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...
| Main Authors: | , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2021-03-01
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| Series: | Frontiers in Aging Neuroscience |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fnagi.2021.629214/full |
| _version_ | 1818439081458139136 |
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| author | Joanne Li Tao Liu Oliver J. Flynn Amy Turriff Zhuolin Liu Ehsan Ullah Jianfei Liu Alfredo Dubra Mary A. Johnson Brian P. Brooks Robert B. Hufnagel Daniel X. Hammer Laryssa A. Huryn Brett G. Jeffrey Johnny Tam |
| author_facet | Joanne Li Tao Liu Oliver J. Flynn Amy Turriff Zhuolin Liu Ehsan Ullah Jianfei Liu Alfredo Dubra Mary A. Johnson Brian P. Brooks Robert B. Hufnagel Daniel X. Hammer Laryssa A. Huryn Brett G. Jeffrey Johnny Tam |
| author_sort | Joanne Li |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-14T17:50:47Z |
| format | Article |
| id | doaj.art-d2b4053401d44236a0a22e03905cf2a9 |
| institution | Directory Open Access Journal |
| issn | 1663-4365 |
| language | English |
| last_indexed | 2024-12-14T17:50:47Z |
| publishDate | 2021-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Aging Neuroscience |
| spelling | doaj.art-d2b4053401d44236a0a22e03905cf2a92022-12-21T22:52:38ZengFrontiers Media S.A.Frontiers in Aging Neuroscience1663-43652021-03-011310.3389/fnagi.2021.629214629214Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics OphthalmoscopyJoanne Li0Tao Liu1Oliver J. Flynn2Amy Turriff3Zhuolin Liu4Ehsan Ullah5Jianfei Liu6Alfredo Dubra7Mary A. Johnson8Brian P. Brooks9Robert B. Hufnagel10Daniel X. Hammer11Laryssa A. Huryn12Brett G. Jeffrey13Johnny Tam14National Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesCenter for Devices and Radiological Health, U.S. Food and Drug Administration, Silver Spring, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesDepartment of Ophthalmology, Stanford University, Palo Alto, CA, Unites StatesOphthalmology and Visual Sciences, University of Maryland School of Medicine, Baltimore, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesCenter for Devices and Radiological Health, U.S. Food and Drug Administration, Silver Spring, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesNational Eye Institute, National Institutes of Health, Bethesda, MD, United StatesDark 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.https://www.frontiersin.org/articles/10.3389/fnagi.2021.629214/fulladaptive opticsscanning laser ophthalmoscopyoptical coherence tomographydark conesvisual functioncolor vision |
| spellingShingle | Joanne Li Tao Liu Oliver J. Flynn Amy Turriff Zhuolin Liu Ehsan Ullah Jianfei Liu Alfredo Dubra Mary A. Johnson Brian P. Brooks Robert B. Hufnagel Daniel X. Hammer Laryssa A. Huryn Brett G. Jeffrey Johnny Tam Persistent Dark Cones in Oligocone Trichromacy Revealed by Multimodal Adaptive Optics Ophthalmoscopy Frontiers in Aging Neuroscience adaptive optics scanning laser ophthalmoscopy optical coherence tomography dark cones visual function color vision |
| 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 | adaptive optics scanning laser ophthalmoscopy optical coherence tomography dark cones visual function color vision |
| url | https://www.frontiersin.org/articles/10.3389/fnagi.2021.629214/full |
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