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PURPOSE: To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease. DESIGN: Multi-institutional consecutive retrospective case series. SUBJECTS: New patients with a diagnosis of IP were seen at the Casey Eye Institute at the Oregon Health and Science University (OHSU), Moran Eye Center, University of Utah, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September 2018. METHODS: Detailed ophthalmoscopic examination and FA were recommended for all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by 2 masked graders on a 3-point scale: 0 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, and 3 = retinal detachment. The presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation. MAIN OUTCOME MEASURES: The proportion of eyes noted to have disease on ophthalmoscopy compared with FA and the severity of retinal disease in those with and without known CNS disease. RESULTS: Retinal pathology was detected in 18 of 35 patients (51%) by indirect ophthalmoscopy and 26 of 35 patients (74%) by FA (P = 0.048) in a predominantly pediatric population (median age, 9 months). Ten patients (29%) had known CNS disease at the time of the eye examination. A Wilcoxon rank-sum test indicated that the retinal severity scores for patients with CNS disease (median, 2) were significantly higher than the retinal severity scores for patients without CNS disease (median, 1), z = -2.12, P = 0.034. CONCLUSIONS: Retinal disease is present in the majority of patients with IP, and ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.
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Doenças do Sistema Nervoso Central , Incontinência Pigmentar , Doenças Retinianas , Humanos , Criança , Lactente , Incontinência Pigmentar/complicações , Incontinência Pigmentar/diagnóstico , Incontinência Pigmentar/epidemiologia , Prevalência , Estudos Retrospectivos , Doenças Retinianas/diagnóstico , Doenças Retinianas/epidemiologia , Doenças Retinianas/etiologia , Retina , Doenças do Sistema Nervoso Central/complicaçõesRESUMO
OBJECTIVE: To evaluate whether pentosan polysulfate maculopathy manifests distinctive imaging features that can be differentiated from those found in age-related macular degeneration (AMD). METHODS: Local databases were queried to identify patients with a diagnosis of interstitial cystitis who were seen at the Emory Eye Center between May 2014 and January 2019 and who had fundus imaging available for review. Ninety patients met the eligibility criteria. Masked graders categorized patients based on imaging characteristics as follows: category 1: pentosan polysulfate maculopathy; category 2: AMD or drusen; category 3: neither; and category 4: unsure. Pentosan polysulfate exposure characteristics were compared among groups. RESULTS: Of the 90 subjects evaluated, 79 (88%) were female and the median age was 61.5 years (range, 30-89). Seventeen patients were placed in category 1; 25 in category 2; 47 in category 3, and; 1 in category 4. Among categories 1 to 4, respectively, 17 (100%), 15 (60%), 28 (60%), and 0 patients had exposure to pentosan polysulfate (pâ¯=â¯0.007). Mean cumulative exposure to pentosan polysulfate across the four categories was 2.1, 0.36, 0.34, and 0 kg, respectively (p < 0.00001). Eyes with pentosan polysulfate maculopathy did not have typical drusen in the macula. CONCLUSION: Although pentosan polysulfate maculopathy resembles some aspects of AMD, the two conditions can be differentiated with the use of multimodal fundus imaging.
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Macula Lutea , Degeneração Macular , Doenças Retinianas , Anticoagulantes , Feminino , Humanos , Degeneração Macular/induzido quimicamente , Degeneração Macular/diagnóstico , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Poliéster Sulfúrico de Pentosana/efeitos adversos , Doenças Retinianas/diagnósticoAssuntos
Infecções Oculares Virais/diagnóstico , Doença pelo Vírus Ebola/diagnóstico , Segmento Posterior do Olho/patologia , Uveíte Posterior/diagnóstico , Adolescente , Adulto , Catarata/diagnóstico , Catarata/virologia , Surtos de Doenças , Ebolavirus/isolamento & purificação , Membrana Epirretiniana/diagnóstico , Membrana Epirretiniana/epidemiologia , Membrana Epirretiniana/virologia , Oftalmopatias/diagnóstico , Oftalmopatias/epidemiologia , Oftalmopatias/virologia , Infecções Oculares Virais/epidemiologia , Infecções Oculares Virais/virologia , Feminino , Doença pelo Vírus Ebola/epidemiologia , Doença pelo Vírus Ebola/virologia , Humanos , Masculino , Pessoa de Meia-Idade , Segmento Posterior do Olho/virologia , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/epidemiologia , Descolamento Retiniano/virologia , Serra Leoa/epidemiologia , Sobreviventes , Uveíte Posterior/epidemiologia , Uveíte Posterior/virologia , Corpo Vítreo/patologia , Adulto JovemRESUMO
PURPOSE: The mainstay empiric treatments of bacterial endophthalmitis are intravitreal vancomycin and ceftazidime. In the United States, up to 10% of the general population has a reported penicillin (PCN) allergy. Despite low cross-reactivity between PCN and later-generation cephalosporins, some providers alter the intravitreal antibiotic choice for endophthalmitis because of concern for allergic reactions. We evaluated the management strategies of infectious endophthalmitis in the setting of self-reported systemic antibiotic allergies and the association with adverse reactions after standard intravitreal antibiotic administration. DESIGN: Single-center, retrospective cohort study. PARTICIPANTS: All patients with endophthalmitis between 2005 and 2019 and documented PCN, PCN-analog, cephalosporin, or vancomycin allergy who received intravitreal antibiotics on the basis of International Classification of Diseases 9th and 10th Revisions, and Current Procedural Terminology codes. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Any allergic reaction after intravitreal injection, additional surgical interventions required for treatment, and visual function at last recorded visit. RESULTS: Of the 65 patients included in this cohort, the most common causes of endophthalmitis were postcataract extraction surgery (n = 23, 35.4%) and postintravitreal injection (n = 11, 16.9%). All patients (65/65) received intravitreal vancomycin, and 81.5% (53/65) received intravitreal ceftazidime. Of the 53 patients who received intravitreal ceftazidime, 46 (86.8%) had allergies to PCNs alone, 5 (9.4%) had a cephalosporin allergy alone, and 2 (3.8%) had reported allergies to both PCN and cephalosporin antibiotics. Two patients (3.1%) with a documented vancomycin allergy received intravitreal vancomycin without complication. No patients exhibited any systemic or local allergic reactions or complications after intravitreal injection. CONCLUSIONS: There were no documented allergic reactions in this cohort of patients with systemic antibiotic allergies who were treated for infectious endophthalmitis. Our findings are consistent with previous reports of a low allergic cross-reactivity between PCN and later-generation cephalosporins. Ophthalmologists should use evidence-based practices and a careful informed consent process when choosing intravitreal antibiotics for patients with specific antibiotic allergies. In the routine patient with suspected bacterial endophthalmitis, PCN allergy may not be an absolute contraindication to intravitreal cephalosporin use.
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Cefalosporinas/uso terapêutico , Hipersensibilidade a Drogas , Endoftalmite/tratamento farmacológico , Infecções Oculares Bacterianas/tratamento farmacológico , Penicilinas/uso terapêutico , Vancomicina/uso terapêutico , Idoso , Antibacterianos/uso terapêutico , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND AND OBJECTIVE: To evaluate the impact of sex differences on the presenting features, treatment patterns, and clinical outcomes in patients with central retinal vein occlusions (CRVO). PATIENTS AND METHODS: This retrospective, longitudinal cohort study included 476 patients diagnosed with CRVO over a 7-year period. Charts were reviewed and clinical data were abstracted. RESULTS: The average age at CRVO onset was lower in males (63.8 years vs. 66.1 years; P = .048). More males (104/224, 54%) had an ischemic CRVO compared to females (113/252, 45%) at 12 months (P = .05). Males with CRVO had a greater central subfield thickness at the baseline (546.7 ± 306.8 µm vs. 438.4 ± 252.9 µm; P = .003) and final visits (343 ± 179.5 µm vs. 304.6 ± 176.2 µm; P = .005). Foveal avascular zone enlargement was more likely in males (39/102, 38% vs. 29/116, 25%; P = .04). CONCLUSIONS: Sex differences exist in presenting features of patients with CRVO. Further inquiry may help provide individualized recommendations for management of CRVO. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:279-285.].
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Inibidores da Angiogênese/administração & dosagem , Oclusão da Veia Retiniana/epidemiologia , Acuidade Visual , Idoso , Feminino , Angiofluoresceinografia , Seguimentos , Fundo de Olho , Humanos , Injeções Intravítreas , Masculino , Pessoa de Meia-Idade , Morbidade/tendências , Oclusão da Veia Retiniana/tratamento farmacológico , Estudos Retrospectivos , Distribuição por Sexo , Fatores Sexuais , Tomografia de Coerência Óptica , Resultado do Tratamento , Estados Unidos/epidemiologiaRESUMO
PURPOSE: To study the relationship between gene expression profile (GEP) subclass and American Joint Committee on Cancer (AJCC) stage in patients with uveal melanoma (UM). METHODS: A retrospective, multicenter study was undertaken with patients entered from nine major ocular oncology centers from across the United States. Three hundred sixty eligible patients had UM and underwent I-125 plaque brachytherapy with concurrent tumor biopsy with GEP testing between January 1, 2010, and October 28, 2014. Patient demographics and UM features were analyzed by both GEP and AJCC status. RESULTS: Gene expression profile class divided the cohort into three groups: Class 1a (n = 186), Class 1b (n = 77), and Class 2 (n = 113). When classified using AJCC staging criteria, we found the following: Stage I in 91 cases (25.3%), Stage IIA in 143 cases (39.7%), Stage IIB in 89 cases (24.7%), Stage IIIA in 36 cases (10%), and Stage IIIB in 1 case (0.3%). There were no Stage IV cases, as lymph node and metastatic data were not collected as a part of this study. Among Stage I tumors, both high tumor height and high largest basal diameter were associated with a higher frequency of Class 2 status (P < 0.05). As UMs progress to a larger AJCC tumor group (T1-T4), the odds ratio of having a worse prognosis based on GEP class was 1.75 (95% CI, 1.36-2.25; P < 0.001). Similarly, as UMs progress to a higher AJCC stage, the odds ratio of having a worse prognosis based on GEP class was 1.69 (95% CI, 1.36-2.10; P < 0.001). CONCLUSION: This report details the differences in clinical features between GEP subclasses and how they are distributed among the AJCC stages. When the tumors were grouped by AJCC staging criteria, both larger AJCC tumor (T) group and worsening AJCC stage were associated with worsening predicted prognosis, based on GEP subclass.
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DNA de Neoplasias/genética , Perfilação da Expressão Gênica/métodos , Melanoma/genética , Estadiamento de Neoplasias , Oftalmologia , Sociedades Médicas , Neoplasias Uveais/genética , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Biópsia por Agulha Fina , Feminino , Humanos , Masculino , Melanoma/diagnóstico , Melanoma/metabolismo , Pessoa de Meia-Idade , Estudos Retrospectivos , Estados Unidos , Neoplasias Uveais/diagnóstico , Neoplasias Uveais/metabolismo , Adulto JovemRESUMO
PURPOSE: To investigate the relationship between surgical approach for intraocular tumor biopsy of uveal melanoma and tumor morphologic features such as size and intraocular location and the effect of these variables on diagnostic yield and biopsy outcome. METHODS: Consecutive patients from nine Ocular Oncology centers with uveal melanoma (UM) undergoing tumor biopsy immediately preceding I125 plaque brachytherapy with tissue sent for gene expression profiling (GEP) testing were reviewed retrospectively. RESULTS: Three hundred sixty patients were included (50% men, mean age 60.2 years). Overall biopsy yield was 99% and 83% for GEP and cytopathology, respectively. Surgeon choice of biopsy approach (trans-vitreal vs. trans-scleral) was found to associate with both tumor location and tumor thickness. A trans-scleral rather than trans-vitreal approach was used more commonly for anteriorly located tumors (92% vs. 38% of posterior tumors, p < 0.001) and thicker tumors (86% vs. 55% of thin tumors, p < 0.001). When performing trans-vitreal biopsies, ocular oncologists with previous vitreoretinal surgery fellowship training were more likely to use wide-field surgical viewing systems, compared with indirect ophthalmoscopy (82.6% vs. 20.6%, p < 0.001). Surgical complications were rare and occurred more frequently with trans-vitreal biopsies (3.6% vs. 0.46%, p = 0.046). CONCLUSIONS: In this multi-center analysis of UM tumor biopsy, surgical yield was high for obtaining tumor tissue for GEP and cytopathology analysis with both trans-scleral and trans-vitreal techniques. Fellowship-trained ocular oncologists' preferred intraocular biopsy techniques associated strongly with tumor location, tumor thickness, and fellowship training of the surgeon. Short-term complication rates were low.
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Biópsia por Agulha Fina/métodos , Braquiterapia/métodos , Perfilação da Expressão Gênica/métodos , Melanoma/diagnóstico , Procedimentos Cirúrgicos Oftalmológicos/métodos , Úvea/patologia , Neoplasias Uveais/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino , Melanoma/genética , Melanoma/terapia , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Neoplasias Uveais/genética , Neoplasias Uveais/terapia , Adulto JovemRESUMO
PURPOSE: Cutaneous melanoma metastatic to the vitreous is very rare. This study investigated the clinical findings, treatment, and outcome of patients with metastatic cutaneous melanoma to the vitreous. Most patients received checkpoint inhibition for the treatment of systemic disease, and the significance of this was explored. DESIGN: Multicenter, retrospective cohort study. PARTICIPANTS: Fourteen eyes of 11 patients with metastatic cutaneous melanoma to the vitreous. METHODS: Clinical records, including fundus photography and ultrasound results, were reviewed retrospectively, and relevant data were recorded for each patient eye. MAIN OUTCOME MEASURES: Clinical features at presentation, ophthalmic and systemic treatments, and outcomes. RESULTS: The median age at presentation of ophthalmic disease was 66 years (range, 23-88 years), and the median follow-up from diagnosis of ophthalmic disease was 23 months. Ten of 11 patients were treated with immune checkpoint inhibition at some point in the treatment course. The median time from starting immunotherapy to ocular symptoms was 17 months (range, 4.5-38 months). Half of eyes demonstrated amelanotic vitreous debris. Five eyes demonstrated elevated intraocular pressure, and 4 eyes demonstrated a retinal detachment. Six patients showed metastatic disease in the central nervous system. Ophthalmic treatment included external beam radiation (30-40 Gy) in 6 eyes, intravitreous melphalan (10-20 µg) in 4 eyes, enucleation of 1 eye, and local observation while receiving systemic treatment in 2 eyes. Three eyes received intravitreous bevacizumab for neovascularization. The final Snellen visual acuity ranged from 20/20 to no light perception. CONCLUSIONS: The differential diagnosis of vitreous debris in the context of metastatic cutaneous melanoma includes intravitreal metastasis, and this seems to be particularly apparent during this era of treatment with checkpoint inhibition. External beam radiation, intravitreous melphalan, and systemic checkpoint inhibition can be used in the treatment of ophthalmic disease. Neovascular glaucoma and retinal detachments may occur, and most eyes show poor visual potential. Approximately one quarter of patients demonstrated ocular disease that preceded central nervous system metastasis. Patients with visual symptoms or vitreous debris in the context of metastatic cutaneous melanoma would benefit from evaluation by an ophthalmic oncologist.
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Antineoplásicos Alquilantes/uso terapêutico , Neoplasias Oculares/tratamento farmacológico , Neoplasias Oculares/secundário , Imunoterapia/métodos , Melanoma/patologia , Melfalan/uso terapêutico , Neoplasias Cutâneas/patologia , Corpo Vítreo/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem , Melanoma Maligno CutâneoRESUMO
PURPOSE: To report a case of necrotic uveal melanoma presenting as orbital cellulitis with an intraocular hemorrhage. OBSERVATIONS: A 61 year-old non-verbal male presented with a two-week history of right eyelid swelling and erythema unresponsive to antibiotics. In addition to these signs of orbital cellulitis, he presented with an opaque media precluding fundus visualization. He was later found to have a collar-button shaped mass consistent with uveal melanoma on B scan ultrasonography during an exam under anesthesia. The patient underwent enucleation with histopathology confirming a necrotic uveal melanoma. CONCLUSION AND IMPORTANCE: This case demonstrates how necrotic uveal melanoma can present as orbital cellulitis and the importance of keeping the diagnosis on the differential.
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BACKGROUND AND OBJECTIVE: To assess the impact of the disorganization of retinal inner layers (DRIL) on visual acuity (VA) and its correlation with ischemic index (IsI) on ultra-widefield fluorescein angiography (UWFFA) in eyes with acute, treatment-naïve branch retinal vein occlusion (BRVO). PATIENTS AND METHODS: Retrospective, longitudinal study of BRVO eyes with 1 year of follow-up or more. Area of intraretinal cysts, DRIL length, extent of disruption of external limiting membrane (ELM), and ellipsoid zone (EZ) were graded on the central 1,000 µm of foveal optical coherence tomography (OCT) scan. Baseline IsI was calculated on UWFFA. RESULTS: Thirty eyes of 30 patients with a mean follow-up of 25.4 months ± 11.0 months were evaluated. At baseline, 50% had DRIL (mean 443.1 µm ± 460.4 µm). DRIL length at baseline was predictive of worse VA at 12 months (P = .029), and DRIL length at 12 months was predictive of worse final VA(P = .011). In multivariate analyses, DRIL length was associated with final VA (P = .008) after controlling for other OCT parameters. There was no association between baseline IsI on UWFFA and DRIL. CONCLUSIONS: DRIL served as an independent OCT biomarker predictive of worse VA during a period of 2 years in acute, treatment-naïve BRVO. Development of DRIL was influenced by presence of CME, intraretinal cyst area, and extent of ELM and EZ disruption, but not by severity of baseline IsI. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:354-364.].
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Retina/patologia , Oclusão da Veia Retiniana/fisiopatologia , Acuidade Visual/fisiologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Angiofluoresceinografia/métodos , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tomografia de Coerência Óptica/métodosRESUMO
PURPOSE: To examine the association between GATA2 deficiency-related myelodysplastic syndrome (MDS) and central retinal vein occlusion (CRVO). METHODS: Clinical ophthalmologic examination and laboratory work-up was performed for a patient with GATA2 deficiency-related MDS who experienced a unilateral CRVO. The literature was reviewed for reports of CRVO in the setting of MDS and allogeneic hematopoietic stem cell transplantation. RESULTS: Ophthalmologic examination revealed findings consistent with unilateral CRVO. Typical hypercoagulable work-up did not reveal an identifiable cause. A review of the patient's medical history revealed multiple potential causes of CRVO, including drug-induced and/or related to her history of MDS and hematopoietic stem cell transplantation. The literature was reviewed for reports of CRVO in association with her risk factors. CONCLUSION: On review of the literature, we determined that GATA2 deficiency-related MDS was a possible cause of this patient's CRVO although it is likely that her multiple risk factors worked synergistically to create a prothrombotic state. This case and review of the literature serve as an important reminder of the long and ever-evolving list of risk factors for the development of CRVO.
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Deficiência de GATA2/complicações , Fator de Transcrição GATA2/genética , Oclusão da Veia Retiniana/etiologia , Adolescente , Evolução Fatal , Feminino , Humanos , Mutação de Sentido IncorretoRESUMO
PURPOSE: To study the relationship between gene expression profile subclass and clinical features in a multicenter cohort of patients with uveal melanoma. METHODS: A retrospective, multicenter study was undertaken with patients entered from nine major ocular oncology centers from across the United States. Eligible patients had uveal melanoma and underwent I-125 plaque brachytherapy with concurrent tumor biopsy with gene expression profile testing between January 1, 2010, and October 28, 2014. Data were collected regarding patient demographics, baseline tumor clinical features, and gene expression profile results. Statistical analyses were performed using the Fisher's exact test, Wilcoxon rank-sum test, Kruskal-Wallis test, and proportional-odds cumulative logit modeling. RESULTS: Inclusion criteria were met for 379 patients. Gene expression profile class divided the cohort into two main groups, Class 1 (n = 263) and Class 2 (n = 113). Class 1 tumors were further subdivided into Class 1a (n = 186) and Class 1b (n = 77). The differences between Class 1 and Class 2 tumors were similar to previous studies, except the finding of Class 2 tumors being more likely to have associated exudative retinal detachment (P < 0.001). There was no statistically significant difference between Class 1 and Class 2 tumors based on the presence of lipofuscin, drusen, or subretinal fluid. Class 1a tumor patients, compared with Class 1b, were significantly older (P = 0.034). Class 2 tumors, when compared with Class 1b, were associated with increasing patient age (P < 0.001), larger tumor height (P = 0.010), ciliary body involvement (P = 0.001), exudative retinal detachment (P = 0.024), and anterior tumor location (P < 0.001). When the tumors were grouped into Collaborative Ocular Melanoma Study size categories, increasing tumor size category was significantly associated with Class 2 status: 6% of small tumors, 32% of medium tumors, and 53% of large tumors were Class 2. CONCLUSION: In a multi-institutional setting, we found that the only significant difference in clinical features between Class 1a and Class 1b tumors was that patients with Class 1a tumors were older at the time of diagnosis. We also found that Class 1a and Class 1b have clinical features distinct from Class 2 tumors. The distribution of the gene expression profile subclasses among the size groups was similar to reported time-to-metastasis data among the same size groupings. Our clinical findings support the current molecular classification-based survival data previously reported in uveal melanoma.
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Biomarcadores Tumorais/genética , Perfilação da Expressão Gênica/métodos , Melanoma/diagnóstico , Estadiamento de Neoplasias , Neoplasias Uveais/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/biossíntese , Biópsia por Agulha Fina , Feminino , Seguimentos , Humanos , Masculino , Melanoma/genética , Melanoma/metabolismo , Pessoa de Meia-Idade , Estudos Retrospectivos , Ultrassonografia , Neoplasias Uveais/genética , Neoplasias Uveais/metabolismo , Adulto JovemRESUMO
INTRODUCTION: The largest Ebola virus (EBOV) outbreak occurred from 2013 - 2016 in West Africa and consequently resulted in the largest cohort of Ebola virus disease (EVD) survivors to date. Ocular disease is among the most common sequelae reported in EVD survivors. This review discusses the prevalence, manifestations, pathogenesis, diagnosis and management of EVD-related ocular disease. AREAS COVERED: An extensive review of the literature was performed to detail the prevalence and manifestations of EVD-related ocular disease. We also review current eye screening and treatment strategies and our current understanding and approach to invasive ophthalmic procedures including surgery. EXPERT OPINION: The ocular sequelae of EVD can lead to vision impairment or blindness, if untreated. Keys to the prevention of such an outcome include timely evaluation and access to appropriate ophthalmic care. The persistence of EBOV in the eye and other immune-privileged sites is the subject of ongoing investigation, but should not be a barrier to care if appropriate screening and biosafety measures are taken. Improved understanding of the pathogenesis of this condition and ongoing clinical care are needed for EVD survivors at-risk for ocular complications.
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"The EVICT study was the first study to demonstrate a step-wise approach on how to safely screen EVD survivors for cataract surgery, providing evidence that vision restoration though surgical management was safe and feasible in this cohort of EVD survivors".
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PURPOSE: To determine whether disorganization of retinal inner layers (DRIL) on optical coherence tomography (OCT) is associated with ischemia on ultra-widefield fluorescein angiography (UWFFA) and with visual outcomes in eyes with acute, treatment-naïve central retinal vein occlusion (CRVO). DESIGN: Retrospective, single-institution, longitudinal cohort study. PARTICIPANTS: Twenty-five consecutive patients with treatment-naïve CRVO and ≥ 1 year follow-up. METHODS: Two independent masked graders evaluated the extent of DRIL, ellipsoid zone disruption, external limiting membrane disruption, and other OCT parameters at the baseline, 6- month, 12-month, and final visits. Baseline UWFFA images were assessed for ischemic index values and foveal avascular zone (FAZ) enlargement. MAIN OUTCOME MEASURES: Associations of DRIL with UWFFA findings and clinical outcomes including corrected visual acuity (VA). RESULTS: The median time to final follow-up was 24 months (range 12.1 - 43.9 months). Median DRIL extent at baseline was 765 µm (range 0 - 1000 µm). Eighteen of 25 eyes (72%) had some degree of DRIL at baseline, and 20 of 25 eyes (80%) had cystoid macular edema (CME). Neither the presence nor extent of DRIL at baseline was associated with presenting VA. In a cross-sectional analysis of each visit, extent of DRIL correlated with worse VA at both the 6-month (ρ = 0.656; p = 0.001) and final (ρ = 0.509; p = 0.016) visits. At final follow-up, DRIL extent was the OCT parameter most strongly correlated with baseline ischemic index (ρ = 0.418; p = 0.047) and baseline enlarged FAZ (p = 0.057) on UWFFA. On multivariate regression analysis, DRIL extent at final follow-up was the only OCT parameter associated with worse VA (p = 0.013) and remained significant when accounting for CME as a potential confounder. CONCLUSIONS: Extent of DRIL was not associated with presenting VA in treatment-naïve eyes with acute CRVO. Following six months of follow-up however, DRIL extent correlated with worse VA and was predictive of worse VA throughout more than 2 years of follow-up. Ischemic features on UWFFA at baseline are predictive of the extent of DRIL development at final follow-up.
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BACKGROUND/AIMS: Plaque brachytherapy is currently the most common treatment for uveal melanoma and has many known potential complications. Here we present 3 cases of early conjunctival and scleral necrosis following iodine-125 plaque. METHODS: This study was conducted as a retrospective case series. RESULTS: We identified 3 cases of early conjunctival and scleral necrosis following iodine-125 plaque. All patients were managed conservatively with resolution of the necrosis. CONCLUSIONS: While delayed corneoscleral necrosis following plaque brachytherapy has been previously reported, occurring many months to years after treatment, the 3 cases in this series presented within 2-6 weeks in the postoperative period. While we were unable to identify a specific etiology, we believe this represents a distinct clinical entity of post-brachytherapy cornea-scleral necrosis that is important to recognize. Possible causes include acute radiation toxicity, mechanical trauma, and/or conjunctival microinfection.
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A 5-year-old girl with Aicardi syndrome and microphthalmia with cyst of the OD presented with progressive enlargement of the cyst causing pain. Microophthalmia with inferior cyst (35 × 25 × 12 mm) was noted at birth, and Aicardi syndrome was diagnosed at 10 months by the presence of the classic triad of callosal agenesis, infantile spasms, and chorioretinal lacunae. She underwent enucleation with cyst resection, and subsequent reconstruction with a dermis fat graft. Histopathologic study revealed adenocarcinoma of the pigmented ciliary epithelium. Full-body metastatic workup was negative. Adenocarcinoma of the pigmented ciliary epithelium is an extremely rare eye tumor with only 4 documented cases in the literature, none arising in a microophthalmic eye with cyst. Aicardi syndrome is also a rare disease that has been associated with increased incidence of malignancy and ocular abnormalities, but has never been described in association with microophthalmia with cyst or with adenocarcinoma of the pigmented ciliary epithelium. Herein, the authors present a review of the case and relevant literature.
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Adenocarcinoma/patologia , Síndrome de Aicardi/complicações , Corpo Ciliar/patologia , Cistos/patologia , Neoplasias Oculares/patologia , Microftalmia , Pré-Escolar , Feminino , HumanosRESUMO
Dislocated nuclear material may be present in the vitreous cavity following complicated cataract surgery, closed globe injury, or spontaneous dislocation of the crystalline lens. The authors report herein a method to remove larger pieces of retained nuclear material during vitrectomy surgery with a retractable basket made from nitinol referred to as the "Frag Bag" (patent pending). Although originally designed for cystoscopic removal of kidney stones, this instrument fits easily through a 23-gauge vitrectomy port. The basket allows for retrieval of larger pieces of retained nuclear material with stabilization of the lens material in the mid-vitreous cavity and softening of the nuclear material to allow efficient and safe removal with the vitreous cutter, away from the retinal surface. Use of the Frag Bag may potentially improve the safety and efficiency of a pars plana lensectomy by obviating the need for a phacofragmatome and decreasing the number of times instruments are brought close to the retinal surface. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:1006-1008.].