AN UNUSUAL PRESENTATION OF MACULAR DYSTROPHY

Abstract

Introduction-Juvenile macular degenerations include a series of conditions affecting children and young adults. This includes Stargardt's disease, Best's vitelliform dystrophy and Juvenile Retinoschisis. They present in the first to second decade and causes central vision loss as the main symptom.Case report-38 year old gentleman presented with complaints of defective vision of 6 months duration, with no other associated ocular or systemic complaints. Vision was found to be defective, not improving with pinhole or glasses. Anterior segment was examined and found to be normal. Fundus showed yellow deposits in the macular area and FFA showed hyperfluorescence. Electrophysiological tests, fields and colour vision testing confirmed the diagnosis of Stargardt's disease. Discussion-In Stargardt's disease, the major defect is in the visual transduction cycle, leading to deposition of yellow flecks in the macular area. There is gradual deterioration of cone photoreceptors. Dark adaptation is affected, but typical night blindness does not occur. Stem cell treatment for retinal pigment epithelial cells is the main treatment studied nowadays.