Budd-Chiari Syndrome (BCS)

Budd-Chiari Syndrome (BCS) is a rare condition associated with obstruction of two or more major hepatic veins leading to hepatic venous outflow obstruction.

Etiology

Hepatic Venous Outflow obstruction in Budd-Chiari Syndrome can be due to-

• Thrombosis of Hepatic veins
• Compression / Invasion of Hepatic veins

Thrombosis of Hepatic veins 

The majority of cases with Budd-Chiari Syndrome are associated with hypercoagulable states causing thrombosis in hepatic veins. The most important causes are the following-

• Polycythemia Vera/ Myeloproliferative Disorders – nearly 50% of cases with Budd-Chiari Syndrome are associated with myeloproliferative disorders (Polycythemia vera is most common).
• OCP/ Pregnancy / Post-partum period – all are hypercoagulable states
• Antiphospholipid antibody syndrome
• Paroxysmal Nocturnal hemoglobinuria (PNH)
• Clotting abnormalities

Compression/Invasion of the Hepatic Vein

• Neoplasm- Intrabdominal malignancies (HCC and RCC are most common)
• Space Occupying lesions Liver – Hepatic cyst, aspergillosis

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Pathophysiology

Obstruction of two or more hepatic veins →hepatic venous outflow obstruction→ impaired blood drainage to inferior vena cava→ increased backflow of the blood →hepatic venous congestion

Hepatic venous congestion→ hepatomegaly →stretching of liver capsule →abdominal pain and tender hepatomegaly

Hepatic venous congestion→ increases sinusoidal pressure → sinusoidal dilation →reduced hepatic blood flow→ cellular hypoxia→ centrilobular necrosis and peripheral fatty degeneration (Nutmeg liver) →liver dysfunction →jaundice, hyperbilirubinemia and increased liver enzyme→ liver failure if left untreated

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Hepatic venous congestion may cause portal hypertension that can lead to

• splenomegaly
• ascites
• increased perfusion of portocaval shunts causing esophageal varices and caput medusae

 

Histopathology

• Centrilobular necrosis and peripheral fatty degeneration give the liver a “Nutmeg appearance”
• Sinusoidal dilation

Clinical features 

• Abdominal pain, tender hepatomegaly (due to stretching of liver capsule), and ascites (the triad of Budd-Chiari Syndrome)
• Splenomegaly
• Jaundice
• Esophageal varices
• Caput Medusae

Diagnosis 

• Clinical presentation and history
• Doppler Ultrasonography (initial investigation) – to visualize thrombosis or hepatic vein obstruction.
• CT/MRI – Confirmatory test (non-invasive modality of choice)
• Venography- gold standard test, reserved for cases with a high level of suspicion where other modalities have failed

Treatment 

• Treat underlying cause
• Prevent propagation of the thrombus – use anticoagulants
• Restore blood flow by localized thrombolysis or angioplasty and Stenting
• TIPS (Trans jugular intrahepatic portosystemic shunt)
• Liver transplant in case of hepatic failure

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