Jean Marie Pagani, OD

Disclosures

February 25, 2016

Case Diagnosis

The correct diagnosis is iridocorneal endothelial (ICE) syndrome. ICE syndrome should be in the differential diagnosis for any young to middle-aged adult who presents with an acquired unilateral disorder associated with iris anomalies, corneal decompensation, and unilateral glaucoma.

Posterior polymorphous corneal dystrophy is an autosomal dominant disorder that presents as a bilateral endothelial corneal dystrophy. Ocular findings include corectopia, corneal edema, iridocorneal adhesions, and angle closure or open-angle glaucoma. Whereas endothelial cells appear on specular microscopy as dark areas with central highlights in ICE syndrome, posterior polymorphous corneal dystrophy shows typical vesicles and bands. On microscopic examination, multilayered endothelial cells look and behave like epithelial cells.[1]

Traumatic glaucoma presents with a history of trauma and elevated intraocular pressure, with potential for optic nerve damage. Angle recession may be seen on gonioscopy.

Axenfeld-Rieger syndrome is an autosomal dominant disorder that presents bilaterally in young patients. Developmental abnormalities can be seen in the anterior chamber angle, iris, and trabecular meshwork. Corectopia, polycoria, ectropion uveae, posterior embryotoxon, and increased intraocular pressure are common findings. Corneal endothelial changes are usually not seen.[1]

Clinical Care and Treatment

Topical medication for the glaucoma is the first-line therapy in the setting of ICE syndrome. Topical beta-blockers, alpha-agonists, and carbonic anhydrase inhibitors are typically prescribed. Because inflammation and the herpes simplex virus are proposed etiologies in ICE syndrome, prostaglandins should be used with caution.[2]

Corneal edema can be a presenting sign and may be exacerbated by elevated intraocular pressure. Thus, patients would benefit from intraocular pressure reduction and the use of hypertonic preparations.

At times, both glaucoma-filtering procedures and corneal transplant are necessary, owing to the challenges associated with treating ICE syndrome.[3]

Case Outcome

This patient (who did not have a sulfa allergy) was treated with dorzolamide hydrochloride ophthalmic solution, 1 drop three times a day in the right eye only. The intraocular pressure in the affected eye was 16 mm Hg at his first follow-up appointment. He will continue to be closely monitored with repeated optical coherence tomography, visual fields, and gonioscopy. If there is any change to the left eye, bilateral treatment will be considered. He also was counseled on the progressive nature of his disease and the need for compliance with both topical drops and follow-up appointments.

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