IRIDOCORNEAL ENDOTHELIAL SYNDROME (ICE): THE ESSENTIAL IRIS ATROPHY VARIANT.

The Iridocorneal endothelial (ICE) syndrome is represented by three entities having in common a proliferative corneal endotheliopathy, extending to the iridocorneal angle then to the iris tissue to cause, often refractory ocular hypertension: essential atrophy of the iris (represented by our case), Chandler syndrome and Cogan-Reese syndrome. It is usually unilateral, non-familial and typically occurs in young women. 

The etiology remains unclear, but may be related to viral infection with Herpes simplex or Epstein-Barr virus. The progressive course of the disease is frequently complicated by secondary glaucoma and corneal decompensation.

We report the case of an 44-year-old man with no particular history presented at the Military Hospital Moulay Ismail, Meknes, Morocco, with right eye pain for 2 days.

The best corrected visual acuity was 20/20 in each eye. Examination of the right eye showed, an intraocular pressure of 37 mmHg, an extensive iris atrophy with pear-shaped corectopia, and a beaten-metal appearance in the posterior corneal surface, with a central predominance. Gonioscopy revealed a narrow iridocorneal angle with peripheral anterior synechiae. The right fundus and left eye examination were unremarkable. The control of the right ocular hypertension was obtained by medical treatment. At the 9-month follow-up, intraocular pressure remains well balanced.

Glaucoma associated with ICE syndrome is often difficult to manage and is usually treated with medications and/or filtering surgery. Glaucoma filtering surgery is usually successful when done early, but may fail due to endothelialization of the fistula by the abnormal corneal endothelium.