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Kiyoko Nakashima, Saddek Mohand-Said, Isabelle Audo, Barbara Lamory, Jose-Alain Sahel, Michel Paques; In vivo Microscopic Imaging of Stargardt Disease using Adaptive Optics. Invest. Ophthalmol. Vis. Sci. 2011;52(14):5006.
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© ARVO (1962-2015); The Authors (2016-present)
This study assessed the ability of an adaptive optics (AO) camera to image individual cones in vivo in Stargardt disease, in order to determine the feasibility of using this instrument in gene therapy trials.
Five patients previously diagnosed for Stargardt disease underwent complete ophthalmic examinations, OCT and SLO examinations (Spectralis HRA+OCT, Heidelberg, Germany) and en face retinal imaging using an infrared flood-illumination AO retinal camera (rtx1, Imagine Eyes, Orsay, France) at eccentricities ranging from 0 deg to 6 deg from the foveola. The resulting AO images were registered with the corresponding OCT and SLO images. Cones and other microscopic details visible in AO images were analyzed in relation with the structure observed using the other imaging modalities.
4 males and 1 female of age ranging between 26 and 40 years were included in the study. Visual acuities ranged from 20/200 to 20/20. All cases showed remarkable thinning of retinal thickness in OCT. Minimum foveal retinal thickness ranged between 11µm and 169µm. In two cases with preserved central visual field response, the foveal photoreceptor outer segments were detected by both the OCT and AO camera. In the other cases, cone-like structures of size ranging between 2 and 4µm were observed in AO images centered at 1 deg eccentricity although outer segments were absent for the corresponding OCT images.
AO fundus imaging enables the visualization of retinal microscopic structure in Stargardt Disease. Our data suggest that AO can detect residual cones within areas of central retinal atrophy. AO imaging may be useful for the interpretation of morpho-functional correlations, the assessment of disease progression, and therapeutic decision especially regarding gene therapy.
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