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.
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.
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.
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.
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.
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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