Abstract

The aim of this retrospective study was to describe the vascular features in eyes with Coats disease, using optical coherence tomography angiography (OCTA), at baseline and after 3 monthly intravitreal injections of ranibizumab. Fifteen eyes of 15 consecutive patients affected by Coats' disease were recruited in this study. All patients underwent the best-corrected visual acuity (BCVA) evaluation, fundus examination, fluorescein angiography (FA), indocyanine green angiography (ICGA), multicolor imaging, structural Spectral Domain (SD)-OCT and OCTA at baseline and 1 month after the third monthly ranibizumab injection (loading phase). Fifteen patients completed the study, of whom nine were males and six females. Mean age was 20.4 ± 2 years. BCVA was 0.46 ± 0.11 logMar and 0.47 ± 0.12 logMar at baseline and after treatment, respectively (p = 0.164). SD-OCT revealed no significant decrease in central macular thickness (486.33 μm ± 93.37 at baseline vs. 483.4 μm ± 80.97 after treatment; p = 0.915). The subretinal exudates persisted in macular region after intravitreal injections. OCTA showed a general vascular rarefaction in superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillary (CC) that did not change after loading phase. This study showed no functional and vascular improvement following 3 monthly ranibizumab injections. OCTA, non-invasive technique, could be useful during follow up of these patients and provide a better understand of pathogenesis of this disorder.

Highlights

  • Coats disease is a retinopathy characterized by idiopathic vascular abnormalities in retinal periphery, subretinal and intraretinal extensive exudation [18]

  • Coats disease leads to vision loss due to exudative retinal detachment, and less frequently neovascular glaucoma [12]

  • Initial treatment includes ablation therapy with cryotherapy or laser photocoagulation of the telangiectasic areas, but subretinal fluid (SRF) and massive exudation are often responsible for therapeutic failure [18]

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Summary

Introduction

Coats disease is an idiopathic retinal vascular disorder, described for the first time by Coats in 1908 [1].This disease is characterized by retinal telangiactasias in the temporal-macular zone, numerous yellowish exudates in the subretinal space containing cholesterol crystals, macular edema, hemorrhages and, in advanced end-stage, by exudative retinal detachment with secondary neovascular glaucoma [2].OCTA in Coats DiseaseThe use of intravitreal anti-vascular endothelial growth factor (VEGF) agents in combination with common treatments, such as laser photocoagulation, has been reported to reduce the subretinal fluid (SRF) and exudation in small case series of Coats disease [3,4,5].The gold standard test for diagnosis and follow-up after treatment for Coats patients is fluorescein angiography (FA), while optical coherence tomography (OCT) allows to identify macular edema and exudates in macular region [6].OCT Angiography (OCTA) is a non-invasive imaging technique that provides a detailed evaluation of retinal and choriocapillaris (CC) microvasculature and it turned to be useful and safe in pediatric patients because it does not need intravenous dye agent injection [7, 8].Previous reports focused on the retinal and CC vascular alterations at OCTA in patients with Coats disease rather than to analyze the vascular changes after VEGF injections [9,10,11,12,13]. Coats disease is an idiopathic retinal vascular disorder, described for the first time by Coats in 1908 [1] This disease is characterized by retinal telangiactasias in the temporal-macular zone, numerous yellowish exudates in the subretinal space containing cholesterol crystals, macular edema, hemorrhages and, in advanced end-stage, by exudative retinal detachment with secondary neovascular glaucoma [2]. The use of intravitreal anti-vascular endothelial growth factor (VEGF) agents in combination with common treatments, such as laser photocoagulation, has been reported to reduce the subretinal fluid (SRF) and exudation in small case series of Coats disease [3,4,5]. OCT Angiography (OCTA) is a non-invasive imaging technique that provides a detailed evaluation of retinal and choriocapillaris (CC) microvasculature and it turned to be useful and safe in pediatric patients because it does not need intravenous dye agent injection [7, 8].

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