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PURPOSE: To compare non-syndromic and syndromic forms of USH2A-related retinitis pigmentosa (RP) by means of structural optical coherence tomography (OCT) and OCT-angiography (OCTA). METHODS: Observational, cross-sectional, multicenter study. All patients underwent best corrected visual acuity (BCVA) measurement, OCT (Spectralis HRA + OCT, Heidelberg Engineering) and OCTA (OCT DRI Topcon Triton, Topcon Corporation). We compared subfoveal choroidal thickness (SCT), choroidal vascularity index (CVI), presence of cystroid macular edema (CME), macular vessel density (VD) at the superficial and deep capillary plexa, as well as VD of the radial peripapillary capillary (RPC) network, between syndromic and non-syndromic patients with USH2A-associated retinopathy. RESULTS: Thirty-four eyes from 18 patients (7 females) were included. Thirteen patients (72.2%) were affected by Usher syndrome type 2, whereas the remaining 5 subjects (27.8%) had non-syndromic retinitis pigmentosa (nsRP). Syndromic patients were younger than nsRP (p = 0.01) and had a worse visual acuity than those with the exclusively retinal phenotype. Patients with Usher syndrome type 2 had a higher prevalence of CME and a thicker choroid compared to nsRP, although these results were not statistically significant (p = 0.775 and p = 0.122, respectively). Similarly, none of the other quantitative OCT and OCTA parameters was statistically different between the two groups. CONCLUSIONS: Despite their younger age, patients with Usher syndrome type 2 displayed similar choroidal and microvascular changes compared to those with nsRP.
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Purpose: To propose a noninvasive way of classifying multimodal imaging of retinal microaneurysms (MA) secondary to diabetic retinopathy (DR). Methods: The research was designed as a cross-sectional, observational study of patients affected by DR. Multimodal imaging included confocal MultiColor imaging, optical coherence tomography (OCT) and OCT angiography (OCTA). MA green- and infrared-reflectance components were assessed by confocal MultiColor imaging, reflectivity properties by OCT, and MA perfusion features by OCTA. In addition, we included high-resolution (HR) and high-speed (HS) OCTA scans to assess HR-HS agreement in detecting retinal MA and to highlight different perfusion features detected by both OCTA acquisitions. Results: We analyzed 216 retinal MAs, divided into green (46; 21%), red (58; 27%) and mixed types (112; 52%). Green MAs were mainly hyper-reflective on OCT, with no or poor filling on OCTA. Red MAs were characterized by an isoreflective signal on OCT and full filling on OCTA. Mixed MAs showed a hyper-reflective border and a hyporeflective core on OCT and partial filling on OCTA. No differences in red MA HR/HS size discrepancy and reflectivity were found, whereas these progressively increased as the MA MultiColor signal changed from infrared to green. MA types significantly correlated with visual acuity, DR duration, and DR severity. Conclusions: Retinal MA can be classified reliably by means of a fully noninvasive multimodal imaging-based assessment. MA types are matched with visual acuity, DR duration and DR severity. Both HR and HS OCTA are highly effective in detecting MA, although HR OCTA is to be preferred in the presence of fibrotic evolution. Translational Relevance: This study outlines a proposed novel MA classification based on noninvasive multimodal imaging. The findings presented in this paper endorse the clinical relevance of this approach, highlighting how this classification is associated with both DR duration and severity.
