Reliability and Repeatability of Cone Density Measurements in Patients with Congenital Achromatopsia. Adv Exp Med Biol 2016;854:277-83
Date
10/03/2015Pubmed ID
26427422Pubmed Central ID
PMC4839591DOI
10.1007/978-3-319-17121-0_37Scopus ID
2-s2.0-84943329540 (requires institutional sign-in at Scopus site) 39 CitationsAbstract
Adaptive optics scanning light ophthalmoscopy (AOSLO) allows non-invasive assessment of the cone photoreceptor mosaic. Confocal AOSLO imaging of patients with achromatopsia (ACHM) reveals an altered reflectivity of the remaining cone structure, making identification of the cells more challenging than in normal retinas. Recently, a "split-detector" AOSLO imaging method was shown to enable direct visualization of cone inner segments in patients with ACHM. Several studies have demonstrated gene replacement therapy effective in restoring cone function in animal models of ACHM and human trials have on the horizon, making the ability to reliably assess cone structure increasingly important. Here we sought to examine whether absolute estimates of cone density obtained from split-detector and confocal AOSLO images differed from one another and whether the inter- and intra-observer reliability is significantly different between these modes. These findings provide an important foundation for evaluating the role of these images as tools to assess the efficacy of future gene therapy trials.
Author List
Abozaid MA, Langlo CS, Dubis AM, Michaelides M, Tarima S, Carroll JAuthors
Joseph J. Carroll PhD Director, Professor in the Ophthalmology and Visual Sciences department at Medical College of WisconsinSergey S. Tarima PhD Associate Professor in the Institute for Health and Equity department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
AdolescentAdult
Child
Color Vision Defects
Cyclic Nucleotide-Gated Cation Channels
Female
Humans
Male
Microscopy, Confocal
Middle Aged
Mutation
Ophthalmoscopy
Reproducibility of Results
Retinal Cone Photoreceptor Cells
Sensitivity and Specificity
Young Adult