Cerebral vascular malformations in hereditary hemorrhagic telangiectasia.
OBJECTIVE
Hereditary hemorrhagic telangiectasia (HHT) is a hereditary disorder characterized by mucocutaneous telangiectasias, frequent nosebleeds, and visceral arteriovenous malformations (AVMs). Few reports have outlined the prevalence of the various cerebral vascular malformations found in patients with HHT. The authors set out to define the prevalence of cerebral vascular malformations in a population of HHT patients who underwent imaging with 3-T imaging (MRI/MR angiography [MRA]) of the brain.


METHODS
A retrospective review of prospectively collected data was carried out using a database of 372 HHT patients who were seen and examined at the Georgia Regents University HHT Center and screened with 3-T MRI/MRA. Data were tabulated for numbers and types of vascular malformations in this population.


RESULTS
Arteriovenous malformations were identified in 7.7%, developmental venous anomalies in 4.3%, and cerebral aneurysms in 2.4% of HHT patients. The HHT AVMs tended to be supratentorial, small, and cortical in this series, findings consistent with other recent studies in the literature. An arteriovenous fistula, cavernous malformation, and capillary telangiectasia were identified in 0.5%, 1%, and 1.9% of HHT patients, respectively.


CONCLUSIONS
Few studies have investigated the prevalence of the various vascular malformations found in HHT patients screened with 3-T MRI/MRA of the brain. Hereditary hemorrhagic telangiectasia AVMs are more likely to be multiple and have a tendency toward small size and cortical location. As such, they are often treated using a single-modality therapy.
Departments of Neurological Surgery.
M Neil
MN
Woodall
Melissa
M
McGettigan
Ramon
R
Figueroa
James R
JR
Gossage
Cargill H
CH
Alleyne
D000328:Adult; D020785:Central Nervous System Vascular Malformations / Q000175:diagnosis* / Q000453:epidemiology* / Q000473:pathology; D002533:Cerebral Angiography; D015897:Comorbidity; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000453:epidemiology* / Q000473:pathology