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Photo Essay
August 2003

Myelination of the Macula Associated With Disabling Photophobia

Arch Ophthalmol. 2003;121(8):1204-1205. doi:10.1001/archopht.121.8.1204

A 7-YEAR-OLD Latino boy with infantile esotropia complained of extreme glare and said that his "eyes were not straight." He wore a very large-billed hat whenever outdoors. He noted that photophobia was eliminated by patching the affected eye, but preferred not wearing the patch for cosmetic reasons. His visual acuity was counting fingers at 1 ft OD, and 20/20 OS. The pupils were normal, with no relative afferent pupillary defect.

Cycloplegic retinoscopy revealed a white reflex without a streak in the right eye and +1.50 OS. He was unable to perform manifest refraction or pseudoisochromatic color plate testing in the right eye because of his poor visual acuity. Myelination of the retinal nerve fibers covered the macula, vascular arcades, and midperiphery, but the fovea was spared (Figure 1). The optic disc was entirely engulfed in myelin. The left fundus was normal.

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