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 of 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 years of age, he developed an acute hemiplegia and was then diagnosed with moyamoya disease. This rare instance of childhood ocular ischemia in conjunction with moyamoya disease and neurofibromatosis demonstrates the serious ocular and systemic sequelae of occlusive vascular disease.

Fundus measurements with indirect ophthalmoscopy. An experimental approach.

We examined the factors influencing the accuracy of indirect ophthalmoscopic estimates of choroidal tumor size by studying indirect ophthalmoscope photographs of solid black domes implanted in the suprachoroidal space of postmortem human eyes. Measurements of the indirect ophthalmoscope photographs showed clinically significant differences in the absolute diameter of the visualized fundus, which varied with differences in condensing lens power and width, anteroposterior location of viewed fundus, and axial length of the experimental eye. Linear regression analysis demonstrated that the field of view of each condensing lens varied similarly with axial length and that the majority of variation in field size for each condensing lens was attributable to axial length variation. Equatorial fields of view averaged 11% smaller than posterior fields of view. The accuracy of estimated absolute intraocular dimensions using indirect ophthalmoscopy potentially approaches +/- 5%.