Budd-Chiari Syndrome

Published on 18/07/2015 by admin

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Last modified 18/07/2015

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 May simulate a large neoplasm within caudate lobe

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

image Spider web pattern of hepatic venous collaterals on CT, MR, angiography
• Large regenerative nodules (form of nodular regenerative hyperplasia) are characteristic of chronic BCS

image Imaging and histology similar to FNH
image May have peripheral halo and central scar
image Hypervascularity persists into venous phase without washout
image Uniform or peripheral delayed retention (bright) on gadoxetate-enhanced MR
• Absent, reversed, or flat flow in hepatic veins; reversed flow in IVC on color Doppler US

PATHOLOGY

• Etiology in western populations is usually a hypercoagulable condition

DIAGNOSTIC CHECKLIST

• Do not mistake BCS for cirrhosis

image 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, prior chemotherapy, or bone marrow transplant
image
(Left) Axial anatomic illustration of Budd-Chiari syndrome demonstrates ascites, venous collaterals image, heterogeneous hepatic parenchyma due to centrilobular necrosis, and hypervascular regenerative nodules image. Note the sparing of the caudate lobe with hypertrophy image, as well as the thrombosed IVC.

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

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

TERMINOLOGY

Abbreviations

• Budd-Chiari syndrome (BCS)

Synonyms

• Hepatic venous outflow obstruction

Definitions

• Global or segmental hepatic venous outflow obstruction

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

IMAGING

General Features

• Best diagnostic clue

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

image Hepatic veins, IVC, or centrilobular veins
• Characteristic finding: Nodular regenerative hyperplasia in a dysmorphic liver

CT Findings

• NECT

image Acute phase

– Diffusely hypodense enlarged liver
– Narrowed IVC and hepatic veins; ascites
– Hyperdense IVC and hepatic veins (due to increased attenuation of thrombus)
image Chronic phase

– Heterogeneous hypodensity and atrophy of peripheral liver

image Hypertrophy of caudate lobe, which is spared
image Caudate often greater in diameter than right lobe
image Normal caudate to right lobe is ≤ 0.6 (60%)
– Nonvisualization of IVC and hepatic veins
• CECT

image Acute phase

– Classic “flip-flop” pattern seen

image Early enhancement of caudate lobe and central portion around IVC, decreased peripheral liver enhancement
image Later decreased enhancement centrally with increased enhancement peripherally
– Narrowed hypodense hepatic veins and IVC with hyperdense walls
image Chronic phase

– Total obliteration of IVC and hepatic veins
– Large regenerative nodules: Focal, multiacinar form of nodular regenerative hyperplasia

image Enhancing 1-4 cm hyperdense nodules, ± hypodense ring, ± central scar
image Usually multiple
• CTA

image Hepatic venous outflow obstruction

MR Findings

• T1WI

image Increased intensity of liver centrally with peripheral heterogeneity
image Narrowed or absent hepatic veins and IVC
image Hyperintense regenerative nodules and enlarged caudate lobe
• T2WI

image Nonvisualized hepatic veins and IVC
image Iso- or hypointense regenerative nodules
• T2* GRE

image No demonstrable flow in hepatic veins or IVC
• T1WI C+

image Tumor thrombus (rare) may show enhancement
image Acute phase

– Damaged parenchyma enhances less than surrounding liver
image Congested liver with increased water content

– Peripheral liver enhances < central liver, secondary to ↑ parenchymal pressure, ↓ blood supply
image Chronic phase

– Enhancement is more variable, may be increased
– Nodules: Intense enhancement that persists into venous phase (no washout)
– Uniform or peripheral delayed retention, bright on gadoxetate-enhanced MR
• MRA

image Depicts thrombus and level of venous obstruction

Ultrasonographic Findings

• Grayscale ultrasound

image Hepatic veins narrowed, nonvisualized, or filled with thrombus
image Hypertrophied caudate lobe
• Color Doppler

image Hepatic veins and IVC

– Absent or flat flow in hepatic veins
– Reversed flow in hepatic veins or IVC
– “Bicolored” hepatic veins due to intrahepatic collateral pathways
– Sensitivity: 87.5%
image Portal vein

– Slow hepatofugal flow ≤ 11 cm/s
image Hepatic artery: Resistive index ≥ 0.75

Angiographic Findings

• Inferior venacavography or hepatic venacavography

image Spider web pattern of hepatic venous collaterals
image Thrombus in hepatic veins or IVC
image 

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