NORD gratefully acknowledges M. Bruce Shields, MD, Marvin L. Sears Professor and Chairman Emeritus, Department of Ophthalmology & Visual Science, Yale University School of Medicine, Yale Eye Center, for assistance in the preparation of this report.
Chandler's syndrome (CS) is a rare eye disorder in which the endothelium, the single layer of cells lining the interior of the cornea, proliferates causing distortion of the iris, corneal edema, and unusually high pressure in the eye (glaucoma). CS is one of three syndromes affecting the eyes (progressive iris atrophy and Cogan-Reese syndrome are the other two) that make up the iridocorneal endothelial syndrome. Chandler's syndrome affects females more often than males and usually presents in young adulthood to middle age.
Most often the condition affects one eye only but the fellow eye often has subclinical involvement. The combination of high pressure within the eye (glaucoma) and corneal edema can result in reduced vision with pain.
Chandler’s syndrome is characterized by proliferation of the cells lining the cornea, swelling of the cornea with distortion of the iris and glaucoma (optic nerve damage from high pressure) within the eye.
The pupil of the eye appears to be out of place, i.e. located in an out-of-the-ordinary position and distorted in shape and size (corectopia). In Chandler’s syndrome, whatever iris atrophy occurs is mild when compared to that associated with progressive iris atrophy and Cogan-Reese syndrome. Typically, the corneal endothelium presents as a hammered, silver surface on the back of the cornea.
The disorder may cause blurred vision, pain in the eye with eventual loss of vision. Usually only one eye is affected; however, the other eye may, very rarely, be clinically involved.
The single layer of cells lining the inside of the surface of the cornea is known as the endothelium. One of the roles played by the endothelium is to pump aqueous humor (fluid inside the eye) from the cornea. If the behavior of the endothelium is interrupted (as in Chandler’s syndrome), the pumping action fails, and fluid accumulates in the cornea (corneal edema) resulting in blurred vision.
The exact cause of Chandler’s syndrome is not known. Some researchers suspect that inflammation or chronic viral infection may be the cause of the disease.
Chandler’s syndrome is a very rare disorder that affects females more often than it does males. The disorder usually appears during young adult to middle aged years.
Treatment of Chandler's Syndrome usually involves the use of drops in the eyes to control the glaucoma and swelling (edema). Prostaglandin analogs, beta blockers, alpha-2 agonists and carbonic anhydrase inhibitors are drugs used for this purpose. If these methods are unsuccessful surgery may be indicated. Keratoplasty for corneal edema, and trabeculectomy for glaucoma are surgical methods used to treat Chandler's Syndrome. Laser surgery is rarely effective.
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Geyer O, Neufelder M, Michaeli-Cohen A, et al. Radiation-induced Chandler’s syndrome. Isr Med Assoc J. 2000;2:241-42.
Huna R, Barak A, Melamed S. Bilateral iridocorneal endothelial syndrome presented as Cogan-Reese and Chandler’s syndrome. J Glaucoma. 1996;5:60-62.
Hirst LW, Bancroft J, Yamauchi K, et al. Immunohistochemical pathology of the corneal endothelium in iridocorneal endothelial syndrome. Invest Ophthalmol Vis Sci. 1995;36:820-27.
Hemady RK, Patel A, Blum S, et al. Bilateral iridocorneal endothelial syndrome: case report and review of the literature. Cornea. 1994;13:368-72.
Devine N. ed. ICE Syndrome Chat Highlights. “ICE Syndrome” July 23, 2003.
Facts About The Cornea and Corneal Disease. National Eye Institute (NEI). 2010 Available at: http://www.nei.nih.gov/health/cornealdisease/#g Accessed:February 6, 2013.
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