Vol. 8, Issue 1, Part A (2022)

Abstract
Straatsma syndrome is a rare disease entity characterized by the traditional triad of unilateral myelinated retinal nerve fibers (MNRF), axial myopia, and amblyopia. The original description of the condition also included strabismus^. Variations of the triad include a “reverse Straatsma syndrome”, in which patient’s exhibit hyperopic instead of myopia. Even though nystagmus and strabismus have not been prominently associated with Straatsma syndrome, either may be present as complimentary findings without precluding one from the diagnosis. A 12-year-old boy presented with report of decreased vision in the right eye for the last year. On examination, his best-corrected visual acuity was 1.78 log MAR in the right eye and 0 log MAR in the left eye. Cycloplegic retinoscopy revealed a refractive error of a −15 dioptre sphere with a −3.5 dioptre cylinder at 180° in the right eye. The patient had right exotropia of 10 prism diopters on modified Krimsky’s test with full extra ocular motility.