Diagnosis and Management of Iridocorneal Endothelial Syndrome

Abstract

The iridocorneal endothelial (ICE) syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative abnormalities of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. The main subtypes of this syndrome are the progressive iris atrophy, the Cogan-Reese syndrome, and the Chandler syndrome. ICE syndrome is usually diagnosed in women in the adult age. Clinical history and complete eye examination including tonometry and gonioscopy are necessary to reach a diagnosis. Imaging techniques, such as in vivo confocal microscopy and ultrasound biomicroscopy, are used to confirm the diagnosis by revealing the presence of “ICE-cells” on the corneal endothelium and the structural changes of the anterior chamber angle. An early diagnosis is helpful to better manage the most challenging complications such as secondary glaucoma and corneal edema. Treatment of ICE-related glaucoma often requires glaucoma filtering surgery with antifibrotic agents and the use of glaucoma drainage implants should be considered early in the management of these patients. Visual impairment and pain associated with corneal edema can be successfully managed with endothelial keratoplasty.

Biomechanical relationships between the corneal endothelium and Descemet's membrane

“…Although the mechanical, topographical, and biochemical properties of DM in these diseases have not been well characterized, we note a recurring incidence of abnormal ultrastructure and the appearance of lesions, excrescences and craters in DM 70,175,180,181,183 . These features very likely offer abnormal topographical and mechanical cues to endothelial cells growing on and interacting with the membrane, which could trigger pathological processes such as EMT 184 or apoptosis 185 .…”

“…Examination of one cornea revealed an irregularly thickened DM, consisting of an abnormal ABL, an abnormal posterior banded zone of varying ultrastructure and thickness, and an absent PNBL 180 . Iridocorneal endothelial syndrome (ICE) is a group of related corneal disorders in which endothelial cells migrate onto the iris and block the drainage angle, leading to iris atrophy and glaucoma 181 . In a study of 27 ICE affected corneas, the majority possessed DMs with a posterior layer of microfibrils embedded in an amorphous matrix.…”

Posterior keratoconus and iris atrophy: a fortuitous association?

“…The source of the second hit or trigger remains unclear. Alvarado et al first postulated a viral etiology for the pathogenesis of ICE syndrome, specifically herpes zoster or herpes simplex, since these viruses are known to affect Descemet's membrane and are primarily unilateral (9) .…”

The frequency of secondary glaucoma in patients with iridocorneal endothelial syndrome in correlation to the presence of uveal ectropion

“…Surgical interventions based on fistulising procedures, have good results in the beginning, but usually shortly after the procedure closure of fistule with proliferated tissue and endothelial membranes occurs [26,27]. In most of the cases re-treatment is needed with obligatory intraoperative administration of antimetabolites or usage of drainage implants, in order to prevent or delay cicatricial closure of the surgical aperture in the limbus or in the filtering bleb [28,29,30].…”