Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration: Classification of Atrophy Meeting Report 4

Robyn H. Guymer, Philip J. Rosenfeld, Christine A. Curcio, Frank G. Holz, Giovanni Staurenghi, K. Bailey Freund, Steffen Schmitz-Valckenberg, Janet Sparrow, Richard F. Spaide, Adnan Tufail, Usha Chakravarthy, Glenn J. Jaffe, Karl Csaky, David Sarraf, Jordi M. Monés, Ramin Tadayoni, Juan Grunwald, Ferdinando Bottoni, Sandra Liakopoulos, Daniel PauleikhoffSergio Pagliarini, Emily Y. Chew, Francesco Viola, Monika Fleckenstein, Barbara A. Blodi, Tock Han Lim, Victor Chong, Jerry Lutty, Alan C. Bird, Srinivas R. Sadda

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalOphthalmology
DOIs
Publication statusAccepted/In press - Jan 1 2019

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Retinal Pigments
Macular Degeneration
Atrophy
Retinal Pigment Epithelium
Consensus
Multimodal Imaging
Aptitude
Choroid
Longitudinal Studies
Disease Progression
Retina
Reading
Outcome Assessment (Health Care)

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Guymer, R. H., Rosenfeld, P. J., Curcio, C. A., Holz, F. G., Staurenghi, G., Freund, K. B., ... Sadda, S. R. (Accepted/In press). Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration: Classification of Atrophy Meeting Report 4. Ophthalmology. https://doi.org/10.1016/j.ophtha.2019.09.035

Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration : Classification of Atrophy Meeting Report 4. / Guymer, Robyn H.; Rosenfeld, Philip J.; Curcio, Christine A.; Holz, Frank G.; Staurenghi, Giovanni; Freund, K. Bailey; Schmitz-Valckenberg, Steffen; Sparrow, Janet; Spaide, Richard F.; Tufail, Adnan; Chakravarthy, Usha; Jaffe, Glenn J.; Csaky, Karl; Sarraf, David; Monés, Jordi M.; Tadayoni, Ramin; Grunwald, Juan; Bottoni, Ferdinando; Liakopoulos, Sandra; Pauleikhoff, Daniel; Pagliarini, Sergio; Chew, Emily Y.; Viola, Francesco; Fleckenstein, Monika; Blodi, Barbara A.; Lim, Tock Han; Chong, Victor; Lutty, Jerry; Bird, Alan C.; Sadda, Srinivas R.

In: Ophthalmology, 01.01.2019.

Research output: Contribution to journalArticle

Guymer, RH, Rosenfeld, PJ, Curcio, CA, Holz, FG, Staurenghi, G, Freund, KB, Schmitz-Valckenberg, S, Sparrow, J, Spaide, RF, Tufail, A, Chakravarthy, U, Jaffe, GJ, Csaky, K, Sarraf, D, Monés, JM, Tadayoni, R, Grunwald, J, Bottoni, F, Liakopoulos, S, Pauleikhoff, D, Pagliarini, S, Chew, EY, Viola, F, Fleckenstein, M, Blodi, BA, Lim, TH, Chong, V, Lutty, J, Bird, AC & Sadda, SR 2019, 'Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration: Classification of Atrophy Meeting Report 4', Ophthalmology. https://doi.org/10.1016/j.ophtha.2019.09.035
Guymer, Robyn H. ; Rosenfeld, Philip J. ; Curcio, Christine A. ; Holz, Frank G. ; Staurenghi, Giovanni ; Freund, K. Bailey ; Schmitz-Valckenberg, Steffen ; Sparrow, Janet ; Spaide, Richard F. ; Tufail, Adnan ; Chakravarthy, Usha ; Jaffe, Glenn J. ; Csaky, Karl ; Sarraf, David ; Monés, Jordi M. ; Tadayoni, Ramin ; Grunwald, Juan ; Bottoni, Ferdinando ; Liakopoulos, Sandra ; Pauleikhoff, Daniel ; Pagliarini, Sergio ; Chew, Emily Y. ; Viola, Francesco ; Fleckenstein, Monika ; Blodi, Barbara A. ; Lim, Tock Han ; Chong, Victor ; Lutty, Jerry ; Bird, Alan C. ; Sadda, Srinivas R. / Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration : Classification of Atrophy Meeting Report 4. In: Ophthalmology. 2019.
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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.",
author = "Guymer, {Robyn H.} and Rosenfeld, {Philip J.} and Curcio, {Christine A.} and Holz, {Frank G.} and Giovanni Staurenghi and Freund, {K. Bailey} and Steffen Schmitz-Valckenberg and Janet Sparrow and Spaide, {Richard F.} and Adnan Tufail and Usha Chakravarthy and Jaffe, {Glenn J.} and Karl Csaky and David Sarraf and Mon{\'e}s, {Jordi M.} and Ramin Tadayoni and Juan Grunwald and Ferdinando Bottoni and Sandra Liakopoulos and Daniel Pauleikhoff and Sergio Pagliarini and Chew, {Emily Y.} and Francesco Viola and Monika Fleckenstein and Blodi, {Barbara A.} and Lim, {Tock Han} and Victor Chong and Jerry Lutty and Bird, {Alan C.} and Sadda, {Srinivas R.}",
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T1 - Incomplete Retinal Pigment Epithelial and Outer Retinal Atrophy in Age-Related Macular Degeneration

T2 - Classification of Atrophy Meeting Report 4

AU - Guymer, Robyn H.

AU - Rosenfeld, Philip J.

AU - Curcio, Christine A.

AU - Holz, Frank G.

AU - Staurenghi, Giovanni

AU - Freund, K. Bailey

AU - Schmitz-Valckenberg, Steffen

AU - Sparrow, Janet

AU - Spaide, Richard F.

AU - Tufail, Adnan

AU - Chakravarthy, Usha

AU - Jaffe, Glenn J.

AU - Csaky, Karl

AU - Sarraf, David

AU - Monés, Jordi M.

AU - Tadayoni, Ramin

AU - Grunwald, Juan

AU - Bottoni, Ferdinando

AU - Liakopoulos, Sandra

AU - Pauleikhoff, Daniel

AU - Pagliarini, Sergio

AU - Chew, Emily Y.

AU - Viola, Francesco

AU - Fleckenstein, Monika

AU - Blodi, Barbara A.

AU - Lim, Tock Han

AU - Chong, Victor

AU - Lutty, Jerry

AU - Bird, Alan C.

AU - Sadda, Srinivas R.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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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