June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Progression of Stargardt disease as measured by spectral-domain optical coherence tomography (SD-OCT) in the ProgStar Study
Author Affiliations & Notes
  • Mohamed A Ibrahim
    Ophthalmology, Johns Hopkins University, Forest Hill, Maryland, United States
  • Yulia Wolfson
    Ophthalmology, Johns Hopkins University, Forest Hill, Maryland, United States
  • Beatriz Munoz
    Ophthalmology, Johns Hopkins University, Forest Hill, Maryland, United States
  • Rupert Wolfgang Strauss
    Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
    Johannes Kepler University, Linz, Austria
  • Sheila West
    Ophthalmology, Johns Hopkins University, Forest Hill, Maryland, United States
  • Swetha Velaga
    Doheny Eye Institute, Los Angeles, California, United States
  • Nizar Saleh Abdelfattah
    Doheny Eye Institute, Los Angeles, California, United States
    David Geffen School of Medicine, UCLA, Los Angeles, California, United States
  • Jianyan Huang
    Doheny Eye Institute, Los Angeles, California, United States
  • Srinivas R Sadda
    Doheny Eye Institute, Los Angeles, California, United States
    David Geffen School of Medicine, UCLA, Los Angeles, California, United States
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Eberhart Zrenner
    Center for Ophthalmology, Eberhard Karls Universität, Tübingen, Germany
  • Hendrik P Scholl
    Ophthalmology, Johns Hopkins University, Forest Hill, Maryland, United States
    Department of Ophthalmology, University of Basel, Basel, Switzerland
  • Footnotes
    Commercial Relationships   Mohamed Ibrahim, None; Yulia Wolfson, None; Beatriz Munoz, None; Rupert Strauss, None; Sheila West, None; Swetha Velaga, None; Nizar Abdelfattah, None; Jianyan Huang, None; Srinivas Sadda, Allergan (F), Allergan (C), Carl Zeiss Meditec (F), Genentech (F), Genentech (C), Iconic (C), Novartis (C), Optos (F), Optos (C), Thrombogenics (C); David Birch, None; Eberhart Zrenner, Acucela Inc. (F), NightstaRx Ltd. (F), QLT, Inc. (C), ReNeuron Group Plc/Ora Inc. (C), Retina Implant AG (F), Retina Implant AG (I), Retina Implant AG (C), Retina Implant AG (P), Retina Implant AG (R), Retina Implant AG (S), Shire (C); Hendrik Scholl, Acucela Inc. (F), Boehringer Ingelheim Pharma GmbH & Co. KG (C), Daiichi Sankyo, Inc. (C), Genentech Inc./F. Hoffmann-La Roche Ltd. (R), Gensight Biologics (C), Genzyme Corp./Sanofi (R), Gerson Lehrman Group (C), Guidepoint (C), Intellia Therapeutics, Inc. (C), NightstaRx Ltd (F), QLT, Inc. (F), ReNeuron Group Plc/Ora Inc. (R), Shire (C), Vision Medicines, Inc. (C)
  • Footnotes
    Support  Supported by the Foundation Fighting Blindness Clinical Research Institute (FFB CRI) and a grant to FFB CRI by the U.S. Department of Defense USAMRMC TATRC, Fort Meade, Maryland (grant numbers W81-XWH-07-1-0720 and W81XWH-09-2-0189)
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 4640. doi:
  • Views
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to Subscribers Only
      Sign In or Create an Account ×
    • Get Citation

      Mohamed A Ibrahim, Yulia Wolfson, Beatriz Munoz, Rupert Wolfgang Strauss, Sheila West, Swetha Velaga, Nizar Saleh Abdelfattah, Jianyan Huang, Srinivas R Sadda, David G Birch, 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. 2017;58(8):4640.

      Download citation file:


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

      ×
  • Supplements
Abstract

Purpose : To evaluate progression of loss in various retinal layers in patients with Stargardt disease type 1 (STGD1) based on 6 months follow-up of SD-OCT findings in the prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) Study.

Methods : Patients with molecularly (ABCA4 gene mutations) confirmed STGD1 were enrolled. SD-OCT scans were obtained at baseline from a 20oX20o scan area centered on the fovea and was repeated at month 6 using the built-in follow-up mode. Retinal layers were semi-automatically segmented to identify: retinal pigment epithelium (RPE), inner segments (IS), outer segments (OS), and outer nuclear layer (ONL). The thicknesses of individual layers and the proportions of damage/tissue loss relative to the scanned area were calculated by a custom software within the total scanned area (TSA), central subfield (CS; 0.5mm radius), inner ring (IR; 0.5-1.5mm) and outer ring (OR; 1.5-3mm).

Results : Study demographics and statistics are shown in Tables 1&2. At baseline, OS and IS had the greatest loss in area within the TSA (31%; SD 30; and 29%; SD 29.6, respectively), followed by RPE (8.2%; SD 10.4) and ONL (1.9%; SD 4.1). CS showed the biggest losses with 96.3% (SD 13.7) loss in OS, 95.6% (SD 15.3) in IS, 66% (SD 4.1) in RPE, and 32% (SD 3.7) in ONL. At month 6, the greatest progression was observed in IS with 2.6% of additional loss within the TSA, followed by RPE (2.2%), ONL (1.5%), and lastly OS (1.4%). Changes in ONL and RPE lost areas were largest within the CS with 17.4% and 9.7% of additional loss, respectively, then within IR (5.4% and 7.3%), and lastly within OR (0.4%, and 1.2%). Changes in IS and OS layers were largest within IR (5% and 2.8%, respectively), then within OR (2.3% and 1.2%), and were minimal within CS (1.3% and 0.9%, respectively). All these changes were significant (p<0.001). ONL showed the biggest loss in mean thickness at month 6 (2µm; p<0.001), which was most pronounced within CS subfield (7.3µm; p<0.001).

Conclusions : Statistically significant loss in outer retinal layers can be detected by SD-OCT over 6 months in patients with STGD1. While several outcome variables including the loss in IS, OS, and ONL areas within the central 6mm look promising as surrogate biomarkers, additional data is needed to validate their use as endpoints to demonstrate halting or slowing of disease progression.

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

 

 

×
×

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.

×