September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
Functional and structural measures of disease severity from a Phase 1 clinical trial in type 2 idiopathic macular telangiectasia.
Author Affiliations & Notes
  • Ferenc B Sallo
    Research & Development, Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
    Cell Biology, UCL Institute of Ophthalmology, London, United Kingdom
  • Irene Leung
    Research & Development, Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
  • Simona Esposti
    Research & Development, Moorfields Eye Hospital NHS Foundation Trust, London, United Kingdom
  • Traci E Clemons
    EMMES Corporation, Rockville, Maryland, United States
  • Daniel Pauleikhoff
    Ophthalmology, St Franziskus Hospital, Munster, Germany
  • Emily Y Chew
    National Eye Institute, Bethesda, Maryland, United States
  • Alan C Bird
    Inherited Eye Disease, Moorfields Eye Hospital, London, United Kingdom
  • Tunde Peto
    NIHR Biomedical Research Centre for Ophthalmology, at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, United Kingdom
  • Footnotes
    Commercial Relationships   Ferenc Sallo, None; Irene Leung, None; Simona Esposti, None; Traci Clemons, None; Daniel Pauleikhoff, None; Emily Chew, None; Alan Bird, None; Tunde Peto, None
  • Footnotes
    Support  Lowy Medical Research Institute
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 4955. doi:
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      Ferenc B Sallo, Irene Leung, Simona Esposti, Traci E Clemons, Daniel Pauleikhoff, Emily Y Chew, Alan C Bird, Tunde Peto; Functional and structural measures of disease severity from a Phase 1 clinical trial in type 2 idiopathic macular telangiectasia.. Invest. Ophthalmol. Vis. Sci. 2016;57(12):4955.

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

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Abstract

Purpose : Type 2 macular telangiectasia (MacTel) is associated with outer retinal abnormalities and focal sensitivity defects near and at the foveal centre. Optical Coherence Tomographic (OCT) topographic mapping of the IS/OS junction layer is a functionally relevant method for assessing disease severity in MacTel. Our aim was to assess the characteristics and utility of microperimetry and 'en face' OCT imaging data in following disease progression.

Methods : Patients were selected from an ongoing Phase I open label clinical trial of CNTF in type 2 MacTel. OCT volume scans of an area at least 15°x10° in size with B-scan intervals ≤47µ were acquired using Heidelberg Spectralis (two patients) or CZM Cirrus OCT4000 units (5 patients). Segmentation and topographic mapping of the IS/OS layer was performed manually using a dedicated 3D imaging software (Visage Imaging Amira v5.0). Functional testing was performed using CenterVue Maia devices, using a customized grid. OCT topographic and microperitmetric data maps were superimposed and analyzed in Adobe Photoshop. Aggregate sensitivity loss was calculated as described earlier.

Results : Fourteen eyes of 7 MacTel patients (ranging in age 48-67 years) were examined at baseline and at 4 subsequent annual follow-up visits. An IS/OS break was apparent in 12 eyes at baseline and in all eyes at 48 months. In 4 eyes of 3 patients however, the break area was consistently too small to be detected by functional testing using the custom grid, in these eyes aggregate loss could not be calculated. Overall, the break area size showed a good correlation with the number of test points <20dB (Spearman's ρ=0.813, p<0.0001, 95%CI 0.710 to 0.881, n=66) as well as with aggregate sensitivity loss (ρ =0.834, P<0.0001, 95%CI 0.716 to 0.906, n=45).

Conclusions : Overall, we found a good correlation between IS/OS break area size and function loss. OCT mapping of the IS/OS demonstrates structural change before microperimetry can detect a focal loss of retinal sensitivity typical of MacTel. In future studies, the minimum lesion size needs to be determined taking into consideration not only the OCT resolution but also the spacing of the microperimeter test grid and the stimulus size. Calibrating for axial length and refractive power may further improve the precision of lesion area measurements in OCT en face images.

This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.

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