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Romano, Francesco MD*,†; Arrigo, Alessandro MD*; Ch'ng, Soon Wai FRCOphth; Battaglia Parodi, Maurizio MD*; Manitto, Maria Pia MD*; Martina, Elisabetta MD*; Bandello, Francesco MD, FEBO*; Stanga, Paulo E. MD†,‡

doi: 10.1097/IAE.0000000000002222
Original Study

Purpose: To assess foveal and parafoveal vasculature at the superficial capillary plexus, deep capillary plexus, and choriocapillaris of patients with X-linked retinoschisis by means of optical coherence tomography angiography.

Methods: Six patients with X-linked retinoschisis (12 eyes) and seven healthy controls (14 eyes) were recruited and underwent complete ophthalmologic examination, including best-corrected visual acuity, dilated fundoscopy, and 3 × 3-mm optical coherence tomography angiography macular scans (DRI OCT Triton; Topcon Corp). After segmentation and quality review, optical coherence tomography angiography slabs were imported into ImageJ 1.50 (NIH; Bethesda) and digitally binarized. Quantification of vessel density was performed after foveal avascular zone area measurement and exclusion. Patients were additionally divided into “responders” and “nonresponders” to dorzolamide therapy.

Results: Foveal avascular zone area resulted markedly enlarged at the deep capillary plexus (P < 0.001), particularly in nonresponders. Moreover, patients disclosed a significant deep capillary plexus rarefaction, when compared with controls (P: 0.04); however, a subanalysis revealed that this damage was limited to the fovea (P: 0.006). Finally, the enlargement of foveal avascular zone area positively correlated with a decline in best-corrected visual acuity (P: 0.01).

Conclusion: Prominent foveal vascular impairment is detectable in the deep capillary plexus of patients with X-linked retinoschisis. Our results correlate with functional outcomes, suggesting a possible vascular role in X-linked retinoschisis clinical manifestations.

Pediatric patients with genetically confirmed X-linked retinoschisis carry significant foveal vascular alterations at the deep capillary plexus. These results correlate with the functional outcome in terms of visual acuity and suggest a possible vascular impairment in the course of this disease.

*Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, Milan, Italy;

Manchester Vision Regeneration (MVR) Lab, Manchester Royal Eye Hospital, NIHR/Wellcome Trust Manchester CRF, Manchester, United Kingdom; and

Division of Evolution & Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom.

Reprint requests: Maurizio Battaglia Parodi, MD, Department of Ophthalmology, University Vita-Salute, Scientific Institute San Raffaele, via Olgettina 60, 20132 Milan, Italy; e-mail:

M. Battaglia Parodi is a consultant for Bausch & Lomb Inc. P. E. Stanga is a consultant for Allergan Plc., Bausch & Lomb Inc., Bayer AG, Novartis AG, Optos Plc., Second Sight Medical Products, Inc., Topcon Corp., and Carl Zeiss AG. F. Bandello is a consultant for Alcon, Allergan Plc., Famila-Thea, Bayer Schering Pharma AG, Bausch & Lomb, Hoffmann-La-Roche, Novartis, Sanofi-Aventis, and Carl Zeiss AG. The remaining authors have no financial/conflicting interests to disclose.

© 2019 by Ophthalmic Communications Society, Inc.