Marfan syndrome is an autosomal dominant inheritance disease that affects connective tissue with phenotypic manifestations involving the skeletal, cardiovascular and ocular systems. The main ocular manifestations are the subluxation of the lens, myopia and retinal detachment. The aim of this article was to report the clinical and surgical management of a patient with Marfan syndrome with luxated lens for the vitreous cavity and who developed a severe phacoanaphylactic reaction characterized by severe secondary glaucoma and corneal decompensation.

X-linked adrenoleukodystrophy (X-ALD) represents a group of diseases characterized by the accumulation of very long chain fattyacids (VLCFAs) in the tissues. Its clinical manifestations are usually manifold. Visual changes may be present, but they often appear later in the disease. We describe here the case of a 9-year-old boy with X-ALD, whose first symptom was visual loss, which began at 8 years of age. His ophthalmologic evaluation revealed no alterations. Shortly thereafter, he suffered a head injury. The magnetic resonance […]

A adrenoleucodistrofia ligada ao X (X-ALD) representa um grupo de doenças caracterizadas pelo acúmulo de ácidos graxos de cadeia muito longa (VLCFAs) nos tecidos. Suas manifestações clínicas costumam ser múltiplas. Alterações visuais podem estar presentes, contudo costumam surgir mais tardiamente na doença. Descrevemos aqui o caso de um menino de 9 anos com X-ALD, cujo primeiro sintoma foi perda visual, iniciada aos 8 anos de idade. A sua avaliação oftalmológica não revelou alterações. Pouco tempo depois, ele sofreu um traumatismo […]

Choroidal melanomas are the most common primary intraocular malignant tumor in adults. They tend to be more malignant; because of their location hidden behind the iris they can not be detected until they become larger. Therapeutic strategy is related by size, extension, number and location of tumor and growth patterns. High frequency ultrasound biomicroscopy (UBM) gives high resolution, cross-sectional images of the anterior segment lesions. Postequatorial lesions and intracranial extension of the melanomas are scanned by magnetic resonance imaging (MRI). […]

Melanomas coroidais são os tumores malignos intra-oculares primários mais comuns em adultos. Eles tendem a ser mais malignos; devido à sua localização ser escondida por detrás da íris eles não podem ser detectados até se tornarem maiores. A estratégia terapêutica está relacionada com tamanho, extensão, número e localização dos padrões tumorais e de crescimento. O biomicroscopio ultra-sónico de alta frequência (BMU) fornece imagens transversais de alta resolução das lesões do segmento anterior. Lesões pós-equatoriais e de extensão intracraniana dos melanomas […]

Wolfram Syndrome consists of a neurodegenerative pathology of genetic character, also known by the acronym DIDMOAD that translates the main findings of this disease, Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy and Deafness. The article report the case of a patient diagnosed clinically with this syndrome in a general ophthalmology out patient clinic. Considering that patients with this genetic alteration have more than one cranial nerve affected by the disease and clinical history without meningitis or other neurological alterations, one has […]

Facing an enormous influx of information from medical research, clinicians need to differentiate robust study findings from spurious ones. The levels of evidence are an important component of Evidence-Based Medicine. Understanding the levels helps the Ophthalmologist to prioritize information and make right clinical decisions. The aim of this article is to describe the hierarchy of studies regarding their scientific evidence focusing on ophthalmology.

Em face a um enorme influxo de informações de pesquisa médica, os clínicos precisam diferenciar os achados de estudos robusto dos espúrios. Os níveis de evidência são um componente importante da Medicina Baseada em Evidências. Compreender os níveis ajuda ooftalmologista a priorizar as informações e tomar decisões clínicas corretas. O objetivo deste artigo é descrever a hierarquia dosestudos em relação à evidência científica com enfoque na oftalmologia.