Multimodal imaging in Schubert-Bornschein congenital stationary night blindness
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Taylor and Francis Ltd.
Abstract
Background: Schubert-Bornschein (SB) is the most common type of people with congenital stationary night blindness (CSNB). The aim of the study is to describe the optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) findings in patients with SB CSNB. Methods: Prospective, observational case series including three patients with genetically confirmed CSNB along with matched controls, who underwent complete ophthalmic examination and multimodal imaging. Results: On SD-OCT, a significant focal outer plexiform layer (OPL) thickening and a corresponding focal outer nuclear layer (ONL) thinning were identified in the macular area (p < 0.001). OCTA analysis overall showed decreased density of macular deep capillary plexus (mDCP) and macular choriocapillaris (mCC) (p = 0.008 and p = 0.033, respectively). DCP vessel density in the area corresponding to OPL thickening was significantly increased compared to the remaining retina (p < 0.001). Conclusion: SB CSNB is characterized by retinal vascular impairment, as detected on OCTA. © 2022 Taylor & Francis Group, LLC.
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Congenital stationary night blindness, Optical coherence tomography angiography, Schubert-bornschein, Fluorescein angiography, Humans, Multimodal imaging, Night blindness, Night blindness, congenital stationary, Prospective studies, Retina, Retinal vessels, Tomography, optical coherence, A wave, Adult, Article, Autofluorescence imaging, B wave, Best corrected visual acuity, Blood vessel density, Cacna1f gene, Central macular thickness, Childhood, Choroid capillary layer, Choroidal thickness, Clinical article, Controlled study, Dark adaptation, Electroretinogram, Eye examination, Gene, Genetic disorder, High throughput sequencing, Human, Image quality, Male, Medical history, Observational study, Prognostic assessment, Prospective study, Radial peripapillary capillary, Refraction error, Retinal outer nuclear layer, Retinal outer plexiform layer, Retinal thickness, Schubert bornschein congenital stationary night blindness, Slit lamp microscopy, Spectral domain optical coherence tomography, X chromosome, Diagnostic imaging, Fluorescence angiography, Genetics, Optical coherence tomography, Retina blood vessel