• Clinical science

Budd-Chiari syndrome

Summary

Budd-Chiari syndrome is a rare condition resulting from hepatic vein obstruction that leads to hepatomegaly, ascites, and abdominal discomfort. It is most commonly due to a thrombotic occlusion secondary to a chronic myeloproliferative neoplasm (e.g., polycythemia vera), but may be caused by other conditions associated with hypercoagulable states. The obstruction of blood flow causes congestion of the liver with subsequent liver cell damage. If left untreated, it may result in progressive liver failure. Doppler ultrasound confirms the diagnosis. Management involves preventing further clotting with anticoagulation therapy, restoring blood flow with radiologic or surgical procedures, and treating the underlying condition.

Etiology

References:[2][3]

Pathophysiology

  • Obstruction (e.g., due to thrombosis or compression) of hepatic veins ↓ blood outflow → hepatic venous congestion → increased sinusoidal pressure, cellular hypoxia, centrilobular necrosis congestive hepatopathy
  • May develop into “nutmeg liver”: Because of ischemia and fatty degeneration, the tissue appears speckled with dark spots.
  • Clinical symptoms depend on the extent of hepatic injury and portal hypertension.

References: [4]

Clinical features

In contrast to congestive heart failure, which can also cause hepatic congestion, Budd-Chiari syndrome does not lead to jugular venous distension.

References:[5][6]

Diagnostics

References:[5][8][6][9]

Treatment

References:[10][6]

Complications

We list the most important complications. The selection is not exhaustive.