Optic nerve hypoplasia is a relatively common, non-progressive, congenital anomaly of the optic nerve causing impaired vision, often manifesting as strabismic amblyopia. It is thought to be a failure of ganglion cell axon migration during embryological development of the eye as a consequence to any insult to the CNS resulting in damage to the developing optic nerve. Three cases of optic nerve hypoplasia are presented demonstrating variable visual profiles. The first case is a 22 year old female with esotropia and Tilted Disk Syndrome. The second is a 6 year old female with esotropia and bilateral optic nerve hypoplasia where occlusion patch therapy was unsuccessful due to the hypoplasia. The final case is a 53 year-old female without strabismus, but a largely reduced visual field consequently causing a functional vision loss. Tips for diagnosis and testing are also offered, stressing the importance of a thorough vision exam. Particularly close attention should be made to Disk-Macula/Disc Diameter ratio, optic nerve head evaluation (pallor with double-ring sign,) visual fields and diagnostic patching.
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