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. Buscarini E et al. Dig Liver Dis 2005 ; 37 : 635 – 645.
Hereditary hemorrhagic telangiectasia
Laparotomy
Multiple tiny & reddish teleangiectases scattered on liver surface
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. 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. 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. 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. 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. 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. 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
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. 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. 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
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.
