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1.
Ophthalmic Plast Reconstr Surg ; 34(3): 280-283, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28661930

RESUMO

PURPOSE: The authors sought to compare the clinical outcomes of simultaneous versus sequential ptosis and strabismus surgery in children. METHODS: Retrospective, single-center cohort study of children requiring both ptosis and strabismus surgery on the same eye. Simultaneous surgeries were performed during a single anesthetic event; sequential surgeries were performed at least 7 weeks apart. Outcomes were ptosis surgery success (margin reflex distance 1 ≥ 2 mm, good eyelid contour, and good eyelid crease); strabismus surgery success (ocular alignment within 10 prism diopters of orthophoria and/or improved head position); surgical complications; and reoperations. RESULTS: Fifty-six children were studied, 38 had simultaneous surgery and 18 sequential. Strabismus surgery was performed first in 38/38 simultaneous and 6/18 sequential cases. Mean age at first surgery was 64 months, with mean follow up 27 months. A total of 75% of children had congenital ptosis; 64% had comitant strabismus. A majority of ptosis surgeries were frontalis sling (59%) or Fasanella-Servat (30%) procedures. There were no significant differences between simultaneous and sequential groups with regards to surgical success rates, complications, or reoperations (all p > 0.28). CONCLUSIONS: In the first comparative study of simultaneous versus sequential ptosis and strabismus surgery, no advantage for sequential surgery was seen. Despite a theoretical risk of postoperative eyelid malposition or complications when surgeries were performed in a combined manner, the rate of such outcomes was not increased with simultaneous surgeries. Performing ptosis and strabismus surgery together appears to be clinically effective and safe, and reduces anesthesia exposure during childhood.


Assuntos
Blefaroptose/cirurgia , Estrabismo/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Músculos Oculomotores/cirurgia , Complicações Pós-Operatórias , Reoperação/estatística & dados numéricos , Estudos Retrospectivos
2.
J AAPOS ; 26(5): 236.e1-236.e6, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36113699

RESUMO

PURPOSE: To determine the characteristics of children diagnosed with glaucoma suspect (GS) status, their clinical outcomes, and risk factors for progression to a diagnosis of glaucoma. METHODS: This was a retrospective sequential cohort study of children <18 years diagnosed as GS between 2013 and 2019, based on clinical (C-GS) and CGRN (CGRN-GS) criteria. Children with penetrating ocular trauma, steroid-response, treated ocular hypertension, and glaucoma at presentation were excluded. Outcomes included glaucoma, treated ocular hypertension, nonglaucomatous cupping (pseudoglaucomatous or physiologic), or persistent GS. Secondary outcomes were characteristics of children who progressed to glaucoma. RESULTS: A total of 887 children (mean age, 9.3 ± 4.7 years) were diagnosed as C-GS, because of optic nerve appearance (83%), family history (25%), ocular hypertension (15%), periocular lesion (4% [eg, Sturge-Weber]), blunt-trauma history (3%), ocular anomaly (2%), and systemic/genetic syndrome (1.5%). Outcomes among 487 children with one or more follow-up visits (mean, 1.7 ± 1.6 years) included 14 (3%) with glaucoma, 98 (20%) with physiologic cupping, 50 (10%) with prematurity-associated cupping, and 1 (0.2%) with treated ocular hypertension; 324 (67%) remained GS. Of children lost to follow-up, 116 (29%) were suspected physiologic or pseudoglaucomatous. Glaucoma diagnosis occurred at a mean age of 8.4 ± 5.5 years, based on elevated intraocular pressure (IOP; 79%), optical coherence tomography changes (43%), disk changes (21%), or field defects (14%). Risk factors for glaucoma were baseline IOP of ≥24 (P = 0.01) and periocular lesion (P = 0.008). Results from 773 children who met CGRN-GS criteria were similar. CONCLUSIONS: Risk of conversion to glaucoma diagnosis among children with glaucoma suspect status appears low. Baseline cup:disk ratio and family history of glaucoma were not predictive of glaucoma diagnosis. Baseline IOP >24 and presence of a periocular lesion carry higher risk.


Assuntos
Glaucoma , Hipertensão Ocular , Criança , Humanos , Pré-Escolar , Adolescente , Pressão Intraocular , Estudos Retrospectivos , Estudos de Coortes , Hipertensão Ocular/diagnóstico , Glaucoma/diagnóstico
3.
Arch Ophthalmol ; 125(11): 1495-500, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17998510

RESUMO

OBJECTIVE: To assess the predictive value of early retinal vascular diameter measurements for the development of severe retinopathy of prematurity (ROP) in high-risk preterm infants. METHODS: Digital fundus images were taken of 78 eyes at risk for ROP with a noncontact camera during fundoscopic examination when infants were 31 to 34 weeks of postconception age. Vessel diameters were measured for the major superior and inferior temporal arteries and veins with the use of VesselMap software. Mean vessel diameters from eyes that developed severe ROP requiring treatment were compared with those that developed less severe ROP not requiring treatment or no ROP. RESULTS: All 4 major temporal retinal vessels showed significantly larger average diameters in the group of eyes that developed severe ROP requiring treatment. Multivariate analysis adjusted by birth weight, gestational age, and chronologic age showed similar results. Early retinal vessel diameter measurements showed good predictive value for the progression to severe ROP requiring treatment (area under the receiver operating characteristic curve, 0.75-0.94). CONCLUSIONS: At 31 to 34 weeks of postconception age, average retinal vessel diameter measurements were significantly larger in infants who developed severe ROP requiring treatment than in infants who developed less severe ROP or no ROP.


Assuntos
Peso ao Nascer , Idade Gestacional , Vasos Retinianos/patologia , Retinopatia da Prematuridade/diagnóstico , Progressão da Doença , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Fotografação , Valor Preditivo dos Testes , Reprodutibilidade dos Testes
4.
Am J Ophthalmol ; 143(6): 1030-2, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17524769

RESUMO

PURPOSE: To evaluate the feasibility of using semiautomated analysis of digital fundus images to quantify effects of photocoagulation on retinal vessel diameter in retinopathy of prematurity (ROP). DESIGN: Case series. METHODS: Fourteen eyes of seven patients with threshold ROP and 12 eyes of six controls were included. Fundus images were captured before and after photocoagulation, or one to three weeks apart in controls, using an NM200D camera (Nidek, Inc, Aichi, Japan). VesselMap software (Imedos, Jena, Germany) measured vessel diameter. The t test analysis was used to assess vessel changes. RESULTS: The authors observed a significant reduction in average largest retinal vein diameter of -18% +/- 16% (+/-1 standard deviation). The average largest artery diameter trended down by -12% +/- 16%. Compared with controls, diameter reduction was significant in veins (P = .014) and arteries (P = .016). CONCLUSIONS: The diameter of retinal vessels decreases after photocoagulation. VesselMap analysis of digital images is feasible and may be helpful in quantifying treatment effects.


Assuntos
Fotocoagulação a Laser , Artéria Retiniana/patologia , Veia Retiniana/patologia , Retinopatia da Prematuridade/cirurgia , Dilatação Patológica , Fundo de Olho , Idade Gestacional , Humanos , Processamento de Imagem Assistida por Computador , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Fotografação
5.
Am J Ophthalmol ; 143(4): 723-5, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-17386296

RESUMO

PURPOSE: To determine the feasibility of using semiautomated analysis of digital fundus images to quantify the differences in retinal vascular diameter between retinopathy of prematurity (ROP) patients with and without plus disease. DESIGN: Case-control study. METHODS: Thirty eyes of 15 patients with ROP were included in this study. Fourteen eyes of seven patients had plus disease and 16 eyes of eight patients had no evidence of plus disease. Digital fundus images were captured using an NM200D (Nidek, Inc, Aichi, Japan) camera. Vessel diameters were determined using VesselMap software (Imedos, Jena, Germany). The Student t test analysis was used to compare diameters of vessels with and without plus disease. RESULTS: We found that the average venous diameter is significantly larger by 15% in the group with plus disease. CONCLUSIONS: VesselMap analysis of digital images is feasible. This method is able to distinguish between veins with and without plus disease, and may be useful in telemedicine screening strategies.


Assuntos
Processamento de Imagem Assistida por Computador , Fotografação/métodos , Artéria Retiniana/patologia , Veia Retiniana/patologia , Retinopatia da Prematuridade/diagnóstico , Estudos de Casos e Controles , Pré-Escolar , Estudos de Viabilidade , Fundo de Olho , Idade Gestacional , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido
6.
Ophthalmology ; 113(9): 1675-80, 2006 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-16828516

RESUMO

PURPOSE: To develop and validate a new parental questionnaire addressing symptoms and health-related quality of life (HRQL) in childhood nasolacrimal duct obstruction (NLDO). DESIGN: Cross-sectional study. PARTICIPANTS: Children ages 6 to younger than 48 months with and without clinical signs of NLDO. METHODS: A new questionnaire was developed using semistructured interviews with parents of children with NLDO and through discussions with expert clinicians. Questionnaires were completed by parents of children with and without NLDO. Cronbach's alpha was calculated as a measure of internal-consistency reliability. Factor analysis was used to evaluate a priori subscales: symptoms and HRQL. Discriminant construct validity was assessed by comparing questionnaire scores between children with and without NLDO and between affected and unaffected eyes of children with unilateral NLDO. Instrument responsiveness was determined by comparing presurgical and postsurgical intervention scores in a subset of NLDO patients who underwent surgical treatment. MAIN OUTCOME MEASURE: The NLDO questionnaire score. RESULTS: Eighty-seven children were enrolled, 56 with and 31 without NLDO. All but 2 questions on the questionnaire showed a good distribution of responses, a high correlation with the rest of the questionnaire, and excellent discrimination between patients with and without NLDO. Cronbach's alpha values were good for the overall questionnaire (0.95), and for 2 predetermined subscales: symptoms (0.95) and HRQL (0.85). On a 0 to 4 scale, NLDO patients had worse scores compared with non-NLDO patients for both symptoms (mean difference, 2.1; 95% confidence interval [CI], 1.9-2.3) and HRQL (mean difference, 1.2; 95% CI, 0.9-1.5) subscales. The NLDO patients had worse scores before intervention compared with after intervention for both the symptoms (mean difference, 2.2; 95% CI, 1.6-2.9) and HRQL (mean difference, 1.4; 95% CI, 0.8-2.1) subscales. Finally, NLDO patients had worse symptom scores for affected eyes compared with unaffected eyes (mean difference, 2.3; 95% CI, 1.9-2.6). CONCLUSIONS: This novel NLDO questionnaire is useful in quantifying parental perception of symptoms and HRQL in childhood NLDO. The questionnaire may have a role in future clinical studies of NLDO.


Assuntos
Obstrução dos Ductos Lacrimais/diagnóstico , Ducto Nasolacrimal/patologia , Qualidade de Vida , Inquéritos e Questionários , Pré-Escolar , Estudos Transversais , Dacriocistorinostomia , Feminino , Inquéritos Epidemiológicos , Humanos , Lactente , Obstrução dos Ductos Lacrimais/congênito , Masculino , Pais , Perfil de Impacto da Doença
7.
J Glaucoma ; 15(6): 524-8, 2006 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17106366

RESUMO

PURPOSE: To determine and compare the central corneal thickness (CCT) and corneal diameter among groups of patients with childhood glaucomas and assess the relationship between CCT and corneal diameter in these patients. DESIGN: A multicenter observational case series using prospective and retrospective data. METHODS: Patients from the Scheie Eye Institute, Children's Hospital of Philadelphia, and Emory and Vanderbilt Medical Centers with childhood glaucomas were eligible to participate. Retrospective data on CCT and corneal diameter of these patients were collected when available; otherwise, patients were asked to return to the ophthalmology clinics for measurements. Patients with corneal edema or central corneal scarring were excluded. One hundred eighty four glaucomatous eyes from 109 patients (median age = 9.0 y; age range = 0 to 60 y) were included. RESULTS: The mean CCT (+/-SE) was 651.1+/-63.5 microm for aphakic, 528.7+/-38.5 microm for Axenfeld-Rieger, and 563.4+/-67.9 microm for 1 degrees infantile eyes. The mean corneal diameter in aphakic, Axenfeld-Rieger, and 1 degrees infantile glaucoma eyes were 11.2+/-1.0, 12.5+/-0.9, and 13.2+/-1.2 mm, respectively. There was a significant difference in CCT and in corneal diameter between aphakic and 1 degrees infantile glaucoma eyes, and between aphakic and Axenfeld-Rieger eyes (P < 0.0001). There was a negative correlation between CCT and corneal diameter in all eyes (r = -0.41, P < 0.0001). CONCLUSIONS: Patients with aphakic glaucoma are different from those with congenital glaucoma or Axenfeld-Rieger in CCT and corneal diameter. A patient with pediatric glaucoma and a larger corneal diameter was more likely to have a thinner CCT. Attention should be paid to the CCT of patients with childhood glaucomas for interpretation of intraocular pressure.


Assuntos
Afacia Pós-Catarata/complicações , Córnea/patologia , Glaucoma/congênito , Glaucoma/etiologia , Adolescente , Adulto , Pesos e Medidas Corporais , Criança , Pré-Escolar , Córnea/diagnóstico por imagem , Feminino , Humanos , Lactente , Recém-Nascido , Pressão Intraocular , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Estudos Retrospectivos , Tonometria Ocular , Ultrassonografia
8.
J AAPOS ; 20(5): 425-430.e1, 2016 10.
Artigo em Inglês | MEDLINE | ID: mdl-27651231

RESUMO

PURPOSE: To examine the feasibility of a retinopathy of prematurity (ROP) telemedicine evaluation system of providing timely feedback to a neonatal intensive care unit (NICU) with at-risk premature infants. METHODS: This was a prospective observational study of premature infants with birth weights of <1251 g in five NICUs in the United States. Infants scheduled for clinically indicated ROP evaluations underwent indirect ophthalmoscopic examinations and digital imaging on the same day. Imaging was performed by nonphysician retinal imagers. Times required were determined from obtaining digital images of both eyes to submission via web-based system to a secure server for grading by trained readers at a central reading center to sending back grading results to the clinical center. RESULTS: A total of 1,642 image sets of eyes of 292 infants were obtained, from 823 imaging sessions. The mean turnaround time from submission of image sets of both eyes to return of the grading results to the clinical center was 10.1 ± 11.3 hours (standard deviation), with a median of 12.0 hours (1st quartile, 0.9 hours; 3rd quartile, 16 hours). Overall, 95.5% of gradings (95% CI, 93.9%-96.7%) were returned within 24 hours. Subgroup analyses found, for image sets submitted to the reading center before 2 p.m. Eastern Standard Time, median time to report was 1.7 hours (1st quartile, 0.7 hours; 3rd quartile, 15.5 hours) compared with those submitted after 2pm (median, 14.1 hours; 1st quartile, 11.2, hours; 3rd quartile, 16.3 hours). CONCLUSIONS: An ROP telemedicine approach can provide timely feedback to the NICU regarding the detection of potentially serious ROP and thus referral to an ophthalmologist for examination and consideration of treatment.


Assuntos
Diagnóstico por Imagem/métodos , Implementação de Plano de Saúde , Triagem Neonatal/métodos , Retinopatia da Prematuridade/diagnóstico , Telemedicina/métodos , Pessoal Técnico de Saúde , Estudos de Viabilidade , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Unidades de Terapia Intensiva Neonatal , Masculino , Estudos Prospectivos , Sensibilidade e Especificidade , Fatores de Tempo
9.
Arch Ophthalmol ; 123(9): 1177-86, 2005 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-16157797

RESUMO

OBJECTIVE: To evaluate dorzolamide hydrochloride in patients younger than 6 years who have an elevated intraocular pressure or glaucoma. DESIGN: A 3-month, controlled, randomized, double-masked, multicenter, clinical trial. Patients were randomized to 2% dorzolamide 3 times daily or timolol maleate gel-forming solution (0.25% for patients <2 years and 0.5% for patients > or =2 but <6 years) once daily plus placebo twice daily. If the intraocular pressure was not controlled through monotherapy, younger patients received concomitant dorzolamide 3 times daily and 0.25% timolol gel-forming solution once daily and older patients received a fixed combination of 2% dorzolamide and 0.5% timolol twice daily. The primary safety variable was the proportion of patients who discontinued therapy for a drug-related adverse experience. Intraocular pressure reduction was a secondary measure. RESULTS: One younger patient (1.8%) of 56 randomized to dorzolamide discontinued concomitant therapy because of bradycardia. Two older patients (3.0%) of 66 discontinued dorzolamide because of ocular adverse experiences. The most frequent ocular adverse experiences were discharge and ocular hyperemia (younger cohort) and ocular hyperemia and burning/stinging (older cohort). At week 12, the mean change in intraocular pressure for dorzolamide was statistically significant from baseline (-7.3 mm Hg [-20.6%] and -7.1 mm Hg [-23.3%]) in the younger and older cohorts, respectively; P<.001 for both. CONCLUSION: Dorzolamide was generally well tolerated and demonstrated efficacy for up to 3 months in patients younger than 6 years.


Assuntos
Anti-Hipertensivos/administração & dosagem , Glaucoma/tratamento farmacológico , Sulfonamidas/administração & dosagem , Tiofenos/administração & dosagem , Timolol/administração & dosagem , Anti-Hipertensivos/efeitos adversos , Pré-Escolar , Método Duplo-Cego , Feminino , Glaucoma/etiologia , Humanos , Lactente , Pressão Intraocular/efeitos dos fármacos , Masculino , Hipertensão Ocular/tratamento farmacológico , Soluções Oftálmicas/administração & dosagem , Estudos Prospectivos , Segurança , Sulfonamidas/efeitos adversos , Tiofenos/efeitos adversos , Timolol/efeitos adversos , Resultado do Tratamento
10.
Am J Ophthalmol ; 160(1): 193-200.e1, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25892124

RESUMO

PURPOSE: To evaluate reactivation of pediatric uveitis during/following treatment with tumor necrosis factor alpha inhibition (anti-TNFα). DESIGN: Retrospective cohort study. METHODS: We assessed the incidence of uveitis reactivation in children ≤18 years who had achieved uveitis quiescence under anti-TNFα. Survival analysis was used to calculate reactivation rates while still on (primary outcome), and following discontinuation of (secondary outcome), anti-TNFα. Potential predictive factors were assessed. RESULTS: Among 50 children observed to develop quiescence of uveitis under anti-TNFα, 39 met criteria to be "at risk" of the primary (19 for the secondary) outcome. 60% were female, ∼half had juvenile idiopathic arthritis, and most were treated with infliximab. Overall, the estimated proportion relapsing within 12 months was 27.8% (95% confidence interval [CI]: 15.9%-45.8%); the estimated probability of reactivation was higher following (63.8% [95% CI: 38.9%-87.7%]) vs before (21.6% [95% CI: 10.8%-40.2%]) anti-TNFα discontinuation. Among those who discontinued anti-TNFα, the likelihood of reactivation was higher for those treated with adalimumab vs infliximab (hazard ratio [HR] 13.4, P = .01, 95% CI: 2.2-82.5) and those with older age at uveitis onset (HR 1.3, P = .09, 95% CI: 1.0-1.7). The duration of suppression, on medication, did not significantly affect the likelihood of reactivation when quiescence was maintained for ≥1.5 years. CONCLUSIONS: Approximately 75% of children remaining on anti-TNFα following achievement of uveitis quiescence remain quiescent at 1 year. However, most reactivate following anti-TNFα discontinuation. These results suggest that infliximab more often is followed by remission, off medication, than adalimumab. The data do not suggest that maintenance of suppression for more than 1.5 years decreases the reactivation risk.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Antirreumáticos/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Uveíte/diagnóstico , Adalimumab , Adolescente , Artrite Juvenil/tratamento farmacológico , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Infliximab , Masculino , Recidiva , Estudos Retrospectivos , Uveíte/tratamento farmacológico
11.
Ophthalmic Genet ; 36(4): 339-48, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-24512365

RESUMO

PURPOSE: To describe the retinal structure in a patient with cobalamin C (cblC) disease. METHODS: A 13-year-old male patient diagnosed with cblC disease during a perinatal metabolic screening prompted by jaundice and hypotony underwent ophthalmic examinations, electroretinography (ERG) and spectral domain optical coherence tomography (SD-OCT). RESULTS: The patient carried a homozygous (c.271dupA) mutation in the methylmalonic aciduria and homocystinuria type C (MMACHC) gene. At age 3 months he had a normal eye exam. A pigmentary maculopathy progressed to chorioretinal atrophy from 5-10 months. ERG at 7 months was normal. A nystagmus remained stable since the age of 2 years. At age 13, visual acuity was 20/250 (right eye) and 20/400 (left eye), with a +5.00 D correction, a level of vision maintained since first measurable at age 5 years. SD-OCT showed bilateral macular coloboma-like lesions; there was also a thickened surface layer with ganglion cell layer thinning. Photoreceptor outer segment loss and thinning of the outer nuclear layer (ONL) transitioned to regions with no discernible ONL with a delaminated, thickened, inner retina. CONCLUSIONS: A thick surface layer near the optic nerve resembling an immature retina and an initially normal macula that rapidly developed coloboma-like lesions suggest there may be an interference with retinal/foveal development in cblC, a mechanism of maculopathy that may be shared by other early onset retinal degenerations. Photoreceptor loss and inner retinal remodeling confirm associated photoreceptor degeneration.


Assuntos
Homocistinúria/diagnóstico , Retina/patologia , Degeneração Retiniana/diagnóstico , Deficiência de Vitamina B 12/congênito , Adolescente , Proteínas de Transporte/genética , Eletrorretinografia , Homocistinúria/genética , Humanos , Masculino , Oxirredutases , Degeneração Retiniana/genética , Células Ganglionares da Retina/patologia , Segmento Externo das Células Fotorreceptoras da Retina/patologia , Tomografia de Coerência Óptica , Acuidade Visual , Deficiência de Vitamina B 12/diagnóstico , Deficiência de Vitamina B 12/genética
12.
Invest Ophthalmol Vis Sci ; 56(13): 7875-87, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26658511

RESUMO

PURPOSE: To describe in detail the retinal structure and function of a group of patients with cobalamin C (cblC) disease. METHODS: Patients (n = 11, age 4 months to 15 years) with cblC disease (9/11, early onset) diagnosed by newborn screening underwent complete ophthalmic examinations, fundus photography, near-infrared reflectance imaging, and spectral-domain optical coherence tomography (SD-OCT). Electroretinograms (ERGs) were performed in a subset of patients. RESULTS: Patients carried homozygous or compound heterozygote mutations in the methylmalonic aciduria and homocystinuria type C (MMACHC) gene. Late-onset patients had a normal exam. All early-onset patients showed a maculopathy; older subjects had a retina-wide degeneration (n = 4; >7 years of age). In general, retinal changes were first observed before 1 year of age and progressed within months to a well-established maculopathy. Pseudocolobomas were documented in three patients. Measurable visual acuities ranged from 20/200 to 20/540. Nystagmus was present in 8/11 patients; 5/6 patients had normal ERGs; 1/6 had reduced rod-mediated responses. Spectral-domain OCT showed macular thinning, with severe ganglion cell layer (GCL) and outer nuclear layer (ONL) loss. Inner retinal thickening was observed in areas of total GCL/ONL loss. A normal lamination pattern in the peripapillary nasal retina was often seen despite severe central and/or retina-wide disease. CONCLUSIONS: Patients with early-onset cblC and MMACHC mutations showed an early-onset, unusually fast-progressing maculopathy with severe central ONL and GCL loss. An abnormally thickened inner retina supports a remodeling response to both photoreceptor and ganglion cell degeneration and/or an interference with normal development in early-onset cblC.


Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/complicações , Degeneração Retiniana/etiologia , Células Ganglionares da Retina/patologia , Adolescente , Erros Inatos do Metabolismo dos Aminoácidos/diagnóstico , Criança , Pré-Escolar , Eletrorretinografia , Feminino , Humanos , Lactente , Masculino , Degeneração Retiniana/diagnóstico , Índice de Gravidade de Doença , Tomografia de Coerência Óptica/métodos , Acuidade Visual
13.
J AAPOS ; 8(6): 549-54, 2004 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-15616502

RESUMO

INTRODUCTION: With a prevalence of 3-5%, amblyopia represents a major public health problem. Effective treatment depends on early detection, and a broad consensus of professional opinion supports vision screening of infants and young children. No single method of screening has been demonstrated to be superior in detecting amblyopia and all methods have significant limitations. METHODS: We assessed a new, "child-friendly" visual evoked potential (VEP) system (ENFANTtrade mark II, Diopsys Corp., Metuchen, NJ) for use in screening. We studied 122 children, aged 6 months to 5 years, comparing test results in a masked fashion to results of standard ophthalmologic examinations. A statistical program analyzed VEP differences between fellow eyes to determine a "pass" or "fail" for each child. For verbal patients, clinical amblyopia was defined as an interocular difference of two or more lines in best-corrected visual acuity. For preverbal patients, clinical amblyopia was defined by the clinician's decision to treat with occlusion or atropine penalization. Preverbal children with significant refractive errors or structural eye pathology were also considered clinically abnormal. RESULTS: The test was completed by 94% of the study group, each child requiring an average of 10 minutes to complete testing of both eyes. The sensitivity was 0.973, the specificity 0.808, the positive predictive value 0.706, and the negative predictive value 0.984. CONCLUSION: With its easy electrode placement and rapid, attractive stimulus, the new system overcomes technical difficulties which were associated with older VEP techniques. The test shows promise as a screening tool for detecting amblyopia and other visual deficits in young children.


Assuntos
Ambliopia/diagnóstico , Potenciais Evocados Visuais , Seleção Visual/métodos , Pré-Escolar , Reações Falso-Positivas , Humanos , Lactente , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
14.
Med Image Comput Comput Assist Interv ; 16(Pt 2): 567-74, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24579186

RESUMO

Despite recent advances, automatic blood vessel extraction from low quality retina images remains difficult. We propose an interactive approach that enables a user to efficiently obtain near perfect vessel segmentation with a few mouse clicks. Given two seed points, the approach seeks an optimal path between them by minimizing a cost function. In contrast to the Live-Vessel approach, the graph in our approach is based on the curve fragments generated with vessel tracing instead of individual pixels. This enables our approach to overcome the shortcut problem in extracting tortuous vessels and the problem of vessel interference in extracting neighboring vessels in minimal-cost path techniques, resulting in less user interaction for extracting thin and tortuous vessels from low contrast images. It also makes the approach much faster.


Assuntos
Algoritmos , Angiofluoresceinografia/métodos , Interpretação de Imagem Assistida por Computador/métodos , Reconhecimento Automatizado de Padrão/métodos , Doenças Retinianas/patologia , Vasos Retinianos/patologia , Interface Usuário-Computador , Humanos , Aumento da Imagem/métodos , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Integração de Sistemas
15.
J Rheumatol ; 40(8): 1394-403, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23818712

RESUMO

OBJECTIVE: To evaluate the outcome of tumor necrosis factor-α inhibition (anti-TNF) for pediatric uveitis. METHODS: We retrospectively assessed children (age ≤ 18 yrs) with noninfectious uveitis receiving anti-TNF at 5 uveitis centers and 1 pediatric rheumatology center. Incident treatment success was defined as minimal or no uveitis activity at ≥ 2 consecutive ophthalmological examinations ≥ 28 days apart while taking no oral and ≤ 2 eyedrops/day of corticosteroids. Eligible children had active uveitis and/or were taking higher corticosteroid doses. RESULTS: Among 56 eligible children followed over 33.73 person-years, 52% had juvenile idiopathic arthritis (JIA) and 75% had anterior uveitis (AU). The Kaplan-Meier estimated proportion achieving treatment success within 12 months was 75% (95% CI 62%-87%). Complete absence of inflammatory signs with discontinuation of all corticosteroids was observed in an estimated 64% by 12 months (95% CI 51%-76%). Diagnoses of JIA or AU were associated with greater likelihood of success, as was the oligoarticular subtype among JIA cases. In a multivariable model, compared to those with JIA-associated AU, those with neither or with JIA or AU alone had a 75%-80% lower rate of achieving quiescence under anti-TNF, independent of the number of immunomodulators previously or concomitantly prescribed. Uveitis reactivated within 12 months of achieving quiescence in 14% of those continuing anti-TNF (95% CI 6%-31%). The incidence of discontinuation for adverse effects was 8%/year (95% CI 1%-43%). CONCLUSION: Treatment with anti-TNF was successful and sustained in a majority of children with noninfectious uveitis, and treatment-limiting toxicity was infrequent. JIA-associated AU may be especially responsive to anti-TNF.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Imunoglobulina G/uso terapêutico , Receptores do Fator de Necrose Tumoral/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Uveíte Anterior/tratamento farmacológico , Uveíte/tratamento farmacológico , Adalimumab , Adolescente , Artrite Juvenil/tratamento farmacológico , Artrite Juvenil/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Comorbidade , Etanercepte , Feminino , Humanos , Lactente , Infliximab , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Uveíte/epidemiologia , Uveíte Anterior/epidemiologia
17.
J AAPOS ; 16(4): 376-81, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22929453

RESUMO

PURPOSE: To evaluate and compare the visual acuity prognosis in the various pediatric glaucoma subtypes and to determine risk factors for vision loss. METHODS: The medical records of pediatric glaucoma patients from 2000 to 2010 at Children's Hospital of Philadelphia were retrospectively reviewed. Visual acuities, surgeries, glaucoma subtype, and etiology of vision impairment were recorded. Univariate and multivariate analyses were performed to determine the risk factors for visual impairment. RESULTS: A total of 133 eyes (36.8% primary congenital glaucoma, 28.6% aphakic glaucoma, 12.0% glaucoma associated with anterior segment dysgenesis, 12.0% Sturge-Weber glaucoma) of 88 patients were included. At last follow-up (median length, 5 years), 46.6% eyes achieved excellent (≥20/70) visual acuity. Of the glaucoma subtypes, primary congenital glaucoma conferred the best visual prognosis, with 69.4% eyes with excellent (≥20/70) visual acuity at final follow-up. Factors most associated with visual impairment (<20/200) were unilateral disease, multiple surgeries, poor vision at diagnosis, and other ocular comorbidities. The most common primary etiology for vision impairment was amblyopia (54.9%). CONCLUSIONS: Patients with glaucoma early in life appear to have a better visual acuity prognosis than previously reported, with those with primary congenital glaucoma faring better than other glaucoma subtypes. Recognition of risk factors for visual impairment can better guide clinical management and counseling of patients.


Assuntos
Glaucoma/fisiopatologia , Transtornos da Visão/fisiopatologia , Acuidade Visual/fisiologia , Adolescente , Criança , Pré-Escolar , Feminino , Glaucoma/classificação , Glaucoma/cirurgia , Implantes para Drenagem de Glaucoma , Humanos , Lactente , Recém-Nascido , Iridectomia , Fotocoagulação a Laser , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Trabeculectomia
18.
J AAPOS ; 16(5): 431-6, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23084379

RESUMO

PURPOSE: To describe the rate of change in retinal vessel width and tortuosity in eyes that develop treatment-requiring, or type 1, retinopathy of prematurity (ROP) versus eyes that do not develop type 1 ROP. METHODS: Posterior poles of eyes of 41 infants at risk for ROP were imaged longitudinally with a 30° fundus camera. Retinal vessel width and tortuosity were measured with computer-assisted image analysis. The rate of change per day in width and tortuosity up to the development of most severe ROP was calculated from linear regression and eyes with (n = 10) and without type 1 ROP (n = 31) were compared. RESULTS: Eyes that developed type 1 ROP had a greater rate of change in width for venules and 3 widest vessels (P < 0.0001), and a greater rate of change in tortuosity for arterioles and 3 most tortuous vessels (P < 0.0001) than eyes that did not develop type 1 ROP. These vessel parameters discriminate the 2 groups well (area under the ROC curve, 0.79-0.90). A combination of venular width and arteriolar tortuosity had the best discriminative ability (area under the ROC curve, 0.96). CONCLUSIONS: In this pilot study, eyes that eventually developed type 1 ROP demonstrated a faster increase in width and tortuosity of retinal vessels compared with those that did not. Further study of the kinetics of retinal vascular change in a larger sample may allow for the earlier identification of vision-threatening ROP.


Assuntos
Vasos Retinianos/patologia , Retinopatia da Prematuridade/patologia , Anormalidade Torcional/patologia , Arteríolas/patologia , Feminino , Idade Gestacional , Humanos , Processamento de Imagem Assistida por Computador , Recém-Nascido , Masculino , Projetos Piloto , Curva ROC , Retinopatia da Prematuridade/etiologia , Fatores de Risco , Vênulas/patologia
19.
J AAPOS ; 16(4): 350-3, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-22824490

RESUMO

BACKGROUND: The clinical response to retinopathy of prematurity (ROP) treatment is currently assessed subjectively. This study aims to quantify treatment response objectively by assessing changes in digital images of posterior pole retinal vessel width and tortuosity. METHODS: Images of 30 right eyes with type 1 ROP obtained at up to three time points were analyzed: before treatment (T = 0) and 1 (T = 1) and/or 2 weeks (T = 2) after treatment. Width and tortuosity of retinal vessels were analyzed from digital images using computer-assisted image analysis software. RESULTS: Vessel width decreased by 20% (P < 0.004) within the first week and remained stable by the second week after laser treatment. Vessel tortuosity did not significantly change by the first week but decreased 27% (P < 0.01) by second week. CONCLUSIONS: Vessel width appears to decrease dramatic within the first week, whereas the regression of tortuosity follows a slower course.


Assuntos
Fotocoagulação a Laser , Vasos Retinianos/patologia , Retinopatia da Prematuridade/cirurgia , Arteríolas/patologia , Feminino , Idade Gestacional , Humanos , Processamento de Imagem Assistida por Computador , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Masculino , Projetos Piloto , Resultado do Tratamento , Vênulas/patologia
20.
JAMA Ophthalmol ; 134(11): 1235-1236, 2016 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-27583563

Assuntos
Imperícia
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