X-linked juvenile retinoschisis is a hereditary disorder which usually occurs in boys rather than in girls, who are rarely affected. First clinical manifestations usually appear during the first decade. It is responsible for variable severity and slowly progressive vision loss. This progression can be characterized by vitreous hemorrhages and recurrent retinal detachments. We report the case of a 17-year old patient with stellar bilateral microcistic macular rearrangement of the eye-ground, centered on the foveola, associated with peripheral schisis with retinal detachment and unilateral tearing of internal and external layers.
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