June 2015
Volume 56, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2015
Three-dimensional characteristics of outer retinal atrophy in type 2 idiopathic macular telangiectasia.
Author Affiliations & Notes
  • Ferenc B Sallo
    Visual Neuroscience, UCL Institute of Ophthalmology, London, United Kingdom
    Research and Development, Moorfields Eye Hospital, London, United Kingdom
  • Irene Leung
    Research and Development, Moorfields Eye Hospital, London, United Kingdom
  • Vincent Rocco
    Research and Development, Moorfields Eye Hospital, London, United Kingdom
  • Meike Zeimer
    Department of Ophthalmology, St Franziskus Hospital, Muenster, Germany
  • Britta Heimes
    Department of Ophthalmology, St Franziskus Hospital, Muenster, Germany
  • Traci E Clemons
    The EMMES Corporation, Rockville, MD
  • Daniel Pauleikhoff
    Department of Ophthalmology, St Franziskus Hospital, Muenster, Germany
  • Emily Y Chew
    NEI, NIH, Bethesda, MD
  • 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; Vincent Rocco, None; Meike Zeimer, None; Britta Heimes, None; Traci Clemons, None; Daniel Pauleikhoff, None; Emily Chew, None; Alan Bird, None; Tunde Peto, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science June 2015, Vol.56, 2783. doi:
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    • Get Citation

      Ferenc B Sallo, Irene Leung, Vincent Rocco, Meike Zeimer, Britta Heimes, Traci E Clemons, Daniel Pauleikhoff, Emily Y Chew, Alan C Bird, Tunde Peto, ; Three-dimensional characteristics of outer retinal atrophy in type 2 idiopathic macular telangiectasia.. Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):2783.

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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 at and near the foveal centre. Optical Coherence Tomographic (OCT) topographic mapping of the IS/OS junction layer (or ellipsoid zone, EZ) is a functionally relevant method for assessing disease severity in MacTel. On OCT, the photoreceptor outer segment tips (POST line or interdigitation zone, IZ) are also associated with a clear signal. Our aim was to examine the characteristics of outer retinal atrophy in in MacTel, in 3 dimensions.

 
Methods
 

Patients were selected from the MacTel Study cohort. OCT volume scans of an area 15°x10° in size with 11µ B-scan intervals were acquired using Heidelberg Spectralis OCT units. Automated segmentation and topographic mapping of the POST (IZ) and IS/OS (EZ) layers was reviewed and corrected manually. OCT topographic maps were superimposed and compared in 3D using a dedicated 3D imaging software.

 
Results
 

Eighteen eyes of 10 MacTel patients (ranging in age 34-68 years, mean age 53.2, SD=13.7 years) were examined. In 13 eyes a break was apparent in both the IS/OS (EZ) and the POST (IZ). Consistently, the POST (IZ) break appeared larger than the break in the IS/OS (EZ). In 11 cases, the IS/OS (EZ) deviated through the POST (IZ) break towards the retinal pigment epithelium (RPE). In 5 eyes no IS/OS (EZ) break was apparent. In 3 of these however, a POST (IZ) break was detectable and the shape of the IS/OS (EZ) at the temporal part of the fovea externa was deformed, deviating towards the RPE.

 
Conclusions
 

A break in the POST (IZ) layer and deviations in the shape of the IS/OS (EZ) may precede a full break in the IS/OS (EZ). This may be interpreted as a shortening of the photoreceptor outer segments before degenerative changes appear in the junctions or the inner segments. A 3D topographic analysis of the outer retina may adjunct current methods and provide a more accurate tool for characterizing early disease as well as clues to the sequence of cellular degeneration in MacTel.

 
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