Iridocorneal endothelial syndrome (ICE-S): classification, clinical picture, diagnosis

The iridocorneal endothelial (ICE) syndrome includes progressive essential iris atrophy, the Cogan-Reese syndrome, Chandler's syndrome and mixed forms. The term ICE-S was proposed by Scheie and Yanoff in 1975 and by Yanoff in 1979. The capacity of migration of the abnormal corneal endothelial cell layer across the anterior chamber angle, and on to the anterior surface of the iris, possible on to the back surface of the iris and across the zonula fibers is responsible for corneal edema, secondary glaucoma, nevi, noduli and atrophy of the iris, and pupillary distortion. The contraction of the migrated membrane-like ICE tissue produces holes in the iris. The diseases are usually unilateral in young patients. The etiology is still not clear. Theories include membrane formation, low grade of inflammation and viral infection with Herpes simplex or Epstein-Barr virus. Glaucoma and edema of the cornea are the main therapeutic problems.