Peripapillary and macular morpho-vascular changes in patients with genetic or clinical diagnosis of autosomal dominant optic atrophy: a case-control study.

PURPOSE: To evaluate the macular and peripapillary morpho-vascular changes in ADOA, using optical coherence tomography (OCT) and OCT angiography (OCTA). METHODS: Prospectively defined, cross-sectional case-control study. Consecutive patients with a genetic or clinical diagnosis of ADOA along with age- and sex-matched controls were included. The radial peripapillary capillary (RPC) density and vessel density (VD) in the parafoveal superficial and deep capillary plexuses (SCP and DCP, respectively) were evaluated with OCTA. The ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL) thickness were determined using structural OCT. We applied a previously validated customized macro (Fiji, SciJava Consortium) to compute RPC density. The remaining parameters were calculated by the built-in software. Non-parametric methods were used for data analysis. The target α level was 0.05, which was adjusted through Bonferroni's correction when multiple outcomes were tested. RESULTS: Fifty-eight eyes (n = 29 control; n = 29 ADOA) from 30 subjects (mean age 42.43 ± 15.30 years; 37.93% male) were included. Parafoveal SCP VD, GCC thickness, RPC VD in the temporal quadrant, as well as RNFL thickness in the nasal and temporal quadrants were decreased in ADOA eyes (all p < 0.001). When only patients with genetically confirmed diagnosis were included, capillary dropout in the circumpapillary superior and inferior quadrants also became evident (both p < 0.001). The GCC/parafoveal SCP ratio was increased in ADOA, relatively to matched controls. In contrast, none of the circumpapillary morpho-vascular ratios was significantly different in ADOA eyes. CONCLUSIONS: The microvascular and structural changes found in ADOA suggest that both the macular and peripapillary regions are involved, although the threshold for damage of the structural and vascular components may be different for each region. Larger series with longitudinal follow-up may validate OCTA biomarkers helpful for disease monitoring.

Ranibizumab for the Treatment of Diabetic Macular Oedema in the Real-World Clinical Setting in Portugal: A Multicentre Study.

PURPOSE: The purpose of this study was to evaluate the 2-year outcome of ranibizumab for diabetic macular oedema (DME) in the real-life clinical practice of five ophthalmology departments of the National Health Service (NHS) in Portugal. METHODS: This is a retrospective multicentre study. The clinical records on consecutive patients with DME from clinical practice treated with 0.5 mg intravitreal ranibizumab and followed up for 24 months were reviewed. Efficacy outcomes comprised the change in best corrected visual acuity (BCVA) and central macular thickness (CMT) evaluated by SD-OCT. Multivariate regression analysis was performed to explore predictors of BCVA. RESULTS: A total of 122 eyes of 93 patients were included. The median BCVA change by 24 months was +5.0 letters (IQR 12.0) (p < 0.001) and the CMT change was -89.0 µm (IQR 165.0) (p < 0.001). By 24 months, 21.4% of the eyes had gained ≥15 letters and 8.6% had lost ≥15 letters. The median number of injections given during follow-up was 5.0 (IQR 4.0). A greater baseline CMT and a more disrupted status of the external limiting membrane were predictive of worse BCVA at 24 months (p ≤ 0.015). CONCLUSION: DME treatment with ranibizumab in the Portuguese NHS is associated with anatomic and functional improvement by 2 years; however, our results are below those reported in major clinical trials, and undertreatment is probably the cause.

Incidence of Age-Related Macular Degeneration in the Central Region of Portugal: The Coimbra Eye Study - Report 5.

PURPOSE: To describe the 6.5-year incidence and progression of age-related macular degeneration (AMD) in a coastal town of central Portugal. METHODS: Population-based cohort study. Participants underwent standardized interviews and ophthalmological examination. Color fundus photographs were graded according to the International Classification and Grading System for AMD and ARM. The crude and age-standardized incidence of early and late AMD was calculated, and progression was analyzed. RESULTS: The 6.5-year cumulative incidence of early AMD was 10.7%, and of late AMD it was 0.8%. The incidence of early AMD was 7.2, 13.1 and 17.7% for participants aged 55-64, 65-74 and 75-84 years (p < 0.001). The late AMD incidence was 0.3, 0.9 and 2.8% for the corresponding age groups (p = 0.003). The age-standardized incidence was 10.8% (95% CI, 10.74-10.80%) for early and 1.0% (95% CI, 1.00-1.02%) for late AMD. The incidence of both neovascular AMD and geographic atrophy was 0.4%. Progression occurred in 17.2% of patients. CONCLUSION: The early AMD incidence in a coastal town of central Portugal was found to be similar to that of major epidemiological studies of European-descent populations; however, the incidence of late AMD was lower, and further analysis on risk factors will be conducted.

Multimodal Evaluation of the Fellow Eye of Patients with Retinal Angiomatous Proliferation.

INTRODUCTION: We conducted a multimodal, cross-sectional evaluation. METHODS: Eyes were divided into 4 study groups: controls, early/intermediate age-related macular degeneration (AMD), fellow eyes of retinal angiomatous proliferation (RAP), and RAP eyes. Patients were evaluated with spectral-domain optical coherence tomography (OCT), enhanced depth imaging-OCT, and OCT angiography (OCTA). OCTA images were processed to generate maps of the vessel density and perfusion density of the superficial and deep retinal layers (SRL and DRL) and the choriocapillaris level (CL). The thickness of the outer nuclear layer and choroid was manually assessed. RESULTS: We included 135 eyes of 100 patients (51 controls, 30 AMD, 42 RAP, and 12 fellow eyes). The fellow eyes showed a significantly lower vascular perfusion of the SRL, DRL, and CL (p < 0.02) than the early/intermediate AMD and control eyes did. Similarly, RAP eyes presented a lower vascular perfusion of the DRL and CL (p < 0.05). Besides, structural analyses of the fellow eyes and RAP eyes revealed a significantly higher prevalence of macular pigmentary changes, atrophy of the retinal pigment epithelium, hyperreflective "clumps" above flat drusen, amongst others, than early/intermediate AMD and control eyes (p < 0.05). CONCLUSION: We present the first report on the OCTA analysis of the fellow eye of patients with RAP. The reduced perfusion density and vessel density observed contributes, in association with clearly defined structural changes, to a wider characterization of RAP as a distinctive phenotype.

Effectiveness of Snoezelen in older adults with neurocognitive and other pathologies: A systematic review of the literature.

Snoezelen Multisensory Stimulation (SMSS) is a non-pharmacological intervention that provides controlled multisensorial environments to stimulate the primary senses: sight, hearing, smell, touch, taste, proprioceptive and vestibular. Even though the use and potential of SMSS have been widespread in the literature regarding certain target populations (autism, developmental disabilities) and settings (e.g. leisure, therapeutic), its effectiveness in older adults with neurocognitive disorders (e.g. dementia, mild cognitive impairment) and other pathologies (e.g. psychiatric disorders, oncological diseases) is still unclear. Therefore, a systematic review of the literature was conducted to address this issue. The recommendations of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) and of the Cochrane Collaboration were followed. An initial search on PubMed and Scopus databases resulted in 86 articles of which 14 met inclusion criteria and were reviewed in detail. The outcomes showed that most of the studies (n = 13) focused on the effects of SMSS on behaviour and mood in older adults with major cognitive disorders (i.e. dementia). Although there is scarce literature on its impact on cognition, psychophysiological measures (e.g. heart rate, oxygen saturation), daily living functionality and quality of life, this type of intervention seems to contribute to delaying the worsening in severity of the neurocognitive disorders from the mildest to the most severe stages. Likewise, it is legitimate to consider the possibility of potential benefits to older adults with less severe neurocognitive disorders or other pathologies, but more research is needed.

Macular atrophy development in neovascular age-related macular degeneration during first year of treatment: Incidence and risk factors.

PURPOSE: To assess the development of macular atrophy, according to the new Classification of Atrophy Meetings criteria, in patients with treatment-naïve neovascular age-related macular degeneration during the first year of treatment with ranibizumab or aflibercept, and to determine baseline factors predictive of atrophy development. METHODS: Retrospective subanalysis of three prospective clinical trials that included eyes with treatment-naïve neovascular age-related macular degeneration. Multimodal evaluation was performed with spectral-domain optical coherence tomography, fluorescein angiography, fundus autofluorescence and color fundus photography at baseline and after 12 months of treatment. The main outcome was the macular atrophy type, classified according to Classification of Atrophy Meeting criteria. Logistic regression models were built to test predictors of macular atrophy development. RESULTS: A total of 85 eyes of 85 patients (63% female; mean age: 78.5 ± 6.3 years old) were included. After 12 months of antiangiogenic therapy, all four Classification of Atrophy Meeting types of macular atrophy developed de novo. The atrophy type with highest incidence at end of follow-up was incomplete retinal pigment epithelium and outer retinal atrophy (63.6%; 95% confidence interval: 45.9%-86.0%). A significant association was observed between development at 12 months and the presence of incomplete retinal pigment epithelium and outer retinal atrophy at baseline (odds ratio (95% confidence interval): 22.4 (1.6, 323.5)). The number of injections was predictive of complete outer retinal atrophy development at end of follow-up (odds ratio (95% confidence interval) 1.5 (1.1, 2.1), p = 0.011). CONCLUSION: Predictors of atrophy development have the potential to change treatment practices. Further research is warranted.

Management of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Insights from Multimodal Imaging with OCTA.

A 28-year-old man presented to the emergency room with blurred vision in the right eye for two days. He reported a preceding flu-like illness one week earlier. His best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/25 in the left eye. There was no anterior chamber inflammation or vitritis in either eye. He presented multiple yellowish-white placoid lesions in the posterior pole, some involving the foveal area, bilaterally. General examination and systemic investigation were unremarkable. Multimodal evaluation with fluorescein angiography, indocyanine green angiography, and spectral domain and optical coherence tomography angiography (OCTA) were consistent with the diagnosis of acute posterior multifocal placoid pigment epitheliopathy. Due to centromacular involvement with decreased BCVA, treatment with oral methylprednisolone was started after infectious causes were ruled out. After two weeks, the patient presented functional and anatomical improvement. OCTA showed partial reperfusion of the choriocapillaris in the affected areas, in both eyes.

Age-Related Macular Degeneration Staging by Color Fundus Photography vs. Multimodal Imaging-Epidemiological Implications (The Coimbra Eye Study-Report 6).

Epidemiology of age-related macular degeneration (AMD) is based on staging systems relying on color fundus photography (CFP). We aim to compare AMD staging using CFP to multimodal imaging with optical coherence tomography (OCT), infra-red (IR), and fundus autofluorescence (FAF), in a large cohort from the Epidemiologic AMD Coimbra Eye Study. All imaging exams from the participants of this population-based study were classified by a central reading center. CFP images were graded according to the International Classification and Grading System for AMD and staged with Rotterdam classification. Afterward, CFP images were reviewed with OCT, IR, and FAF and stage update was performed if necessary. Early and late AMD prevalence was compared in a total of 1616 included subjects. In CFP-based grading, the prevalence was 14.11% for early AMD (n = 228) and 1.05% (n = 17) for late AMD, nine cases (0.56%) had neovascular AMD (nAMD) and eight (0.50%) geographic atrophy (GA). Using multimodal grading, the prevalence increased to 14.60% for early AMD (n = 236) and 1.61% (n = 26) for late AMD, with 14 cases (0.87%) of nAMD and 12 (0.74%) of GA. AMD staging was more accurate with the multimodal approach and this was especially relevant for late AMD. We propose that multimodal imaging should be adopted in the future to better estimate and compare epidemiological data in different populations.

Salzmann nodular degeneration features in a case of Kabuki make-up syndrome.

Kabuki syndrome (KS) is a multiple congenital anomaly syndrome with diversified ophthalmological manifestations. We report a case of a boy with bilateral features of Salzmann nodular degeneration (SND) associated with KS. An 18-year-old Caucasian man with KS presented for a second opinion regarding incapacitating photophobia in his right eye, refractory to medical therapy. Biomicroscopy revealed bilateral subepithelial nodules in the midperiphery of the cornea, less extensive in the left eye, consistent with SND. Symptomatic improvement was achieved after superficial keratectomy, manually performed with a blade and adjuvant application of mitomycin C. We report a rare case of a KS patient with SND. Since KS manifestations may vary widely, it is important to perform an early ophthalmological examination for prompt detection and treatment of ocular abnormalities and thus improve life quality in these patients.

Macular OCT-angiography parameters to predict the clinical stage of nonproliferative diabetic retinopathy: an exploratory analysis.

BACKGROUND: To test whether a single or a composite set of macular vascular density parameters, evaluated with optical coherence tomography angiography (OCTA), are able to predict nonproliferative diabetic retinopathy (NPDR) staging according to the gold-standard ETDRS-grading scheme. METHODS: Prospectively defined, cross-sectional study in which macular structural and vascular parameters of diabetic eyes with nonproliferative DR (up to ETDRS Level 53) were evaluated with OCTA (Avanti RTVue-XR 100, Optovue Inc, Fremont, CA). Seven-field photographs of the fundus were taken for DR staging according to the ETDRS-grading scheme. The vessel density in the superficial and deep capillary plexus (SCP and DCP, respectively), as well as in the choriocapillaris (CC), were calculated using automated software. Univariate and multivariate ordered logistic regression models were used in the analysis. P < 0.05 was considered statistically significant. RESULTS: We included 101 eyes from 56 subjects (mean (SD) age 62.64 (11.74) years; 57.4% were male). On univariate analysis, several OCTA parameters were found to be associated with higher ETDRS level (parafoveal SCP density: OR = 0.87 (95% CI 0.76-0.99), p = 0.039; parafoveal DCP density: OR = 0.79 (95% CI 0.72-0.87), p < 0.001; CC density: OR = 0.89 (95% CI 0.80-0.99)), p = 0.036). In the final model, while also adjusting for relevant clinical features, only parafoveal vessel density in the DCP remained as a significant predictor of NPDR ETDRS level (OR = 0.54 (95% CI 0.32-0.92), p = 0.024). CONCLUSION: Our results suggest that parafoveal vessel density in the DCP is the parameter most robustly associated with ETDRS level. OCTA analysis may provide objective imaging biomarkers to monitor NPDR clinical progression.

Radius-Maumenee syndrome: a rare cause of glaucoma.

Case presentation of a 41-year-old woman with long-standing bilateral eye injection whose clinical findings included bilateral episcleral vessel engorgement and tortuosity, raised intraocular pressure with open iridocorneal angles and left optic disc cupping. All remaining objective examination was normal. She underwent cranial and orbits CT and CT-angiography scans which were unremarkable. Optical coherence tomography of the peripapillary nerve fibre layer and automated perimetry were compatible with advanced glaucomatous damage on the left eye. The patient was diagnosed with idiopathic elevated episcleral venous pressure or Radius-Maumenee syndrome. If medical therapy is unable to prevent disease progression, left eye filtering surgery will be proposed.

Intervención Snoezelen en personas mayores institucionalizadas: bienestar y utilidad instántanea y recordada / Snoezelen intervention in institutionalized elderly people: Well-being and instant and remembered utilities / Intervenção Snoezelen em pessoas idosas institucionalizadas: bem-estar e utilidades instantâneas e recordadas

El presente estudio trata de evaluar el impacto de la intervención Snoezelen en el bienestar de personas mayores institucionalizadas (n=20) en residencias de larga estancia en diversos periodos, durante sesiones y fuera de sesiones, a lo largo de 6 semanas. Utilizando un diseño de reversión para establecer una conexión causal entre Snoezelen y sus efectos en las variables dependientes. Se ha verificado una reducción de las oscilaciones en el bienestar en las semanas en que disfrutaron de Snoezelen, comparativamente a las semanas en las que no lo disfrutaron.

The present study aimed to evaluate the impact of Snoezelen on the well-being of institutionalized elderly people (n = 20) in long-term residences, at various periods, during the session and outside the session, over 6 weeks. A reversal design was used to establish a causal connection between Snoezelen and its effects on the dependent variables. There has been a reduction in fluctuations in their well-being during the weeks in which they enjoyed Snoezelen, compared to the weeks when they did not enjoy it.

O presente estudo procurou avaliar o impacto do Snoezelen no bem-estar das pessoas idosas institucionalizadas (n=20) em residências de longa permanência em diversos períodos, durante a sessão e fora da sessão, ao longo de 6 semanas. Utilizou-se um desenho de reversão para estabelecer uma conexão causal entre o Snoezelen e os seus efeitos nas variáveis dependentes. Verificou-se uma redução das oscilações no seu bem-estar nas semanas em que usufruíram de Snoezelen, comparativamente às semanas em que não o usufruíram.