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PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY

PURPOSE: To characterize outer retina structure in best vitelliform macular dystrophy (BVMD) and to determine the effect of macular lesions on overlying and adjacent photoreceptors. METHODS: Five individuals with BVMD were followed prospectively with spectral domain optical coherence tomography and...

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Autores principales: Scoles, Drew, Sulai, Yusufu N., Cooper, Robert F., Higgins, Brian P., Johnson, Ryan D., Carroll, Joseph, Dubra, Alfredo, Stepien, Kimberly E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Retina 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5362286/
https://www.ncbi.nlm.nih.gov/pubmed/27467379
http://dx.doi.org/10.1097/IAE.0000000000001203
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author Scoles, Drew
Sulai, Yusufu N.
Cooper, Robert F.
Higgins, Brian P.
Johnson, Ryan D.
Carroll, Joseph
Dubra, Alfredo
Stepien, Kimberly E.
author_facet Scoles, Drew
Sulai, Yusufu N.
Cooper, Robert F.
Higgins, Brian P.
Johnson, Ryan D.
Carroll, Joseph
Dubra, Alfredo
Stepien, Kimberly E.
author_sort Scoles, Drew
collection PubMed
description PURPOSE: To characterize outer retina structure in best vitelliform macular dystrophy (BVMD) and to determine the effect of macular lesions on overlying and adjacent photoreceptors. METHODS: Five individuals with BVMD were followed prospectively with spectral domain optical coherence tomography and confocal and nonconfocal split-detector adaptive optics scanning light ophthalmoscopy (AOSLO). The AOSLO cone photoreceptor mosaic images were obtained within and around retinal lesions. Cone density was measured inside and outside lesions. In 2 subjects, densities were compared with published measurements acquired ∼2.5 years before. One subject was imaged 3 times over a 5-month period. RESULTS: The AOSLO imaging demonstrated that photoreceptor morphology within BVMD retinal lesions was highly variable depending on the disease stage, with photoreceptor structure present even in advanced disease. The AOSLO imaging was repeatable even in severe disease over short-time and long-time intervals. Photoreceptor density was normal in retinal areas immediately adjacent to lesions and stable over ∼2.5 years. Mobile disk-like structures possibly representing subretinal macrophages were also observed. CONCLUSION: Combined confocal and nonconfocal split-detector AOSLO imaging reveals substantial variability within clinical lesions in all stages of BVMD. Longitudinal cellular photoreceptor imaging could prove a powerful tool for understanding disease progression and monitoring emerging therapeutic treatment response in inherited degenerations such as BVMD.
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spelling pubmed-53622862018-04-01 PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY Scoles, Drew Sulai, Yusufu N. Cooper, Robert F. Higgins, Brian P. Johnson, Ryan D. Carroll, Joseph Dubra, Alfredo Stepien, Kimberly E. Retina Original Study PURPOSE: To characterize outer retina structure in best vitelliform macular dystrophy (BVMD) and to determine the effect of macular lesions on overlying and adjacent photoreceptors. METHODS: Five individuals with BVMD were followed prospectively with spectral domain optical coherence tomography and confocal and nonconfocal split-detector adaptive optics scanning light ophthalmoscopy (AOSLO). The AOSLO cone photoreceptor mosaic images were obtained within and around retinal lesions. Cone density was measured inside and outside lesions. In 2 subjects, densities were compared with published measurements acquired ∼2.5 years before. One subject was imaged 3 times over a 5-month period. RESULTS: The AOSLO imaging demonstrated that photoreceptor morphology within BVMD retinal lesions was highly variable depending on the disease stage, with photoreceptor structure present even in advanced disease. The AOSLO imaging was repeatable even in severe disease over short-time and long-time intervals. Photoreceptor density was normal in retinal areas immediately adjacent to lesions and stable over ∼2.5 years. Mobile disk-like structures possibly representing subretinal macrophages were also observed. CONCLUSION: Combined confocal and nonconfocal split-detector AOSLO imaging reveals substantial variability within clinical lesions in all stages of BVMD. Longitudinal cellular photoreceptor imaging could prove a powerful tool for understanding disease progression and monitoring emerging therapeutic treatment response in inherited degenerations such as BVMD. Retina 2017-04 2017-04-07 /pmc/articles/PMC5362286/ /pubmed/27467379 http://dx.doi.org/10.1097/IAE.0000000000001203 Text en This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (http://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.
spellingShingle Original Study
Scoles, Drew
Sulai, Yusufu N.
Cooper, Robert F.
Higgins, Brian P.
Johnson, Ryan D.
Carroll, Joseph
Dubra, Alfredo
Stepien, Kimberly E.
PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY
title PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY
title_full PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY
title_fullStr PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY
title_full_unstemmed PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY
title_short PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY
title_sort photoreceptor inner segment morphology in best vitelliform macular dystrophy
topic Original Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5362286/
https://www.ncbi.nlm.nih.gov/pubmed/27467379
http://dx.doi.org/10.1097/IAE.0000000000001203
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