June 2013
Volume 54, Issue 15
Free
ARVO Annual Meeting Abstract  |   June 2013
Characterization of Stargardt disease using polarisation-sensitive spectral-domain optical coherence tomography and fundus autofluorescence imaging
Author Affiliations & Notes
  • Markus Ritter
    Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
  • Stefan Zotter
    Center for Medical Physics and Biomedical Engineering, Medical University of Vienna, Vienna, Austria
  • Gabor Deak
    Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
  • Michael Pircher
    Center for Medical Physics and Biomedical Engineering, Medical University of Vienna, Vienna, Austria
  • Stefan Sacu
    Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
  • Christoph Hitzenberger
    Center for Medical Physics and Biomedical Engineering, Medical University of Vienna, Vienna, Austria
  • Ursula Schmidt-Erfurth
    Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
  • Footnotes
    Commercial Relationships Markus Ritter, None; Stefan Zotter, Canon Inc. (F); Gabor Deak, None; Michael Pircher, None; Stefan Sacu, None; Christoph Hitzenberger, Canon Inc. (F), Canon Inc. (C); Ursula Schmidt-Erfurth, Alcon (C), Bayer Healthcare (C), Novartis (C)
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2013, Vol.54, 1476. doi:
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      Markus Ritter, Stefan Zotter, Gabor Deak, Michael Pircher, Stefan Sacu, Christoph Hitzenberger, Ursula Schmidt-Erfurth; Characterization of Stargardt disease using polarisation-sensitive spectral-domain optical coherence tomography and fundus autofluorescence imaging. Invest. Ophthalmol. Vis. Sci. 2013;54(15):1476.

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

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Abstract

Purpose: To identify disease-specific changes in Stargardt disease (STGD) based on imaging with polarisation-sensitive spectral-domain optical coherence tomography (PS-OCT) and to compare structural changes to those visible on fundus autofluorescence (FAF) imaging.

Methods: Twenty-eight eyes of 14 patients diagnosed with STGD and a recently described ABCA4-associated phenotype characterized on OCT by a gap in the subfoveal outer segment layer (foveal cavitation) were imaged using a novel high-speed, large-field PS-OCT system and FAF. In patients with STGD, the ophthalmoscopic phenotype was classified into 3 groups as suggested by Fishman et al. (Arch Ophthalmol.1999). ABCA4 mutation detection was performed by PCR and direct sequencing.

Results: STGD phenotype 1 (8 eyes) showed sharply delineated areas of absent RPE signal on RPE segmentation B scans of PS-SD-OCT correlating with areas of hypofluorescenece on FAF. Adjacent areas of increased flourescence correlated with an irregular, partly interrupted RPE segmentation line with absence of overlaying photoreceptor layers . Hyperfluorescent flecks on FAF in phenotype 2 STGD (8 eyes) were identified as clusters of depolarizing material at the level of the RPE, some of them extended into the inner segment/outer segment junction line of photoreceptors. Distribution of flecks could be depicted on depolarizing material thickness maps. An increased amount of depolarizing material in the choroid was characteristic for STGD Phenotype 3 (8 eyes). 4 eyes characterized by a foveal cavitation showed increased perifoveal fluorescence but a normal RPE segmentation line on PS-SD-OCT.

Conclusions: PS-OCT together with FAF identified characteristic patterns of changes in different stages of the disease. PS-OCT is a promising new tool for diagnosis, disease follow-up and evaluation of future treatment modalities in ABCA4-associated retinal dystrophies.

Keywords: 552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • 696 retinal degenerations: hereditary  
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