Sonography in Budd-Chiari Syndrome

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1 Image Presentation Sonography in udd-chiari Syndrome Nitin Chaubal, MD, DMRD, Manjiri Dighe, MD, DMRE, Vijay Hanchate, MD, Hemangini Thakkar, MD, Hemant Deshmukh, MD, Krantikumar Rathod, MD Objective. The objective of this presentation is to provide an overview of sonographic manifestations of udd-chiari syndrome (CS). Methods. Patients were scanned with ultrasound systems using mainly a 2- to 5-MHz curvilinear transducer and in some patients a 5- to 12-MHz linear transducer. The patients were asked to fast from the previous night or for at least 6 hours. Color and spectral Doppler sonography was performed in all patients. Results. Commonly seen findings in CS include inferior vena cava (IVC) webs and thrombi, IVC narrowing, hepatic venous thrombosis, enlarged caudate lobes, ascites, intrahepatic or extrahepatic collaterals, monophasic to absent flow in the hepatic veins, and high flow velocities in areas of stenosis in the IVC or hepatic veins. Inferior vena cava stents used in the treatment of CS could also be seen. Conclusions. udd-chiari syndrome is an uncommon disorder; outcome is poor in many cases; and the condition is often misdiagnosed or underdiagnosed. Sonography is a noninvasive and effective modality for diagnosis of CS. Key words: udd-chiari syndrome; hepatic Doppler sonography; inferior vena cava abnormalities. bbreviations CS, udd-chiari syndrome; IVC, inferior vena cava Received September 26, 2005, from Thane Ultrasound Centre, Thane, India (N.C.); King Edward Hospital and Seth G. S. Medical College, Mumbai, India (N.C., V.H., H.T., H.D., K.R.); University of Washington Medical Center, Seattle, Washington US (M.D.); and Jaslok Hospital and Research Center, Mumbai, India (N.C.). Revision requested October 3, Revised manuscript accepted for publication October 19, ddress correspondence to Nitin Chaubal, MD, DMRD, Thane Ultrasound Centre, Shanti Nivas, Dr Moose Road, Talaopali, Thane , India. chaubal@bom3.vsnl.net.in udd-chiari syndrome (CS) is a rare condition that is variable in its presentation and natural history. It can be life threatening if not diagnosed and treated promptly. 1 udd-chiari syndrome is a manifestation of hepatic venous outflow obstruction that was first described by George udd in 1845 and then expounded on by Hans Chiari, who presented 13 cases in The hepatic venous obstruction occurs late at the level of the inferior vena cava (IVC), the hepatic veins, and, depending on the classification and nomenclature, possibly at the level of the hepatic venule. 2 udd-chiari syndrome can be classified according to etiology, site of obstruction, manifestations, and duration of the disease. udd-chiari syndrome is considered primary when obstruction of the hepatic venous outflow tract is the result of an endoluminal venous lesion (thrombosis or web) or secondary when the obstruction results from the presence in the lumen of material not originating from the venous system or from extrinsic compression by a neighboring tumor. 3 In practice, CS is regarded as primary when no causes of secondary obstruction are found. CS can also be classified according to the level of obstruction (Table 1) or duration of disease (Table 2) by the merican Institute of Ultrasound in Medicine J Ultrasound Med 2006; 25: /06/$3.50

2 Sonography in udd-chiari Syndrome Table 1. Classification of CS ccording to the Level of Obstruction Type I II III Level of Obstruction Obstruction of IVC with or without secondary hepatic vein occlusion Obstruction of major hepatic veins Obstruction of the small centrilobular venules (considered by some as veno-occlusive disease). Table 2. Classification of CS ccording to the Duration of Disease Type Fulminant cute Subacute Chronic Duration Present with hepatic encephalopathy within 8 wk of development of jaundice Short duration, ascites, hepatic necrosis without formation of venous collaterals Insidious onset, ascites, minimal hepatic necrosis and portal and hepatic venous collaterals Complications of cirrhosis in addition to findings in subacute form Hypercoagulable states, hereditary or acquired, account for 75% of the cases of CS in the United States. 4 Hematologic disorders, such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria, and myeloproliferative diseases, account for 10% to 40% of the cases of CS. 5 Postpartum status accounts for another 20%, and tumors account for 9%. Inferior vena cava webs and stenosis are uncommon causes of CS in the United States; however, more than 50% of cases of CS in the Southeast sian countries result from these causes. 6 bdominal pain and hepatomegaly are present in almost all patients with the CS. Splenomegaly and jaundice may also be present in some patients. Sonographic Findings in CS Sonographic features of hepatic vein involvement include a partial or complete inability to see the hepatic veins (Figure 2), stenosis with proximal dilatation, intraluminal echogenicity, thickened walls, and thrombosis (Figure 3). The IVC may show narrowing due to compression by the enlarged caudate lobe (Figure 4), long segment narrowing without associated caudate lobe enlargement, or localized, marked narrowing consistent with a web (Figure 5) or a thrombus (Figure 6). Membranous webs may be seen as echogenic areas or focal obliteration of the lumen. Webs may be difficult to visualize in cirrhotic patients in whom the hepatic veins are difficult to see. 8 Uncommonly, an aneurysm may be seen in the IVC (Figure 7, and ). Intrahepatic collaterals are a specific diagnostic criterion for CS, 9 except for the paraumbilical vein, which is also seen in portal hypertension. Collaterals are either intrahepatic, which communicate with systemic vessels via subcapsular collaterals (Figure 8, and ), or from an occluded hepatic vein to a nonoccluded hepatic vein (Figure 9) or to the caudate lobe veins (Figure 9). Rarely, collaterals are also seen from hepatic veins to the suprahepatic IVC close to the right atrium (Figure 10). Color Doppler and Spectral Doppler Findings On Color Doppler imaging, no flow or flow in an abnormal direction is seen in all or part of the hepatic veins (Figure 11, and ). If CS is caused by stenosis of a hepatic vein, high flow Figure 1. Sagittal sonogram in the epigastric region showing massive caudate lobe enlargement (asterisks) causing compression of the IVC (arrow). Doppler sonography of the liver, with sensitivity and specificity of 85% or greater, is the technique of choice for initial investigation when CS is suspected. 7 -Mode Findings On -mode sonography, the liver is typically large and bulbous in the acute phase. Hemorrhagic infarction may produce substantially altered regional echogenicity. The caudate lobe is often spared in CS as the emissary veins drain directly into the IVC at a lower level than the involved main hepatic veins, leading to caudate hypertrophy (Figure 1) J Ultrasound Med 2006; 25:

3 Chaubal et al essential because thrombosis precludes decompression of the liver via a portosystemic shunt. Changes seen in chronic CS include cirrhosis, splenomegaly, portal hypertension, and ascites, in addition to the hepatic venous collaterals. Chronic CS may be difficult to distinguish from primary cirrhosis and portal hypertension, and angiographic delineation of hepatic veins, collaterals in the form of a spiderweb network, IVC thrombi, or webs may be necessary for diagnosing chronic CS. 13 summary of the sonographic findings in CS is presented in Table 3. Figure 2. Transverse sonogram of the liver with inability to show the hepatic veins and with a probable middle hepatic vein seen as a linear hyperechoic area (arrow). velocity is seen at the site of stenosis (Figure 12). The normal blood flow in the IVC and hepatic veins is phasic in response to both the cardiac and respiratory cycles. 10 In CS, flow in the IVC, hepatic veins, or both changes from phasic to absent, reversed, turbulent, or continuous (Figure 13). Continuous flow has been called a pseudoportal Doppler signal and appears to reflect either partial IVC obstruction or extrinsic IVC compression. 11 The portal blood flow also may be affected and is slowed or reversed. 12 ssessment of potency of the portal vein is Figure 4. Sagittal image in the epigastric region showing compression of the IVC by the enlarged caudate lobe (, white arrow) with high flow velocities seen at the site of narrowing in the IVC () on a spectral Doppler image. R indicates right atrium. Figure 3. Thrombus in the middle and left hepatic veins seen as echogenic areas (thick arrows) in the transverse subcostal image. Narrowing is also seen at the distal end of the middle hepatic vein as it joins the IVC (thin arrow). J Ultrasound Med 2006; 25:

4 Sonography in udd-chiari Syndrome Therapeutic interventions in CS include thrombolytic therapy, angioplasty with or without stenting (Figure 14), transjugular intrahepatic portosystemic shunting, surgical shunting, and liver transplantation. Percutaneous or transhepatic angioplasty or stenting of a narrowed hepatic vein or IVC webs may relieve symptoms in 70% of patients. ecause the rate of restenosis is high, regular follow-up is necessary to assess for restenosis or stent occlusion. 14,15 Figure 5., Sagittal image through the superior IVC showing localized narrowing in the IVC (arrow), consistent with a membrane with the corresponding IVC angiogram image () showing complete occlusion of the IVC (single arrow) with multiple collaterals (double arrows). Figure 6. Small thrombus (white arrow) seen in the IVC possibly proximal to a web (black arrow). Measurement calipers are seen at the thrombus, which measures approximately 1.5 cm. R indicates right atrium. Figure 7. Transverse image through the upper abdomen showing aneurysm formation in the IVC (, arrow) in a patient with CS, confirmed on angiography (, arrow). O indicates aorta. 376 J Ultrasound Med 2006; 25:

5 Chaubal et al Conclusions udd-chiari syndrome is an uncommon disorder; outcome is poor in many cases; and the condition is often misdiagnosed or underdiagnosed. Therefore, a successful diagnostic and therapeutic approach is of vital importance. Sonography is a noninvasive and effective modality for diagnosis of CS. It gives accurate anatomic and physiologic information and helps determine the patency and direction of flow in hepatic veins and portal venous system. Sonography is extremely helpful in documentation of complex intrahepatic venous anastomosis and is an excellent noninvasive test to assess stent patency. Figure 8., Capsular collaterals seen along the left subhepatic space (arrow) exiting through the liver in a transverse image through the left lobe. The middle hepatic vein is hepatofugal (blue), but the left hepatic vein is reversed (red) and flowing toward the large collateral (blue)., Collateral seen along the liver surface (arrow) in a transverse image acquired by a linear high-frequency transducer (12 MHz). Figure 9. Images through the epigastric region showing an intrahepatic collateral between hepatic veins (arrow) seen as an abnormal vessel communicating between the 2 hepatic veins (double arrows) on a transverse image () and an enlarged caudate lobe (asterisks) with a collateral seen as an abnormal enlarged vessel (arrow) in the caudate lobe on a sagittal image (). Figure 10. Transverse image in the epigastric region showing a large collateral (white arrow) entering into the suprahepatic IVC (double black arrows). J Ultrasound Med 2006; 25:

6 Sonography in udd-chiari Syndrome Figure 11. Transverse images through the liver showing liver with a small nodular contour and heterogeneous appearance () suggesting cirrhotic changes with reversal of flow in the left and middle hepatic veins (red) and with normal direction of flow in the right hepatic vein (blue) and reversal of flow in the middle hepatic vein (, red, white arrow) with normal direction of flow in the left hepatic vein (blue, black arrow). Figure 12., Transverse sonogram showing aliasing (white arrow) at the hepatic vein-ivc anastomosis., Doppler spectrum at the corresponding level showing high flow velocities with turbulence at the level of the stenosis and low flow velocity (arrows) proximally due to patient breathing. Figure 13. Doppler spectral image in the middle hepatic vein showing monophasic low-velocity flow. Figure 14. Sagittal image through the epigastric region showing a stent in the IVC (arrow) as 2 linear hyperechoic areas. Flow is present within the stent, suggesting patency. 378 J Ultrasound Med 2006; 25:

7 Chaubal et al Table 3. Summary of Sonographic Findings in CS Imaging Type Findings -mode Liver Hepatic veins IVC Hepatomegaly; selective caudate lobe enlargement (Figure 1), hepatic infarction Partial or complete inability to see the hepatic veins (Figure 2); stenosis with proximal dilatation, intraluminal echogenicity, thickened walls, and thrombosis (Figure 3) Narrowing due to compression by the enlarged caudate lobe (Figure 4); long segment narrowing without associated caudate lobe enlargement or localized, marked narrowing consistent with a web (Figure 5) or a thrombus (Figure 6), uncommonly, aneurysm formation (Figure 7, and ) Collaterals Intrahepatic collaterals that communicate with systemic vessels via subcapsular collaterals (Figure 8, and ) from an occluded hepatic vein to a nonoccluded hepatic vein (Figure 9) or to the caudate lobe veins (Figure 9); hepatic veins to the suprahepatic IVC close to the right atrium (Figure 10) and to the portal system. Doppler Color Doppler No flow or flow in an abnormal direction in all or part of the hepatic veins (Figure 11, and ); stenosis of hepatic veins, aliasing at the hepatic ostium (Figure 12) Spectral Doppler Stenosis of a hepatic vein - high flow velocity is seen at the site of stenosis (Figure 12); phasic to absent, reversed, turbulent or continuous flow in the IVC, hepatic veins, or both (Figure 13) References 1. Janssen HL, Garcia-Pagan J-C, Elias E, et al. udd-chiari syndrome: a review by an expert panel. J Hepatol 2003; 38: Ludwig J, Hashimoto E, McGill D, et al. Classification of hepatic venous outflow obstruction: ambiguous terminology of the udd-chiari syndrome. Mayo Clin Proc 1990; 65: Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for udd-chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 1998; 28: Menon NKV, Shah V, Kamath P. The udd-chiari syndrome. N Engl J Med 2004; 350: Valla D, Casaevall N, Lacombe C, et al. Primary myeloproliferative disorder and hepatic vein thrombosis: a prospective study of erythroid colony formation in vitro in 20 patients with udd-chiari syndrome. nn Intern Med 1985; 103: De K, De KK, Sen S, et al. Etiology based prevalence of udd-chiari syndrome in eastern India. J ssoc Physicians India 2000; 48: Taylor KJW, urns PN, Woodcock JP, Wells PN. lood flow in deep abdominal and pelvic vessels: ultrasonic pulsed Doppler analysis. Radiology 1985; 154: Keller MS, Taylor KJW, Riely C. Pseudo-portal Doppler signal in the partially obstructed inferior vena cava. Radiology 1989; 170: Hosoki T, Kuroda C, Tokunaga K, Marukawa T, Masuike M, Kozuka T. Hepatic venous outflow obstruction: evaluation with pulsed Duplex sonography. Radiology 1989; 170: Tisnado JC, Carithers RL Jr, Goldschmidt R. The udd- Chiari syndrome: angiographic pathologic correlation. Radiographics 1983; 3: Yang XL, Cheng TO, Chen CR. Successful treatment by percutaneous balloon angioplasty of udd-chiari syndrome caused by membranous obstruction of inferior vena cava: 8 year follow-up study. J m Coll Cardiol 1996; 28: Fisher NC, McCafferty I, Dolapci M, et al. Managing udd- Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut 1999; 44: olondi L, Gaiani S, Li Nassi S, et al. Diagnosis of udd- Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology 1991; 100: Wiethers CE, Wilson SR. The Liver. In: Diagnostic Ultrasound. St Louis, MO: Mosby Year ook; 1991: Menu Y, lison D, Lorphelin JM, Valla D, elghiti J, Nahum H. udd-chiari syndrome: US evaluation. Radiology 1985; 157: J Ultrasound Med 2006; 25: