OCULAR ISCHEMIC SYNDROME IN A CHILD WITH MOYAMOYA DISEASE AND NEUROFIBROMATOSIS

Ocular ischemic syndrome is extremely rare in childhood. Patients with moyamoya disease may be particularly susceptible to the development o f ocular ischemia due to the associated carotid occlusion. A 19-month- old boy presented with neurofibromatosis and signs of ocular ischemia. At 29 months of age, he developed dense right vitreous hemorrhage and eventually lost vision in that eye due to phthisis. At almost six yea rs of age, he developed an acute hemiplegia and tvas then diagnosed wi th moyamoya disease. This rare instance of childhood ocular ischemia i n conjunction with moyamoya disease and neurofibromatosis demonstrates the serious ocular and systemic sequelae of occlusive vascular diseas e.