Etienne M Schönbach1, Yulia Wolfson1, Rupert W Strauss2, Mohamed A Ibrahim1, Xiangrong Kong1, Beatriz Muñoz1, David G Birch3, Artur V Cideciyan4, Gesa-Astrid Hahn5, Muneeswar Nittala6, Janet S Sunness7, SriniVas R Sadda8, Sheila K West1, Hendrik P N Scholl9. 1. Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland. 2. Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland2Moorfields Eye Hospital, London, United Kingdom3Department of Ophthalmology, Johannes Kepler University, Linz, Austria4Department of Ophthalmology, Medical University, Graz, Austria5Department of Ophthalmology, University of Basel, Switzerland. 3. Retina Foundation of the Southwest, Dallas, Texas. 4. Scheie Eye Institute, University of Pennsylvania, Philadelphia. 5. Center for Ophthalmology, Eberhard Karls Universität, Tübingen, Germany. 6. Doheny Eye Institute, Los Angeles, California. 7. Hoover Low Vision Rehabilitation Services, Greater Baltimore Medical Center, Baltimore, Maryland. 8. Doheny Eye Institute, Los Angeles, California11University of California, Los Angeles David Geffen School of Medicine. 9. Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland5Department of Ophthalmology, University of Basel, Switzerland.
Abstract
Importance: New outcome measures for treatment trials for Stargardt disease type 1 (STGD1) and other macular diseases are needed. Microperimetry allows mapping of light sensitivity of the macula and provides topographic information on visual function beyond visual acuity. Objective: To measure and analyze retinal light sensitivity of the macula in STGD1 using fundus-controlled perimetry (microperimetry). Design, Setting, and Participants: This was a multicenter prospective cohort study. A total of 199 patients and 326 eyes with molecularly confirmed (ABCA4) STGD1 underwent testing with the Nidek MP-1 microperimeter as part of the multicenter, prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) study. Sensitivity of 68 retinal loci was tested, and the mean sensitivity (MS) was determined; each point was categorized as "normal," "relative," or "deep" scotoma. Main Outcomes and Measures: Mean sensitivity and the number of points with normal sensitivity, relative, or deep scotomas. Results: Mean (SD) patient age was 34.2 (14.7) years, mean (SD) best-corrected visual acuity of all eyes was 47.8 (16.9) Early Treatment Diabetic Retinopathy Study letter score (approximately 20/100 Snellen equivalent), and mean MS of all eyes of all 68 points was 11.0 (5.0) dB. The median number of normal points per eye was 49 (mean [SD], 41.3 [20.8]; range, 0-68); abnormal sensitivity and deep scotomas were more prevalent in the central macula. Mean sensitivity was lower in the fovea (mean [SD], 2.7 [4.4] dB) than in the inner (mean [SD], 6.8 [5.8] dB) and outer ring (mean [SD], 12.7 [5.3] dB). Overall MS per eye was 0.086 dB lower per year of additional age (95% CI, -0.13 to -0.041; P < .001) and 0.21 dB lower per additional year of duration of STGD1 (95% CI, -0.28 to -0.14; P < .001). Longer duration of STGD1 was associated with worse MS (β = -0.18; P < .001), with a lower number of normal test points (β = -0.71; P < .001), and with a higher number of deep scotoma points (β = -0.70; P < .001). We found 11 eyes with low MS (<6 dB) but very good best-corrected visual acuity of at least 72 Early Treatment Diabetic Retinopathy Study letter score (20/40 Snellen equivalent). Conclusions and Relevance: We provide an extensive analysis of macular sensitivity parameters in STGD1 and demonstrate their association with demographic characteristics and vision. These data suggest microperimetry testing provides a more comprehensive assessment of retinal function and will be an important outcome measure in future clinical trials.
Importance: New outcome measures for treatment trials for Stargardt disease type 1 (STGD1) and other macular diseases are needed. Microperimetry allows mapping of light sensitivity of the macula and provides topographic information on visual function beyond visual acuity. Objective: To measure and analyze retinal light sensitivity of the macula in STGD1 using fundus-controlled perimetry (microperimetry). Design, Setting, and Participants: This was a multicenter prospective cohort study. A total of 199 patients and 326 eyes with molecularly confirmed (ABCA4) STGD1 underwent testing with the Nidek MP-1 microperimeter as part of the multicenter, prospective Natural History of the Progression of Atrophy Secondary to Stargardt Disease (ProgStar) study. Sensitivity of 68 retinal loci was tested, and the mean sensitivity (MS) was determined; each point was categorized as "normal," "relative," or "deep" scotoma. Main Outcomes and Measures: Mean sensitivity and the number of points with normal sensitivity, relative, or deep scotomas. Results: Mean (SD) patient age was 34.2 (14.7) years, mean (SD) best-corrected visual acuity of all eyes was 47.8 (16.9) Early Treatment Diabetic Retinopathy Study letter score (approximately 20/100 Snellen equivalent), and mean MS of all eyes of all 68 points was 11.0 (5.0) dB. The median number of normal points per eye was 49 (mean [SD], 41.3 [20.8]; range, 0-68); abnormal sensitivity and deep scotomas were more prevalent in the central macula. Mean sensitivity was lower in the fovea (mean [SD], 2.7 [4.4] dB) than in the inner (mean [SD], 6.8 [5.8] dB) and outer ring (mean [SD], 12.7 [5.3] dB). Overall MS per eye was 0.086 dB lower per year of additional age (95% CI, -0.13 to -0.041; P < .001) and 0.21 dB lower per additional year of duration of STGD1 (95% CI, -0.28 to -0.14; P < .001). Longer duration of STGD1 was associated with worse MS (β = -0.18; P < .001), with a lower number of normal test points (β = -0.71; P < .001), and with a higher number of deep scotoma points (β = -0.70; P < .001). We found 11 eyes with low MS (<6 dB) but very good best-corrected visual acuity of at least 72 Early Treatment Diabetic Retinopathy Study letter score (20/40 Snellen equivalent). Conclusions and Relevance: We provide an extensive analysis of macular sensitivity parameters in STGD1 and demonstrate their association with demographic characteristics and vision. These data suggest microperimetry testing provides a more comprehensive assessment of retinal function and will be an important outcome measure in future clinical trials.
Authors: Maria Fernanda Abalem; Benjamin Otte; Chris Andrews; Katherine A Joltikov; Kari Branham; Abigail T Fahim; Dana Schlegel; Cynthia X Qian; John R Heckenlively; Thiran Jayasundera Journal: Am J Ophthalmol Date: 2017-10-14 Impact factor: 5.258
Authors: Etienne M Schönbach; Rupert W Strauss; Beatriz Muñoz; Yulia Wolfson; Mohamed A Ibrahim; David G Birch; Eberhart Zrenner; Janet S Sunness; Michael S Ip; SriniVas R Sadda; Sheila K West; Hendrik P N Scholl Journal: JAMA Ophthalmol Date: 2020-07-01 Impact factor: 7.389
Authors: Xiangrong Kong; Mohamed Ibrahim-Ahmed; Millena G Bittencourt; Rupert W Strauss; David G Birch; Artur V Cideciyan; Ann-Margaret Ervin; Alexander Ho; Janet S Sunness; Isabelle S Audo; Michel Michaelides; Eberhart Zrenner; SriniVas Sadda; Michael S Ip; Sheila West; Hendrik P N Scholl Journal: Am J Ophthalmol Date: 2021-10-23 Impact factor: 5.258
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Authors: Jason Charng; Tina M Lamey; Jennifer A Thompson; Terri L McLaren; Mary S Attia; Ian L McAllister; Ian J Constable; David A Mackey; John N De Roach; Fred K Chen Journal: Transl Vis Sci Technol Date: 2020-09-09 Impact factor: 3.283
Authors: Jacque L Duncan; Eric A Pierce; Amy M Laster; Stephen P Daiger; David G Birch; John D Ash; Alessandro Iannaccone; John G Flannery; José A Sahel; Donald J Zack; Marco A Zarbin Journal: Transl Vis Sci Technol Date: 2018-07-18 Impact factor: 3.283