Straatsma Syndrome - Report of Two Cases

Abstract

Introduction: Straatsma syndrome is a rare disease characterized clinically by the exuberant persistence of myelin fibers in the retina associated or not with the presence of myopia, strabismus and amblyopia. The present study is aimed to describe two cases of this entity. Methods: Retrospective patient's clinical records review. Results: In case 1, a 17-year-old male was referred to the ophthalmology consultation due to decreased visual acuity in the right eye (RE). With no relevant systemic or ophthalmological history, he had a better corrected visual acuities (BCVA) of 20/200 in the RE and 20/20 without correction in the left eye (LE), with no oculomotricity changes and fundoscopy revealed the persistence of myelinated retinal nerve fibers (MRNF) near the upper temporal arch and myopic choroid in the RE and no alteration in the LE. In case 2, a 39-year-old male with family history of myopia was referred with a diagnosis of rhegmatogenous retinal detachment on the LE. On ophthalmic examination, BCVA were 20/20 on the RE and hand motion on the LE. During the pars plana vitrectomy, a total rhegmatogenous retinal detachment was observed in the LE, with MRNF accompanying the temporal arcades and a macular hole (MH). After three pars plana vitrectomies, including an inverted ILM flap technique attempted to close the MH, and eight months after silicone oil extraction, the aphakic LE presented a 20/200 BCVA with the retina fully attached and the MH closed. Conclusion:Despite some controversy in its features, Straatsma syndrome should be included in the differential diagnosis of leukocoria and must be suspected in the presence of refractive errors with anisometropia. The general prognosis is poor to moderate, but better results can be achieved with a prompt diagnosis and amblyopia management.