Biettis crystalline dystrophy - a rare case report

Abstract

We report the clinical and spectral domain optical coherence tomography findings in a rare case of Biettis        crystalline dystrophy.A 23 year old male patient presented with defective vision,night blindness and paracentral scotoma for a duration of 9 months.Fundus examination showed         characteristic yellowish white crystals scattered over the       posterior pole with areas of patchy retinal pigment epithelial degeneration.The typical age of onset during 2nd or 3rd            decade of life,progressive visual impairment,night                     blindness,paracentral scotoma,asymmetry of presentation between the two eyes were suggestive of Biettis                       dystrophy.Spectral domain optical coherence tomography ( SD-OCT ) is of value in diagnosis and during follow up.          SD-OCT showed deposition of crystals in the retinal pigment epithelium associated with atrophy.