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Sonography of Budd-Chiari Syndrome

¹ All authors: Department of Radiology, Hospital Clinic, C/Villarroel, 170, Barcelona, Spain 08036.

View larger version (92K): [in a new window]   Fig. 1A —45-year-old woman with 1-month history of weakness and enlargement of abdominal diameter. Gray-scale sonogram obtained in right subcostal plane shows echogenic bands replacing right and left hepatic veins (open arrowheads) and failed to show middle hepatic vein. Liver parenchyma is heterogeneous with large central hypoechoic area (arrows) representing edema caused by congestion. Note perihepatic ascites. Transjugular intrahepatic portosystemic shunt (TIPS) placement was mandatory. This case illustrates acute Budd-Chiari syndrome with obstruction of three major hepatic veins combining signs related to hepatic vein obstruction and those related to portal hypertension (liver edema and ascites). However, presence of fibrous cords replacing veins in this acute setting would support endophlebitis as pathogenic factor.

View larger version (85K): [in a new window]   Fig. 1B —45-year-old woman with 1-month history of weakness and enlargement of abdominal diameter. Color Doppler sonogram obtained in right subcostal plane 1 year later shows capsular hypertrophied vessels (open arrowheads).

View larger version (91K): [in a new window]   Fig. 2A —40-year-old woman with subacute Budd-Chiari syndrome (2 months since onset of symptoms) presenting as thrombosis of right and middle hepatic veins. Transverse Doppler sonogram shows lack of visualization of distal part of middle hepatic vein. Segment near inferior vena cava is filiform (open arrowheads) and has no flow. Irregular pulsed Doppler signals that are obtained represent artifactual transmission of cardiac contraction.

View larger version (84K): [in a new window]   Fig. 2B —40-year-old woman with subacute Budd-Chiari syndrome (2 months since onset of symptoms) presenting as thrombosis of right and middle hepatic veins. Gray-scale sonogram obtained 1 year later shows fibrous cords replacing hepatic veins (open arrowheads). ivc = inferior vena cava.

View larger version (121K): [in a new window]   Fig. 2C —40-year-old woman with subacute Budd-Chiari syndrome (2 months since onset of symptoms) presenting as thrombosis of right and middle hepatic veins. Color Doppler sonogram (C) and pulsed Doppler sonogram (D) display spontaneous direct portacaval shunt (arrow) between right portal vein (rpv) and inferior vena cava (ivc). This spontaneous shunt was by itself unable to provide enough hepatic decompression, and transjugular intrahepatic portosystemic shunt (TIPS) placement was necessary. Note that spectral Doppler waveform in D shows noncontinuous flow.

View larger version (80K): [in a new window]   Fig. 2D —40-year-old woman with subacute Budd-Chiari syndrome (2 months since onset of symptoms) presenting as thrombosis of right and middle hepatic veins. Color Doppler sonogram (C) and pulsed Doppler sonogram (D) display spontaneous direct portacaval shunt (arrow) between right portal vein (rpv) and inferior vena cava (ivc). This spontaneous shunt was by itself unable to provide enough hepatic decompression, and transjugular intrahepatic portosystemic shunt (TIPS) placement was necessary. Note that spectral Doppler waveform in D shows noncontinuous flow.

View larger version (81K): [in a new window]   Fig. 3A —35-year-old woman. At time of Budd-Chiari syndrome diagnosis, patient had 1-year history of mild symptoms (unspecific abdominal pain). Patient developed important collateral circulation in follow-up. Color Doppler sonogram obtained in intercostal plane displays attempt to replace obstructed right hepatic vein by means of fragmented, little veins (open arrowheads), which follow same track as that of previous occluded vein.

View larger version (103K): [in a new window]   Fig. 3B —35-year-old woman. At time of Budd-Chiari syndrome diagnosis, patient had 1-year history of mild symptoms (unspecific abdominal pain). Patient developed important collateral circulation in follow-up. Color Doppler sonogram obtained in intercostal plane shows spontaneous portacaval shunt. ivc = inferior vena cava.

View larger version (86K): [in a new window]   Fig. 4A —50-year-old woman with 2-month history of refractory ascites. Color Doppler sonogram obtained in intercostal plane shows uncolored right hepatic vein (open arrowheads) that is occupied by fine echoes thought to be related to acute thrombosis. Fine perivenous vessels (arrows) represent incipient collateral circulation.

View larger version (96K): [in a new window]   Fig. 4B —50-year-old woman with 2-month history of refractory ascites. Color Doppler sonogram obtained in intercostal plane shows large tortuous subcapsular vein going to drain to inferior vena cava (open arrowheads). Hypertrophied subcapsular veins may shunt blood from liver to systemic veins (azygos vein, intercostal veins) or directly to inferior vena cava creating new intrahepatic and extrahepatic circulation.

View larger version (115K): [in a new window]   Fig. 5A —50-year-old man with secondary Budd-Chiari syndrome. Tumoral invasion of hepatic veins may be seen in hepatocellular carcinoma and other invasive tumors such as sarcoma. Extended hepatocarcinoma tends to invade vascular structures, although isolated invasion of hepatic vein does not have clinical correlation. Gray-scale sonogram obtained in intercostal plane shows large tumor (arrows) with central anechoic area (arrowheads) that represents necrosis. Histologic study diagnosed paraganglioma.

View larger version (120K): [in a new window]   Fig. 5B —50-year-old man with secondary Budd-Chiari syndrome. Tumoral invasion of hepatic veins may be seen in hepatocellular carcinoma and other invasive tumors such as sarcoma. Extended hepatocarcinoma tends to invade vascular structures, although isolated invasion of hepatic vein does not have clinical correlation. Gray-scale sonogram obtained in right subcostal plane shows tumor invading middle hepatic vein (mhv). Thin arrows show vein invasion. Note presence of small vessels (thick arrows) going toward patent left hepatic vein (lhv). In this case, patient shows no symptoms associated with vascular infiltration.

View larger version (115K): [in a new window]   Fig. 5C —50-year-old man with secondary Budd-Chiari syndrome. Tumoral invasion of hepatic veins may be seen in hepatocellular carcinoma and other invasive tumors such as sarcoma. Extended hepatocarcinoma tends to invade vascular structures, although isolated invasion of hepatic vein does not have clinical correlation. Color Doppler sonogram in right subcostal plane shows small vessels in blue (arrows) going from middle hepatic vein (mhv) to left hepatic vein (lhv). Note aliasing in middle hepatic vein caused by proximal obstruction. ivc = inferior vena cava.

View larger version (110K): [in a new window]   Fig. 6A —59-year-old woman with orthotopic liver transplantation for chronic hepatitis C virus. Color Doppler sonogram obtained in right intercostal plane shows right hepatic vein (rhv) with inverted flow (red). mhv = middle hepatic vein.

View larger version (101K): [in a new window]   Fig. 6B —59-year-old woman with orthotopic liver transplantation for chronic hepatitis C virus. Color Doppler sonogram slightly tilted relative to previous image shows right hepatic vein (out of plane) draining through collateral vessel (arrowhead) to middle hepatic vein (mhv), resulting in presence of "bicolored" hepatic vein. Collateral vessel connecting occluded (or stenotic) hepatic vein to neighboring patent hepatic vein (blue) results in presence of "bicolored" hepatic veins. Thus, "bicolored" phenomenon consists of flow, which turns away and toward transducer in same vessel. In this case, it was incidental finding that was thought to be related to stenosis of hepatic vein because junction with vena cava was not clearly depicted.

View larger version (101K): [in a new window]   Fig. 7A —55-year-old man with paroxysmal nocturnal hemoglobinuria (PNH) who presented with 6-week history of generalized edema and diffuse abdominal pain. Sonographic study was performed showing that right and left hepatic veins were obstructed. Term "spiderweb" was initially used in description of angiographic findings in Budd-Chiari syndrome, and it means presence of very small interwoven veins. This split image (gray scale on right and color Doppler, shown here in gray scale, on left) shows example of spiderweb circulation (arrowheads) with Budd-Chiari syndrome. Note that nonconspicuity of these small-size veins made its visualization really difficult on gray-scale sonography. On right, only intrahepatic portal vein (arrow) is evident.

View larger version (64K): [in a new window]   Fig. 7B —55-year-old man with paroxysmal nocturnal hemoglobinuria (PNH) who presented with 6-week history of generalized edema and diffuse abdominal pain. Sonographic study was performed showing that right and left hepatic veins were obstructed. Pulsed Doppler sonogram obtained 1 year later illustrates anomalous curvilinear vessel (arrowheads) draining to inferior vena cava (ivc) replacing obstructed right hepatic vein.

View larger version (70K): [in a new window]   Fig. 8A —43-year-old man with subacute Budd-Chiari syndrome. Split image (gray scale on left and color Doppler on right) obtained in intercostal plane. On gray scale, note curvilinear, fragmented vessels (arrowheads) that are characteristic for Budd-Chiari syndrome.

View larger version (89K): [in a new window]   Fig. 8B —43-year-old man with subacute Budd-Chiari syndrome. Venovenous collaterals are anomalous curvilinear vessels that usually drain to inferior vena cava, although sometimes inferior vena cava does not show evidence of connections with other vessels. Color Doppler sonogram in intercostal plane shows multiple anomalous, curvilinear veins (arrows) draining to inferior vena cava (arrowheads).

View larger version (114K): [in a new window]   Fig. 9 —35-year-old woman suffering from chronic Budd-Chiari syndrome related to myelofibrosis. Color Doppler sonogram, shown here in black-and-white, displays characteristic collateral vessel in hockey-stick appearance (arrow). These subcapsular vessels are typical and may connect with extrahepatic circulation.

View larger version (135K): [in a new window]   Fig. 10A —36-year-old man with primary thrombocythemia developed chronic Budd-Chiari syndrome that required transjugular intrahepatic portosystemic shunt (TIPS) placement. Patient has multiple benign nodules largely followed without changes. Sonogram shows distal thrombosis of middle and left hepatic veins (mhv and lhv, respectively) with collateral circulation within them (arrowhead). Note TIPS (arrows).

View larger version (122K): [in a new window]   Fig. 10B —36-year-old man with primary thrombocythemia developed chronic Budd-Chiari syndrome that required transjugular intrahepatic portosystemic shunt (TIPS) placement. Patient has multiple benign nodules largely followed without changes. Sonogram in sagittal epigastric line shows caudate hypertrophy with prominent caudate lobe vein (arrowheads).

View larger version (111K): [in a new window]   Fig. 10C —36-year-old man with primary thrombocythemia developed chronic Budd-Chiari syndrome that required transjugular intrahepatic portosystemic shunt (TIPS) placement. Patient has multiple benign nodules largely followed without changes. Color Doppler sonogram, shown here in gray scale, discloses two iso- and hyperechoic nodules (arrowheads) surrounded by thin hypoechoic halo. Spectral study shows low resistance arterial waveform with high velocity.

View larger version (108K): [in a new window]   Fig. 10D —36-year-old man with primary thrombocythemia developed chronic Budd-Chiari syndrome that required transjugular intrahepatic portosystemic shunt (TIPS) placement. Patient has multiple benign nodules largely followed without changes. Helical CT after IV contrast injection displays enhancing nodule (arrows) surrounded by low-attenuating area related to vascular disorders. On helical CT, benign regenerative nodules are homogeneously hyperattenuating on arterial phase and remain slightly hyperattenuating on portal vein phase.

View larger version (57K): [in a new window]   Fig. 11A —39-year-old woman with Budd-Chiari syndrome diagnosed 1 year ago. Split image showing portal thrombosis. Color Doppler sonogram shows portal thrombus (thin arrows). Note hepatofugal flow in right portal vein (arrowhead). Thick arrow points to hepatic artery.

View larger version (114K): [in a new window]   Fig. 11B —39-year-old woman with Budd-Chiari syndrome diagnosed 1 year ago. Gray-scale sonogram obtained in transversal epigastric plane shows evident caudate lobe vein (arrowheads). Note changes in parenchymal echogenicity (arrows) reflecting vascular disorder. ivc = inferior vena cava.

View larger version (72K): [in a new window]   Fig. 12A —32-year-old man with multiple small liver nodules without changes in 4 years. On color Doppler sonography, regenerative nodules can be isoechoic or mildly hyperechoic with hypoechoic halo. Poor border definition is also frequent finding. Most nodules show arterial vascularization (radial and peripheral) at pulsed Doppler interrogation. Power Doppler sonogram shows small isoechoic hypervascular nodule (arrows).

View larger version (70K): [in a new window]   Fig. 12B —32-year-old man with multiple small liver nodules without changes in 4 years. On color Doppler sonography, regenerative nodules can be isoechoic or mildly hyperechoic with hypoechoic halo. Poor border definition is also frequent finding. Most nodules show arterial vascularization (radial and peripheral) at pulsed Doppler interrogation. Pulsed color Doppler sonogram shows arterial nature of vessels (arrows).

View larger version (71K): [in a new window]   Fig. 12C —32-year-old man with multiple small liver nodules without changes in 4 years. On color Doppler sonography, regenerative nodules can be isoechoic or mildly hyperechoic with hypoechoic halo. Poor border definition is also frequent finding. Most nodules show arterial vascularization (radial and peripheral) at pulsed Doppler interrogation. MR T1-weighted image after gadolinium injection shows multiple peripheral enhancing nodules (arrows). On MRI, benign regenerative nodules are hyperintense on T1-weighted and commonly hyperintense on T2-weighted images (distinctive finding). There is hypervascularity at dynamic MR study.

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