Rare case of iridocorneal endothelial syndrome with secondary glaucoma

Abstract

A rare case of 40 years old female, came with complaints of pain and blurring of vision in the left eye for the past week. The patient had a diminution of vision since birth in her left eye. On examination left eye vision was found to be 6/60 not improving with pinhole, also defective color vision 20/25. The right-eye vision was found to be normal. The left eye showed significant corneal edema, and moderate anterior chamber depth, with iris features of Corectopia, Pseudopolycoria, and iris atrophy. Pressures were found to be 30mm hg in the left eye, gonioscopy revealed high peripheral anterior synechiae extending anteriorly above Schwalbe’s line. Left eye fundoscopy was done and it showed increased CDR of 0.8 to 0.9 following which visual field analysis was done, it showed inferior field defect. The patient was started on e/d timolol and hypertonic saline for a week. The patient was reviewed after a week showing reduced pressures of 22mmg in the left eye with reduced corneal edema and pain. Diagnosis of iridocorneal endothelial syndrome-essential iris atrophy type with secondary glaucoma was made and now the patient is being followed up regularly. An iridocorneal endothelial syndrome is a group of disorders with three clinical variants as Chandler syndrome, Essential iris atrophy, and Cogan Reese syndrome. Pathology is the presence of abnormal endothelial cells, which behave as epithelial cells, able to proliferate and migrate. The iridocorneal endothelial syndrome is frequently associated with secondary glaucoma.