MASSIVE RETINAL NEOVASCULARIZATION IN VON HIPPEL-LINDAU DISEASE: ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR, VITRECTOMY, IMMUNOHISTOCHEMISTRY, AND OPTICAL COHERENCE TOMOGRAPHY FEATURES.

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.

MULTIMODAL IMAGING OF FOCAL CHOROIDAL EXCAVATION COMPLICATED BY CHOROIDAL NEOVASCULARIZATION IN A PATIENT WITH ANGIOID STREAKS AND PSEUDOXANTHOMA ELASTICUM.

PURPOSE: To describe a case of focal choroidal excavation (FCE) complicated with Type-2 choroidal neovascularization (CNV) in a patient with angioid streaks secondary to pseudoxanthoma elasticum before and after treatment with bevacizumab. METHODS: Fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography (SD-OCT) and SD-OCT angiography were performed in a 60-year-old white woman with angioid streaks and bilateral FCE. Spectral domain OCT images were taken before and after three-monthly intravitreal injections of bevacizumab. Histopathological analysis of the dermis established the diagnosis of pseudoxanthoma elasticum. RESULTS: Multimodal imaging revealed bilateral FCE and CNV. Spectral domain OCT diagnosed bilateral FCE and Type-1 and Type-2 CNV in the right eye, and irregular vascular network, along macular streak, in both eyes, which were not observed on fluorescein neither on indocyanine green angiography, but only on SD-OCT angiography. Patient presented good anatomical and functional response to intravitreal injections of bevacizumab. CONCLUSION: Focal choroidal excavation is a rare condition detected mainly by SD-OCT, which may be associated with angioid streaks secondary to pseudoxanthoma elasticum and complicated by CNV. Multimodal imaging is important for diagnosis and follow-up of such patients, even in the absence of signs of CNV, and anatomical and functional response to anti-vascular endothelial growth factor therapy is good.

An innovative proposal for anophthalmic cavity reconstruction surgery with bone cement: a case report / Proposta inovadora para reconstrução de cavidades anoftálmicas utilizando cimento ósseo: um relato de caso

ABSTRACT We present a patient who underwent evisceration surgery after spontaneous rupture of the ocular globe due to long-data uncontrolled glaucoma, with posterior placement of an orbital implant made of a bone cement compound based on polymethylmethacrylate as alternative materials were not available. Such a compound is characterized by excellent biocompatibility and low cost, which makes it an interesting alternative for treatment. The anophthalmic socket was successfully filled, providing proper esthetic results and favorable conditions for the posterior scleral prosthesis implantation. No complications were observed during 10 months of follow-up. We believe that, in the absence of alternative materials, low-cost materials may be used in emergency settings to repair anophthalmic cavities and provide satisfactory functional and esthetic outcomes.

RESUMO Apresentamos um paciente que foi submetido à cirurgia de evisceração após ruptura espontânea do globo ocular devido a glaucoma não controlado de longa data, com posterior colocação de implante orbital feito de cimento ósseo, composto à base de polimetilmetacrilato, diante da indisponibilidade de materiais alternativos. Tal composto se caracteriza pela excelente biocompatibilidade e baixo custo, o que o torna uma alternativa interessante para o tratamento. A cavidade anoftálmica foi preenchida com sucesso, fornecendo resultados estéticos adequados e condições favoráveis para o implante posterior de prótese escleral. Nenhuma complicação foi observada durante os 10 meses de seguimento. Acredi­tamos que, na ausência de materiais alternativos, materiais de baixo custo podem ser usados em situações emergenciais para preencher cavidades anoftálmicas e prover resultados funcionais e estéticos satisfatórios.

An innovative proposal for anophthalmic cavity reconstruction surgery with bone cement: a case report.

We present a patient who underwent evisceration surgery after spontaneous rupture of the ocular globe due to long-data uncontrolled glaucoma, with posterior placement of an orbital implant made of a bone cement compound based on polymethylmethacrylate as alternative materials were not available. Such a compound is characterized by excellent biocompatibility and low cost, which makes it an interesting alternative for treatment. The anophthalmic socket was successfully filled, providing proper esthetic results and favorable conditions for the posterior scleral prosthesis implantation. No complications were observed during 10 months of follow-up. We believe that, in the absence of alternative materials, low-cost materials may be used in emergency settings to repair anophthalmic cavities and provide satisfactory functional and esthetic outcomes.

Spontaneous outer retinal layer recovery in a case of hypertensive choroidopathy secondary to pre-eclampsia: a multimodal evaluation / Recuperação espontânea das camadas externas da retina em um caso de coroidopatia hipertensiva secundária a pré-eclâmpsia: avaliação multimodal

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.

Spontaneous outer retinal layer recovery in a case of hypertensive choroidopathy secondary to pre-eclampsia: a multimodal evaluation.

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.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts / Facoemulsificação e colírio de atropina a 1% para tratamento de microcoria congênita antimetrópica associada a catarata

ABSTRACT A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts. Phacoemulsification was performed using an iris expansion device and the anterior capsule was stained using the "trypan down under" technique. Preoperative considerations, the surgical approach, and postoperative management are discussed.

RESUMO Um caso raro de microcoria congênita bilateral associada à antimetropia em um homem de 47 anos de idade é descrito aqui. O paciente queixava-se de perda visual progressiva em seu olho direito nos últimos 5 anos. Um exame com lâmpada de fenda e biomicroscopia ultrassônica confirmaram microcoria congênita e catarata. A facoemulsificação foi realizada usando dispositivo de expansão iriana, e a cápsula anterior foi corada através da técnica "trypan down under". Considerações pré-operatórias, abordagem cirúrgica e manejo pós-operatório são discutidos.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts.

A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts. Phacoemulsification was performed using an iris expansion device and the anterior capsule was stained using the "trypan down under" technique. Preoperative considerations, the surgical approach, and postoperative management are discussed.

Diagnostic value of pediatric blood culture bottles for acute postoperative endophthalmitis.

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.

Diagnostic value of pediatric blood culture bottles for acute postoperative endophthalmitis

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.

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.