Irene Leung, Ferenc B. Sallo*, Roberto Bonelli, Traci E. Clemons, Daniel Pauleikhoff, Emily Y. Chew, Alan C. Bird, Tunde Peto, Jose Alain Sahel, Robyn Guymer, Gisele Soubrane, Alain Gaudric, Steven Schwartz, Ian Constable, Michael Cooney, Catherine Egan, Lawrence Singerman, Mark C. Gillies, Martin Friedlander, Daniel PauleikhoffJoseph Moisseiev, Richard Rosen, Robert Murphy, Frank Holz, Grant Comer, Barbara Blodi, Diana Do, Alexander Brucker, Raja Narayanan, Sebastian Wolf, Philip Rosenfeld, Paul S. Bernstein, Lawrence Yannuzzi, Jacque Duncan, Mina Chung, Jiong Yan, David Weinberg, Clasien Oomen, Joan W. Miller

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

27 Scopus citations


Purpose: Pigment in the midretina is a characteristic sign in Type 2 idiopathic macular telangiectasia (MacTel) and is considered to characterize the late stage of the disease. Our aim was to investigate its incidence, and relationship with risk factors for MacTel, including outer retinal vascularization and subretinal neovascular proliferation (SRNV). Methods: Pigment extent was measured in fundus autofluorescence images of 150 eyes of 75 MacTel probands, using the Region Finder tool of Heidelberg Eye Explorer. A linear mixed model was used to analyze the dynamics of pigment and its associations with other features of the phenotype. The relative incidence of pigment and of outer retinal outer retinal vascularization and SRNV was analyzed within the full MacTel Study cohort (1,244 probands). Results: Mean pigment area at baseline was 0.157 mm2 (range = 0–1.295 mm2, SD = 0.228 mm2, n = 101). Progression demonstrated a nonlinear pattern (P, 0.001) at an overall rate of 0.0177 mm2/year and was associated with the initial plaque size and with SRNV. There was a strong correlation between fellow eyes (P # 0.0001). In approximately 25% of all SRNV cases, SRNV may coincide with or precede pigment. Conclusion: Our data may be useful for refining the current system for staging disease severity in MacTel.

Original languageEnglish
Pages (from-to)S43-S50
StatePublished - 1 Jan 2018


FundersFunder number
Australian Government NHMRC
Lowy Medical Research Institute
State Government of Victoria


    • Degeneration
    • Imaging
    • MacTel
    • Müller cells
    • OCT
    • Pigment
    • Retina
    • Telangiectasia


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