ICE syndrome,a group of rare genetic disorder,frequently affecting young women.We report a case of 34 years old female patient presenting with gradual unilateral loss of vision with raised IOP, pseudopolycoria,pigment dispersion over endothelium and CDR of 0.8:1 on fundoscopy and partially closed angle in gonioscopy. Patient received mannitol 20%(as immediate intervention)and other anti-glaucoma drugs but to no avail ;eventually undergone Trabeculotomy Surgery with Peripheral Iridectomy. Purpose of this report is due to its complexity ,diagnostic dilemma surrounding it and progression to Glaucomatous changes to optic nerve head.
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