NORD gratefully acknowledges Sarwat Salim, MD, FACS, Professor of Ophthalmology, Chief, Glaucoma Service, Medical College of Wisconsin, for assistance in the preparation of this report.
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.
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. These variants may represent different stages of one disease. (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.
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.
There is a hypothesis that ICE syndromes stem from an in-vitro herpes infection localized in the endothelial layer. According to this theory, one eye is infected first and the second eye develops immunity before it can be affected.
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 affected family members.
Secondary Glaucoma and Treatment:
In general, glaucoma is one of the leading causes of blindness in the world.. Glaucoma is characterized by increased pressure within the eye. If left untreated, the increased pressure affects the optic nerve, resulting in eventual blindness. The etiology of glaucoma in unclear and remains an active area of research. The American Academy of Ophthalmology recommends a complete eye exam by the age of 40 or earlier for those at increased risk. Important elements of the examination include visual acuity test, tonometry to measure intraocular pressure, gonioscopy to assess if the drainage angle is open or closed, slit lamp examination to assess the anterior segment of the eye, use of special lenses to examine the optic nerve and posterior segment of the eye, and visual field test to assess the loss of peripheral or central vision.
Glaucoma may occur as a secondary disorder to Cogan-Reese syndrome. The mechanism of glaucoma in ICE syndrome (all three variants) is believed to be related to a cellular membrane secreted by the abnormal endothelial cells. This membrane covers the trabecular meshwork of the drainage angle, thereby obstructing aqueous outflow facility and elevating intraocular pressure. In the early stages, the angle may appear open clinically although it is covered by this transparent membrane. Over time, contraction of this membrane leads to peripheral anterior synechiae and secondary angle closure glaucoma.
Treatment of Cogan-Reese syndrome usually involves the use of drops in the eyes to control the glaucoma and swelling (edema). Mild cases or corneal edema are often managed with soft contact lenses and hypertonic saline solutions. In advanced cases penetrating or endothelial keratoplasty may be required, although the failure rate is high with need for repeat corneal grafts. In some individuals, the corneal edema may be improved with reduction in intraocular pressure. Medical therapy for glaucoma is usually initiated with aqueous suppressants, including beta blockers, alpha-2 agonists and carbonic anhydrase inhibitors. Prostaglandin analogues may be helpful in some cases. Surgical intervention for glaucoma is eventually required in a high percentage of patients with ICE syndrome. The most commonly performed procedure is trabeculectomy, with variable success rates. Glaucoma drainage devices have shown favorable outcomes in a small number of patients, but further studies are warranted to validate these results in a large series. Laser surgery is rarely effective.
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