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Iridocorneal syndrome: causes, symptoms, diagnosis, treatment

Medical expert of the article

Ophthalmologist
, medical expert
Last reviewed: 08.07.2025

Iridocorneal syndrome is a group of secondary closed-angle glaucomas with symptoms of angle block. This syndrome includes the following pathologies:

  • essential iris atrophy;
  • Chandler syndrome;
  • Cogan-Reese syndrome (iris nevus.

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Epidemiology of iridocorneal syndrome

Iridocorneal syndrome is rare, and its exact prevalence is unknown. Typically, the disease is seen in middle-aged women, with one eye affected.

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Pathophysiology of iridocorneal syndrome

The three iridocorneal syndromes share the same pathophysiological mechanism. The corneal endothelium abnormally grows through the anterior chamber angle and covers the surface of the iris, giving the iris its characteristic features. Initially, the anterior chamber angle is open but blocked. Over time, the endothelial membrane contracts, secondarily closing the angle and deforming the pupil and iris.

Symptoms of iridocorneal syndrome

In the early stages of the disease, patients have no symptoms. Later, patients notice decreased vision in one eye and an abnormal appearance of the iris. As intraocular pressure increases, the patient complains of pain and/or redness of the eye.

Diagnosis of iridocorneal syndrome

Biomicroscopy

In one eye, the endothelial layer of the cornea looks like a thin layer of forged metal.

Some anomalies of the iris are noted, more specific for each individual disease.

  • Essential iris atrophy: areas of thinning, a dystopic and deformed pupil are visible, appearing as the endothelial membrane that tightens the iris contracts.
  • Chandler syndrome: iris changes are almost identical to those in essential iris atrophy, but there is marked corneal edema and corneal changes are more noticeable.
  • Cogan-Reese syndrome: The iris has a flattened appearance with small nodules in the normal iris tissue protruding from openings in the endothelial layer, giving it a "mushroom spot" appearance.

Gonioscopy

At the early stage of the disease, gonioscopy of the anterior chamber angle does not reveal any pathology. Later, wide and uneven peripheral anterior synechiae block part of the angle or the entire angle.

Posterior pole

The posterior pole is unchanged, except for a certain degree of glaucomatous excavation of the optic disc with an increase in intraocular pressure.

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Treatment of iridocorneal syndrome

It is impossible to control intraocular pressure with the help of drug therapy; surgical intervention is necessary. The following operations are performed: trabeculectomy with the use of an antimetabolite drug, drainage implantation and cyclodestructive procedures. In case of significant vision loss due to corneal edema, keratoplasty is performed.


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