An uncommon condition affecting the liver, Budd Chiari syndrome occurs when the veins that drain blood from the liver become blocked or narrowed, causing blood to back up and damage the liver. A variety of complications can result, including liver failure, portal hypertension, and other complications.
The causes of budd chiari syndrome are not fully understood, but some factors that may increase the risk of developing it are:
Blood clotting disorders, such as polycythemia vera, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, or factor V Leiden mutation
Pregnancy or use of oral contraceptives
Liver cancer or other tumors that compress the hepatic veins
Infections, such as tuberculosis, syphilis, or malaria
Trauma or surgery to the abdomen
Inflammatory diseases, such as Behçet’s disease, sarcoidosis, or Crohn’s disease
Congenital abnormalities of the hepatic veins or inferior vena cava
The symptoms of budd chiari syndrome may vary depending on the severity and location of the vein obstruction. Some common symptoms are:
Abdominal pain and swelling
Ascites (fluid accumulation in the abdomen)
Jaundice (yellowing of the skin and eyes)
Varices (enlarged veins) in the esophagus, stomach, or rectum, which may bleed
Hepatic encephalopathy (confusion, drowsiness, or coma due to liver dysfunction)
Splenomegaly (enlarged spleen)
Weight loss
Fatigue
Fever
The diagnosis of budd chiari syndrome is based on the medical history, physical examination, and imaging tests. Some of the tests that may be used are:
Blood tests, to check the liver function, blood clotting factors, and markers of inflammation
Doppler ultrasound, to visualize the blood flow and detect any blockage or narrowing of the hepatic veins
Computed tomography (CT) scan or magnetic resonance imaging (MRI), to provide detailed images of the liver and the veins
Liver biopsy, to obtain a sample of the liver tissue and examine it under a microscope
Angiography, to inject a dye into the veins and take x-rays to show the anatomy and location of the obstruction
Treatment for Budd Chiari syndrome depends on the cause, extent, and severity of the vein obstruction. The main goals are to restore blood flow, prevent complications, and treat the underlying condition.
Anticoagulants, to prevent or dissolve blood clots
Diuretics, to reduce the fluid retention and swelling
Beta blockers, to lower the blood pressure in the portal vein and prevent variceal bleeding
Shunt surgery, to create a bypass between the portal vein and the inferior vena cava, or between the hepatic vein and the right atrium, to relieve the pressure and improve the blood flow
Balloon angioplasty or stent placement, to widen the narrowed or blocked vein and keep it open
Thrombolysis, to inject a clot-busting drug into the vein to dissolve the clot
Transjugular intrahepatic portosystemic shunt (TIPS), to insert a metal tube through the jugular vein into the liver, creating a new channel for the blood to flow
Liver transplantation, to replace the damaged liver with a healthy one from a donor
There is no specific way to prevent budd chiari syndrome, but some general measures that may reduce the risk are:
Avoiding or treating the conditions that may cause blood clots, such as infections, cancers, or inflammatory diseases
Using blood thinners as prescribed by the doctor, if there is a history of blood clotting disorders
Avoiding or limiting the use of oral contraceptives, especially if there are other risk factors
Maintaining a healthy weight and lifestyle, with regular exercise and a balanced diet
Quitting smoking and limiting alcohol intake
Seeking medical attention promptly if any symptoms of budd chiari syndrome occur
