¿Can The Endothelium Migrate? A Rare Syndrome Leading To Corneal Disease And Secondary Glaucoma.

To resurface the importance of unusual differential diagnosis in corneal diseases, which can lead to early onset of corneal edema, lamellar keratoplasties and glaucoma surgery, such as the Cogan-Reese Syndrome with a short case report.

Cogan-Reese syndrome, also known as iris nevus syndrome, belongs to a group of rare pathologies called Iridocorneal Endothelial Syndrome (ICE), it encompasses three clinical variants with a common etiopathogenesis. It is caused by proliferation of abnormal corneal endothelial cells that migrate toward the iridocorneal angle and the anterior iris surface, causing in many occasions secondary glaucoma and corneal edema.

A 64-year-old woman diagnosed with unilateral glaucoma in the left eye at the age of 48. The onset was with acute glaucoma. She was treated with topical antiglucomatous medications but due to the unsuccessful intraocular pressure control (IOP), trabeculectomy was performed three years later. After almost 10 years of good IOP control, topical medications needed to be added again. Throughout the follow up, she developed optic nerve damage and visual field deterioration, currently having a visual field index of 67%. In the latest follow up she showed characteristic iris nodules, corectopia with iridian atrophic areas, uveal ectropion, and corneal endothelial guttae which lead to the diagnosis of Cogan-Reese syndrome.

Certain rare corneal diseases with long-term-onset can be difficult to diagnose; in our initial ophthalmological exploration ICE was considered, even though the subtype was not completely clear; after 16 years of clinical history developing a rare type of secondary glaucoma due to the pathogenesis of the mentioned disease. In the last follow-up iris nodules had been observed leading to the diagnosis of Cogan-Reese Syndrome. In its typical presentation, middle aged woman require surgery earlier and fail earlier compared to other types of secondary glaucoma, also corneal edema can manifest and require lamellar transplants. In short, we cannot fully distress the importance of considering every possible diagnosis such as the rare ICE-Syndromes.