Abstract
Purpose. This study aimed at assessing the prevalence of pathologies presenting retinal inner nuclear layer (RINL) microcystic perimacular changes associated with optic nerve atrophy (OA). The charts of patients presenting a significant defect of the Retinal Nerve Fiber Layer (RNFL) were included prospectively in this study. Patients were classified according to the etiology of the RNFL defect. Two hundred and one eyes of 138 patients were enrolled in this analysis. Retinal images obtained showed the typical hyporeflective perifoveal crescent-shaped lesion composed of small round hyporeflective microcysts confined to the RINL in 35.3% of the eyes. Those findings were found in 75% of eyes presenting hereditary OA, 50% of eyes presenting ischemic optic neuritis, 50% of eyes with drusen of the optic nerve (ON), 44.4% of eyes presenting a compressive OA, 32% of eyes presenting inflammatory optic neuropathy from multiple sclerosis, 18.5% of eyes presenting OA from undetermined origin, and 17.6% of eyes having primary open-angle glaucoma. This study demonstrates that microcystic changes in RINL are not specific to a disease but are found in OA of various etiologies. Moreover, their incidence was found to be dependent upon the cause of OA, with the highest incidence occurring in genetic OA.
Highlights
Optic nerve atrophy (OA) is a wide spectrum of hereditary or acquired optic neuropathies arising from various etiologies
Of the 201 eyes presenting those Retinal Nerve Fiber Layer (RNFL) defects, there were 40 eyes (19.9%) presenting with hereditary optic atrophy [mitochondrial or autosomal dominant optic atrophy (ADOA)], 6 eyes (3%) with ischemic optic neuritis (ION), 4 eyes (2%) with drusen of the optic nerve (ON), 9 eyes (4.5%) with compressive OA as diagnosed with brain and orbital Magnetic Resonance Imaging (MRI), 27 eyes (13.4%) with inflammatory optic neuropathy from multiple sclerosis (MS), 27 patients (13.4%) with OA from undetermined origin, 85 eyes (42.3%) with primary open-angle glaucoma (POAG), 2 eyes (1%) with idiopathic intracranial hypertension (IIH), and 1 eye (0.5%) presenting with juxtapapillary toxoplasmic retinochoroiditis. Further analysis of those eyes with RNFL defects with scanning laser ophthalmoscope infrared (SLO-IR), B-scans, and “en face” SD-OCT showed in 71 eyes (35.3%) a hyporeflective perifoveal crescent-shaped lesion composed of small round hyporeflective microcysts confined to the retinal inner nuclear layer (RINL) without any extension to the adjacent layers
No similar lesion was found in eyes presenting IIH or toxoplasmic retinochoroiditis
Summary
Optic nerve atrophy (OA) is a wide spectrum of hereditary or acquired optic neuropathies arising from various etiologies. High-resolution retinal imaging technologies, such as scanning laser ophthalmoscope infrared (SLO-IR) imaging and Spectral Domain Optical Coherence Tomography (SDOCT), and both B-scans and “en face,” were shown to help define the location and extent of structural damage occurring in several chorioretinal diseases [3]. In a previous study [4], the authors had noticed the presence of macular microcysts in the retinal inner nuclear layer (RINL) in patients suffering from advanced OA. These microcysts are never observed in normal eyes. In this study, using high-resolution retinal imaging technologies, the authors analyzed prospectively the incidence, in different ocular pathologies, of these RINL microcystic changes that are associated with atrophy of the optic nerve (ON)
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