What is iridocorneal endothelial syndrome, or ICE syndrome?

Dr. Jeanne Morrison, PhD
Family Practitioner

More common in women and usually diagnosed in people between ages 30 and 50, iridocorneal endothelial syndrome, or 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 fluids inside the eye cannot drain properly. ICE syndrome 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.

This answer is based on source information from National Eye Institute.

Continue Learning about Eye Conditions

What is the difference between a pterygium and a pinguecula?
American Academy of Ophthalmology's EyeSmartAmerican Academy of Ophthalmology's EyeSmart
A pinguecula is a yellowish patch or bump on the conjunctiva, most often on the side closest to the ...
More Answers
How common is uveal coloboma?
Diana MeeksDiana Meeks
Uveal coloboma is a rare condition that is not always well documented. Depending on the study and wh...
More Answers
How is keratoconus treated?
Dr. David R. Demartini, MDDr. David R. Demartini, MD
Keratoconus is the cone-like steepening of a patient's cornea that causes significant distortion of ...
More Answers
What Is the Leading Cause of Blindness?
What Is the Leading Cause of Blindness?

Important: This content reflects information from various individuals and organizations and may offer alternative or opposing points of view. It should not be used for medical advice, diagnosis or treatment. As always, you should consult with your healthcare provider about your specific health needs.