Juvenile X-linked Retinoschisis responsive to Intravitreal C
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X-linked retinoschisis (XLRS) is an early onset genetic macular degeneration generally characterized by bilateral foveal and peripheral schsis at the layer of the nerve fiber layer. Abnormalities in the gene XLRS1, which codes for a protein retinoschisin, is thought to be important in cell adhesion and cause for the XLRS phenotype. Patients can present with mild to severe visual impairment which correlates with the variable degree of schisis seen on clinical examination and optical coherence tomography (OCT). Currently, there is no approved treatment for XLRS and management involves treating complications, which commonly include vitreous hemorrhage and retinal detachments. Here we describe a case of unilateral progressive XLRS in a patient who underwent retinal detachment repair and was stabilized using intravitreal corticosteroids.

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