You are here: Home / Rare Disease Information / Rare Disease Database

Search Rare Diseases

Enter a disease name or synonym to search NORD's database of reports.

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

Chandler's Syndrome

NORD is very grateful to 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.

Synonyms of Chandler's Syndrome

  • iridocorneal endothelial syndrome
  • iris atrophy with corneal edema and glaucoma

Disorder Subdivisions

  • No subdivisions found.

General Discussion

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.

Symptoms

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.

Causes

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.

Affected Populations

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.

Related Disorders

Symptoms of the following disorders can be similar to those of Chandler's syndrome. Comparisons may be useful for a differential diagnosis:

Progressive (or essential) iris atrophy is a very similar disorder to Chandler's syndrome and their symptoms may even overlap. Progressive iris atrophy is characterized by gross distortion of the pupil eye and the development of holes in the iris. This condition also causes increased intraocular pressure and corneal edema. If left untreated, vision loss may occur.

Cogan-Reese syndrome is characterized by distortion of iris tissue and the development of small wart-like growths on the iris. Increased pressure within the eye and corneal swelling are also evident. This disorder differs from Cogan corneal dystrophy, which is inherited as an autosomal dominant disorder. (For more information on this disorder, choose "Cogan-Reese" as your search term in the Rare Disease Database.)

Glaucoma occurs as a secondary characteristic of Chandler's syndrome. If left untreated, the increased pressure may affect the optic nerve, resulting in eventual blindness. Some symptoms for persons to be aware of are: blurred vision, rainbow colored halos around lights, and loss of side vision resulting in "tunnel vision". A simple test can measure the pressure in a person's eye, and this testing is recommended annually for persons over age 40, along with a complete eye exam. Treatment may consist of medicated eye drops and if these are unsuccessful surgery may be necessary.

Iridocorneal epithelial syndrome (ICE) is an umbrella term used to include the essential or progressive iris atrophy, Cogan-Reese syndrome, and Chandler's syndrome. The three disorders share symptoms such as corneal endothelial dysfunction and iris atrophy.

Standard Therapies

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.

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

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Organizations related to Chandler's Syndrome

References

TEXTBOOKS
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.

JOURNAL ARTICLES
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.

INTERNET
Devine N. ed. ICE Syndrome Chat Highlights. "ICE Syndrome" July 23, 2003.
http://willsglaucoma.org/ice-syndrome-2

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.

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: 2013/02/07 00:00:00 GMT+0

0-9 - A - B - C - D - E - F - G - H - I - J - K - L - M - N - O - P - Q - R - S - T - U - V - W - X - Y - Z

NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.

 
Copyright ©2014 NORD - National Organization for Rare Disorders, Inc. All rights reserved.