Atypical presentation and review of the ICE syndrome

The different variants of the iridocorneal endothelial (ICE) syndrome were separately described in the early to mid-1900's and were eventually linked to a common etiology of an abnormal corneal endothelium. The ICE syndrome typically manifests in early to middle adulthood, usually occurs in women, and is almost always unilateral. Management of patients with any variant of the ICE syndrome challenges even the best clinicians.

We present a 52-year-old patient with Chandler's syndrome. Our patient shared many of the classic characteristics of Chandler's syndrome, including peripheral anterior synechiae and corneal edema, with one unusual aspect--the patient was male. Details regarding the subsets of the ICE syndrome, differential diagnosis, and current understanding of the pathophysiology are also reviewed.

The ICE syndrome is a progressive anterior segment disease that is quite difficult to manage. Corneal edema, increased IOP, and glaucoma are all sequelae of these conditions and, even in the best hands, are extremely challenging to manage. Today, nearly 100 years after Harms described the first patient with an ICE syndrome, we still do not know the exact mechanism for the corneal endothelial changes. Many investigations have been made into the causative agent or stimulus for abnormal endothelial growth in the ICE syndrome. No definitive proof has been established, but a relationship may exist with the Herpes Simplex and Epstein-Barr viruses. Further investigation is needed to determine the most-appropriate treatment and management of the ICE syndrome.