Budd–Chiari syndrome

Budd–Chiari syndrome is a rare, life-threatening condition characterized by obstruction of the hepatic veins that drain the liver. It presents with the classical triad of abdominal pain, ascites, and liver enlargement. The syndrome can be fulminant, acute, chronic, or asymptomatic. The prognosis is poor without treatment, but survival rates have improved with the advent of new therapeutic interventions.

Etiology

The cause of Budd–Chiari syndrome is thrombosis in the hepatic veins, which subsequently obstructs them. This can be due to a number of factors, including polycythemia vera, paroxysmal nocturnal hemoglobinuria, pregnancy, oral contraceptive use, and liver cancer. In many cases, the cause is idiopathic.

Symptoms

The symptoms of Budd–Chiari syndrome can vary greatly from person to person. They may include abdominal pain, ascites, hepatomegaly, jaundice, and variceal bleeding. In severe cases, patients may develop liver failure.

Diagnosis

Diagnosis of Budd–Chiari syndrome is typically made through a combination of clinical history, physical examination, and imaging studies. Ultrasound is often the first imaging modality used, but CT scan and MRI can also be helpful. In some cases, a liver biopsy may be necessary.

Treatment

Treatment of Budd–Chiari syndrome is aimed at reducing the hepatic venous pressure gradient, preventing thrombosis, and managing complications. This may involve anticoagulation therapy, thrombolysis, transjugular intrahepatic portosystemic shunt (TIPS), or liver transplantation in severe cases.

Prognosis

The prognosis of Budd–Chiari syndrome is variable and depends on the severity of the disease and the response to treatment. Without treatment, the condition is often fatal, but with appropriate management, survival rates have significantly improved.

See also

• Hepatic vein
• Liver disease
• Thrombosis

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