Chandler’s Syndrome

Disease Overview

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 corneal edema, distortion of the iris, 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 (ICE syndrome). The spectrum is an acquired, unilateral disorder, which typically occurs in early to middle adulthood and predominantly affects women. Chandler’s syndrome is the most commonly encountered clinical variant of this spectrum.

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, frequently requiring surgical intervention with variable success rates.

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Synonyms

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

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Signs & 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. Because iris alternations may be minimal in the early stage of the disease, CS is often not recognized initially by the clinicians.

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.

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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. In addition, the abnormal endothelial cells can migrate as a membrane over adjacent structures, including the iris and the trabecular meshwork (the drainage canal of the eye). Contraction of this membrane leads to associated iris changes (corectopia) and iridotrabecular synechiae causing obstruction of aqueous outflow in the trabecular meshwork resulting in secondary glaucoma.

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. Herpes simplex virus DNA has been demonstrated with polymerase chain reaction in corneal specimens obtained from patients with ICE syndrome.

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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. However, its occurrence in a child and a teenager has been reported. ICE syndrome has been described in different ethnic groups, although the prevalence of the 3 clinical variants may vary among ethnicities.

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Disorders with Similar Symptoms

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.)
The prevalence of glaucoma in ICE syndrome has been reported to be as high as 82%. Glaucoma appears to be more frequent and severe in patients with progressive iris atrophy and Cogan-Reese syndrome as compared with Chandler’s syndrome. The anterior chamber angle is usually open early in the disease process, and some patients may develop glaucoma at this stage. As the disease progresses and contraction of the abnormal basement membrane occurs, broad peripheral anterior synechiae (broad iris strands to the drainage angle structures, including trabecular meshwork and Schwalbe’s line) lead to aqueous humor outflow obstruction and secondary angle closure glaucoma. 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.

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Standard Therapies

Treatment

Treatment of Chandler’s 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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Clinical Trials and Studies

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/

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References

TEXTBOOKS

Salim S, Shields MB. Iridocorneal Endothelial Syndromes. In: Kahook and Schuman, eds. Chandler and Grant’s Glaucoma. Slack Incorporated. New Jersey. 2013:319-326.

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. Available at: https://willsglaucoma.org/ice-syndrome-2 Accessed March 30, 2016.

Facts About The Cornea and Corneal Disease. National Eye Institute (NEI). Last Update: May 2013. Available at: https://www.nei.nih.gov/health/cornealdisease/#g Accessed March 30, 2016.

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Programs & Resources

• Assistance Programs
• Patient Organizations

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