Selim Bolukbasi1, Cezmi Dogan2, Ertugrul Kiykim3, Akin Cakir4, Burak Erden4, Alper Halil Bayat4, Mustafa Nuri Elcioglu4, Ayse Cigdem Aktuglu Zeybek3. 1. Department of Ophthalmology, Okmeydani Training and Research Hospital, University of Health Sciences, Istanbul, Turkey. selimbolukbasi@hotmail.com. 2. Department of Ophthalmology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey. 3. Department of Pediatrics, Division of Nutrition and Metabolism, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey. 4. Department of Ophthalmology, Okmeydani Training and Research Hospital, University of Health Sciences, Istanbul, Turkey.
Abstract
PURPOSE: To evaluate accumulation patterns of deposits in retinal layers of type B Niemann-Pick patients by multimodal imaging. METHODS: Seven patients with type B Niemann-Pick disease were included in this study. All participants underwent a complete ophthalmologic evaluation, high-resolution digital colour imaging, spectral-domain optical coherence tomography, blue light fundus autofluorescence and optical coherence tomography angiography (OCTA). RESULTS: We demonstrated different accumulation patterns in the retinal ganglion cell layer, the retinal nerve fibre layer and the subfoveolar region by multimodal imaging. Local retinal capillary nonflow areas in the superficial plexus, increased vascular tortuosity and deformed foveal avascular areas were shown in OCTA scans. CONCLUSION: Multimodal imaging including OCTA is a useful technique for the identification of different types of accumulation patterns, diagnosis and follow-up of type B Niemann-Pick patients.
PURPOSE: To evaluate accumulation patterns of deposits in retinal layers of type B Niemann-Pickpatients by multimodal imaging. METHODS: Seven patients with type B Niemann-Pick disease were included in this study. All participants underwent a complete ophthalmologic evaluation, high-resolution digital colour imaging, spectral-domain optical coherence tomography, blue light fundus autofluorescence and optical coherence tomography angiography (OCTA). RESULTS: We demonstrated different accumulation patterns in the retinal ganglion cell layer, the retinal nerve fibre layer and the subfoveolar region by multimodal imaging. Local retinal capillary nonflow areas in the superficial plexus, increased vascular tortuosity and deformed foveal avascular areas were shown in OCTA scans. CONCLUSION: Multimodal imaging including OCTA is a useful technique for the identification of different types of accumulation patterns, diagnosis and follow-up of type B Niemann-Pickpatients.
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