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Iridocorneal Endothelial Syndrome (ICE) syndrome is a rare, unilateral, non-hereditary, progressive ocular condition characterized by abnormal proliferation of corneal endothelium, which extends over the angle and iris, leading to secondary angle-closure glaucoma, iris atrophy, and corneal changes.
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| Feature | Description |
|---|---|
| Sex | Predominantly affects females |
| Age | Onset typically between 20–50 years |
| Laterality | Almost always unilateral |
| Etiology | Exact cause unknown; Herpes simplex virus (HSV) DNA detected in some cases, suggesting viral association |
Subtypes of ICE Syndrome
| Subtype | Features |
|---|---|
| Essential (Progressive) Iris Atrophy | Marked iris atrophy, corectopia (pupil displacement), polycoria (multiple pupils), PAS |
| Chandler Syndrome | Most common subtype; prominent corneal edema, mild iris changes, minimal glaucoma |
| Cogan-Reese Syndrome (Iris Nevus Syndrome) | Pigmented iris nodules, mild iris atrophy, high glaucoma risk |
(a) Eye with progressive iris atrophy after AGV implantation, (a1) shows the same eye after DSEK and intraocular lens implantation. (b) Eye with Cogan-Reese syndrome, (b1) same eye after trabeculectomy with MMC. (c) Eye with Chandler syndrome, (c1) same eye after trabeculectomy with MMC and DSEK
Chandran P, Rao HL, Mandal AK, Choudhari NS, Garudadri CS, Sirisha Senthil. Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. PLoS ONE. 2017;12(3):e0171884-e0171884. doi:https://doi.org/10.1371/journal.pone.0171884
| System | Findings |
|---|---|
| Cornea | "Beaten bronze" or "hammered silver" appearance on specular reflection |
| Iris | Atrophy, corectopia, pseudopolycoria, nodules (in Cogan-Reese) |
| Anterior Chamber Angle | Broad PAS on gonioscopy |
| Intraocular Pressure | Often elevated; resistant glaucoma |
| Vision | Blurred due to corneal edema or optic neuropathy from glaucoma |
Representative pictures of anterior segment findings seen in patients clinically suspected to have ICE syndrome (a) small patch of iris atrophy (yellow arrow) with slight corectopia (white arrow) (b) multiple patches of iris atrophy with corectopia (c) ectropion uvea with a distorted pupil (d and e) iris atrophy, iris holes and severe corectopia (f) diffuse corneal edema with iris details visible faintly (g) broad PAS seen on gonioscopy (yellow arrow) (h) “hammered silver” appearance of endothelium seen on slit lamp biomicroscopy (white arrow)
Malhotra, Chintan; Seth, Natasha G; Pandav, Surinder S; Jain, Arun K; Kaushik, Sushmita; Gupta, Amit; Raj, Srishti; Dhingra, Deepika. Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. Indian Journal of Ophthalmology 67(5):p 604-610, May 2019. | DOI: 10.4103/ijo.IJO_1237_18
| Modality | Findings |
|---|---|
| Slit-lamp Biomicroscopy | Shows corneal changes, iris abnormalities. |
| Gonioscopy | Detects PAS, closed angles. |
| Specular Microscopy | Abnormal endothelial cells with epithelial features. |
| Anterior Segment OCT / UBM | Shows irido-corneal adhesions, angle anatomy. |
| Tonometry | Raised IOP with poor response to medical therapy. |