June 2020
Volume 61, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2020
Functional consequences of disrupted cone photoreceptor reflectivity visualized using multimodal adaptive optics retinal imaging in oligocone trichromacy
Author Affiliations & Notes
  • Joanne Li
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Tao Liu
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Oliver Flynn
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Amy Turriff
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Zhuolin Liu
    Center for Devices and Radiological Health, U.S. Food and Drug Administration, Silver Spring, Maryland, United States
  • Jianfei Liu
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Alfredo Dubra
    Department of Ophthalmology, Stanford University, Palo Alto, California, United States
  • Robert Hufnagel
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Mary A Johnson
    Department of Ophthalmology & Visual Sciences, University of Maryland School of Medicine, Baltimore, Maryland, United States
  • Brian Patrick Brooks
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Daniel X. Hammer
    Center for Devices and Radiological Health, U.S. Food and Drug Administration, Silver Spring, Maryland, United States
  • Laryssa Huryn
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Brett G Jeffrey
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Johnny Tam
    National Eye Institute, National Institutes of Health, Bethesda, Maryland, United States
  • Footnotes
    Commercial Relationships   Joanne Li, None; Tao Liu, None; Oliver Flynn, None; Amy Turriff, None; Zhuolin Liu, None; Jianfei Liu, None; Alfredo Dubra, None; Robert Hufnagel, None; Mary Johnson, None; Brian Brooks, None; Daniel Hammer, None; Laryssa Huryn, None; Brett Jeffrey, None; Johnny Tam, None
  • Footnotes
    Support  Intramural Research Program of the National Eye Institute, National Institutes of Health
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 3026. doi:
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      Joanne Li, Tao Liu, Oliver Flynn, Amy Turriff, Zhuolin Liu, Jianfei Liu, Alfredo Dubra, Robert Hufnagel, Mary A Johnson, Brian Patrick Brooks, Daniel X. Hammer, Laryssa Huryn, Brett G Jeffrey, Johnny Tam; Functional consequences of disrupted cone photoreceptor reflectivity visualized using multimodal adaptive optics retinal imaging in oligocone trichromacy. Invest. Ophthalmol. Vis. Sci. 2020;61(7):3026.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Dark cones are photoreceptors that retain inner segments despite disrupted waveguiding properties, which may be attributed to partial disruption of the cone outer segments. Although dark cones have been observed in numerous conditions, their clinical significance remains uncertain. In this study, we longitudinally followed and evaluated the presence and impact of dark cones discovered in a patient with oligocone trichromacy (OT) using multimodal adaptive optics (AO) imaging alongside clinical assessments.

Methods : A patient with OT was imaged annually for 3 years with a custom-built multimodal AO imaging system, which incorporates confocal reflectance, nonconfocal split detection, and spectral-domain optical coherence tomography (SD-OCT) modes. The proportion of dark cones was determined using spatially co-registered confocal reflectance and split detection images to assess cone reflectivity and inner segment status, respectively. Cone inner segment spacing and density were quantified. In addition, this patient underwent functional clinical assessments, including visual acuity (VA), electroretinogram (ERG), and color vision tests (Hardy-Rand-Rittler, anomaloscope, and Cambridge Color Test).

Results : Overall, dark cones were observed throughout the retina, comprising more than 50% of the total imaged cones. These dark cones were relatively stable, persisting across visits. At non-foveal regions, the proportion of dark cones was 10-20% higher than in the fovea, with corresponding apparent disruptions in the outer retinal layers on AO SD-OCT. On average, quantitative assessment of cone inner segments in the fovea showed that cone spacing was approximately two-fold higher when compared to published normative data (73% decrease in cone density). The results of functional clinical assessments, in which bilateral best corrected VA was 20/32, cone ERG responses were reduced, and color vision was normal at both central and peripheral locations, suggested that cone function is only partially disrupted.

Conclusions : There is a widespread population of persistent dark cones identified in this patient with OT. Despite the disrupted cone structure, color vision is maintained across eccentricities. This characterization of the structure and function of dark cones represents an important step toward understanding their clinical significance.

This is a 2020 ARVO Annual Meeting abstract.

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