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Iridocorneal Endothelial Syndrome

More common in women and usually diagnosed between ages 30-50, iridocorneal endothelial (ICE) syndrome has three main features: (1) Visible changes in the iris, the colored part of the eye that regulates the amount of light entering the eye; (2) Swelling of the cornea; and (3) The development of glaucoma, a disease that can cause severe vision loss when normal fluid inside the eye cannot drain properly. ICE is usually present in only one eye.

ICE syndrome is actually a grouping of three closely linked conditions: iris nevus (or Cogan-Reese) syndrome; Chandler's syndrome; and essential (progressive) iris atrophy (hence the acronym ICE). The most common feature of this group of diseases is the movement of endothelial cells off the cornea onto the iris. This loss of cells from the cornea often leads to corneal swelling, distortion of the iris, and variable degrees of distortion of the pupil, the adjustable opening at the center of the iris that allows varying amounts of light to enter the eye. This cell movement also plugs the fluid outflow channels of the eye, causing glaucoma.

The cause of this disease is unknown. While we do not yet know how to keep ICE syndrome from progressing, the glaucoma associated with the disease can be treated with medication, and a corneal transplant can treat the corneal swelling.