Brancatelli et al.

H e p a t o b i l i a r y I m ag i n g • P i c t o r i a l E s s ay
Budd-Chiari Syndrome

Budd-Chiari Syndrome:
Spectrum of Imaging Findings
Giuseppe Brancatelli1,2,3 OBJECTIVE. The objective of our study was to illustrate the imaging findings of Budd-
Valérie Vilgrain4 Chiari syndrome, including CT, MRI, sonographic, and angiographic findings.
Michael P. Federle3 CONCLUSION. The key imaging findings in Budd-Chiari syndrome are occlusion of the
Antoine Hakime4 hepatic veins, inferior vena cava, or both; caudate lobe enlargement; inhomogeneous liver en-
Roberto Lagalla2 hancement; and the presence of intrahepatic collateral vessels and hypervascular nodules.
Awareness of these findings is important for early diagnosis and appropriate treatment.
Riccardo Iannaccone5
Dominique Valla6
he term Budd-Chiari syndrome is tion originating from an endoluminal venous
Brancatelli G, Vilgrain V, Federle MP, et al.
T applied to the clinical manifesta-
tions of hepatic venous outflow
obstruction at any level from the
lesion, such as thrombus (Fig. 1) or a mem-
brane [1]. Budd-Chiari syndrome is consid-
ered secondary when the obstruction of he-
small hepatic veins to the junction of the infe- patic venous outflow results from the
rior vena cava and the right atrium regardless presence in the lumen of material not origi-
of the cause of obstruction [1, 2]. Early diag- nating from the venous system (Fig. 2) or
nosis of Budd-Chiari syndrome is important from extrinsic compression [1] (Fig. 3). Re-
for establishing appropriate treatment. Be- gardless of cause, blockage of hepatic
cause of inhomogeneous distribution of dis- venous outflow prevents normal drainage of
ease in the liver, normal biopsy findings do blood. Therefore portal hypertension and in-
Keywords: CT, Budd-Chiari syndrome, liver disease, MRI
not exclude this entity [1]. Therefore imaging trahepatic and extrahepatic collateral vessels
DOI:10.2214/AJR.05.0168 studies combined with clinical information typically develop in patients with Budd-
are often essential for reaching a definitive di- Chiari syndrome.
Received February 1, 2005; accepted after revision agnosis. Evaluation of occlusion of the he-
October 6, 2005.
patic veins and inferior vena cava, caudate Imaging Findings
1Sezione di Radiologia, Ospedale Specializzato in lobe enlargement, inhomogeneous liver en- The imaging findings of Budd-Chiari syn-
Gastroenterologia, Saverio de Bellis, IRCCS, Castellana hancement, and presence of intrahepatic col- drome are variable and depend on the stage of
Grotte, Italy. lateral vessels and hypervascular nodules is the disease (i.e., acute or chronic).
2Istituto di Radiologia, Università
di Palermo, Via the most important role of imaging examina-
Villaermosa 29, 90139 Palermo, Italy. Address tions of patients with Budd-Chiari syndrome. Sonographic Findings
correspondence to G. Brancatelli (gbranca@yahoo.com). We illustrate the spectrum of imaging find- Conventional gray-scale sonography can
3Department of Radiology, University of Pittsburgh Medical ings in Budd-Chiari syndrome, including CT, show enlargement of the caudate lobe; as-
Center, Pittsburgh, PA, 15213. MR, sonographic, and angiographic findings. cites; splenomegaly; and narrowing, lack of
4Service
visualization, and thrombosis of the hepatic
de Radiologie, Hopital Beaujon, Clichy 92118,
France.
Epidemiologic, Etiologic, and veins. Color Doppler studies show absent or
Pathogenetic Aspects flat flow in the hepatic veins; reversed flow
5Istituto di Radiologia, Università
di Roma La Sapienza, Budd-Chiari syndrome can occur at any in the hepatic veins, inferior vena cava, or
Policlinico Umberto I, Roma 00161, Italy.
age, and it is more common in women. Pre- both; and intrahepatic collateral pathways
6Service de Hepatologie, Hopital Beaujon, Clichy 92118, sentation varies from fulminant signs and [3] (Fig. 4). At the level of the portal vein,
France. symptoms to an asymptomatic condition color Doppler sonography shows slow
WEB recognized fortuitously, depending on the hepatofugal flow (< 11 cm/s). Bargallo et al.
This is a Web exclusive article. temporal nature of the disease (acute, sub- [4] found that visualization of a caudate
AJR 2007; 188:W168–W176
acute, or chronic). With regard to cause, vein ≥ 3 mm in diameter on gray-scale
Budd-Chiari syndrome can be classified into sonography strongly suggests the diagnosis
0361–803X/07/1882–W168
primary or secondary. The primary type is of Budd-Chiari syndrome in the appropriate
© American Roentgen Ray Society caused by hepatic venous outflow obstruc- clinical setting.

W168 AJR:188, February 2007


CT Findings
In acute Budd-Chiari syndrome, the mor-
phologic features of the liver usually are
normal, and occlusion of the hepatic veins
with severe ascites is the typical finding.
The liver exhibits patchy, decreased periph-
eral enhancement caused by portal and sinu-
soidal stasis and stronger enhancement of
the central portion of the liver parenchyma
[5, 6] (Fig. 1). The thrombosed hepatic
veins are hypoattenuating, and the inferior
vena cava is compressed by the enlarged
caudate lobe. Ascites and splenomegaly are
usually present [5, 6].
In subacute or chronic Budd-Chiari syn-
drome, the morphologic changes in the liver
are the result of the type of venous involve-
ment, and portosystemic and intrahepatic col-
lateral vessels are often found. Contrast-en-
hanced CT is useful for depicting regions of
hypoperfused liver parenchyma (Fig. 5). The
diameter of the hepatic artery is usually en-
larged compared with that of the splenic ar-
tery. Chronic thrombosis of the inferior vena
cava can evolve into calcification (Fig. 6).
Moreover, portal vein thrombosis can develop
as the result of underlying thrombophilia and
stagnation of portal flow caused by outflow
block [2] (Fig. 7). Sometimes the only clue to
the diagnosis of Budd-Chiari syndrome is
large (comma-shaped) intrahepatic collateral
vessels with no ascites or major morphologic
changes [1, 2] (Fig. 8).
Chronic Budd-Chiari syndrome is also
characterized by the development of multiple
regenerative nodules, which can be viewed as
a response to a focal loss of portal perfusion
and hyperarterialization in areas with pre-
served hepatic venous outflow [7–11]. These
nodules are usually multiple and have a typi-
cal diameter of 0.5–4.0 cm. On multiphasic
helical CT, regenerative nodules are markedly
and homogeneously hyperattenuating on arte-
rial phase images and remain slightly hyper-
attenuating on portal venous phase images,
allowing differential diagnosis from hepato-
cellular carcinoma. Hepatocellular carcinoma
usually exhibits heterogeneous hyperattenua-
tion in relation to the liver on arterial phase
images and hypoattenuation on unenhanced
and portal venous phase images [11].
Although to our knowledge the role of
MDCT in patients with Budd-Chiari syn-
drome has been evaluated in only one study
[12], it is likely that multiplanar reformation
from submillimeter isotropic voxels will pro-
vide a useful adjunct to the axial plane images
in the near future (Fig. 9).
AJR:188, February 2007
Budd-Chiari Syndrome
MRI Findings
In Budd-Chiari syndrome, MR images ob-
tained with T2-weighted sequences usually
show heterogeneously increased signal inten-
sity in the peripheral portion of the liver [13]
(Fig. 10). Images acquired with T2-weighted
sequences do not depict the hepatic veins
or inferior vena cava. Acquisition of T2*-
weighted gradient-recalled echo sequences is
useful for showing absence of flow in the he-
patic veins and inferior vena cava.
MR images acquired with T1-weighted se-
quences after contrast administration clearly
show occlusion of the hepatic veins, inferior
vena cava, or both. In acute Budd-Chiari syn-
drome, the caudate lobe exhibits normal or in-
creased enhancement with decreased en-
hancement in the periphery of the liver [13]
(Fig. 10). In chronic Budd-Chiari syndrome,
the enhancement is more variable, and such
differences may be more subtle [13].
Regenerative nodules are bright on T1-
weighted MR images and strongly hypervascu-
lar after IV bolus administration of gadolinium
contrast material. The nodules are predomi-
nantly isointense or hypointense relative to the
liver on T2-weighted images [7–11] (Fig. 11).
Therefore, large regenerative nodules in patients
with Budd-Chiari syndrome have features on
MRI that distinguish them from hepatocellular
carcinoma, which is usually hypointense in re-
lation to the liver on T1-weighted images and
hyperintense on T2-weighted images [11].
Multiphase contrast-enhanced 3D MR an-
giography of patients with Budd-Chiari syn-
drome has been described [14, 15] as an effec-
tive, noninvasive approach to the evaluation
of the hepatic artery, portal and hepatic veins,
and inferior vena cava, especially for assess-
ment of vessel patency.
Angiographic Findings
Inferior venacavography or hepatic vena-
cavography shows a spiderweb pattern of col-
lateral vessels pathognomonic of Budd-
Chiari syndrome [3] (Fig. 12). In addition,
angiographic studies usually show the pres-
ence of thrombus within the hepatic veins or
inferior vena cava. Another typical feature of
Budd-Chiari syndrome is long segmental
compression of the inferior vena cava caused
by caudate lobe hypertrophy (Fig. 9). The he-
patic arteries tend to be dilated and associated
with arterioportal shunts.
Pathologic Aspects
At gross pathologic examination, liver af-
fected by acute Budd-Chiari syndrome ap-
pears enlarged and congested by occlusion of
the hepatic veins or inferior vena cava [16]. In
the chronic phase, the liver has a nodular,
shrunken, sometimes cirrhotic appearance
with hypertrophy of the caudate lobe and vari-
able atrophy of the other portions of the liver
[16]. The compensatory hypertrophy of the
caudate lobe is explained by preservation of
its veins draining directly into the inferior
vena cava [16] (Fig. 13). Typical microscopic
features of Budd-Chiari syndrome include
centrilobular congestion, sinusoidal dilata-
tion, fibrosis, necrosis, and cell atrophy [16].
Pathologic examination shows nodules com-
posed of fairly normal or hyperplastic hepato-
cytes, large arteries extending radially from the
center of the nodule, and a central scar in larger
lesions [9–10] (Figs. 8 and 14). Such nodules
can resemble focal nodular hyperplasia, and in
rare cases they can be difficult to differentiate
from hepatocellular carcinoma (Fig. 15). To
our knowledge, there have been no published
cases of large regenerative nodules in Budd-
Chiari syndrome degenerating into malignancy.
Treatment
The therapeutic approach to Budd-Chiari
syndrome should be adapted to disease sever-
ity. If patients with signs of portal hypertension
or of liver failure do not respond to anticoagu-
lation or nutritional therapy, careful follow-up
[17] is mandatory to determine the suitable
time for treatments such as a surgical shunt
(usually portacaval, mesocaval, or mesoatrial)
(Fig. 14), placement of metallic stents in the
inferior vena cava or hepatic vein (Fig. 16),
transjugular portosystemic shunt, and liver
transplantation [1, 2]. In patients with mem-
branous occlusion of the inferior vena cava or
hepatic veins, balloon angioplasty, laser treat-
ment, or stent insertion can be performed. An
alternative treatment is surgical membranot-
omy or membranectomy. For patients who
have undergone stent placement, imaging fol-
low-up is necessary to determine the long-term
success of the procedure [18].
Summary
Budd-Chiari syndrome is a rare clinical en-
tity characterized by hepatic venous outflow
obstruction. Awareness of its imaging find-
ings is important for early diagnosis and ap-
propriate treatment.
Acknowledgments
We thank Marie-Pierre Vullierme and Do-
minique Cazals-Hatem for help with radio-
logic and pathologic interpretation.
W169

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Fig. 1—49-year-old woman with Budd-Chiari syndrome.
A, Unenhanced transverse CT scan shows dysmorphic liver with enlarged left lobe and caudate which has normal attenuation compared with other, low-density portions of
liver. Ascites (a) is evident.
B, Contrast-enhanced transverse CT scan obtained during portal phase shows intense enhancement of caudate lobe and relatively low enhancement of peripheral portions
of liver. Inferior vena cava (IVC) is compressed by enlarged caudate lobe. Thrombosis of left hepatic vein (arrow) is evident.
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Lencioni R. Large regenerative nodules in Budd-
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pathologic correlation. AJR 2002; 178:877–883
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nodules in Budd-Chiari syndrome: radiologic-
pathologic correlation with emphasis on the central
scar. AJR 2002; 178:869–875
10. Cazals-Hatem D, Vilgrain V, Genin P, et al. Arterial
and portal circulation and parenchymal changes in
Budd-Chiari syndrome: a study in 17 explanted liv-
ers. Hepatology 2003; 37:510–519
11. Brancatelli G, Federle MP, Grazioli L, Golfieri R,
Lencioni R. Benign regenerative nodules in Budd-
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12. Shan H, Zhu KS, Xiao XS, et al. Budd-Chiari syn-
drome with occlusion of hepatic vein: multi-slice
spiral CT diagnosis and its clinical significance in
the treatment [in Chinese]. Zhonghua Yi Xue Za Zhi
2005; 85:303–307
13. Noone TC, Semelka RC, Siegelman ES, et al.
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Budd-Chiari syndrome: spectrum of appearances
of acute, subacute, and chronic disease with mag-
netic resonance imaging. J Magn Reson Imaging
2000; 11:44–50
14. Erden A, Erden I, Karayalcin S, Yurdaydin C.
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contrast-enhanced three-dimensional MR angiog-
raphy. AJR 2002; 179:1287–1292
15. Lin J, Chen XH, Zhou KR, et al. Budd-Chiari syn-
drome: diagnosis with three-dimensional contrast-
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16. Wanless IR. Vascular disorders. In MacSween RN,
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17. Ruh J, Malago M, Busch Y, et al. Management
of Budd-Chiari syndrome. Dig Dis Sci 2005;
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18. Weernink EE, Huisman AB, van Baarlen J, ten Napel
CH. Treatment of the Budd-Chiari syndrome by in-
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taneous transluminal angioplasty: the necessity of fol-
low-up. Eur J Gastroenterol Hepatol 1996; 8:85–88
AJR:188, February 2007
 Budd-Chiari Syndrome
Fig. 2—61-year-old man with hepatocellular carcinoma causing Budd-Chiari
syndrome. Contrast-enhanced transverse CT scan obtained during portal phase
shows hepatocellular carcinoma invading and expanding inferior vena cava (vertical
arrow). Note hepatocellular carcinoma satellite lesion (arrowhead) and metastatic
lesion (horizontal arrow) involving right chest wall.
Fig. 4—44-year-old man with Budd-Chiari syndrome and intrahepatic collateral
vessels. Transverse color Doppler sonogram obtained at level of caudate lobe shows
lack of color flow in distal portion of middle hepatic vein. Intrahepatic venovenous
collateral vessel (arrowheads) is alternative pathway for venous return from
proximal patent middle hepatic vein (arrow) to inferior vena cava (IVC).
AJR:188, February 2007
Fig. 3—40-year-old man with iatrogenic variant of Budd-Chiari syndrome 30 days after
receiving liver transplant from live donor. Contrast-enhanced transverse CT scan
obtained during portal phase shows occlusion of branch of middle hepatic vein (arrow)
with congestion, edema, and ischemia of anterior part of right lobe of liver. Sharp
demarcation (arrowheads) is evident between normal and abnormally drained liver.
Fig. 5—43-year-old woman with systemic lupus erythematosus and Budd-Chiari
syndrome with impaired circulation in caudate lobe. Contrast-enhanced transverse CT
scan obtained during portal phase shows enlarged left lobe and caudate lobe, normal
enhancement of ventral portion of caudate lobe, and lack of enhancement of dorsal
portion. Straight line (arrowheads) separates normally from abnormally perfused zones.
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 Brancatelli et al.

A B C
Fig. 6—71-year-old woman with chronic Budd-Chiari syndrome, inferior vena caval calcification, and portal vein occlusion.
A, Contrast-enhanced transverse CT scan obtained during arterial phase shows increased peripheral enhancement of liver in relation to portal vein occlusion (not shown).
Liver exhibits peripheral atrophy with compensatory caudate hypertrophy.
B, Contrast-enhanced transverse CT scan obtained during portal phase shows homogeneous enhancement of liver.
C, Contrast-enhanced transverse CT scan cephalic in relation to B obtained during portal phase shows hepatic venous occlusion and coarse calcification (arrow) within
inferior vena cava caused by chronic thrombosis.

A B C
Fig. 7—22-year-old woman with chronic Budd-Chiari syndrome and portal vein occlusion.
A and B, Gadolinium-enhanced T1-weighted MR image (A) and contrast-enhanced transverse CT scan obtained during portal phase (B) show occlusion of vein (arrow,
A and B) draining caudate lobe.
C, Contrast-enhanced transverse CT scan caudal in relation to B obtained during portal phase shows cavernous transformation of portal vein (arrow) and gallbladder varices
(arrowhead). Ascites is evident. This case is unusual in that caudate lobe has impaired venous drainage with resulting heterogeneous enhancement.

W172 AJR:188, February 2007

 Budd-Chiari Syndrome

A B C
Fig. 8—39-year-old woman with Budd-Chiari syndrome. Fig. 9—25-year-old woman with Budd-Chiari
A, Contrast-enhanced transverse CT scan obtained during portal phase shows development of intrahepatic (black syndrome. Contrast-enhanced coronal maximum-
arrow) and extrahepatic (white arrow) subcutaneous collateral vessels after hepatic venous thrombosis. Ascites intensity-projection CT scan obtained during portal
and dilatation of azygos vein (arrowhead) are evident. phase shows patent inferior vena cava with extrinsic
B, Contrast-enhanced transverse CT scan caudal in relation to A obtained during portal phase shows multiple compression (arrow) of enlarged caudate lobe (C).
hypervascular nodules resembling focal nodular hyperplasia. Largest nodule exhibits central scar (arrow).

A B

Fig. 10—43-year-old woman with Budd-Chiari syndrome.

A, Contrast-enhanced transverse CT scan obtained during portal phase shows
caudate lobe hypertrophy and left lobe atrophy. Central portion of liver is enhanced,
and liver periphery is hypoperfused. More cephalic section (not shown) showed
hepatic veins as areas of hypoattenuation due to thrombosis.
B, Gadolinium-enhanced T1-weighted MR image obtained during portal phase
shows straight demarcation (arrows) between normally perfused central portion of
liver and hypoperfused periphery.
C, T2-weighted fat-saturated MR image shows hyperintensity of liver periphery.
Ascites and splenomegaly are evident.
C

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 Brancatelli et al.

A B
Fig. 11—77-year-old man with Budd-Chiari syndrome and typical large regenerative nodules.
A, Gadolinium-enhanced T1-weighted MR image obtained during arterial phase shows numerous homogeneous hyperintense lesions (arrows) in liver parenchyma.
B, T2-weighted fat-saturated MR image shows only one nodule (arrow) of many as hypointense lesion. On gross specimen (not shown) diffuse nutmeg pattern caused by
chronic hepatovenous outflow obstruction was evident along with multiple scattered, round, orange–brown, well-demarcated nodules in both lobes. Histopathologic
examination of nodules showed different-sized hepatocytes with proliferation of bile ductules, and nodules were called large (multiacinar) regenerative nodules.

A B C
Fig. 12—42-year-old woman with Budd-Chiari Fig. 13—17-year-old girl with Budd-Chiari syndrome.
syndrome. Hepatic venogram obtained with selective A, Gadolinium-enhanced T1-weighted MR image obtained during arterial phase. Hyperintense structures
catheterization of hepatic vein shows spiderweb represent portal venules (arrows), which are visible because of postsinusoidal portal hypertension.
pattern. Because of hepatic vein obstruction, collateral B, Gadolinium-enhanced T1-weighted MR image caudal in relation to A obtained during portal phase better shows
channels developed within liver. enlarged caudate lobe that has pseudotumor appearance. Enhancement is still patchy in periphery and
homogeneous in center of liver. Direct venous drainage (arrow) of caudate lobe into inferior vena cava is evident.

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 Budd-Chiari Syndrome

Fig. 14—35-year-old man with Budd-Chiari syndrome

and large regenerative nodules over 3-year period.
A, Contrast-enhanced transverse CT scan obtained
during late hepatic artery phase shows multiple
hypervascular nodules. Largest nodule has central
scar (arrow), resembling focal nodular hyperplasia.
Inferior vena caval stent (S) is evident.
B, Contrast-enhanced transverse CT scan obtained
during late hepatic artery phase 3 years after and at
same level as A shows much less conspicuous
nodules. Retraction of liver capsule (arrow) adjacent to
largest nodule is evident. Mesoatrial surgical shunt (S)
was inserted 2 years before A.
C, Photograph of cut section of fresh explant shows
two nodules. At histologic examination, nodules
exhibited extensive fibrosis, and typical features of
large regenerative nodules were not found. Capsular
retraction (arrow) is evident adjacent to larger
nodule. Relation between decrease in size of
hypervascular nodules and mesoatrial shunt
placement can be hypothesized.

A B

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 Brancatelli et al.

A B
Fig. 15—24 year-old woman with Budd-Chiari syndrome and large regenerative nodules with atypical features.
A, Contrast-enhanced transverse CT scan obtained during arterial phase shows multiple homogeneous hyperattenuating lesions (arrows). Intrahepatic collateral vessel
(arrowhead) is evident.
B, Contrast-enhanced transverse CT scan obtained during delayed phase shows hypoattenuating nodules (arrows) with hyperattenuating ring. Case is unusual in that lesions
are hypoattenuating in relation to surrounding liver parenchyma on delayed phase images. Presence of hyperattenuating capsule is uncommon and would raise concern
about hepatocellular carcinoma.

Fig. 16—40-year-old man with chronic asymptomatic

Budd-Chiari syndrome and intrahepatic collateral
vessels.
A, Gadolinium-enhanced T1-weighted MR image
obtained during portal phase shows liver with
lobulated contours and no flow in hepatic veins.
Inferior vena caval stent (arrow) is evident.
B, Gadolinium-enhanced T1-weighted MR image
cephalic in relation to A obtained during portal phase
shows subcapsular collateral veins (arrowheads)
draining into inferior vena cava, providing route that is
alternative to occluded hepatic veins.
A B

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