April 2014
Volume 55, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2014
Spectral Domain Optical Coherence Tomography Reveals a Novel Biomarker for Early Stargardt Disease
Author Affiliations & Notes
  • Jerome Sherman
    Clinical Sciences, SUNY College of Optometry, New York, NY
    SEI, SUNY College of Optometry, New York, NY
  • Sanjeev Nath
    NY Eye Institute and Laser Center, New York, NY
  • Sarah MacIver
    University of Waterloo School of Optometry, Waterloo, ON, Canada
  • Sherry J Bass
    Clinical Sciences, SUNY College of Optometry, New York, NY
  • K Bailey Freund
    Vitreous, Retina, Macula Consultants of New York, New York, NY
Investigative Ophthalmology & Visual Science April 2014, Vol.55, 1432. doi:
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      Jerome Sherman, Sanjeev Nath, Sarah MacIver, Sherry J Bass, K Bailey Freund; Spectral Domain Optical Coherence Tomography Reveals a Novel Biomarker for Early Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2014;55(13):1432.

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

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Abstract
 
Purpose
 

To report a novel finding of foveal SD-OCT hyper-reflectivity extending above the external limiting membrane which may precede photoreceptor atrophy in eyes with Stargardt Disease.

 
Methods
 

This was a retrospective review of SD-OCT and fundus autofluorescence imaging from 3 institutions. Imaging of both Stargardt patients and of their asymptomatic siblings, some having genetic confirmation of their diagnosis, were analyzed.

 
Results
 

Six patients were identified as having the novel SD-OCT biomarker including 2 asymptomatic young siblings (4 and 5 yrs old) of an 8-year-old boy with genetically confirmed Stargardt disease and 20/200 VA. Ultra-widefield autofluorescece (UWF-AF) imaging showed mildly increased FAF in the young siblings and marked hyper-AF in the 8 year-old with clinical findings. Additional cases included a 23-year-old (VA 20/25) and a 27-year-old (VA 20/200), both with genetic confirmation of Stargardt disease. The SD-OCT abnormality was not detected in any Stargardt patient over 40 yrs.

 
Conclusions
 

The proposed SD-OCT biomarker appears to be present prior to symptomatic vision loss in patients as young as 4 yrs. We hypothesize that cellular degeneration at the level of the outer nuclear layer may produce this finding. The biomarker is likely transient and may be lost with further outer retina degeneration.

 
 
OCT of 4 yo asymptomatic sister with 20/25 VA. Other eye and both eyes of 5 yo sister are virtually identical.
 
OCT of 4 yo asymptomatic sister with 20/25 VA. Other eye and both eyes of 5 yo sister are virtually identical.
 
 
UWF-AF of 5 yo asymptomatic sister with 20/25 VA. Other eye is virtually identical.
 
UWF-AF of 5 yo asymptomatic sister with 20/25 VA. Other eye is virtually identical.
 
Keywords: 552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • 696 retinal degenerations: hereditary • 539 genetics  
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