Pediatric Budd-Chiari Syndrome

• Etiology: Impaired hepatic venous outflow due to obstruction anywhere between hepatic veins to junction of inferior vena cava with right atrium
• Imaging: Diagnosis requires radiologic evidence of thrombosis and stenosis in hepatic vein and or inferior vena cava
• Imaging:
  — Overall see thrombus or stenosis in hepatic vein and or inferior vena cava
  — Hepatomegaly and or splenomegaly
  — Heterogeneous liver due to perfusion abnormality
  — Hemorrhage or infarction
  — Ascites
  — Caudate lobe hypertrophy (50%)
  — Chronic phase has regenerative nodules
• Imaging Doppler US:
  — Spider web of collaterals near hepatic veins ostia and absent hepatic vein
  — Replacement of hepatic veins by fibrous echogenic cord
  — Small subcapsular intrahepatic collaterals (in 80%)
  — Monophasic or absent or reversed or turbulent flow in hepatic veins
  — Slow bi-directional or hepatofugal flow in portal veins
• Imaging CT Acute:
  — Lack of opacification of hepatic veins on delayed phase imaging
  — Patchy central enhancement
  — Delayed or minimal enhancement in periphery and around hepatic veins
• Imaging CT Chronic:
  — Liver atrophy
  — Caudate lobe hypertrophy
  — Intra and extrahepatic collaterals
  — Large and multifocal regenerative nodules
• Imaging MR Acute:
  — Central liver hypointense on T1 and hyperintense on T2
  — Lack of enhancement of hepatic veins
  — Decreased enhancement at periphery even on delayed images
• Imaging MR Sub-acute: Peripheral liver is hypointense on T1 and hyperintense on T2
• Imaging MR Chronic:
  — No difference in central and peripheral signal intensity
  — Hepatic atrophy
  — Regenerative nodules
  — Collaterals
• Imaging MRA: Useful in evaluating inferior vena cava
• DDX:
• Complications:
• Treatment:
• Clinical: Rare in children and more common in adults

Radiology Cases of Budd-Chiari Syndrome