June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Progression of Ellipsoid Zone Loss correlates with Change of Scotoma Size in Macular Telangiectasia Type 2.
Author Affiliations & Notes
  • Tjebo FC Heeren
    NIHR Biomedical Research Centre, Moorfields Eye Hospital, London, England, United Kingdom
    Ophthalmology, University of Bonn, Bonn, Germany
  • Diána Kitka
    NIHR Biomedical Research Centre, Moorfields Eye Hospital, London, England, United Kingdom
  • Daniela Florea
    NIHR Biomedical Research Centre, Moorfields Eye Hospital, London, England, United Kingdom
  • Alan C Bird
    NIHR Biomedical Research Centre, Moorfields Eye Hospital, London, England, United Kingdom
  • Emily Chew
    Epidemiology & Clinical Applications, National Eye Institute, Bethesda, Maryland, United States
  • Traci E Clemons
    Statistics, Emmes Corporation, Rockville, Maryland, United States
  • Daniel Pauleikhoff
    Ophthalmology, St Franziskus Hospital, Münster, Germany
  • Frank G Holz
    Ophthalmology, University of Bonn, Bonn, Germany
  • Tunde Peto
    NIHR Biomedical Research Centre, Moorfields Eye Hospital, London, England, United Kingdom
    Centre for Public Health, Queen's University Belfast, Belfast, United Kingdom
  • Footnotes
    Commercial Relationships   Tjebo Heeren, None; Diána Kitka, None; Daniela Florea, None; Alan Bird, None; Emily Chew, None; Traci Clemons, None; Daniel Pauleikhoff, None; Frank Holz, None; Tunde Peto, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 1280. doi:
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      Tjebo FC Heeren, Diána Kitka, Daniela Florea, Alan C Bird, Emily Chew, Traci E Clemons, Daniel Pauleikhoff, Frank G Holz, Tunde Peto; Progression of Ellipsoid Zone Loss correlates with Change of Scotoma Size in Macular Telangiectasia Type 2.. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1280.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Macular telangiectasia type 2 (MacTel) is a potential bilateral neurodegenerative disease with ellipsoid zone (EZ) loss detected on spectral domain optical coherence tomography (SD-OCT) as a common feature. While best corrected visual acuity (BCVA) often remains stable until late disease stages, visual function loss is typically characterized by the development of a paracentral (nasal) scotoma that enlarges over time as measured by microperimetry. It has been shown that EZ loss correlates with aggregate sensitivity loss, a measure for scotoma depth. Seeking further evidence that EZ loss correlates with functional loss is important, as EZ loss is a potential outcome parameter for interventional clinical trials. Therefore we compared EZ loss with change in scotoma size over time.

Methods : Participants of the MacTel Natural History Observation Study (NHOS) underwent annual exams including BCVA, microperimetry (MP1, Nidek), and high density SD-OCT volume scans (Spectralis, Heidelberg Engineering). Scotoma size was derived from counting the number of stimuli above a threshold of 12dB as well as the number of stimuli with absolute scotoma (‘thresholding’). EZ loss was measured using en face SD-OCT scans (Heidelberg Eye Explorer, Heidelberg Engineering) and compared with change of scotoma size and loss of BCVA. Analysis was performed with mixed effects models, accounting for correlation between eyes.

Results : Thirtyone participants (56 eyes) from one center were followed for 4.5±1.2 years (range 2.3-7.6 years). EZ loss was 18600±3917.3 pixel at baseline (≈0.59mm2) and increased 2627.8±427.9 pixel (≈0.08mm2) per year. BCVA decreased 2.2 ± 0.9 letters per year. EZ loss correlated significantly with progression of relative and absolute scotomas (r = 0.62; p-value < 0.0001 and : r = 0.72; p-value < 0.0001, respectively). Microperimetry showed a less discernible progression than EZ loss and a higher rate of ‘regression’, indicating higher variability of the measurement. No correlation was found between EZ loss and loss of BCVA.

Conclusions : Progression of EZ loss correlates well with progression of scotoma size corroborating EZ loss as clinical surrogate outcome parameter for visual function. Moreover, EZ loss, as an objective parameter compared with functional parameters such as microperimetry or BCVA, may be more suitable as primary end point in future interventional clinical trials.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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