Ophthalmic Artery Ischemic Syndrome Associated With Neurofibromatosis and Moyamoya Syndrome

Figure 1. Magnetic resonance image and magnetic resonance angiogram. A, Magnetic resonance image of the brain showing severe right-sided hemiatrophy with areas of laminar necrosis in the right frontal (arrow), parietal, occipital, and temporal lobes, indicative of a prior ischemic event. The cerebral ventricles are enlarged due to cerebral atrophy (arrowhead). B, Three-dimensional, time-of flight magnetic resonance angiogram revealing a moderately attenuated right internal carotid artery (ICA) (arrowhead) and severely attenuated right middle (arrow), posterior, and bilateral anterior cerebral arteries. MCA indicates middle cerebral artery.

Figure 2. Retcam (Clarity Medical Systems, Inc) color photograph and fluorescein angiogram. A, Retcam color photograph of the right eye showing a pale optic nerve (arrowhead), attenuated retinal arcade vessels, and severe chorioretinal scarring nasal to the optic nerve (arrow). B, Retcam fluorescein angiogram of the right eye at 1 minute demonstrating attenuation of the retinal vessels (arrowhead) as well as atrophy of the retinal pigment epithelium and choroidal (arrow) vasculature nasal to the optic nerve.