Iridocorneal endothelial syndrome

Iridocorneal endothelial syndrome (ICE syndrome) is a rare, progressive eye disorder that affects the cornea, iris, and the endothelium of the eye. It is characterized by abnormal cell proliferation on the corneal endothelium, which can lead to corneal edema, iris atrophy, and secondary glaucoma.

Classification

ICE syndrome is classified into three clinical variants:

• Chandler syndrome
• Essential iris atrophy
• Cogan-Reese syndrome

Pathophysiology

The exact cause of ICE syndrome is unknown, but it is believed to be related to an abnormality in the corneal endothelial cells. These cells proliferate and migrate across the anterior chamber, leading to the formation of a membrane that can contract and cause structural changes in the iris and cornea. This membrane can obstruct the trabecular meshwork, leading to increased intraocular pressure and secondary glaucoma.

Symptoms

Symptoms of ICE syndrome can vary depending on the specific variant but generally include:

• Blurred vision
• Halos around lights
• Pain or discomfort in the eye
• Progressive loss of vision
• Changes in the appearance of the iris

Diagnosis

Diagnosis of ICE syndrome is typically made through a comprehensive eye examination, which may include:

• Slit-lamp examination
• Gonioscopy
• Specular microscopy
• Optical coherence tomography (OCT)

Treatment

There is no cure for ICE syndrome, and treatment focuses on managing symptoms and preventing complications. Treatment options may include:

• Medications to reduce intraocular pressure
• Corneal transplant in cases of severe corneal edema
• Surgical procedures to manage glaucoma

Prognosis

The prognosis for individuals with ICE syndrome varies. While some patients may maintain good vision with appropriate treatment, others may experience significant vision loss due to complications such as glaucoma and corneal edema.

Related Pages

• Cornea
• Iris (anatomy)
• Endothelium
• Glaucoma
• Corneal transplant
• Optical coherence tomography