OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY: A NEW IMAGING APPROACH FOR THE ASSESSMENT OF MACULAR CHANGES IN X-LINKED JUVENILE RETINOSCHISIS

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.