Cogan Reese Syndrome

Cogan Reese Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Cogan Reese Syndrome 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, Cogan-Reese Type
  • Iridocorneal Endothelial (ICE) Syndrome, Cogan-Resse Type
  • Iris Naevus Syndrome
  • Iris Nevus Syndrome

Disorder Subdivisions

  • None

General Discussion

Cogan-Reese Syndrome is an extremely rare eye disorder characterized by a matted or smudged appearance to the surface of the iris; the development of small colored lumps on the iris (nodular iris nevi); the attachment of portions of the iris to the cornea (peripheral anterior synechiae); and/or increased pressure in the eye (glaucoma). Secondary glaucoma may lead to vision loss. This disorder most frequently appears in young and middle-aged females, usually affecting only one eye (unilateral) and developing slowly over time.

Symptoms

Cogan-Reese Syndrome is one of the iridocorneal endothelial (ICE) syndromes, all of which usually affect one eye of young to middle-aged women. The ICE syndromes (Essential Iris Atrophy, Chandler's Syndrome, and Cogan-Reese Syndrome) are distinct from one another. However, since these disorders all affect the eye and some of their symptoms overlap, it may be difficult to distinguish between them. (For more information on Chandler's Syndrome and Essential Iris Atrophy, see the Related Disorders section of this article.)



Major characteristics of Cogan-Reese Syndrome include a matted or smudged appearance to the surface of the iris (nevus), yellow or brown lumps or nodules on the iris (nodular iris nevi), the attachment of portions of the iris to the cornea (peripheral anterior synechiae), and increased pressure in the eye (glaucoma). The development of Cogan-Reese Syndrome is gradual, and may be preceded by symptoms of Essential Iris Atrophy and/or Chandler's Syndrome. The matted appearance of the iris and development of nodules on the iris distinguish Cogan-Reese Syndrome from the other iridocorneal endothelial syndromes.



Other features of Cogan-Reese Syndrome may include swelling of the cornea (corneal edema) and/or abnormalities in the cells lining the cornea (corneal endothelium). These changes may be responsible for the glaucoma that is characteristic of this disorder. Glaucoma may lead to vision loss. The edge of the pupil may turn outward (ectropion uveae) and/or a transparent membrane may appear across the surface of the iris.

Causes

The cause of Cogan-Reese Syndrome is not known. Some researchers suspect that inflammation or chronic infection may be the cause of the disease. Others suggest that the primary disorder involves the cells that line the cornea (corneal endothelium), with the impact on the iris as a secondary or associated disorder. Some scientists suggest that the three iridocorneal (ICE) syndromes may represent different stages of one disease process.

Affected Populations

Cogan-Reese Syndrome is a very rare disorder that predominantly affects females in the middle adult years, although cases have been reported in children. Most affected individuals are white. The male to female ratio ranges from 1:2 to 1:5. A family history usually shows no other cases.

Standard Therapies

Treatment of Cogan-Reese Syndrome 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. Surgery using a laser beam to reduce pressure within the eye (trabeculectomy) and corneal transplant (penetrating keratoplasty) are surgical methods that have been used to treat Cogan-Reese Syndrome.

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

Durcan FJ. Cogan-Reese Syndrome. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:644.



Shields MB, Iridocorneal Endothelial Syndromes. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:649.



Kanski JJ. Ed. Clinical Ophthalmology. 4th ed. Butterworth-Heinemann. Oxford, UK; 1999:233-34,323.



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



JOURNAL ARTICLES

Halhal M, D'hermies F, Morel X, et al. [Iridocorneal endothelial syndrome. Series of 7 cases] J Fr Ophthalmol. 2001;24:628-34. French.



Teekhasaenee C, Ritch R. Iridocorneal endothelial syndrome in Thai patients: clinical variations. Arch Ophthalmol. 2000;118:187-92.



Ozdemir Y, Onder F, Cosar CB, et al. Clinical and histopathologic finding of iris nevus (Cogan-Reese) syndrome. Acta Ophthalmol Scand.1999;77:234-37.



Tester RA, Durcan FJ, Mamalis N, et al. Cogan-Reese syndrome. Progressive growth of endothelium over iris. Arch Ophthalmol. 1998;116:1126-27.



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



Wilson MC, Shields MB. A comparison of clinical variations of the iridocorneal endothelial syndrome. Arch Ophthamol. 1989;107:1465-68.



Makley TA, Kapetansky FM. Iris nevus syndrome. Ann Ophthalmol. 1988;20:311-15.



FROM THE INTERNET

Kaiser P. ed. Digital Journal of Ophthalmology, DJO Grand Rounds. Iridocorneal Endothelial (ICE) Syndrome. nd. 3pp.

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



Kaiser P. ed. Digital Journal of Ophthalmology, DJO Grand Rounds. Iridocorneal Endothelial (ICE) Syndrome. nd. 3pp.

www.djo.harvard.edu/meei/GR/Amin102596/amin102596Dx.html



Cogan-Reese Syndrome. EyeMDLink.com. Last Updated: 12/21/2001, 2pp.

www.eyemdlink.com/Condition.asp?ConditionID=114

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



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/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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