June 2020
Volume 61, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2020
Structure and function from en-face optical coherence tomography, fundus autofluorescence, and rod and cone perimetry in Stargardt disease
Author Affiliations & Notes
  • Brandon Winward
    University of Texas Southwestern Medical Center, Dallas, Texas, United States
  • Vivienne C Greenstein
    Ophthalmology, Columbia University Medical Center, New York, New York, United States
    Ophthalmology, New York University School of Medicine, New York, New York, United States
  • Donald C Hood
    Ophthalmology, Columbia University Medical Center, New York, New York, United States
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
    University of Texas Southwestern Medical Center, Dallas, Texas, United States
  • Footnotes
    Commercial Relationships   Brandon Winward, None; Vivienne Greenstein, None; Donald Hood, Heidelberg Eng. (F), Heidelberg Eng. (C), Heidelberg Eng. (R), Novartis (F), Novartis (C), Novartis (R), Topcon Inc. (F), Topcon Inc. (C); David Birch, None
  • Footnotes
    Support  R01 EY09076, unrestricted grant from Research to Prevent Blindness
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 1048. doi:
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    • Get Citation

      Brandon Winward, Vivienne C Greenstein, Donald C Hood, David G Birch; Structure and function from en-face optical coherence tomography, fundus autofluorescence, and rod and cone perimetry in Stargardt disease. Invest. Ophthalmol. Vis. Sci. 2020;61(7):1048.

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

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Abstract

Purpose : To compare rod and cone sensitivity loss to structural changes on en-face optical coherence tomography (OCT) and fundus autofluorescence (AF) in patients with Stargardt disease (STGD1).

Methods : Eight patients aged 16-70 yrs. with genetically confirmed STGD1 were enrolled. Spectral domain (SD)-OCT cube scans (9x6mm, 97 B-scans), short-wavelength (SW)-AF, and near-infrared (NIR)-AF images (30 and 55°) were obtained from the better eye. For the OCT, the automated segmentation of the inner/outer segment (IS/OS) junction and RPE/BM boundaries were manually corrected, and en-face images were generated with these boundaries as references using commercial software. RPE atrophy (a central hyper-reflective area on the subRPE slab) and IS/OS junction loss (an abnormal reflective area on the IS/OS slab) were measured using ImageJ. The areas of hypoSW-AF, hyper+hypoSW-AF, and hypoNIR-AF were also measured. Rod and cone system sensitivities were assessed with two-color dark-adapted fundus perimetry (Nidek MP-1S) using a 10-2 grid centered on the fovea (size 3 stimulus, 200-ms duration, 68 points). The rod and cone sensitivity maps were superimposed on SW-AF (Fig.), NIR-AF, and en-face images. Scotopic visual fields were measured in 5 eyes using a Medmont dark-adapted chromatic perimeter (size 5 stimulus, 200-ms duration, 505 nm, 103 points, 144°x72°).

Results : Five eyes had foveal sparing. For 7 eyes, the area of IS/OS junction loss was larger than that of RPE atrophy (p=0.03), and the hyper+hypoSW-AF area was larger than the hyperreflective area on the subRPE slab (p=0.003). Agreement between the area of IS/OS junction loss and the hyper+hypoSW-AF area was good. For 5 eyes, the hypoNIR area was larger than the hypoSW-AF, but the hyper+hypoSW-AF area was larger than the hypoNIR-AF area (p=0.01). Rod sensitivity was more affected than cone sensitivity (p<0.001) adjacent to areas of RPE atrophy, IS/OS junction loss, and surrounding the hypo-AF areas (Fig.) Rod visual fields for 5 eyes showed rod sensitivity was markedly decreased within the central 20°, but comparable to normal in the periphery.

Conclusions : Decreased rod and cone sensitivities are associated with photoreceptor and RPE structural changes that extend beyond atrophy in STGD1.

This is a 2020 ARVO Annual Meeting abstract.

 

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