The aim of this article is to report two cases of Straatsma syndrome, a rare disease characterized by the traditional triad of unilateral myelinated retinal nerve fibres, axial myopia, and amblyopia. The cases were a five-year-old and a three-year-old girl, both with unilateral myopia, visual acuity of 0.1 (decimal), and myelinated retinal nerve fibres in fundoscopy. Prescription of cycloplegic refraction findings and occlusion of the involved eye was attempted in both cases, but visual acuity did not improve. Poor-prognostic factors of Straatsma syndrome include a high degree of anisometropia and extensive myelination.
Keywords: anisometric amblyopia; anisometropia; high myopia; oct (optical coherence tomography); rnfl (retinal nerve fibre layer).
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