Doppler ultrasound of Budd Chiari syndrome & SOS

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  • Consider only direct visualization of obstruction, and/or collaterals, of a hepatic vein or inferior vena cava, as definite evidence for the diagnosis.
  • Term “spiderweb” was initially used in description of angiographic findings in Budd-Chiari syndrome, and it means presence of very small interwoven veins.Interwoven: منسوجة
  • 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.
  • “Bicolored” hepatic vein
  • HCC appears to be a significant long-term complication (11 of 97 patients followed-up for a mean of 5 years).αFP more specific for dg than with other liver diseases. Risk of HCC in long-standing IVC obstruction 70-fold higher than those with pure hepatic vein involvement.
  • HVOD frequently develops before day 20 of bone marrow transplantation.clinically suspected when there is jaundice, painful hepatomegaly, and fluid retention. Intensive myeloablativechemotherapy or radiation therapy before transplantation is presumed to be the cause.Clinical criteria for diagnosis: Seattle Criteria & Baltimore Criteria (weight gain – ascites – hepatomegaly - hyperbilirubinemia). The diagnosis may be supported by imaging, which will demonstrate the presence of hepatomegaly and ascites and rule out biliary obstruction due to benign or malignant causes, but imaging is currently not diagnostic by itself. The best-studied modality is gray-scale and color Doppler ultrasonography. The majority of studies suggest that no single ultrasound parameter is diagnostic for SOS.Findings that are highly suggestive of SOS are reversal of portal venous flow, attenuation of hepatic venous flow, gallbladder wall edema, and perhaps increased resistive indices to hepatic artery flow. A composite score of gray-scale and color Doppler ultrasound criteria has been proposed, but may be too cumbersome for routineclinical use. One study has suggested that the presence of flow in the para-umbilical vein is more common in moderate and severe SOS, but this will need to be validated by other investigators.
  • Doppler ultrasound of Budd Chiari syndrome & SOS

    1. 1. Doppler ultrasound of Budd-Chiari syndrome Samir Haffar M.D. Assistant Professor of gastroenterology
    2. 2. Causes of portal hypertension Pre-sinusoidal Congenital hepatic fibrosis Sarcoidosis Schistosomiasis Lymphoma Hyperdynamic Arterio-portal fistula or malformation Robinson KA et al. Ultrasound Quarterly 2009 ; 25 : 3 – 13. Intra-hepatic Post-sinusoidal Cirrhosis Causes Disease Extra-hepatic Portal vein thrombosis or compression most common cause Supra-hepatic Budd-Chiari syndrome Right heart insufficiency
    3. 3. Budd-Chiari syndrome Narayanan Menon KV et al. N Engl J Med 2004 ; 350 : 578 – 85. Occlusion of a single hepatic vein usually clinically silent Two or three hepatic veins can be occluded without clear symptoms
    4. 4. Doppler US in BCS Obstruction &/or collaterals of HV or IVC* * DeLeve L et al. AASLD practice guidelines. Hepatology 2009 ; 49 : 1729 – 1764. Obstructed HV Presence of solid endoluminal material Hyperechoic cord replacing normal vein Reversed flow in large hepatic vein Dilatation of vein upstream to obstacle HV collaterals Sipder web in vicinity of HV ostia Subcapsular or HV to intercostal or HV veins Caudate lobe hypertrophy with dilated veins IVC Web – Thrombosis – Inversion of flow
    5. 5. BCS / Solid endoluminal material in HV Solid endoluminal material in middle & left hepatic veins Narrowing at distal end of middle hepatic vein as it joins IVC Chaubal N et al. J Ultrasound Med 2006 ; 25 : 373 – 379. Transverse subcostal image
    6. 6. BCS / Hyperechoic cord Hepatic veins transformed to fibrotic cords “Hepatic vein star” Boozari B et al. J Hepatol 2008 ; 49 : 572 – 580.
    7. 7. BCS / Reversed flow in large HV Inverted flow in right hepatic vein Normal flow in middle hepatic vein Right intercostal view Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
    8. 8. BCS / Reversed flow in HV upstream to obstacle Solid endo-luminal material in distal part of MHV Reverse flow in proximal part of MHV
    9. 9. BCS / Sipder web in vicinity of HV ostia Vilgrain V. Eur Radiol 2001 ; 11 : 1563 – 1577. Segev D L. Liver Transpl 2007 ; 13 : 1285 – 1294. Gray-scale US Small interwoven veins near IVC Hepatic venogram Typical “spider web” pattern
    10. 10. BCS / Large subcapsular vein Large tortuous subcapsular vein draining into IVC Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
    11. 11. BCS / HV draining into another HV Occluded RHV draining through collateral vessel into MHV Flow away & toward transducer in same vessel “Bicolored hepatic vein” Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42.
    12. 12. BCS / Collateral from HV to caudate lobe vein Brancatelli G et al. Am J Roentgenol 2007 ; 188 : W168 – W176. Transverse Doppler US at level of caudate lobe Lack of flow in distal portion of MHV Collateral from MHV to caudate lobe vein
    13. 13. BCS / Suggestive intra-hepatic collateral Erden A. Eur J Radiol 2007 ; 61 : 44 – 56. Undulated course “h-shaped” Hockey-stick Curvilinear Curved
    14. 14. BCS / Caudate lobe hypertrophy Erden A. Eur J Radiol 2007 ; 61 : 44 – 56. Sagittal gray-scale US Enlarged caudate lobe Antero-posterior diameter: 7.6 cm
    15. 15. BCS / Dilated caudate lobe vein 75% of cases Bargalló X et al. Am J Roentgenol 2003 ; 181 : 1641 – 1645. Mildly dilated caudate vein 7 mm Largely dilated caudate vein 21 mm Caudate vein (≥ 3 mm) suggests diagnosis Except for cardiac failure
    16. 16. BCS / Membranous obstruction of IVC Kandpal H et al. RadioGraphics 2008 ; 28 : 669 – 689. 30-year-old woman, abdominal pain & distention of 3 y duration Ostial HV narrowing Multiple IH collaterals Tapered IVC occlusion at cavo-atrial junction Reversed flow in IVC Loss of cardiac pulsations
    17. 17. Budd-Chiari syndrome & liver hydatid disease Retrospective study of 13 patients with HDL & BCS Yilmaz C et al. Radiol Oncol 2009 ; 43 : 225 – 232. Heterogeneous mass representing degenerated & collapsed membranes Large subcapsular vein draining into suprahepatic IVC
    18. 18. BCS / IVC thrombosis Behçet disease – Secondary BCS Sagittal image of IVC distended with echogenic thrombus Secondary BCS due to renal cell carcinoma Rumack CM, Wilson SR, & Charboneau JW. Diagnostic Ultrasound. Elsevier-Mosby, St. Louis, Missouri, USA, 3rd edition, 2005.
    19. 19. BCS / Thrombosis of PV 15% of patients – Poor prognosis Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42. Thrombosis of portal vein Hepatofugal flow in right portal vein Dilated hepatic artery
    20. 20. BCS / Benign regenerative nodules Multiple (> 10) – Small ( < 4 cm) – Hypervascular Vilgrain V et al. Radiology 1999 ; 210 : 443 – 450. Bargalló X et al. Am J Roentgenol 2006 ; 187 : W33 – W42. Two iso- & hyperechoic nodules surrounded by thin hypoechoic halo Low resistance arterial waveform with high velocity
    21. 21. Proposed diagnostic strategy for BCS Valla DC. Gut 2008 ; 57 : 1469 – 1478. DeLeve L et al. AASLD practice guidelines. Hepatology 2009 ; 49 : 1729 – 1764.
    22. 22. Doppler US in SOS Non specific • Main PV Decreased, to-and-fro, or reversed flow • Hepatic artery Significant elevation of RI (> 0.80) • Hepatic veins Normal direction – Monophasic flow • IVC Patent with flow toward heart McGahan J et al. Diagnostic ultrasound, Informa Healthcare, 2nd edition, 2008. * Lassau N et al. Radiology 1997 ; 204 : 545 – 552. • US findings Thickened GB wall – Ascites
    23. 23. Sinusoidal obstruction syndrome (SOS) BMT for acute myelogenous leukemia Desser TS et al. Am J Roentgenol 2003 ; 180 : 1583 – 1591. Contrast-enhanced CT Heterogeneous hepatic enhancement Color & duplex US of HV Monophasic flow in MHV Imaging currently not diagnostic by itself
    24. 24. Thank You

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