DR. SNEHA GUHATHAKURTA

DR. JAYA BISWAS, DR. MOUSUMI BANERJEE

Abstract

MRCS syndrome is a rare, genetic retinal dystrophy characterized by bilateral microcornea, rod-cone dystrophy, cataract, posterior staphyloma in absence of any other systemic manifestations. Typically presenting as nyctalopia, the disease may progress to cause significant vision loss, and may be associated with strabismus, nystagmus, angle closure glaucoma. We are reporting one such case of MRCS syndrome with incidental finding of angle closure glaucoma. He presented with chief complaints of diminution of vision in low light condition, progressively worsening since 1 year. His systemic examination was within normal limit. Retinoscopic finding revealed refractive error of -6.5D and -7.5D in RE and LE respectively. Anterior segment examination showed ITC in all four quadrants with IOP 22mmHg and 28mmHg in RE and LE respectively. Fundus examination of both eye revealed atypical retinitis pigmentosa, optic nerve head drusen, posterior staphyloma and generalized chorioretinal atrophy.