Browsing by Author "Dias-Santos, A"
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- Alzheimer's Disease: a Review of its Visual System Neuropathology. Optical Coherence Tomography-a Potential Role As a Study Tool in VivoPublication . Cunha, JP; Moura-Coelho, N; Proença, R; Dias-Santos, A; Ferreira, J; Louro, C; Castanheira-Dinis, AAlzheimer's disease (AD) is a prevalent, long-term progressive degenerative disorder with great social impact. It is currently thought that, in addition to neurodegeneration, vascular changes also play a role in the pathophysiology of the disease. Visual symptoms are frequent and are an early clinical manifestation; a number of psychophysiologic changes occur in visual function, including visual field defects, abnormal contrast sensitivity, abnormalities in color vision, depth perception deficits, and motion detection abnormalities. These visual changes were initially believed to be solely due to neurodegeneration in the posterior visual pathway. However, evidence from pathology studies in both animal models of AD and humans has demonstrated that neurodegeneration also takes place in the anterior visual pathway, with involvement of the retinal ganglion cells' (RGCs) dendrites, somata, and axons in the optic nerve. These studies additionally showed that patients with AD have changes in retinal and choroidal microvasculature. Pathology findings have been corroborated in in-vivo assessment of the retina and optic nerve head (ONH), as well as the retinal and choroidal vasculature. Optical coherence tomography (OCT) in particular has shown great utility in the assessment of these changes, and it may become a useful tool for early detection and monitoring disease progression in AD. The authors make a review of the current understanding of retinal and choroidal pathological changes in patients with AD, with particular focus on in-vivo evidence of retinal and choroidal neurodegenerative and microvascular changes using OCT technology.
- Análise Retrospectiva e Caracterização Epidemiológica de 151 Casos de Inflamação OcularPublication . Lisboa, M; Dias-Santos, A; Vieira, L; Rosa, R; Cardoso, M; Domingues, IObjectivo: Analisar e caracterizar uma amostra de doentes de uma consulta de inflamação ocular. Material e Métodos: Análise retrospectiva de 503 consultas realizadas por um clínico entre 1 de Agosto de 2012 e 31 de Agosto de 2013 no Centro Hospitalar de Lisboa Central com recurso aos respectivos processos clínicos. Na análise da casuística da consulta foram incluídos 151 doentes. Desses, 24 padeciam de doenças auto-imunes em seguimento para monitorização de toxicidade a fármacos mas sem registo de qualquer episódio de uveíte, pelo que foram excluídos da avaliação estatística referente às uveítes. Dos 127 doentes com uveíte foram incluídos 197 olhos. Resultados: A média de idades foi de 53,8±16,5 anos, sendo 60% do sexo feminino e 40% masculino. A inflamação foi bilateral em 70 e unilateral em 57 doentes. O tipo de uveíte mais frequente foi a anterior (51,2%), seguida da panuveíte (21,3%), posterior (19,7%), intermédia (3,9%), episclerite (3,2%) e esclerite (0,8%). As etiologias foram agrupadas em: doenças sistémicas (34%), doenças infecciosas (30%), idiopáticas (27%) e patologias oculares específicas (9%). A acuidade visual média nos olhos com uveíte anterior foi 0.8, panuveíte 0.2, uveíte posterior 0.2, uveíte intermédia 0.7, episclerite e esclerite 1.0. Dos 197 olhos com uveíte, 27 (13,7%) foram submetidos a cirurgia de catarata e 5 (2,5%) a cirurgia de glaucoma. Conclusões: Apesar de se tratar de uma amostra relativamente pequena, reveste-se de importância dado ser fundamental conhecer a realidade em cada centro de referência de forma a optimizar os recursos disponíveis e a melhorar a abordagem clínica.
- Choroidal Thickness in Nonarteritic Anterior Ischaemic Optic Neuropathy: A Study with Optical Coherence TomographyPublication . Dias-Santos, A; Ferreira, J; Abegão Pinto, L; Vicente, A; Anjos, R; Cabugueira, A; Flores, R; Cunha, JPNonarteritic anterior ischemic optic neuropathy (NA-AION) is the most common nonglaucomatous optic neuropathy in adults over 50 years of age. It is usually related to cardiovascular risk factors. The primary objective of this study was to evaluate choroidal thickness in patients with chronic NA-AION, and the secondary objective was to evaluate macular thickness in these patients. This cross-sectional study compared two groups: group 1 included 20 eyes of 20 patients with chronic NA-AION, and group 2 included 31 eyes of 31 healthy controls. In both groups, the choroidal thickness was measured using the enhanced depth imaging program of Heidelberg Spectralis® optical coherence tomography (Heidelberg Engineering, Heidelberg, Germany). The macular thickness was also measured using the automatic software of the same device. The mean follow-up time after NA-AION in group 1 was 57.17 ± 26.92 months. The mean choroidal thickness of the posterior pole was 244.38 ± 61.03 µm in group 1 and 214.18 ± 65.97 µm in group 2 (p = 0.004). The mean macular thickness was higher in group 2. Macular thickness is reduced in eyes that had an episode of NA-AION, whereas choroidal thickness is generally higher in these eyes when compared with normal eyes. The increase in choroidal thickness may be due to a local dysfunction in vascular autoregulatory mechanisms, which may predispose to ischemic phenomena.
- Choroidal Thinning: Alzheimer's Disease and AgingPublication . Cunha, JP; Proença, R; Dias-Santos, A; Melancia, D; Almeida, R; Águas, H; Oliveira Santos, B; Alves, M; Ferreira, J; Papoila, AL; Louro, C; Castanheira-Dinis, AINTRODUCTION: The purpose of this study was to measure and to compare macular choroidal thickness (CT) between patients with mild Alzheimer's disease (AD), patients without AD, and elderly patients. METHODS: CT was measured manually in 13 locations at 500-μm intervals of a horizontal and a vertical section from the fovea. Linear regression models were used to analyze the data. RESULTS: Fifty patients with a diagnosis of mild AD (73.1 years), 152 patients without AD (71.03 years), and 50 elderly without AD (82.14 years) were included. In the AD patients, CT was significantly thinner in all 13 locations (P < .001-comparing with age-match group), and comparing with the elderly group, a more pronounced difference was found in two locations temporal to the fovea. DISCUSSION: Patients with AD showed a significant choroidal thinning even when compared with elderly subjects. The reduction of CT may aid in the diagnoses of AD, probably reflecting the importance of vascular factors in their pathogenesis.
- Expression of Focal Adhesion Kinase in RetinoblastomaPublication . Dias-Santos, A; Bravo-Filho, V; Faingold, D; Odashiro, A; Odashiro, P; Jagan, L; Ballazsi, M; Burnier Jr, MNIntrodução: As cinases de adesão focal (CAF) são tirosina-cinases citoplasmáticas, não receto- ras, importantes e no processo oncogénico. O objetivo deste estudo é avaliar o nível de expressão das CAF no retinoblastoma e correlacioná-la com fatores prognósticos histopatológicos. Material e Métodos: a expressão de CAF e da sua forma ativa fosforilada - CAF [pY397] - foi avaliada em vinte espécimes de retinoblastomas por imuno-histoquímica. A imunorreatividade foi correlacionada com a idade, grau de diferenciação do tumor e com a presença ou ausência de invasão da câmara anterior, coroideia, vítreo e nervo ótico. Resultados: A avaliação histopatológica revelou 55% de tumores pouco diferenciados, 30% moderadamente diferenciados e 15% bem diferenciados. A invasão da câmara anterior foi obser- vada em 80% dos casos, da coróide em 35%, do vítreo em 25% e do nervo ótico em 70%. Vinte e cinco por cento dos tumores apresentaram uma forte expressão de CAF [pY397], 60% expressão moderada e 15% expressão fraca. Relativamente à imunorreatividade para CAF não fosforilada, 20% dos tumores revelaram-se fortemente positivos, 75% moderadamente positivos e 5% fraca- mente positivos. Não houve correlação significativa entre a expressão destas moléculas e a idade ou fatores de prognóstico histopatológicos. Conclusão: Este estudo foi o primeiro a avaliar a expressão desta molécula no retinoblastoma. Todos os espécimes apresentaram expressão de CAF ou CAF [pY397], tendo mais de 80% exi- bido expressão moderada ou forte de ambas. Os resultados sugerem uma potencial relação destas moléculas com o processo oncogénico, abrindo perspetivas relativamente a novas estratégias de intervenção terapêutica no retinoblastoma.
- Higher Order Aberrations in Amblyopic Children and their Role in Refractory AmblyopiaPublication . Dias-Santos, A; Rosa, R; Ferreira, J; Cunha, JP; Brito, C; Paixão, A; Toscano, AObjective: Some studies have hypothesized that an unfavourable higher order aberrometric profile could act as an amblyogenic mechanism and may be responsible for some amblyopic cases that are refractory to conventional treatment or cases of “idiopathic” amblyopia. This study compared the aberrometric profile in amblyopic children to that of children with normal visual development and compared the aberrometric profile in corrected amblyopic eyes and refractory amblyopic eyes with that of healthy eyes. Methods: Cross-sectional study with three groups of children – the CA group (22 eyes of 11 children with unilateral corrected amblyopia), the RA group (24 eyes of 13 children with unilateral refractory amblyopia) and the C group (28 eyes of 14 children with normal visual development). Higher order aberrations were evaluated using an OPD-Scan III (NIDEK). Comparisons of the aberrometric profile were made between these groups as well as between the amblyopic and healthy eyes within the CA and RA groups. Results: Higher order aberrations with greater impact in visual quality were not significantly higher in the CA and RA groups when compared with the C group. Moreover, there were no statistically significant differences in the higher order aberrometric profile between the amblyopic and healthy eyes within the CA and RA groups. Conclusions: Contrary to lower order aberrations (e.g., myopia, hyperopia, primary astigmatism), higher order aberrations do not seem to be involved in the etiopathogenesis of amblyopia. Therefore, these are likely not the cause of most cases of refractory amblyopia.
- Macular and Optic Disc Edema and Retinal Vascular Leakage in Familial Amyloid Polyneuropathy with a Transthyretin Val30Met Mutation: a Case ReportPublication . Dias-Santos, A; Ferreira, J; Cunha, JPIntroduction: Familial amyloid polyneuropathy is a group of autosomal dominant disorders characterized by extracellular amyloid deposition in several target organs. This paper aims to report an unusual manifestation of retinal vascular leakage including optic disc and macular edema in a patient with familial amyloid polyneuropathy. Case presentation: A 37-year-old Portuguese Caucasian man with Val30Met transthyretin-related familial amyloid polyneuropathy presented with rapidly progressing visual loss in his left eye. He had undergone liver transplantation at the age of 30 with neurologic stabilization. Fundoscopy and fluorescein angiogram revealed optic disc and macular edema as well as vessel wall staining with leakage in the posterior pole and mid-periphery, without vitreous opacities. A diagnostic work-up for infectious, autoimmune and neoplasic conditions was negative. Systemic immunosuppression was increased but without improvement. Sustained resolution of macular edema was observed after intravitreal injection of dexamethasone implant and laser panretinal photocoagulation. Conclusions: To the best of our knowledge, this is the first report of a rare ocular manifestation of familial amyloid polyneuropathy which represents a new therapeutic challenge. Intravitreal injection of sustained release dexamethasone implant and panretinal photocoagulation may be an effective eye-saving therapeutic approach.
- OCT in Alzheimer's Disease: Thinning of the RNFL and Superior HemiretinaPublication . Cunha, JP; Proença, R; Dias-Santos, A; Almeida, R; Águas, H; Alves, M; Papoila, AL; Louro, C; Castanheira-Dinis, ABACKGROUND: Peripapillary retinal nerve fiber layer (pRNFL) and internal macular layer thinning have been demonstrated in Alzheimer's disease (AD) with optical coherence tomography (OCT) studies. The purpose of this study is to compare the pRNFL thickness and overall retinal thickness (RT) in AD patients with non-AD patients, using spectral domain optical coherence tomography (SD-OCT) and determine the sectors most characteristically affected in AD. METHODS: A cross-sectional study was performed to determine the pRNFL and overall macular RT thicknesses in AD and non-AD patients, attending a tertiary hospital center. For pRNFL, the global and six peripapillary quadrants were calculated, and for overall RT values, the nine Early Treatment Diabetic Retinopathy Study (ETDRS) areas were used. A multiple regression analysis was applied to assess the effects of disease, age, gender, spherical equivalent, visual acuity, intraocular pressure, axial length and blood pressure on pRNFL and overall macular RT. RESULTS: A total of 202 subjects, including 50 eyes of 50 patients with mild AD (mean age 73.10; SD = 5.36 years) and 152 eyes of 152 patients without AD (mean age 71.03; SD = 4.62 years). After Bonferroni correction, the pRNFL was significantly thinner for the AD group globally and in the temporal superior quadrant (10.76 μm and 20.09 μm mean decrease, respectively). The RT thickness was also decreased in superior sectors S3 and S6 (mean thinning of 9.92 μm and 11.65 μm, respectively). Spearman's correlation coefficient showed a direct association between pRNFL in the temporal superior quadrant and RT in superior S6 and S3 sectors (rS = 0.41; p < 0.001 and rS = 0.28; p < 0.001, respectively). CONCLUSIONS: Patients with AD showed a significant thickness reduction in global and temporal superior quadrants in pRNFL and in superior pericentral and peripheral sectors of RT. These findings may reflect a peripapillary and retinal changes characteristic of AD, suggesting the importance of SD-OCT as a potential adjuvant in early diagnosis of AD. Further studies are needed to understand which retinal layers and macular sectors are more useful as potential ocular biomarker over time in AD.
- Ocular Involvement in Systemic Lupus Erythematosus Patients: a Paradigm Shift Based on the Experience of a Tertiary Referral CenterPublication . Dias-Santos, A; Ferreira, J; Pinheiro, S; Cunha, JP; Alves, M; Papoila, AL; Moraes-Fontes, MF; Proença, RObjective: To evaluate ocular involvement in a cohort of systemic lupus erythematosus (SLE) patients of a tertiary referral center and to compare the results with the existing literature. Methods: Patients underwent a complete ophthalmological evaluation, including visual acuity, slit-lamp examination, fluorescein staining, Schirmer-I test, Goldmann applanation tonometry, fundoscopy, 10-2 automated threshold visual fields, fundus autofluorescence and spectral-domain optical coherence tomography to screen for hydroxychloroquine (HCQ) macular toxicity. Results: A total of 161 patients (16 men and 145 women) were enrolled in this study. The mean age was 47.6 years and the mean disease duration was 11.5 years. Fifty patients (31.1%) had at least one ocular manifestation of SLE. The most frequent manifestation was dry eye syndrome (12.4%), immediately followed by cataracts (11.2%) and HCQ macular toxicity (11.2%). Among patients with HCQ maculopathy, two presented with an atypical spectral-domain optical coherence tomography pattern. Five patients (3.1%) presented with glaucoma, two patients (1.2%) presented with SLE retinopathy while only one presented with lupus choroidopathy (0.6%). Conclusions: Compared with previous studies, we conclude there has been a significant reduction in disease-related ocular complications, particularly those associated with poor systemic disease control. On the other hand, drug and age-related complications are assuming a prominent role in the ophthalmic care of these patients.
- Outcomes of Cystoid Macular Edema Following Descemet's Membrane Endothelial Keratoplasty in a Referral Center for Keratoplasty in Spain: Retrospective StudyPublication . Moura-Coelho, N; Papa-Vettorazz, R; Santiesteban-García, I; Dias-Santos, A; Manero, F; Cunha, JP; Güell, JThe aim of this study was to analyze the outcomes of eyes with visually significant cystoid macular œdema (vs-CMO) after Descemet membrane endothelial keratoplasty (DMEK) in a referral center for keratoplasty in Spain. We conducted a retrospective, single-surgeon case series of eyes that developed post-DMEK vs-CMO performed between January 2011 and December 2020. Data collected included: indication for DMEK; biometric data; ocular comorbidities; past medical history; time to detection of vs-CMO after DMEK (T, weeks); best-corrected visual acuity (BCVA, logMAR) and central retinal thickness (CRT, µm) at diagnosis of vs-CMO, after resolution of CMO, and at last follow-up; and management strategy. Main outcomes analyzed were incidence of vs-CMO, improvement in BCVA and CRT after treatment of vs-CMO. Of 291 consecutive DMEK surgeries, 14 eyes of 13 patients (4.8%) developed vs-CMO. Five patients (38.5%) had history of CMO, and 28.6% of eyes had ophthalmic comorbidities. Median (P25-P75) T was 4 (3-10) weeks. Treatment success was observed in 12/13 eyes (92.3%), two of which required second-line treatment. In successful cases (median time-to-resolution 3.0 (2.0-3.5) months), median BCVA improved from 0.60 (0.40-0.80) logMAR to 0.30 (0.15-0.40) logMAR (p = 0.002) after treatment, and median CRT improved from 582.5 (400.0-655.0) µm to 278.0 (258.0-294.0) µm (p = 0.005). In our study, we found a 4.8% rate of post-DMEK vs-CMO, with most cases occurring in the first 3 months after surgery. Good functional and anatomical outcomes are expected in most eyes, without treatment-related complications or implications in graft outcomes. Additional studies are encouraged to determine a standardized protocol for post-DMEK vs-CMO.