July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Autofluorescence of the photoreceptors in Stargardt disease (SD) identified using fluorescence adaptive optics scanning light ophthalmoscopy (FAOSLO)
Author Affiliations & Notes
  • Hongxin Song
    Beijing Tongren Eye Center, Beijing, China
    Center for Visual Science, University of Rochester, Rochester, New York, United States
  • Ethan A Rossi
    Department of Ophthalmology, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
    Department of Bioengineering, University of Pittsburgh, Pittsburgh, Pennsylvania, United States
  • Lisa Latchney
    Flaum Eye Institute, Rochester, New York, United States
  • Mina M Chung
    Flaum Eye Institute, Rochester, New York, United States
    Center for Visual Science, University of Rochester, Rochester, New York, United States
  • Footnotes
    Commercial Relationships   Hongxin Song, None; Ethan Rossi, None; Lisa Latchney, None; Mina Chung, None
  • Footnotes
    Support  EY021786, EY021669, EY001319, EY014375, and EY004367
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 4635. doi:
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    • Get Citation

      Hongxin Song, Ethan A Rossi, Lisa Latchney, Mina M Chung; Autofluorescence of the photoreceptors in Stargardt disease (SD) identified using fluorescence adaptive optics scanning light ophthalmoscopy (FAOSLO). Invest. Ophthalmol. Vis. Sci. 2018;59(9):4635.

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

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Abstract

Purpose : Stargardt disease (SD) is characterized by changes in the retinal pigment epithelium (RPE) that can be identified by ophthalmoscopy. The causative ABCA4 gene encodes a protein localizing to photoreceptor outer segments. The disease sequence by which ABCA4 mutations lead to defects in the RPE remains unclear. In this study, we imaged photoreceptors and RPE cells in SD using FAOSLO.

Methods : Three SD patients underwent a comprehensive ophthalmic examination and conventional imaging including fundus photography and fundus autofluorescence (HRA, Heidelberg). FAOSLO was used to image the photoreceptor and RPE layers at the single cell level, and compared to age-matched control subjects. Cone and rod photoreceptors were counted and RPE segmentation was performed.

Results : Cone and rod spacing was increased in all patients at all locations where photoreceptors were visible; cones had a dark appearance. FAOSLO showed structures consistent with RPE cells in the periphery. In a transition zone in the peripheral macula outside areas of atrophy, the autofluorescent (AF) pattern was consistent with photoreceptors. No AF cellular patterns were identified within areas of atrophy at the foveal center.

Conclusions : This study provides the first in vivo high resolution images of cones, rods and RPE cells in SD. The AF pattern imaged in the transition zone appears to arise from photoreceptors. This pattern may be due to the accumulation of lipofuscin or lipofuscin precursors within the photoreceptors, and may represent an important early stage in the pathophysiology of SD.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

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