To evaluate the prevalence of magnetic resonance angiography (MRA) findings and clinically characterize neonates with arterial ischemic stroke (AIS) who have abnormal or variable vasculature.

This was a single-center, retrospective study of patients with neonatal stroke from 1991 to 2012. We reviewed charts and neuroimaging, including MRA, in neonates with AIS. Clinical data of patients with MRA findings were compared with the control group of neonates with AIS and a normal MRA.

We identified 142 cases of neonatal AIS, of which 81 patients had magnetic resonance imaging and MRA. Among the neonates with arterial neuroimaging, 29 had arterial findings (for a prevalence rate of 20%-35%). The majority of the findings were stenotic or hypoplastic branches. Two patients had presumed carotid artery dissection. Low Apgar scores and the presence of sepsis were significantly (P < .05) more common in neonates with MRA findings.

The prevalence of arterial abnormalities or variations in neonatal AIS has been underestimated because neurovascular imaging is often not performed. We recommend an MRA for neonates with AIS, particularly those who have low Apgar scores and/or sepsis, to rule out a vasculopathy that may warrant therapeutic intervention.