Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION) associated with Paracentral Acute Middle Maculopathy (PAMM) and Cilioretinal Artery Occlusion (CILRAO): multimodal imaging of a newly described clinical association.

PURPOSE: To report multimodal imaging features of a novel association of non-arteritic anterior ischemic optic neuropathy (NAION), paracentral acute middle maculopathy (PAMM), and cilioretinal artery occlusion (CILRAO). METHODS: A 74-year-old male patient presented complaining of an acute subjective decline in visual acuity in the right eye over the previous days. This patient underwent a comprehensive ophthalmological assessment, including multimodal retinal imaging and visual field test. RESULTS: The eye examination revealed yellowish optic disc edema in the right eye with peripapillary hemorrhages and a whitish lesion extending from the temporal margin of the optic nerve head towards the macula. Multimodal imaging confirmed the diagnosis of NAION in the right eye. Notably, the structural B-scan and en face OCT images revealed hyperreflective areas in the inner and mid retinal layers, indicating an association with CILRAO and PAMM. CONCLUSION: We herein describe the clinical findings of a previously unreported association among NAION, CILRAO and PAMM.

Inter-Reader and Inter-Modality Variability in Macular Atrophy Quantification in Neovascular AMD: Comparison of Six Imaging Modalities.

PURPOSE: Macular atrophy is a common complication in neovascular age-related macular degeneration (AMD) and is associated with poorer visual outcomes. This study evaluated inter-reader and inter-modality variability in measuring macular atrophy in previously-treated neovascular AMD eyes without exudation, using six imaging modalities. DESIGN: Prospective, cohort study. PARTICIPANTS: Thirty participants with previously-treated neovascular AMD, showing no signs of exudation at the time of enrollment, and exhibiting macular atrophy. METHODS: During the same clinic visit, patients were imaged using six different imaging modalities: color fundus photography (CFP; Clarus, Carl Zeiss Meditec), near-infrared imaging (NIR; Spectralis; Heidelberg Engineering, Heidelberg, Germany), structural optical coherence tomography (OCT; Spectralis; Heidelberg Engineering, Heidelberg, Germany), green fundus autofluorescence (GAF; Clarus, Carl Zeiss Meditec), blue fundus autofluorescence (BAF; Spectralis; Heidelberg Engineering, Heidelberg, Germany), and pseudocolor imaging (MultiColor; Spectralis; Heidelberg Engineering, Heidelberg, Germany). Two readers independently measured the macular atrophy area. MAIN OUTCOME MEASURES: Inter-reader and inter-modality agreement. RESULTS: The 95% coefficient of repeatability (CR) was 5.98 mm2 for CFP, 4.46 mm2 for MultiColor, 3.90 mm2 for BAF, 3.92 mm2 for GAF, 4.86 mm2 for NIR, and 3.55 mm2 for OCT. Similarly, the coefficient of variation (CV) was lowest for the OCT-based grading at 0.08 and highest for the NIR-based grading at 0.28. Accordingly, the intraclass correlation coefficient (ICC) was 0.742 for CFP, 0.805 for MultiColor, 0.857 for BAF, 0.850 for GAF, 0.755 for NIR, and 0.917 for OCT. The 6 different imaging modalities presented measurements with different mean values, with only a limited number of comparisons not significantly different between the instruments, although measurements were correlated. The largest size of macular atrophy was with the structural OCT-based grading (median=4.65 mm2; interquartile range [IQR]=4.78 mm2) and the smallest was with the CFP-based grading (median=3.86 mm2; IQR=5.06 mm2). Inconsistencies arose from various factors. CONCLUSIONS: In patients with neovascular AMD, macular atrophy measurements vary significantly depending on the imaging technique used. CFP-based assessments yielded the smallest macular atrophy sizes, while structural OCT-based assessments produced the largest. These discrepancies stem from both the inherent limitations of each modality in assessing RPE atrophy and factors related to neovascularization, such as the coexistence of fibrosis.

Deep Learning in Neovascular Age-Related Macular Degeneration.

Background and objectives: Age-related macular degeneration (AMD) is a complex and multifactorial condition that can lead to permanent vision loss once it progresses to the neovascular exudative stage. This review aims to summarize the use of deep learning in neovascular AMD. Materials and Methods: Pubmed search. Results: Deep learning has demonstrated effectiveness in analyzing structural OCT images in patients with neovascular AMD. This review outlines the role of deep learning in identifying and measuring biomarkers linked to an elevated risk of transitioning to the neovascular form of AMD. Additionally, deep learning techniques can quantify critical OCT features associated with neovascular AMD, which have prognostic implications for these patients. Incorporating deep learning into the assessment of neovascular AMD eyes holds promise for enhancing clinical management strategies for affected individuals. Conclusion: Several studies have demonstrated effectiveness of deep learning in assessing neovascular AMD patients and this has a promising role in the assessment of these patients.

Mucopolysaccharidosis type II B complicated by optic disc swelling, pigmentary retinopathy and macular edema.

PURPOSE: To report clinical and imaging features of optic nerve and retinal involvement in a patient with mucopolysaccharidosis (MPS) type II B. METHODS: A 27-year-old man, diagnosed with MPS type II B and undergoing enzymatic substitution therapy for the past 19 years, was referred to the retina service. An ophthalmological evaluation, which included multimodal imaging, was conducted to investigate potential retinal and optic disc involvement. RESULTS: The eye examination revealed a pigmentary retinopathy with a predominant loss of the outer retinal loss, primarily in the parafoveal and perifoveal regions. Notably, multimodal imaging identified macular edema without any signs of leakage, implying an association between macular edema and retinal neurodegeneration. Additionally, both eyes exhibited an optic disc with blurred margins. CONCLUSION: We herein describe the multimodal imaging findings of retinal and optic disc involvement in a patient with MPS type II B. This report describes for the first-time the presence of macular edema without leakage alongside photoreceptor damage and optic disc swelling.

Blindness and visual impairment: quality of life and accessibility in the city of Turin.

Purpose: Despite the increase in socio-health conditions and, in general, the focus on health worldwide, many diseases still adversely affect the quality of life (QoL), including those causing vision loss. The main purpose of this study was to evaluate the QoL of people with visual impairments through a questionnaire and identify issues concerning everyday life in the urban and extra-urban areas of Turin. Patients and methods: A personalized questionnaire including 25 questions was distributed to 100 enrolled patients. It was designed by integrating the most widely used questionnaires related to the QoL of people with visual impairment with questions concerning the city of Turin. The inclusion criteria were any degree of visual impairment (from mild defect to complete blindness), according to Law n. 138/2001 classification. The exclusion criteria were mental disability and residence in care homes. Finally, statistical analysis was performed. Pearson's Chi-Square test was used to evaluate the strength of the association between two qualitative variables in different sections of the questionnaire. The results were classified as statistically significant with a p-value of ≤0.05 or borderline (0.05 < p-value<0.10). Results: Based on responses to question 7 (Q7), 67% of selected patients stated that sight markedly influences their QoL. Moreover, 49% of patients responding to question 12 considered themselves almost completely dependent on other people regarding mobility and movement in and around Turin. In total, 57% used public transport (Q13); however, 50% of them found it challenging to access (Q14). Personal aids (e.g., white cane and magnifying glasses) were adopted only by 51% (Q15), and 63% of patients responding to question 18 suggested a refinement of urban aids (e.g., road signs). Of the 53 patients, 30 patients (56.6%) considered Turin a livable city for visually impaired people (Q19); however, 44 patients (84.6%) reported no significant improvements in Turin's urban logistics during the last 5 years and highlighted the urgent need to improve urban aids (Q21). Furthermore, the statistical associations studied showed that the loss of vision plays a significant role in influencing the perception of one's QoL (association of questions 7 and 8, X2 = 112.119, Cramer's V = 0.548, p-value <0.001). In addition, it is more difficult for visually impaired patients living outside the city to move outdoors (Chi-Square = 10.637, Cramer's V = 0.326, p - 245 value = 0.031) and to cross the street (Chi-Square = 14.102, Cramer's V = 0.376, p-250 value = 0.007). Finally, those who feel independent perceive their lives to be more fulfilling (Chi-Square = 268, X2 = 37.433; Cramer's V = 0.306, p value = 0.002). Conclusion: Our study showed how vision loss plays a remarkable role in influencing the perception of one's QoL. Furthermore, it highlighted how the implementation of mobility and the use of personal aids for living in a city, such as Turin, were associated with a better perception of QoL by visually impaired patients. However, it is necessary to improve urban technological development according to the needs of people with visual disability.

Structural OCT and OCT angiography biomarkers associated with the development and progression of geographic atrophy in AMD.

BACKGROUND: Geographic atrophy (GA) is an advanced, irreversible, and progressive form of age-related macular degeneration (AMD). Structural optical coherence tomography (OCT) and OCT angiography (OCTA) have been largely used to characterize this stage of AMD and, more importantly, to define biomarkers associated with the development and progression of GA in AMD. METHODS: Articles pertaining to OCT and OCTA biomarkers related to the development and progression of GA with relevant key words were used to search in PubMed, Researchgate, and Google Scholar. The articles were selected based on their relevance, reliability, publication year, published journal, and accessibility. RESULTS: Previous reports have highlighted various OCT and OCTA biomarkers linked to the onset and advancement of GA. These biomarkers encompass characteristics such as the size, volume, and subtype of drusen, the presence of hyperreflective foci, basal laminar deposits, incomplete retinal pigment epithelium and outer retinal atrophy (iRORA), persistent choroidal hypertransmission defects, and the existence of subretinal drusenoid deposits (also referred to as reticular pseudodrusen). Moreover, biomarkers associated with the progression of GA include thinning of the outer retina, photoreceptor degradation, the distance between retinal pigment epithelium and Bruch's membrane, and choriocapillaris loss. CONCLUSION: The advent of novel treatment strategies for GA underscores the heightened need for prompt diagnosis and precise monitoring of individuals with this condition. The utilization of structural OCT and OCTA becomes essential for identifying distinct biomarkers associated with the initiation and progression of GA.