Hepatic vein obstruction (Budd-Chiari syndrome)

General Considerations

Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction, independent of the level or mechanism of obstruction.

-Obstruction can occur anywhere from the small hepatic venous tributaries within the liver parenchyma to the major hepatic venous trunks to the inferior vena cava up to the level of the right atrium.

-The obstruction is not due to cardiac disease, pericardial disease, or sinusoidal obstruction syndrome (veno-occlusive disease).

–The most common reason is thrombosis.

-Causes of Budd-Chiari syndrome: Polycythemia rubra vera, pregnancy, oral contraceptives, coagulation disorders, and factor V Leiden.

Symptoms & signs

Fever, right upper quadrant abdominal pain, anorexia, postprandial bloating, jaundice, lower extremity edema, gastrointestinal bleeding

Diagnosis

-The screening test of choice is contrast-enhanced, color, or pulsed-Doppler abdominal ultrasonography

-The definitive radiographic study to confirm the diagnosis: Hepatic venography

Treatment

All patients with hepatic vein obstruction should be hospitalized.

Treatment goals: prevent the propagation of the clot, prevent and manage the complications, decompress the congested liver and restore the patency of thrombosed veins

Anticoagulation, thrombolytic therapy, percutaneous angioplasty, TIPS

Prognosis

Without treatment, the prognosis is poor