September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Scotopic and photopic macular functions as assessed with microperimetry (MP1) in patients with Stargardt disease type 1 – The SMART Study
Author Affiliations & Notes
  • Mohamed A Ibrahim
    Wilmer Eye Institute at the Johns Hopkins University, Baltimore, Maryland, United States
  • Rupert Wolfgang Strauss
    Wilmer Eye Institute at the Johns Hopkins University, Baltimore, Maryland, United States
    Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom
  • Xiangrong Kong
    Wilmer Eye Institute at the Johns Hopkins University, Baltimore, Maryland, United States
  • Ann-Margret Ervin
    Wilmer Eye Institute at the Johns Hopkins University, Baltimore, Maryland, United States
  • Alexander Ho
    Doheny Eye Institute, Los Angeles, California, United States
  • Janet S Sunness
    Greater Baltimore Medical Center, Baltimore, Maryland, United States
  • Isabelle S Audo
    CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DHOS CIC 1423, Sorbonne Universités, UPMC Univ Paris 06, INSERM, CNRS, Institut de la Vision, Paris, France
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Srinivas R Sadda
    Doheny Eye Institute, Los Angeles, California, United States
    David Geffen School of Medicine, UCLA, Los Angeles, California, United States
  • Millena G Bittencourt
    Wilmer Eye Institute at the Johns Hopkins University, Baltimore, Maryland, United States
  • Hendrik P Scholl
    Wilmer Eye Institute at the Johns Hopkins University, Baltimore, Maryland, United States
  • Footnotes
    Commercial Relationships   Mohamed Ibrahim, None; Rupert Strauss, None; Xiangrong Kong, None; Ann-Margret Ervin, None; Alexander Ho, None; Janet Sunness, None; Isabelle Audo, None; David Birch, None; Srinivas Sadda, Allergan (F), Allergan (C), Avalanche (C), Bayer (C), Carl Zeiss Meditec (F), Genentech (F), Genetech (C), Iconic (C), Novartis (C), Optos (F), Optos (C), Regeneron (C), Stem Cell Inc (C), Thrombogenetics (C); Millena Bittencourt, 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, 2696. doi:
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    • Get Citation

      Mohamed A Ibrahim, Rupert Wolfgang Strauss, Xiangrong Kong, Ann-Margret Ervin, Alexander Ho, Janet S Sunness, Isabelle S Audo, David G Birch, Srinivas R Sadda, Millena G Bittencourt, Hendrik P Scholl; Scotopic and photopic macular functions as assessed with microperimetry (MP1) in patients with Stargardt disease type 1 – The SMART Study. Invest. Ophthalmol. Vis. Sci. 2016;57(12):2696.

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      © 2017 Association for Research in Vision and Ophthalmology.

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Abstract

Purpose : To characterize scotopic and photopic macular function with MP1S in patients with Stargardt disease type 1 (STGD1) in the SMART Study (Scotopic Microperimetric Assessment of Rod Function in Stargardt Disease)

Methods : Patients with confirmed mutations in the ABCA4 gene and STGD1 phenotype were enrolled (sub-cohort of the ProgStar Study). One eye from every patient (best eye) underwent testing using a custom pattern on the MP1S system (Nidek Technologies), before (mesopic; m) and after (scotopic; s) dark-adaptation. Mesopic testing used a white stimulus; scotopic testing a blue stimulus with a combined neutral density filter to align sensitivity with the dynamic range of the MP1S and a 500nm short-pass filter. Tests were graded at the Doheny Image Reading Center. Mean sensitivity (MS) in the ring 4-10 degrees from the fovea was calculated for both the mesopic (mMS) and scotopic (sMS) tests. The distance of center of gravity of fixation points (preferred retinal locus or PRL) from the anatomical fovea was measured along with the 2SD bivariate contour ellipse area (BCEA). Descriptive statistics and correlation coefficients were used to assess the relationship between photopic and scotopic outcome variables.

Results : 136 patients from 7 centers were enrolled. 54% were female, 82% were white, mean age was 34.5 (SD±14) years, mean age of onset was 24.6 (SD±13.7) years, and mean disease duration (DD) was 10 (SD±7.3) years. The mean BCVA was 53.4 letters (SD±17.9). The mean mMS was 12.2 (SD±4.6) dB and the mean sMS was 11.1 (SD±5.0) dB with the one-log unit filter. mMS and sMS were highly correlated with Pearson correlation coefficient of 0.81 (p<0.0001). The mean mPRL was 4.9 (SD±3.9) degrees and the mean sPRL was 5.34 (SD±3.8) degrees. mPRL and sPRL were highly correlated with correlation coefficient of 0.77 (p<0.0001). The mean 2SD mBCEA was 26.8 (SD±34.7) deg2 and the mean sBCEA was 30.4 (SD±31) deg2. mBCEA and sBCEA were correlated with correlation coefficients of 0.68 (p<0.0001).

Conclusions : In our cohort of patients with STGD1, photopic and scotopic macular performance were highly correlated when assessed using mean macular sensitivity in the perifoveal area. There were no significant differences in the distance of PRL from fovea or the 2SD BCEA of fixation.

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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