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Samira Anwar, Sarim Mohammad, Muhammad Babar Elahi, Irene Gottlob; Optic Nerve Hypoplasia in Association with Retinal Changes. Invest. Ophthalmol. Vis. Sci. 2011;52(14):2990.
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Optic nerve hypoplasia (ONH) refers to the clinical appearance of a small optic nerve which may be unilateral or bilateral. Little is known about the macular architecture in ONH. The purpose of this study was to investigate affected individuals by examining the retinal appearance using ultra high resolution optical coherence tomography (UHR-OCT).
We describe four patients with isolated unilateral optic nerve hypoplasia and normal neuroimaging in whom we have investigated UHR-OCT appearances of the macula and compared it to the unaffected normal fellow eye. The average age of all four patients was 11.75 years with range from 10 years to 13 years. There was 1 right eye and 3 left eyes clinically affected with optic nerve hypoplasia. Corrected distance visual acuities (VA) in the normal clinically unaffected eye ranged from 0.0 to 0.1 (LogMar) and VA in affected eyes with optic nerve hypoplasia ranged from 1.0 (LogMar) to PL. In all patients a relative afferent pupil defect (RAPD) was noted in the affected eye. Spectral optical coherence tomography (SOCT) ((UHR-OCT) (SOCT Copernicus, 94 OPTOPOL Technology S.A., Zawiercie, Poland) was performed in both eyes for each patient.
In all four patients the retinal layers were thinner than normal in the affected eye. The retinal nerve fibre layer appeared to be absent and the retinal ganglion cell layer was difficult to distinguish. In 2 out of 4 patients, there was complete absence of the foveal pit. In the unaffected eye of all four patients the foveal pit was present but the overall retinal thickness was reduced. However in all eyes examined the photoreceptor layer appeared to be normal.
In ONH the retinal morphology is significantly abnormal. This involves mostly the inner retinal layers. The foveal architecture may be severely affected as described above. Inner retinal layer abnormalities were also noted in the clinically unaffected fellow eye with normal vision but to a lesser degree.
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