Introduction:
Occlusion of the hepatic veins and/ or the suprahepatic inferior vena cava.
- Classical triad:
- Abdominal pain
- Ascites
- Hepatomegaly
Aetiology
Primary Budd–Chiari syndrome (75% cases)
- Thrombosis of the hepatic vein (Decreasing order of frequency):
- Polycythemia vera
- Pregnancy
- Postpartum state
- Use of oral contraceptives
- Paroxysmal nocturnal hemoglobinuria
- Hepatocellular carcinoma
- Lupus anticoagulants
Secondary Budd–Chiari syndrome (25% cases)
- Compression of the hepatic vein by an outside structure (e.g. a tumor, abscess, cysts)
Clinical features
Chronic form (common)
- Hepatomegaly
- Abdominal distension
- Portal hypertension
Acute disorder (uncommon)
- Abdominal pain
- Ascites
- Hepatomegaly
- Rapidly progressive hepatic failure
Inferior vena cava block:
- Back veins become prominent, dilated and tortuous with flow from below upwards
Diagnosis
- Doppler ultrasound
- Venography
Management
Restoring the patency of hepatic vein/inferior vena cava
Radiological management:
- Angioplasty
- Stenting
- Trans-jugular intrahepatic portosystemic shunt (TIPS)
Surgical management:
- Mesoatrial shunt
- Mesocaval shunt
Orthotopic liver transplant
End-stage liver disease