ICE Syndrome

THE COGAN-REESE SYNDROME OF IRIS NODULES, ECTOPIC DESCEMET'S membrane, and unilateral glaucoma is now considered part of the spectrum of the iridocorneal endothelial (ICE) syndrome.' The primary pathology in this syndrome is thought to be an abnormality in the corneal endothelium. The sequence of events begins with proliferation of this endothelium, leading to corneal edema and extension over the trabecular meshwork onto the iris, which then can contract to produce broad peripheral anterior synechiae and iris atrophy.2 Although this hypothesis can explain Chandler's syndrome and essential iris atrophy, it falls short of explaining the occurrence of multiple nevi on the iris surface and does not provide an explanation of the etiology of the endothelial cell proliferation and basement membrane production that covers the trabecular meshwork and anterior iris surface. The purpose of this report is to present two cases of the ICE syndrome, one of which is an iris nevus syndrome, and to present an alternative hypothesis, drawing upon the more recent evidence of involvement of abnormalities in neural crest cell migration and terminal differentiation.