Straatsma Syndrome and cataract: case report and review of the literature.

Abstract

Straatsma Syndrome is known as unilateral myopia, amblyopia, and myelinated retinal nerve fibers (MRNF). The syndrome can be associated with other findings such as nystagmus, strabismus, and optic nerve hypoplasia among others. However, no cases associated with cataract have been reported. The visual prognosis depends on the myelinated retinal nerve fibers extension, the early amblyopia therapy, and the coexistence of other signs. We present the case of a 4-year-old girl with Straatsma Syndrome and cataract in the left eye. Despite the cataract surgical treatment with the refractive error correction and the amblyopia therapy, no visual improvement has been reported. Abbreviations: MRNF = Myelinated retinal nerve fibers, LE = Left eye, PD = Prism dioptres, BCVA = Best-corrected visual acuity, RE = Right eye, HM = Hand movement, CF = Counting fingers.