Essential iris atrophy with secondary angle closure glaucoma: A rare spectrum of Irido-corneal endothelial syndrome

Abstract

Iridocorneal Endothelial syndrome (ICE) is a rare unilateral progressive disease of unknown Etiology, predominantly occurring in middle aged females. It consists of three overlapping disorders namely progressive iris atrophy, Cogan Reese syndrome and Chandler’s syndrome. Cogan-Reese syndrome is characterised by diffuse iris naevus or pedunculated iris nodules. Chandler’s syndrome is associated with corneal changes portraying “hammered silver” appearance. Iris changes are prominent in progressive iris atrophy. The management is targeted to control corneal edema and secondary glaucoma. The following report is a case of essential iris atrophy, a rare condition under the spectrum of ICE syndrome. The proliferative Endotheliopathy results in characteristic iris changes, Irido-trabecular Synechiae, Corectopia and secondary angle-closure glaucoma. Gonioscopy revealed the characteristic “high peripheral anterior synechiae”. The fundus examination showed unilateral glaucomatous cupping and intraocular pressure was managed with anti-glaucoma medications. Regular monitoring is required for early diagnosis of vision threatening complications in ICE syndrome.