Abstract

A 16 year old male with history of night blindness,diminution of vision since 10 years.Vision-OD and OS-Perception of light present with projection of rays inaccurate.Anterior segment(OU) :within normal limits with alternating exotropia. Fundoscopy -OU-Media-clear,Disc-normal.Macula OU-large sharply demarcated lesion with shiny beaten bronze appearance with pigment clumps. Mid periphery OU- pigment spicules.History of consanginous marriage.FFA-Hypofluroscent lesion with hyperfluroscence at edges.His younger sister,13 year old with similar complaints since 4 years.Vision-OU-6/36.Anterior segment(OU): within normal limits with alternating exotropia. Fundoscopy(OU):Media-clear.Disc -normal. Macula-Less extensive similar lesion noted,mid-periphery-pigmentary spicules and specks noted.OCT both patients-OU-Inner retinal layer degeneration with high reflectivity of choroid secondary to gross RPE atrophy.FFA-Lesion-window defect with blocked fluroscence.OU-Mottled appearance at midperiphery.