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 compression of the inferior vena cava by an elevated right hemidiaphragm. Magnetic resonance images demonstrated 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, stenosis or obstruction—Budd-Chiari syndrome.
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 apparent 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 performed 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 hypertension, other signiﬁcant liver disease, or lower extremity edema.
Budd-Chiari syndrome involves hepatic venous outﬂow obstruction 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, malignancy, and miscellaneous . In Budd-Chiari syndrome, some hepatic venous drainage 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 collaterals draining into the IVC [2–5]. Compensatory hypertrophy 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 . Some
Correspondence to: D. G. Mitchell
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).
P. N. Kim et al.: Hepatic venous obstruction by an elevated diaphragm
Fig. 1. A–C Axial FMPSPGR images (TR/TE 150/2.4, NEX 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
vein (large arrow) is not connected to the IVC. The azygos and hemiazygos veins are dilated. D Coronal FMPSPGR image (TR/TE 130/2.3, NEX 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 connected to the IVC, but the accessory hepatic veins are drained into the IVC.
A 69-year-old woman was transferred for further evaluation of multiple cystic lesions in the liver. Laboratory values were normal. CT showed
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 atrophy 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 6.8/2.1, ﬂip angle 20°, NEX 0.5) taken approximately 1 min after administration of gadolinium chelate. A shows counterclockwise rotation (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
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 intrahepatic 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.
moderate atrophy of the anterior segment of the right lobe, and compensatory hypertrophy of the caudate and central liver. Tortuous intrahepatic venous collaterals were demonstrated in the right lobe of the
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.
P. N. Kim et al.: Hepatic venous obstruction by an elevated diaphragm
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 principal 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 function of affected hepatic segments . Regions with completely 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 regeneration, hypertrophy, and atrophy depend in part on the degree of portal perfusion , Budd-Chiari syndrome is typically associated with peripheral hepatic atrophy and caudate and central hypertrophy. Because of central hypertrophy of the liver, the portahepatis may be displaced toward the anterior portion of the liver , and the IVC can be compressed . 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 . Hypertrophy of the liver may posteriorly displace the IVC. In our cases, elevation of the right hemidiaphragm caused acute angulation of the hepatic venous connection to the IVC, resulting in obstruction. 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 hemidiaphragm, 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 . The syndrome has been associated with hypercoagulable states, neoplasms, trauma, medications, and congenital abnormalities . With Budd-Chiari syndrome due to trauma, the IVC was compressed by hematoma or enlarged liver , or the hepatic veins were obstructed by hepatic hernia into the thorax . 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
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 posterolaterally 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.  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 hepatic 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 . In the present cases, MR demonstrated narrowing of the central hepatic veins and the IVC, intrahepatic collaterals, and heterogeneous enhancement of the liver. Budd-Chiari syndrome can result from extrinsic compression by the diaphragm. MR shows hepatomegaly due to hypertrophy, elevation of the diaphragm, narrowing of the hepatic veins, stenosis of the IVC and intrahepatic collaterals, and heterogeneous enhancement of the liver.
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