Iridocorneal Endothelial Syndrome: iris nevus syndrome

ABSTRACT

Iridocorneal endothelial syndrome (ICE syndrome) embraces a group of rare diseases with similar characteristics in the anterior segment, namely Essential iris atrophy, Chandler`s syndrome, and Iris nevus syndrom (Cogan Reese syndrome). The origin and pathogenesis of ICE syndrome remain unkown, but it appears that it is an abnormality of the corneal endothelial cells that is fundamental to all three conditions and leads to characteristic changes in the cornea, iris, and angle. Finding by specular microscopy of abnormal cells on the posterior corneal surface has led to the speculation of possible pathogenensis of this condition. We have experienced two patients with Iris nevus syndrome. Both cases presented with glaucoma as the chief clinical problem, and they illustrated difficult challenges in the glaucoma managemant in this disorder.