July 2019
Volume 60, Issue 9
Free
ARVO Annual Meeting Abstract  |   July 2019
Structural Markers Predictive of Visual Acuity in Choroideremia
Author Affiliations & Notes
  • Kurt Scavelli
    Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Drew Scoles
    Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Leona Serrano
    Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Katherine E. Uyhazi
    Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Jessica Ijams Wolfing Morgan
    Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Tomas S Aleman
    Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Footnotes
    Commercial Relationships   Kurt Scavelli, None; Drew Scoles, None; Leona Serrano, None; Katherine Uyhazi, None; Jessica Morgan, AGTC:Code F (F), US Patent 8226236 (P); Tomas Aleman, None
  • Footnotes
    Support  National Institutes of Health (NEI-K12EY015398-10, NIH R01EY028601), Research to Prevent Blindness, Foundation Fighting Blindness, Hope for Vision, Macula Vision Research, the Paul and Evanina Bell Mackall Foundation Trust and The Pennsylvania Lions Sight Conservation
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 4523. doi:
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    • Get Citation

      Kurt Scavelli, Drew Scoles, Leona Serrano, Katherine E. Uyhazi, Jessica Ijams Wolfing Morgan, Tomas S Aleman; Structural Markers Predictive of Visual Acuity in Choroideremia. Invest. Ophthalmol. Vis. Sci. 2019;60(9):4523.

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

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Abstract

Purpose : To correlate changes in retinal structure with visual function in choroideremia (CHM).

Methods : Patients with CHM, ages 6 to 82 years were evaluated by ocular examination. Retinal imaging was performed with spectral domain optical coherence tomography (SD-OCT). SD-OCT analysis included measurement of the horizontal extent of uninterrupted inner segment ellipsoid zone (EZ), length of the posterior outer segment (POS), and foveal thickness. These values were then correlated to visual acuity and visual thresholds measured with automated static perimetry.

Results : One hundred and twenty five molecularly confirmed CHM patients were included. Twenty-two of these patients had longitudinal follow up spanning 11 months to four years. The median rate of change in the total horizontal extent of the ellipsoid zone was a loss of 197 um/year. Visual acuity inversely correlated with shortening of the POS, central foveal thickness, and the extent of the ellipsoid zone. There was an inverse relationship between visual acuity and foveal sensitivity.

Conclusions : Shortening of the cone POS, thinning of the central fovea and centripetal movement of transition zones of severe structural abnormalities towards the fovea correlated with decline in visual acuity in CHM. These are parameters that may be used to monitor disease progression and predict visual acuity decline. The results have implications for the planning of future gene therapy trials that intend to target the central retina.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

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