AJDRAJNR - American Journal of Neuroradiology

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Angiographic and Clinical Characteristics of Patients with Cerebral Arteriovenous Malformations Associated with Hereditary Hemorrhagic Telangiectasia

Shunji Matsubaraa, Jennifer L. Manziaa, Karel ter BruggeGo,a, Robert A. Willinskya, Walter Montaneraa and Marie E. Faughnana

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.



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FIG 1. Angiograms of a 21-year-old man who presented with a grand mal seizure, a history of epistaxis, lower-lip telangiectasia, and a family history of HHT.

A, Lateral-view angiogram of the right internal carotid artery shows the small AVM involving right parietal lobe cortex filled by the two branches of the right middle cerebral artery with two draining veins (open arrows).

B, Anteroposterior-view angiogram of the vertebral artery shows a second AVM (micro AVM) (arrow), involving the left occipital cortex, fed by a branch of the posterior cerebral artery and draining into a single vein (open arrows).

C, Lateral-view angiogram shows another micro AVM (arrow) involving the right occipital lobe cortex supplied by a parieto-occipital artery with a single draining vein (open arrows).



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FIG 2. Images from the case of a 15-year-old woman who presented with right hemiplegia, a history of recurrent epistaxis, and a family history of HHT. She was also subsequently diagnosed with a pulmonary AVM.

A, Unenhanced CT scan shows an intracerebral hemorrhage and subarachnoid hemorrhage.

B, Lateral-view angiogram of the left internal carotid artery shows an AVF (arrow) involving the left frontal lobe cortex, arising from a middle cerebral artery branch, with cortical drainage and venous pouch (open arrow).

C, Lateral-view angiogram of the right internal carotid artery shows another AVF (arrow) involving the right parietal cortex supplied by the right pericallosal artery with deep drainage and venous pouch (open arrow).