Essential Iris Atrophy

Essential Iris Atrophy

National Organization for Rare Disorders, Inc.

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 Subdivisions

  • 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.

Symptoms

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.

Causes

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.

Affected Populations

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.

Standard Therapies

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.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Kanski JJ. Ed. Clinical Ophthalmology. 4th ed. Butterworth-Heinemann. Oxford, UK; 1999:232-34.



Newell FW. Ed. Ophthalmology: Principles and Concepts. 7th ed. Mosby Year Book, St. Louis, MO; 1991:275-76.



JOURNAL ARTICLES

Lakosha HM, Pavlin CJ, Simpson ER. Essential Iris atrophy mimicking iris neoplasm: an ultrasound biomicroscopic study. Can J Ophthalmol. 2000;35:390-93.



Howell DN, Damms T, Burchette JL Jr, et al. Endothelial metaplasia in the iridocorneal endothelial syndrome. Invest Ophthalmol Vis Sci. 1997;38:1896-901.



Huna R, Barak A, Melamed S. Bilateral iridocorneal endothelial syndrome presented as Cogan-Reese and Chandler's syndrome. J Glaucoma;1996;5:60-62.



DeBroff BM, Thoft RA. Surgical Results of penetrating keratoplasty in essential iris atrophy. J Refract Corneal Surg. 1994;10:428-32.



Alvarado JA, Murphy CG, Juster RP, et al. Pathogenesis of Chandler's syndrome, essential iris atrophy and the Cogan-Reese syndrome. II. Estimated age at disease onset. Invest Ophthalmol Vis Sci. 1986;27:873-82.



Alvarado JA, Murphy CG, Maglio M, et al. Pathogenesis of Chandler's syndrome, essential iris atrophy and the Cogan-Reese syndrome. I. Alterations of the corneal endothelium. Invest Ophthalmol Vis Sci. 1986;27:853-72.



FROM THE INTERNET

Kaiser P. Iridocorneal Endothelial (ICE) Syndromes. Digital Journal of Ophthalmology (DJO). nd. 2pp.

www.djo.hravard.edu/meei/GR/Kaiser120195/Kaiser120195Dx.html



Devine N. (ed.) Monday Night Chat Room. Wills Glaucoma Service & Foundation. Glaucoma. nd. 5pp.

www.wills-glaucoma.org/supportgroup/20010917.html



Burk R. Classification of secondary glaucomas.

Resources

Lighthouse International

111 E 59th St

New York, NY 10022-1202

Tel: (800)829-0500

Email: info@lighthouse.org

Internet: http://www.lighthouse.org



National Association for Parents of Children with Visual Impairments (NAPVI)

P.O. Box 317

Watertown, MA 02272-0317

Tel: (617)972-7441

Fax: (617)972-7444

Tel: (800)562-6265

Email: napvi@perkins.org

Internet: http://www.napvi.org



Prevent Blindness America

211 West Wacker Drive

Suite 1700

Chicago, IL 60606

Tel: (312)363-6001

Fax: (312)363-6052

Tel: (800)331-2020

Email: info@preventblindness.org

Internet: http://www.preventblindness.org



Glaucoma Research Foundation

251 Post Street

Suite 600

San Francisco, CA 94108

Tel: (415)986-3162

Fax: (415)986-3763

Tel: (800)826-6693

Email: info@glaucoma.org

Internet: http://www.glaucoma.org



NIH/National Eye Institute

31 Center Dr

MSC 2510

Bethesda, MD 20892-2510

United States

Tel: (301)496-5248

Fax: (301)402-1065

Email: 2020@nei.nih.gov

Internet: http://www.nei.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

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