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Images in Neurology |

Multiple Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia Complicated by Embolic Strokes

Julien Morier, MD; Vasco Goncalves-Matoso, MD; Patrik Michel, MD
Arch Neurol. 2011;68(5):672. doi:10.1001/archneurol.2011.96.
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In 2008, a 67-year-old woman experienced a left posterior choroidal artery stroke with right motor and sensitive hemiparesis (National Institutes of Health Stroke Scale score, 5). Her medical history included migraine with visual aura, major epistaxis since childhood, an isolated pulmonary arteriovenous malformation (PAVM) surgically removed in 1969, and a diagnosis of hereditary hemorrhagic telangiectasia (HHT) syndrome1 based on recurrent epistaxis, New York Heart Association Functional Classification II dyspnea, digital clubbing, and cutaneous telangiectases. Clinical examination uncovered cutaneous telangiectases on the face, lips, and fingertips (Figure 1) as well as digital clubbing associated with a relative cyanosis of extremities. Cerebral computed tomography and magnetic resonance imaging showed asymptomatic infarcts in the left cerebellum and right basal ganglia, confirmed the left posterior choroidal artery stroke (Figure 2B), and excluded any intracranial vascular malformations. Chest x-ray raised the suspicion of multiple PAVMs, which was confirmed on thoracic computed tomography (Figure 2A). Contrast echocardiography and transcranial Doppler showed a massive right-to-left shunt. A splenic aneurysm and a hepatic arteriovenous fistula were also discovered.

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Figure 1.

A, Cutaneous telangiectases are seen on the face, oral mucosa, and fingertips. B, Digital clubbing can also be seen.

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Figure 2.

A, Multiple pulmonary arteriovenous malformations at the arterial (in red) and venous (in purple) junctions (arrows) are shown using computed tomography. B, Posterior choroidal artery stroke is shown on diffusion-weighted magnetic resonance imaging.

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