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Original research
Characterisation of vascular changes in different stages of Stargardt disease using double swept-source optical coherence tomography angiography
  1. Michael Reich,
  2. Andreas Glatz,
  3. Bertan Cakir,
  4. Daniel Böhringer,
  5. Stefan Lang,
  6. Sebastian Küchlin,
  7. Lutz Joachimsen,
  8. Wolf Lagreze,
  9. Hansjuergen T Agostini,
  10. Clemens Lange
  1. Eye Center, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany
  1. Correspondence to Dr Clemens Lange; clemens.lange{at}uniklinik-freiburg.de

Abstract

Objective To describe vascular changes in different stages of Stargardt disease (STGD) via double swept-source optical coherence tomography angiography.

Methods and analysis Prospective, cross-sectional case–control study. Twenty-three patients (45 eyes) with ABCA4 mutations graded according to the Fishman STGD classification and 23 controls (23 eyes) were included. Two independent investigators quantified the foveal avascular zone (FAZ) in the superficial and deep capillary plexus (SCP/DCP) and the areas presenting rarefied flow and complete vascular atrophy in the outer retina to choriocapillaris (ORCC) and choriocapillaris (CC) slab.

Results The mean age at first diagnosis of STGD was 24.0 years (range 9–50) and 37.9 years (range 18–74) at the time of examination. Eleven patients were assigned to the Fishman STGD classification stage (S) 1, three to S2, eight to S3 and one to S4. The FAZ in SCP and DCP was increased in all stages compared with controls (p<0.01). Areas with rarefied flow signal and vascular atrophy were detected in the ORCC and the CC layer and grew with increasing stage of disease (p<0.01). The duration of disease correlated with the extent of the enlarged FAZ in the SCP/DCP and with the area of reduced flow in the ORCC and CC layer (p<0.01). Best corrected visual acuity correlated negatively with the extent of the enlarged FAZ in the SCP/DCP (p<0.0001), as well as with enlarged atrophic area in the ORCC and CC layer (p=0.026 and p=0.074).

Conclusions Patients with STGD reveal vascular changes in the retina and CC in all disease stages. The avascular zone in the SCP/DCP and areas with rarefied flow signal in the ORCC/CC increase with the duration and stage of disease, indicating progressive vascular decay most likely secondary to retinal pigment epithelium and neuronal loss. Furthermore, increased vascular damage is associated with decreased vision.

  • Retina
  • Dystrophy
  • Degeneration

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/bmjophth-2019-000318

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    Footnotes

    • Contributors MR has conducted the acquisition of data, data analysis and interpretation, has made substantial contribution to conception and design of the manuscript and has been involved in drafting the manuscript. AG has conducted the data analysis, has made substantial contributions to acquisition of data and has been involved in drafting the manuscript. BC, SL, SK and LJ have made substantial contributions to acquisition of data, and have been involved in drafting the manuscript. DB has conducted the data and statistical analysis and has been involved in drafting the manuscript. WL and HTA have made substantial contributions to acquisition of data, and have been involved in revising the manuscript critically for important intellectual content. CL has made substantial contributions to analysis and interpretation of data and has been involved in revising the manuscript critically for important intellectual content. In addition, all authors have given final approval of the version to be published. Each author has participated sufficiently in the work to take public responsibility for appropriate portions of the content and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors read and approved the final manuscript.

    • Funding The Plex Elite OCTA system (Zeiss PLEX Elite 9000, Germany) was loaned to our clinic by Zeiss. The article processing charge was funded by the German Research Foundation (DFG) and the University of Freiburg in the funding programme Open Access Publishing.

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Ethics approval The study was approved by our institutional ethics committee and adhered to the tenets of the Declaration of Helsinki. Informed written consent was obtained from all patients with STGD and all controls.

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

    • Data availability statement Data are available upon request.