Appendix
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Ophthalmology

Iridocorneal Endothelial Syndrome

Iridocorneal endothelial syndrome (ICE) is a rare condition characterized by the increased proliferation and migration of corneal endothelial cells to the iris and iridocorneal angle, leading to secondary angle-closure glaucoma, corneal edema, and atrophy of the iris. ICE is a group of disorders with three clinically distinct variants, including essential iris atrophy (EIA), Chandler syndrome (CS), and Cogan-Reese syndrome (CRS). If left untreated, each variant may progress to blindness. However

v1 · Reviewed Nov 7, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • If left untreated, each variant may progress to blindness.
  • prompt diagnosis and multidisciplinary management can mitigate this catastrophic outcome.
  • Vision loss typically occurs due to the progression of secondary glaucoma and corneal decompensation.
  • Patients may also develop ocular pain or headache if they have a severe increase in IOP due to secondary glaucoma.
  • Early diagnosis of ICE is essential to prevent the complications of corneal edema and secondary glaucoma.
  • A thorough physical examination includes a slit-lamp examination, a careful exam of the angle with gonioscopy, and tonometry or applanation.
  • Techniques such as specular microscopy and in vivo corneal confocal microscopy (IVCM) can reveal pathognomonic findings.
  • Ophthalmic examination of the corneal endothelium may reveal “hammered silver” or “beaten bronze” irregularities during slit-lamp examination, a reversal light pattern during specular microscopy, or changes in the iridocorneal angle on gonioscopy.
  • Cupping of the optic nerve may be seen in patients with secondary glaucoma).
  • If gonioscopy is challenging due to corneal edema, ultrasound biomicroscopy can be used to visualize the anterior chamber angle and detect abnormalities.
  • Patients with ICE and subsequent secondary glaucoma should be evaluated with Humphrey visual field testing, central corneal thickness measurements, intraocular pressure evaluation with Goldmann applanation tonometry, evaluation of the iridocorneal angle and trabecular meshwork with gonioscopy, optic nerve evaluation on physical examination, as well as optical coherence tomography of the optic nerve head and retinal nerve fiber layer.
  • The treatment of ICE is typically centered around managing corneal decompensation and preventing irreversible glaucomatous vision loss.
  • Endothelial keratoplasty techniques such as Descemet stripping automated endothelial keratoplasty (DSAEK) and Descemet membrane endothelial keratoplasty (DMEK) are preferred over penetrating keratoplasty (PK) for the treatment of ICE-related corneal edema.
  • The primary treatment regimen for secondary glaucoma includes topical beta blockers, carbonic anhydrase inhibitors, and alpha agonists.
beta blockers
topical
Adult
Not specified in source
Renal
Not specified in source
Duration
Not specified in source
carbonic anhydrase inhibitors
topical
Adult
Not specified in source
Renal
Not specified in source
Duration
Not specified in source
alpha agonists
topical
Adult
Not specified in source
Renal
Not specified in source
Duration
Not specified in source
  • Topical prostaglandins have been associated with intraocular reactivation of HSV and should be avoided in patients with ICE.
  • If patients have failed multiple keratoplasty procedures, keratoprosthesis may be considered.
  • ICE-induced secondary glaucoma may be very challenging to control.
  • If IOP is not adequately controlled with topical ophthalmic medications, surgical interventions such as goniotomy, trabeculectomy with antifibrotic agents such as mitomycin-C or 5-fluorouracil, glaucoma drainage implants, or cyclodestructive procedures must be considered.
  • Goniotomy is an excellent surgical option to relieve the obstruction to aqueous outflow caused by the ICE membrane over the angle.
  • Femtosecond-assisted keratopigmentation (KTP) has shown promising results in eliminating the diplopia and photophobia common in patients with ICE.
  • Alternatively, multiple endocapsular iris prosthesis devices can be implanted inside the capsular bag in front of the intraocular lens to address iris atrophy and photophobia and improve cosmesis.
  • The complications of ICE include iris abnormalities, corneal edema and decompensation, and secondary glaucoma.
  • Glaucoma occurs in approximately 50% of patients with ICE and is more common in the EIA variant.
  • Corneal edema and decompensation, however, are higher in the CS subtype.
  • Patients with ICE and subsequent secondary glaucoma should be evaluated with Humphrey visual field testing, central corneal thickness measurements, intraocular pressure evaluation with Goldmann applanation tonometry, evaluation of the iridocorneal angle and trabecular meshwork with gonioscopy, optic nerve evaluation on physical examination, as well as optical coherence tomography of the optic nerve head and retinal nerve fiber layer.
  • Ensuring patients are educated about ICE, including its symptoms, available treatment options, and the significance of medication adherence and follow-up visits, is paramount.
  • review
    Iridocorneal Endothelial Syndrome. StatPearls [Internet] — Shibandri Das, Koushik Tripathy — StatPearls Publishing, 2023

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov