• Lauren O'Loughlin, Mari L Groves, Neil R Miller, and Monica S Pearl.
• Division of Interventional Neuroradiology, The Johns Hopkins University School of Medicine, 1800 Orleans Street, Bloomberg Building Room 7218, Baltimore, MD, 21287, USA.
• Childs Nerv Syst. 2017 Mar 1; 33 (3): 513-516.

AbstractCerebrofacial arteriovenous metameric syndrome (CAMS) is a recent classification of vascular malformations that encompasses a spectrum of phenotypic expression involving arteriovenous malformations (AVMs) of the cerebral, orbital, and facial region. Recognizing the embryologic basis of CAMS is important for diagnosing other AVMs along the same metameric level. Visual loss is the most common presentation prompting ophthalmologic evaluation followed by neuroimaging. We present two pediatric patients with ipsilateral optic nerve and chiasmal AVMs without cutaneous manifestations, characteristic of CAMS 2. The diagnosis of cerebral AVMs was made by magnetic resonance imaging of the brain and confirmed with cerebral angiography. High-resolution flat-panel computed tomography was performed in one patient and was useful to demonstrate the intraneural invasion of the optic nerve by the AVM.

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