E Boulanger-Scemama1, J Akesbi2, S Tick3, S Mohand-Said3,4, J-A Sahel1,3,4,5, I Audo6,7. 1. Fondation Ophtalmologique Adolphe de Rothschild, Paris, France. 2. Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Service II, 75012, Paris, France. 3. DHUSight Restore, INSERM-DGOS CIC 1423, Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, France. 4. INSERM U968, CNRS UMR_7210, Institut de la Vision, UPMC Univ Paris 06, Sorbonne Universités, 17 Rue Moreau, 75012, Paris, France. 5. Department of Ophthalmology, The University of Pittsburgh School of Medicine, Pittsburgh, PA, 15213, USA. 6. DHUSight Restore, INSERM-DGOS CIC 1423, Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, France. isabelle.audo@inserm.fr. 7. INSERM U968, CNRS UMR_7210, Institut de la Vision, UPMC Univ Paris 06, Sorbonne Universités, 17 Rue Moreau, 75012, Paris, France. isabelle.audo@inserm.fr.
Abstract
PURPOSE: Patients with unusual macular retinal pigment epithelium (RPE) hypopigmentation are described and analyzed using retinal multimodal imaging. METHODS: We report three cases of patients with unilateral (2) or bilateral (1) macular lesions discovered incidentally on fundoscopy. A comprehensive ophthalmic examination including visual acuity, fundoscopy, spectral-domain optical coherence tomography (SD-OCT), short-wavelength light and near-infrared autofluorescence, fluorescein angiography, microperimetry, multifocal electroretinogram, adaptive optics (AO), and OCT-angiography (OCT-A) has been performed. RESULTS: Visual acuity was 20/20 in both eyes of all patients. The lesion appeared hyperautofluorescent on short-wavelength light and hypoautofluorescent on near-infrared light. Fluorescein angiography revealed a sharply demarcated macular hyperfluorescence without any leakage, suggesting a window defect. Interestingly, SD-OCT revealed only a choroidal hyperreflectivity in relation to the lesions without any abnormality of the outer retinal layers. Microperimetry was normal except for 1 patient with bilateral lesion and subtle decrease in macular sensitivity. Mf ERG was normal in all three patients. AO showed a well-preserved cone mosaic, suggesting that the abnormality was localized under the photoreceptor layers. OCT-A revealed hyperreflectivity just below the RPE layer, corresponding to the macular lesion observed on fundoscopy and the choroidal hyperreflectivity seen on SD-OCT. CONCLUSIONS: Macular RPE hypopigmentation should be considered in case of an isolated macular lesion without functional visual impairment or anatomical defect on SD-OCT.
PURPOSE:Patients with unusual macular retinal pigment epithelium (RPE) hypopigmentation are described and analyzed using retinal multimodal imaging. METHODS: We report three cases of patients with unilateral (2) or bilateral (1) macular lesions discovered incidentally on fundoscopy. A comprehensive ophthalmic examination including visual acuity, fundoscopy, spectral-domain optical coherence tomography (SD-OCT), short-wavelength light and near-infrared autofluorescence, fluorescein angiography, microperimetry, multifocal electroretinogram, adaptive optics (AO), and OCT-angiography (OCT-A) has been performed. RESULTS: Visual acuity was 20/20 in both eyes of all patients. The lesion appeared hyperautofluorescent on short-wavelength light and hypoautofluorescent on near-infrared light. Fluorescein angiography revealed a sharply demarcated macular hyperfluorescence without any leakage, suggesting a window defect. Interestingly, SD-OCT revealed only a choroidal hyperreflectivity in relation to the lesions without any abnormality of the outer retinal layers. Microperimetry was normal except for 1 patient with bilateral lesion and subtle decrease in macular sensitivity. Mf ERG was normal in all three patients. AO showed a well-preserved cone mosaic, suggesting that the abnormality was localized under the photoreceptor layers. OCT-A revealed hyperreflectivity just below the RPE layer, corresponding to the macular lesion observed on fundoscopy and the choroidal hyperreflectivity seen on SD-OCT. CONCLUSIONS: Macular RPE hypopigmentation should be considered in case of an isolated macular lesion without functional visual impairment or anatomical defect on SD-OCT.
Authors: Carol L Shields; Aparna Ramasubramanian; W Benjamin Kunz; Ekta Aggarwal; Jerry A Shields Journal: Ophthalmology Date: 2009-11-24 Impact factor: 12.079
Authors: Husam Alghanem; Tapas R Padhi; Adrienne Chen; Leslie M Niziol; Maria Fernanda Abalem; Natalie Dakki; Timothy Steffens; Chris Andrews; David C Musch; K Thiran Jayasundera; Naheed W Khan Journal: Transl Vis Sci Technol Date: 2019-09-27 Impact factor: 3.283