Jean Marie Pagani, OD

Disclosures

February 25, 2016

Discussion

ICE syndrome is a rare progressive disorder whose etiology is unknown, but inflammation and a viral origin (ie, herpes simplex virus) are thought to be associated.[2,4] It is characterized by proliferative and structural abnormalities of the corneal endothelium.[5] Secondary corneal edema, peripheral anterior synechiae, progressive obstruction of the iridocorneal angle, and abnormalities of the iris stroma are common features. These changes lead to corneal decompensation and glaucoma.

ICE syndrome comprises three clinical entities based on the levels of iris involvement and the type and severity of iris abnormalities: progressive iris atrophy, Cogan-Reese syndrome, and Chandler syndrome.[6] The unifying abnormality in these three entities is a unilateral beaten-bronze appearance of the corneal endothelium.[7]

Pathogenesis may be related to reparative activities induced by the injury of endothelial cells caused by inflammation or a viral infection. These abnormal endothelial cells develop the unique characteristics of epithelial cells and proliferate and migrate into surrounding tissue. The final result of these cellular alterations is endothelial cells that migrate posteriorly beyond the Schwalbe line to obstruct the iridocorneal angle and into the anterior chamber to cover the iris. This abnormally formed basement membrane eventually contracts, creating pupil anomalies, iris damage, and formation of peripheral anterior synechiae.[2]

ICE syndrome can result in glaucomatous optic neuropathy in more than 50% of patients. Glaucoma is a result of trabecular meshwork obstruction from migrating corneal endothelial cells and peripheral anterior synechiae formation within the angle. Corneal edema and decompensation is seen with altered corneal endothelial cells and endothelial pump function insufficiency. Iris atrophy, corectopia, polycoria, and ectropion uveae occur secondary to contraction of the migrated corneal endothelial cells.

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