Unilateral myelinated retinal nerve fibers with high myopia and strabismus: Clinical insight into Straatsma syndrome

We present a case of a 23-year-old young male who had progressive outward deviation of left eye over the past two years. His left eye had a refractive error of -8.5DS/-0.75DC at 170°, and exhibited an exotropia of 60 prism diopters. The axial length of the right eye was 24.36 mm, compared to 28.61 mm in the left eye. There was also associated extensive myelinated nerve fibres with sparing of macula and vitreoretinal interface changes noted on optical coherence tomography (OCT) that could predispose to macular hole in future. This showcases a unique presentation of Straatsma syndrome and highlights the importance of OCT in such cases.