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ObjectiveTo evaluate the risk factors and outcomes of central retinal vein occlusion (CRVO) in young patients (

Purpose: To report the use of Spectralis High Magnification Module (HMM) as part of multimodal imaging in Multiple Evanescent White Dot Syndrome (MEWDS). Observations: HMM imaging showed a blurry mosaic pattern corresponding to MEWDS-related photoreceptors’ lesions. These abnormalities remained detectable at later stages of the disease while other imaging modalities were negative. Conclusions and importance: HMM can be a useful technique to monitor the structure of the outer retina during the different stages of MEWDS.

Central retinal vein occlusion (CRVO) is a common retinal disease. Recent works mentioned spontaneous retinal arterial pulsations (SRAPs) as a feature of some CRVOs. This is a retrospective study on patients presenting with CRVO who were followed up for at least 6 months. The objective was to identify SRAP in the acute phase of the disease and determine their relationship with patients’ characteristics and visual prognosis. A 10-second infrared film centered on the optic disc was recorded within a month of the onset of symptoms, and SRAPs were detected in two-thirds of the cases. Patients with SRAP were significantly younger than those without SRAP. Mean central macular thickness was significantly higher in the absence of SRAP, which was translated into a more severe macular edema; however, this difference faded with time. BCVA tended to be higher in the presence of SRAP at the 6-month follow-up when adjusted to baseline. This study demonstrates that SRAPs are a frequent finding, easily detected by infrared fundus video recording, and associated with a younger age and lesser macular edema.

Abstract EMAP (Extensive Macular Atrophy with Pseudodrusen) is a maculopathy we recently described that shares pseudodrusen and geographic atrophy with Age-related Macular Disease (AMD). EMAP differs from AMD by an earlier age of onset (50-55 years) and a characteristic natural history comprising a night blindness followed by a severe visual loss. In a prospective case-control study, ten referral centers included 115 EMAP (70 women, 45 men) patients and 345 matched controls to appraise dietary, environmental, and genetic risk factors. The incidence of EMAP (mean 2.95/1.106) was lower in Provence-Côte d’Azur with a Mediterranean diet (1.9/1.106), and higher in regions with intensive farming or industrialized activities (5 to 20/1.106). EMAP patients reported toxic exposure during professional activities (OR 2.29). The frequencies of common AMD complement factor risk alleles were comparable in EMAP. By contrast, only one EMAP patient had a rare AMD variant. This study suggests that EMAP could be a neurodegenerative disorder caused by lifelong toxic exposure and that it is associated with a chronic inflammation and abnormal complement pathway regulation. This leads to diffuse subretinal deposits with rod dysfunction and cone apoptosis around the age of 50 with characteristic extensive macular atrophy and paving stones in the far peripheral retina.

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.

Purpose. The recent use of “en-face” enhanced-depth imaging spectral-domain optical coherence tomography (EDI SD-OCT) helps distinguish the retinal layers involved in the physiopathology of multiple evanescent white dot syndrome (MEWDS). Methods. Four patients presenting with MEWDS underwent a comprehensive ocular examination including C-scan (“en-face”) EDI SD-OCT at the initial visit and during follow-up. Results. C-scans combined with the other multimodal imaging enabled the visualization of retinal damage. Acute lesions appeared as diffuse and focal disruptions occurring in the ellipsoid and interdigitation zones. The match between autofluorescence imaging, indocyanine green angiography, and “en-face” OCT helped identify the acute microstructural damages in the outer retina further than the choroid. Follow-up using “en-face” EDI-OCT revealed progressive and complete recovery of the central outer retinal layers. Conclusion. “En-face” EDI SD-OCT identified the site of initial damage in MEWDS as the photoreceptors and the interdigitation layers rather than the choroid. Moreover, “en-face” OCT is helpful in the follow-up of these lesions by being able to show the recovery of the outer retinal layers.

Purpose. To report B-scan and “En-face” spectral-domain optical coherence tomography (SD-OCT) findings in acute retinal pigment epitheliitis (ARPE). Methods. Two patients (3 eyes) with ARPE were examined. Fluorescein and indocyanine green (ICGA) angiography, B-scan, and “En-face” SD-OCT were performed in each patient at initial and follow-up visits. Results. Both patients presented with acute onset of blurred vision, and one with bilateral involvement. B-can OCT revealed disruption of the macular retinal pigment epithelial (RPE) inner band layer and photoreceptors’ inner and outer segment (IS-OS) junction. Hyperreflective dots were observed in the outer nuclear layer (ONL) above the RPE/IS-OS disruption. Just around these hyperreflective dots, slight thickening of the hyperreflective IS/OS junction was observed. During the late phase, indocyanine green angiography (ICGA) showed a macular cockade-like hyperfluorescent halo. “En-face” OCT showed the same cockade-like appearance with a hyporeflective center and a hyperreflective border matching the pattern observed on ICGA. At followup, as vision improved without treatment, B-scan OCT demonstrated progressive resolution of the hyperreflective and disrupted lesions; “en-face” OCT also showed disappearance of the macular cockade-like halo with a transient discrete hyperreflective macular star at the RPE level in one eye. Conclusion. “En-face” OCT associated with B-scan SD-OCT analysis appears to be very helpful in the diagnosis and followup of ARPE. The pathophysiology of ARPE remains complex and still poorly understood. These techniques help define the location and extent of structural damage occurring in this disease.

Purpose. “En face” is an emerging imaging technique derived from spectral domain optical coherence tomography (OCT). It produces frontal sections of retinal layers, also called “C-scan OCT.” Outer retinal tubulations (ORTs) in age-related macular degeneration (AMD) are a recent finding evidenced by spectral-domain OCT. The aim of this study is to characterize the morphology of ORT according to the form of AMD, using “en-face” spectral domain OCT. Methods. “En face” OCT imaging was prospectively performed in 26 consecutive eyes with AMD that also had ORT. Results. There were 15 neovascular, 8 atrophic, and 3 eyes with a mixed (fibrotic and atrophic) form of AMD. Among the neovascular group, the most frequent tubulation pattern on “en-face” OCT was a branching network emanating from a fibrovascular scar; we term this pattern as “pseudodendritic.” It did not require treatment when observed as an isolated finding. In all cases of atrophic AMD, the tubular network was located at the edge of the geographic atrophy area, and formed a “perilesional” pattern. Six atrophic cases showed tubular invaginations inside this area. Conclusion. “En face” OCT is a valuable technique in the diagnosis and followup of macular disease. It revealed the main characteristic patterns of ORT associated with neovascular and atrophic AMD.