June 2021
Volume 62, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2021
Adaptive optics scanning laser ophthalmoscopy (AOSLO) measures in USH2A-related retinal degeneration
Author Affiliations & Notes
  • Jacque L Duncan
    University of California San Francisco, San Francisco, California, United States
  • Wendi Liang
    Jaeb Centre for Health Research, Tampa, Florida, United States
  • Joseph Carroll
    Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Jenna Cava
    Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Jessica Wong
    University of California San Francisco, San Francisco, California, United States
  • Michel Michaelides
    Moorfields Eye Hospital NHS Foundation Trust, London, London, United Kingdom
    University College London, London, London, United Kingdom
  • Angelos Kalitzeos
    Moorfields Eye Hospital NHS Foundation Trust, London, London, United Kingdom
    University College London, London, London, United Kingdom
  • Isabelle Audo
    Institut de la vision, Paris, Île-de-France, France
    Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, Île-de-France, France
  • Kate Grieve
    Institut de la vision, Paris, Île-de-France, France
    Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, Île-de-France, France
  • Natalie Norberg
    Institut de la vision, Paris, Île-de-France, France
    Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, Île-de-France, France
  • Michel Paques
    Institut de la vision, Paris, Île-de-France, France
    Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, Île-de-France, France
  • Allison Ayala
    Jaeb Centre for Health Research, Tampa, Florida, United States
  • Travis C Porco
    University of California San Francisco, San Francisco, California, United States
  • Footnotes
    Commercial Relationships   Jacque Duncan, 4D Therapeutics (C), Acucela (F), AGTC (C), Allergan/Abbvie (F), Astellas (C), Biogen/Nightstarx Therapeutics (F), DTx Therapeutics (C), Editas (C), Eloxx (C), Eyevensys (C), Gyroscope (C), Horama (C), Neurotech USA (F), Partner-RxSight (I), ProQR Therapeutics (C), Second Sight Medical Products (F), Sparing Vision (C), Spark Therapeutics (C), Vedere Bio (C); Wendi Liang, None; Joseph Carroll, AGTC (F), Meira GTx (C), Meira GTx (F), Translational Imaging Innovations (I), Translational Imaging Innovations (P); Jenna Cava, None; Jessica Wong, None; Michel Michaelides, None; Angelos Kalitzeos, None; Isabelle Audo, Agence Nationale de la Recherche within the Investissements d'Avenir program [ANR-11-IDEX-0004-0] (F), Biogen (C), Gamut Tx (C), IHU FOReSIGHT [ANR-18-IAHU-0001] (F), LABEX LIFESENSES [reference ANR-10-LABX-65] (F), MeiraGTX (C), Novartis (C), Retina France (F), Roche (C); Kate Grieve, None; Natalie Norberg, None; Michel Paques, None; Allison Ayala, None; Travis Porco, None
  • Footnotes
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Investigative Ophthalmology & Visual Science June 2021, Vol.62, 22. doi:
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    • Get Citation

      Jacque L Duncan, Wendi Liang, Joseph Carroll, Jenna Cava, Jessica Wong, Michel Michaelides, Angelos Kalitzeos, Isabelle Audo, Kate Grieve, Natalie Norberg, Michel Paques, Allison Ayala, Travis C Porco; Adaptive optics scanning laser ophthalmoscopy (AOSLO) measures in USH2A-related retinal degeneration. Invest. Ophthalmol. Vis. Sci. 2021;62(8):22.

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

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Abstract

Purpose : Although USH2A-related retinal degeneration is among the most common forms of Usher syndrome type 2 (USH2) and autosomal recessive retinitis pigmentosa (ARRP), prior studies have not described cone structure with high-resolution images. Adaptive Optics Scanning Laser Ophthalmoscopy (AOSLO) is a non-invasive method of visualizing cone photoreceptors with high resolution. We describe baseline cone spacing and explore inter-grader and inter-test variability using data collected in a multicenter, international natural history study of USH2A-related retinal degeneration.

Methods : Two baseline AOSLO montages of the cone mosaic were obtained from each of 14 participants (6 USH2, 8 ARRP; 4 female; 11 white) with USH2A-related retinal degeneration. Two graders masked to disease category measured cone spacing, cone density and an image quality score; poor quality images were confirmed not gradable by a masked clinician. Cone spacing measures were converted to Z-scores based on 37 normal subjects. Inter-test and inter-grader variability were evaluated using intraclass coefficient (ICC) and Bland-Altman plots. Association between cone spacing and clinical characteristics were assessed by linear mixed effects regression.

Results : Median age of participants at enrollment was 38 years (range: 20-59). Median disease duration was 14 years (interquartile range, IQR: 11-19) in USH2 and 6 years (IQR: 4-14) in ARRP participants. Cone spacing ranged from 0.78-2.44 arc minutes. Cone spacing was associated with eccentricity (P < 0.001), but no significant difference was observed by clinical diagnosis or disease duration. Bland-Altman plots showed high concordance between baseline 1 and baseline 2 (mean differences near 0, ICC = 0.71). Grader 2 reported slightly lower values than grader 1 (ICC=0.7). Quality score was higher in baseline 1 than baseline 2 (P = 0.02). Greater inter-grader variation was found in regions with poor image quality (P < 0.001).

Conclusions : The baseline AOSLO data revealed similar cone spacing between USH2 and ARRP participants. Image quality was significantly lower for the second baseline montage, suggesting one baseline image is sufficient. Adding a qualitative image quality score can be a useful approach to handle inter-grader inconsistencies while minimizing the loss of data.

This is a 2021 ARVO Annual Meeting abstract.

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