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R. Michels, A. Muellner–Eidenboeck, E. Moser; Incidence and Clinical Aspects of Optic Nerve Hypoplasia in a European University Eye Hospital . Invest. Ophthalmol. Vis. Sci. 2006;47(13):773.
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To evaluate incidence and clinical aspects of optic nerve hypoplasia in a European university eye hospital.
Between 1999 and 2005 44 patients were diagnosed with optic nerve hypoplasia (ONH) and were retrospectively evaluated with regard to visual acuity, complete ophthalmologic exam, prenatal risk factors, time of diagnosis and associated malformations.
38 of 44 patients had bilateral ONH, 5 had unilateral ONH and one patient had no clinically detectable abnormalities but evidence of a septo–optic dysplasia (SOD). 59% of patients were diagnosed before the age of 1. Retinal vascular tortuosity (RVT) seemed to be a predictor for SOD since 10 of the 17 patients with RVT were diagnosed with SOD. The other most common clinical characteristics of ONH were a small optic disc (95,5%), strabism (47.7%), nystagmus (31.8%) and the double ring sign (29.5%). 50% of patients had a visual acuity of less than 20/400 and 38.6% of patients had minimal or no visual impairment.
ONH is the most common congenital malformation of the optic nerve and shows a large variability in clinical and functional aspects.
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