Doppler ultrasound of the liver in hereditary hemorrhagic telangiectasia

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  • Curaçao diagnostic criteria for HHT1. Epistaxis Spontaneous, recurrent nose bleeds2. Telangiectases Multiple, at characteristic sites: Lips, oral cavity, nose, fingers3. VMs GI, pulmonary, hepatic, cerebral, spinal4. Family history First degree relative with HHTDefinite diagnosis If three criteria are presentPossible diagnosis If two criteria are presentUnlikely If fewer than two criteria are present
  • Causes of enlargement of hepatic artery:1- Cirrhosis2- Hepatic diseases associated with alcoholism3- Vascular tumors4- Hereditary hemorrhagic telangiectasia
  • Once the diagnosis of hepatic VMs has been established, the severity & type of the predominant intrahepatic shunt should determine what, if any, further testing is required. Early stages (grades 0 +, 1 and 2): Do not call for any other testsEchocardiography: Advised for grades 3 & 4 to evaluate cardiac function & overload as well as pulmonary pressure. Arterioportal shunts HGI endoscopy should be obtained to detect esophageal varices & other signs of PHT.
  • Doppler ultrasound of the liver in hereditary hemorrhagic telangiectasia

    1. 1. Doppler Ultrasound of Hereditary hemorrhagic telangiectasia Samir Haffar M.D. Department of Internal Medicine
    2. 2. Curaçao diagnostic criteria for HHT 1. Epistaxis Spontaneous, recurrent nose bleeds 2. Telangiectases Multiple, at characteristic sites: Lips, oral cavity, nose, fingers 3. VMs GI, pulmonary, hepatic, cerebral, spinal 4. Family history First degree relative with HHT Definite diagnosis: 3 criteria are present Possible diagnosis: 2 criteria are present Unlikely: Less than 2 criteria present Criteria VMs: Vascular Malformations Shovlin CL et al. Am J Med Genet 2000 ; 91 : 66 – 7.
    3. 3. Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645. Hereditary hemorrhagic telangiectasia Laparotomy Multiple tiny & reddish teleangiectases scattered on liver surface
    4. 4. Doppler US findings in HHT Prevalence of hepatic involvement: at least 33% • HA Dilated main HA & its branches High-velocity flow (aliased or turbulent) • Vms Porto-systemic shunt PV to HV Arterio-portal shunt HA to PV Arterio-systemic shunt HA to HV Systemic-to-systemic shunt HV to HV Liver biopsy should be avoided (high risk of bleeding) VMs: Vascular Malformations Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55.
    5. 5. Hereditary hemorrhagic telangiectasia Dilated main hepatic artery Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645. Dilated hepatic artery: 8.7 mm Measured 2 cm distal to its origin from celiac trunk
    6. 6. Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Hereditary hemorrhagic telangiectasia Dilated hepatic artery branches - Straight Transverse US of left lobe “double channel sign” Dilated hepatic artery branch Power Doppler US
    7. 7. Hereditary hemorrhagic telangiectasia Dilated hepatic artery branches – Tortuous Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Tortuous & tangled hepatic artery branches Power Doppler USGray-scale US
    8. 8. Slightly dilated HA: 6.2 mm High PSV: 129 cm/sec Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Hereditary hemorrhagic telangiectasia Duplex US of hepatic artery
    9. 9. Hereditary hemorrhagic telangiectasia Intra-hepatic arterial hypervascularization Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645. Tortuous small arterial branches Color Doppler USGray-scale US No abnormality demonstrated
    10. 10. Hereditary hemorrhagic telangiectasia Intrahepatic arterial hypervascularization Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Prominent intrahepatic arterial hypervascularization Color Doppler US of liver
    11. 11. Hereditary hemorrhagic telangiectasia Arterio-portal fistulas Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538. Low-resistance arterial flow Arterialized & inverted PV flow Dilated tortuous structures Dilated vascular structures with aliasing
    12. 12. Hereditary Hemorrhagic Telangiectasia Arterio-systemic shunt Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. Dilated hepatic artery branch & right hepatic vein Shunt of hepatic artery branch to right hepatic vein
    13. 13. Hereditary Hemorrhagic Telangiectasia Systemic-to-systemic shunt Oblique color Doppler US Large communication between right & middle hepatic veins RHV MHV Bertolotto M et al. J Clin Ultrasound 2008 ; 36 : 527 – 538.
    14. 14. Hereditary hemorrhagic telangiectasia Ocran K et al. Ultraschall Med 2004 ; 25 : 191 – 194. Dilated portal vein, hepatic artery, right hepatic vein & IVC Dilated tortuous intrahepatic arteries
    15. 15. US criteria for hepatic involvement in HHT Criteria Major criteria Dilated common hepatic artery (> 7 mm) Intrahepatic arterial hypervascularization Caselitz M et al. Hepatology 2003 ; 37 : 1139 – 1146. Diagnosis: two major criteria or one major with two minor Minor criteria PSV of proper hepatic artery > 110 cm/s RI of proper hepatic artery < 0.60 Vmax of portal vein > 25 cm/s Tortuous course of extrahepatic hepatic artery Facultative findings Dilated portal vein (> 13 mm) Dilated liver veins (> 11 mm) Hepatomegaly (> 15 cm in MCL) Nodular liver margin
    16. 16. Severity grading of hepatic VMs in HHT Buscarini E et al. Ultraschall Med 2004 ; 25 : 348 – 55. VM grading 0+ HA diameter 5 – 6 mm, and/or PFV > 80 cm/sec, and/or RI < 0.55, and/or Peripheral hepatic hypervascularization 1 HA dilatation, only extrahepatic > 6 mm, and PFV > 80 cm/sec, and/or RI < 0.55 2 HA dilatation, extra- and intrahepatic (“double channel” aspect) and PFV > 80 cm/sec Possibly associated with moderate flow abnormality of HA &/or PV 3 Complex changes in HA & its branches (tortuous and tangled) with marked flow abnormalities associated with – moderate dilatation of hepatic and/or portal veins – and/or abnormality of hepatic and/or portal vein flow 4 Decompensation of arteriovenous shunt such as: – marked dilatation of hepatic and/or portal vein – marked flow abnormalities in both arteries and veins
    17. 17. Thank You

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