Consensus Definition for Atrophy Associated with Age-Related Macular Degeneration on OCT

Classification of Atrophy Report 3
Published:November 02, 2017DOI:


      To develop consensus terminology and criteria for defining atrophy based on OCT findings in the setting of age-related macular degeneration (AMD).


      Consensus meeting.


      Panel of retina specialists, image reading center experts, retinal histologists, and optics engineers.


      As part of the Classification of Atrophy Meetings (CAM) program, an international group of experts surveyed the existing literature, performed a masked analysis of longitudinal multimodal imaging for a series of eyes with AMD, and reviewed the results of this analysis to define areas of agreement and disagreement. Through consensus discussions at 3 meetings over 12 months, a classification system based on OCT was proposed for atrophy secondary to AMD. Specific criteria were defined to establish the presence of atrophy.

      Main Outcome Measures

      A consensus classification system for atrophy and OCT-based criteria to identify atrophy.


      OCT was proposed as the reference standard or base imaging method to diagnose and stage atrophy. Other methods, including fundus autofluorescence, near-infrared reflectance, and color imaging, provided complementary and confirmatory information. Recognizing that photoreceptor atrophy can occur without retinal pigment epithelium (RPE) atrophy and that atrophy can undergo an evolution of different stages, 4 terms and histologic candidates were proposed: complete RPE and outer retinal atrophy (cRORA), incomplete RPE and outer retinal atrophy, complete outer retinal atrophy, and incomplete outer retinal atrophy. Specific OCT criteria to diagnose cRORA were proposed: (1) a region of hypertransmission of at least 250 μm in diameter, (2) a zone of attenuation or disruption of the RPE of at least 250 μm in diameter, (3) evidence of overlying photoreceptor degeneration, and (4) absence of scrolled RPE or other signs of an RPE tear.


      A classification system and criteria for OCT-defined atrophy in the setting of AMD has been proposed based on an international consensus. This classification is a more complete representation of changes that occur in AMD than can be detected using color fundus photography alone. Longitudinal information is required to validate the implied risk of vision loss associated with these terms. This system will enable such future studies to be undertaken using consistent definitions.

      Abbreviations and Acronyms:

      AMD (age-related macular degeneration), CAM (Classification of Atrophy Meetings), CFP (color fundus photography), CNV (choroidal neovascularization), cRORA (complete retinal pigment epithelium and outer retinal atrophy), FAF (fundus autofluorescence), GA (geographic atrophy), iRORA (incomplete retinal pigment epithelium and outer retinal atrophy), RPD (reticular pseudodrusen), RPE (retinal pigment epithelium)
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        • Ferris 3rd, F.L.
        • Wilkinson C.P.
        • Bird A.
        • et al.
        Clinical classification of age-related macular degeneration.
        Ophthalmology. 2013; 120: 844-851
        • Holz F.G.
        • Strauss E.C.
        • Schmitz-Valckenberg S.
        • van Lookeren Campagne M.
        Geographic atrophy: clinical features and potential therapeutic approaches.
        Ophthalmology. 2014; 121: 1079-1091
        • Gass J.D.
        Stereoscopic Atlas of Macular Diseases.
        1st ed. The C. V. Mosby Company, St. Louis1970
        • Schmitz-Valckenberg S.
        • Sadda S.
        • Staurenghi G.
        • et al.
        Geographic atrophy: semantic considerations and literature review.
        Retina. 2016; 36: 2250-2264
        • Haab O.
        Erkrankungen der Macula Lutea.
        Centralblat Augenheilkd. 1885; 9: 384-391
        • Nettleship E.
        Central senile areolar choroidal atrophy.
        Trans Ophthalmol Soc U K. 1884; 4: 165-167
        • Behr C.
        Die Heredodegeneration der Makula.
        Monatsblätter für Augenheilkunde. 1920; 65: 465-505
        • Sarks S.H.
        Senile choroidal sclerosis.
        Br J Ophthalmol. 1973; 57: 98-109
        • Klien B.A.
        The heredodegeneration of the macula lutea—diagnostic and differential diagnostic considerations and a histopathologic report.
        Am J Ophthalmol. 1950; 33: 371-379
        • Klein R.
        • Davis M.D.
        • Magli Y.L.
        • et al.
        The Wisconsin age-related maculopathy grading system.
        Ophthalmology. 1991; 98: 1128-1134
        • Age-Related Eye Disease Study Group
        The Age-Related Eye Disease Study system for classifying age-related macular degeneration from stereoscopic color fundus photographs: the Age-Related Eye Disease Study report number 6.
        Am J Ophthalmol. 2001; 132: 668-681
        • Sarks J.P.
        • Sarks S.H.
        • Killingsworth M.C.
        Evolution of geographic atrophy of the retinal pigment epithelium.
        Eye (Lond). 1988; 2: 552-577
        • Sunness J.S.
        • Bressler N.M.
        • Tian Y.
        • et al.
        Measuring geographic atrophy in advanced age-related macular degeneration.
        Invest Ophthalmol Vis Sci. 1999; 40: 1761-1769
        • Mitchell P.
        • Smith W.
        • Attebo K.
        • Wang J.J.
        Prevalence of age-related maculopathy in Australia. The Blue Mountains Eye Study.
        Ophthalmology. 1995; 102: 1450-1460
        • Bird A.C.
        • Bressler N.M.
        • Bressler S.B.
        • et al.
        An international classification and grading system for age-related maculopathy and age-related macular degeneration. The International ARM Epidemiological Study Group.
        Surv Ophthalmol. 1995; 39: 367-374
        • Schmitz-Valckenberg S.
        • Sahel J.A.
        • Danis R.
        • et al.
        Natural history of geographic atrophy progression secondary to age-related macular degeneration (Geographic Atrophy Progression Study).
        Ophthalmology. 2016; 123: 361-368
        • Jaffe G.J.
        • Schmitz-Valckenberg S.
        • Boyer D.
        • et al.
        Randomized trial to evaluate tandospirone in geographic atrophy secondary to age-related macular degeneration: the GATE study.
        Am J Ophthalmol. 2015; 160: 1226-1234
        • von Ruckmann A.
        • Fitzke F.W.
        • Bird A.C.
        Distribution of fundus autofluorescence with a scanning laser ophthalmoscope.
        Br J Ophthalmol. 1995; 79: 407-412
        • Schmitz-Valckenberg S.
        • Brinkmann C.K.
        • Alten F.
        • et al.
        Semiautomated image processing method for identification and quantification of geographic atrophy in age-related macular degeneration.
        Invest Ophthalmol Vis Sci. 2011; 52: 7640-7646
        • Hwang J.C.
        • Chan J.W.
        • Chang S.
        • Smith R.T.
        Predictive value of fundus autofluorescence for development of geographic atrophy in age-related macular degeneration.
        Invest Ophthalmol Vis Sci. 2006; 47: 2655-2661
        • Lindner M.
        • Boker A.
        • Mauschitz M.M.
        • et al.
        Directional kinetics of geographic atrophy progression in age-related macular degeneration with foveal sparing.
        Ophthalmology. 2015; 122: 1356-1365
        • Wolf-Schnurrbusch U.E.
        • Wittwer V.V.
        • Ghanem R.
        • et al.
        Blue-light versus green-light autofluorescence: lesion size of areas of geographic atrophy.
        Invest Ophthalmol Vis Sci. 2011; 52: 9497-9502
        • Pfau M.
        • Goerdt L.
        • Schmitz-Valckenberg S.
        • et al.
        Green-light autofluorescence versus combined blue-light autofluorescence and near-infrared reflectance imaging in geographic atrophy secondary to age-related macular degeneration.
        Invest Ophthalmol Vis Sci. 2017; 58: BIO121-BIO130
        • Schmitz-Valckenberg S.
        • Fleckenstein M.
        • Gobel A.P.
        • et al.
        Evaluation of autofluorescence imaging with the scanning laser ophthalmoscope and the fundus camera in age-related geographic atrophy.
        Am J Ophthalmol. 2008; 146: 183-192
        • Fleckenstein M.
        • Charbel Issa P.
        • Helb H.M.
        • et al.
        High-resolution spectral domain-OCT imaging in geographic atrophy associated with age-related macular degeneration.
        Invest Ophthalmol Vis Sci. 2008; 49: 4137-4144
        • Yehoshua Z.
        • de Amorim Garcia Filho C.A.
        • Nunes R.P.
        • et al.
        Comparison of geographic atrophy growth rates using different imaging modalities in the COMPLETE Study.
        Ophthalmic Surg Lasers Imaging Retina. 2015; 46: 413-422
        • Hu Z.
        • Medioni G.G.
        • Hernandez M.
        • et al.
        Segmentation of the geographic atrophy in spectral-domain optical coherence tomography and fundus autofluorescence images.
        Invest Ophthalmol Vis Sci. 2013; 54: 8375-8383
        • Lujan B.J.
        • Rosenfeld P.J.
        • Gregori G.
        • et al.
        Spectral domain optical coherence tomographic imaging of geographic atrophy.
        Ophthalmic Surg Lasers Imaging. 2009; 40: 96-101
        • Yehoshua Z.
        • Rosenfeld P.J.
        • Gregori G.
        • et al.
        Progression of geographic atrophy in age-related macular degeneration imaged with spectral domain optical coherence tomography.
        Ophthalmology. 2011; 118: 679-686
        • Schaal K.B.
        • Gregori G.
        • Rosenfeld P.J.
        En face optical coherence tomography imaging for the detection of nascent geographic atrophy.
        Am J Ophthalmol. 2017; 174: 145-154
        • Giocanti-Auregan A.
        • Tadayoni R.
        • Fajnkuchen F.
        • et al.
        Predictive value of outer retina en face OCT imaging for geographic atrophy progression.
        Invest Ophthalmol Vis Sci. 2015; 56: 8325-8330
        • Wu Z.
        • Luu C.D.
        • Ayton L.N.
        • et al.
        Optical coherence tomography-defined changes preceding the development of drusen-associated atrophy in age-related macular degeneration.
        Ophthalmology. 2014; 121: 2415-2422
        • Csaky K.G.
        • Richman E.A.
        • Ferris 3rd, F.L.
        Report from the NEI/FDA Ophthalmic Clinical Trial Design and Endpoints Symposium.
        Invest Ophthalmol Vis Sci. 2008; 49: 479-489
        • Spaide R.F.
        Outer retinal atrophy after regression of subretinal drusenoid deposits as a newly recognized form of late age-related macular degeneration.
        Retina. 2013; 33: 1800-1808
        • Abdelfattah N.S.
        • Al-Sheikh M.
        • Pitetta S.
        • et al.
        Macular atrophy in neovascular age-related macular degeneration with monthly versus treat-and-extend ranibizumab: findings from the TREX-AMD Trial.
        Ophthalmology. 2017; 124: 215-223
        • Bhisitkul R.B.
        • Mendes T.S.
        • Rofagha S.
        • et al.
        Macular atrophy progression and 7-year vision outcomes in subjects from the ANCHOR, MARINA and HORIZON studies (SEVEN-UP Study).
        Am J Ophthalmol. 2015; 159: 915-924
        • Pilotto E.
        • Convento E.
        • Guidolin F.
        • et al.
        Microperimetry features of geographic atrophy identified with en face optical coherence tomography.
        JAMA Ophthalmol. 2016; 134: 873-879
        • Grunwald J.E.
        • Pistilli M.
        • Ying G.S.
        • et al.
        Growth of geographic atrophy in the comparison of age-related macular degeneration treatments trials.
        Ophthalmology. 2015; 122: 809-816
        • Grunwald J.E.
        • Pistilli M.
        • Daniel E.
        • et al.
        Incidence and growth of geographic atrophy during 5 years of Comparison of Age-Related Macular Degeneration Treatments Trials.
        Ophthalmology. 2017; 124: 97-104
        • Staurenghi G.
        • Sadda S.
        • Chakravarthy U.
        • Spaide R.F.
        Proposed lexicon for anatomic landmarks in normal posterior segment spectral-domain optical coherence tomography: the IN*OCT consensus.
        Ophthalmology. 2014; 121: 1572-1578
        • Holz F.G.
        • Sadda S.R.
        • Staurenghi G.
        • et al.
        Imaging protocols in clinical studies in advanced age-related macular degeneration: recommendations from Classification of Atrophy Consensus Meetings.
        Ophthalmology. 2017; 124: 464-478
        • Zanzottera E.C.
        • Messinger J.D.
        • Ach T.
        • et al.
        The Project MACULA retinal pigment epithelium grading system for histology and optical coherence tomography in age-related macular degeneration.
        Invest Ophthalmol Vis Sci. 2015; 56: 3253-3268
        • Curcio C.A.
        • Messinger J.D.
        • Sloan K.R.
        • et al.
        Subretinal drusenoid deposits in non-neovascular age-related macular degeneration: morphology, prevalence, topography, and biogenesis model.
        Retina. 2013; 33: 265-276
        • Polyak S.L.
        The Retina.
        The University of Chicago Press, Chicago1941
        • Ooto S.
        • Vongkulsiri S.
        • Sato T.
        • et al.
        Outer retinal corrugations in age-related macular degeneration.
        JAMA Ophthalmol. 2014; 132: 806-813
        • Tan A.C.S.
        • Astroz P.
        • Dansingani K.K.
        • et al.
        The evolution of the plateau, an optical coherence tomography signature seen in geographic atrophy.
        Invest Ophthalmol Vis Sci. 2017; 58: 2349-2358
        • Green W.R.
        • Enger C.
        Age-related macular degeneration histopathologic studies. The 1992 Lorenz E. Zimmerman Lecture.
        Ophthalmology. 1993; 100: 1519-1535
        • Schaal K.B.
        • Rosenfeld P.J.
        • Gregori G.
        • et al.
        Anatomic clinical trial endpoints for nonexudative age-related macular degeneration.
        Ophthalmology. 2016; 123: 1060-1079
        • Lek J.J.
        • Brassington K.H.
        • Luu C.D.
        • et al.
        Subthreshold nanosecond laser intervention in intermediate age-related macular degeneration.
        Ophthalmol Retina. 2017; 1: 227-239
        • Danis R.P.
        • Domalpally A.
        • Chew E.Y.
        • et al.
        Methods and reproducibility of grading optimized digital color fundus photographs in the Age-Related Eye Disease Study 2 (AREDS2 report number 2).
        Invest Ophthalmol Vis Sci. 2013; 54: 4548-4554

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      • Corrigendum
        OphthalmologyVol. 126Issue 1
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          The authors of “Consensus Definition for Atrophy Associated with Age-related Macular Degeneration on OCT: Classification of Atrophy Report 3” (Ophthalmology. 2018;125:537–548) would like to issue the following correction:
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