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Clinical science
Reduced vessel density in deep capillary plexus correlates with retinal layer thickness in choroideremia
  1. Alessandro Arrigo1,
  2. Francesco Romano1,2,
  3. Maurizio Battaglia Parodi1,
  4. Peter Charbel Issa3,4,
  5. Johannes Birtel3,4,5,
  6. Francesco Bandello1,
  7. Robert E Maclaren6
  1. 1 Department of Ophthalmology, Scientific Institute San Raffaele, University Vita-Salute, via Olgettina, 60, 20132, Milan, Italy
  2. 2 Eye Clinic, Department of Biomedical and Clinical Science, Luigi Sacco University Hospital, Milano, Italy
  3. 3 Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
  4. 4 Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
  5. 5 Department of Ophthalmology, University of Bonn, Bonn, Germany
  6. 6 Oxford Eye Hospital, Oxford University Hospitals NHS Foundation Trust, and Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
  1. Correspondence to Alessandro Arrigo, Department of Ophthalmology, IRCCS Ospedale San Raffaele, University Vita-Salute, Via Olgettina 60, Milan 20132, Italy; alessandro.arrigo{at}hotmail.com

Abstract

Background To assess retinal layer thickness in choroideremia (CHM) and to reveal its correlation with optical coherence tomography (OCT) angiography (OCTA) findings.

Methods The study was designed as an observational, cross-sectional clinical series of patients with CHM, which included 14 CHM eyes and 14 age-matched controls. Multimodal imaging included OCT and OCTA. The vessel density (VD) of superficial capillary (SCP), deep capillary (DCP) and choriocapillaris (CC) plexuses was analysed by OCTA. The apparently preserved retinal islet and atrophic regions were investigated separately. Main outcome measures were as follows: best-corrected visual acuity (BCVA), total retinal layers, ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer (INL), outer plexiform layer (OPL), outer nuclear layer (ONL), ellipsoid zone–retinal pigment epithelium (EZ-RPE) layer, choroidal thickness and VDs of SCP, DCP and of CC.

Results Mean BCVA was 0.0±0.0 LogMAR in both groups. GCL, ONL, EZ-RPE and choroid were significantly thinned in CHM, particularly in the atrophic region. OPL was unaffected in the apparently preserved islet, whereas INL and IPL were similarly thinned in the atrophic and apparently preserved retina. DCP appeared severely affected in both regions, while CC was only altered in the atrophic retina. Significant correlations were found between OCT and OCTA parameters.

Conclusions Our study showed severe alterations in both outer and inner retinal layers of patients with CHM. The extended retinal involvement might be the consequence of neuronal and vascular trophic factor reduction produced by the primarily altered RPE and/or secondary Müller glial cell reaction.

  • Imaging
  • Macula
  • Retina
  • Dystrophy
  • Genetics
  • choroideremia
http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/bjophthalmol-2020-316528

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    Footnotes

    • Contributors AA and FR were involved in study design, data analysis, data interpretation and manuscript draft. PCI and JB were involved in data acquisition, data analysis and manuscript revision. MBP, FB and REM were involved in data interpretation, manuscript revision, study supervision.

    • Funding This study was supported by the National Institute of Health Research (NIHR) Oxford Biomedical Research Centre (BRC). The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health. This work was supported by the Dr. Werner Jackstädt Foundation, Wuppertal, Germany (Grant S0134-10.22 to JB). Francesco Bandello is a consultant for Alcon (Fort Worth, Texas, USA), Alimera Sciences (Alpharetta, Georgia, USA), Allergan (Irvine, California, USA), Farmila-Thea (Clermont-Ferrand, France), Bayer Shering-Pharma (Berlin, Germany), Bausch + Lomb (Rochester, New York, USA), Genentech (San Francisco, California, USA), Hoffmann-La-Roche (Basel, Switzerland), Novagali Pharma (Évry, France), Novartis (Basel, Switzerland), Sanofi-Aventis (Paris, France), Thrombogenics (Heverlee, Belgium), Zeiss (Dublin, USA). Peter Charbel Issa is a consultant for Gyroscope and receives research support from Heidelberg Engineering. Robert MacLaren is a consultant to Biogen (Boston, USA).

    • Competing interests None declared.

    • Data sharing statement No data are available.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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