A case of bilateral straatsma syndrome

Abstract

Straatsma syndrome is characterized by a constellation of conditions including amblyopia, nystagmus, and myopia. We present the case of a 10-year-old boy who complained of involuntary back-and-forth eye movements and reduced vision since birth. His visual acuity was measured at 2/60 in both eyes. Examination revealed horizontal pendular nystagmus without ocular misalignment. Fundus examination showed myopic changes, including an anomalous optic disc with extensive myelinated nerve fibers extending from the optic disc along the superior and inferior arcades to the fovea. Axial length measurements were 28.34 mm and 27.96 mm in the right and left eyes, respectively. SD-OCT imaging confirmed thickening of the retinal nerve fiber layer. Treatment included cycloplegic refraction and occlusion therapy, with regular follow-ups every three months utilizing contact lenses with cycloplegic refraction. The etiology of retinal fiber myelination remains unclear; however, it is hypothesized that blurred retinal imaging during critical ocular development stages may lead to axial elongation and subsequent myopia, potentially delaying the development of the lamina cribrosa and facilitating myelination extension.