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ABSTRACT A young woman presented at our clinic with sudden visual loss in the right eye, recurrent vertigo, and right-sided tinnitus. We performed a complete ophthalmological evaluation. This revealed effects of the condition on the small arterioles of the peripheral retina. Susac syndrome is characterized by the clinical triad of retinal arteriolar occlusions, cochleovestibular manifestations, and encephalopathy (which can be identified by neuroimaging abnormalities). Early diagnosis and immunosuppressive therapy improved the patient's visual acuity and the remission of her other symptoms. Hemi-central retinal artery occlusion is an atypical neuro-ophthalmological finding in this disease. However, its identification as a sign of Susac syndrome may facilitate timely diagnosis and accurate treatment.

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Introducción: Se reconoce la asociación entre los factores de riesgo aterogénico y las alteraciones microvasculares de la retina, pero no hay consenso sobre si estas afectaciones en la retina preceden o son una respuesta fisiopatológica a dichos factores. Objetivo: Determinar si la presencia de los factores de riesgo aterogénico predice las alteraciones vasculares retinianas, a través del fondo de ojo y la retinografía. Métodos: Estudio trasversal en 55 sujetos mayores de 19 años de edad, de cualquier sexo, sin opacidades en los medios transparentes del ojo. Se estudiaron las variables edad, sexo, dislipidemia, hábito de fumar, consumo de alcohol, hipertensión arterial, diabetes mellitus tipo 2, presión arterial sistólica y diastólica, índice de masa corporal, colesterol, glicemia, triglicéridos, creatinina, lipoproteínas de alta densidad, urea, eritrosedimentación y conteo leucocitario. Resultados: El 65,45 % presentó alteraciones en el fondo de ojo: aumento del brillo arteriolar (53,03 %) y disminución del calibre arteriolar generalizado (52,24 %). La retinografía mostró daño en el 58,18 %: rectificación de los cruces arteriovenosos (65,71 %), tortuosidad venosa (28,21 %) y cruces arteriovenosos con aplastamiento (85,71 %). El aumento del colesterol sérico (p= 0,003) se asoció con la presión arterial sistólica (p= 0,037) en el fondo de ojo, y con el antecedente de hipertensión arterial (p= 0,023) en la retinografía. Conclusiones: El colesterol sérico, las cifras elevadas de tensión arterial sistólica y antecedentes de hipertensión arterial son los factores de riesgo que mejor predicen el daño vascular retinal.

Introduction: The association between atherogenic risk factors and retinal microvascular alterations is recognized, but there is no consensus on whether these retinal disorders precede or are a pathophysiological response to these factors. Objective: To determine if the presence of atherogenic risk factors predicts retinal vascular alterations, through fundus examination and retinography. Methods: Cross-sectional study in 55 subjects over 19 years of age, of either sex, without opacities in the transparent media of the eye. The variables studied were age, sex, dyslipidemia, smoking habit, alcohol consumption, arterial hypertension, type 2 diabetes mellitus, systolic and diastolic blood pressure, body mass index, cholesterol, glycemia, triglycerides, creatinine, high-density lipoproteins, urea, erythrocyte sedimentation rate and leukocyte count. Results: 65.45% presented alterations in the fundus of the eye: increased arteriolar brightness (53.03%) and decreased generalized arteriolar caliber (52.24%). Retinography showed damage in 58.18%: rectification of arteriovenous crossings (65.71%), venous tortuosity (28.21%), and arteriovenous crossings with crushing (85.71%). The increase in serum cholesterol (p= 0.003) was associated with systolic blood pressure (p= 0.037) in the fundus, and with a history of arterial hypertension (p= 0.023) in retinography. Conclusions: Serum cholesterol, high systolic blood pressure and a history of hypertension are the risk factors that best predict retinal vascular damage.

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ABSTRACT This case series describes four patients who presented with retinal and optic nerve vascular occlusions after administration of different COVID-19 vaccines. The first patient received the ChAdOx1 nCoV-19 vaccine (AZD1222; Oxford/AstraZeneca) and 42 days later developed central retinal artery occlusion. The second patient developed a painless visual impairment in the left eye and was diagnosed with anterior ischemic optic neuropathy 6 days after receiving the Sinovac-CoronaVac vaccine. The third patient presented with the same condition 22 days after receiving the third dose of the COVID-19 Pfizer (Comirnaty®) vaccine. The fourth patient developed bilateral retrobulbar optic neuritis after receiving the Oxford/AstraZeneca vaccine. The purpose of this case series is to discuss the possibility of a causal association between ischemic eye alterations and COVID-19 virus vaccination. Long-term follow-up and evaluation of similar cases will help elucidate the degree of the association between the vaccine and ischemic ocular events.

RESUMO Esta série de casos descreve quatro casos de pacientes que apresentaram oclusões vasculares de retina e nervo óptico após a administração de tipos diferentes de vacinas contra COVID-19. O primeiro paciente tomou a vacina ChAdOx1 nCoV-19 (AZD1222; Oxford/AstraZeneca) e 42 dias depois desenvolveu oclusão da artéria central da retina. O segundo paciente teve défice visual indolor no olho esquerdo após 6 dias da vacina Sinovac (CoronaVac) e foi diagnosticado com neuropatia óptica isquêmica anterior. O terceiro paciente apresentou o mesmo quadro após 22 dias da terceira dose da vacina COVID-19 Pfizer (Comirnaty®). O quarto paciente desenvolveu neurite óptica retrobulbar bilateral após vacina Oxford/AstraZeneca. O objetivo da nossa série de casos é discutir a possibilidade de correlação entre os quadros oculares isquêmicos e a vacinação contra a COVID-19. Nossos pacientes receberam vacinas contra COVID-19 com tecnologias diferentes e apresentaram quadros isquêmicos oculares relacionados temporalmente à vacinação. O acompanhamento e a avaliação a longo prazo de novos estudos semelhantes elucidarão o grau de associação entre a vacina e esse possível evento adverso.

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RESUMO Com o crescimento da indústria cosmética global, a busca pela estética e pelo rejuvenescimento impulsionou o aumento de procedimentos estéticos. A gordura autóloga é o tipo de preenchimento mais comum e mais grave relacionado à cegueira iatrogênica. A oclusão iatrogênica da artéria oftálmica é uma complicação rara (mas devastadora) das injeções de preenchimento facial. Embora diversos tratamentos tenham sido relatados e propostos, até o momento não há eficácia comprovada. Na identificação da oclusão de artéria central da retina, o tempo é de fundamental importância para o prognóstico visual do paciente. Os resultados, em geral, são insatisfatórios, e há pouca ou nenhuma evidência favorável. O objetivo deste trabalho foi relatar o caso de uma paciente que apresentou oclusão de artéria central da retina após procedimento cosmético com gordura autóloga em região nasolabial.

ABSTRACT The global cosmetic industry growth and the desire for aesthetics and rejuvenation have increased the aesthetic procedures. Autologous fat is the most common and most serious type of filling related to iatrogenic blindness. Iatrogenic occlusion of the ophthalmic artery is a rare but devastating complication of facial filler injections. Although several treatments have been reported and proposed, to date there is no proven efficacy. In identifying central retinal artery occlusion, timing is crucial to the patient's visual prognosis. The results, in general, are unsatisfactory and there is little or no favorable evidence. This paper aimed to report the case of a patient who presented central retinal artery occlusion after a cosmetic procedure with autologous fat in the nasolabial region.

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ABSTRACT Antiphospholipid syndrome is an acquired autoimmune disease characterized by hypercoagulability associated with recurrent venous and arterial thromboembolism in the presence of antiphospholipid antibodies. Herein, we report a case of rapid sequential retinal vein and artery occlusion as the first manifestation of a primary antiphospholipid syndrome triggered by an acute Mycoplasma infection in a previously healthy 11-year-old patient. On day 1, ophthalmoscopy revealed a central retinal vein occlusion. The patient developed temporal branch retinal artery occlusion the next day. On day 3, a central retinal artery occlusion was observed. Serum lupus anticoagulant, immunoglobulin (Ig) G anticardiolipin, IgG anti-β2-glycoprotein 1 antibody, and Mycoplasma pneumoniae IgM antibody levels were increased. Thus, retinal vascular occlusions can be the first manifestation of primary antiphospholipid syndrome. Although it may not improve visual prognosis, prompt diagnosis and treatment are essential to avoid further significant morbidity.

RESUMO A síndrome antifosfolipide é uma doença autoimune adquirida caracterizada por hipercoagulabilidade associada a tromboembolismo venoso e arterial recorrente na presença de anticorpos antifosfolipídicos. Aqui, relatamos um caso clínico de oclusão sequencial de veia e artéria da retina como primeira manifestação de uma síndrome antifosfolipíde primária desen­cadeada por uma infeção aguda por Mycoplasma num paciente de 11 anos previamente saudável. No primeiro dia, a oftalmoscopia revelou uma oclusão da veia central da retina. No dia seguinte, o paciente desenvolveu uma oclusão do ramo temporal da artéria central da retina. No terceiro dia, uma oclusão da artéria central da retina foi diagnosticada. Os níveis de anticoagulante lúpico sérico, anticorpos IgG anticardiolipina e IgG anti-β2-glicoproteína 1 e anticorpos IgM para Mycoplasma pneumoniae estavam aumentados. As oclusões vasculares retinianas podem ser a primeira manifestação da síndrome antifosfolipíde primária. Apesar do prognóstico visual ser reservado, o seu diagnóstico e o tratamento imediatos são essenciais para evitar outras morbilidades associadas.

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ABSTRACT A 51-year-old non-obese woman presented with a one-week history of progressive blurry vision within the inferior visual field of her left eye. Her only relevant past medical history was long-standing hypothyroidism and recent vaccination against Coronavirus Disease 2019 (COVID-19) with an mRNA vaccine 12 days before the onset of symptoms. At examination, the anterior segment was unremarkable, but the retinal fundus revealed a central retinal vein occlusion associated with a branch retinal artery occlusion of the superior temporal branch in her left eye. Ancillary tests to rule out thrombophilia, hyperviscosity, hypercoagulability, or inflammation were negative. Ultrasound tests were also negative for a cardiac or carotid origin of the branch retinal artery occlusion. At two-month follow-up, no new retinal vascular occlusive events were observed. Although the best-corrected visual acuity at presentation was 8/10 in the left eye, the final best-corrected visual acuity remained 3/10.

RESUMO Uma mulher de 51 anos, não obesa, apresentou história de uma semana de visão embaçada progressiva no campo visual inferior do olho esquerdo. Seu único histórico médico anterior relevante era hipotireoidismo de longa data e uma recente vacinação contra a Doença de Coronavírus 2019 (COVID-19), com vacina de mRNA, 12 dias antes do início dos sintomas. O exame mostrou segmento anterior normal, mas o fundo da retina revelou uma oclusão da veia central da retina associada a uma oclusão de ramo arterial da retina do ramo temporal superior no olho esquerdo. Testes auxiliares para descartar trombofilia, hiperviscosidade, hipercoagulabilidade ou inflamação apresentaram resultados negativos. Testes de ultrassom também foram negativos quanto a uma origem cardíaca ou da carótida da oclusão do ramo da artéria da retina. Após dois meses de acompanhamento, nenhum novo evento vascular oclusivo retiniano foi observado. Embora, a acuidade visual melhor corrigida na apresentação tenha sido de 8/10 no olho esquerdo, a acuidade visual final melhor corrigida permaneceu em 3/10.

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INTRODUCTION: This study aimed to determine whether there was any correlation between coronary artery disease (CAD) and retinal artery diameter at an academic tertiary medical center in Trinidad and Tobago. METHODS: This prospective study evaluated patients (n = 77) with recent invasive coronary angiography (CAG) and the Synergy between Percutaneous Coronary Intervention with Taxus and Cardiac Surgery (SYNTAX) score who subsequently underwent optical coherence tomography-angiography (OCT-A) at the Eric Williams Medical Sciences Complex (EWMSC) from January 2021 to March 2021. Routine medical history and cardiovascular medications were also recorded. Spearman's rank correlation coefficient and Mann-Whitney U-tests were used to compare correlations and medians between groups. RESULTS: The average patient age was 57.8 years old, with the majority being male [n = 55 (71.4%)] and of South Asian ethnicity [n = 53 (68.8%)]. Retinal artery diameter was negatively correlated with the SYNTAX score (-0.332 for the right eye, p = 0.003 and -0.237 for the left eye, p = 0.038). A statistically significant relationship was also demonstrated in females and diabetic patients. There were no serious adverse events (SAEs). CONCLUSION: A significantly negative correlation was observed between retinal artery diameter and SYNTAX score. This study alludes to the practical use of optical coherence tomography-angiography (OCT-A) as a noninvasive diagnostic modality for patients with cardiovascular disease (CVD). Further large-scale, multicentric studies are required to confirm these exploratory findings. TRIAL REGISTRATION NUMBER: NCT04233619.

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We describe a 68-year-old female patient with unilateral central retinal artery occlusion (CRAO) with cilioretinal artery sparing post-coronavirus disease 2019 (COVID-19) infection. The patient presented with acute vision loss in the left eye 11 days after discharge from a severe COVID-19 infection, with altered D-dimer, C-reactive protein, and fibrinogen levels. The best-corrected visual acuity (BCVA) in the left eye was 20/400; fundoscopic examination revealed diffuse pallor retina with a patent arterial branch from the optic disk to the fovea, confirmed by fluorescein angiography. CRAO with a cilioretinal artery sparing post-COVID-19 may be considered an additional ocular manifestation of the post-acute COVID-19 syndrome spectrum.

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This case report is a multimodal analysis of a pregnant patient with branch retinal artery occlusion (BRAO) associated to patent foramen ovale (PFO). A 28-year-old woman presented at the clinic 20 h after an acute, painless black spot appearance in the inferior temporal visual field of the right eye (OD). At that time, she was 18 weeks pregnant and had no report of complications in her previous pregnancy. Best-corrected visual acuity was 1.0 in both eyes. Color fundus photo, perimetry, and OCT angiography were required. The results clearly showed an embolus in the superior nasal retinal arteriole, associated with a pallor in the distal retina. Patient was referred to a cardiologist and a transcranial Doppler with contrast indicated a right-to-left intracardiac shunt, confirmed by the presence of a PFO at the transesophageal echocardiography. Thrombophilic conditions were excluded. Enoxaparin 1 mg/kg was started and kept until the delivery. Now, a PFO surgical closure is on schedule. This case highlights the noteworthiness of considering PFO as a source of embolism for BRAO in young patients, the capability of OCTA as a dye-free method for use in pregnancy and emphasizes the importance of systemic evaluation in patients with BRAO.

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ABSTRACT Purpose: The study aimed to investigate the correlation between arterial hemodynamics measured by color Doppler ultrasonography and retinal microarchitecture parameters determined by spectral-domain optical coherence tomography (SD-OCT) in pseudoexfoliation glaucoma. Methods: This prospective study included 82 participants. Peripapillary retinal nerve fiber layer, ganglion cell inner plexiform layer, and ganglion cell complex values were measured. Ophthalmic artery and central retinal artery flows were evaluated with color Doppler ultrasonography, and resistivity index values were calculated. Results: The study included 47 controls and 35 pseudoexfoliation glaucoma cases. In pseudoexfoliation glaucoma group, mean peripapillary retinal nerve fiber layer and ganglion cell complex thickness were statistically significantly lower in all quadrants compared to controls (p<0.001). Resistivity index values of the ophthalmic and central retinal arteries were significantly higher in pseudoexfoliation glaucoma group than in the controls (p<0.001 and r=0.684). Resistivity index values of the ophthalmic and central retinal arteries with ganglion cell complex thickness correlated significantly. On the other hand, no significant relationship for retinal nerve fiber layer thickness was identified. Conclusions: Structural changes (ganglion cell complex and ganglion cell inner plexiform layer) in patients with pseudoexfoliation glaucoma and early glaucomatous loss showed a significant correlation with changes in ocular vascular hemodynamics. In cases where systemic vascular resistance is increased, ganglion cell complex and ganglion cell inner plexiform layer may not exactly reflect glaucoma state. In such cases, thickness changes in the retinal nerve fiber layer may give more realistic results regarding glaucoma. We have seen that pseudoexfoliation glaucoma-induced structural deterioration and increased resistance in ocular hemodynamics correlated with ganglion cell complex, but not retinal nerve fiber layer.

RESUMO Objetivo: Investigar a correlação entre a hemodi­nâmica arterial, medida pela ultrassonografia com Doppler colorido, e os parâmetros de microarquitetura da retina, determinados pela tomografia de coerência óptica de domínio espectral (SD-OCT) no glaucoma pseudoexfoliativo. Métodos: Foram incluídos 82 participantes neste estudo prospectivo. Foram medidos os valores da camada de fibras nervosas da retina peripapilar, da camada plexiforme interna de células ganglionares e do complexo de células ganglionares. Os fluxos da artéria oftálmica e da artéria central da retina foram avaliados com ultrassonografia por Doppler colorida e foram calculados os valores do índice de resistividade. Resultados: Foram incluídos no estudo 47 casos de controle e 35 casos de glaucoma pseudoexfoliativo. No grupo com glaucoma pseudoexfoliativo, a média da camada de fibras nervosas da retina peripapilar e a espessura do complexo de células ganglionares foram menores em todos os quadrantes em comparação com os controles, com significância estatística (p<0,001). Os valores do índice de resistividade das artérias oftálmica e central da retina foram significativamente maiores no grupo com glaucoma pseudoexfoliativo que nos controles (p<0,001 e r=0,684). Ao se compararem os valores do índice de resistividade das artérias oftálmica e central da retina com a espessura do complexo de células ganglionares, foi encontrada uma correlação significativa entre elas. Por outro lado, não detectamos uma relação significativa para a espessura da camada de fibras nervosas da retina. Conclusões: Alterações estruturais (complexo de células ganglionares, camada plexiforme interna de células ganglionares) em pacientes com glaucoma pseudoexfoliativo com perda glaucomatosa precoce mostraram uma correlação significativa com alterações na hemodinâmica vascular ocular. Nos casos em que a resistência vascular sistêmica é aumentada, o complexo de células ganglionares e a camada plexiforme interna de células ganglionares podem não refletir exatamente o estado do glaucoma. Nesses casos, alterações na espessura da camada de fibras nervosas da retina podem dar resultados mais realistas em relação ao glaucoma. Observou-se uma correlação da deterioração estrutural induzida pelo glaucoma pseudoexfoliativo e do aumento da resistência na hemodinâmica ocular com o complexo de células ganglionares, mas não com a camada de fibras nervosas da retina.

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Introducción: El uso del medicamento ranibizumab intravítreo favorece la reducción del edema macular generador de las oclusiones vasculares retinianas causantes de la pérdida visual. Objetivo: Evaluar la eficacia y seguridad de la administración intravítreo de ranibizumab en el cambio de espesor macular central en las oclusiones vasculares retinianas analizado mediante tomografía de coherencia óptica. Material y métodos: Se desarrolló un estudio de tipo retrospectivo, analítico, correlacional y observacional de campo con diseño no experimental en 125 pacientes mayores de 30 años con oclusión vascular retiniana diagnosticados en la consulta de oftalmología del Hospital "Teodoro Maldonado Carbó" durante enero de 2017 a junio de 2018. La técnica ANOVA compara las medias para determinar mediante el proceso de contraste de hipótesis si existen diferencias estadísticamente significativas entre estas. Resultados: El análisis de la agudeza visual con escala logMAR demostró diferencias estadísticamente significativas entre los promedios obtenidos 3 meses antes y después de la aplicación del tratamiento (p=0,0001). Se encontró 28,8 por ciento de efectos adversos. Con frecuencia en aumento de presión intraocular (4 por ciento), sequedad ocular (16 por ciento) y hemorragia conjuntival (11,2 por ciento). Conclusiones: El ranibizumab en oclusiones vasculares retinianas proporciona una mejor agudeza visual corregida en relación con el grosor macular, favorece el desarrollo de nuevos vasos sanguíneos a partir de vasos preexistentes desde migración de células endoteliales(AU)

Introduction: The use of the intravitreal ranibizumab favors the reduction of the macular edema that generates retinal vascular occlusions that cause visual loss. Objective: To evaluate the efficacy and safety of the intravitreal administration of ranibizumab in the change in central macular thickness in retinal vascular occlusions analyzed by optical coherence tomography. Material and methods: A retrospective, analytical, correlational and observational field study with a non-experimental design was carried out on 125 patients over 30 years of age diagnosed with retinal vascular occlusion in the Ophthalmology Service of "Teodoro Maldonado Carbó" Hospital during the period between January 2017 and June 2018. The ANOVA technique was used to compare means in order to determine, through the hypothesis contrast process, if there are statistically significant differences between them. Results: Visual acuity analysis using the logMAR scale showed statistically significant differences between the averages obtained 3 months before and after the application of the treatment (p =0.0001). In addition, 28,8 percent of adverse effects were found. The most frequent ones included increased intraocular pressure (4 percent), dry eyes (16 percent), and conjunctival hemorrhage (11,2 percent). Conclusions: In retinal vascular occlusions, Ranibizumab provides a better corrected visual acuity in relation to macular thickness, favors the development of new blood vessels from pre-existing vessels from endothelial cell migration(AU)

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PURPOSE: Report the case of a patient with a history of central retinal artery occlusion in her right eye and amaurosis fugax associated with acute ischemic changes in her left eye related to a prothrombin G20210A gene variant, in which OCT-A was used as a diagnostic and therapeutic tool. CASE PRESENTATION: 55-year-old woman with a history of central retinal artery occlusion in her right eye and prothrombin gene G20210A (F2) variant diagnosis. She presented to our consultation with amaurosis fugax in her left eye. As medical history, she had an episode of bilateral posterior scleritis diagnosed asynchronously with the current episode. Vascular, autoimmune, and metabolic prothrombotic diseases were ruled out. OCT-A showed areas suggesting acute ischemia consistent with macular retinopathy in her left eye. Anticoagulant therapy with Apixaban was initiated, considering the risk for her vision. Control OCT-A showed perfusion improvement in the previous site of the occlusive vascular event. We also considered the extent of the inflammatory response due to posterior scleritis as a differential diagnosis. Nevertheless, it is less likely, considering the temporality between scleritis and the retinal-vascular episodes. CONCLUSIONS: While the G20210A prothrombin gene (F2) variant is a rare cause of retinal artery occlusion, it is important to consider it a differential diagnosis. Good visual outcomes can be achieved with prompt initiation of antithrombotic treatment. In addition, OCT-A is useful for diagnosing ischemic retinal changes that cannot be observed with other diagnostic methods and monitoring them.

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ABSTRACT Dermal filler injection is among facial rejuvenation treatments that have been increasingly used. Despite being a minimally invasive procedure, it can lead to severe complications such as blindness. A review of all cases of filler- -induced visual loss in the world literature was conducted to summarize the mechanisms, anatomical considerations, and clinical ophthalmologic course, current strategies of prevention and management, and trends over the years. We identified 233 cases of filler-induced visual loss, and 172 patients had a severe visual impairment in at least one eye. The typical patients are young women who received injections of hyaluronic acid or autologous fat in the glabella or nose, and the typical presentations were sudden ocular pain, ptosis, and ophthalmoplegia due to vascular occlusion. The findings of this study also suggest an increase in the number of unlicensed professionals performing the procedure. Even though the continued development of dermal fillers has improved the treatment options available, further studies and strategies are necessary to reduce the incidence and minimize the consequences of filler-induced visual loss.

RESUMO O uso de preenchedores dérmicos é uma prática bem estabelecida de rejuvenescimento facial. Embora seja um procedimento minimamente invasivo, pode levar a complicações graves como cegueira. Uma revisão de casos de perda visual pós preenchimento facial estético foi conduzida para descrever os mecanismos, considerações anatômicas, quadro oftalmológico, atuais estratégias de prevenção e manejo, e tendências ao longo dos anos. Foram identificados 233 casos, e 172 pacientes tiveram ao menos um olho com baixa visão ao final do seguimento. O paciente típico é uma mulher jovem submetida a preenchimento de ácido hialurônico na glabela ou nariz, apresentando dor ocular súbita, ptose e oftalmoplegia devido à oclusão vascular. Este estudo também destaca um possível aumento de profissionais não habilitados realizando este procedimento. Apesar do contínuo desenvolvimento dos preenchedores dérmicos e aprimoramento das opções de tratamento disponíveis, mais estudos e estratégias são necessários para reduzir a incidência de complicações e minimizar suas consequências.

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Susac syndrome is a disorder thought to be mediated by an autoimmune response towards endothelial cells, leading to a characteristic clinical triad of encephalopathy, visual disturbances due to branch arterial occlusions and sensorineural hearing impairment. Although it is a rare disease, three reasons make it important. First, given its variable presentation, Susac syndrome is underdiagnosed. Second, it is considered an important differential diagnosis in different neurological, psychiatric, ophthalmological and hearing disorders, and consequently is frequently misdiagnosed. Third, in many cases, Susac syndrome is diagnosed and treated late, with significant irreversible sequelae including dementia, blindness and hearing loss. Neuropathology findings derived from both Susac syndrome patient tissue and novel transgenic mouse models indicate cytotoxic CD8+ T cells adhere to microvessels, inducing endothelial cell swelling, vascular narrowing and occlusion, causing microinfarcts. Anti-endothelial cell antibodies are present in serum in 25% of Susac syndrome patients, but it is unclear whether they are aetiologically related to the disease, or an epiphenomenon. The clinical triad comprising encephalopathy, branch arterial occlusions, and sensorineural hearing impairment is considered pathognomonic, although great variability is found in presentation and natural course of disease. At first evaluation, only 13-30% of patients exhibit the full clinical triad, making diagnosis difficult. Retinal fluorescein angiography, optic coherence tomography, MRI and tonal audiometry are helpful methods for diagnosing and monitoring disease activity during treatment. By contrast, there are no reliable objective immune markers to monitor disease activity. Immunosuppression is the current treatment, with high-dose corticosteroid therapy as the mainstay, but additional therapies such as intravenous immunoglobulins, cyclophosphamide, rituximab and mycophenolate mofetil are often necessary, because the disease can be devastating, causing irreversible organ damage. Unfortunately, low rates of disease, variability in presentation and paucity of objective biomarkers make prospective controlled clinical trials for Susac syndrome treatment difficult. Current immunosuppressive treatments are therefore based on empirical evidence, mainly from retrospective case series and expert opinion. In this review, we draw attention to the need to take consider Susac syndrome in the differential diagnosis of different neurological, psychiatric, ophthalmological and hearing disorders. Furthermore, we summarize our current knowledge of this syndrome, in reference to its pathophysiology, diagnosis and management, emphasizing the need for prospective and controlled studies that allow a better therapeutic approach.

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ABSTRACT Ophthalmologic complications of nonocular surgeries are rare events, but can lead to irreversible conditions of low visual acuity. They are often associated with spine, heart and neck surgery, however they can occur after procedures on other systems. The main local causes are ischemic optic neuropathies, vascular occlusions, cortical lesions, and acute angle-closure glaucoma. We report two cases of sudden low visual acuity secondary to vascular occlusions after gastrointestinal procedures. In the first case, a 57-year-old patient electively admitted for colon reconstruction after Hartmann's colostomy, progressed with intra- and postoperative complications and required subsequent complementary surgeries. Once month later he presented with sudden bilateral low visual acuity, painless and non-altitudinal, and was diagnosed as papillophlebitis, which resolved spontaneously with the progression of the condition. The second case, a 69-year-old patient with no comorbidities underwent rectal resection due to suspected malignant tumor, and progressed on the third postoperative day, with pain and bilateral low visual acuity secondary to acute angle-closure glaucoma, and branch retinal artery occlusion in right eye; treated with iridotomy and ocular hypotensive eye drops, with only slight recovery of vision. The article aims to discuss the etiological mechanisms of the reported conditions and present a literature review.

RESUMO Complicações oftalmológicas de cirurgias não oculares são raras, mas podem levar a condições irreversíveis de baixa acuidade visual. Em geral são associadas à cirurgia de coluna, coração ou pescoço, mas podem ocorrer após procedimentos em outros sistemas. As principais causas são neuropatias ópticas isquêmicas, oclusões vasculares, lesões corticais, e glaucoma agudo de ângulo fechado. Relatamos dois casos de baixa acuidade visual súbita, secundária a oclusões vasculares, após procedimentos cirúrgicos gastrointestinais. No primeiro caso, um paciente de 57 anos foi internado de forma eletiva para reconstrução do cólon após colostomia de Hartmann. Evoluiu com complicações nos períodos intra- e pós-operatório, e necessitou de outras cirurgias complementares. Um mês depois apresentou baixa acuidade visual bilateral súbita, indolor e não altitudinal, e foi diagnosticado como papiloflebite, com resolução espontânea na evolução. O segundo caso, uma paciente de 69 anos, sem comorbidades, foi submetida à ressecção do reto por suspeita de tumor maligno e, no terceiro dia de pós-operatório, evoluiu com dor e baixa acuidade visual bilateral, secundária a glaucoma agudo de ângulo fechado, e oclusão de ramo da artéria retiniana no olho direito; tratada com iridotomia e colírios hipotensores, com recuperação parcial da visão. O objetivo do artigo é discutir os mecanismos etiológicos das doenças relatadas, e apresentar uma revisão da literatura.

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ABSTRACT Patent foramen ovale might cause cryptogenic strokes, including retinal artery occlusion. Herein, we describe a previously healthy young man who presented with central retinal artery occlusion in the setting of patent foramen ovale and explore the need for transesophageal echocardiogram for its diagnosis. Cardiovascular workup and neuroimaging were unremarkable. Transthoracic echocardiogram bubble study revealed a right to left atrial shunt and subsequent transesophageal echocardiogram disclosed patent foramen ovale. This congenital cardiac anomaly was the likely conduit for a thrombo-embolic central retinal artery occlusion. We identified seven patients with patent foramen ovale associated with central retinal artery occlusion in the literature. Transthoracic echocardiogram was diagnostic in only one patient (14.3%), whereas transesophageal echocardiogram was required to reveal patent foramen ovale in the remaining six (85.7%). Our case and the previous reports support the link between central retinal artery occlusion and patent foramen ovale. Therefore, providers should consider the more sensitive transesophageal echocardiogram during the initial evaluation of young patients without immediately identifiable causes of retinal artery occlusion.

RESUMO O forame oval patente pode estar associado a derrames criptogênicos que incluem a oclusão da artéria retiniana. Descrevemos aqui um jovem previamente saudável que apresentou oclusão da artéria central da retina associada ao forame oval patente, sendo considerado portanto, a necessidade de um ecocardiograma transesofágico para seu diagnóstico. A avaliação cardiovascular e a neuroimagem não foram significativas. O estudo da bolha no ecocardiograma transtorácico revelou um shunt atrial direito-esquerdo e o ecocardiograma transesofágico subsequente revelou um forame oval patente. Esta anomalia cardíaca congênita foi o provável conduíte para uma oclusão tromboembólica da artéria central retiniana Na literatura, foram identificadossete pacientes com forame oval patente associado à oclusão da artéria central retiniana. O ecocardiograma transtorácico diagnosticou apenas um paciente (14,3%), enquanto o ecocardiograma transesofágico foi necessário para revelar o forame oval patente nos seis casos restantes (85,7%). Nosso caso e relatos anteriores suportam a ligação entre a oclusão da artéria central retiniana e o forame oval patente. Os profissionais devem considerar, como sendo mais sensível, o ecocardiograma transesofágico na avaliação inicial de pacientes jovens sem causas imediatamente identificáveis de oclusões da artéria retiniana.

RESUMO

BACKGROUND: Central retinal artery occlusion (CRAO) is a rare acute disease associated with great morbidity. It is reported as a complication of surgical procedures, but rarely associated with brain surgery and no reports before due to parasagittal meningioma resection. CASE DESCRIPTION: We present the case of a 41-year-old female who underwent surgery for a parasagittal meningioma and developed a bilateral CRAO as an acute postoperative complication. Most common causes, such as cardiac embolism, carotid pathology and coagulation problems, were discussed and all clinical and neuroradiological exams performed were reported. CONCLUSION: Bilateral CRAO as results of brain surgery is extremely rare; however, if it occurs, it should be early recognized and treated to minimize its high morbidity.

RESUMO

PURPOSE: To report a case of central retinal artery (CRA) occlusion secondary to prepapillary loop in a 13-year-old girl. METHODS: A 13-year-old girl presented with a history of sudden visual loss in her left eye. RESULTS: Fundus examination confirmed thrombosis in a prepapillary arterial loop causing CRA occlusion and extensive retinal ischemia. Macular region was watered by an anomalous macular branch, which explained her 20/20 vision central vision. CONCLUSION: Congenital prepapillary vascular loops are rare and usually asymptomatic. We report a case of central artery occlusion confirmed by multimodal imaging.

RESUMO

ABSTRACT The case of a 55-year-old male who presented central retinal vein occlusion with marked macular ischemia in left eye is reported. Despite the intervention with sustained-release dexamethasone polymer injection and other clinical measures, the visual acuity was severely reduced in left eye. After 8 months, he returned to the emergency with acute visual loss of 2 hours of progression in right eye due to a central retinal artery occlusion, sparing only the territory of the cilioretinal artery. Patient underwent clinical maneuvers with anterior chamber paracentesis and intravenous injection of tissue plasminogen activator. Fluorescein angiography immediately after the procedures showed recanalization, but despite arterial vasodilation, no complete recanalization was observed after 24 hours. The patient developed retinal atrophy.

RESUMO Apresenta-se o caso de um paciente do sexo masculino, de 55 anos, com oclusão de veia central retiniana com acentuada isquemia macular em olho esquerdo. Apesar da intervenção com injeção de polímero de liberação lenta de dexametasona e outras medidas clínicas tomadas, ele evoluiu com severa baixa da acuidade visual em olho esquerdo. Após 8 meses, retornou à emergência com perda visual aguda de 2 horas de evolução em olho direito devido à oclusão de artéria central retiniana, poupando apenas o território da artéria ciliorretiniana. O paciente foi submetido a manobras clínicas, com paracentese de câmara anterior e injeção endovenosa de ativador do plasminogênio tecidual. A angiografia fluoresceínica imediatamente após as manobras mostrou recanalização, porém, a despeito do vasodilatador arterial, não foi observada completa recanalização com 24 horas. O paciente evoluiu com atrofia retiniana.

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