July 2019
Volume 60, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2019
Progressive Neurodegeneration in Macular Telangiectasia Type 2 (MacTel)
Author Affiliations & Notes
  • Alec Lee Amram
    University of Iowa, Iowa City, Iowa, United States
  • S. Scott whitmore
    University of Iowa, Iowa City, Iowa, United States
  • Christine Clavell
    University of Iowa, Iowa City, Iowa, United States
  • Lance Lyons
    University of Texas Medical Branch, Galveston, Texas, United States
  • Alexander Michael Rusakevich
    Retina Consultants of Houston, Houston, Texas, United States
  • Ian Han
    University of Iowa, Iowa City, Iowa, United States
  • James C Folk
    University of Iowa, Iowa City, Iowa, United States
  • H Culver Boldt
    University of Iowa, Iowa City, Iowa, United States
  • Karen Gehrs
    University of Iowa, Iowa City, Iowa, United States
  • Edwin M Stone
    University of Iowa, Iowa City, Iowa, United States
  • Stephen R Russell
    University of Iowa, Iowa City, Iowa, United States
  • Kyungmoo Lee
    University of Iowa, Iowa City, Iowa, United States
  • Michael David Abramoff
    University of Iowa, Iowa City, Iowa, United States
  • Charles Clifton Wykoff
    Retina Consultants of Houston, Houston, Texas, United States
  • Elliott H Sohn
    University of Iowa, Iowa City, Iowa, United States
  • Footnotes
    Commercial Relationships   Alec Amram, None; S. whitmore, None; Christine Clavell, None; Lance Lyons, None; Alexander Rusakevich, None; Ian Han, None; James Folk, None; H Boldt, None; Karen Gehrs, None; Edwin Stone, None; Stephen Russell, None; Kyungmoo Lee, None; Michael Abramoff, Alimera Life Sciences (C), IDx (I), IDx (E), IDx (C), University of Iowa (P); Charles Wykoff, None; Elliott Sohn, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science July 2019, Vol.60, 2570. doi:
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      Alec Lee Amram, S. Scott whitmore, Christine Clavell, Lance Lyons, Alexander Michael Rusakevich, Ian Han, James C Folk, H Culver Boldt, Karen Gehrs, Edwin M Stone, Stephen R Russell, Kyungmoo Lee, Michael David Abramoff, Charles Clifton Wykoff, Elliott H Sohn; Progressive Neurodegeneration in Macular Telangiectasia Type 2 (MacTel). Invest. Ophthalmol. Vis. Sci. 2019;60(9):2570.

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

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Abstract

Purpose : MacTel has a complex pathogenesis that involves the retinal vasculature with a significant neurodegenerative component. In addition to outer retinal abnormalities (e.g. ellipsoid zone) that correlate with visual dysfunction, patients with MacTel have been shown to have retinal ganglion cell layer (GCL) and nerve fiber layer (NFL) thinning, but the rate of thinning of retinal layers is unclear. We sought to quantify the change in thickness over time of retinal layers in patients with non-proliferative MacTel.

Methods : This was an IRB-approved retrospective study of patients with MacTel presenting to the University of Iowa and Retina Consultants of Houston with at least two Heidelberg Spectralis SD-OCT scans separated by over 10 months. Exclusion criteria included: glaucoma, history of optic nerve disease, proliferative diabetic retinopathy, diabetic macular edema, choroidal neovascularization from any cause, vascular occlusion, prior plaque radiotherapy, anti-VEGF therapy, or advanced age-related macular degeneration. OCTs were segmented into 11 retinal layers using the Iowa Reference Algorithms (ver. 5.0.0). For each layer, mean thickness was computed for the 8 noncentral regions of the ETDRS grid. A mixed effects model was fit to each layer and region using lme4 for R (thickness ~ time + laterality + time:laterality + (1|subject)). Percent change was calculated as the rate of change / baseline thickness.

Results : 117 patients met criteria for this study; of these, 37 have been analyzed to date, with 81% female, median age 66 years old. Average NFL, GCL, and INL thickness decreased by 0.47%, 0.93%, and 2.1% per year in the parafoveal temporal region, respectively. Greatest percent loss was observed in the outer zone of the outer segments, with 7 of 8 regions having percent loss greater than 1.8% per year, and mean loss of 2.16% per year.

Conclusions : Patients with non-proliferative MacTel sustain progressive neurodegeneration that may exceed those with diabetes and no clinical retinopathy. Ongoing analyses will more definitively delineate the relationship between MacTel and neuroretinal thinning.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.

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