Robyn H Guymer1, Philip J Rosenfeld2, Christine A Curcio3, Frank G Holz4, Giovanni Staurenghi5, K Bailey Freund6, Steffen Schmitz-Valckenberg4, Janet Sparrow7, Richard F Spaide6, Adnan Tufail8, Usha Chakravarthy9, Glenn J Jaffe10, Karl Csaky11, David Sarraf12, Jordi M Monés13, Ramin Tadayoni14, Juan Grunwald15, Ferdinando Bottoni5, Sandra Liakopoulos16, Daniel Pauleikhoff17, Sergio Pagliarini18, Emily Y Chew19, Francesco Viola20, Monika Fleckenstein4, Barbara A Blodi21, Tock Han Lim22, Victor Chong23, Jerry Lutty24, Alan C Bird8, Srinivas R Sadda25. 1. Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, University of Melbourne, Department of Surgery (Ophthalmology), Melbourne, Australia. Electronic address: rhg@unimelb.edu.au. 2. Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida. 3. Department of Ophthalmology and Visual Sciences, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama. 4. Department of Ophthalmology, University of Bonn, Bonn, Germany. 5. Eye Clinic, Department of Biomedical and Clinical Sciences "Luigi Sacco," Luigi Sacco Hospital, University of Milan, Milan, Italy. 6. Vitreous Retina Macula Consultants of New York, New York, New York. 7. Departments of Ophthalmology and Pathology and Cell Biology, Columbia University Medical Center, New York, New York. 8. Institute of Ophthalmology, University College London, London, United Kingdom. 9. Center for Public Health, The Queen's University of Belfast, Belfast, United Kingdom. 10. Department of Ophthalmology, Duke University, Durham, North Carolina. 11. Retina Foundation of the Southwest, Dallas, Texas. 12. Stein Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California. 13. Institut de la Màcula and Barcelona Macula Foundation, Barcelona, Spain. 14. Department of Ophthalmology, Hôpital Lariboisière, AP-HP, Université Paris 7-Sorbonne, Paris, France. 15. Department of Ophthalmology, University of Pennsylvania, Philadelphia, Pennsylvania. 16. Department of Ophthalmology, University of Cologne, Cologne, Germany. 17. Department of Ophthalmology, St. Franziskus Hospital, Münster, German. 18. Department of Ophthalmology, University Hospitals Coventry & Warwickshire, Coventry, United Kingdom. 19. National Eye Institute, National Institutes of Health, Bethesda, Maryland. 20. Cà Granda Foundation, Ospedale Maggiore Policlinico, University of Milan, Milan, Italy. 21. Department of Ophthalmology and Visual Sciences, Fundus Photograph Reading Center, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin. 22. National Healthcare Group Eye Institute, Tan Tock Seng Hospital, Singapore, Republic of Singapore. 23. University of Oxford, Oxford, United Kingdom. 24. Wilmer Ophthalmological Institute, Johns Hopkins Hospital, Baltimore, Maryland. 25. Doheny Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California.
Abstract
PURPOSE: To describe the defining features of incomplete retinal pigment epithelium (RPE) and outer retinal atrophy (iRORA), a consensus term referring to the OCT-based anatomic changes often identified before the development of complete RPE and outer retinal atrophy (cRORA) in age-related macular degeneration (AMD). We provide descriptive OCT and histologic examples of disease progression. DESIGN: Consensus meeting. PARTICIPANTS: Panel of retina specialists, including retinal imaging experts, reading center leaders, and retinal histologists. METHODS: As part of the Classification of Atrophy Meeting (CAM) program, an international group of experts analyzed and discussed longitudinal multimodal imaging of eyes with AMD. Consensus was reached on a classification system for OCT-based structural alterations that occurred before the development of atrophy secondary to AMD. New terms of iRORA and cRORA were defined. This report describes in detail the CAM consensus on iRORA. MAIN OUTCOME MEASURES: Defining the term iRORA through OCT imaging and longitudinal cases showing progression of atrophy, with histologic correlates. RESULTS: OCT was used in cases of early and intermediate AMD as the base imaging method to identify cases of iRORA. In the context of drusen, iRORA is defined on OCT as (1) a region of signal hypertransmission into the choroid, (2) a corresponding zone of attenuation or disruption of the RPE, and (3) evidence of overlying photoreceptor degeneration. The term iRORA should not be used when there is an RPE tear. Longitudinal studies confirmed the concept of progression from iRORA to cRORA. CONCLUSIONS: An international consensus classification for OCT-defined anatomic features of iRORA are described and examples of longitudinal progression to cRORA are provided. The ability to identify these OCT changes reproducibly is essential to understand better the natural history of the disease, to identify high-risk signs of progression, and to study early interventions. Longitudinal data are required to quantify the implied risk of vision loss associated with these terms. The CAM classification provides initial definitions to enable these future endeavors, acknowledging that the classification will be refined as new data are generated.
PURPOSE: To describe the defining features of incomplete retinal pigment epithelium (RPE) and outer retinal atrophy (iRORA), a consensus term referring to the OCT-based anatomic changes often identified before the development of complete RPE and outer retinal atrophy (cRORA) in age-related macular degeneration (AMD). We provide descriptive OCT and histologic examples of disease progression. DESIGN: Consensus meeting. PARTICIPANTS: Panel of retina specialists, including retinal imaging experts, reading center leaders, and retinal histologists. METHODS: As part of the Classification of Atrophy Meeting (CAM) program, an international group of experts analyzed and discussed longitudinal multimodal imaging of eyes with AMD. Consensus was reached on a classification system for OCT-based structural alterations that occurred before the development of atrophy secondary to AMD. New terms of iRORA and cRORA were defined. This report describes in detail the CAM consensus on iRORA. MAIN OUTCOME MEASURES: Defining the term iRORA through OCT imaging and longitudinal cases showing progression of atrophy, with histologic correlates. RESULTS:OCT was used in cases of early and intermediate AMD as the base imaging method to identify cases of iRORA. In the context of drusen, iRORA is defined on OCT as (1) a region of signal hypertransmission into the choroid, (2) a corresponding zone of attenuation or disruption of the RPE, and (3) evidence of overlying photoreceptor degeneration. The term iRORA should not be used when there is an RPE tear. Longitudinal studies confirmed the concept of progression from iRORA to cRORA. CONCLUSIONS: An international consensus classification for OCT-defined anatomic features of iRORA are described and examples of longitudinal progression to cRORA are provided. The ability to identify these OCT changes reproducibly is essential to understand better the natural history of the disease, to identify high-risk signs of progression, and to study early interventions. Longitudinal data are required to quantify the implied risk of vision loss associated with these terms. The CAM classification provides initial definitions to enable these future endeavors, acknowledging that the classification will be refined as new data are generated.
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