September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016

Associations between fundus autofluorescence and spectral-domain optical coherence tomography in Stargardt disease at baseline of the prospective ProgStar study.
Author Affiliations & Notes
  • Yulia Wolfson
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Beatriz E Munoz
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Rupert Wolfgang Strauss
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
    Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
  • Alexander Ho
    Doheny Eye Institute, Los Angeles, California, United States
  • Anamika Jha
    Doheny Eye Institute, Los Angeles, California, United States
  • Elias I Traboulsi
    Cole Eye Institute, Cleveland, Ohio, United States
    Center for Graduate Medical Education, Education Institute, Cleveland Clinic, Cleveland, Ohio, United States
  • Artur V Cideciyan
    Scheie Eye Institute, Univ of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Michel Michaelides
    Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
  • Srinivas R Sadda
    Doheny Eye Institute, Los Angeles, California, United States
    David Geffen School of Medicine, UCLA, Los Angeles, California, United States
  • Sheila K West
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Hendrik P Scholl
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Yulia Wolfson, None; Beatriz Munoz, None; Rupert Strauss, None; Alexander Ho, None; Anamika Jha, None; Elias Traboulsi, None; Artur Cideciyan, None; Michel Michaelides, Athena Vision (C); Srinivas Sadda, Allergan (F), Allergan (C), Avalanche (C), Bayer (C), Carl Zeiss Meditec (F), Genentech (F), Genentech (C), Iconic (C), Novartis (C), Optos (F), Optos (C), Regeneron (C), Stem Cells Inc (C), Thrombogenics (C); Sheila West, None; Hendrik Scholl, None
  • 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 September 2016, Vol.57, No Pagination Specified. doi:
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      Yulia Wolfson, Beatriz E Munoz, Rupert Wolfgang Strauss, Alexander Ho, Anamika Jha, Elias I Traboulsi, Artur V Cideciyan, Michel Michaelides, Srinivas R Sadda, Sheila K West, Hendrik P Scholl;
      Associations between fundus autofluorescence and spectral-domain optical coherence tomography in Stargardt disease at baseline of the prospective ProgStar study.. Invest. Ophthalmol. Vis. Sci. 201657(12):.

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      © 2017 Association for Research in Vision and Ophthalmology.

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Abstract

Purpose : The multicentre observational ProgStar study (the Natural History of the Progression of Atrophy Secondary to Stargardt Disease Studies) aims to characterize the natural history of Stargardt disease (STGD1). The associations are estimated between characteristics derived from fundus autofluorescence (AF) and retinal architecture derived from spectral-domain optical coherence tomography (SD-OCT) at baseline visit of the prospective ProgStar study.

Methods : AF and SD-OCT images from genetically confirmed STGD1 participants were sent from nine clinical sites to a central reading center. For AF, areas of definitely decreased AF (DDAF), well-demarcated questionably decreased AF (WD-QDAF) and poorly-demarcated AF (PD-QDAF) were quantified using Heidelberg Engineering’s RegionFinder tool, and the total area of abnormally decreased AF was calculated as the sum of DDAF, WD-QDAF and PD-QDAF. Areas of preserved retinal pigment epithelium (RPE), photoreceptor outer segments (OS) and inner segments (IS), outer nuclear complex (ONC), and inner retina (InR) were derived from SD-OCT volume scans using proprietary software. OCT defect areas were calculated by subtracting the preserved areas from total scanned areas. Linear models with generalized estimating equations were used to assess the relationships between AF (outcome) and SD-OCT defect areas while adjusting for within-subject (between-eye) correlations.

Results : AF and SD-OCT images were graded for 212 study eyes of 113 participants at the baseline visit. Mean age at enrolment was 33.4 (± 15.4 standard deviation) years. AF mean lesion sizes were: 2.14 ± 3.32 mm2 for DDAF; 0.31 ± 0.97 mm2 for WD-QDAF; 1.42 ± 1.70 mm2 for PD-QDAF. SD-OCT defect mean areas were: 3.28 ± 4.30 mm2 for RPE; 9.67 ± 9.82 mm2 for OS; 8.44 ± 9.27 mm2 for IS; 0.73 ± 1.23 mm2 for ONC; 0.004 ± 0.02 mm2 for InR. Significant positive associations were observed between DDAF and total AF lesion areas and the total RPE, IS, OS and ONC defect areas in the SD-OCT (Table 1).

Conclusions : The structural changes in FAF are consistent with the structural changes in SD-OCT.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

 

Table 1: Regression coefficients and 95% confidence intervals for an increase of 1 standard deviation in the estimated defect area in the SD-OCT.

Table 1: Regression coefficients and 95% confidence intervals for an increase of 1 standard deviation in the estimated defect area in the SD-OCT.

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