A seven-year-old boy presented with gradual progressive deterioration of vision for last two years. On examination, his best corrected visual acuity was finger counting at 1/2 meter in right eye and finger counting at 1 m in left eye from aphakic area. He was on therapy with dorzolamide and timolol 0.5% in both eyes. Applanation intraocular pressure was 44 mm of Hg in RE and 42 mm of Hg in LE. His horizontal corneal diameters were 11.5 mm right eye (RE) and 11.5 mm left eye (LE), with pachymetry of 650 µm in both eyes.
His lens was cataractous and superiorly subluxated in both eyes. Fundus evaluation revealed a vertical cup to disc diameter ratio was 0.9:1 RE and 0.8:1 LE, with foveal hypoplasia in both eyes. What is the likely diagnosis? Source: Clinical Cases in Opthalmology *This case is from Docplexus Editorial team for educative purpose only The answer is posted in comment…
