July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Interocular symmetry of foveal cone topography in achromatopsia (ACHM)
Author Affiliations & Notes
  • Katie M Litts
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Christopher S Langlo
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
    Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Emily J Patterson
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Rebecca Mastey
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Jenna A Cava
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Patrick May
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Byron L Lam
    Bascom Palmer Eye Institute, University of Miami, Miami, Florida, United States
  • Gerald A Fishman
    Pangere Center for Inherited Retinal Disease, The Chicago Lighthouse, Chicago, Illinois, United States
  • Mark E Pennesi
    Casey Eye Institute, Oregon Health & Science University, Portland, Oregon, United States
  • Christine N. Kay
    Vitreoretinal Associates, Gainesville, Florida, United States
  • Alfredo Dubra
    Byers Eye Institute, Stanford University, Palo Alto, California, United States
  • William W Hauswirth
    Ophthalmology, University of Florida, Gainesville, Florida, United States
  • Kathleen N Beasley
    Applied Genetic Technologies Corporation, Alachua, Florida, United States
  • Jeffrey D Chulay
    Applied Genetic Technologies Corporation, Alachua, Florida, United States
  • Joseph Carroll
    Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
    Cell Biology, Neurobiology and Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Footnotes
    Commercial Relationships   Katie Litts, None; Christopher Langlo, None; Emily Patterson, None; Rebecca Mastey, None; Jenna Cava, None; Patrick May, None; Byron Lam, Applied Genetic Technologies Corporation (F); Gerald Fishman, Applied Genetic Technologies Corporation (F); Mark Pennesi, Applied Genetic Technologies Corporation (F); Christine Kay, Applied Genetic Technologies Corporation (F); Alfredo Dubra, 8,226,236 (P), Meira GTx (C); William Hauswirth, Applied Genetic Technologies Corporation (I), Applied Genetic Technologies Corporation (R); Kathleen Beasley, Applied Genetic Technologies Corporation (E); Jeffrey Chulay, Applied Genetic Technologies Corporation (E); Joseph Carroll, Applied Genetic Technologies Corporation (F), Meira GTx (R)
  • Footnotes
    Support  NIH Grants R01EY017607, P30EY001931, R24EY022023, T32EY014537, T32GM080202
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 4992. doi:
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    • Get Citation

      Katie M Litts, Christopher S Langlo, Emily J Patterson, Rebecca Mastey, Jenna A Cava, Patrick May, Byron L Lam, Gerald A Fishman, Mark E Pennesi, Christine N. Kay, Alfredo Dubra, William W Hauswirth, Kathleen N Beasley, Jeffrey D Chulay, Joseph Carroll; Interocular symmetry of foveal cone topography in achromatopsia (ACHM). Invest. Ophthalmol. Vis. Sci. 2018;59(9):4992.

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

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Abstract

Purpose : Interocular symmetry of foveal cone structure in ACHM has received little attention, despite significant variation across patients.[1] The variability between eyes may be important for selection of patients and monitoring outcomes in clinical trials. Here, we assess interocular symmetry of foveal cone topography in ACHM using non-confocal split-detection adaptive optics scanning light ophthalmoscopy (AOSLO).

Methods : Split-detection AOSLO images of the foveal cone mosaic were acquired from both eyes of subjects with CNGA3- or CNGB3-associated ACHM (n = 4 and 16, respectively). Cones were identified manually or semi-automatically[2] within a manually-delineated rod-free zone.[1] Peak cone density was determined using a 55 x 55 µm sampling window. Inter-cell distance (ICD) between a cell and its immediate neighbors was calculated for each cell in the rod-free zone. The mean and standard deviation (SD) of ICD for each mosaic was calculated to derive the coefficient of variation (CV).

Results : Representative images of remnant cone structure in both eyes of subjects with CNGA3- and CNGB3-associated ACHM are shown (Figure). Peak cone density (mean ± SD: 19,868 ± 12,259 and 18,744 ± 11,508 cones/mm2 for OD and OS, respectively) was similar between eyes with ACHM (p = 0.35, Wilcoxon test). ICD (mean ± SD: 12.98 ± 4.37 µm for OD and 12.76 ± 4.04 µm for OS) was not different between eyes with ACHM (p = 0.57, paired t-test). CV values (mean ± SD: 0.28 ± 0.05 for OD and 0.26 ± 0.06 for OS) demonstrate similar mosaic topography between eyes (p = 0.29, paired t-test).

Conclusions : These results demonstrate high interocular symmetry of the foveal cone mosaic (density and packing) in ACHM. This is consistent with data showing high interocular symmetry of foveal outer nuclear layer thickness in ACHM.[3] The results are of potential value in longitudinal monitoring of patients during treatment trials and further suggest that both eyes of a given subject may have similar therapeutic potential.

[1] PMID: 27479814
[2] PMID: 27231641
[3] 2017 ARVO E-Abstract 6005

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

 

Figure. Remnant foveal cone structure in ACHM. In a subject with CNGA3-associated ACHM (top), peak cone density OD is 9,917 cones/mm2 and OS is 9,917 cones/mm2. In a subject with CNGB3-associated ACHM (bottom), peak cone density OD is 13,223 cones/mm2 and OS is 12,893 cones/mm2.

Figure. Remnant foveal cone structure in ACHM. In a subject with CNGA3-associated ACHM (top), peak cone density OD is 9,917 cones/mm2 and OS is 9,917 cones/mm2. In a subject with CNGB3-associated ACHM (bottom), peak cone density OD is 13,223 cones/mm2 and OS is 12,893 cones/mm2.

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