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Budd-Chiari syndrome: causes, symptoms, diagnosis, treatment
Medical expert of the article
Last reviewed: 04.07.2025
Budd-Chiari syndrome is an occlusion with impaired venous outflow through the hepatic veins, which can be localized at the level from the right atrium to small branches of the hepatic veins. The manifestation of this lesion varies from asymptomatic to fulminant liver failure. Diagnosis is based on ultrasound. Treatment of Budd-Chiari syndrome includes symptomatic drug therapy and is aimed at restoring venous patency by thrombolysis, decompression bypass, and long-term anticoagulant therapy.
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What causes Budd-Chiari syndrome?
Occlusion of small branches of the hepatic veins is associated with venous occlusive diseases. In Western countries, the main cause of Budd-Chiari syndrome is thrombosis of the inferior vena cava entry of the hepatic veins. The main etiologies include thrombotic coagulopathy (eg, protein C or S deficiency, antithrombin III, pregnancy, oral contraceptive use), hematological disorders (eg, polycythemia, paroxysmal nocturnal hemoglobinopathy, myeloproliferative disorders), inflammatory bowel disease, connective tissue diseases, and trauma. Other causes include infections (eg, hydatid cysts, amebiasis) and tumor invasion of the hepatic veins (eg, hepatocellular carcinoma or renal cell carcinoma). The cause of venous obstruction is often unknown. In Asia and South Africa, the cause is often membranous obstruction of the inferior vena cava above the liver, probably due to recanalization of a thrombus formed in adulthood or a malformation in childhood.
The consequences of venous occlusion are ascites, portal hypertension and hypersplenism.
Symptoms of Budd-Chiari Syndrome
Manifestation of the syndrome varies from asymptomatic to fulminant development of liver failure or cirrhosis. Acute occlusion (classic Budd-Chiari syndrome) is manifested by pain in the right upper quadrant of the abdomen, nausea, vomiting, moderate jaundice, enlarged and painful liver, ascites. With complete occlusion of the inferior vena cava, edema of the abdominal wall and lower extremities develops with pronounced dilation of the superficial veins of the abdomen from the pelvis to the costal arch. Subacute course (< 6 months) is manifested by hepatomegaly, coagulopathy, ascites, splenomegaly, bleeding from varicose veins and hepatorenal syndrome. In most cases, a chronic process (> 6 months) is observed, accompanied by fatigue, the presence of tortuous superficial abdominal veins, and in some patients, bleeding from varices, ascites and decompensated liver cirrhosis.
Where does it hurt?
Diagnosis of Budd-Chiari syndrome
Budd-Chiari syndrome is suspected when hepatomegaly, ascites, liver failure or cirrhosis develop and when abnormal liver function tests are combined with thrombosis risk factors. Doppler ultrasound results demonstrate blood flow disturbances and occlusion areas. Indications for CT and MRI are uninformative ultrasound scans. If surgery is planned, angiography is necessary. Laboratory tests are not diagnostic, but they help in assessing the functional state of the liver.
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Prognosis and treatment of Budd-Chiari syndrome
In most cases, patients with complete venous occlusion die of liver failure within 3 years. With incomplete occlusion, the course of the disease is variable.
Treatment of Budd-Chiari syndrome includes symptomatic therapy of complications (eg, ascites, liver failure) and decompression. Thrombolysis allows lysing acute thrombi and reducing hepatic congestion. In case of membranous obstruction of the vena cava or stenosis of the hepatic veins, outflow is restored using percutaneous transluminal balloon angioplasty with intraluminal stenting. Intrahepatic transjugular stenting and many surgical bypass techniques also provide decompression. In case of encephalopathy, bypass is usually not used due to the progression of liver failure and impairment of the synthetic function of the liver. In addition, bypass is associated with the risk of thrombosis, especially in hematological disorders. Long-term anticoagulant therapy is often necessary to prevent relapse. To save the life of a patient with fulminant forms of the disease or decompensated liver cirrhosis, a liver transplant can be performed.