A 20-year-old man presented with history of diminution of vision since 8 years. On examination his best corrected visual acuity was 20/20 in the right eye and 20/200 in the left eye. Anterior segment examination was essentially normal in both eyes. Right eye fundus showed stellate maculopathy with schitic changes in the foveal region (figure 1A). Left eye fundus showed stellate maculopathy with schitic changes in the foveal region and an isolated wedge-shaped retinochoroidal coloboma in the inferior fundus (figure 1B). The retinochoroidal coloboma in the left eye was not involving the disc or macula. Optical coherence tomography of both eyes showed altered foveal contour, increased central macular thickness and schitic changes in the inner retinal layers with splitting of the retinal layers at the level of outer plexiform and inner nuclear layer (figure 1C, D). Electroretinogram of both eyes showed reduced photopic and scotopic responses. Both eyes showed reduced oscillatory potential (figure 1E). A diagnosis of juvenile retinoschisis was made based on fundus features and ancillary tests. The patient and family members were counselled regarding the disease nature, prognosis and genetic association. Topical dorzolamide BD was prescribed for the macular schitic changes.
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