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.
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.
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).
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: firstname.lastname@example.org
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.