Morphological and functional analyses of adult onset vitelliform macular dystrophy.
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Aim: To evaluate the morphology and visual function of the macula in eyes with adult onset vitelliformmacular dystrophy (AVMD).Methods: 12 eyes of six patients with AVMD were examined by ophthalmoscopy, scanning laser ophthalmoscopy(SLO), optical coherence tomography (OCT), and multifocal electroretinography(mfERGs). The macular lesions were bilateral in all patients and varied from the typical vitelliform (fiveeyes), faded vitelliform changes with retinal pigment epithelium (RPE) atrophy (five eyes), and a normalfovea associated with small flecks around the macula (two eyes).Results: SLO demonstrated small abnormal bright spots in the deep retina throughout the posteriorretina in all cases. OCT showed a highly reflective fusiform thickened layer at the level of the RPE andchoriocapillaris in patients with a submacular yellow vitelliform lesion. A well circumscribed, opticallyclear space was observed beneath the retinal layer in the macular lesions with RPE atrophy. ThemfERGs were significantly reduced not only in the macular area but also in the outermost ring (20–30°)of the mfERGs.Conclusions: The submacular materials that accumulate within the RPE or subepithelial layers reportedin previous histopathological studies of vitelliform lesions can be detected by OCT. In the macularlesions with RPE atrophy, the material may have disappeared leaving a subretinal or subepithelial opticalclear space. These SLO and mfERG observations suggest that the morphological and functionalabnormalities may not be localised just in the macular area but may be present throughout the posteriorpole in eyes with AVMD.東邦大学博士医学
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