Adaptive Optics Imaging in Patients Affected by Pseudoxanthoma Elasticum
| Autor: | Dario Giorgio, Dario Pasquale Mucciolo, Andrea Sodi, Federica Boraldi, Daniela Quaglino, Gianni Virgili, Lucia Finocchio, Laura Pavese, Vittoria Murro, Fabrizio Giansanti |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Adult
Male medicine.medical_specialty Visual acuity Adolescent genetic structures Fundus Oculi Visual Acuity Fundus (eye) Multimodal Imaging Ophthalmoscopy Young Adult 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Retinal Diseases Ophthalmology medicine Humans Peau d'orange Fluorescein Angiography Pseudoxanthoma Elasticum Aged Retrospective Studies 030304 developmental biology 0303 health sciences medicine.diagnostic_test business.industry Optical Imaging Retinal Middle Aged medicine.disease Pseudoxanthoma elasticum eye diseases Angioid streaks chemistry 030221 ophthalmology & optometry Female sense organs Multidrug Resistance-Associated Proteins medicine.symptom business Tomography Optical Coherence Photoreceptor Cells Vertebrate Retinopathy |
| Popis: | Purpose To describe the retinal findings of patients affected by pseudoxanthoma elasticum (PXE) using a multimodal imaging approach including flood-illumination adaptive optics ophthalmoscopy (AO). Design Retrospective case series. Materials and Methods Patients affected by PXE were retrospectively studied. Clinical data, color, infrared and autofluorescence fundus imaging, optical coherence tomographic scans, and AO examinations were collected. Furthermore, the photoreceptor count was assessed. PXE diagnosis was confirmed by a positive skin biopsy and/or genetic testing. Results Twenty-one eyes of 18 patients (11 females and 7 males) were included in the study. In 3 patients, both eyes were studied. The mean age at examination was 37.7 ± 16.4 years (range 14-66) and the mean best-corrected visual acuity (BCVA) was 0.1 ± 0.2 logMAR (range 0-1). We identified 3 types of angioid streaks (AS) using AO: “crack,” “band,” and “hypopigmented.” The first 2 were very similar and they differed in size; the third type showed specific clinical features. Comet lesions appeared as hyper-reflective round lesions on AO imaging. In all eyes, the cone mosaic appeared reduced inside the streaks compared to the neighboring areas (13,532.8 ± 1,366.5 cones/mm2 vs 16,817.1 ± 1,263.0 cones/mm2 respectively). Conclusion Using AO imaging in PXE-related retinopathy, we were able to observe the presence of the photoreceptors within the angioid streaks, differentiate 3 types of angioid streaks, based on size and reflective features, and identify the very small crystalline bodies not identifiable using other retinal imaging techniques. |
| Databáze: | OpenAIRE |
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