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ABSTRACT This case report presents the details of a 33-year-old female patient who was referred to a specialized retina service because of mild vision loss in her right eye). The patient's visual acuity was 20/25 in right eye and 20/50 in the left eye (; amblyopic); the spherical equivalent was -12.75 diopters (right eye) and -14.75 diopters (left eye). Multimodal retinal imaging showed peripapillary schisis in both the inner and outer retinal layers, grade II posterior vitreous detachment, and a tessellated fundus. Using Humphrey perimetry and MP-3 microperimetry, the functional evaluation indicated macular sensitivity within normal limits and decreased sensitivity in the peripapillary region, especially in right eye. The pattern-re versal visual evoked potential was measured. The N75 and P100 latency and amplitude in right eye were within normal values for checks of 1º. However, the amplitude was low for checks of 15′. Highly myopic patients who have posterior staphyloma that involves the optic nerve are susceptible to posterior hyaloid traction, and the resulting peripapillary vitreous traction may compromise vision.

RESUMO Este relato de caso apresenta um paciente feminino de 33 anos encaminhado para um serviço especializado de retina devido à leve perda de visão em olho direito. A acuidade visual foi de 20/25 no olho direito e 20/50 no olho esquerdo, o equivalente esférico foi de -12,75 dioptrias e -14,75 dioptrias, respectivamente. Avaliações multimodais revelaram isquese peripapilar nas camadas internas e externas da retina, descolamento vítreo posterior grau II e fundo tesselado. Avaliação funcional com perimetria Humphrey e microperimetria MP-3 revelaram sensibilidade macular normais e diminuição da sensibilidade na região peripapilar, especialmente no olho direito. Potencial visual evocado de padrão reverso apresentou no olho direito latência e amplitude N75 e P100 dentro dos valores normais para verificação de 1º. Entretanto, a amplitude foi baixa para a de 15′. Pacientes alto míopes com esfiloma posterior envolvendo o nervo óptico são suscetíveis à tração da hialoide posterior. Portanto a tração vitreopapilar resultante pode causar comprometimento da visão.

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PURPOSE: To describe a case of retinitis pigmentosa and nanophthalmos in a patient with attenuated Hunter's syndrome. METHODS: Fundus photography, total field electroretinogram, ultrasound, computerized visual field examination, biochemical examination and genetic testing were obtained. RESULTS: The fundus exam showed diffuse arteriolar attenuation, optic disc with regular contours, and pigment agglomerates like "bone spicules" in the middle periphery. Ultrasound examination revealed scleral thickening and short axial diameter in both eyes. The total field electroretinogram exam showed a subnormal result with greater impairment of the scotopic phase of the exam. Computerized visual field examination demonstrated a diffuse reduction in retinal sensitivity in the periphery. Biochemical examination showed increased urine glycosaminoglycan excretion and iduronate-2-sulphatase activity (IDS) deficiency in leukocytes, confirming the type II mucopolysaccharidosis. Molecular analysis revealed a novel missense mutation (p.A77D) in the IDS gene. CONCLUSION: The case report is about a patient presented an attenuated form of the syndrome, with no cognitive impairment. Ophthalmologic follow-up is still an important part of multidisciplinary treatment for Hunter's syndrome.

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This case report presents the details of a 33-year-old female patient who was referred to a specialized retina service because of mild vision loss in her right eye). The patient's visual acuity was 20/25 in right eye and 20/50 in the left eye (; amblyopic); the spherical equivalent was -12.75 diopters (right eye) and -14.75 diopters (left eye). Multimodal retinal imaging showed peripapillary schisis in both the inner and outer retinal layers, grade II posterior vitreous detachment, and a tessellated fundus. Using Humphrey perimetry and MP-3 microperimetry, the functional evaluation indicated macular sensitivity within normal limits and decreased sensitivity in the peripapillary region, especially in right eye. The pattern-re versal visual evoked potential was measured. The N75 and P100 latency and amplitude in right eye were within normal values for checks of 1º. However, the amplitude was low for checks of 15'. Highly myopic patients who have posterior staphyloma that involves the optic nerve are susceptible to posterior hyaloid traction, and the resulting peripapillary vitreous traction may compromise vision.

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BACKGROUND: Papilledema is the main ocular finding in patients with idiopathic intracranial hypertension (IIH) although several chorioretinal abnormalities may also occur and contribute to visual loss. The purpose of this paper is to describe two cases of chorioretinal abnormalities associated with idiopathic intracranial hypertension: one with choroidal folds and another with polypoidal choroidal vasculopathy, an extremely unusual ocular complication in the disease. CASE PRESENTATION: Case 1: A 47-year-old woman previous diagnosed with idiopathic intracranial hypertension treated with weight loss and acetazolamide that over the following 6 months had optic disc edema gradually resolved. The patient was follow-up for a period of 10 years and the papilledema disappeared, but choroidal folds remained unchanged. Case 2: A 61-year-old female patient was seen as a follow-up examination of a 5-year history of IIH that presented with papilledema. The patient was asymptomatic but fundoscopy evaluation revealed a yellowish white peripapillary subretinal nodular lesion temporally in OD. Multimodal imaging studies were made, and the patient was diagnosed with a rare and just recent described association of IIH and polypoidal choroidal vasculopathy. CONCLUSION: Papilledema, RNFL and retinal ganglion cell loss are the most common structural complications of IIH, but chorioretinal complications are important findings and should be carefully evaluated in such patients. Awareness of such occurrence and the use of appropriated clinical and multimodal imaging studies are of great importance for its early detection, leading to proper treatment and prevention of further visual loss.

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PURPOSE: To report a rare presentation of ocular von Hippel-Lindau disease associated with a massive epiretinal vascular proliferation over the macula removed by vitrectomy and submitted to histological analysis. METHODS: Interventional case report. RESULTS: A 13-year-old woman with von Hippel-Lindau disease reported progressive visual loss in the right eye over the preceding 6 months. Best-corrected visual acuity was 20/100 in the right eye. Fundoscopy showed retinal neovascularization (RNV) with macular traction and a small superotemporal hemangioblastoma. Spectral-domain optical coherence tomography confirmed increased macular thickness and macular traction secondary to RNV. Bevacizumab was injected intravitreally, resulting in partial regression of RNV. Five days after the injection, the patient underwent complete removal of fibrovascular proliferation via pars plana vitrectomy, followed by peripheral tumor photocoagulation. The specimen was subjected to histopathological and immunohistochemical analyses. At 2 years of follow-up, vision had improved to 20/30, and anatomical improvement was confirmed on both fundoscopy and spectral-domain optical coherence tomography. CONCLUSION: Ocular von Hippel-Lindau disease may be associated with RNV and macular traction. In such cases, RNV is likely responsive to anti-vascular endothelial growth factor and may be removed surgically along a cleavage plane between the tissue proliferation and the inner retina. In the reported case, the procedure was found to be safe and associated with macular anatomical improvement and vision recovery.

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BACKGROUND: Acute post-cataract endophthalmitis (APE) is a rare complication potentially causing irreversible visual loss. A 10-year study of APE was conducted to determine its incidence, microbiological spectra and antibiotic resistance profile of APE-related pathogens at a major tertiary referral center in Brazil. METHODS: APE cases reported between January 2010 and December 2019 were included. Phacoemulsification and extracapsular cataract techniques were eligible; combined procedures, traumatic and congenital cataract were excluded. Vitreous samples were cultured and antimicrobial resistance was compared for the periods of 2010-2014 and 2015-2019. The results were analyzed with Fisher's exact test. RESULTS: Our sample consisted of 40,491 cataract surgeries and 51 (0.126%) APE cases. Culture was positive in 35 cases (71.4%), of which 31 (88.6%) Gram-positive, 3 (8.6%) Gram-negative, and 1 (2.9%) fungal. The most frequently isolated organism was Staphylococcus epidermidis (n = 17/35, 48.6%), followed by Staphylococcus aureus (n = 4/35, 11.4%). From 2010-2014 to 2015-2019, antimicrobial resistance increased against moxifloxacin (11.1-54.5%, p = 0.07), ciprofloxacin (54.5-72.7%, p = 0.659) and oxacillin (66.7-93.3%, p = 0.13). CONCLUSIONS: The observed incidence and microbial spectra were compatible with previous studies. A trend towards growing moxifloxacin and ciprofloxacin resistance was observed. Surveillance remains crucial to prevent treatment failure from antimicrobial resistance.

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ABSTRACT A 26-year-old woman presented at 28 weeks gestation with hypertensive choroidopathy associated with pre-eclampsia. Fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed in both eyes in the immediate postoperative period. SD-OCT images were obtained before delivery and during a 3-month follow-up. Fundus autofluorescence exhibited patchy hyper- and hypoautofluorescent lesions; fluorescein and indocyanine green angiography revealed areas of choroidal ischemia; and SD-OCT showed disorganization of the outer retinal layers and disruption of the ellipsoid zone. After her blood pressure was stabilized, progressive recovery of the outer retinal layer was monitored on SD-OCT.

RESUMO Uma mulher de 26 anos de idade, com 28 semanas de gestação apresentando coroidopatia hipertensiva associada à pré-eclâmpsia. Retinografia, autofluorescência, tomografia de coerência óptica de domínio espectral, angiofluoresceínografia e angiografia com indocianina verde foram realizadas em ambos os olhos no período pós-operatório imediato do parto. Imagens da tomografia de coerência óptica de domínio espectral foram obtidas antes do parto e durante o seguimento de 3 meses. A autofluorescência apresentou lesões heterogêneas hiper e hipoautofluorescentes, a angiofluoresceínografia e angiografia com indocianina verde revelaram áreas de isquemia de coroide, e a tomografia de coerência óptica de domínio espectral apresentou desorganização das camadas externas da retina e interrupção da zona elipsóide. Após a estabilização da pressão sanguínea, a recuperação progressiva da camada externa da retina foi monitorada pela tomografia de coerência óptica de domínio espectral.

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A 26-year-old woman presented at 28 weeks gestation with hypertensive choroidopathy associated with pre-eclampsia. Fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography, and indocyanine green angiography were performed in both eyes in the immediate postoperative period. SD-OCT images were obtained before delivery and during a 3-month follow-up. Fundus autofluorescence exhibited patchy hyper- and hypoautofluorescent lesions; fluorescein and indocyanine green angiography revealed areas of choroidal ischemia; and SD-OCT showed disorganization of the outer retinal layers and disruption of the ellipsoid zone. After her blood pressure was stabilized, progressive recovery of the outer retinal layer was monitored on SD-OCT.

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OBJECTIVE: To report our experience using conventional culture methods (CM) and pediatric blood culture bottles (PBCBs) for vitreous sample culture of acute postoperative endophthalmitis. METHODS: A retrospective study was conducted at the Department of Ophthalmology, Hospital das Clinicas, HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, BR, from January 2010 to December 2015, and it included 54 patients with clinically suspected acute postoperative endophthalmitis. Vitreous samples were obtained by vitreous tap or vitrectomy. Samples from January 2010 to December 2011 were cultivated in CM, whereas samples from January 2012 to December 2015 were inoculated in PBCBs. The measured outcome was the yield of positive cultures. RESULTS: Twenty cases were included in the CM group, and 34 cases were included in the PBCB group. The yield of positive cultures in PBCBs (64.7%) was significantly higher than that in conventional CM (35%, p=0.034). Staphylococcus epidermidis and Streptococcus viridans were the two most commonly found agents. CONCLUSION: PBCBs can be used successfully in clinically suspected endophthalmitis. The method showed a higher yield of positive cultures than the conventional method. This technique appears to have several advantages over the traditional method: it saves time, as only one medium needs to be inoculated; transportation to a laboratory is easier than in the traditional method, and there is no need to maintain a supply of fresh agar media. The use of PBCBs may be recommended as the primary method for microbiological diagnosis and is especially suitable for office settings and remote clinics.

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OBJECTIVE: To report our experience using conventional culture methods (CM) and pediatric blood culture bottles (PBCBs) for vitreous sample culture of acute postoperative endophthalmitis. METHODS: A retrospective study was conducted at the Department of Ophthalmology, Hospital das Clinicas, HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, BR, from January 2010 to December 2015, and it included 54 patients with clinically suspected acute postoperative endophthalmitis. Vitreous samples were obtained by vitreous tap or vitrectomy. Samples from January 2010 to December 2011 were cultivated in CM, whereas samples from January 2012 to December 2015 were inoculated in PBCBs. The measured outcome was the yield of positive cultures. RESULTS: Twenty cases were included in the CM group, and 34 cases were included in the PBCB group. The yield of positive cultures in PBCBs (64.7%) was significantly higher than that in conventional CM (35%, p=0.034). Staphylococcus epidermidis and Streptococcus viridans were the two most commonly found agents. CONCLUSION: PBCBs can be used successfully in clinically suspected endophthalmitis. The method showed a higher yield of positive cultures than the conventional method. This technique appears to have several advantages over the traditional method: it saves time, as only one medium needs to be inoculated; transportation to a laboratory is easier than in the traditional method, and there is no need to maintain a supply of fresh agar media. The use of PBCBs may be recommended as the primary method for microbiological diagnosis and is especially suitable for office settings and remote clinics.

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INTRODUCTION: We report a case of retinal and posterior ocular findings in a 33-year-old man diagnosed with Hunter syndrome (Mucopolysaccharidosis type II) in a multimodal imaging way. CASE PRESENTATION: Our patient was complaining of blurred night vision for the past 3 years. He had not received any systemic treatment for Hunter syndrome. Vision acuity was 20/20 in both eyes and corneas were clear. Fundus examination revealed bilateral crowded and hyperemic optic nerve heads (elevated in the ocular ultrasound) and areas of subretinal hypopigmentation. There was hyperautofluorescence at the central fovea and perifovea, and a diffuse bilateral choroidal fluorescence in angiography. Macular SD-OCT showed a thinning of the external retina at the perifovea in both eyes. Visual field testing showed a bilateral ring scotoma. The full field ERG was subnormal, with a negative response in the scotopic phase. Visual Evoked Potencial test and cranial MRI were normal. CONCLUSION: Our multimodal analysis reported here attempted to contribute to the knowledge of the natural history of GAG deposition in the eye, focusing on the retina and retinal pigment epithelium. Defining this natural history is essential for a proper comparison with Hunter patients receiving systemic treatment, thus determining if it can or cannot improve retinal function in humans with this disorder.

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Idiopathic organ transplant chorioretinopathy is a rare disease associated with kidney and heart transplantation. We present a case secondary to liver transplantation including its multimodal imaging, differential diagnosis, and physiopathology discussion.

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The Shaken Baby Syndrome (SBS) is characterized by subdural hematomas (SH), retinal hemorrhages (RH), and multiple fractures of long bones without external evidence of head trauma. Subinternal limiting membrane (ILM) hemorrhage, also known as macular schisis, is a characteristic finding of this entity. There is no guideline on the right time to indicate surgical treatment. This report describes an abused child with massive sub-ILM hemorrhage, which showed spontaneous absorption after less than two months of follow-up. Due to the possible spontaneous resolution, we suggest an initial conservative treatment in cases of sub-ILM hemorrhage related to SBS.

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PURPOSE: To evaluate the effect of a single intravitreal bevacizumab injection on visual acuity, contrast sensitivity and optical coherence tomography-measured central macular thickness in eyes with macular edema from branch retinal vein occlusion. METHODS: Seventeen eyes of 17 patients with macular edema from unilateral branch retinal vein occlusion were treated with a single bevacizumab injection. Patients were submitted to a complete evaluation including best corrected visual acuity, contrast sensitivity and optical coherence tomography measurements before treatment and one and three months after injection. Visual acuity, contrast sensitivity and optical coherence tomography measurements were compared to baseline values. RESULTS: Mean visual acuity measurement improved from 0.77 logMAR at baseline to 0.613 logMAR one month after injection (P=0.0001) but worsened to 0.75 logMAR after three months. Contrast sensitivity test demonstrated significant improvement at spatial frequencies of 3, 6, 12 and 18 cycles/degree one month after injection and at the spatial frequency of 12 cycles/degree three months after treatment. Mean ± standard deviation baseline central macular thickness (552 ± 150 µm) reduced significantly one month (322 ± 127 µm, P=0.0001) and three months (439 ± 179 µm, P=0.01) after treatment. CONCLUSIONS: Bevacizumab injection improves visual acuity and contrast sensitivity and reduces central macular thickness one month after treatment. Visual acuity returns to baseline levels at the 3-month follow-up, but some beneficial effect of the treatment is still present at that time, as evidenced by optical coherence tomography-measured central macular thickness and contrast sensitivity measurements.

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PURPOSE: To evaluate the effect of a single intravitreal bevacizumab injection on visual acuity, contrast sensitivity and optical coherence tomography-measured central macular thickness in eyes with macular edema from branch retinal vein occlusion. METHODS: Seventeen eyes of 17 patients with macular edema from unilateral branch retinal vein occlusion were treated with a single bevacizumab injection. Patients were submitted to a complete evaluation including best corrected visual acuity, contrast sensitivity and optical coherence tomography measurements before treatment and one and three months after injection. Visual acuity, contrast sensitivity and optical coherence tomography measurements were compared to baseline values. RESULTS: Mean visual acuity measurement improved from 0.77 logMAR at baseline to 0.613 logMAR one month after injection (P=0.0001) but worsened to 0.75 logMAR after three months. Contrast sensitivity test demonstrated significant improvement at spatial frequencies of 3, 6, 12 and 18 cycles/degree one month after injection and at the spatial frequency of 12 cycles/degree three months after treatment. Mean ± standard deviation baseline central macular thickness (552 ± 150 µm) reduced significantly one month (322 ± 127 µm, P=0.0001) and three months (439 ± 179 µm, P=0.01) after treatment. CONCLUSIONS: Bevacizumab injection improves visual acuity and contrast sensitivity and reduces central macular thickness one month after treatment. Visual acuity returns to baseline levels at the 3-month follow-up, but some beneficial effect of the treatment is still present at that time, as evidenced by optical coherence tomography-measured central macular thickness and contrast sensitivity measurements.

OBJETIVO: Avaliar o efeito de uma única injeção intravítrea de bevacizumabe na função visual, sensibilidade ao contraste, em olhos com edema macular causado por oclusão de ramo venoso retiniano. MÉTODOS: Dezessete olhos de 17 pacientes com edema macular unilateral causado por oclusão de ramo venoso retiniano foram tratados com uma única injeção intravítrea de bevacizumabe. Pacientes previamente foram submetidos a exame ocular completo, sensibilidade ao contraste, variável de maior interesse, melhor acuidade visual corrigida e tomografia de coerência óptica e foram reavaliados no 1º e 3º mês de seguimento. RESULTADOS: O teste de sensibilidade ao contraste demonstrou melhora significante nas frequências espaciais 3, 6, 12 e 18 ciclos/grau (c/g) no primeiro mês de seguimento e na frequência espacial 12 c/g após o 3º mês de tratamento. A média da acuidade visual melhorou de 0,77 no pré-tratamento para 0,61 logMAR no 1º mês de seguimento (P=0,0001), com piora ao final do 3º mês, 0,75 logMAR. A média da espessura foveal central (552 ± 150 µm) reduziu significantemente no 1º (322 ± 127 µm, P=0,0001) e 3º (439 ± 179 µm, P=0,01) mês de seguimento. CONCLUSÃO: No edema macular causado por oclusão de ramo venoso retiniano, uma única injeção intravítrea de bevacizumabe melhora a sensibilidade ao contraste, acuidade visual e reduz a espessura foveal central após 1 mês de tratamento. Após 3 meses de seguimento, ainda é possível observar benefício com o tratamento, como foi evidenciado pela sensibilidade ao contraste e a medida da espessura foveal central à tomografia de coerência óptica.

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Este trabalho descreve o caso de uma paciente de 41 anos, com diagnóstico da síndrome da hipertensão intracraniana idiopática (síndrome do pseudotumor cerebral) que desenvolveu perda visual em um dos olhos decorrente de membrana neovascular sub-retiniana envolvendo a região macular. Apesar do tratamento clínico adequado e da realização de fenestração da bainha do nervo óptico com boa regressão do edema de papila não houve resolução da membrana neovascular sub-retiniana mesmo após três injeções intravítreas seqüenciais de bevacizumab. Chamamos a atenção para esta complicação incomum do papiledema, revisamos a literatura a este respeito e discutimos as possíveis causas, bem como a evolução mais comum e as modalidades de tratamento disponíveis para tratamento da membrana neovascular sub-retiniana em pacientes com a síndrome do pseudotumor cerebral.

We report a 41-years-old woman with idiopathic intracranial hyperthension (psedotumor cerebri syndrome) that presented with visual loss in one eye due to subretinal neovascular membrane involving the macular area. Despite the introduction of adequate medical treatment followed by optic nerve sheath fenestration that lead to optic disc edema regression there was no improvement in the subretinal neovascular membrane even after performing three sequential intravitreal injection of bevacizumab. We stress the importance of recognizing this uncommon complication of papilledema and review the literature regarding such an occurrence. We also discuss the possible causes for developing subretinal membrane in papilledema, its most common outcome and the treatment modalities available for managing subretinal neovascular membrane in patients with pseudotumor cerebri syndrome.

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A telangiectasia justafoveolar idiopática (TJI) é uma vasculopatia retiniana da idade adulta, pode causar baixa acuidade visual e ser de difícil diagnóstico. Estudou-se os prontuários de 19 pacientes com TJI usando-se a classificaçäo de GASS & BLODI. Observou-se 8 pacientes no grupo 1 (42 p/c), 11 no grupo 2 (58 p/c) e nenhum no grupo 3. Todos os do grupo 1 apresentaram doença unilateral, com as telangiectasias facilmente visíveis, depósitos lipídicos e edema cistóide de mácula. A idade média foi de 55,8 anos, com cinco pacientes do sexo masculino (63 p/c) e três do feminino (37 p/c). Todos os do grupo 2 tinham doença bilateral com telangiectasias evidenciadas apenas à angiofluoresceinografia. A idade média foi de 54,5 anos, com dois

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Os autores descrevem dois casos de uma entidade rara, o osteoma de coróide, complicado por descolamento seroso do epitélio neuro-sensorial da retina e por membrana neovascular subretiniana. Analisam a apresentação clínica, características fundoscópicas e angiográficas, padrão ecográfico e tomográfico. Discutem tratamento das complicações, diagnóstico diferencial e patogênese, revisando a literatura.

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Os autores analisaram 544 casos de CCS, com o objetivo de discutir dados epidemiológicos desta patologia. Observou-se: 1- média de idade de 40,6 anos; 2- predomínio do sexo masculino (81,9 por cento); 3- diferença na distribuiçäo etária de acordo com o sexo. Os homens apresentaram pico de incidência na terceira década com declínio acentuado na sexta década, enquanto que esta distribuiçäo foi mais homogênea em mulheres, com predomínio relativo destas na sexta década; 4- distribuiçäo semelhante entre olhos direito esquerdo; bilateralidade em 12,4 por cento; 5- predomínio acentuado dos casos na raça branca (87,8 por cento); näo houve perfil sazonal na incidência da CCS

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