June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Month 18 changes in microperimetric mean sensitivity, fixation location, and fixation stability in
Stargardt disease: The ProgStar Study
Author Affiliations & Notes
  • Etienne Schonbach
    Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Xiangrong Kong
    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
    Moorfields Eye Hospital, London, United Kingdom
    Johannes Kepler University, Linz, Austria
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Artur V Cideciyan
    Scheie Eye Institute, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Muneeswar Gupta Nittala
    Doheny Eye Institute, Los Angeles, California, United States
  • Janet S Sunness
    Hoover Low Vision Rehabilitation Services, Greater Baltimore Medical Center, Baltimore, Maryland, United States
  • Eberhart Zrenner
    Center for Ophthalmology, Eberhard Karls Universität, Tübingen, Germany
  • Srinivas R Sadda
    UCLA David Geffen School of Medicine, Los Angeles, California, United States
    Doheny Eye Institute, Los Angeles, California, United States
  • Sheila 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
    Department of Ophthalmology, University of Basel, Basel, Switzerland
  • Footnotes
    Commercial Relationships   Etienne Schonbach, Leopoldina Fellowship Program Grant No LPDS 2015-14 (F); Xiangrong Kong, None; Mohamed Ibrahim, None; Rupert Strauss, None; David Birch, None; Artur Cideciyan, None; Muneeswar Nittala, None; Janet Sunness, None; Eberhart Zrenner, 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); Sheila West, None; Hendrik Scholl, Acucela Inc. (F), Boehringer Ingelheim Pharma GmbH & Co. KG (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  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, 4635. doi:
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    • Get Citation

      Etienne Schonbach, Xiangrong Kong, Mohamed A Ibrahim, Rupert Wolfgang Strauss, David G Birch, Artur V Cideciyan, Muneeswar Gupta Nittala, Janet S Sunness, Eberhart Zrenner, Srinivas R Sadda, Sheila West, Hendrik P Scholl; Month 18 changes in microperimetric mean sensitivity, fixation location, and fixation stability in
      Stargardt disease: The ProgStar Study. Invest. Ophthalmol. Vis. Sci. 2017;58(8):4635.

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

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Abstract

Purpose : Degeneration of photoreceptors and the retinal pigment epithelium in autosomal recessive Stargardt disease (type 1; STGD1) eventually leads to bilateral reduction of visual acuity and macular sensitivity. Affected patients begin to use an eccentric, less affected, location of the retina to fixate once the center of the macula is involved, often with considerably reduced stability of fixation. Here, we present the month 18 longitudinal changes of the microperimetric mean sensitivity (MS), fixation location, and fixation stability in the prospective, multicenter, longitudinal, observational ProgStar study (NCT01977846).

Methods : The MP-1 Microperimeter (Nidek Technologies) was used to measure MS of the central 20° of the macula, fixation stability through the 95.4%-Bivariate Contour Ellipse Area (BCEA), and fixation location as the distance of the preferred retinal locus (PRL) from the anatomical fovea in patients with ABCA4-related STGD1. Data were graded for month 0, 6, 12, and 18. Linear mixed effects models were used to estimate the rate of change of MP parameters per year.

Results : At baseline, 238 patients (105 males, 44 %) with 444 eyes and a median age of 32 years (mean, 33.8 years; range, 7-69 years) were included in this analysis. Median MS at baseline was 12.1 dB (mean, 10.8 dB; range, 0- 19.9 dB). MS at month 18 was 10.9 dB (mean, 9.76 dB; range, 0-20.0 dB) and a yearly change of -0.69 dB (95 % CI, -0.83 to -0.55 dB) was calculated. The median BCEA at baseline was 21.4 deg2 (mean, 30.8 deg2; range, 0.37-220.6 deg2) and 21.4 deg2 at month 18 (mean, 31.1 deg2; range, 0.37- 266.9 deg2) with a non signficant yearly increase of 0.06 deg2 (95 % CI, -1.7 to 1.8 deg2). The median PRL distance was 6 deg from the anatomical fovea (mean, 6.24 deg; range, 0 to 25 deg) at baseline and remained stable at month 18 (mean, 6.16 deg; range, 0 to 26 deg) with a yearly change of -0.10 deg (95 % CI, -0.27 to 0.07 deg). Both improvements and worsening of eccentricity and fixation stability were observed.

Conclusions : In our cohort of 238 cases of STGD1 with 18 months of follow-up, the annual rate of change in microperimetric measures was not statistically significant for fixation location or stability. Mean sensitivity showed a statistically significant annual decline of 0.69 dB.

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

 

Longitudinal follow-up of mean sensitivity in eyes with ABCA4- related Stargardt disease.

Longitudinal follow-up of mean sensitivity in eyes with ABCA4- related Stargardt disease.

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