BILATERAL STRAATSMA SYNDROME WITH NYSTAGMUS

In Brief Purpose: This case report presents a rare case of bilateral Straatsma syndrome with nystagmus and documents additional findings related to spectral domain optical coherence tomography and ocular fundus images. Methods: Case report with fundus photography, fluorescein angiography, indocyanine green angiography, and spectral domain optical coherence tomography correlations. Results: A 22-year-old man presented with extensive bilateral myelination of the retinal nerve fiber layer that encompassed both posterior poles. The patient had concomitant ocular findings of high myopia, strabismus, amblyopia, and congenital nystagmus. Conclusion: This case describes an unusual presentation of Straatsma syndrome and investigates the clinical features of this disease. The patient's constellation of findings is most easily explained as bilateral myelination serving as the catalyst for the associated findings of myopia and nystagmus. Straatsma syndrome is the triad of myelinated nerve fibers, amblyopia, and high myopia. Traditionally occurring unilaterally with wedge-shaped myelination, the patient exhibits a rare variant with bilateral diffuse myelination in conjunction with nystagmus.