Full-field electroretinogram behavior in Vogt-Koyanagi-Harada disease: a 24-month longitudinal study in patients from acute onset evaluated with multimodal analysis.

PURPOSE: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset. METHODS: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases. Inflammation was systematically evaluated with clinical and posterior segment imaging (PSI) exams (fluorescein angiography, FA, indocyanine green angiography, ICGA, enhanced depth imaging optical coherence tomography, EDI-OCT). A ff-ERG was performed upon enrollment as well as at predefined intervals. Scotopic ff-ERG parameters changes between the 12th and 24th months defined the ERG-stable or ERG-worsening groups. "Flare" was defined as an appearance or worsening of inflammatory signs (after the initial 6 months following disease onset) under the predefined treatment protocol. RESULTS: ff-ERG parameters initially improved in all eyes; in the evaluation between the 12th and 24th months, ff-ERG results were stable in 17 eyes (71 %) and worsened in 7 eyes (29 %). Subnormal ff-ERG results were observed in 15 eyes (62 %) at the 24th month. On the other hand, the flare was observed in 8 eyes (33 %) as cells in the anterior chamber and in 24 eyes (100 %) as any PSI inflammatory sign. The ERG-worsening group presented thicker subfoveal choroid at the first month (p = 0.001) and fluctuations in choroidal thickness more often during follow-up when compared to the ERG-stable group (p = 0.02). CONCLUSIONS: Scotopic ff-ERG parameters worsened between the 12th and 24th months in a quarter of the patients. Subclinical inflammation detected as an increase in CT seems to be related to worsening in visual function measured with ffERG.

Acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) associated with choroidal melanoma.

BACKGROUND: To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. CASE PRESENTATION: A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. CONCLUSIONS: We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.

Paracentral acute middle maculopathy in patient with atherosclerosis and angioplasty with stents in the coronary artery.

Sophisticated imaging systems have helped to redefine the clinical presentation of acute macular neuroretinopathy and have markedly enhanced diagnostic sensitivity. The proposed mechanism of paracentral acute middle maculopathy is related to ischemia at the level of the superficial and deep retinal capillary plexi. This is a case report of a patient who developed an acute macular neuroretinopathy after an uneventful angioplasty with stents in the coronary artery.

Novel low-cost approach to the treatment of ocular surface squamous neoplasia using pattern scanning laser photocoagulation.

PURPOSE: To evaluate the safety and 12-month effect of treatment with pattern scanning laser photocoagulation for ocular surface squamous neoplasia in a low-resource setting with extremely limited access to an operating room. METHODS: Adult patients with a clinical diagnosis of ocular surface squamous neoplasia underwent a complete ophthalmologic examination. After topical anesthesia and instillation of toluidine blue 1%, the lesion was treated using pattern scanning photocoagulation for a duration time that varied from 20 to 100 ms and power from 600 to 1,800 mW. Patients were examined on a weekly basis for the first month and underwent weekly retreatment of the remaining lesions, as necessary. Patients had a minimum follow-up of 12 months. RESULTS: Thirty-eight patients (38 eyes) were included. All patients had clinical ocular surface squamous neoplasia that was confirmed by impression cytology. The age of patients ranged from 40 to 83 years (average: 65.5 years) and 28 of them were males (74%). The patients were divided into two groups: group I (immunocompetent) and group II (immuno-suppressed). In group I, 23 patients (74%) presented complete response with lesion control after laser treatment alone. In group II, two of seven patients (28%) showed treatment response during the follow-up. The average number of treatments was 2.5 (one to six laser treatments). Procedures were well tolerated. CONCLUSION: Short-term results of the laser photocoagulation approach for the treatment of ocular surface squamous neoplasia conjunctival lesions were favorable, with a 74% success rate observed in immunocompetent patients. This novel strategy is a less resource-intensive alternative that could demonstrate its usefulness in settings with shortages in operating rooms and in recurrent cases. Studies with longer follow-ups and larger sample sizes are warranted to confirm our findings and evaluate the effectiveness of laser treatment in association with topical chemotherapy.

Frosted branch angiitis and cerebral venous sinus thrombosis as an initial onset of neuro-Behçet's disease: a case report and review of the literature.

BACKGROUND: Frosted branch angiitis is a rare, severe condition. It can be either a primary or a secondary condition and is characterized by rapid deterioration of vision and fulminant retinal vasculitis that manifests as diffuse sheathing of retinal vessels, macular edema, papillitis, vitritis and anterior uveitis. We aimed to describe a case of frosted branch angiitis and cerebral venous sinus thrombosis as an initial neuro-Behçet's disease onset. Diagnosis of Behçet's disease was based on the current 2014 International Criteria for Behçet's Disease and the International consensus recommendation criteria for neuro-Behçet's disease. In addition, a literature review using search parameters of "frosted branch angiitis", "Behçet" and "neuro-Behçet" in the PubMed database is presented. CASE PRESENTATION: A 28-year-old Brazilian pardo woman presented to our hospital with abrupt bilateral vision loss associated with recurrent aphthous oral ulcers 6 months before visual symptom onset. A fundus examination showed bilateral widespread retinal vasculitis with venous and arterial white sheathing, optic disc swelling, macular edema, and retinal hemorrhages, leading to the diagnosis of frosted branch angiitis. An extensive systemic workup for retinal vasculitis was uneventful, except for brain magnetic resonance imaging demonstrating cerebral venous sinus thrombosis and lymphocytic aseptic meningitis. A diagnosis of neuro-Behçet's disease was made, and treatment was started with methylprednisolone therapy 1 g/day for 5 consecutive days, followed by oral mycophenolate mofetil and infliximab 5 mg/kg infusion. The patient's response was rapid, with improvement of visual acuity to hand movement and counting fingers by day 7 and final visual acuity of counting fingers and 20/130. CONCLUSIONS: Frosted branch angiitis may be associated with infectious, noninfectious, or idiopathic causes. An extensive workup should be done to exclude systemic vasculitis such as Behçet's disease. Treatment with systemic steroids must be promptly initiated in association with specific treatment aimed at inflammation control and blindness risk reduction.

Paracentral acute middle maculopathy in patient with atherosclerosis and angioplasty with stents in the coronary artery / Maculopatia média aguda paracentral em paciente com arterioesclerose e angioplastia com stent na artéria coronária

ABSTRACT Sophisticated imaging systems have helped to redefine the clinical presentation of acute macular neuroretinopathy and have markedly enhanced diagnostic sensitivity. The proposed mechanism of paracentral acute middle maculopathy is related to ischemia at the level of the superficial and deep retinal capillary plexi. This is a case report of a patient who developed an acute macular neuroretinopathy after an uneventful angioplasty with stents in the coronary artery.

RESUMO Sistemas de imagem sofisticados ajudaram a redefinir a apresentação clínica da neurorretinopatia macular aguda e têm sensibilidade diagnóstica marcadamente aumentada. A maculopatia média aguda paracentral tem sido relacionada à isquemia ao nível dos plexos capilares superficial e profundo da retina. Este é um relato de caso de paciente que desenvolveu uma neurorretinopatia macular aguda após uma cirurgia de angioplastia com stents da artéria coronária sem complicações.

Mycophenolate mofetil as an immunomodulator in refractory noninfectious uveitis.

PURPOSE:: To evaluate the efficacy and tolerance of mycophenolate mofetil (MMF) for the treatment of noninfectious uveitis using the methods advocated by the Standardization of Uveitis Nomenclature (SUN) Working Group and to compare this with other studies of immunosuppression in ocular inflammation. METHODS:: Retrospective case series. Patients with noninfectious uveitis, followed at a tertiary Uveitis Service in São Paulo, Brazil, from 2007 to 2014 and receiving oral MMF for a minimum of 6 months, were retrospectively reviewed. After reaching an optimal dose of MMF, patients were evaluated after 6 (T6), 12 (T12), and 24 months (T24). The optimal dose varied for each patient (medium 2.2 g/day, range 1.0-3.0 g/day). The main outcome measures were: 1) success on achieving complete control of inflammation in both eyes and/or oral prednisone dosage reduction to ≤10 mg per day, and 2) the length of time required to reduce oral prednisone to ≤10 mg/day, partial control of ocular inflammation, and side effects. RESULTS:: In a cohort of 16 patients with refractory noninfectious uveitis, 67% reached the ideal prednisone dose after 1 year of MMF treatment and 83% after 2 years of MMF treatment. Complete or partial inflammation control was achieved in 43.7% at T12. Two patients (14%) had disease remission after 4.7 years of MMF treatment. Adverse effects were gastrointestinal disturbances, infection, insomnia, and liver function abnormalities at a rate of 0.03 patient-year each. CONCLUSIONS:: This small retrospective case series is consistent with the literature concerning the high efficacy and moderate tolerability of MMF in noninfectious uveitis. Observation of patients should be continued for at least 1 year to clearly determine MMF efficacy.

Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes.

Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1*0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration. The current review discusses the general features of VKHD, including epidemiology, classification into categories, differential diagnosis and current therapeutic approaches.

Novel low-cost approach to the treatment of ocular surface squamous neoplasia using pattern scanning laser photocoagulation / Nova abordagem de baixo custo para neoplasia escamosa da superficie ocular usando fotocoagulação tipo pattern scanning

ABSTRACT Purpose: To evaluate the safety and 12-month effect of treatment with pattern scanning laser photocoagulation for ocular surface squamous neoplasia in a low-resource setting with extremely limited access to an operating room. Methods: Adult patients with a clinical diagnosis of ocular surface squamous neoplasia underwent a complete ophthalmologic examination. After topical anesthesia and instillation of toluidine blue 1%, the lesion was treated using pattern scanning photocoagulation for a duration time that varied from 20 to 100 ms and power from 600 to 1,800 mW. Patients were examined on a weekly basis for the first month and underwent weekly retreatment of the remaining lesions, as necessary. Patients had a minimum follow-up of 12 months. Results: Thirty-eight patients (38 eyes) were included. All patients had clinical ocular surface squamous neoplasia that was confirmed by impression cytology. The age of patients ranged from 40 to 83 years (average: 65.5 years) and 28 of them were males (74%). The patients were divided into two groups: group I (immunocompetent) and group II (immuno­suppressed). In group I, 23 patients (74%) presented complete response with lesion control after laser treatment alone. In group II, two of seven patients (28%) showed treatment response during the follow-up. The average number of treatments was 2.5 (one to six laser treatments). Procedures were well tolerated. Conclusion: Short-term results of the laser photocoagulation approach for the treatment of ocular surface squamous neoplasia conjunctival lesions were favorable, with a 74% success rate observed in immunocompetent patients. This novel strategy is a less resource-intensive alternative that could demonstrate its usefulness in settings with shortages in operating rooms and in recurrent cases. Studies with longer follow-ups and larger sample sizes are warranted to confirm our findings and evaluate the effectiveness of laser treatment in association with topical chemotherapy.

RESUMO Objetivo: Avaliar a segurança e o efeito de 12 meses de tratamento com fotocoagulação pelo pattern scanning laser para neoplasia escamosa da superfície ocular em um ambiente com poucos recursos e acesso extremamente limitado a um tratamento cirúrgico. Métodos: Pacientes adultos com diagnóstico de neoplasia escamosa de superfície ocular foram submetidos a exame oftalmológico completo. Após anestesia tópica e instilação de azul de toluidina 1%, a lesão foi tratada com laser por um tempo de duração que variou de 20 a 100 ms e potência de 600 a 1800 mW. Os pacientes foram examinados semanalmente durante o primeiro mês e fo­ram retratados semanalmente das lesões restantes, conforme necessário. Os pacientes tiveram um seguimento mínimo de 12 meses. Resultados: Trinta e oito pacientes (38 olhos) foram incluídos no estudo. Todos os pacientes apresentaram neoplasia escamosa da superfície ocular clínica, confirmada por citologia de impressão. A idade dos pacientes variou entre 40 e 83 anos (média de 65.5 anos) e 28 deles eram do sexo masculino (74%). Os pacientes foram divididos em dois grupos: Grupo I (imunocompetente) e grupo II (imunossuprimido). No grupo I, 23 pacientes (74%) apresentaram resposta completa com o controle da lesão após o tratamento com laser. No grupo II, dois dos sete pacientes (28%) apresentaram resposta ao tratamento durante o acompanhamento. A média de aplicações de laser foi de 2,5 (1 a 6 aplicações). Os procedimentos foram bem tolerados. Conclusões: Os resultados a curto prazo da abordagem de fotocoagulação a laser para o tratamento das lesões conjuntivais de neoplasia escamosa de superfície ocular foram favoráveis, com uma taxa de sucesso de 74% observada em pacientes imunocompetentes. Essa nova estratégia é uma alternativa menos intensiva em recursos que pode demonstrar sua utilidade em ambientes com escassez de salas cirúrgicas e em casos recorrentes. Estudos com acompanhamentos mais longos e amostras maiores são necessários para confirmar nossos achados e avaliar a eficácia do tratamento a laser asso­ciado à quimioterapia tópica.

Mycophenolate mofetil as an immunomodulator in refractory noninfectious uveitis / Micofenolato mofetila como agente imunomodulador no tratamento das uveítes não infecciosas refratárias

ABSTRACT Purpose: To evaluate the efficacy and tolerance of mycophenolate mofetil (MMF) for the treatment of noninfectious uveitis using the methods advocated by the Standardization of Uveitis Nomenclature (SUN) Working Group and to compare this with other studies of immunosuppression in ocular inflammation. Methods: Retrospective case series. Patients with noninfectious uveitis, followed at a tertiary Uveitis Service in São Paulo, Brazil, from 2007 to 2014 and receiving oral MMF for a minimum of 6 months, were retrospectively reviewed. After reaching an optimal dose of MMF, patients were evaluated after 6 (T6), 12 (T12), and 24 months (T24). The optimal dose varied for each patient (medium 2.2 g/day, range 1.0-3.0 g/day). The main outcome measures were: 1) success on achieving complete control of inflammation in both eyes and/or oral prednisone dosage reduction to ≤10 mg per day, and 2) the length of time required to reduce oral prednisone to ≤10 mg/day, partial control of ocular inflammation, and side effects. Results: In a cohort of 16 patients with refractory noninfectious uveitis, 67% reached the ideal prednisone dose after 1 year of MMF treatment and 83% after 2 years of MMF treatment. Complete or partial inflammation control was achieved in 43.7% at T12. Two patients (14%) had disease remission after 4.7 years of MMF treatment. Adverse effects were gastrointestinal disturbances, infection, insomnia, and liver function abnormalities at a rate of 0.03 patient-year each. Conclusions: This small retrospective case series is consistent with the literature concerning the high efficacy and moderate tolerability of MMF in noninfectious uveitis. Observation of patients should be continued for at least 1 year to clearly determine MMF efficacy.

RESUMO Objetivo: Avaliar a eficácia e tolerância do micofenolato de mofetila (MMF) para o tratamento das uveítes não infecciosas refratárias, utilizando os métodos de análises definidos pelo "Standardization of Uveitis Nomenclature Working Group." Método: Estudo retrospectivo de série de casos. Foram incluídos pacientes com uveíte não infecciosa, em tratamento oral com MMF por um período mínimo de seis meses, acompanhados no Serviço de Uveítes, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brasil, no período de 2007 a 2014. Todos os pacientes faziam uso de pelo menos um imunossupressor e apresentavam doença ocular ativa. Os pacientes foram avaliados aos seis meses (T6), 12 meses (T12) e 24 meses (T24) após atingir a dose ótima do MMF. A média da dose ótima foi 2,2g/dia (intervalo 1,0-3,0g/dia). Os principais desfechos analisados foram: 1) Sucesso no controle total da inflamação em ambos os olhos e/ou redução da dose de prednisona oral para ≤10 mg/dia; 2) Intervalo até a redução da prednisona oral para ≤10 mg/dia, controle parcial de inflamação ocular e efeitos adversos. Resultados: Na presente coorte com 16 pacientes com uveíte não infecciosa refratária, observou-se 67% e 83% de probabilidade de alcançar a dose ideal de prednisona em T12 e T24, respectivamente. Controle total ou parcial da inflamação foi observado em 43,7% dos pacientes em T12. Dois pacientes (14%) tiveram remissão da doença após 4,7 anos do início de MMF. Os efeitos adversos foram distúrbios gastrintestinais, infecção, insônia e anormalidade da função hepática com 0,03 eventos paciente-ano (PPY) respectivamente. Conclusões: Esta pequena série retrospectiva de casos ratifica os achados na literatura sobre a alta eficácia e tolerância moderada de MMF em uveítes não infecciosas. Uma importante observação é que, para melhor avaliar a eficácia do MMF, deve se esperar o intervalo mínimo de um ano.