PHOTORECEPTOR INNER SEGMENT MORPHOLOGY IN BEST VITELLIFORM MACULAR DYSTROPHY. Retina 2017 Apr;37(4):741-748
Date
10/18/2016Pubmed ID
27467379Pubmed Central ID
PMC5362286DOI
10.1097/IAE.0000000000001203Scopus ID
2-s2.0-84980022412 (requires institutional sign-in at Scopus site) 31 CitationsAbstract
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.
Author List
Scoles D, Sulai YN, Cooper RF, Higgins BP, Johnson RD, Carroll J, Dubra A, Stepien KEAuthors
Joseph J. Carroll PhD Director, Professor in the Ophthalmology and Visual Sciences department at Medical College of WisconsinRobert F. Cooper Ph.D Assistant Professor in the Biomedical Engineering department at Marquette University
MESH terms used to index this publication - Major topics in bold
AdolescentAdult
Cell Count
Female
Humans
Male
Middle Aged
Ophthalmoscopy
Prospective Studies
Retinal Cone Photoreceptor Cells
Retinal Photoreceptor Cell Inner Segment
Tomography, Optical Coherence
Vitelliform Macular Dystrophy
Young Adult
