Investigative Ophthalmology & Visual Science Cover Image for Volume 59, Issue 9
July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Progression of Stargardt disease as measured by spectral-domain optical coherence tomography (SD-OCT) in the ProgStar Study
Rupert Wolfgang Strauss; Xiangrong Kong; Beatriz E Munoz; Sheila K West; Michael S Ip; Alexander Ho; Anamika Jha; Paul S Bernstein; David G Birch; Artur V Cideciyan; Michel Michaelides; José-Alain Sahel; Janet S Sunness; Elias I Traboulsi; Eberhart Zrenner; Hendrik P Scholl
Author Affiliations & Notes
  • Rupert Wolfgang Strauss
    Moorfields Eye Hosp & Inst of Ophthal UCL, London, ENGLAND, United Kingdom
    Department of Ophthalmology, University of Basel, Basel, Switzerland
  • Xiangrong Kong
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Beatriz E Munoz
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Sheila K West
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Michael S Ip
    Doheny Imaging Reading Center, David Geffen School of Medicine, Los Angeles, California, United States
  • Alexander Ho
    Doheny Imaging Reading Center, David Geffen School of Medicine, Los Angeles, California, United States
  • Anamika Jha
    Doheny Imaging Reading Center, David Geffen School of Medicine, Los Angeles, California, United States
  • Paul S Bernstein
    Moran Eye Institute, University of Utah, Salt Lake City, Utah, United States
  • David G Birch
    Retinafoundation of the Southwest, Dallas, Texas, United States
  • Artur V Cideciyan
    University of Pennsylvania, Scheie Eye Institute, Philadelphia, Pennsylvania, United States
  • Michel Michaelides
    Moorfields Eye Hosp & Inst of Ophthal UCL, London, ENGLAND, United Kingdom
  • José-Alain Sahel
    Sorbonnes University, Institute de la vVsion, Paris, France
  • Janet S Sunness
    Hoover Low Vision Rehabilitation Services, Greater Baltimore Medical Center, Baltimore, Maryland, United States
  • Elias I Traboulsi
    Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio, United States
  • Eberhart Zrenner
    Center for Ophthalmology, Eberhard-Karls University Hospital, Tuebingen, Germany
  • Hendrik P Scholl
    Department of Ophthalmology, University of Basel, Basel, Switzerland
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Rupert Strauss, None; Xiangrong Kong, None; Beatriz Munoz, None; Sheila West, None; Michael Ip, None; Alexander Ho, None; Anamika Jha, None; Paul Bernstein, None; David Birch, 4D Molecular Therapeutics (F), Acucela C (F), AGTC (F), Genentech (C), Ionis Pharmaceuticals (F), Nacuity (C), NightstaRX C (F); Artur Cideciyan, None; Michel Michaelides, None; José-Alain Sahel, Banque publique d'Investissement (F), Chronocam (I), Chronolife (I), ERC Synergy "HELMHOLTZ" (F), Foundation Fighting Blindness (F), Genesignal (C), GenSight Biologics (C), GenSight Biologics (I), LabEx LIFESENSES (ANR-10-LABX-65) (F), Pixium Vision (C), Pixium Vision (I), Sparing Vision (I), Tilak Healthcare (I); Janet Sunness, None; Elias Traboulsi, None; Eberhart Zrenner, None; Hendrik Scholl, Boehringer Ingelheim Pharma GmbH & Co. KG (C), Daiichi Sankyo, Inc (C), Gerson Lehrman Group (C), Guidepoint (C), Shire (C)
  • Footnotes
    Support  Foundation Fighting Blindness Clinical Research Institute and by a grant to that entity by the United States Department of Defense USAMRMC TATRC, Fort Meade, Maryland (grant nos.: W81-XWH-07-1-0720 and W81XWH-09-2-0189)
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 1568. doi:
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      Rupert Wolfgang Strauss, Xiangrong Kong, Beatriz E Munoz, Sheila K West, Michael S Ip, Alexander Ho, Anamika Jha, Paul S Bernstein, David G Birch, Artur V Cideciyan, Michel Michaelides, José-Alain Sahel, Janet S Sunness, Elias I Traboulsi, Eberhart Zrenner, Hendrik P Scholl; Progression of Stargardt disease as measured by spectral-domain optical coherence tomography (SD-OCT) in the ProgStar Study. Invest. Ophthalmol. Vis. Sci. 2018;59(9):1568.

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

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Abstract

Purpose : To evaluate the change of individual retinal layer thicknesses in patients with Stargardt disease type 1 (STGD1) over 12 months of follow-up using SD-OCT in the prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Study.

Methods : 259 patients with molecularly confirmed STGD1 (disease causing ABCA4 gene mutations) were enrolled. SD-OCT scans using Heidelberg Spectralis (HE, Germany) were obtained at baseline from a 20° x 20° scan area centered on the fovea and repeated at month 6 and 12 using the built-in follow-up mode (where possible) with gradable images available for 427 eyes. Retinal layers were semi-automatically segmented to identify the following bands: retinal pigment epithelium (RPE), outer segments (OS), inner segments (IS), outer nuclear complex (ONC) and inner retina. The mean thicknesses (MT) of individual layers and the areas of damage/tissue loss were calculated by a custom software within the total scanned area (TSA), central subfield (CS; 0.5mm radius from the foveal center), inner ring (IR; 0.5-1.5mm) and outer ring (OR; 1.5-3mm) of the ETDRS grid.

Results : Study demographics are shown in table 1. In CS, 377 eyes (86%) had MT=0 for IS and OS layers, and CS was excluded from further analysis as there would be no further progression. In IR, total retina thickness decreased -3.04 (95% CI: -4.12, -1.96) µm/year, ONC -7.23 (-8.37, -6.09), IS -1.96 (-2.28, -1.64) µm/year, OS -0.55 (-0.75, -0.35) µm/year and RPE -3.42 (-4.00, -2.85) (table 2). In IR, also intact area showed a statistically significant decrease in all layers over a 12-months period; in the outer ring, a statistically significant decrease was found in RPE and ONC (table 2).

Conclusions : A statistically significant loss was observed in the outer retinal layers by SD-OCT over a 12 month period in patients with STGD1. This was especially significant in the IR – with loss of thickness and/or intact area of the RPE, IS, OS and ONL potentially serving as structural endpoints for clinical trials that aim to slow disease progression.

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

 

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Copyright © 2025 Association for Research in Vision and Ophthalmology.
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