Chandler's Syndrome

Chandler's Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Chandler's 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

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

Disorder Subdivisions

  • None

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.

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/

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.

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



Schepens Eye Research Institute

20 Staniford Street

Boston, MA 02114-2500

Tel: (617)912-0100

Fax: (617)912-0101

Email: richard.godfrey@schepens.harvard.edu

Internet: http://www.theschepens.org/



National Association for Parents of Children with Visual Impairments (NAPVI)

P.O. Box 317

Watertown, MA 02272-0317

Tel: (617)972-7441

Fax: (617)972-7444

Tel: (800)562-6265

Email: napvi@perkins.org

Internet: http://www.napvi.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



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/



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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