Secondary glaucoma in iridocorneal endothelial syndrome

Abstract

Introduction. Iridocorneal endothelial (ICE) syndrome is a rare ophthalmic disorder of unknown aetiology. It affects adult women and is characterized by proliferative and structural abnormalities of the corneal endothelium, progressive obliteration of the iridocorneal angle which may decompensate to secondary glaucoma. ICE is typically unilateral and comprises a spectrum of three clinical entities: progressive essential iris atrophy, Chandler syndrome and Cogan-Reese syndrome. Aim of the study: We present a clinical case of ICE syndrome with predominant features of essential iris atrophy that results in secondary glaucoma. Material and methods: This is a descriptive case report of a ICE syndrome based on medical documentation, patient observation, surgical intervention and follow-ups. Results: A 55 years old patient, female, presented in emergency room complaining about decreased visual acuity, ocular pain, photophobia in the left eye for 1 week. The first examination revealed decreased visual acuity, elevated intraocular pressure. The slit lamp examination showed corneal edema, multiple peripheral anterior synechia, iris atrophy, change of the pupil shape. We performed gonioscopy, OCT, specular microscopy. Antiglaucoma surgery was performed. Conclusion: ICE is a rare ocular disorder. A clinical history and a full ophthalmic exam (visual acuity, IOP, gonioscopy, specular microscopy, OCT, visual field, ocular ultrasound) are essential to make the correct diagnosis. This case was challenging due to its rarity, diagnostic and therapeutic intricacy.