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.
Major symptoms of essential iris atrophy may include a displaced and/or distorted pupil, patchy areas of degeneration (atrophy) on the iris, and/or holes in the iris. The edge of the pupil may turn outward (ectropion uveae). The onset of this disorder is gradual, and the changes in the shape and placement of the pupil are usually noticed before any change in vision occurs. Degeneration and holes in the iris may develop over a period of several years.
Other features of essential iris atrophy may include the attachment of portions of the iris to the cornea (peripheral anterior synechiae), swelling of the cornea (corneal edema), and/or abnormalities in the cells lining the cornea (corneal endothelium). These changes may lead to increased pressure in the eye (glaucoma) and vision loss.
The cause of essential iris atrophy or any other of the iridocorneal endothelial syndromes is not known. They are thought to be the result of the same mechanism and, according to one theory, a particular membrane (the corneal endothelial membrane) is the site of the primary defect. This idea proposes that the primary disorder is of the cells that line the cornea (corneal endothelium), with the impact on the iris and associated glaucoma as secondary or associated disorders.
Other researchers suspect that inflammation or chronic infection may be the cause of the disease.
Essential iris atrophy is a very rare disorder that is usually recognized in early to middle adulthood and occurs slightly more often among women than among men.
Treatment of essential iris atrophy is usually directed to the secondary glaucoma. Eye drops may be used to control the glaucoma and corneal swelling (edema). If these methods are unsuccessful surgery may be indicated. The use of a laser beam to reduce pressure within the eye (trabeculectomy) and corneal transplant (penetrating keratoplasty) are surgical methods that have been used to treat essential iris atrophy.
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FROM THE INTERNET
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