Straatsma syndrome: the diagnostic value of dilated funduscopy in anisometropic amblyopia

Straatsma syndrome is a rare condition defined by the triad of unilateral high axial myopia, amblyopia and myelinated retinal nerve fibre layer (MRNFL). Visual prognosis is often guarded when myelination is extensive, the macula is involved, or anisometropia is high. We present a case of classic Straatsma syndrome in a boy in his middle childhood from the Arabian Gulf region. In the right eye, he had extensive type 2 MRNFL involving the superior macula, high axial myopia and amblyopia. Given his age and extensive MRNFL, retinoblastoma was excluded. We describe his longitudinal clinical course and multimodal imaging findings. Despite recommended occlusion therapy and refractive correction, the family declined treatment, and best-corrected visual acuity remained at counting fingers at 1-year follow-up. The case highlights the value of dilated fundus examination in children with anisometropic amblyopia or unilateral high myopia to facilitate timely diagnosis and appropriate prognostic counselling.