• It is usually treated through medications, by placing shunts, or performing certain surgical procedures, to relieve obstruction. In some cases, blood thinners may also be used
• Without proper treatment, Budd-Chiari Syndrome can result in a fatal liver failure. It may also affect the kidneys and brain 

Who gets Budd-Chiari Syndrome? (Age and Sex Distribution)

• Budd-Chiari Syndrome is a very rare disorder that commonly affects young to middle-aged adults, who are in their third or fourth decade (between ages 20 and 40 years). However, it can be seen in any age group
• Both males and females are equally affected 

What are the Risk Factors for Budd-Chiari Syndrome? (Predisposing Factors)

The risk factors of Budd-Chiari Syndrome include:

• Any disturbance in the equilibrium among red blood cells, blood vessel lining, and blood flow, results in blood clots. When these blood clots occur in the hepatic veins, it may cause Budd-Chiari Syndrome
• When there are abnormally high number of blood cells, blood becomes more viscous and predisposes to clot formation
• Certain blood clotting disorders can also predispose an individual to blood clots in the hepatic veins
• Pregnancies and post-partum periods (immediately after delivery) are high-risk periods for blood clots
• Membranous webs have been identified in Asian population that may obstruct hepatic veins
• Other clinical conditions that result in chronic inflammation and chronic infection may make an individual vulnerable to viscous blood and formation of blood clots
• When there are tumors in the body, the circulating malignant cells may provoke blood clots. Tumors that are close to the hepatic veins may press on them, resulting in blockage of the veins
• Chemotherapy drugs, like Dacarbazine, certain prescription drugs, like birth control pills, and some herbal medicines, may also cause blood clots, obstructing the flow in hepatic veins

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider. 

What are the Causes of Budd-Chiari Syndrome? (Etiology)

• The causes of Budd-Chiari Syndrome include blood disorders, like:
  • Polycythemia vera - where there are abnormally high number of red blood cells)
  • Paroxysmal nocturnal hemoglobinuria - a condition resulting in destruction of the red blood cells)
  • Essential thrombocytosis - excess number of platelets in circulation
  • Antiphospholipid antibody syndrome - a blood clotting disorder resulting from antibodies against the cell membrane
• Other blood clotting disorders, like factor V Leiden mutation, protein C and S deficiency, and anti-thrombin III deficiency, can also cause Budd-Chiari Syndrome
• Excess estrogen and progesterone during pregnancy, or hormone replacement therapy, can increase the tendency to cause blood clots
• Infections, like tuberculosis, aspergillosis, syphilis, hydatid cysts (due to tapeworm infection), and amebic abscesses
• Malignancies, like liver cancer, kidney tumors, or adrenal tumors, can press on the hepatic veins causing the symptoms related to Budd-Chiari Syndrome
• Chronic autoimmune and inflammatory conditions, like sarcoidosis, systemic lupus erythematosus, or inflammatory bowel disease, may also be responsible for the condition 

What are the Signs and Symptoms of Budd-Chiari Syndrome?

The signs and symptoms of Budd-Chiari Syndrome include:

• Enlarged liver, fluid in the abdomen, and abdominal pain, are the most common symptoms
• There are four clinical variants of Budd-Chiari Syndrome - acute, sub-acute, chronic, and fulminant forms
  • Acute and sub-acute forms are usually characterized by jaundice, abdominal swelling, and enlarged liver. Renal failure may also be seen in these forms
  • Chronic form is not associated with jaundice. There is progressive swelling of the abdomen and renal failure is also observed
  • Fulminant form is the most serious of all forms. Along with the clinical features of acute and sub-acute forms, the liver is enlarged and tender. There are also features of multi-organ failure 

How is Budd-Chiari Syndrome Diagnosed?

A diagnosis of Budd-Chiari Syndrome may involve:

• Physical examination with thorough medical history evaluation
• Basic laboratory tests may delineate elevated liver enzymes. Any related blood disorders, like elevated red blood cells or elevated platelets may be identified. Renal failure may also be observed in a few patients, as mentioned above
• An ultrasound scan with color Doppler provides valuable information about the blood flow in hepatic veins. The ultrasound may help delineate the fluid in the abdomen (ascitic fluid). This fluid may be drained and further tests may be performed to establish the underlying cause
• Other imaging studies, like MRI of the liver and hepatic venography, may also be utilized to further elucidate the clinical picture
• Liver biopsy may be indicated in some cases, if the underlying cause is not identified. The pathology may shows congestion of the hepatic veins; fibrosis may also be seen

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Budd-Chiari Syndrome?

The complications of Budd-Chiari Syndrome may include the following:

• Change in mental status, also called as hepatic encephalopathy, may develop in some patients. This occurs as a result of building-up of toxins, like ammonia, which are not cleared by liver any more
• Gastrointestinal bleeding is another complication that can occur as a result of back pressure in the blood vessels. This back pressure is also called as portal hypertension. When portal hypertension worsens, blood vessels can rupture and bleed. These abnormal dilated blood vessels are known as varicose veins
• Kidneys get affected in Budd-Chiari Syndrome as a result of the build-up of toxins and fluid. When kidneys are involved due to this liver disease, it is termed as hepato-renal syndrome 

How is Budd-Chiari Syndrome Treated?

The primary goal of Budd-Chiari Syndrome treatment is to relieve obstruction in the hepatic veins and treat related symptoms.

• Blood thinners, like heparin or warfarin may be used, especially when clotting disorders are identified
• Clot busters (anti-thrombolytic therapy) may be used to break-up the clots in the hepatic veins
• Sometimes, a balloon angioplasty (inflating balloon in the narrowed blood vessels) can help relieve obstruction. A stent may be placed in the narrowed blood vessel to keep it open
• Ascites (fluid in the abdomen) may be drained to help alleviate abdominal distention and discomfort. Low salt diet and water pills may be used to take out the fluid
• Transjugular intrahepatic porto-systemic shunt (TIPS): This procedure may be performed in few of the recalcitrant cases of ascites. In this procedure, a stent is placed through the jugular vein into the hepatic vein
• Liver transplant may be recommended in cases of irreversible liver damage 

How can Budd-Chiari Syndrome be Prevented?

Preventive measures for Budd-Chiari Syndrome may include:

• Ensuring adequate anti-coagulation measures in individuals with risk, such as those with inherited or acquired clotting disorders, should be maintained
• Birth control pills should be avoided in women with risk factors
• Lifelong anti-coagulation measures should be initiated in those with clotting disorders 

What is the Prognosis of Budd-Chiari Syndrome? (Outcomes/Resolutions)

• An appropriate treatment of Budd-Chiari Syndrome improves the prognosis. Younger age groups have more favorable outcomes
• Low Child-Pugh score: A score used to assess the prognosis of liver disease. It comprises an assessment of bilirubin levels, serum albumin levels, prothrombin time, the presence of ascites, and brain damage
• Renal parameters, such as a low serum creatinine, indicates that the kidneys are functioning well
• TIPS procedure has a 5-year survival rate between 38-87%
• Liver transplantation increases the 5-year survival rate by up to 70%

Additional and Relevant Useful Information for Budd-Chiari Syndrome:

• Polycythemia vera is a blood-related disorder, in which there is too much red blood cells being produced by the body

The following article link will help you understand polycythemia vera:

https://www.dovemed.com/diseases-conditions/polycythemia-vera/

• Antiphospholipid syndrome is a condition in which the body produces antibodies against its own tissues. As a result of this, excessive blood clots are formed in both arteries and veins, as well as in the placenta in pregnant women

The following article link will help you understand antiphospholipid syndrome:

http://www.dovemed.com/diseases-conditions/antiphospholipid-syndrome-aps/