Advertisement

Progression of vascular changes in macular telangiectasia type 2: comparison between SD-OCT and OCT angiography

  • Daniel PauleikhoffEmail author
  • Frederic Gunnemann
  • Marius Book
  • Kai Rothaus
Retinal Disorders

Abstract

Purpose

To investigate the different appearances of vascular changes in macular telangiectasia type 2 (MacTel) and to describe their possible progression, the vascular patterns in different retinal layers were analyzed with optical coherence tomography angiography (OCT-A) and the findings were correlated with a spectral-domain OCT (SD-OCT) disease severity scale based on the extent of ellipsoid zone (EZ) loss.

Methods

Participants from the MacTel Study Group in Muenster and a healthy cohort were investigated with OCT-A using RTVue XR Avanti. After segmentation of the superficial capillary network, the deep capillary network, and the outer retina (OR), flow density was analyzed using Optovue software. Then, the images were exported using the software Fiji (National Institute of Mental Health, Bethesda, MD, USA) and analyzed with the automated MATLAB program (Mathworks, Version R2014b). Four parameters (total vessel length, number of vessel branches, number of vessel segments, and fractal dimension) were examined on the vascular skeletons (temporal, foveal, nasal, and total fields of the ETDRS grid). In addition, linear and area measurements of EZ loss were performed on SD-OCT volume scans. Progression characteristics and correlation between linear and area measurements were analyzed using linear mixed effects models.

Results

Twenty eyes of healthy probands (20 OCT-A and 20 SD-OCT scans) and 122 eyes of 61 MacTel patients were included. In order to classify the severity of the disease, MacTel eyes were assigned to a SD-OCT “disease severity scale” (grade 1 = no EZ loss; grade 2 = EZ loss temporal to the fovea; grade 3 = EZ loss including the fovea and the region nasal to the fovea). Flow density and total vessel length showed only limited differences between healthy eyes and different grades of MacTel, but particularly the numbers of branches and vessel segments, as well as the fractal dimension values, demonstrated significant and progressive reduction in the superficial and deep capillary networks of the temporal, nasal, and total ETDRS fields. Moreover, the outer retina showed a progressive presence of hyperreflective material in SD-OCT grades 2 and 3 eyes with associated vascular patterns in the OR on OCT-A.

Conclusions

In SD-OCT, the severity of MacTel is characterized by progressive EZ loss, which may be used as a simple clinical “disease severity scale”. In addition, OCT-A enables visualization and quantification of vascular patterns with mathematical methods. The morphological progression of the disease correlated significantly with progressive vascular changes, especially in respect of the numbers of branches and vessel segments as well as fractal dimension. This suggests that the severity of neurodegenerative and neurovascular changes develops in parallel and that the analysis and quantification of the vascular changes in the superficial and deep capillary networks may become an additional parameter for future treatment trials. Moreover, the significant association between hyperreflective material progressively visible on SD-OCT in the OR, which most often contains vessels in OCT-A, and advancing SD-OCT severity grades, as well as vascular changes in OCT-A, supports the concept of retinal neovascularization in the OR in patients with advanced MacTel.

Keywords

Macular telangiectasia type 2 MacTel OCT Disease severity scale OCT angiography Vascular changes 

Notes

Acknowledgments

Many thanks to Mrs. Susanne Wirtz-Kirchberg for her invaluable analysis of the SD-OCT scans and en-face images.

Compliance with ethical standards

Conflict of interest

Author D.P. is a member of the international MacTel Consortium and has received travel support for the meeting of this Study Group from the LMRI; Author F.G. declares that he has no conflict of interest. Author M.B. declares that he has no conflict of interest. Author K.R. declares that he has no conflict of interest.

Ethical approval and informed consent

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. In addition, a positive vote from the ethical committee of the University of Münster and the Westphalian Doctors Association was achieved and informed consent was obtained from all individual participants included in the study.

References

  1. 1.
    Powner MB, Gillies MC, Tretiach M et al (2010) Perifoveal Müller cell depletion in a case of macular telangiectasia type 2. Ophthalmology 117:2407–2416CrossRefGoogle Scholar
  2. 2.
    Powner MB, Gillies MC, Zhu M et al (2013) Loss of Müller’s cells and photoreceptors in macular telangiectasia type 2. Ophthalmology 120:2344–2352CrossRefGoogle Scholar
  3. 3.
    Issa PC, Gillies MC, Chew EY et al (2013) Macular telangiectasia type 2. Prog Retin Eye Res 34:49–77CrossRefGoogle Scholar
  4. 4.
    Gass JDM (1968) A fluorescein angiographic study of macular dysfunction secondary to retinal vascular disease: V. retinal telangiectasis. Arch Ophthalmol 80:592–605CrossRefGoogle Scholar
  5. 5.
    Gass JD, Oyakawa RT (1982) Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 100:769–780CrossRefGoogle Scholar
  6. 6.
    Gass JDM, Blodi BA (1993) Idiopathic juxtafoveolar retinal telangiectasis: update of classification and follow-up study. Ophthalmology 100:1536–1546CrossRefGoogle Scholar
  7. 7.
    Esposti SD, Egan C, Bunce C et al (2012) Macular pigment parameters in patients with macular telangiectasia (MacTel) and normal subjects: implications of a novel analysis. Invest Ophthalmol Vis Sci 53:6568–6575CrossRefGoogle Scholar
  8. 8.
    Zeimer MB, Padge B, Heimes B, Pauleikhoff D (2010) Idiopathic macular telangiectasia type 2: distribution of macular pigment and functional investigations. Retina 30:586–595CrossRefGoogle Scholar
  9. 9.
    Issa PC, Berendschot TTJM, Staurenghi G et al (2008) Confocal blue reflectance imaging in type 2 idiopathic macular telangiectasia. Invest Ophthalmol Vis Sci 49:1172–1177CrossRefGoogle Scholar
  10. 10.
    Sallo FB, Leung I, Zeimer M et al (2018) Abnormal retinal reflectivity to short-wavelength light in type 2 idiopathic macular teleangiectasis. Retina 38:S79–S88CrossRefGoogle Scholar
  11. 11.
    Gaudric A, de Lahitte GD, Cohen SY et al (2006) Optical coherence tomography in group 2A idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol 124:1410–1419CrossRefGoogle Scholar
  12. 12.
    Sallo FB, Leung I, Clemons TE et al (2015) Multimodal imaging in type 2 idiopathic macular teleangiectasis. Retina 35:742–749CrossRefGoogle Scholar
  13. 13.
    Sallo FB, Peto T, Egan C et al (2012) “En face” OCT imaging of the IS/OS junction line in type 2 idiopathic macular telangiectasia. Invest Ophthalmol Vis Sci 53:6145–6152CrossRefGoogle Scholar
  14. 14.
    Sallo FB, Peto T, Egan C et al (2012) The IS/OS junction layer in the natural history of type 2 idiopathic macular telangiectasia. Invest Ophthalmol Vis Sci 53:7889–7895CrossRefGoogle Scholar
  15. 15.
    Mukherjee D, Lad EM, Vann RR et al (2017) Correlation between macular integrity assessment and optical coherence tomography imaging of ellipsoid zone in macular telangiectasia type 2. Invest Ophthalmol Vis Sci 58:291–299CrossRefGoogle Scholar
  16. 16.
    Heeren TFC, Kitka D, Florea D et al (2018) Longitudinal correlation of ellipsoid zone loss and functional loss in macular telangiectasia type 2. Retina 38:S20–S26Google Scholar
  17. 17.
    Okada M, Robson AG, Egan CA et al (2018) Electrophysiological characterization of macular telangiectasia type 2 and structure-function correlation. Retina 38:S33–S42Google Scholar
  18. 18.
    Peto T, Heeren TFC, Clemons TE et al (2018) Correlation of clinical and functional progression with visual acuity loss in macular telangiectasia type 2: macTel project report no. 6–the macTel research group. Retina 38:S8–S13Google Scholar
  19. 19.
    Chew EY, Clemons TE, Jaffe GJ, et al (2019) Effect of ciliary neurotrophic factor on retinalneurodegeneration in patients with macular telangiectasia type 2: a randomized clinical trial. Ophthalmology 126(4):540–549Google Scholar
  20. 20.
    Jia Y, Tan O, Tokayer J et al (2012) Split-spectrum amplitude-decorrelation angiography with optical coherence tomography. Opt Express 20:4710–4725CrossRefGoogle Scholar
  21. 21.
    Zeimer M, Gutfleisch M, Heimes B et al (2015) Association between changes in macular vasculature in optical coherence tomography- and fluorescein-angiography and distribution of macular pigment in type 2 idiopathic macular teleangiectasia. Retina 35:2307–2316CrossRefGoogle Scholar
  22. 22.
    Gaudric A, Krivosic V, Tadayoni R (2015) Outer retina capillary invasion and ellipsoid zone loss in macular teleangiectasia type 2 imaged by OCT angiography. Retina 35:2300–2306CrossRefGoogle Scholar
  23. 23.
    Spaide RF, Marco RD, Yannuzzi LA (2018) Vascular distortion and dragging related to apparent tissue contraction in macular teleangictasis type 2. Retina 38:S51–S60Google Scholar
  24. 24.
    Spaide RF, Yannuzzi LA, Maloca PM (2018) Retinal-choroidal anastomosis in macular teleangictasis type 2. Retina 38:1920–1929CrossRefGoogle Scholar
  25. 25.
    Faatz H, Rothaus K, Gunnemann F et al (2017) Changes in OCT angiography of type 2 CNV in neovascular AMD during anti-VEGF treatment. Klin Monatsbl Augenheilkd 234:1125–1131CrossRefGoogle Scholar
  26. 26.
    Pauleikhoff D, Bonelli R, Dubis A et al (2019) Progression characteristics of ellipsoid zone loss in macular teleangiectasia type 2. Ophthalmologica: Epub ahead of printGoogle Scholar
  27. 27.
    Faatz H, Farecki ML, Rothaus K et al (2019) Optical coherence tomography angiography of type 1 and 2 neovascularisations in age-related macular degeneration under anti-VEGF therapy: evaluation of a new quantitative method. Eye: Epub ahead of printGoogle Scholar
  28. 28.
    Rothaus K, Jiang X (2005) Multi-scale midline extraction using creaseness. Pattern recognition and image analysis. Springer, Berlin, pp 502–511CrossRefGoogle Scholar
  29. 29.
    López AM, Lloret D, Serrat J, Villanueva JJ (2000) Multilocal creaseness based on the level-set extrinsic curvature. Comput Vis Image Underst 77:111–144CrossRefGoogle Scholar
  30. 30.
    Leung I, Sallo FB, Bonelli R et al (2018) Characteristics of pigmented lesions in type 2 idiopathic macular teleangiectasia. Retina 38:43–50CrossRefGoogle Scholar
  31. 31.
    Milam AH, Li ZY, Fariss RN (1998) Histopathology of the human retina in retinitis pigmentosa. Prog Retin Eye Res 17:175–205CrossRefGoogle Scholar
  32. 32.
    Kupitz EH, Heeren TFC, Holz FG, Charbel Issa P (2015) Poor long-term outcome of anti-VEGF therapy in nonproliferative macular teleangiectasia type 2. Retina 35:2619–2626CrossRefGoogle Scholar
  33. 33.
    Spaide RF, Klancnik JM, Cooney MJ (2015) Retinal vascular layers in macular telangiectasia type 2 imaged by optical coherence tomographic angiography. JAMA Ophthalmol 133:66–73CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of OphthalmologySt. Franziskus HospitalMuensterGermany
  2. 2.Department of OphthalmologyUniversity of Duisburg-EssenEssenGermany

Personalised recommendations