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Cogan Reese Syndrome

Synonyms of Cogan Reese Syndrome

  • ICE Syndrome, Cogan-Reese Type
  • Iridocorneal Endothelial (ICE) Syndrome, Cogan-Resse Type
  • Iris Naevus Syndrome
  • Iris Nevus Syndrome

Disorder Subdivisions

  • No subdivisions found.

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Cogan-Reese Syndrome. Comparisons may be useful for a differential diagnosis:

Chandler's Syndrome is a very rare disorder characterized by increased development of the cells lining the cornea, drying up of the iris, corneal swelling, and unusually high pressure in the eye (glaucoma). This disorder may result in vision loss. The colored nodules characteristic of Cogan-Reese Syndrome do not usually appear in Chandler's Syndrome. (For more information on this disorder, choose "Chandler" as your search term in the Rare Disease Database.)

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. Attachment of portions of the iris to the cornea (peripheral anterior synechiae) and abnormalities in the cornea may lead to secondary glaucoma and vision loss. The colored nodules characteristic of Cogan-Reese Syndrome do not usually appear in Essential Iris Atrophy. (For more information on this disorder, choose "Essential Iris Atrophy" as your search term in the Rare Disease Database.)

Axenfeld's Anomaly is characterized by attachment of portions of the iris to the cornea (peripheral anterior synechiae). Axenfeld's Anomaly is considered to be an inherited, developmental defect, while the iridocorneal syndromes (Cogan-Reese Syndrome, Chandler's Syndrome, and Essential Iris Atrophy) are thought to be acquired disorders. There is some confusion in the medical literature as to whether Axenfeld's and Rieger's Anomalies are separate disorders or whether they occur together in what is called the Axenfeld-Rieger Syndrome.

Rieger's Anomaly is characterized by attachment of portions of the iris to the cornea, a distorted pupil, clouding of the edges of the cornea (peripheral corneal opacification), displacement of iris tissue (hypoplasia), and/or secondary glaucoma. When Rieger's Anomaly occurs in association with dental abnormalities (i.e., a decrease in the number of teeth) and facial malformations (i.e., displacement of the jaw) it is referred to as Rieger's Syndrome. Rieger Anomaly is considered to be an inherited, developmental defect, while the iridocorneal syndromes (Cogan-Reese Syndrome, Chandler's Syndrome, and Essential Iris Atrophy) are thought to be acquired disorders. There is some confusion in the medical literature as to whether Axenfeld's and Rieger's Anomalies are separate disorders or whether they occur together in what is called the Axenfeld-Rieger Syndrome.

The following disorders may be associated with Cogan-Reese Syndrome as secondary characteristics. They are not necessary for a differential diagnosis:

Glaucoma is a common eye disorder that occurs as a secondary disorder to Cogan-Reese Syndrome. Glaucoma is characterized by increased pressure within the eye. If left untreated the increased pressure may affect the lens and the optic nerve, resulting in eventual blindness. Glaucoma usually occurs for unknown reasons, however, it is more prevalent in people with diabetes. Some symptoms to be aware of are blurred vision, rainbow-colored halos around lights, and loss of side vision (tunnel vision). A simple test can measure the pressure in an individual's eye and this testing is recommended annually for persons over age forty. Treatment may consist of medicated eye drops. If medication does not resolve the symptoms surgery may be necessary.

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

Cogan Reese Syndrome Resources

Organizations:

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

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2008/03/18 00:00:00 GMT+0

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