June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Reliability of Counting Cone Inner Segments in Choroideremia
Author Affiliations & Notes
  • Grace K. Han
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Robert F Cooper
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
    Department of Psychology, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Drew Scoles
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Jean Bennett
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, Department of Ophthalmology, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Albert M Maguire
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, Department of Ophthalmology, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Tomas S Aleman
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, Department of Ophthalmology, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Jessica Ijams Wolfing Morgan
    Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States
    Center for Advanced Retinal and Ophthalmic Therapeutics, Department of Ophthalmology, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Footnotes
    Commercial Relationships   Grace Han, None; Robert Cooper, None; Drew Scoles, None; Jean Bennett, Biogen (F), Gensight Biologics (S), Limelight (F), Sanofi (C), Spark Therapeutics (P), Spark Therapeutics (S); Albert Maguire, Spark Therapeutics (F); Tomas Aleman, None; Jessica Morgan, AGTC (F), Canon, Inc. (F), University of Rochester US Patent 8226236 (P)
  • Footnotes
    Support  NIH U01EY025477, U01EY025864, Foundation Fighting Blindness, Research to Prevent Blindness Stein Innovation Award, the F. M. Kirby Foundation, the Paul and Evanina Mackall Foundation Trust.
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 1258. doi:
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    • Get Citation

      Grace K. Han, Robert F Cooper, Drew Scoles, Jean Bennett, Albert M Maguire, Tomas S Aleman, Jessica Ijams Wolfing Morgan; Reliability of Counting Cone Inner Segments in Choroideremia. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1258.

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

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Abstract

Purpose : Recent advances in adaptive optics scanning light ophthalmoscopy (AOSLO) have allowed the visualization of cone inner segments (IS) and enabled quantification of IS cone density in normal and diseased retina (Scoles, et al. IOVS, 2014). Understanding the reliability of IS cone density measurements is essential for using changes in IS cone density as an outcome measure in longitudinal studies of disease progression and assessment of experimental intervention. Here, we examine the inter-observer reliability for measuring IS cone density in patients with Choroideremia.

Methods : The central two mm of 11 eyes from 11 Choroideremia subjects age 9 to 37 were imaged along the horizontal and vertical meridians using nonconfocal split detection AOSLO. AOSLO images were semi-automatically montaged using a custom MATLAB algorithm (Chen, et al. Biomed. Optics Ex., 2016). Regions of interest (ROIs) containing cone IS were selected at 190, 350, 500, and 900 μm in all four directions from the fovea. An ROI was included for density analysis if the image fell within the patient’s central island of intact retina but excluded if the ROI fell outside of the atrophic border, resulting in 136 ROIs. Three trained observers (previously shown to exhibit high agreement in normal images, Han G, et al. IOVS 2016;57:ARVO E-abstract 4640) manually identified the cone IS using custom software. Cone density was extracted from each ROI, and intraclass correlation coefficients with 95% confidence intervals (CI) were calculated to assess the inter-observer agreement in cone density measurements.

Results : Cone IS density was consistent with our previously reported cone densities in Choroideremia. Inter-observer agreement for cone density measurements was good at 350 and 500 μm (0.684, CI 0.526-0.810 and 0.741, CI 0.0.594-0.851) and moderate at 190 and 900 μm (0.543, CI 0.372-0.696 and 0.527, CI 0.283-0.739). Overall inter-observer agreement in cone density was lower for Choroideremia images than for normal images.

Conclusions : The dependence on inter-observer agreement with retinal eccentricity is consistent with the expected difficulty associated with identifying cone IS at different retinal locations in Choroideremia. Cone identification appeared consistent within observers, however further study of intra-observer repeatability is required. These studies will be important for using cone density as an outcome measure to assess disease progression in Choroideremia.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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