![]() ![]() The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. This investigation was conducted in part in a facility constructed with support from a Research Facilities Improvement Program (grant number C06RR016511) from the National Center for Research Resources, National Institutes of Health. Supported by grants from the National Eye Institute, National Institutes of Health (R01EY017607, R01EY024969, P30EY010572, R24EY022023, T32EY014537, T32GM080202, F32EY029148) by the National Center for Advancing Translational Sciences under award number UL1TR001436 by unrestricted grants from Research to Prevent Blindness to the Casey Eye Institute and University of Florida and by the Foundation Fighting Blindness. Translational Relevance: Until AOSLO is successful in all patients with ACHM and albinism, the possibility of the reported data from a particular cohort not being representative of the entire population remains an important issue to consider when interpreting results from AOSLO studies. In albinism, OCT metrics of cone structure did not differ between groups.Ĭonclusions: Previously reported AOSLO-based cone density measures in ACHM may not necessarily reflect the degree of remnant cone structure in these patients. There was a significant trend between ellipsoid zone appearance and ability to quantify AOSLO (P = 0.0028). Older age and better BCVA were found in subjects with quantifiable AOSLO images for both ACHM (P = 0.0214 and P = 0.0276, respectively) and albinism (P = 0.0073 and P < 0.0004, respectively). Results: Forty-nine percent of subjects with ACHM and 57% of subjects with albinism had quantifiable AOSLO images. AOSLO images were graded as quantifiable if a peak cone density could be measured and/or usable if the location of peak density could be identified and the parafoveal mosaic was quantifiable. Foveal OCT scans were assessed for outer retinal structure, outer nuclear layer thickness, and hypoplasia. Methods: The study included 166 subjects (84 with ACHM 82 with albinism) with previously acquired OCT scans, AOSLO images, and best-corrected visual acuity (BCVA, if available). Here, we tested whether optical coherence tomography (OCT) measures of foveal structure differed between patients for whom AOSLO images were either quantifiable or unquantifiable. Purpose: Adaptive optics scanning light ophthalmoscopy (AOSLO) imaging in patients with achromatopsia (ACHM) and albinism is not always successful. ![]()
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