Important

It is possible that the main title of the report Essential Iris Atrophy is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• ICE Syndrome, Essential Iris Atrophy Type
• Iridocorneal Endothelial (ICE) Syndrome, Essential Iris Atrophy
• Progressive Essential Iris Atrophy

 Disorder Subdivision

• None

 General Discussion

Essential iris atrophy is a very rare, progressive disorder of the eye characterized by a pupil that is out of place and/or distorted, areas of degeneration on the iris (atrophy), and/or holes in the iris. This disorder most frequently affects only one eye (unilateral) and develops slowly over time. Attachment of portions of the iris to the cornea (peripheral anterior synechiae) and/or abnormalities in the cornea may lead to secondary glaucoma and vision loss.

Essential iris atrophy is one of three iridocorneal endothelial (ICE) syndromes, each of which usually affects one eye of young to middle-aged men and women. The ICE syndromes (essential iris atrophy, Chandler syndrome, and Cogan-Reese syndrome) are distinct from one another. However, these disorders all affect the eye. Some of their symptoms overlap; making it difficult to distinguish between them.