Abdom Imaging 24:267–271 (1999)
Abdominal
Budd-Chiari syndrome: hepatic venous obstruction by an elevated
diaphragm
P. N. Kim, D. G. Mitchell, E. K. Outwater
Department of Radiology, 1096 Main Building, Thomas Jefferson University Hospital, 132 South 10th Street, Philadelphia, PA 19107-5244,
USA
Received: 13 April 1998/Accepted: 3 June 1998
Abstract
We describe two cases of Budd-Chiari syndrome detected
by magnetic resonance imaging that resulted from com-
pression of the inferior vena cava by an elevated right
hemidiaphragm. Magnetic resonance images demon-
strated elevation of the right hemidiaphragm and medial
deviation of the inferior vena cava with short segmental
narrowing. The hepatic veins and inferior vena cava were
patent but discontinuous. Hepatic venous drainage was
assisted by multiple large intrahepatic collaterals.
Key words: Liver, MR—Hepatic veins, thrombosis—
Hepatic veins, stenosis or obstruction—Venae cavae, ste-
nosis or obstruction—Budd-Chiari syndrome.
Budd-Chiari syndrome involves hepatic venous outflow ob-
struction from the sinusoidal bed of the liver, resulting in
portal hypertension, ascites, and progressive hepatic failure.
The hepatic veins in Budd-Chiari syndrome can occlude at
the small hepatic venules, the major hepatic veins, and the
inferior vena cava (IVC). Each level of obstruction is related
to a different etiology. Occlusion of the major hepatic veins
is, in many instances, secondary to an underlying disease,
and three main causes are hypercoagulable conditions, ma-
lignancy, and miscellaneous [1].
In Budd-Chiari syndrome, some hepatic venous drain-
age is usually preserved for the caudate lobe and the
central regions of the left and right lobes through the
accessory hepatic veins, caudate veins, and other collat-
erals draining into the IVC [2–5]. Compensatory hyper-
trophy of spared areas is typical, especially in the caudate
lobe, which may become massive and take over the
function of the more severely affected liver [1]. Some
Correspondence to: D. G. Mitchell
Imaging
© Springer-Verlag New York Inc. 1999
venous drainage is also preserved in the periphery of the
liver through capsular veins, although this route is usually
not sufficient to prevent peripheral hepatic atrophy in
patients with Budd-Chiari syndrome [6, 7].
We report two cases of Budd-Chiari syndrome in
which elevation of the right hemidiaphragm was the ap-
parent cause of hepatic venous obstruction due to an acute
angle at the junction of the hepatic veins and IVC.
Case reports
During a 5-year period, magnetic resonance (MR) imaging was per-
formed in 20 patients with Budd-Chiari syndrome. In two patients, the
right hemidiaphragm was markedly elevated, thereby obstructing the
junction of the hepatic veins with the IVC. In addition, the intrahepatic
portion of the IVC was medially deviated with a short segmental
narrowing. Both patients had an extensive network of intrahepatic
venous collaterals. Neither patient had clinical signs of portal hyperten-
sion, other significant liver disease, or lower extremity edema.
Case 1
A 50-year-old woman with a history of a severe motor vehicle accident
20 years before was transferred to evaluate a parenchymal abnormality
of the right lobe of the liver, incidentally seen by chest computed
tomography (CT). Laboratory values including coagulation profile were
normal except for total bilirubin of 1.2 mg/dL. CT showed mottled
enhancement throughout the right lobe of the liver and hypertrophy of
the lateral segment of the left lobe. There was a large hepatic vein in the
hepatic dome region. The right accessory hepatic veins were dilated
caudal to the portal vein. The portal and hepatic veins and the IVC were
patent. MR also demonstrated severe atrophy of the medial segment of
the left lobe, a lesser degree of atrophy involving the anterior right lobe,
and compensatory hypertrophy of the posterior right lobe and the lateral
segment of the left lobe. The right lobe of the liver was abnormally
enhanced with a heterogeneous mosaic appearance, as seen with hepatic
venous obstruction. There were tortuous hepatic venous collaterals
anteriorly, the right hepatic vein drained to an anomalous vein, and the
connection to the IVC was not apparent. The right hemidiaphragm was
markedly elevated, and the IVC was medially deviated with a short
segmental narrowing (Fig. 1).
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Fig. 1. A–C Axial FMPSPGR images (TR/TE 5 150/2.4, NEX 5 1.0) show,
A, a large hepatic vein at the hepatic dome (arrow), most consistent with the
right hepatic vein, draining into an abnormal collateral. B Image obtained 20
mm caudal to that shown in A shows a tortuous collateral vein (white arrows)
in the anterior liver and the right portal vein (black arrow). The IVC is located
anterior to the vertebra. C Image obtained 20 mm caudal to that shown in B
shows severe atrophy of the medial segment of the left lobe of the liver, two
right accessory hepatic veins draining into the IVC, and the main portal vein
(arrowhead). The IVC is dilated and located anterolateral to the vertebra with
a medial beak (long arrow) indicating twisting. A strongly enhanced abnormal
Case 2
A 69-year-old woman was transferred for further evaluation of multiple
cystic lesions in the liver. Laboratory values were normal. CT showed
P. N. Kim et al.: Hepatic venous obstruction by an elevated diaphragm
vein (large arrow) is not connected to the IVC. The azygos and hemiazygos
veins are dilated. D Coronal FMPSPGR image (TR/TE 5 130/2.3, NEX 5
1.0) shows a markedly elevated right hemidiaphragm, hepatomegaly including
hypertrophy of the lateral segment of the liver, and medial displacement of the
intrahepatic IVC with a short segmental narrowing of the IVC (arrow). E Axial
projection image obtained during the portal venous phase shows contrast-filled
portal veins in both lobes and a dilated IVC with a medial beak (arrow). The
hepatic veins are not enhanced. F Axial projection view obtained 2 min later
shows the hepatic and portal venous systems. The hepatic veins are not con-
nected to the IVC, but the accessory hepatic veins are drained into the IVC.
three hypodense lesions in the right lobe of the liver with no contrast
enhancement. The largest lesion in the hepatic dome region had an
irregular margin, suggesting necrosis. MR images showed severe atro-
phy of the posterior segment of the right lobe at the hepatic dome,
P. N. Kim et al.: Hepatic venous obstruction by an elevated diaphragm
Fig. 2. A–D Axial fat-suppressed 3DFT gradient echo images (TR/TE 5
6.8/2.1, flip angle 5 20°, NEX 5 0.5) taken approximately 1 min after
administration of gadolinium chelate. A shows counterclockwise rota-
tion (curved arrow) of a small right (R), middle (M), and left (L) hepatic
veins due to right lobe atrophy and left lobe hypertrophy. A low signal
intensity lesion in the anterior segment suggests necrosis (short black
arrow). The IVC is located anterior to the vertebra. The azygos vein
(long black arrow) is enlarged. B Image obtained 16 mm caudal to that
shown in A shows tortuous intrahepatic collaterals (arrow) in the right
moderate atrophy of the anterior segment of the right lobe, and com-
pensatory hypertrophy of the caudate and central liver. Tortuous intra-
hepatic venous collaterals were demonstrated in the right lobe of the
269
lobe of the liver and a flat IVC. The umbilical portion of the left portal
vein is prominent. C Image obtained 16 mm caudal to that shown in B
shows a small hepatic vein (arrow) from the central liver draining into
the IVC and the normal-sized IVC that is located anterolateral to the
vertebra. D, E Axial projection images shows multiple tortuous intra-
hepatic collaterals in the anterior segment of the right lobe (arrows)
draining into the right or middle hepatic veins. The posterior segment of
the right lobe is atrophied. F Coronal projection image shows an abrupt
narrowing of the IVC at the level of the right hemidiaphragm.
liver, consistent with hepatic venous obstruction. The hepatic veins were
patent, but the right hepatic vein was narrowed, and its drainage into the
IVC was interrupted by acute angulation. The intrahepatic IVC was
270
medially displaced with a short segmental narrowing just below the
level of the elevated right hemidiaphragm (Fig. 2). The right accessory
hepatic veins were enlarged.
Discussion
In most cases of Budd-Chiari syndrome, hepatic venous
outflow is not completely eliminated because a variety of
accessory hepatic veins drain above or below the princi-
pal site of obstruction [2– 4]. Because the caudate lobe
drains directly into the IVC by small caudate veins, the
caudate lobe may hypertrophy as compensation for the
loss of functioning liver tissue, thus taking over the func-
tion of affected hepatic segments [1]. Regions with com-
pletely obstructed hepatic venous outflow must drain
through shunting of the hepatic veins and arteries to the
portal veins, thereby producing regional reversal of portal
venous flow. Thus, these regions of the liver will be
deprived of portal vein supply. Because hepatic regener-
ation, hypertrophy, and atrophy depend in part on the
degree of portal perfusion [8], Budd-Chiari syndrome is
typically associated with peripheral hepatic atrophy and
caudate and central hypertrophy. Because of central hy-
pertrophy of the liver, the portahepatis may be displaced
toward the anterior portion of the liver [2], and the IVC
can be compressed [5].
The disease process often starts in the right superior
segment of the liver and may cause extreme hyperplasia
of the left lobe, thereby producing gross changes in the
shape of the liver [9]. Hypertrophy of the liver may
posteriorly displace the IVC. In our cases, elevation of the
right hemidiaphragm caused acute angulation of the he-
patic venous connection to the IVC, resulting in obstruc-
tion. Hypertrophy of the central liver at the hepatic dome
may have exacerbated the IVC obstruction. The medial
segment of the left lobe was atrophied. The intrahepatic
portion of the IVC was medially deviated with a short
segmental narrowing at the level of the right hemidia-
phragm, consistent with compression by the diaphragm.
One case demonstrated a medial beak from the IVC,
indicating twisting of the IVC.
The majority of Budd-Chiari syndrome is idiopathic
[2]. The syndrome has been associated with hypercoag-
ulable states, neoplasms, trauma, medications, and con-
genital abnormalities [10]. With Budd-Chiari syndrome
due to trauma, the IVC was compressed by hematoma or
enlarged liver [11], or the hepatic veins were obstructed
by hepatic hernia into the thorax [12]. We are not aware
of any prior reports about compression of the hepatic
vein–IVC confluence by an elevated diaphragm. Because
the position of the hepatic venous drainage into the IVC
is fixed by the unaltered position of the heart, elevation of
the liver into the chest interrupts hepatic venous drainage
into the IVC. In case 1, the hypertrophy of the hepatic
dome proximal to the right accessory hepatic veins may
P. N. Kim et al.: Hepatic venous obstruction by an elevated diaphragm
have exacerbated the obstructed hepatic venous outflow,
medially displacing the IVC. Extrinsic compression of the
IVC caused a medial beak of the IVC at the level of the
right hemidiaphragm and dilatation of the extrahepatic
IVC. In case 2, the IVC had an oblique course because the
junction between the right atrium and the IVC was fixed
and the extrahepatic portion of the IVC was posterolat-
erally displaced due to severe central hypertrophy of the
liver. As a result, the IVC was compressed at the level of
the diaphragm. The extensive network of collaterals in
both cases presumably accounts for the lack of obvious
clinical manifestations.
Budd-Chiari syndrome can produce a mass lesion in
the acute stage due to hemorrhagic necrosis [13, 14], and
Bayraktar et al. [15] reported the disappearance of these
lesions on follow-up. In case 2, an irregular cystic lesion
with a thick wall was found in the right lobe of the liver,
consistent with an area of necrosis.
Ultrasound, CT, MR, and venography are all useful in
the diagnosis of patients with Budd-Chiari syndrome by
showing reduced caliber or complete absence of the he-
patic veins, marked constriction of the intrahepatic IVC,
and intrahepatic collateral veins with “comma-shaped” or
curled-tubular appearance [16, 17]. Dynamic enhanced
MR imaging demonstrates early discrepant enhancement
of central and peripheral portions of the liver, depending
on chronicity and the development of portal and hepatic
collaterals [18]. In the present cases, MR demonstrated
narrowing of the central hepatic veins and the IVC, in-
trahepatic collaterals, and heterogeneous enhancement of
the liver.
Budd-Chiari syndrome can result from extrinsic
compression by the diaphragm. MR shows hepatomeg-
aly due to hypertrophy, elevation of the diaphragm,
narrowing of the hepatic veins, stenosis of the IVC and
intrahepatic collaterals, and heterogeneous enhance-
ment of the liver.
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