Budd–Chiari syndrome

A condition caused by occlusion of the hepatic veins

Budd–Chiari syndrome is a rare condition characterized by the obstruction of the hepatic veins that drain the liver. This obstruction can lead to liver damage, portal hypertension, and ascites. The syndrome is named after the British physician George Budd and the Austrian pathologist Hans Chiari.

Pathophysiology

Diagram of the hepatic veins and inferior vena cava.

The primary cause of Budd–Chiari syndrome is the blockage of the hepatic veins, which can occur due to thrombosis or compression. This blockage impedes the normal outflow of blood from the liver into the inferior vena cava, leading to increased pressure in the liver and subsequent liver damage. The increased pressure can cause the liver to become engorged with blood, leading to hepatomegaly and potentially liver failure.

Causes

The causes of Budd–Chiari syndrome can be classified into primary and secondary causes:

• Primary causes: These involve intrinsic factors affecting the hepatic veins, such as thrombosis due to hypercoagulable states.
• Secondary causes: These involve external compression of the hepatic veins, such as from a tumor or cyst.

Symptoms

The symptoms of Budd–Chiari syndrome can vary depending on the severity and duration of the obstruction. Common symptoms include:

• Abdominal pain
• Ascites
• Hepatomegaly
• Jaundice
• Splenomegaly

Diagnosis

Diagnosis of Budd–Chiari syndrome typically involves imaging studies such as ultrasound, CT scan, or MRI to visualize the hepatic veins and assess for any blockages. Doppler ultrasound is particularly useful for evaluating blood flow in the hepatic veins.

Treatment

Treatment of Budd–Chiari syndrome aims to relieve the obstruction and manage complications. Options include:

• Anticoagulation therapy to prevent further thrombosis.
• Thrombolysis to dissolve existing clots.
• Angioplasty or stenting to open narrowed veins.
• Surgical shunting to redirect blood flow around the obstruction.
• Liver transplantation in cases of severe liver damage.

Prognosis

The prognosis for Budd–Chiari syndrome varies depending on the underlying cause and the effectiveness of treatment. Early diagnosis and intervention can improve outcomes significantly.

Related pages

• Portal hypertension
• Liver disease
• Thrombosis

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