CHARACTERIZING THE NATURAL HISTORY OF FOVEAL-SPARING ATROPHIC LATE-ONSET RETINAL DEGENERATION. Issue 6 (June 2021)

• Record Type: Journal Article
• Authors: Borooah, Shyamanga; Papastavrou, Vasileios T.; Lando, Leonardo; Moghimi, Sasan; Lin, Tiezhu; Dans, Kunny; Motevasseli, Tahmineh; Cameron, James R.; Freeman, William R.; Dhillon, Baljean; Browning, Andrew C.
• Is Part Of: Retina. Volume 41:Issue 6(2021)
• Publication Date: 2021-06
• Subjects: retina -- macular degeneration -- optical coherence tomography -- fundus autofluorescence; Retina -- Diseases -- Periodicals; Retinal Diseases; Vitreous Body; 617.735
• DOI: 10.1097/IAE.0000000000003017
• Languages: English
• ISSNs: 0275-004X

Abstract

Abstract : Purpose: To identify quantifiable markers of disease progression in patients with foveal-sparing atrophic late-onset retinal degeneration using fundus autofluorescence and spectral-domain optical coherence tomography imaging. Methods: Natural history study evaluating patients within a 3-year interval. Disease progression was assessed based on the area of retinal atrophy, macular topographic distribution of lesions, retinal and choroidal thickness and volume, and choroidal vascularity index. Results: Twenty-four eyes (12 individuals) were included for fundus autofluorescence, and 31 eyes (16 individuals) for spectral-domain optical coherence tomography studies. Measurements were symmetrical between eyes of the same patient. The area of atrophy significantly enlarged ( P = 0.002), with a growth rate of 2.67 mm 2 /year (SD: 2.13; square rooted: 0.57 mm/year, SD = 0.34). Baseline area of atrophy and progression both correlated with age. Most atrophic lesions were found in the temporal macula and progressed nasally at follow-up. Central choroidal and retinal thicknesses and volume in late-onset retinal degeneration cases were significantly reduced compared with controls, but only central retinal thickness decreased significantly at follow-up. Conclusion: This study identifies the area of atrophy and central retinal thickness, but not chorioretinal volume or choroidal thickness, as markers of short-term progression in late-onset retinal degeneration. These findings may be useful for disease monitoring and late-onset retinal degeneration interventional studies. Abstract : Supplemental Digital Content is Available in the Text.In this prospective, a natural history study of late-onset retinal degeneration, area of atrophy, and central retinal thickness changed significantly with follow-up. Choroidal thickness and volume were significantly reduced compared with normal controls. All measures were validated and had a high interocular correlation.