Vitor K L Takahashi1,2,3, Júlia T Takiuti1,2,4, Jose R L Carvalho-Jr1,2,3,5, Christine L Xu1,2, Jimmy K Duong6, Vinit B Mahajan7,8, Stephen H Tsang9,10,11,12. 1. Department of Ophthalmology, Columbia University, New York, NY, USA. 2. Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Departments of Ophthalmology, Pathology & Cell Biology, Columbia Stem Cell Initiative, Institute of Human Nutrition, Columbia University, New York, NY, USA. 3. Department of Ophthalmology, Federal University of São Paulo, Sao Paulo, Brazil. 4. Division of Ophthalmology, University of São Paulo Medical School, Sao Paulo, Brazil. 5. Empresa Brasileira de Servicos Hospitalares (EBSERH) - Hospital das Clinicas de Pernambuco (HCPE), Departament of Ophthalmology, Federal University of Pernambuco (UFPE), Recife, Brazil. 6. Department of Biostatistics, Columbia University, New York, NY, USA. 7. Omics Laboratory, Department of Ophthalmology, Byers Eye Institute, Stanford University School of Medicine, Palo Alto, CA, USA. 8. Veterans Affairs Palo Alto Health Care System, Palo Alto, CA, USA. 9. Department of Ophthalmology, Columbia University, New York, NY, USA. sht2@cumc.columbia.edu. 10. Jonas Children's Vision Care, and the Bernard & Shirlee Brown Glaucoma Laboratory, Departments of Ophthalmology, Pathology & Cell Biology, Columbia Stem Cell Initiative, Institute of Human Nutrition, Columbia University, New York, NY, USA. sht2@cumc.columbia.edu. 11. Department of Pathology & Cell Biology, Stem Cell Initiative (CSCI), College of Physicians and Surgeons, Institute of Human Nutrition, Columbia University, New York, NY, USA. sht2@cumc.columbia.edu. 12. Columbia University Medical Center, Harkness Eye Institute, 635 West 165th Street, Box 212, New York, NY, 10032, USA. sht2@cumc.columbia.edu.
Abstract
PURPOSE: To evaluate the progression of retinitis pigmentosa (RP) due to mutations in rhodopsin (RHO) by measuring the short-wavelength autofluorescence (SW-AF) increased autofluorescence ring and ellipsoid zone (EZ)-line width. METHODS: Fundus autofluorescence (FAF) and spectral domain optical coherence tomography (SD-OCT) images were obtained from 10 patients with autosomal dominant RP due to mutations in the RHO gene. Measurements of ring area on FAF images, as well as the EZ line width on SD-OCT images and horizontal, vertical diameter, were performed by two independent masked graders. RESULTS: The ring area decreased by a rate of 0.6 ± 0.2 mm2 per year. We observed that the EZ line width decreased by an average of 152 ± 37 μm per year, while the horizontal and vertical diameters decreased by 106 ± 35 μm and 125 ± 29 μm per year, respectively. Progression rates were similar between eyes. CONCLUSIONS: We observed SW-AF ring constriction and a progressive loss of EZ line width over time.
PURPOSE: To evaluate the progression of retinitis pigmentosa (RP) due to mutations in rhodopsin (RHO) by measuring the short-wavelength autofluorescence (SW-AF) increased autofluorescence ring and ellipsoid zone (EZ)-line width. METHODS: Fundus autofluorescence (FAF) and spectral domain optical coherence tomography (SD-OCT) images were obtained from 10 patients with autosomal dominant RP due to mutations in the RHO gene. Measurements of ring area on FAF images, as well as the EZ line width on SD-OCT images and horizontal, vertical diameter, were performed by two independent masked graders. RESULTS: The ring area decreased by a rate of 0.6 ± 0.2 mm2 per year. We observed that the EZ line width decreased by an average of 152 ± 37 μm per year, while the horizontal and vertical diameters decreased by 106 ± 35 μm and 125 ± 29 μm per year, respectively. Progression rates were similar between eyes. CONCLUSIONS: We observed SW-AF ring constriction and a progressive loss of EZ line width over time.
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