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.

Analysis of phenotype and CYP4V2 gene variants in two pedigrees affected with Bietti crystalline corneoretinal dystrophy / 中华医学遗传学杂志

OBJECTIVE@#The CYP4V2 gene of two pedigrees affected with Bietti crystalline corneoretinal dystrophy was analyzed to indentify the cause of the disease and provide a basis for clinical diagnosis.@*METHODS@#The probands were subjected to next generation sequencing (NGS). Suspected variants were verified by Sanger sequencing. Pathogenicity of the variants were searched through relevant databases and PubMed by following the ACMG guidelines.@*RESULTS@#A homozygous variant in the CYP4V2 gene c. (802-8) _810delTCATACAGGTCATCGCTinsGC was detected in proband from pedigree 1, parents did not detect; CYP4V2 genes c. (802-8)_810delTCATACAGGTCATCGCTinsGC and c. 958 C>T (p.Arg320X) compound heterozygous variants existed in the proband of pedigree 2,both parents were variant carriers. The results of Sanger sequencing showed that the variant of CYP4V2 gene in the two families was consistent with the NGS sequencing. The c. (802-8)_810delTCATACAGGTCATCGCTinsGC of CYP4V2 gene was splicing variant, and both splicing variant and nonsense variant could produce truncated nonfunctional protein products. Based on standards and guidelines by American College of Medical Genetics and Genomics, the CYP4V2 genes c. (802-8)_810del TCATACAGGTCATCGCTinsGC and c. 958 C>T (p.Arg320X) were predicted to be pathogenic variants (PVS1+PS1+PM2+PM3).@*CONCLUSION@#The homozygous variant c. (802-8) _810delTCATACAGGTCATCGCTinsGC and the complex heterozygous variants c. (802-8) _810delTCATACAGGTCATCGCTinsGC and c.958C>T (p.Arg320X) in CYP4V2 gene are the cause of the disease in the probands of two pedigrees , respectively.

Association between Chagas disease and changes in the optic nerve and retinal nerve fiber layer / Associação entre doença de Chagas e alterações do nervo óptico e camada de fibras nervosas

ABSTRACT Purpose: To test the hypothesis that Chagas disease predisposes to optic nerve and retinal nerve fiber layer alterations. Methods: We conducted a cross-sectional study including 41 patients diagnosed with Chagas disease and 41 controls, paired by sex and age. The patients underwent ophthalmologic examinations, including intraocular pressure measurements, optic nerve and retinal nerve fiber layer screening with retinography, optical coherence tomography, and standard automated perimetry. Results: All of the patients with Chagas disease had a recent cardiologic study; 15 (36.6%) had heart failure, 14 (34.1%) had cardiac form without left ventricular dysfunction, and 12 (29.3%) had indeterminate form. Optic nerve/retinal nerve fiber layer alterations were observed in 24 patients (58.5%) in the Chagas disease group and 7 controls (17.1%) (p£0.01). Among these, optic nerve pallor, optic nerve alterations suggestive of glaucoma, notch, peripapillary hemorrhage, and localized retinal nerve fiber layer defect were detected. Alterations were more prominent in patients with Chagas disease and heart failure (11 patients), although they also occurred in those with Chagas disease without left ventricular dysfunction (7 patients) and those with indeterminate form (6 patients). Optical coherence tomography showed that themean of the average retinal nerve fiber layer thickness measured 89 ± 9.7 mm, and the mean of retinal nerve fiber layer superior and inferior thickness measured 109 ± 17.5 and 113 ± 16.8 mm, respectively were lower in patients with Chagas disease. In controls, these values were 94 ± 10.6 (p=0.02); 117 ± 18.1 (p=0.04), and 122 ± 18.4 mm (p=0.03). Conclusion: Changes in optic nerve/ retinal nerve fiber layer were more prevalent in patients with Chagas disease.

RESUMO Objetivo: Testar a hipótese de que a doença de Chagas predispõe a alterações no nervo óptico e camada de fibras nervosas peripapilar. Métodos: Foi realizado um estudo transversal com 41 pacientes diagnosticados com doença de Chagas e 41 controles, pareados por sexo e idade. Os pacientes foram submetidos a exames oftalmológicos, incluindo medida da pressão intraocular, avaliação do nervo óptico e camada de fibras nervosas através de retinografia, tomografia de coerência óptica e perimetria automatizada padrão. Resultados: Todos os pacientes com doença de Chagas apresentavam estudo cardiológico recente; 15 pacientes (36,6%) apresentavam insuficiência cardíaca; 14 (34,1%) forma cardíaca sem disfunção de ventrículo esquerdo e 12 (29,3%), forma indeterminada. Alterações do nervo óptico/camada de fibras nervosas foram observadas em 24 pacientes (58,5%) do grupo com doença de Chagas e 07 controles (17,1%) (p£0,01). Dentre estas, palidez do nervo óptico, alterações do nervo óptico sugestivas de glaucoma, entalhe, hemorragia peripapilar e defeito da camada de fibras localizado foram detectados. As alterações foram mais proeminentes nos pacientes com doença de Chagas e insuficiência cardíaca (11 pacientes) embora também ocorressem naqueles com doença de Chagas sem disfunção de ventrículo esquerdo (7 pacientes) e com forma indeterminada (6 pacientes). A tomografia de coerência óptica mostrou que a média da espessura da camada de fibras nervosas da retina mediu 89 ± 9,7 mm), e a média da espessura da camada de fibras nervosas superior e inferior mediu 109 ± 17,5 e 113 ± 16,8 mm, respectivamente, foi menor em pacientes com doença de Chagas. Nos controles, esses valores foram de 94 ± 10,6 mm (p=0,02); 117 ± 18,1 (p=0,04) e 122 ± 18,4 mm (p=0,03). Conclusão: Alterações do nervo óptico/camada de fibras nervosas da retina foram mais prevalentes nos pacientes com doença de Chagas.

Effects of caffeic acid phenethyl ester on retinal damage after traumatic brain injury / Efectos del ester fenetílico del ácido cafeico en el daño de la retina después de una lesion cerebral traumática

SUMMARY: Head trauma damages the optic nerve visual function and visual acuity.Effects of head trauma on the retina was investigated with biochemical, histological and immunohistochemical respects.The study was conducted on 30 rats with three groups: group 1 was control group (n=10). Second group was head-traumatized group (n=10) and last group was head-traumatized+Caffeic acid phenethyl ester (CAPE, i.p. 20ml/kg/day). Upon head was traumatized, CAPE was applied to trauma+CAPE group and then for the following four days. At the end of 5th day, rats were anesthetized with ketamine hydroxide and then blood samples were taken for biochemical analysis. MDA and GSH-Px values were compared. After blood sample, total eyes of rats were dissected for histopathological and immunohistochemical analysis. In trauma group, degeneration in retinal photoreceptor cells, disintegrity and in inner and outer nuclear layers, hypertrophy in ganglion cells, and hemorrhage in blood vessels were observed. In the group treated with CAPE, lesser degeneration in photoreceptor cells, regular appearances of inner and outer nuclear layers, mild hemorrhage in blood vessels of ganglionic cell layer were observed. The apoptotic changes caused by trauma seen in photoreceptor and ganglionic cells were decreased and cellular organization was preserved due to CAPE treatment. CAPE was thought to induce healing process on traumatic damages.

RESUMEN: El trauma craneal daña la función visual del nervio óptico y la agudeza visual. Se investigaron los efectos del traumatismo craneal en la retina con aspectos bioquímicos, histológicos e inmunohistoquímicos. El estudio se realizó en 30 ratas distribuidas en tres grupos: grupo control (n = 10); grupo con traumatismo craneal (n = 10); grupo con traumatismo craneoencefálico + Éster fenetílico de ácido cafeico (CAPE, i.p. 20 ml / kg / día). Sobre la cabeza traumatizada, se aplicó CAPE a trauma + grupo CAPE durante los siguientes cuatro días. Al final del día 5, las ratas se anestesiaron con hidróxido de ketamina y luego se tomaron muestras de sangre para el análisis bioquímico. Se compararon los valores de MDA y GSH-Px. Después de la muestra de sangre, se disecaron los ojos de las ratas para su análisis histopatológico e inmunohistoquímico. En el grupo de traumatismos, se observó degeneración en las células fotorreceptoras retinianas, desintegridad en capas nucleares internas y externas, hipertrofia en células ganglionares y hemorragia en los vasos sanguíneos. En el grupo tratado con CAPE, se observó una menor degeneración en las células fotorreceptoras, apariciones regulares de capas nucleares internas y externas, hemorragia leve en los vasos sanguíneos de la capa de células ganglionares. Los cambios apoptóticos causados por el trauma visto en el fotorreceptor y las células ganglionares disminuyeron y la organización celular se conservó debido al tratamiento con CAPE. Se concluyó que CAPE induce un proceso de curación en daños traumáticos.

Neuroprotective effects of erythropoietin on rat retinas subjected to oligemia

OBJECTIVES: Erythropoietin may have neuroprotective potential after ischemia of the central nervous system. Here, we conducted a study to characterize the protective effects of erythropoietin on retinal ganglion cells and gliotic reactions in an experimentally induced oligemia model. METHODS: Rats were subjected to global oligemia by bilateral common carotid artery occlusion and then received either vehicle or erythropoietin via intravitreal injection after 48 h; they were euthanized one week after the injection. The densities of retinal ganglion cells and contents of glial fibrillary acidic protein (astrocytes/Müller cells) and cluster of differentiation 68 clone ED1 (microglia/macrophages), assessed by fluorescence intensity, were evaluated in frozen retinal sections by immunofluorescence and epifluorescence microscopy. RESULTS: Retinal ganglion cells were nearly undetectable one week after oligemia compared with the sham controls; however, these cells were partially preserved in erythropoietin-treated retinas. The contents of glial fibrillary acidic protein and cluster of differentiation 68 clone ED1, markers for reactive gliosis, were significantly higher in retinas after bilateral common carotid artery occlusion than those in both sham and erythropoietin-treated retinas. CONCLUSIONS: The number of partially preserved retinal ganglion cells in the erythropoietin-treated group suggests that erythropoietin exerts a neuroprotective effect on oligemic/ischemic retinas. This effect could be related to the down-modulation of glial reactivity, usually observed in hypoxic conditions, clinically observed during glaucoma or retinal artery occlusion conditions. Therefore, glial reactivity may enhance neurodegeneration in hypoxic conditions, like normal-tension glaucoma and retinal ischemia, and erythropoietin is thus a candidate to be clinically applied after the detection of decreased retinal blood flow.

Benign familial fleck retina: multimodal imaging including optical coherence tomography angiography / Benign familial fleck retina: estudo multimodal incluindo OCT angiography

ABSTRACT This report presents multimodal imaging of a 27-year-old woman diagnosed with benign familial fleck retina (OMIM 228980), an uncommon disorder. Fundus photographs revealed retinal flecks that affected her post-equatorial retina but spared the macular area. Fundus autofluorescence and infrared imaging demonstrated a symmetrical pattern of yellow-white fleck lesions that affected both eyes. Her full-field electroretinogram and electrooculogram were normal. An optical coherence tomography B-scan was performed for both eyes, revealing increased thickness of the retinal pigmented epithelium leading to multiple small pigmented epithelium detachments. The outer retina remained intact in both eyes. Spectral-domain optical coherence tomography angiography with split-spectrum amplitude decorrelation algorithm and 3 × 3 mm structural en face optical coherence tomography did not show macular lesions. Benign familial fleck retina belongs to a heterogenous group of so-called flecked retina syndromes, and should be considered in patients with yellowish-white retinal lesions without involvement of the macula.

RESUMO O objetivo do presente relato é demonstrar um estudo multimodal de um paciente com diagnóstico de Benign Familial Fleck Retina (BFFR) (OMIM 228980), uma alteração retinana muito rara. Retinografia colorida demonstrou "flecks" na retina posterior ao equador, poupando mácula. Tanto autofluorescência quando imagem "infrared," nota-se padrão simétrico de lesões amareladas em ambos os olhos. Eletrorretinograma padrão de campo total e EOG não evidenciaram alterações. SD OCT B-scan demostrou pequenos e múltiplos descolamentos do epitélio pigmentado (EPR), com retina externa intacta em ambos os olhos. Angiografia por OCT com "split-spectrum amplitude decorrelation algorithm" e "structural" "en face" OCT 3 x 3 mm não apontaram anormalidades na mácula. BFFR pertence ao heterogêneo grupo chamado "flecked retina syndromes," devendo ser considerada em pacientes com flecks retinianos poupando mácula.

Prevalência de cicatrizes coriorretinianas em exames angiográficos / Prevalence of chorioretinal scars in angiographics exams

RESUMO A toxoplasmose é uma zoonose que tem como agente etiológico o Toxoplasma gondii, que se caracteriza por ser uma doença infecciosa de distribuição mundial. Considera-se que no mundo existam mais de 2 bilhões de pessoas infectadas pela toxoplasmose. É bem conhecido que a toxoplasmose é uma causa frequente de cicatriz coriorretiniana. Nesse grupo de doenças, o exame angiográfico (ou angiofluoresceinografia de retina) é de fundamental importância para o diagnóstico. Objetivo: Realizar um levantamento da prevalência de cicatrizes coriorretinianas em angiografias em serviço privado na região de Cascavel (PR), Brasil. Métodos: Realização um estudo retrospectivo, transversal, com levantamento de documentos fonte, onde foram analisados 8719 laudos de angiografias de retina realizadas no Instituto da Visão na cidade de Cascavel (PR), Brasil, entre os anos de 2000 a 2011, sendo selecionados como primeiro diagnóstico de cada paciente, completando um total de 4928 exames válidos. As alterações encontradas foram classificadas de acordo com o tipo de cicatriz visualizada, e divididas conforme frequência percentual. Resultados: Observou-se uma prevalência de cicatrizes de 6,38%, sendo que o percentual de cicatriz coriorretiniana encontrado foi de 6,14%. Conclusão: A baixa prevalência de cicatrizes coriorretinianas encontrada na região de Cascavel (PR), Brasil, quando comparada à de Erechim (RS), Brasil, se deve a diversos fatores, tanto climáticos, socioculturais e institucionais. Além das cicatrizes coriorretinianas, que representaram o maior número entre as cicatrizes, foram encontrados outros subtipos cicatriciais com prevalências menores.

ABSTRACT Toxoplasmosis is a zoonosis whose etiologic agent is Toxoplasma gondii, which is characterized as an infectious disease of worldwide distribution. There are thought to be more than 2 billion people globally infected with toxoplasmosis. It is well known that toxoplasmosis is a frequent cause of chorioretinal scarring. In this group of diseases, the angiographic examination (or retinal fluorescein angiography) is very important for diagnosis. Objective: To perform a survey on the prevalence of chorioretinal scars in angiography at a private clinic in the area of Cascavel, Paraná State, Brazil. Methods: This was a retrospective, cross-sectional study with collection of data from source documents, involving the analysis of 8,719 reports of retinal angiograms performed at the Instituto da Visão of Cascavel, Brazil, between the years 2000-2011. The first diagnosis of each patient was selected, completing a total of 4,928 valid exams. The changes were classified according to the type of visualized scarring, and divided as percentage frequency. Results: We observed a prevalence of 6.38% scars, and the percentage of chorioretinal scars was found to be 6.14%. Conclusion: The low prevalence of chorioretinal scars found in the region of Cascavel, Brazil, when compared with the region of Erechim (Rio Grande do Sul State), Brazil, is due to climatic, sociocultural and institutional factors. Besides the chorioretinal scars, which represent the largest group of scars, other subtypes of scars with lower prevalence were found.

Relationship between retinal sensitivity and disease activity in patients with psoriasis vulgaris

OBJECTIVES: Psoriasis is a hyperproliferative chronic inflammatory skin disease of unknown etiology and ocular structures and visual pathways can also be affected during the course of this disease. Subclinical optic neuritis has previously been observed in psoriatic patients in visual evoked potential studies. This trial was designed to evaluate retinal sensitivity in patients with psoriasis vulgaris. METHODS: A total of 40 eyes of 40 patients with chronic plaque-type psoriasis and 40 eyes of 40 age- and sex-matched control subjects were included in this study. The diagnosis of psoriasis was confirmed by skin biopsy. The severity was determined using the Psoriasis Area and Severity Index and the duration of the disease was recorded. After a full ophthalmological examination, including tests for color vision and pupil reactions, the visual field of each subject was assessed using both standard achromatic perimetry and short wavelength automated perimetry. RESULTS: The mean Psoriasis Area and Severity Index was 22.05±6.40′. There were no significant differences in the visual field parameters of subjects versus controls using either method. There were correlations between disease severity and the mean deviations in standard achromatic perimetry and short wavelength automated perimetry and between disease severity and the corrected pattern standard deviation and pattern standard deviation of short wavelength automated perimetry (r = -0.363, r = -0.399, r = 0.515 and r = 0.369, respectively). CONCLUSIONS: Retinal sensitivity appears to be affected by the severity of psoriasis vulgaris. .

Forensic Application of Microperimetry and Visual Evoked Potential in Macular Disease / 法医学杂志

OBJECTIV@#e To find the correlation between real best corrected visual acuity (BCVA) and testing results of microperimetry and visual evoked potential (VEP) and to explore a new method in recording BCVA in macular disease.@*METHODS@#Sixty-two patients with macular disease (macular disease group, 62 eyes) and eighteen healthy volunteers (control group, 36 eyes) had BCVA, microperimetry and VEP recorded.@*RESULTS@#(1) By microperimetry, the values of retinal mean sensitivity and fixation percentage in macular disease group were lower than that in control group. The bicurve ellipse area in macular disease group was higher than that in control group. By VEP, P100 amplitude under 0.5 cpd and 2 cpd in macular disease group were significantly higher than that in control group and the latency was prolonged (P < 0.05). (2) In macular disease group, BCVA had significant positive correlation with retinal mean sensitivity, bicurve ellipse area, macular central 2 degrees and 4 degrees fixation percentage, respectively (P < 0.05). There was a significant correlation between retinal mean sensitivity and P100 amplitude (P < 0.05). (3) Multiple linear regression equation was y = 0.053 x1+0.008 x3+3.897 (y was BCVA, while x1 was retinal mean sensitivity and x3 was P100 amplitude under 2 cpd).@*CONCLUSION@#Combined use of microperimetry and VEP is useful in the assessment of BCVA in macular disease.

Hallazgo por neuroimaginología de microangiopatía cerebral retiniana con calcificaciones y quistes / Neuroimaging findings in cerebroretinal microangiopathy with calcifications and cysts

La microangiopatía cerebral retiniana con calcificaciones y quistes es una enfermedad poco frecuente, caracterizada por alteraciones cerebrales, retinianas y óseas, así como por predisposición al sangrado gastrointestinal. Existen pocos reportes de casos de esta condición, especialmente en adultos, en quienes la incidencia es baja. Los hallazgos por medio de neuroimágenes son característicos, con calcificaciones bilaterales y múltiples formaciones quísticas. El propósito de este artículo fue hacer una revisión bibliográfica e ilustrar dos casos cuyo diagnóstico fue posible con la ayuda de neuroimágenes.

Cerebroretinal microangiopathy with calcifications and cysts is a rare condition characterized by brain, retinal and bone anomalies, as well as a predisposition to gastrointestinal bleeding. There are few reported cases of this condition in adults, among whom the incidence is low. Neuroimaging findings are characteristic, with bilateral calcifications, leukoencephalopathy and intracranial cysts. The purpose of this article was to do a literature survey and illustrate two cases diagnosed with the aid of neuroimaging.

Transient spectral domain optical coherence tomography findings in classic MEWDS: a case report / Alterações transitórias evidenciadas na tomografia de coerência óptica de domíno espectral em quadro clássico de MEWDS: relato de caso

The purpose of this study was to describe a patient with multiple evanescent white dot syndrome (MEWDS) who presented with classic retinal findings and transient changes in outer retinal anatomy. A 20-year-old man presented with mild blurred vision in the left eye, reporting flu-like symptoms 1 week before the visual symptoms started. Fundus examination of the left eye revealed foveal granularity and multiple scattered spots deep to the retina in the posterior pole. Fluorescein angiography and indocyanine green angiography showed typical MEWDS findings. Spectral Domain Optical Coherence Tomography has shown transient changes in outer retinal anatomy with disappearance of inner segment-outer segment junction and mild attenuation of external limiting membrane. Six months later, Spectral Domain Optical Coherence Tomography has shown complete resolution with recovery of normal outer retinal aspect.

O propósito deste estudo é descrever o caso de um paciente com síndrome dos múltiplos pontos brancos evanescentes (MEWDS), apresentando achados retinianos clássicos e alterações transitórias na anatomia retiniana externa. Paciente do sexo masculino, 20 anos de idade, apresentando embaçamento visual no olho esquerdo, relatando sintomas gripais uma semana antes do início dos sintomas visuais. Fundoscopia do olho esquerdo revelou granularidade foveal, múltiplos pontos brancos retinianos no polo posterior. A angiografia fluoresceínica e a indocianinografia verde evidenciaram achados típicos de MEWDS. A tomografia de coerência óptica de domínio espectral evidenciou alterações transitórias na anatomia retiniana externa como desaparecimento da junção dos segmentos interno-externo dos fotorreceptores e leve atenuação da membrana limitante externa. Após 6 meses, a tomografia de coerência óptica mostrou completa resolução com recuperação total da anatomia retiniana externa.

novel navigated laser system brings new efficacy to the treatment of retinovascular disorders

The laser photocoagulation is now treatment of choice for various retinovascular disorders. Conventional slit-lamp based laser delivery systems have many limitations including, questionable accuracy, need of contact lens with local anesthesia, and inadvertent damage to fovea. Navigated laser system, a fundus camera based laser delivery system with computer based laser planning and laser treatment without contact lens achieves improved patient compliance, improved accuracy, and treatment ease for the physician, efficient panretinal photocoagulation pattern laser, excellent documentation, and advanced laser training. This article compares navigated laser systems with available conventional and PASCAL laser systems based on the literature and personal experience of the authors

Isolated retinal cotton wool spot after coronary angiography

Visual symptoms after coronary angiography are rarely encountered and mostly related to contrast induced transient cortical blindness or retinal artery occlusions. We report an intriguing case of a 50-year-old woman, who presented with vision deterioration in her right eye 12 h after coronary angiography for cardiac palpitation. Fundoscopy and optical coherence tomography scan revealed an isolated parafoveal cotton wool spot in her right eye that has totally resolved 6 weeks after initial presentation. This is the first case report of this rare post coronary angiography complication

Electroretinogram and histopathologic changes of the retina after methanol intoxication / 法医学杂志

In order to study the functional and structural alterations of the retina in SD rat model after methanol intoxication, 35 rats were divided randomly into five groups administrated with saline, 3-day high dose, 7-day high dose, 3-day low dose and 7-day low dose methanol separately. The retinal function of each group was assessed by flash electroretinogram (F-ERG) 3 and 7 days after methanol poisoning. The microstructure and ultrastructure of the retina were observed at the same time. The high-dose methanol intoxication induced irreversible retinal functional and structural damages 3 days after poisoning, which included prolonged latency and reduced amplitude of the Max-reaction of F-ERG. These injuries were aggravated 7 days after poisoning. Meanwhile, the latency and amplitude of the Cone-reaction of F-ERG were also affected 3 days after poisoning, but there were no further worsening tendency 7 days after poisoning. The retinal histological analysis showed cellular edema, heteromorphy and disarrangement, tissular loosen of the inner nuclear layer and photoreceptors layer. The mitochondrial damage began at the photoreceptors layer and developed further into the inner nuclear layer. The low-dose methanol intoxication only caused transient damage of the retina. Our results showed that the function and structure of the photoreceptor and inner nuclear layer were the primary target of methanol intoxication and that the rod cells were more sensitive to methanol intoxication than the cone cells. The mitochondrial damage developed from outer layer to inner layer of the retina.

Tomografia de coerência óptica para avaliação de doenças da retina / Optical coherence tomography for evaluation of retinal diseases

A Tomografia de Coerência Óptica (OCT) é um método de exame oftalmológico não invasivo e de não contato que permite a realização de cortes transversais da retina (segmento posterior), gerando imagens tomográficas de alta resolução. Sua aplicação é especialmente útil para aplicações diagnósticas oftalmológicas devido ao fácil acesso óptico às estruturas do segmento posterior do olho, permitindo detectar sinais microscópicos de alterações precoces do tecido estudado, além de alterações anatômicas coroido-retinianas na profundidade da retina. A realização do exame costuma durar em média 10 minutos e é realizado pelo próprio oftalmologista ou por tecnólogo capacitado. O diagnóstico normalmente é feito de forma imediata exclusivamente pelo médico oftalmologista. O Tomográfo de Coerência Óptica é um equipamento de imaginologia que se incorporou ao arsenal oftalmológico de forma mais consistente desde meados da presente década (2005 em diante) devido à sua capacidade de avaliar a histologia retiniana através da confecção de cortes tomográficos de retina obtidos por um feixe de luz que tem precisão de 3 micrômetros. Dessa forma, tornou-se possível a avaliação da estrutura macular (região central da retina, responsável pela maior qualidade da visão humana) de forma precisa e não invasiva. Novas hipóteses fisiopatológicas para um número significativo de doenças retinianas têm sido propostas a partir das imagens obtidas pela OCT, as quais são consideradas padrão-ouro para o diagnóstico de algumas dessas doenças, sendo decisivas na classificação do estágio das mesmas e sua conseqüente definição terapêutica ­ como, por exemplo, no Buraco Macular (estadiamento) e na Degeneração Macular Relacionada à Idade (diagnóstico, acompanhamento terapêutico, indicação de retratamento). Há duas tecnologias de captura das imagens pelos aparelhos de tomografia de coerência óptica. A primeira tecnologia, chamada de Domínio Espectral, ainda é a mais comum nos aparelhos existentes no Brasil. A outra tecnologia, Domínio Fourrier, oferece uma maior resolução de imagens, permitindo melhor capacidade diagnóstica em casos limítrofes.Embora não haja um procedimento específico no SUS para ressarcimento dos exames, o Tomógrafo de Coerência Óptica está disponibilizado na relação de equipamentos financiáveis pelo Ministério da Saúde desde 2006. Alguns exemplos das principais indicações clínicas para a realização do exame de Tomografia de Coerência Óptica são: a) Doenças da retina; b) Doenças do nervo óptico; c) Doenças do segmento anterior; Tumores de íris. Os membros da CONITEC presentes na 9ª reunião do plenário do dia 11/10/2012, por unanimidade, ratificaram a decisão de recomendar a incorporação do procedimento de Tomografia de Coerência Óptica para utilização em casos de doenças da retina. A Portaria N.º 26, de 12 de junho de 2013 - Decisão de incorporar o procedimento tomografia de coerência óptica para utilização em casos de doenças da retina no Sistema Único de Saúde - SUS.

Foveal Thickness between Stratus and Spectralis Optical Coherence Tomography in Retinal Diseases

PURPOSE: To compare the foveal thickness (FT) parameters measured by Stratus optical coherence tomography (OCT) and Spectralis OCT in various retinal diseases and to construct conversion formulas between the two types of OCT devices. METHODS: We examined 366 consecutive patients (475 eyes) with retinal diseases and 13 normal controls (13 eyes). The patients were categorized into eight retinal disease groups. The mean amount and distribution of foveal thickness differences (FTD) measured by Stratus and Spectralis OCT were determined, and conversion formulas were constructed for Stratus OCT FT from Spectralis OCT FT for each retinal disease group. RESULTS: Among retinal diseases, the mean FTD was significantly larger in exudative age-related macular degeneration (AMD) patients (mean +/- SD, 94.0 +/- 55.0 microm) compared to normal subjects (66.2 +/- 11.7 microm; p < 0.0001). The proportion of eyes with a mean FTD outside 1.96 standard deviations of normal subject FTD was greatest in the exudative AMD (50.0%) group and smallest in the macular hole (18.2%) group. The predicted FTs obtained through the conversion formulas showed lower variance than the actual FTD values, especially in the exudative AMD group. The prediction line for exudative AMD deviated most from that of normal subjects. CONCLUSIONS: FTD shows diverse values and variances among various retinal diseases, especially in exudative AMD, which indicates that Stratus OCT FT cannot be predicted from Spectralis OCT FT by FTD value alone. We constructed statistically significant conversion formulas, which provided more reliable methods to predict Stratus OCT-measured FT from Spectralis OCT measurements for different retinal disease groups.

Alterações retinianas em jovens portadores de anemia falciforme (hemoglobinopatias) em hospital universitário no nordeste do Brasil / Retinal impairment in young individuals with sickle cell anemia (hemoglobin SS disease) in university hospital in Northeastern of Brazil

OBJETIVOS: Descrever e classificar alterações retinianas encontradas em portadores de anemia falciforme com genótipo SS, bem com comparar métodos diagnósticos (mapeamento de retina e angiofluoresceinografia). MÉTODOS: Neste estudo transversal foram avaliados pacientes portadores de anemia falciforme com idade igual ou superior a sete anos. Esses pacientes foram submetidos a mapeamento de retina e angiofluoresceinografia. Os achados do mapeamento de retina foram agrupados em três classes: sem alterações; alterações não proliferativas e alterações proliferativas. Os resultados à angiofluoresceinografia foram classificados de acordo com os estágios de Goldberg, variando de I a V e expressando gradiente crescente de gravidade. RESULTADOS: Foram avaliados 61olhos de 31pacientes. A retinopatia falciforme foi encontrada em 38/61 (62,3%) dos olhos examinados. A média de idade do grupo de portadores de retinopatia foi menor que dos pacientes sem retinopatia (14,4 versus 17,4 anos, p=0,04). Observou-se elevada freqüência de retinopatia não proliferativa, especialmente as tortuosidades vasculares (27,9%), seguidas por anastomoses arteriovenosas na periferia da retina (24,6%) e oclusões arteriolares (8,2%). Em um olho foi observado neovascularização. Em 16,4% dos olhos obteve-se resultado normal no mapeamento de retina e alterado à angiofluoresceinografia. CONCLUSÕES: As alterações retinianas do tipo não proliferativa são frequentes e precoces nos portadores de anemia falciforme do tipo SS, sendo a angiofluoresceinografia mais sensível no diagnóstico quando comparada ao mapeamento de retina.

PURPOSES: To describe and categorize retinal vascular changes in patients with sickle cell anemia, as well as to compare diagnostic methods (indirect ophthalmoscopy and fluorescein angiography). METHODS: Patients with sickle cell anemia over the age of seven were examined. Complete ophthalmologic examination with indirect ophthalmoscopy and angiography was performed in each patient. The fundoscopy results were grouped in 3 classes: normal; non-proliferative retinopathy, which includes vascular tortuosity, black sunburst, salmon-patch and peripheral closure/anastomoses; and proliferative retinopathy, related to neovascular proliferation. Angiography results were classified according to Goldberg classifications from stage I to V. RESULTS: Retinopathy related to sickle cell anemia was seen in 62.3% (38/61) of the eyes checked. Neovascularization was observed in one eye. The frequency of bilateral changes in angiography was high. Non-proliferative retinopathy was more common, especially vascular tortuosities (17/61), followed by arteriovenous anastomoses in the retinal periphery (15/61) and arterial occlusions (5/61). The mean age of retinopathy group was 14.4 years old, significantly lower than the mean age of non-retinopathy group, which was 17.4. The result was normal in16.4% (10/61) of the eyes in the fundoscopy exam, while angiography showed alterations. CONCLUSIONS: All the results pointed to the conclusion that the non-proliferative retinal vascular changes are frequent and precocious in patients with sickle cell anemia (SS genotype). Fluorescein angiography is more sensitive in the diagnosis of retinopathy when compared to indirect ophthalmoscopy.

Intravitreal ranibizumab for the treatment of choroidal neovascularization secondary to ocular toxoplasmosis.

The purpose of the study was to report a case of choroidal neovascularization (CNV) secondary to ocular toxoplasmosis in an 18-year-old female patient. She was treated with a single intravitreal injection of ranibizumab. The CNV resolved as confirmed by fluorescein angiography and optical coherence tomography (OCT). The visual acuity improved to 20/30, which was maintained till the last follow-up visit at two years, without requisition of a repeat injection.

Optical coherence tomography evaluation of retinal nerve fiber layer in longitudinally extensive transverse myelitis / Avaliação da camada de fibras nervosas da retina na mielite transversal longitudinalmente extensa usando tomografia de coerência óptica

OBJECTIVE: To compare optical coherence tomography (OCT) measurements on the retinal nerve fiber layer (RNFL) of healthy controls and patients with longitudinally extensive transverse myelitis (LETM) without previous optic neuritis. METHOD: Twenty-six eyes from 26 patients with LETM and 26 control eyes were subjected to automated perimetry and OCT for comparison of RNFL measurements. RESULTS: The mean deviation values from perimetry were significantly lower in patients with LETM than in controls (p<0.0001). RNFL measurements in the nasal quadrant and in the 3-o'clock segment were significantly smaller in LETM eyes than in controls. (p=0.04 and p=0.006, respectively). No significantly differences in other RNFL measurements were found. CONCLUSION: Patients with LETM may present localized RNFL loss, particularly on the nasal side of the optic disc, associated with slight visual field defects, even in the absence of previous episodes of optic neuritis. These findings emphasize the fact that patients with LETM may experience attacks of subclinical optic nerve damage.

OBJETIVO: Comparar as medidas da camada de fibras nervosas da retina (CFNR) usando a tomografia de coerência óptica (TCO) em indivíduos normais e pacientes com mielite transversal longitudinalmente extensa (MTLE) sem episódio prévio de neurite óptica. MÉTODO: Vinte e seis olhos de 26 pacientes com MTLE e 26 olhos normais foram submetidos à campimetria computadorizada e TCO para comparação das medidas da CFNR. RESULTADOS: Valores do parâmetro desvio médio da campimetria computadorizada foram significativamente menores nos pacientes com MTLE do que nos controles (p<0,001). Medidas da CFNR no quadrante nasal e no segmento 3 horas foram significativamente menores nos olhos dos pacientes com MTLE do que nos olhos normais (p=0,04 e p=0,006, respectivamente). Não foi encontrada diferença significante nas outras medidas da CFNR avaliadas. CONCLUSÃO: Pacientes com MTLE podem apresentar perda localizada da CFNR, particularmente na região nasal do disco óptico, associada a defeitos discretos de campo visual, mesmo na ausência de episódio prévio de neurite óptica. Estes achados sugerem que pacientes com MTLE podem apresentar acometimento subclínico do nervo óptico.

Hemorrhagic intraretinal macrocyst: Differential diagnoses and report of an unusual case

Retinal 'cysts' may be single or multiple, ranging from two-to-ten disc diameters in size, and occur in eyes with longstanding retinal detachment. The authors describe a retinal macrocyst larger than ten disc diameters, with a blood-filled cavity, and its ultrasound findings. Improved retinal nourishment following retinal reattachment gradually reverses the process responsible for cystic degeneration, with the eventual collapse of the cyst [within days or weeks]. Surprisingly, this giant cyst did not collapse for almost three years despite retinal reattachment. The internal mobile echogenic contents were suggestive of blood. The possible reason of blood in the cyst could be rupture of the retinal blood vessels in the cyst cavity. This could be a recurrent phenomenon, which did not allow the cyst to collapse. The Hemorrhagic Intraretinal Macrocyst needs to be differentiated from mimicking clinical conditions, namely, retinoschisis, choroidal melanoma, subretinal abscess, choroidal hemangioma, and the like. It could take up to a few years to collapse spontaneously, following successful retinal reattachment