Table 6

Screening studies for liver vascular malformations (VMs) in hereditary haemorrhagic telangiectasia (HHT)

StudyNumberPopulation% with HHTType of studyTestFindings for liver VMsFrequency of abnormality in liver VMsPrevalence of liver VMs detectedGold standard
Memeo et al 2005112105HHT, consecutive patients100%

  • Screening

  • Descriptive

CT

  • Telangiectasia CVMs

  • AV shunt

  • AP shunt

  • AV & AP shunt

  • Perfusion abN PHT

  • 50/78 (64%)

  • 20/78 (26%)

  • 40/78 (51%)

  • 16/78 (21%)

  • 22/78 (28%)

  • 46/78 (59%)

  • 46/78 (59%)

78/100 (78%)No
Ravard et al 200411324
24		HHT, consecutive patients controls100%

  • Screening

  • Descriptive comparative

CT

  • Dilated HA

  • Telangiectasia

  • AV shunt

  • AP shunt

  • 16/16 (100%)

  • 12/16 (75%)

  • 5/16 (31%)

  • 3/16 (19%)

16/24 (67%)No
Buscarini et al 2004114346HHT, members of HHT families221 (64%)

  • Screening

  • Descriptive

Doppler US

  • Mild

  • Moderate

  • Severe

  • 11/92 (12%)

  • 70/92 (76%)

  • 11/92 (12%)

92/221(41%)No
Buscarini et al 199711573HHT, one family40 (55%)

  • Screening

  • Descriptive

Doppler US

  • Mild

  • Moderate

  • Severe

  • 3/13 (23%)

  • 3/13 (23%)

  • 7/13 (46%)

13/40 (32%)Angio12/13
Ocran et al 200411622HHT consecutive patients100%

  • Screening

  • Descriptive

Doppler US

  • Dilated HA

  • Dilated intra HA

  • AV shunts

  • 14/16 (88%)

  • 15/16 (94%)

  • 16/16 (100%)

16/22 (73%)No

  • Clinical liver VMs, patients with clinical signs or symptoms of liver VMs.

  • abN, abnormal; AP, arterioportal; AV, arteriovenous; CVM, confluent vascular malformations; HA, hepatic artery; PHT, portal hypertension; PV, portovenous; US, ultrasonography.