Abstract The presence of retinal myelinated nerve fibers is not a rare finding during routine examinations, and it is usually a benign and isolated finding. However, in some rare cases, it can be associated with other ophthalmological conditions. We describe a case of a patient with the triad myelin nerve fibers, myopia and ambliopia, which configures the Straatsma Syndrome.

This article reports two cases of Straatsma Syndrome, a rare disease, emphasizing its clinical features that inclued myopia, strabismus and amblyopia associated with persistent myelinated fibers in the retina. Ophthalmic examination, color retinography and optical coherence tomography were performed.

Este artigo relata dois casos de síndrome de Straatsma, uma doença rara, enfatizando suas características clínicas que incluem miopia, estrabismo e ambliopia associada a persistência de fibras de mielina na retina. Foram realizados exame oftalmológico, retinografia e tomografia de coerência óptica.