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Iridocorneal Endothelial Syndrome (ICE)

URGENT — Glaucoma

Symptoms

usually asymptomatic in early stages

later: irregular pupil shape, blurred vision, or monocular diplopia

pain and photophobia if iop rises or corneal edema develops

strictly unilateral; typically affects women aged 20–50

sporadic presentation (not inherited)

Clinical Findings

Signature endothelial signs

  • "beaten-bronze" or hammered-silver appearance of the posterior cornea
  • specular microscopy (diagnostic): large, pleomorphic, dark "ice cells" with reversed polarity
  • corneal edema can occur even at normal or only mildly elevated iop

Iris & angle changes

  • progressive corectopia (displaced pupil) and polycoria (multiple iris holes)
  • gonioscopy: high PAS extending anterior to schwalbe line (nearly pathognomonic)
  • secondary angle-closure glaucoma from contracting endothelial membranes over the trabecular meshwork

Clinical subtypes

  • chandler syndrome: corneal edema and "beaten-bronze" signs dominate; minimal iris changes
  • progressive iris atrophy: marked full-thickness iris holes and corectopia
  • cogan–reese syndrome: pedunculated nodules (islands of iris) on the anterior iris surface

Etiology

Pathogenesis

  • abnormal corneal endothelium acquires epithelial-like properties and migrates across the angle and iris
  • a contracting basement-membrane-like sheet pulls the iris forward, creating holes and closing the angle
  • etiology is unknown; a viral trigger (hsv or ebv) has been postulated

Red Flags

unilateral corneal edema with pupillary distortion in a middle-aged woman

progressive secondary glaucoma that is refractory to medical therapy

bilateral findings: reconsider axenfeld-rieger or posterior polymorphous dystrophy

rapid endothelial graft failure due to recipient cell migration

Investigations

Diagnostics

  • specular microscopy: essential to identify characteristic ice cells
  • gonioscopy: to identify anteriorly displaced pas and angle closure
  • slit-lamp exam: document corectopia, iris nodules, and "beaten-bronze" reflex

Monitoring

  • baseline glaucoma workup: iop, optic nerve assessment, and visual fields
  • ultrasound biomicroscopy (ubm): if corneal edema obscures angle visualization

Treatment

IOP management

  • medical: aqueous suppressants are first-line
  • surgical: tube shunts are generally preferred over trabeculectomy (which often fails due to membrane overgrowth)

Corneal management

  • dmek or dsaek for corneal decompensation
  • note: recipient endothelium may continue to migrate, leading to graft failure

Iris management

  • surgical reconstruction or prosthetic iris for cosmetic or functional (glare) concerns

Clinical Pearl

A middle-aged woman with unilateral iris atrophy, corectopia, and secondary glaucoma has ICE syndrome — the abnormal endothelium crawls across the angle and iris, and tube shunts outperform trabeculectomy here.

Differential Diagnosis

  • axenfeld–rieger syndrome (bilateral, inherited, systemic signs)
  • posterior polymorphous corneal dystrophy (ppcd) (bilateral, inherited)
  • iridoschisis
  • prior uveitis with posterior synechiae

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