Budd-Chiari Syndrome

Causes

Budd-Chiari syndrome is obstruction of hepatic venous outflow that originates anywhere from the small hepatic veins inside the liver to the inferior vena cava and right atrium. The most common cause is a clot obstructing the hepatic veins and the adjacent inferior vena cava. Clots commonly result from the following:

• Thrombotic conditions
• Hematologic disorders
• Inflammatory bowel disease
• Connective tissue disorders
• Trauma
• Infection
• Tumor invasion of the hepatic vein

Symptoms and Signs

Manifestations range from none (asymptomatic) to fulminant liver failure or cirrhosis. Symptoms vary depending on whether the obstruction occurs acutely or over time. Acute obstruction (in about 20%) causes fatigue, right upper quadrant pain, nausea, vomiting, mild jaundice, tender hepatomegaly, and ascites. It typically occurs during pregnancy.

Diagnosis

• Clinical evaluation and liver function tests
• Vascular imaging

Prognosis

Without treatment, most patients with complete venous obstruction die of liver failure within 3 to 5 years. For patients with incomplete obstruction, the course varies.

Treatment

• Supportive care
• Restoration and maintenance of adequate venous outflow