Abstract
Purpose: To describe the baseline characteristics of a large international cohort of patients with macular telangiectasia type 2 (MacTel Type 2) in anticipation of a longitudinal natural history study to evaluate structural and functional changes, identify potential risk factors and related outcomes.
Methods: Images including fundus photographs, fluorescein angiograms, optical coherence tomography and fundus autofluorescence were collected. A grading system for MacTel type 2 was developed by the central reading center to evaluate lesion characteristics. Relationships between lesion characteristics and visual acuity were evaluated.
Results: A total of 310 participants have been enrolled in the study. The mean time since diagnosis was 3 years (range 0 to 25 years). The mean age at the baseline examination was 61 ± 9 years. The mean visual acuity in the better eye was approximately 20/32 Snellen equivalents and approximately 20/50 in the worse eye. The visual acuity in the better eye of half of the participants was 20/32 or better. We found some relationships between visual acuity and lesions characteristic of MacTel Type 2.
Conclusions: This is the first large-scale study of patients with MacTel Type 2. More than half of the patients had 20/32 or better vision in their better eye, which is a sign that decreased function in these participants may not be reflected in central visual acuity. These findings highlight the limitation of using visual acuity measurements as a measure of function and as an outcome measure in potential clinical trials in patients with MacTel Type 2.
ACKNOWLEDGMENTS
This study was supported by the Lowy Medical Research Institute, LTD. The investigations were performed according to the guidelines of the Declaration of Helsinki and Institutional Review Board approval was obtained.
Declaration of Interest:
Participating Principal Investigators and Centers:
Jose-Alain Sahel, MD, PhD, Centre Hopitalier National D’Optalmologie des Quinze-Vingts, Paris, France; Robyn Guymer, MD, Centre for Eye Research, East Melbourne, Australia; Gisele Soubrane, MD, PhD, FEBO, Clinique Ophtalmolgie de Creteil, Creteil, France; Alain Gaudric, MD, Hopital Lariboisiere, Paris, France; Steven Schwartz, MD, Jules Stein Eye Institute, UCLA, Los Angeles, CA (USA); Ian Constable, MD, Lions Eye Institute, Nedlands, Australia; Michael Cooney, MD, MBA, Manhattan Eye, Ear, & Throat Hospital, New York, NY (USA); Cathy Egan, MD, Moorfields Eye Hospital, London, England (UK); Lawrence Singerman, MD, Retina Associates of Cleveland, Cleveland, OH (USA); Mark Gillies, MD, PhD, Save Sight Institute, Sydney, Australia; Martin Friedlander, MD, PhD, Scripps Research Institute, La Jolla, CA (USA); Daniel Pauleikhoff, Prof. Dr., St. Franziskus Hospital, Muenster, Germany; Joseph Moisseiev, MD, The Goldschleger Eye Institute, Tel Hashomer, Israel; Richard Rosen, MD, The New York Eye and Ear Infirmary, New York, NY (USA); Robert Murphy, MD, The Retina Group of Washington, Fairfax, VA (USA); Frank Holz, MD, University of Bonn, Bonn Germany; Grant Comer, MD, University of Michigan, Kellogg Eye Center, Ann Arbor, MI (USA); Barbara Blodi, MD, University of Wisconsin, Madison, WI (USA); Diana Do, MD, The Wilmer Eye Institute, Baltimore, MD (USA); Alexander Brucker, MD, Scheie Eye Institute, Philadelphia, PA (USA); Raja Narayanan, MD, LV Prasad Eye Institute, Hyderabad, India; Sebastian Wolf, MD, PhD, University of Bern, Bern, Switzerland; Philip Rosenfeld, MD, PhD, Bascom Palmer, Miami, FL (USA).