Budd Chiari Syndrome

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Budd-Chiari Syndrome

• occlusion of hepatic vein leading to venous obstruction

Causes

• unknown in ¹/₃ of cases
• hypercoagulability
  • Polycythaemia Rubra Vera
  • Oral Contraceptive Pill
  • Leukaemia
• posterior abdominal wall sarcoma
• renal tumours
• adrenal tumours
• hepatocellular carcinoma
• hepatic infections
• congenital venous webs
• radiotherapy
• trauma

Clinical Features

Acute

• abdominal pain
• nausea
• vomiting
• tender hepatomegaly
• Ascites

Chronic

• hepatomegaly
  • typically caudate lobe
• mild jaundice
• Ascites
• negative hepatojugular reflex
• splenomegaly
  • due to portal hypertension

Histology

• centrilobular congestion
• hepatocyte atrophy

Investigations

• peritoneal aspiration
  • high protein content
• liver biopsy
• Imaging
  • USS
  • CT
  • MRI
    • hepatic vein occlusion
    • diffuse abnormal parenchyma
      • spared caudate lobe
• + identify cause

Differential Diagnosis

• inferior venal cava obstruction
• right-sided Heart Failure
• Constrictive Pericarditis

Treatment

• Ascites
  • peritoneal-jugular (LeVeen shunt) if resistant
• Surgical
  • resection of webs
  • portocaval / splenorenal anastomosis
  • Liver Transplantation

Prognosis

• can survive for years

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