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K.L. Mayer, T.J. Mcculley, E.J. Holland, J. Simpson; Congenital Aniridia and Optic Nerve Hypoplasia . Invest. Ophthalmol. Vis. Sci. 2003;44(13):620.
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Purpose: This study evaluates the prevalence of optic nerve hypoplasia and its association with foveal hypoplasia in patients with congenital aniridia. Methods: The medical records of 56 patients with congenital aniridia (31 female, 25 male, mean age 33 years, range 2 to 74 years) were evaluated in a retrospective chart review for the presence of optic nerve hypoplasia and foveal hypoplasia. Results: Six of 56 patients (10.7%) had optic nerve hypoplasia which was found in both eyes of 5 binocular patients and in one monocular patient. The prevalence of foveal hypoplasia was higher in aniridia patients with optic nerve hypoplasia than in those without (50.0% vs. 6.0%); this difference did not achieve statistical significance. Conclusions: Clinically apparant optic nerve hypoplasia is found in roughly 10% of patients with aniridia and may occur independently or in association with foveal hypoplasia.
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