Unexpected good results after therapy for anisometropic amblyopia associated with unilateral peripapillary myelinated nerve fibers

Unilateral extensive myelination of the peripapillary nerve fibers may be associated with anisometropic myopia, strabismus, and reduced vision. Despite aggressive occlusion of the normal eye, visual results are often disappointing, presumably due to associated structural abnormalities in the macula which limit visual potential. We report two cases, a 21-month-old child and a 23-month-old child with unilateral peripapillary myelination, ipsilateral high myopia, and dense amblyopia. Despite an abnormal macular reflex in each child, vision improved to 20/30 in one child and 20/50 in the other child after occlusion therapy. Visual results in these patients suggest that aggressive amblyopia therapy should be considered in patients with anisometropic amblyopia associated with extensive myelination continuous with the optic nerve. Not all patients with unilateral peripapillary myelinated nerve fibers, an abnormal macula, and myopia will have refractory amblyopia.