June 2015
Volume 56, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2015
Quantitative Characteristics of Spectral-Domain Optical Coherence Tomography (SDOCT) in Corresponding Areas of Decreased Autofluorescence in Patients with Stargardt Disease
Author Affiliations & Notes
  • Alexander Ho
    Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, CA
  • Laura Kuehlewein
    Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, CA
    Doheny Eye Center, UCLA David Geffen School of Medicine, Los Angeles, CA
  • Amirhossein Hariri
    Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, CA
    Doheny Eye Center, UCLA David Geffen School of Medicine, Los Angeles, CA
  • Yulia Wolfson
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Rupert Wolfgang Strauss
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Hendrik P Scholl
    Wilmer Eye Institute, Johns Hopkins University, Baltimore, MD
  • Srinivas R Sadda
    Doheny Image Reading Center, Doheny Eye Institute, Los Angeles, CA
    Doheny Eye Center, UCLA David Geffen School of Medicine, Los Angeles, CA
  • Footnotes
    Commercial Relationships Alexander Ho, None; Laura Kuehlewein, None; Amirhossein Hariri, None; Yulia Wolfson, None; Rupert Strauss, None; Hendrik Scholl, QLT Inc. (C), QLT Inc. (F), Sanofi-Fovea Pharmaceuticals (C), Vision Medicines, Inc. (C); Srinivas Sadda, Carl Zeiss Meditec (C), Carl Zeiss Meditec (F), Carl Zeiss Meditec (R), Optos (C), Optos (F), Optos (R)
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2015, Vol.56, 5924. doi:
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      Alexander Ho, Laura Kuehlewein, Amirhossein Hariri, Yulia Wolfson, Rupert Wolfgang Strauss, Hendrik P Scholl, Srinivas R Sadda, ; Quantitative Characteristics of Spectral-Domain Optical Coherence Tomography (SDOCT) in Corresponding Areas of Decreased Autofluorescence in Patients with Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):5924.

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

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Abstract
 
Purpose
 

Fundus autofluorescence (FAF) has been proposed as a method to monitor Stargardt disease (STGD), but various patterns of abnormal FAF may be observed, potentially confounding longitudinal assessments. Outer retinal substructure alterations on optical coherence tomography (OCT) have also been demonstrated in STGD patients to correlate with visual outcomes. In this analysis, we explored the relationship between these OCT alterations and areas of definitely and questionably decreased autofluorescence (DDAF, QDAF).

 
Methods
 

34 eyes from 19 STGD patients with exclusively DDAF or QDAF in the central subfield on confocal scanning laser ophthalmoscope FAF images were identified from the ongoing Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) studies. DDAF was defined as regions of decreased FAF greater than 125 µm in diameter with a blackness level comparable to the optic disc. QDAF was defined as regions of decreased FAF greater than 125 µm in diameter and relatively less dark than the optic disc, but still substantially more hypo-autofluorescent than the background (Figure 1). Corresponding SDOCT B-scans (49-section 20°x20° high resolution volume scans) were independently segmented using proprietary software (OCTOR) for the following layers: inner retina (inner limiting membrane [ILM] to dendritic outer plexiform layer [OPL]), outer nuclear layer (ONL; dendritic OPL to external limiting membrane [ELM]), and combined layers between ELM and Bruch’s membrane (BM). Central subfield layer metrics from DDAF and QDAF were compared using the Mann-Whitney-Wilcoxon test.

 
Results
 

The averages and standard deviations of mean thicknesses and preserved areas in the central subfield are shown for DDAF and QDAF in Table 1. The ONL and ELM-BM layer were both significantly thinner in areas of DDAF compared to QDAF (p<0.05).

 
Conclusions
 

Thickness and preservation of the ONL and ELM-BM layer appear to be linked to the visible differences in DDAF and QDAF lesions. This finding is consistent with the proposed models for STGD progression, if QDAF is considered a transition state between healthy retina and later stages of STGD.  

 
Figure 1. Examples of lesions categorized as (1a) DDAF and (1b) QDAF.
 
Figure 1. Examples of lesions categorized as (1a) DDAF and (1b) QDAF.
 
 
Table 1. Averages and standard deviations of mean thicknesses and areas of layers in the central subfield per SDOCT.
 
Table 1. Averages and standard deviations of mean thicknesses and areas of layers in the central subfield per SDOCT.

 
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