Functional Relevance of Hyper-Reflectivity in Macular Telangiectasia Type 2

Invest Ophthalmol Vis Sci. 2021 Mar 1;62(3):6. doi: 10.1167/iovs.62.3.6.

Abstract

Purpose: The purpose of this study was to quantify hyper-reflective lesions on en face optical coherence tomography (OCT) and study its functional relevance in macular telangiectasia type 2 (MacTel).

Design: This was a retrospective, cross-sectional cohort study.

Methods: Baseline image and functional data from participants of a phase II clinical trial (NCT01949324) that studied the effect of Ciliary Neurotrophic Factor in patients with MacTel were analyzed. The projection of hyper-reflectivity within different layers on OCT was used to generate an en face view and measure the en face size of hyper-reflectivity. Ellipsoid zone (EZ)-loss was additionally evaluated, and en face images were superimposed onto microperimetry sensitivity maps, allowing to estimate mean retinal sensitivity within areas displaying hyper-reflectivity and EZ-loss, respectively. Best-corrected visual acuity (BCVA) and reading speed were also analyzed.

Results: Fifty-two eyes from 52 patients were analyzed. Hyper-reflectivity was present in 32 eyes (62%), and EZ-loss in 50 (96%) eyes. Mean lesion size was 0.11 mm² (range = 0.01-0.26) for hyper-reflectivity and 0.51 mm² (range = 0.02-1.34) for EZ-loss, and lesion sizes correlated strongly (Spearman r = 0.79, P < 0.001). Although both hyper-reflectivity and EZ-loss were associated with a significant decrease in retinal sensitivity, mean sensitivity thresholds differed significantly between lesions (0.9 dB vs. 16.3 dB; P < 0.001), indicating an almost complete loss of sensitivity in hyper-reflective areas. No correlations were found between the size of hyper-reflectivity and BCVA (r = 0.09) or reading speed (r = -0.17).

Conclusions: En face OCT can be used to quantify the area of hyper-reflective lesions in MacTel. Hyper-reflectivity in MacTel is associated with severe functional impairment, leading to an almost complete loss of retinal sensitivity as observed on microperimetry.

Publication types

  • Clinical Trial, Phase II
  • Multicenter Study
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Cross-Sectional Studies
  • Female
  • Fluorescein Angiography
  • Humans
  • Male
  • Middle Aged
  • Retina / diagnostic imaging
  • Retina / physiopathology*
  • Retinal Telangiectasis / diagnostic imaging
  • Retinal Telangiectasis / physiopathology*
  • Retrospective Studies
  • Scotoma / diagnostic imaging
  • Scotoma / physiopathology*
  • Telangiectasia, Hereditary Hemorrhagic / diagnostic imaging
  • Telangiectasia, Hereditary Hemorrhagic / physiopathology*
  • Tomography, Optical Coherence
  • Visual Acuity / physiology
  • Visual Field Tests
  • Visual Fields / physiology

Supplementary concepts

  • Osler-rendu-weber syndrome 2