- Usually diagnosed in women in the adult age.
In 1903 Harms extensively described a rare ocular condition characterized by iris atrophy and glaucoma, known as “progressive essential iris atrophy”. Five decades later, Chandler described a rare, unilateral ocular condition characterized by iris atrophy associated with corneal endothelial alterations, corneal edema, and glaucoma. Subsequently, it was suggested that this “Chandler syndrome” and the “progressive essential iris atrophy” are two different forms of the same disease. When Cogan and Reese described a similar condition associated with iris nodules, a third clinical entity was identified and subsequently named “iris nevus” or “Cogan-Reese syndrome”. Subsequent studies confirmed that these clinical entities show similar history and clinical findings and share the same pathogenic mechanisms characterized by an abnormal proliferation of corneal endothelium and the unifying term of “iridocorneal endothelial syndrome” was suggested by Yanoff.
It comprises a spectrum of three clinical entities: progressive essential iris atrophy, Cogan-Reese syndrome, and Chandler syndrome.
Progressive essential iris atrophy:Characterized by marked iris atrophy and hole(s) formation
Chandler syndrome:Corneal edema
Cogan-Reese syndrome:Presence of multiple iris nodules, hence also known as iris nevus syndrome
- Change in the shape/position of pupil
- Impairment of visual function: Blurred vision and/or halos around lights or reduction in visual acuity (due to glaucoma)
- Fine, “hammered-silver” or “beaten-bronze” appearance of the endothelium
- Changes of corneal endothelium
In vivo confocal microscopy (IVCM):High resolution imaging technique that represents a useful diagnostic tool in ICE syndrome, also in patients with corneal edema.
- Presence of “ICE-cells” on the corneal endothelium and the structural changes of the anterior chamber angle
Ultrasound biomicroscopy (UBM):Detection of changes of the anterior chamber angle structures in ICE syndrome, especially in the presence of corneal edema that does not allow gonioscopy visualization
- Corneal endothelial disorders:
- Posterior polymorphous dystrophy (PPCD)
- Fuchs endothelial dystrophy
- Iris disorders:
- Axenfeld-Rieger syndrome
- Iris melanoma
- Inflammatory iris nodules
Final therapeutic target is the prevention and management of the visual impairing complications, namely, corneal edema and glaucoma.
Topical antiglaucomatous medicationsFirst line of treatment, since a reduction in intraocular pressure can also improve corneal edema
- Suppressants of aqueous humor production:
- Topical beta blockers
- Alpha agonists
- Carbonic anhydrase inhibitors
Corneal surgeryTo improve visual function and reduce pain.
- Penetrating keratoplasty (PK)
- Descemet stripping with endothelial keratoplasty (DSEK)