Progression characteristics of ellipsoid zone loss in macular telangiectasia type 2

Daniel Pauleikhoff; Roberto Bonelli; Adam M Dubis; Frederic Gunnemann; Kai Rothaus; Peter Charbel Issa; Tjebo Fc Heeren; Tunde Peto; Traci E Clemons; Emily Y Chew; Alan C Bird; Ferenc B Sallo; MacTel Study Group

doi:10.1111/aos.14110

Progression characteristics of ellipsoid zone loss in macular telangiectasia type 2

Acta Ophthalmol. 2019 Nov;97(7):e998-e1005. doi: 10.1111/aos.14110. Epub 2019 Apr 9.

Authors

Daniel Pauleikhoff^{1

2}, Roberto Bonelli^{3

4}, Adam M Dubis^{5

6}, Frederic Gunnemann¹, Kai Rothaus¹, Peter Charbel Issa⁷, Tjebo Fc Heeren^{5

6

8}, Tunde Peto^{9

10}, Traci E Clemons¹¹, Emily Y Chew¹², Alan C Bird¹³, Ferenc B Sallo^{5

6

14}; MacTel Study Group

Collaborators

MacTel Study Group:
Sophie Bakri, Paul S Bernstein, Barbara Blodi, Alexander Brucker, Felicitas Bucher, Mina Chung, Grant Comer, Ian Constable, Michael Cooney, Diana Do, Jacque Duncan, Catherine Egan, Michael J Elman, Amani Fawzi, Martin Friedlander, Alain Gaudric, Mark C Gillies, Roger Goldberg, Joseph M Googe Jr, Robyn Guymer, Patrick Higgins, Frank Holz, Odette Houghton, Carel B Hoyng, Jean-Pierre Hubschman, Chirag Jhaveri, Arshad Khanani, David Lally, Cecilia Lee, Michael Lee, Joan W Miller, Daniel Miller, Joseph Moisseiev, Robert Murphy, Raja Narayanan, Sandeep Randhawa, Paul V Raphaelian, Ryan Rich, Richard Rosen, Philip Rosenfeld, Joke Ruys, Jose-Alain Sahel, Steven Schwartz, Lawrence Singerman, Scott Sneed, Gisele Soubrane, Johannes R Vingerling, David Warrow, David Weinberg, Sebastian Wolf, Charles Wykoff, Jiong Yan, Lawrence A Yannuzzi, Stanislav A Zhuk

Affiliations

¹ Department of Ophthalmology, St. Franziskus Hospital, Münster, Germany.
² Department of Ophthalmology, University of Duisburg-Essen, Duisburg, Germany.
³ Population Health and Immunity, Walter and Eliza Hall Institute of Medical Research, Parkville, Victoria, Australia.
⁴ Department of Medical Biology, University of Melbourne, Melbourne, Victoria, Australia.
⁵ UCL Institute of Ophthalmology, London, UK.
⁶ Department of Research and Development, Moorfields Eye Hospital, London, UK.
⁷ Nuffield Laboratory of Ophthalmology, Department of Clinical Neurosciences, Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, University of Oxford, Oxford, UK.
⁸ Department of Ophthalmology, University Hospital Bonn, Bonn, Germany.
⁹ Faculty of Medicine, Health and Life Sciences, Queen's University Belfast, Belfast, UK.
¹⁰ NIHR Biomedical Research Center for Ophthalmology, UCL Institute of Ophthalmology, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
¹¹ The Emmes Corporation, Rockville, Maryland, USA.
¹² National Eye Institute, National Institutes of Health, Bethesda, Maryland, USA.
¹³ Inherited Eye Disease, Moorfields Eye Hospital, London, UK.
¹⁴ Department of Ophthalmology, Hôpital Ophtalmique Jules-Gonin, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland.

Abstract

Purpose: To investigate the progression characteristics of ellipsoid zone (EZ) loss in eyes with macular telangiectasia type 2 (MacTel) as reflected by area and linear measurements, and their relevance for visual acuity.

Methods: Participants were selected from the MacTel Study cohort. Linear and area measurements of EZ loss were performed in Spectral-Domain Optical Coherence Tomograph (SD-OCT) volume scans. Progression characteristics and correlations between linear and area measurements were analysed using linear mixed effects models.

Results: A total of 134 eyes of 70 patients were included (85 eyes with follow-up, mean 4.7 years, range: 1.4-8 years). Ellipsoid zone (EZ) loss significantly progressed at a mean annual increment of 0.057 mm² (p = 0.005). The progression rate was non-linear and interacted significantly with initial EZ lesion size indicating an exponential growth before reaching a plateau. There was a strong heterogeneity in area sizes between fellow eyes. EZ break length had a significant linear effect on EZ break area (b = 1.06, p < 0.001) and could predict it. The location of the EZ break had a significant impact on visual acuity.

Conclusion: Ellipsoid zone (EZ) loss in MacTel has a non-linear progression characteristic, and its rate depends on area size at baseline, which must be taken into account at sample selection in clinical trials. Our results show a good correlation of linear and area measures of EZ loss and a segregation of best-corrected visual acuity by EZ location, which may help routine clinical practice.

Keywords: OCT; MacTel; ellipsoid zone; en face image; macular telangiectasia type 2.

MeSH terms

Adult
Aged
Aged, 80 and over
Disease Progression
Female
Fluorescein Angiography / methods*
Follow-Up Studies
Fundus Oculi
Humans
Macula Lutea / pathology*
Male
Middle Aged
Prognosis
Retinal Pigment Epithelium / pathology
Retinal Telangiectasis / diagnosis*
Retinal Vessels / pathology*
Time Factors
Tomography, Optical Coherence / methods*
Visual Acuity*

Abstract

MeSH terms

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