Budd-Chiari Syndrome

Published on 18/07/2015 by admin

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Last modified 22/04/2025

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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 Long segmental compression or stenosis of IVC

– Acute phase: Due to diffuse hepatomegaly
– Chronic phase: Hypertrophy of caudate lobe
image Hepatic arteries

– Acute phase: Narrowing, stretching, bowing
– Chronic phase: Dilated with arterioportal shunts

Imaging Recommendations

• Best imaging tool

image Multiphasic CT or MR

DIFFERENTIAL DIAGNOSIS

Hepatic Cirrhosis

• Hypertrophy: Caudate and lateral segment of left lobe
• Atrophy: Right lobe and medial segment of left lobe
• Portosystemic varices, rather than intrahepatic and body wall collaterals
• Patent hepatic veins and IVC
• Cirrhotic regenerative nodules

image Usually small in size compared to nodules in Budd-Chiari
image Cirrhotic nodules often have increased iron

– Usually hypovascular, decreased signal on T2WI
image Vascular masses in cirrhosis are often hepatocellular carcinoma (HCC); washout on venous phase

PATHOLOGY

General Features

• Etiology

image Primary: Venous outflow membranous obstruction

– Controversial etiology
– Congenital, injury, or infection
– Rarely encountered in western populations
image Secondary: Thrombotic, rarely nonthrombotic

– Obstruction of central and sublobular veins

image Chemotherapy and radiation
– Obstruction of major hepatic veins

image Hypercoagulable states (e.g., oral contraceptives, polycythemia, protein C deficiency)
image This is the dominant cause and type of BCS
– Obstruction of small centrilobular veins (venoocclusive disease)

image Bone marrow transplantation, antineoplastic drugs
– Nonthrombotic: Hepatic and extrahepatic masses
• Embryology/anatomy

image Primary type: Total or incomplete membranous obstruction of hepatic venous outflow
image Deviations of complex embryologic process of IVC

Staging, Grading, & Classification

• 3 classifications based on location

image Type I: Occlusion of IVC ± hepatic veins
image Type II: Occlusion of major hepatic veins ± IVC
image Type III: Occlusion of small centrilobar veins (venoocclusive disease)

Gross Pathologic & Surgical Features

• Acute phase

image Congested enlarged liver with occlusion of hepatic veins and IVC
• Chronic phase

image Shrunken nodular liver; may be cirrhotic
image Hypertrophied caudate lobe, atrophy of other lobes
image Extensive collateral veins

Microscopic Features

• Centrilobular congestion, dilated sinusoids
• Fibrosis, necrosis, cell atrophy

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Acute phase: Rapid onset RUQ pain, tender liver, hypotension
image Chronic phase: RUQ pain, hepatomegaly, splenomegaly, jaundice, ascites, varices
• Lab data

image Acute

– Liver function tests: Mildly to markedly increased
– Hypercoagulable
image Chronic

– Transaminases: Normal or moderately increased
– Albumin and prothrombin: Decreased

Demographics

• Age

image Onset typically occurs in young adulthood
• Gender

image M < F
• Epidemiology

image Primary (congenital-membranous): Common in Japan, India, Israel, South Africa
image Secondary (thrombotic): Most common in western countries, usually due to hypercoagulable state
image Secondary (nonthrombotic): 2nd most common in western countries

Natural History & Prognosis

• Complications

image Acute: Liver failure, emboli from IVC thrombus
image Chronic: Variceal bleeding (cirrhosis), portal hypertension
image Membranous obstruction of IVC

– Complicated by HCC in 20-40% of cases in Japan and South Africa
• Prognosis

image Based on rate/degree of hepatic outflow obstruction

– Mild-moderate obstruction: Good
– Severe obstruction: Poor
image Acute early phase: Good
image Acute late phase: Poor
image Chronic phase: Poor ± treatment

Treatment

• Medical management: Steroids, nutritional therapy, anticoagulants
• Balloon angioplasty, stent insertion for occlusion of IVC and hepatic veins
• Transjugular intrahepatic portosystemic shunt (TIPS)
• Liver transplantation may be curative

DIAGNOSTIC CHECKLIST

Consider

• Distinguish BCS from cirrhosis; do not mistake focal large regenerative nodules of BCS for HCC

Image Interpretation Pearls

• Recognize large benign regenerative nodules and caudate pseudotumor
image
(Left) Axial CECT shows thrombosis of all the major hepatic veins image and a heterogeneous liver.

image
(Right) Axial CECT in same patient shows striking hypertrophy of the caudate lobe but normal enhancement relative to the spleen. The peripheral liver is atrophic and enhances minimally. This is a classic example of the caudate pseudotumor of Budd-Chiari syndrome, in which the caudate is so enlarged and different in attenuation than the remainder of the liver that it might be mistaken for a neoplasm.
image
(Left) Axial arterial phase CECT of a 45-year-old woman with ascites and lower extremity swelling shows 1 of multiple hypervascular masses image.

image
(Right) Arterial phase image in the same patient shows another hypervascular mass image, with a central scar, resembling a focal nodular hyperplasia (FNH). These large (multiacinar) regenerative nodules, also called focal nodular regenerative hyperplasia, occur commonly in BCS and resemble FNH on both imaging and histology.
image
(Left) Axial venous phase CECT in the same patient shows an intrahepatic collateral vein image, a large azygous vein image, and subcutaneous collaterals image bypassing the occluded IVC.

image
(Right) Venous phase CECT in the same patient shows occlusion of the retrohepatic IVC image. The liver is dysmorphic and enhances heterogeneously, especially in the periphery. Ascites is present.
image
(Left) Axial T2WI MR of a 25-year-old man with liver malfunction shows ascites image and a dysmorphic liver, with central hypertrophy and peripheral atrophy.

image
(Right) Axial early arterial phase contrast-enhanced T1WI MR in the same patient shows 2 of several focal nodules image that are hyperintense on this sequence. These are large regenerative nodules, the focal multiacinar form of nodular regenerative hyperplasia, that occur in BCS and other conditions that cause chronic hepatic ischemia.
image
(Left) Axial venous phase T1WI MR in the same patient shows persistent enhancement of a focal regenerative nodule image.

image
(Right) Axial delayed phase enhanced T1WI MR in the same patient shows persistent enhancement of 1 of the nodules image while another shows peripheral ring or halo enhancement image. The IVC is occluded, and large azygous collaterals are seen image.
image
(Left) Film from an inferior vena cavogram shows occlusion of the intrahepatic IVC and numerous collateral veins image, resembling a spider web.

image
(Right) Subsequent film from the same procedure shows deployment of metallic stents within the IVC image and right hepatic vein image following balloon dilation of these vessels. This patient was diagnosed as having BCS due to a hypercoagulable condition, the most common etiology in western countries.
image
(Left) Axial CECT shows numerous collateral veins image bypassing the obstructed IVC image. Ascites is present, and the liver enhances heterogeneously. The central right and caudate lobe enhance normally, and the peripheral right and left lobes enhance poorly. These are typical findings of subacute BCS.

image
(Right) Inferior vena cavography in the same patient demonstrates numerous collateral veins image bypassing the obstructed IVC.
image
(Left) Axial CECT shows massive ascites, heterogeneous “mosaic” enhancement of the liver parenchyma, and marked IVC narrowing image. No patent hepatic veins were identified.

image
(Right) Axial CECT in the same patient shows narrowing of a more caudal section of the IVC image. The liver is heterogeneous but does not yet show substantial atrophy or segmental hypertrophy. These findings are typical of acute BCS.
image
(Left) Axial CECT shows a heterogeneously enhancing liver, ascites, and subcutaneous venous collaterals image. The central liver enhances normally image and is hypertrophied, while the peripheral liver is hypodense and scarred.

image
(Right) Axial gadolinium-enhanced GRE image demonstrates normal enhancement and hypertrophy of the caudate lobe image with peripheral hepatic congestion and atrophy. Note the TIPS artifact image. A short, patent vein image drains the caudate lobe into the IVC.
image
Transverse color Doppler ultrasound shows acute Budd-Chiari syndrome (BCS). Note the reversal of flow in the right hepatic vein image.

image
Transverse color Doppler ultrasound of the middle hepatic vein in the same patient demonstrates acute thrombosis with absence of flow image.
image
Axial CECT of BCS shows marked enhancement of the hypertrophied caudate lobe with diminished peripheral flow image.
image
Color Doppler ultrasound in the same patient reveals prominent caudate lobe collaterals image.
image
Axial CECT shows acute Budd-Chiari due to polycythemia vera. Note the thrombosis of the IVC image and mottled enhancement of the liver.
image
Axial CECT in the same patient shows the level of confluence of the hepatic veins, demonstrating low-density thrombus in the right and left hepatic veins image.
image
Axial CECT shows a large hypervascular mass (regenerative nodule) image within the heterogeneously enhancing dysmorphic liver.
image
Axial CECT on portal venous phase shows multiple hyperdense regenerative nodules image with extensive subcutaneous and intrahepatic collaterals image.
image
Axial CECT shows caudate hypertrophy, peripheral scarring, and heterogeneous enhancement.
image
Hepatic venography shows no patency of hepatic veins. Note the filling of the collateral veins and the spider web pattern of the intrahepatic collateral vessels.
image
Color Doppler ultrasound shows a large “bicolored” intrahepatic collateral vein.
image
Axial CECT in arterial phase shows a dysmorphic liver, subcutaneous collaterals, and ascites. Also note the hypervascular nodules image, the largest of which resembles focal nodular hyperplasia, and a central scar image.
image
Axial CECT in portal venous phase shows less apparent hypervascular nodules image nearly isodense to the liver.

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