ICE syndrome

ICE Syndrome ICE Syndrome, or Iridocorneal Endothelial Syndrome, is a rare eye disorder characterized by abnormalities in the cornea, iris, and the drainage angle of the eye. This condition can lead to secondary glaucoma and vision loss if not properly managed.

Overview

ICE Syndrome is a unilateral condition, meaning it typically affects only one eye. It is more common in women and usually presents in early to middle adulthood. The syndrome is named for the three main components it affects: the iris, cornea, and endothelial cells.

Pathophysiology

The exact cause of ICE Syndrome is unknown, but it is believed to involve an abnormality in the corneal endothelium. This abnormal endothelium proliferates and migrates across the anterior chamber, leading to changes in the iris and angle structures.

Corneal Changes

The corneal endothelium in ICE Syndrome becomes abnormal, leading to corneal edema and changes in transparency. This can result in decreased vision and discomfort.

Iris Changes

The iris may develop atrophy, corectopia (displacement of the pupil), and polycoria (multiple pupils). These changes can affect the appearance of the eye and contribute to visual disturbances.

Angle Changes

The angle of the anterior chamber may become obstructed due to the proliferation of endothelial cells, leading to increased intraocular pressure and secondary glaucoma.

Clinical Presentation

Patients with ICE Syndrome may present with:

• Blurred vision
• Halos around lights
• Eye discomfort or pain
• Changes in the appearance of the iris

Diagnosis

Diagnosis of ICE Syndrome is primarily clinical, based on the characteristic findings in the eye. Gonioscopy and slit-lamp examination are essential tools for identifying the changes in the cornea, iris, and angle.

Subtypes

ICE Syndrome is divided into three subtypes:

• Chandler Syndrome: Characterized by corneal edema and mild iris changes.
• Cogan-Reese Syndrome: Features nodules on the iris surface and more pronounced iris changes.
• Essential Iris Atrophy: Marked by severe iris atrophy and corectopia.

Treatment

There is no cure for ICE Syndrome, but treatment focuses on managing symptoms and preventing complications. This may include:

• Medications to lower intraocular pressure
• Surgical interventions for glaucoma
• Corneal transplantation in severe cases of corneal edema

Prognosis

The prognosis for ICE Syndrome varies depending on the severity of the condition and the effectiveness of treatment. Regular monitoring and management are crucial to prevent vision loss.

Research

Ongoing research aims to better understand the pathogenesis of ICE Syndrome and develop more effective treatments. Genetic studies and investigations into the role of viral infections are areas of active exploration.

See Also

• Glaucoma
• Corneal Endothelium
• Anterior Chamber of the Eye

External Links

• [Link to a reputable medical resource]

NIH genetic and rare disease info

• NIH info on ICE syndrome

ICE syndrome is a rare disease.