September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Cross-sectional evaluation of microperimetric fixation location and stability in Stargardt disease in the ProgStar study
Author Affiliations & Notes
  • Etienne M. Schonbach
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Mohamed A Ibrahim
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Rupert Wolfgang Strauss
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
    Moorfields Eye Hospital, London, United Kingdom
  • Xiangrong Kong
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Alexander Ho
    Doheny Eye Institute, Los Angeles, California, United States
  • Paul S Bernstein
    Moran Eye Center, University of Utah School of Medicine, Salt Lake City, Utah, United States
  • Janet S Sunness
    Greater Baltimore Medical Center, Baltimore, Maryland, United States
  • Eberhart Zrenner
    Center for Ophthalmology, Institute for Ophthalmic Research, Eberhard Karls Universität, Tübingen, Baden- Württemberg, Germany
  • Srinivas R Sadda
    Doheny Eye Institute, Los Angeles, California, United States
    David Geffen School of Medicine, Los Angeles, California, United States
  • Sheila K West
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Hendrik P Scholl
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Etienne Schonbach, None; Mohamed Ibrahim, None; Rupert Strauss, None; Xiangrong Kong, None; Alexander Ho, None; Paul Bernstein, None; Janet Sunness, None; Eberhart Zrenner, None; 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, 2694. doi:
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      Etienne M. Schonbach, Mohamed A Ibrahim, Rupert Wolfgang Strauss, Xiangrong Kong, Alexander Ho, Paul S Bernstein, Janet S Sunness, Eberhart Zrenner, Srinivas R Sadda, Sheila K West, Hendrik P Scholl; Cross-sectional evaluation of microperimetric fixation location and stability in Stargardt disease in the ProgStar study. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2694.

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      © 2017 Association for Research in Vision and Ophthalmology.

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Abstract

Purpose : To investigate the relationship between microperimetric fixation location (FL) and fixation stability (FS), demographic features, and best-corrected visual acuity (BCVA) in Stargardt disease (STGD1) as part of the multicenter ProgStar study (The Natural History of the Progression of Atrophy Secondary to Stargardt Disease).

Methods : We enrolled patients with ABCA4-related STGD1 in a multicenter, prospective, cross-sectional, observational study. We used MP1 data (Nidek Technologies): FL was expressed as the eccentricity of the preferred retinal locus (PRL) from the anatomical fovea, FS as the 95.4 %-bivariate contour ellipse area (BCEA), and best- corrected visual acuity (BCVA) as ETDRS letters. Linear models with generalized estimating equations were used for statistical analysis while accounting for between-eye correlations.

Results : Of 238 patients, 105 (44 %) were males. Median age was 32 years (mean, 33.8 years; range, 7- 69 years) and the median duration of symptoms was 9 years (mean, 11.8 years; range, 0- 55 years). Median PRL eccentricity from the fovea was 6° (mean, 6.2°; range, 0°- 25°), median BCEA was 21.4 deg2 (mean, 31.1 deg2; range, 0.370 deg2- 267 deg2), median BCVA was 42 ETDRS letters (mean, 46.2 letters; range, 20- 88 letters). Each year of additional disease duration was associated with 0.11° more eccentric FL (p =0.0008) but not with FS (p =0.936). Each year of later onset of symptoms of STGD1 was associated with 0.14° more central FL (p < 0.0001) and 0.84 deg2 smaller BCEA (p <0.0001). Each year of additional age was associated with 0.67° more central fixation (p =0.0007) and 0.46 deg2 smaller BCEA (p =0.0014). A single linear model best described the relationship between FL and BCVA: one degree farther PRL eccentricity was associated with 2.3 letters loss of BCVA (p <0.0001) and one additional letter of BCVA with 0.15° more central FL (p <0.0001). The association of FL with BCVA was robust after adjusting for BCEA. Pearson correlation coefficients between patients’ right and left eyes were 0.892 (p <0.0001) for FL and 0.851 (p <0.0001) for FS. After 10 years of disease duration, 82 % of patients had more than 2° PRL eccentricity in both eyes.

Conclusions : Longer disease duration is associated with an increase of eccentricity and loss of stability of fixation in STGD1.

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

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