Budd-Chiari Syndrome

 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 Long segmental compression or stenosis of IVC
– Acute phase: Due to diffuse hepatomegaly

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Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Budd-Chiari Syndrome

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