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Transl Vis Sci Technol. 2016 Mar 11;5(2):6. eCollection 2016 Mar.

Correlating Photoreceptor Mosaic Structure to Clinical Findings in Stargardt Disease.

Author information

1
Department of Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, WI, USA ; Alexandria Faculty of Medicine, Alexandria University, Alexandria, Egypt.
2
Department of Biomedical Engineering, Marquette University, Milwaukee, WI, USA.
3
Department of Cell Biology, Neurobiology & Anatomy, Medical College of Wisconsin, Milwaukee, WI, USA.
4
Department of Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, WI, USA.
5
The Pangere Center for Hereditary Retinal Diseases, the Chicago Lighthouse for People Who Are Blind or Visually Impaired, Chicago, IL, USA ; Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, IL, USA.
6
The Pangere Center for Hereditary Retinal Diseases, the Chicago Lighthouse for People Who Are Blind or Visually Impaired, Chicago, IL, USA.
7
Department of Ophthalmology & Visual Sciences, Medical College of Wisconsin, Milwaukee, WI, USA ; Department of Biomedical Engineering, Marquette University, Milwaukee, WI, USA ; Department of Cell Biology, Neurobiology & Anatomy, Medical College of Wisconsin, Milwaukee, WI, USA ; Department of Biophysics, Medical College of Wisconsin, Milwaukee, WI, USA.

Abstract

PURPOSE:

To demonstrate a method for correlating photoreceptor mosaic structure with optical coherence tomography (OCT) and microperimetry findings in patients with Stargardt disease.

METHODS:

A total of 14 patients with clinically diagnosed Stargardt disease were imaged using confocal and split-detection adaptive optics scanning light ophthalmoscopy. Cone photoreceptors were identified manually in a band along the temporal meridian. Resulting values were compared to a normative database (n = 9) to generate cone density deviation (CDD) maps. Manual measurement of outer nuclear layer plus Henle fiber layer (ONL+HFL) thickness was performed, in addition to determination of the presence of ellipsoid zone (EZ) and interdigitation zone (IZ) bands on OCT. These results, along with microperimetry data, were overlaid with the CDD maps.

RESULTS:

Wide variation in foveal structure and CDD maps was seen within this small group. Disruption of ONL+HFL and/or IZ band was seen in all patients, with EZ band preservation in regions with low cone density in 38% of locations analyzed. Normality of retinal lamellar structure on OCT corresponded with cone density and visual function at 50/78 locations analyzed. Outer retinal tubulations containing photoreceptor-like structures were observed in 3 patients.

CONCLUSIONS:

The use of CDD color-coded maps enables direct comparison of cone mosaic local density with other measures of retinal structure and function. Larger normative datasets and improved tools for automation of image alignment are needed.

TRANSLATIONAL RELEVANCE:

The approach described facilitates comparison of complex multimodal data sets from patients with inherited retinal degeneration, and can be expanded to incorporate other structural imaging or functional testing.

KEYWORDS:

image analysis; photoreceptors; Stargardt disease; retina; imaging

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