Supernormal foveal photoreceptor density in Alport syndrome: A case report.
作者信息
Ameln Julius, Reiniger Jenny L, Hess Kristina, Holz Frank G, Harmening Wolf M
机构信息
Department of Ophthalmology, University of Bonn, Bonn, Germany.
Division of Epidemiology and Clinical Research, National Eye Institute, National Institute of Health, Bethesda, MD, USA.
出版信息
Eur J Ophthalmol. 2023 Jul;33(4):NP51-NP54. doi: 10.1177/11206721221093197. Epub 2022 Apr 12.
PURPOSE
To investigate foveal photoreceptor configuration in Alport syndrome, a rare inherited disease characterized by Collagen IV dysfunction.
METHODS
Adaptive optics scanning laser ophthalmoscope (AOSLO) imaging of the foveal center and quantitative analysis of cone photoreceptor topography in a 17-year-old male patient with Alport syndrome presenting absence of a foveal avascular zone (FAZ) and foveal hypoplasia in both eyes.
RESULTS
Cone density analysis based on AOSLO images revealed an unusual linear cone topography profile displaying supernormal densities within the fovea (z-scores up to + 3.57 and + 2.97 in right and left eyes, respectively).
CONCLUSION
Foveal hypoplasia has previously been associated with normal or reduced cone density. Our observation is the first case of disease-related supernormal cone density within the foveola, shedding light upon the role of Collagen IV in foveal maturation.
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