Correlating Photoreceptor Mosaic Structure to Clinical Findings in Stargardt Disease. Transl Vis Sci Technol 2016 Mar;5(2):6
Date
03/17/2016Pubmed ID
26981328Pubmed Central ID
PMC4790429DOI
10.1167/tvst.5.2.6Scopus ID
2-s2.0-85005952066 (requires institutional sign-in at Scopus site) 26 CitationsAbstract
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.
Author List
Razeen MM, Cooper RF, Langlo CS, Goldberg MR, Wilk MA, Han DP, Connor TB Jr, Fishman GA, Collison FT, Sulai YN, Dubra A, Carroll J, Stepien KEAuthors
Joseph J. Carroll PhD Director, Professor in the Ophthalmology and Visual Sciences department at Medical College of WisconsinThomas B. Connor MD Professor in the Ophthalmology and Visual Sciences department at Medical College of Wisconsin
Robert F. Cooper Ph.D Assistant Professor in the Biomedical Engineering department at Marquette University