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Fig.2.33  Albinotic optic disc. Note small size, situs inversus of vessels and abnormal course of retinal vessels (Courtesy of Stephen C. Pollock, M.D.)

Optic nerve hypoplasia would then be inevitable, unless other nerve fiber bundles contained a proportionately larger number of axons. Several histological studies have estimated that animals with albinism have approximately 7% fewer optic nerve fibers than their normally pigmented counterparts.38,95 These findings raise the possibility that optic nerve hypoplasia is a component of albinism. In one study, highresolution MR imaging of the intracranial optic nerves in human albinos shows no diminution in size,53 while a more recent study detected hypoplasia anterior visual pathways.271

Clinically, the diagnosis of mild optic nerve hypoplasia is usually predicated on finding either subnormal visual acuity or visual field abnormalities, which are usually present in albinos by virtue of the associated macular hypoplasia and nystagmus. Because neither ophthalmoscopy nor MR imaging alone can definitively distinguish mild forms of optic nerve hypoplasia from variants of normal, resolution of this controversy awaits neuropathological examination of human albino optic nerves.

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