Novel optical coherence tomography findings in a patient with methanol toxicity: a case report.

We report a case of acute methanol toxicity with unique optical coherence tomography findings. A 56-year-old man was referred to our ophthalmology clinic with a history of handmade vodka consumption and vision loss. On ophthalmologic examination, his vision was 20/100 in his right eye and 20/200 in his left eye. Bilateral mild optic disk hyperemia was detected on fundus examination. Because of the severity of systemic symptoms in such cases, it is very difficult to include optical coherence tomography in the ophthalmologic examination. However, we managed to perform optical coherence tomography and recorded shallow subretinal fluid and a prominent middle limiting membrane sign as acute retinal structural changes in the patient. The patient was treated with hemodialysis, intravenous ethanol, and sodium bicarbonate. On the fourth day of treatment, visual acuity improved to 20/20 in both eyes. In addition, the prominent middle limiting membrane sign and subretinal fluid disappeared. In this unusual case, retinal pigment epithelium damage and retinal ischemia may have contributed to the prominent middle limiting membrane and subretinal fluid, which are novel optical coherence tomography findings of methanol toxicity.

Laser cutting machine-induced maculopathy and spontaneous recovery.

The thermal effects of laser cutting machines could damage the macula. A few studies in the literature have described macular injury induced by industrial laser burns. The aim of this study was to report the clinical, visual, and optical coherence tomography findings in a gold refinery worker with laser-induced maculopathy. A 21-year-old male gold refinery worker had vision loss in his right eye after using a laser cutting machine without wearing laser eye protection gear. At the first visit (24 h later), his best-corrected visual acuity was 7/10 in the right eye and 10/10 in the left eye. The anterior segment examination was normal. In fundus examination, focal, round, and yellowish lesion was detected within the fovea. The optical coherence tomography findings were foveal outer retinal disruptions and irregularities extending from the outer plexiform layer to the retina pigment epithelium. After 4 months, the best-corrected visual acuity had improved to 1.0, and the optical coherence tomography findings had resolved.

PARACENTRAL ACUTE MIDDLE MACULOPATHY AFTER AORTIC ANEURYSM REPAIR.

PURPOSE: We report a case of paracentral acute middle maculopathy secondary to cholesterol embolization syndrome after an endovascular aortic aneurism repair (EVAR). METHODS: Functional and anatomical damage was assessed by Snellen visual acuity, fundoscopy, and optical coherence tomography. RESULTS: A 67-year-old man presented with sudden vision loss in the right eye for one day with concomitant livedo reticularis in both feet. His medical history included an abdominal EVAR repair three days before the symptoms. The best-corrected visual acuity was 20/40 in the right eye and 20/20 in the left eye. Fundoscopy showed intraarteriolar cholesterol plaques (Hollenhorst) bilaterally, and a localized retinal whitening in the right eye. Optical coherence tomography at the lesion site revealed a hyperreflective band on the level of inner nuclear layer. The patient was referred for imaging and complete systemic evaluation. He was then closely followed up by a vascular team, without additional intervention. Retinal stigma partially regressed at six months with visual improvement. CONCLUSION: This case describes paracentral acute middle maculopathy as a rare ocular sign of cholesterol embolization in postoperative intraaortic operations. The diagnosis of this complication led to prompt clinical care with a multidisciplinary approach.

Coexistence of Straatsma syndrome without macular extension and abnormal ellipsoid zone / Coexistência da síndrome de Straatsma sem extensão macular e zona elipsoide anormal

ABSTRACT Myelinated retinal nerve fibers are rare congenital anomalies that appear as gray-white patches. They may be present in a syndrome characterized by ipsilateral myelinated retinal nerve fibers, myopia and amblyopia. The author reported an ellipsoid zone defect on spectral domain optical coherence tomography in a case of Straatsma syndrome without macular extension.

RESUMO Fibras nervosas retinais mielinizadas são anomalias congênitas raras que aparecem como manchas branco-acinzentadas. Eles podem se apresentar em uma síndrome caracterizada por fibras nervosas retinais mielinizadas ipsilaterais, miopia e ambliopia. O autor relatou um defeito na zona elipsoide na tomografia de coerência óptica de domínio espectral em um caso de síndrome de Straatsma sem extensão macular.

Azoor: Retinopatia externa oculta zonal aguda associada com doença autoimune / Azoor: Acute Zonal Occute Outer retinopathy associated with autoimmune disease

Resumo Retinopatia externa oculta zonal aguda (AZOOR) foi descrita pela primeira vez por Gass em 1993 como uma síndrome com perda rápida de uma ou mais zonas extensas dos segmentos externos da retina. Paciente masculino, 35 anos, portador de doença de Crohn, queixando-se de dor ocular eventual e nictalopia em olho direito desde infância. Em uso regular de azatioprina e mesalazina. melhor acuidade visual 20/20 AO. À fundoscopia, lesões hiperpigmentadas em arcada temporal inferior de olho direito, em treliça, acompanhando vasculatura local. Após exclusão de diagnósticos diferenciais chegou-se ao diagnóstico de Azoor. Azoor é uma síndrome idiopática caracterizada por um quadro agudo início de fotopsia, escotoma ou ambos e é tipicamente associado a uma perda persistente de função visual que envolve uma ou mais zonas da retina externa. Apesar dos sintomas clássicos de fotopsia, o paciente em questão teve uma apresentação clínica atípica. Descrevemos um caso ocorrido em indivíduo do sexo masculino em região periférica apresentando afinamento coroidiano e com doença autoimune associada. Dessa forma, acreditamos que é necessária maior investigação para verificar a etiologia da alteração coroideana e da associação com a doença específica.

Abstract Acute zonal occult external retinopathy (AZOOR) was first described by Gass in 1993 as a syndrome with rapid loss of one or more large areas of the external retinal segments. Male, 35 years, with Crohn's disease complaining of occasional eye pain and right eye nictalopia since childhood. In regular use of azathioprine and mesalazine. better visual acuity 20/20 OU. At fundoscopy, hyperpigmented lesions in the right temporal arcade of the right eye, in trellis, accompanying local vasculature. After exclusion of differential diagnoses, Azoor's diagnosis was reached. Azoor is an idiopathic syndrome characterized by an acute onset of photopsy, scotoma or both and is typically associated with a persistent loss of visual function involving one or more areas of the external retina. Despite classic photopsy symptoms, the patient in question had an atypical clinical presentation. We describe a case of a peripheral male with choroidal thinning and associated autoimmune disease. Thus, we believe that further investigation is necessary to verify the etiology of choroidal alteration and its association with the specific disease.

Wide-Field (15â€Š× 9 mm) Swept-Source Optical Coherence Tomography Angiography Following Plaque Radiotherapy of Choroidal Melanoma: An Analysis of 105 eyes.

PURPOSE: The aim of this study was to evaluate retinal microvascular abnormalities following plaque radiotherapy of choroidal melanoma (CM) using wide-field swept-source optical coherence tomography angiography (OCTA). DESIGN: Single-centre retrospective review. METHODS: Retrospective case series of 105 CM patients treated with I-125 plaque radiotherapy and imaged with wide-field (15 × 9 mm) SS-OCTA from March 2018 to August 2018 at the Ocular Oncology Service, Wills Eye Hospital (Philadelphia, PA). RESULTS: At mean follow-up of 49 months (range 4-297) after plaque radiotherapy, there were 52 eyes (50%) with clinically evident radiation retinopathy (CERR) and 53 eyes (50%) without CERR. Comparison (CERR vs controls) revealed foveal avascular zone enlargement (1.7 vs 0.23 mm, P = 0.03) and reduction of capillary vascular density (CVD) in the superficial and deep plexus in the total wide-field (43% vs 47%, P < 0.001, and 46% vs 48%, P = 0.001, respectively), peripapillary region (66% vs 77%, P < 0.001, and 66% vs 72%, P = 0.001, respectively), and papillomacular bundle (60% vs 68%, P < 0.001, and 61% vs 64%, P = 0.03, respectively). Comparison (no CERR vs controls) revealed nonsignificant foveal avascular zone enlargement (1.20 vs 0.23 mm, P = 0.16) and reduction of CVD in the superficial plexus (46% vs 47%, P = 0.008), and not the deep plexus (48% vs 48%, P = 0.42) of the total wide-field. Comparison of irradiated eyes (CERR vs no CERR) showed reduction of CVD in the superficial and deep plexus of the total wide-field (43% vs 46%, P < 0.006, and 46% vs 48% P < 0.02, respectively), peripapillary region (66% vs 74%, P < 0.001, and 66% vs 72% P < 0.01, respectively), and superficial plexus in the papillomacular bundle (60% vs 65%, P = 0.03). CONCLUSIONS: Following plaque radiotherapy for choroidal melanoma, wide-field swept-source optical coherence tomography angiography demonstrates retinal microvascular abnormalities in the CVD in eyes with and without CERR. These findings are important in early detection and monitoring of radiation retinopathy.

Achados de fundoscopia de pacientes diabéticos e/ou hipertensos / Fundoscopy findings of diabetic and/or hipertensive patients

Resumo Objetivo: Analisar a prevalência de alterações na fundoscopia de pacientes portadores de Diabetes Mellitus (DM) e/ou Hipertensão Arterial Sistêmica (HAS) encaminhados ao Serviço de Referência de Oftalmologia, localizado em uma Unidade Básica de Saúde do município de Patos, Paraíba (PB). Metodos: Estudo de caráter descritivo, transversal e com abordagem quantitativa, que envolveu um total de 22 pacientes. Para a coleta de dados, foi utilizado um questionário sobre a saúde ocular. Além disso, os integrantes da pesquisa participaram do teste da acuidade visual de Snellen e acuidade visual para perto, da medida da pressão intra-ocular e do exame da oftalmoscopia direta. Os que obtiveram pior acuidade visual foram conduzidos para a realização de retinografia. Os dados foram avaliados através de análises estatísticas utilizando o Statistical Package for the Social Sciences - SPSS. Resultados: Do total de 22 pacientes, 11 foram submetidos ao exame de retinografia. Destes, 36,3 % eram apenas diabéticos; 27,4% eram apenas hipertensos e 36,3% eram diabéticos e hipertensos. As principais alterações encontradas foram retinopatia diabética, retinopatia hipertensiva, catarata, glaucoma, nevus de coroide, retinose pigmentar e estafiloma peripapilar. Conclusão: Percebe-se que DM e HAS tem grande impacto negativo sobre a saúde ocular. Para diminuir esse efeito nocivo é necessário que o exame de fundo de olho seja realizado anualmente com a finalidade de diagnosticar precocemente certas patologias e evitar complicações futuras, culminando em menores custos para o sistema de saúde e mais qualidade de vida para os pacientes.

Abstract Objective: To analyze the prevalence of alterations in funduscopy of patients with Diabetes Mellitus (DM) and/or Systemic Arterial Hypertension (SAH) referred to the Ophthalmology Reference Service, located in a Basic Health Unit of the city of Patos, Paraíba). Methods: A descriptive, cross-sectional study with a quantitative approach, which involving a total of 22 patients. For data collection, a questionnaire on ocular health was used. In addition, the research members participated in the Snellen visual acuity test and visual acuity near, intraocular pressure measurement and direct ophthalmoscopy examination. Those who obtained worse visual acuity were conducted for retinography. The data were evaluated through statistical analysis using the Statistical Package for the Social Sciences - SPSS. Results: Of the total of 22 patients, 11 were submitted to retinography. Of these, 36.3% were diabetics only; 27.4% were hypertensive only and 36.3% were diabetic and hypertensive. The main alterations found were diabetic retinopathy, hypertensive retinopathy, cataract, glaucoma, choroidal nevus, pigmentary retinitis and peripapillary staphyloma. Conclusion: It is noticed that DM and SAH have a great negative impact on ocular health. To reduce this harmful effect it is necessary that the fundus eye exam be performed annually for the purpose of early diagnosis of certain pathologies and avoid future complications, culminating in lower costs for the health system and more quality of life for patients.

Evaluation of fundus autofluorescence maging of diabetic patients without retinopathy / Avaliação do exame de imagem de autofluorescência do fundo do olho em pacientes diabéticos sem retinopatia

ABSTRACT Purpose: To evaluate the usefulness of fundus autofluorescence imaging of diabetic patients without retinopathy to investigate early retinal damage. Methods: Fundus autofluorescence images of patients with type 2 diabetes mellitus without retinopathy (diabetic group) and age-sex matched healthy patients (control group) were recorded with a CX-1 digital mydriatic retinal camera after detailed ophthalmologic examinations. MATLAB 2013a software was used to measure the average pixel intensity and average curve width of the macula and fovea. Results: Fifty-six eyes of 28 patients, as the diabetic group, and 54 eyes of 27 healthy patients, as the control group, were included in this study. The mean aggregation index was 168.32 ± 37.18 grayscale units (gsu) in the diabetic group and 152.27 ± 30.39 gsu in the control group (p=0.014). The mean average pixel intensity value of the fovea was 150.87 ± 35.83 gsu the in diabetic group and as 141.51 ± 31.10 gsu in the control group (p=0.060). The average curve width value was statistically higher in the diabetic group than in the control group (71.7 ± 9.2 vs. 59.4 ± 8.6 gsu, respectively, p=0.03). Conclusion: Fundus autofluorescence imaging analysis revealed that diabetic patients without retinopathy have significant fluorescence alterations. Therefore, a noninvasive imaging technique, such as fundus autofluorescence, may be valuable for evaluation of the retina of diabetic patients without retinopathy.

RESUMO Objetivo: Avaliar a utilidade da autofluorescência do fundo de olho de pacientes diabéticos sem retinopatia para investigar lesões precoces na retina. Métodos: Imagens de autofluorescência do fundo de olho de pacientes com diabetes mellitus do tipo 2 sem retinopatia (grupo diabético) e indivíduos saudáveis pareados por idade e sexo (grupo controle) foram registrados com uma câmera retiniana digital midriática CX-1 após exames oftalmológicos detalhados. O software MATLAB 2013a foi usado para medir a intensidade média do pixel e a largura média da curva da mácula e fóvea. Resultados: Cinquenta e seis olhos de 28 pacientes, como o grupo diabético, e 54 olhos de 27 indivíduos saudáveis, como grupo controle, foram incluídos neste estudo. O índice médio de agregação foi de 168,32 ± 37,18 unidades de escala de cinza (gsu) no grupo diabético e em 152,27 ± 30,39 gsu no grupo controle (p = 0,014). O valor médio da intensidade de pixel na fóvea foi de 150,87 ± 35,83 gsu no grupo diabético e de 141,51 ± 31,10 gsu no grupo controle (p=0,060). O valor médio da largura da curva foi estatisticamente maior no grupo diabético do que no grupo controle (71,7 ± 9,2 vs. 59,4 ± 8,6 gsu, respectivamente; p = 0,03). Conclusão: A análise por imagens de autofluorescência de fundo de olho revelou que pacientes diabéticos sem retinopatia apresentam alterações significativas de fluorescência. Portanto, uma técnica de imagem não invasiva, como a autofluorescência de fundo de olho, pode ser valiosa para a avaliação da retina de pacientes diabéticos sem retinopatia.

Classification of diabetes-related retinal diseases using a deep learning approach in optical coherence tomography.

BACKGROUND AND OBJECTIVES: Spectral Domain Optical Coherence Tomography (SD-OCT) is a volumetric imaging technique that allows measuring patterns between layers such as small amounts of fluid. Since 2012, automatic medical image analysis performance has steadily increased through the use of deep learning models that automatically learn relevant features for specific tasks, instead of designing visual features manually. Nevertheless, providing insights and interpretation of the predictions made by the model is still a challenge. This paper describes a deep learning model able to detect medically interpretable information in relevant images from a volume to classify diabetes-related retinal diseases. METHODS: This article presents a new deep learning model, OCT-NET, which is a customized convolutional neural network for processing scans extracted from optical coherence tomography volumes. OCT-NET is applied to the classification of three conditions seen in SD-OCT volumes. Additionally, the proposed model includes a feedback stage that highlights the areas of the scans to support the interpretation of the results. This information is potentially useful for a medical specialist while assessing the prediction produced by the model. RESULTS: The proposed model was tested on the public SERI-CUHK and A2A SD-OCT data sets containing healthy, diabetic retinopathy, diabetic macular edema and age-related macular degeneration. The experimental evaluation shows that the proposed method outperforms conventional convolutional deep learning models from the state of the art reported on the SERI+CUHK and A2A SD-OCT data sets with a precision of 93% and an area under the ROC curve (AUC) of 0.99 respectively. CONCLUSIONS: The proposed method is able to classify the three studied retinal diseases with high accuracy. One advantage of the method is its ability to produce interpretable clinical information in the form of highlighting the regions of the image that most contribute to the classifier decision.

Evaluation of fundus autofluorescence imaging of diabetic patients without retinopathy.

PURPOSE: To evaluate the usefulness of fundus autofluorescence imaging of diabetic patients without retinopathy to investigate early retinal damage. METHODS: Fundus autofluorescence images of patients with type 2 diabetes mellitus without retinopathy (diabetic group) and age-sex matched healthy patients (control group) were recorded with a CX-1 digital mydriatic retinal camera after detailed ophthalmologic examinations. MATLAB 2013a software was used to measure the average pixel intensity and average curve width of the macula and fovea. RESULTS: Fifty-six eyes of 28 patients, as the diabetic group, and 54 eyes of 27 healthy patients, as the control group, were included in this study. The mean aggregation index was 168.32 ± 37.18 grayscale units (gsu) in the diabetic group and 152.27 ± 30.39 gsu in the control group (p=0.014). The mean average pixel intensity value of the fovea was 150.87 ± 35.83 gsu the in diabetic group and as 141.51 ± 31.10 gsu in the control group (p=0.060). The average curve width value was statistically higher in the diabetic group than in the control group (71.7 ± 9.2 vs. 59.4 ± 8.6 gsu, respectively, p=0.03). CONCLUSION: Fundus autofluorescence imaging analysis revealed that diabetic patients without retinopathy have significant fluorescence alterations. Therefore, a noninvasive imaging technique, such as fundus autofluorescence, may be valuable for evaluation of the retina of diabetic patients without retinopathy.

Evaluation of toxic retinopathy caused by antimalarial medications with spectral domain optical coherence tomography / Avaliação da frequência de retinopatia por antimaláricos com o exame tomografia de coerência óptica de domínio espectral

ABSTRACT Purpose: To investigate the frequency of toxic retinopathy in patients with lupus erythematosus and rheumatoid arthritis with long-term use of chloroquine diphosphate or hydroxychloroquine through spectral domain optical coherence tomography and the outcomes of ophthalmological exams (visual acuity - Snellen's table, color vision test - Ishihara's table, fundoscopy, and retinography - red-free). Methods: A cross-sectional study was carried out involving the ophthalmologic evaluation of patients using regular chloroquine diphosphate or hydroxychloroquine for a period of 1 year or longer. The patients completed a questionnaire on their opinions and treatment regularity. The same patients underwent ophthalmologic examination and spectral domain optical coherence tomography. Results: The prevalence of toxic retinopathy caused by antimalarials was 4.15% (9 of 217 patients), 7.4% (4 of 54 patients) following chloroquine diphosphate usage, and 0.82% (1 of 121 patients) following hydroxychloroquine usage. Only patients with advanced stage maculopathy presented abnormalities during the ophthalmologic exam: the color vision test was altered in 11.1%, and visual acuity and fundoscopy were altered in 33.3%. Identification of early toxic retinopathy, detected in six patients, was possible using spectral domain optical coherence tomography. The mean duration of antimalarial drug usage among patients with toxic retinopathy was 10.4 years. Only 31% of the patients reported some symptoms during treatment, and although 24% were afraid to use the medication, they did so as prescribed. Conclusion: Use of spectral domain optical coherence tomography was essential for the diagnosis of early-stage antimalarial toxic retinopathy in patients with the following characteristics: asymptomatic, antimalarial use 7 days a week for a period of more than 5 years, and normal clinical ophthalmologic examination.

RESUMO Objetivo: Investigar a frequência da retinopatia tóxica em pacientes com lúpus eritematoso e artrite reumatóide com uso crônico de difosfato de cloroquina ou hidroxicloroquina, através de tomografia de coerência óptica de domínio espectral e os resultados dos exames oftalmológicos (acuidade visual - tabela de Snellen, teste de visão de cor - tabela de Ishihara, fundoscopia e retinografia - red free). Métodos: Foi realizado um estudo transversal envolvendo a avaliação oftalmológica de pacientes em uso regular de difosfato de cloroquina ou hidroxicloroquina por um período de um ano ou mais. Os pacientes responderam a um questionário sobre a sua opinião e regularidade do tratamento. Os mesmos pacientes realizaram exame oftalmológico clínico e tomografia de coerência óptica de domínio espectral. Resultados: A prevalência de retinopatia tóxica por antimaláricos foi de 4,15% (9 dos 217 pacientes), 7,4% (4 de 54 pacientes) após uso de difosfato de cloroquina e 0,82% (1 de 121 pacientes) após uso de hidroxicloroquina. Apenas os pacientes com maculopatia em fase avançada apresentaram alterações durante os exames clínicos: teste de visão de cores alterado em 11,1%, e a acuidade visual e fundoscopia foram alteradas em 33,3%. A identificação de retinopatia tóxica precoce, detectada em seis pacientes, foi possível por meio da tomografia de coerência óptica de domínio espectral. A duração média do tempo de uso de drogas antimaláricas entre os pacientes com retinopatia tóxica foi de 10,4 anos. Apenas 31% dos pacientes relataram algum sintoma durante o tratamento e apesar de 24% terem medo de usar a medicação, eles o fizeram conforme prescrito. Conclusão: O uso da tomografia de coerência óptica de domínio espectral foi essencial para o diagnóstico de retinopatia tóxica antimalárica em estágio inicial em pacientes com as seguintes características: uso assintomático, antimalárico 7 dias por semana por um período maior que cinco anos e exame oftalmológico clínico normal.

Evaluation of toxic retinopathy caused by antimalarial medications with spectral domain optical coherence tomography.

PURPOSE: To investigate the frequency of toxic retinopathy in patients with lupus erythematosus and rheumatoid arthritis with long-term use of chloroquine diphosphate or hydroxychloroquine through spectral domain optical coherence tomography and the outcomes of ophthalmological exams (visual acuity - Snellen's table, color vision test - Ishihara's table, fundoscopy, and retinography - red-free). METHODS: A cross-sectional study was carried out involving the ophthalmologic evaluation of patients using regular chloroquine diphosphate or hydroxychloroquine for a period of 1 year or longer. The patients completed a questionnaire on their opinions and treatment regularity. The same patients underwent ophthalmologic examination and spectral domain optical coherence tomography. RESULTS: The prevalence of toxic retinopathy caused by antimalarials was 4.15% (9 of 217 patients), 7.4% (4 of 54 patients) following chloroquine diphosphate usage, and 0.82% (1 of 121 patients) following hydroxychloroquine usage. Only patients with advanced stage maculopathy presented abnormalities during the ophthalmologic exam: the color vision test was altered in 11.1%, and visual acuity and fundoscopy were altered in 33.3%. Identification of early toxic retinopathy, detected in six patients, was possible using spectral domain optical coherence tomography. The mean duration of antimalarial drug usage among patients with toxic retinopathy was 10.4 years. Only 31% of the patients reported some symptoms during treatment, and although 24% were afraid to use the medication, they did so as prescribed. CONCLUSION: Use of spectral domain optical coherence tomography was essential for the diagnosis of early-stage antimalarial toxic retinopathy in patients with the following characteristics: asymptomatic, antimalarial use 7 days a week for a period of more than 5 years, and normal clinical ophthalmologic examination.

The use of optical coherence tomography for the detection of ocular toxicity by ethambutol.

OBJECTIVES: To evaluate, through (OCT), alterations in retinal thickness, secondary to use of ethambutol in the treatment of patients with tuberculosis. In addition to studying the use of simpler semiological tools, such as Amsler and Ishihara, in the screening of these cases. METHODS: Thirty patients with ethambutol were recruited from the reference service of tuberculosis treatment at the Federal University of Espírito Santo from May 2015 to July 2016. After clinical history, the following parameters were analyzed; best corrected visual acuity, biomicroscopy, tonometry, photomotor reflex testing, Ishihara test, Amsler's grid test, color digital retinography and optical coherence tomography with CIRRUS HD-OCT (Humphrey-Zeiss) every 2 months during treatment with ethambutol. They were divided into two groups according to the treatment: (1) standard group, two months of ethambutol; (2) extended group, nine to twelve months of ethambutol. RESULTS: There was a significant reduction in OCT thickness between the pre and post treatment times in ten eyes of the extended group, mean reduction of 7,8 microns and in seven eyes of the standard group, with an average of 5.57 microns. During the study, a significant reduction of retinal thickness was observed in both groups at two months of treatment, and the delta percentage was higher in those patients who presented reduction of visual acuity and / or change in the Ishihara test. CONCLUSION: There was a significant reduction in the thickness of the nerve fiber layer by OCT in the patients studied, being more pronounced in those submitted to the extended treatment regimen. This reduction was observed two months after the start of therapy, and was more significant in the cases that presented changes in the Ishihara test.

Morphological and functional findings in Alström syndrome: a study of two families / Achados morfológicos e funcionais na síndrome de Alström: um estudo de duas famílias

ABSTRACT Alström syndrome is a rare disorder characterized by mutations to the ALMS1 gene and clinical findings of childhood obesity, diabetes mellitus, dilated cardiomyopathy, sensorineural hearing loss, and progressive cone-rod dystrophy, which may result in blindness. Ocular manifestations occur in the first decade of life with nystagmus, blepharospasm, and photophobia leading to progressive and severe reductions in visual acuity. This study describes the retinal structure and functional aspects of four patients (8 eyes) from two different families as determined by optical coherence tomography (OCT), fundus autofluorescence, and full-field electroretinography. There was a correlation between morphological and functional findings, evidenced by typical funduscopic changes of retinal dystrophy in spectral domain-OCT and electrophysiological analyses. Foveal characteristics include a single layer of undifferentiated photoreceptors with retinal disorganization mainly from external segments, in agreement with previous reports in the literature. Fundus autofluorescence showed areas of hyperautofluorescence interspersed by hypoautofluorescence dots suggesting, respectively, involvement and atrophy of retinal pigmented epithelial cells in the macular zone. Electroretinographic analyses showed early dysfunction of the cones followed by rapid rod deterioration.

RESUMO A síndrome de Alström é uma doença rara caracterizada por mutações no gene AMLS 1 e achados clínicos de obesidade infantil, diabetes mellitus, cardiomiopatia dilatada, surdez neurossensorial e distrofia de cones e bastonetes progressiva, que podem resultar em cegueira. Manifestações oftalmológicas ocorrem na primeira década de vida com nistagmo, blefaroespasmo e fotofobia, levando a reduções progressivas e graves na acuidade visual. Este estudo descreve a estrutura da retina e os aspectos funcionais de quatro pacientes (oito olhos) de duas famílias dis tintas, conforme determinado por tomografia de coerência óptica, autoflourescência de fundo de olho e eletrorretinograma de campo total. Houve correlação entre os achados morfológicos e funcionais evidenciados por alterações fundoscópicas típicas da distrofia retiniana no domínio espectral-OCT e análises eletrofisiológicas. As características foveais incluem uma única camada de fotorreceptores indiferenciados com desorganização retiniana principalmente nos segmentos externos, de acordo com relatos prévios da literatura. A autofluorescência de fundo mostrou áreas de hiperautofluorescência, sugerindo, respectivamente, envolvimento e atrofia das células do epitélio pigmentar da retina na região macular. Análises eletrorretinográficas mostram disfunção precoce de cones, seguida de rápida deteriorização da haste.

Morphological and functional findings in Alström syndrome: a study of two families.

Alström syndrome is a rare disorder characterized by mutations to the ALMS1 gene and clinical findings of childhood obesity, diabetes mellitus, dilated cardiomyopathy, sensorineural hearing loss, and progressive cone-rod dystrophy, which may result in blindness. Ocular manifestations occur in the first decade of life with nystagmus, blepharospasm, and photophobia leading to progressive and severe reductions in visual acuity. This study describes the retinal structure and functional aspects of four patients (8 eyes) from two different families as determined by optical coherence tomography (OCT), fundus autofluorescence, and full-field electroretinography. There was a correlation between morphological and functional findings, evidenced by typical funduscopic changes of retinal dystrophy in spectral domain-OCT and electrophysiological analyses. Foveal characteristics include a single layer of undifferentiated photoreceptors with retinal disorganization mainly from external segments, in agreement with previous reports in the literature. Fundus autofluorescence showed areas of hyperautofluorescence interspersed by hypoautofluorescence dots suggesting, respectively, involvement and atrophy of retinal pigmented epithelial cells in the macular zone. Electroretinographic analyses showed early dysfunction of the cones followed by rapid rod deterioration.

Retinal toxicity due to hydroxychloroquine: frequency in an Ophthalmology ambulatory / Toxicidade retiniana pela hidroxicloroquina: frequência em um ambulatório de Oftalmologia

Abstract Hydroxychloroquine is widely used by rheumatologists for the treatment of various diseases, such as systemic lupus erythematosus and rheumatoid arthritis because of its safety and low cost. However, it can cause retinal abnormalities. Until today, there is no Brazilian protocol for screening for retinal changes in these patients. We reviewed the medical records and optical coherence tomography of all patients who had attended at Hychloroquine Ambulatory of HFSE, in the period from March/ 2015 until November/2016.

Resumo A Hidroxicloroquina é amplamente utilizada por reumatologistas para o tratamento de várias condições, como os lúpus eritematoso sistêmico e artrite reumatoide, pelo seu baixo custo e relativa segurança. Porém, esta droga pode causar danos à retina. Até o presente momento, não há protocolo brasileiro para o screening de alterações retinianas devido ao uso desta medicação. Foi realizada revisão de prontuário e análise de imagens de tomografia de coerência óptica de pacientes atendidos no período de Março de 2015 a Novembro de 2016 no ambulatório de Hidroxicloroquina do Hospital Federal dos Servidores do Estado do Rio de Janeiro.

FREQUENCY OF OPHTHALMOLOGICAL POSTERIOR SEGMENT FINDINGS IN PATIENTS WITH INFLAMMATORY BOWEL DISEASE.

BACKGROUND: Inflammatory bowel disease is a systemic inflammatory disease classified as Crohn disease or ulcerative colitis. It could present extra intestinal findings, such as fever, weight loss, arthralgia, mucocutaneous lesions, hepatic, renal and ophthalmological involvement. Among ophthalmological findings, posterior segment findings are present in less than 1% of patients with inflammatory bowel disease, however, these findings could bring definitive visual impairment. OBJECTIVE: Our study objective was to evaluate ocular posterior segment findings is patients with inflammatory bowel disease, through retinal mapping, color fundus retinography, optical coherence tomography (OCT) and OCT angiography, and compare our results to literature. METHODS: We evaluated eighty patients with inflammatory bowel disease through complete ophthalmological examination and posterior segment assessment. Color fundus retinography, OCT and OCT angiography was performed with Topcon Triton (Topcon ® , Tokyo, Japan). Macula and posterior pole were evaluated with binocular indirect ophthalmoscopy and fundus biomicroscopy. RESULTS: Participants mean age was 44.16 years (18.08-68.58), 28 (35%) male patients and 52 (65%) female patients. Thirty-five (44%) with diagnosis of Crohn disease, 41 (52%) patients with diagnosis of ulcerative colitis and 3 (4%) had non-conclusive Crohn disease or ulcerative colitis classification. We found abnormal exams in 21 (26.25%) patients. CONCLUSION: Our study found similar prevalence of ophthalmological posterior segment commitment compared to previous literature prevalence. The findings were predominantly unrelated to inflammatory bowel disease, rather than primarily related to it. The most prevalent, and non-previous reported, finding was increased arteriolar tortuosity, probably occurs due to systemic vascular impairment in inflammatory bowel disease.