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Beyoğlu Göz Eğitim Araştırma Hastanesi, İstanbul, Uzm. Dr. The aim of this study is to report the different findings in optical coherence tomography (OCT) of three patients from the same family diagnosed with X-linked juvenile retinoschisis (XLRS). A 16-year-old boy complaining of visual deterioration and strabismus was diagnosed with XLRS on the basis of a fundus examination followed by OCT, FFA, and electrophysiology studies. Two other patients in the same family suffering from diminished vision were also invited for evaluation. One first degree relative (brother) and one second degree relative (cousin) of the patient also had similar findings.
OCT revealed consecutively wide hyporeflective cystoid spaces that split the neurosensory retina at the center of the fovea and small cystic spaces that formed bridges between the outer and inner retinal layers in the perifoveal area, atrophic retinal changes with increased reflectivity of the pigment epithelial layer and accompanying small cystic spaces that formed bridges between the outer and inner retinal layers perifoveally. These findings demonstrated that OCT is useful in the diagnosis of XLRS, especially in early and suspected cases. Keywords : X-linked juvenile retinoschisis, OCT, XLRS