American Journal of Neuroradiology 21:1016-1020 (6 2000)
© 2000 American Society of Neuroradiology
ARTICLE
Angiographic and Clinical Characteristics of Patients with Cerebral Arteriovenous Malformations Associated with Hereditary Hemorrhagic Telangiectasia
a From the Department of Medical Imaging (S.M., K.t.B., R.A.W., W.M.), The Toronto Western Hospital and Brain Vascular Malformation Study Group, University of Toronto, Toronto, Canada; the Department of Neurological Surgery (S.M.), School of Medicine, the University of Tokushima, Tokushima, Japan; and the Department of Medicine (J.L.M., M.E.F.), Division of Respirology, St. Michael's Hospital (Wellesley Central Site), Toronto, Ontario, Canada.
BACKGROUND AND PURPOSE: Cerebral arteriovenous malformations (AVMs) are occasionally associated with hereditary hemorrhagic telangiectasia (HHT), which is characterized by the presence of multiple mucocutaneous telangiectasia, epistaxis, and familial inheritance. We analyzed the angiographic and clinical characteristics of patients with cerebral AVMs related to HHT.
METHODS: Among 638 patients with cerebral AVMs, we identified 14 patients with HHT. The AVMs were classified as those with nidi of 1 cm or less (micro AVMs), those with nidi between 1 and 3 cm (small AVMs), and those of the fistulous type (arteriovenous fistulas [AVFs]).
RESULTS: A total of 28 AVMs were found; seven of 14 patients had multiple AVMs. The 28 AVMs were categorized as 12 micro AVMs, eight small AVMs, and eight AVFs. All except one micro AVM were asymptomatic, whereas all small AVMs were symptomatic. Three of eight AVFs were asymptomatic. All 28 AVMs were located on the cortex. All micro AVMs and AVFs had single feeders and single draining veins, whereas the small AVMs had multiple feeders in all lesions and single draining veins in six of eight lesions.
CONCLUSION: Multiple, cortical, micro AVMs or AVFs harboring single feeding arteries and single draining veins should raise clinical suspicion of HHT-related AVMs.
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