Ocular Ischemic Syndrome due to Internal Carotid Artery Occlusion with Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF-1) is associated with multiple vascular abnormalities, including internal carotid artery (ICA) stenosis/occlusion. Depending on the site of stenosis/occlusion of the ICA, both cerebral circulation and ocular circulation can be impaired. We describe a rare pediatric case of ocular ischemic syndrome (OIS) due to ICA occlusion in NF-1. The patient diagnosed with NF-1 suffered right ICA occlusion at 12 years of age, and developed right dense vitreous hemorrhage due to OIS at 13 years of age. The patient underwent right cerebral revascularization surgery to improve cerebral and ocular ischemia, but the visual acuity of the right eye did not improve. This case suggests that attention should be paid not only to cerebral ischemia but also to ocular ischemia in patients with ICA stenosis/occlusion of NF-1. Surgical interventions such as cerebral revascularization surgery should be considered in the early stages of OIS to protect visual function, and careful follow-up is required.