Budd-Chiari Syndrome: Spectrum of Imaging Findings
Giuseppe Brancatelli1,2,3,
Valérie Vilgrain4,
Michael P. Federle3,
Antoine Hakime4,
Roberto Lagalla2,
Riccardo Iannaccone5 and
Dominique Valla6
1 Sezione di Radiologia, Ospedale Specializzato in Gastroenterologia, Saverio de
Bellis, IRCCS, Castellana Grotte, Italy.
2 Istituto di Radiologia, Università di Palermo, Via Villaermosa 29,
90139 Palermo, Italy.
3 Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh,
PA, 15213.
4 Service de Radiologie, Hopital Beaujon, Clichy 92118, France.
5 Istituto di Radiologia, Università di Roma La Sapienza, Policlinico
Umberto I, Roma 00161, Italy.
6 Service de Hepatologie, Hopital Beaujon, Clichy 92118, France.
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Fig. 1A —49-year-old woman with Budd-Chiari syndrome. 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.
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Fig. 1B —49-year-old woman with Budd-Chiari syndrome. 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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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.
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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.
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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).
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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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Fig. 6A —71-year-old woman with chronic Budd-Chiari syndrome, inferior vena
caval calcification, and portal vein occlusion. 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.
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Fig. 6B —71-year-old woman with chronic Budd-Chiari syndrome, inferior vena
caval calcification, and portal vein occlusion. Contrast-enhanced transverse
CT scan obtained during portal phase shows homogeneous enhancement of
liver.
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Fig. 6C —71-year-old woman with chronic Budd-Chiari syndrome, inferior vena
caval calcification, and portal vein occlusion. 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.
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Fig. 7A —22-year-old woman with chronic Budd-Chiari syndrome and portal vein
occlusion. 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.
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Fig. 7B —22-year-old woman with chronic Budd-Chiari syndrome and portal vein
occlusion. 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.
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Fig. 7C —22-year-old woman with chronic Budd-Chiari syndrome and portal vein
occlusion. 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.
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Fig. 8A —39-year-old woman with Budd-Chiari syndrome. Contrast-enhanced
transverse CT scan obtained during portal phase shows development of
intrahepatic (black arrow) and extrahepatic (white arrow)
subcutaneous collateral vessels after hepatic venous thrombosis. Ascites and
dilatation of azygos vein (arrowhead) are evident.
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Fig. 8B —39-year-old woman with Budd-Chiari syndrome. Contrast-enhanced
transverse CT scan caudal in relation to A obtained during portal phase
shows multiple hypervascular nodules resembling focal nodular hyperplasia.
Largest nodule exhibits central scar (arrow).
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Fig. 9 —25-year-old woman with Budd-Chiari syndrome. Contrast-enhanced
coronal maximumintensity-projection CT scan obtained during portal phase shows
patent inferior vena cava with extrinsic compression (arrow) of
enlarged caudate lobe (C).
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Fig. 10A —43-year-old woman with Budd-Chiari syndrome. 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.
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Fig. 10B —43-year-old woman with Budd-Chiari syndrome. Gadolinium-enhanced
T1-weighted MR image obtained during portal phase shows straight demarcation
(arrows) between normally perfused central portion of liver and
hypoperfused periphery.
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Fig. 11A —77-year-old man with Budd-Chiari syndrome and typical large
regenerative nodules. Gadolinium-enhanced T1-weighted MR image obtained during
arterial phase shows numerous homogeneous hyperintense lesions
(arrows) in liver parenchyma.
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Fig. 11B —77-year-old man with Budd-Chiari syndrome and typical large
regenerative nodules. 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.
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Fig. 12 —42-year-old woman with Budd-Chiari syndrome. Hepatic venogram
obtained with selective catheterization of hepatic vein shows spiderweb
pattern. Because of hepatic vein obstruction, collateral channels developed
within liver.
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Fig. 13 —17-year-old girl with Budd-Chiari syndrome. A,
Gadolinium-enhanced T1-weighted MR image obtained during arterial phase.
Hyperintense structures represent portal venules (arrows), which are
visible because of postsinusoidal portal hypertension. B,
Gadolinium-enhanced T1-weighted MR image caudal in relation to A
obtained during portal phase better shows 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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Fig. 14A —35-year-old man with Budd-Chiari syndrome and large regenerative
nodules over 3-year period. 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.
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Fig. 14B —35-year-old man with Budd-Chiari syndrome and large regenerative
nodules over 3-year period. 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.
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Fig. 14C —35-year-old man with Budd-Chiari syndrome and large regenerative
nodules over 3-year period. 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.
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Fig. 15A —24 year-old woman with Budd-Chiari syndrome and large regenerative
nodules with atypical features. Contrast-enhanced transverse CT scan obtained
during arterial phase shows multiple homogeneous hyperattenuating lesions
(arrows). Intrahepatic collateral vessel (arrowhead) is
evident.
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Fig. 15B —24 year-old woman with Budd-Chiari syndrome and large regenerative
nodules with atypical features. 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.
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Fig. 16A —40-year-old man with chronic asymptomatic Budd-Chiari syndrome and
intrahepatic collateral vessels. 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.
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Fig. 16B —40-year-old man with chronic asymptomatic Budd-Chiari syndrome and
intrahepatic collateral vessels. 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.
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Copyright © 2007 by the American Roentgen Ray Society.
