Budd-Chiari Syndrome


KEY FACTS

Terminology

  • Hepatic injury due to global or segmental hepatic venous outflow or inferior vena cava (IVC) obstruction

Imaging

  • Multiphasic CT, or MR [± gadoxetate (Eovist) enhancement]

    • Characteristic findings: Nodular regenerative hyperplasia in dysmorphic liver with venous collateral and ascites

    • Hypertrophied caudate lobe with atrophy and necrosis of peripheral liver ("pseudotumor")

    • Intrahepatic and systemic venous collaterals bypass obstructed hepatic veins and IVC

      • Spider web pattern of hepatic venous collaterals on CT, MR, angiography

    • Large regenerative nodules (form of nodular regenerative hyperplasia) are characteristic of chronic Budd-Chiari syndrome (BCS)

      • Imaging and histology are similar to FNH

      • May have peripheral halo and central scar

      • Hypervascularity persists into venous phase, usually without washout

      • Uniform or peripheral delayed retention (bright) on gadoxetate-enhanced MR

  • US

    • Absent, reversed, or flat flow in hepatic veins; reversed flow in IVC on color Doppler US

    • Venovenous collaterals

    • Heterogeneous liver parenchyma

Diagnostic Checklist

  • Do not mistake BCS for cirrhosis

    • Pathogenesis, imaging findings, prognosis, and treatment are very different

  • Do not mistake caudate hypertrophy or large regenerative nodules for hepatocellular carcinoma

  • Check for hypercoagulable conditions (most common cause)

Axial anatomic illustration of Budd-Chiari syndrome demonstrates ascites, venous collaterals
, heterogeneous hepatic parenchyma due to centrilobular necrosis, and hypervascular regenerative nodules
. Note the sparing of the caudate lobe with hypertrophy
as well as the thrombosed inferior vena cava (IVC).

Axial CECT shows caudate hypertrophy, a large caudate collateral vein
, and peripheral atrophy and heterogeneity. The hepatic veins were occluded.

Transverse color Doppler ultrasound of the liver in a 48-year-old woman with known polycythemia vera, RUQ pain, and elevated liver function tests reveals a lack of flow within the right hepatic vein
.

Color Doppler ultrasound in the same patient demonstrates a large intrahepatic collateral vein
bypassing the occluded hepatic veins.

TERMINOLOGY

Abbreviations

  • Budd-Chiari syndrome (BCS)

Synonyms

  • Hepatic venous outflow obstruction

Definitions

  • Global or segmental hepatic venous outflow obstruction

    • At level of large hepatic veins or suprahepatic segment of inferior vena cava (IVC)

IMAGING

General Features

  • Best diagnostic clue

    • Caudate hypertrophy, peripheral atrophy, ascites, and collateral veins bypassing occluded IVC

  • Location

    • Hepatic veins, IVC, or centrilobular veins

  • Characteristic finding: Nodular regenerative hyperplasia in dysmorphic liver

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