Juvenile X-linked retinoschisis-A case series

Abstract

Aim To report a case series of an unusual  congenital condition- X-linked juvenile retinoschisis. Methods A 21 year- old male patient with a history of defective vision in the left eye ( 2 months duration) presented for a second  opinion after having been advised emergency surgery for retinal detachment in the right eye. The patients 2 younger male siblings (19 years and 16 years) were asymptomatic but were also examined. Results In the symptomatic patient, fundus examination showed typical stellate striations at the fovea (suggestive of foveal schisis) and multiple large inner layer holes with inferior retinoschisis in the right eye. The patients left eye showed large areas of pigment deposition at the periphery. Both siblings also showed similar stellate striations suggestive of foveal schisis. Optical coherence tomographic assessment of all three showed characteristic vertically - ovoid cystic spaces in the fovea, consistent with a diagnosis of X-linked juvenile retinoschisis. Conclusion  Ophthalmologists must be aware of the clinical features and presentation of X linked juvenile retinoschisis in order to  differentiate it from retinal detachment and there in spare the patient unnecessary surgical interventions.