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Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy

Dark cone photoreceptors, defined as those with diminished or absent reflectivity when observed with adaptive optics (AO) ophthalmoscopy, are increasingly reported in retinal disorders. However, their structural and functional impact remain unclear. Here, we report a 3-year longitudinal study on a p...

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Autores principales: Li, Joanne, Liu, Tao, Flynn, Oliver J., Turriff, Amy, Liu, Zhuolin, Ullah, Ehsan, Liu, Jianfei, Dubra, Alfredo, Johnson, Mary A., Brooks, Brian P., Hufnagel, Robert B., Hammer, Daniel X., Huryn, Laryssa A., Jeffrey, Brett G., Tam, Johnny
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7985087/
https://www.ncbi.nlm.nih.gov/pubmed/33767618
http://dx.doi.org/10.3389/fnagi.2021.629214
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author Li, Joanne
Liu, Tao
Flynn, Oliver J.
Turriff, Amy
Liu, Zhuolin
Ullah, Ehsan
Liu, Jianfei
Dubra, Alfredo
Johnson, Mary A.
Brooks, Brian P.
Hufnagel, Robert B.
Hammer, Daniel X.
Huryn, Laryssa A.
Jeffrey, Brett G.
Tam, Johnny
author_facet Li, Joanne
Liu, Tao
Flynn, Oliver J.
Turriff, Amy
Liu, Zhuolin
Ullah, Ehsan
Liu, Jianfei
Dubra, Alfredo
Johnson, Mary A.
Brooks, Brian P.
Hufnagel, Robert B.
Hammer, Daniel X.
Huryn, Laryssa A.
Jeffrey, Brett G.
Tam, Johnny
author_sort Li, Joanne
collection PubMed
description Dark cone photoreceptors, defined as those with diminished or absent reflectivity when observed with adaptive optics (AO) ophthalmoscopy, are increasingly reported in retinal disorders. However, their structural and functional impact remain unclear. Here, we report a 3-year longitudinal study on a patient with oligocone trichromacy (OT) who presented with persistent, widespread dark cones within and near the macula. Diminished electroretinogram (ERG) cone but normal ERG rod responses together with normal color vision confirmed the OT diagnosis. In addition, the patient had normal to near normal visual acuity and retinal sensitivity. Occasional dark gaps in the photoreceptor layer were observed on optical coherence tomography, in agreement with reflectance AO scanning light ophthalmoscopy, which revealed that over 50% of the cones in the fovea were dark, increasing to 74% at 10° eccentricity. In addition, the cone density was 78% lower than normal histologic value at the fovea, and 20–40% lower at eccentricities of 5–15°. Interestingly, color vision testing was near normal at locations where cones were predominantly dark. These findings illustrate how a retina with predominant dark cones that persist over at least 3 years can support near normal central retinal function. Furthermore, this study adds to the growing evidence that cones can continue to survive under non-ideal conditions.
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spelling pubmed-79850872021-03-24 Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy Li, Joanne Liu, Tao Flynn, Oliver J. Turriff, Amy Liu, Zhuolin Ullah, Ehsan Liu, Jianfei Dubra, Alfredo Johnson, Mary A. Brooks, Brian P. Hufnagel, Robert B. Hammer, Daniel X. Huryn, Laryssa A. Jeffrey, Brett G. Tam, Johnny Front Aging Neurosci Neuroscience Dark cone photoreceptors, defined as those with diminished or absent reflectivity when observed with adaptive optics (AO) ophthalmoscopy, are increasingly reported in retinal disorders. However, their structural and functional impact remain unclear. Here, we report a 3-year longitudinal study on a patient with oligocone trichromacy (OT) who presented with persistent, widespread dark cones within and near the macula. Diminished electroretinogram (ERG) cone but normal ERG rod responses together with normal color vision confirmed the OT diagnosis. In addition, the patient had normal to near normal visual acuity and retinal sensitivity. Occasional dark gaps in the photoreceptor layer were observed on optical coherence tomography, in agreement with reflectance AO scanning light ophthalmoscopy, which revealed that over 50% of the cones in the fovea were dark, increasing to 74% at 10° eccentricity. In addition, the cone density was 78% lower than normal histologic value at the fovea, and 20–40% lower at eccentricities of 5–15°. Interestingly, color vision testing was near normal at locations where cones were predominantly dark. These findings illustrate how a retina with predominant dark cones that persist over at least 3 years can support near normal central retinal function. Furthermore, this study adds to the growing evidence that cones can continue to survive under non-ideal conditions. Frontiers Media S.A. 2021-03-09 /pmc/articles/PMC7985087/ /pubmed/33767618 http://dx.doi.org/10.3389/fnagi.2021.629214 Text en Copyright © 2021 Li, Liu, Flynn, Turriff, Liu, Ullah, Liu, Dubra, Johnson, Brooks, Hufnagel, Hammer, Huryn, Jeffrey and Tam. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Li, Joanne
Liu, Tao
Flynn, Oliver J.
Turriff, Amy
Liu, Zhuolin
Ullah, Ehsan
Liu, Jianfei
Dubra, Alfredo
Johnson, Mary A.
Brooks, Brian P.
Hufnagel, Robert B.
Hammer, Daniel X.
Huryn, Laryssa A.
Jeffrey, Brett G.
Tam, Johnny
Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy
title Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy
title_full Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy
title_fullStr Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy
title_full_unstemmed Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy
title_short Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy
title_sort persistent dark cones in oligocone trichromacy revealed by multimodal adaptive optics ophthalmoscopy
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7985087/
https://www.ncbi.nlm.nih.gov/pubmed/33767618
http://dx.doi.org/10.3389/fnagi.2021.629214
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