Abstract

We report a case of Straatsma syndrome which is a rare disease entity characterized by unilateral myelinated retinal nerve fibers (MNRF), axial myopia, amblyopia and occasionally strabismus.

Our patient 34 yr/M presented with chief complaints of sudden diminution of vision in his right eye (6/6 with temporal visual field loss) 1 month ago, following RTA sustaining head injury with loss of consciousness. The patient gave history of outward deviation and decreased vision(HM+) in his left eye since childhood, which has remained unchanged throughout. Confrontation and perimetry revealed temporal hemianopia in right eye with fundus showing temporal disc pallor secondary to RTA; rest of fundus:WNL. On left eye examination exotropia and high myopia(-12.75D) with axial length = 27mm (OS 23mm) were noted.  Left eye fundus examination showed extensive myelination of retinal nerve fibres contiguous with optic nerve head along superior and inferior temporal arcades involving macula (type 2 MRNF).