September 2016
Volume 57, Issue 12
Open Access
ARVO Annual Meeting Abstract  |   September 2016
OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: A NEW IMAGING APPROACH FOR THE ASSESSMENT OF MACULAR CHANGES IN X-LINKED JUVENILE RETINOSCHISIS
Author Affiliations & Notes
  • Francesco Stringa
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
  • ALESSANDRO PAPAYANNIS
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
  • EMMANOUIL TSAMIS
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
    Institute of Human Development, Faculty of Medical and Human Sciences, University of Manchester, Manchester, United Kingdom
  • Katarzyna M. Chwiejczak
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
  • Susmito Biswas
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
    Institute of Human Development, Faculty of Medical and Human Sciences, University of Manchester, Manchester, United Kingdom
  • Assad Jalil
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
  • Paulo E Stanga
    Manchester Vision Regeneration (MVR) Lab at Manchester Royal Eye Hospital & NIHR/Wellcome Trust Manchester CRF and Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
    Institute of Human Development, Faculty of Medical and Human Sciences, University of Manchester, Manchester, United Kingdom
  • Footnotes
    Commercial Relationships   Francesco Stringa, None; ALESSANDRO PAPAYANNIS, None; EMMANOUIL TSAMIS, None; Katarzyna Chwiejczak, None; Susmito Biswas, None; Assad Jalil, None; Paulo Stanga, Topcon Corp (F), Topcon Corp (C), Topcon Corp (R)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science September 2016, Vol.57, 5510. doi:
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      Francesco Stringa, ALESSANDRO PAPAYANNIS, EMMANOUIL TSAMIS, Katarzyna M. Chwiejczak, Susmito Biswas, Assad Jalil, Paulo E Stanga; OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: A NEW IMAGING APPROACH FOR THE ASSESSMENT OF MACULAR CHANGES IN X-LINKED JUVENILE RETINOSCHISIS. Invest. Ophthalmol. Vis. Sci. 2016;57(12):5510.

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

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Abstract

Purpose : X-linked juvenile retinoschisis (XLRS) is a retinal degenerative disease characterized by splitting of the retinal layers. Swept Source Optical Coherence Tomography Angiography (SS OCT-A) is a new non-invasive imaging technique to evaluate retinal and choroidal vasculature. We describe macular SS OCT-A features in paediatric patients affected by XLRS.

Methods : Serial case report. Nine eyes of five males (mean age 11.5 years) were imaged on Topcon DRI OCT-1 Atlantis® SS OCT-A. Retinal segmentation provided analysis of: superficial neurovascular layer, from inner limiting membrane (ILM) to boundary between inner plexiform layer and inner nuclear layer (IPL/INL); deep neurovascular layer, from IPL/INL to the junction of inner and outer photoreceptor’s segment (IS/OS); and choroidal layer, from retinal pigmented epithelium (RPE) to choroidal-scleral interface (CSI). All images were analysed by two independent ophthalmologists.

Results : All eyes showed macular petaloid-shaped hypo-reflective areas within the deep neuroretinal plexus. All eyes showed perifoveal microvascular telangectatic-like changes and capillary dropout in both superficial and deep plexuses (Figure 1). All eyes showed areas of hypo- and hyper-reflectivity on choroidal layer analysis that where not associated with any evident vascular alteration. However, these could be attributed to alterations of the SS OCT-A signal due to optical shadowing (i.e. hypo-reflectivity) or to RPE irregularities (i.e. hyper-reflectivity). There was 100% agreement between the reviewers.

Conclusions : To the best of our knowledge, this is the first description of SS OCT-A findings in XLRS. The microvascular changes described could only be picked up on SS OCT-A. These changes could potentially serve as biomarkers of prognosis. We conclude that SS OCT-A can be a useful method for the study of both the structural and vascular macular changes in XLRS disease.

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

 

Figure 1. a) Superficial neurovascular layer. b) Deep neurovascular layer. c) Choroidal layer. Red arrows: Telangiectatic changes. Yellow arrows: capillary dropout areas.

Figure 1. a) Superficial neurovascular layer. b) Deep neurovascular layer. c) Choroidal layer. Red arrows: Telangiectatic changes. Yellow arrows: capillary dropout areas.

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