Objective: The aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease. Methods: A group of 51 sickle cell disease patients (36 hemoglobin SS and 15 hemoglobin SC) with ages ranging from 4 to 18 years was studied. Ophthalmological examinations were performed in all patients. Moreover, a fluorescein angiography was also performed in over 10-year-old patients. Results: The most common ocular lesions were vascular tortuosity, which was found in nine (25%) hemoglobin SS patients, and black sunburst, in three (20%) hemoglobin SC patients. Peripheral arterial closure was observed in five (13.9%) hemoglobin SS patients and in three (13.3%) hemoglobin SC patients. Arteriovenous anastomoses were present in six (16.5%) hemoglobin SS patients and six (37.5%) hemoglobin SC patients. Neovascularization was not identified in any of the patients. Conclusions: This study supports the use of early ophthalmological examinations in young sickle cell disease patients to prevent the progression of retinopathy to severe disease and further blindness.

OBJETIVO: O objetivo deste estudo foi determinar a prevalência e gravidade das complicações oculares em pacientes com mucopolissacaridoses (MPS). MÉTODOS: Vinte e nove pacientes com diagnóstico de mucopolissacaridoses foram estudados. Foram avaliados: idade, sexo, acuidade visual, presença de estrabismo, erros refrativos, exame de fundo de olho, pressão intraocular, espessura corneal central e ultrassonografia ocular. RESULTADOS: Foram avaliados três pacientes com MPS I (12%), 11 pacientes com MPS II (37,9%), um paciente com MPS III (3,4%) e 14 pacientes com MPS VI (48,3%). A média de idade foi de 9,5 anos (DP 5,5). Observou-se hipermetropia em 88,5% (23 pacientes) e astigmatismo em 51,7% (15 pacientes). A média da acuidade visual corrigida foi de 0,45 logMAR (DP 0,68). A média do equivalente esférico foi +3,57 D (DP 2,46) e da pressão intraocular foi 17 mmHg (DP 3,9). Os achados mais comuns foram: espessamento palpebral 24,1% (7 pacientes); opacidade da córnea, 55,2% dos casos (16 pacientes); atrofia do nervo óptico, 23,1% (6 pacientes); dobras radiais na retina 24% (7 pacientes). O fundo de olho não foi examinado em 3 pacientes devido à opacidade de córnea. A média da espessura do complexo esclera-retina-coroide (ERC) medida por ultrassom foi de 1,78 mm (DP 0,51). CONCLUSÃO: Os achados oftalmológicos mais proeminentes foram espessamento palpebral, diminuição da acuidade visual, hipermetropia moderada, opacidade da córnea, dobras radiais na retina perimacular e atrofia do nervo óptico.

PURPOSE: The objective of this study was to determine the prevalence and severity of ocular complications in patients with mucopolysaccharidosis (MPS). METHODS: Twenty-nine patients with diagnosis of mucopolysaccharidosis were studied. Age, gender, visual acuity, presence of strabismus, refractive error, fundus examination, intraocular pressure, central corneal thickness and ocular echography were assessed for each individual. RESULTS: There were three patients with MPS I (12%), eleven patients with MPS II (37.9%), one patient with MPS III (3.4%) and fourteen patients with MPS VI (48.3%). Mean age was 9.5 years (ranged from 1.2 to 20 years, DP 5.5). Refraction was available in 26 patients, from which 88.5% (23 patients) were hyperopic, and 53.8% (14 patients) presented astigmatism. Best corrected visual acuity was available in 18 patients and the mean was 0.45 logMAR (DP 0.68). The mean spherical equivalent was +3.57 D (SD 2.46) and intraocular pressure was 17 mmHg (SD 3.9). The most common findings were: eyelid thickening in 24.1% (7 patients); corneal opacity in 55.2% of cases (16 patients); optic nerve atrophy in 23.1% (6 patients); and radial folds in the retina in 24% (7 patients). The fundus was examined in 26 out of 29 patients because corneal opacity avoided the exam in 3 of them. The average thickness of the complex sclera-retina-choroid (SRC was 1.78 mm (SD 0.51). CONCLUSION: The most prominent ophthalmologic findings were eyelid thickening, decreased visual acuity, high hyperopia, corneal opacity, perimacular radial folds in the retina and optic nerve atrophy.

OBJETIVO: Avaliar a flutuação das medidas da pressão intraocular obtidas pela tonometria de aplanação de Goldmann, tonometria de contorno dinâmico e pela tonometria de não-contato com compensação corneana durante o período ambulatorial, em participantes com glaucoma e saudáveis. Esse estudo também correlacionou as flutuações da pressão intraocular com as flutuações da histerese corneana, espessura corneana central, média da curvatura corneana central e amplitude de pulso ocular. MÉTODOS: Um total de 12 controles (24 olhos) e 21 pacientes com glaucoma de ângulo aberto (38 olhos) foram selecionados. A pressão intraocular medida pela tonometria de aplanação de Goldmann, tonometria de contorno dinâmico e tonometria de não-contato com compensação corneana, amplitude de pulso ocular, a curvatura central e espessura corneanas, a histerese corneana e o fator de resistência foram medidos em intervalos de 2 horas, entre 9 AM e 5 PM. RESULTADOS: A pressão intraocular flutuou significativamente durante o dia em indivíduos controles e com glaucoma em todos os tonômetros (P<0,001). Não houve variação estatisticamente significante na média da curvatura corneana central (P=0,048 em controles; P=0,04 em glaucomatosos) ou na histerese no período pesquisado (P=0,12 em controles; P=0,36 em glaucomatosos). A amplitude de pulso ocular mostrou uma flutuação diurna significativa em ambos os grupos (P<0,001). Houve uma correlação significante entre a pressão intraocular medida pela tonometria de contorno dinâmico e a amplitude de pulso ocular (P<0,001). CONCLUSÃO: Houve uma flutuação significante da pressão intraocular ao longo do período ambulatorial nas medidas realizadas pela tonometria de aplanação de Goldmann, tonometria de contorno dinâmico, e tonometria de não-contato com compensação corneana em indivíduos normais e com glaucoma. A pressão intraocular variou independente da variação da histerese, espessura e curvatura central corneanas. Entretanto, houve correlação significante entre a amplitude de pulso ocular e as medidas de pressão intraocular realizadas pela tonometria de contorno dinâmico.

PURPOSE: The purpose of this study was to evaluate the fluctuation of intraocular pressure measurements obtained by Goldmann applanation tonometry, dynamic contour tonometry, and corneal compensated non-contact tonometry during office hours in glaucoma and healthy participants. This study also aims at correlating the intraocular pressure fluctuations with fluctuations of corneal hysteresis, central corneal thickness, mean central corneal curvature and ocular pulse amplitude. METHODS: A total of 12 controls (24 eyes) and 21 patients (38 eyes) with open-angle glaucoma were recruited. Intraocular pressure measured by Goldmann applanation tonometry, dynamic contour tonometry and corneal compensated non-contact tonometry, ocular pulse amplitude, central corneal curvature and thickness, corneal hysteresis, and resistance factor were obtained at intervals of 2 hours, between 9 AM and 5 PM. RESULTS: Intraocular pressure fluctuated significantly throughout the day in controls and glaucoma individuals with all tonometers (P<0.001). There was no statistically significant variation in mean corneal curvature (P=0.048 in controls; P=0.04 in glaucomatous) or hysteresis over time (P=0.12 in controls; P=0.36 in glaucomatous). The ocular pulse amplitude showed a significant diurnal fluctuation in both groups (P<0.001). There was a significant correlation between the intraocular pressure measured by dynamic contour tonometry and ocular pulse amplitude (P<0.001). CONCLUSION: There was significant intraocular pressure fluctuation over office hours on measurements performed by Goldmann applanation tonometry, dynamic contour tonometry, and corneal compensated non-contact tonometry in normal and glaucoma individuals. Intraocular pressure varied independently of corneal hysteresis, central corneal thickness, and central curvature variation. However, there was significant correlation between ocular pulse amplitude and intraocular pressure measurements performed by dynamic contour tonometry.