CLINICAL SIGNIFICANCE OF RETINAL VASCULAR OCCLUSION IN MOYAMOYA DISEASE: Case Series and Systematic Review.

PURPOSE: Although moyamoya disease primarily affects the carotid artery in the ophthalmic artery bifurcation area, retinal vascular abnormalities in moyamoya disease have rarely been reported. The purpose of this report is to describe clinical findings of patients with retinal vascular occlusion in patients with moyamoya disease and present its clinical significance. METHODS: We reviewed and analyzed patients with moyamoya disease with retinal vascular occlusions. For this, a retrospective medical chart review was performed in a tertiary medical center and a literature search was performed using PubMed and EMBASE until September 2020. RESULTS: Patients with retinal artery occlusion (RAO) were significantly younger than patients with retinal vein occlusion (25.0 vs. 40.1 years, P = 0.023). Of 14 patients, retinal vascular occlusion was the presenting sign of moyamoya disease in 8 (57.1%) patients. The occlusion site at the carotid artery was proximal to the ophthalmic artery bifurcation area in 8 (57.1%) patients. Legal blindness occurred in 8 (57.1%) patients at final visits. CONCLUSION: Retinal vascular occlusion is a rare but sight-threatening ocular complication in patients with moyamoya disease. Overall, younger age may be a risk factor for RAO, whereas older age for retinal vein occlusion. Retinal vascular occlusion can be an important indicator of moyamoya disease screening, especially in relatively younger and healthy patients.