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Iridocorneal endothelial syndrome.

Hua Ma1, Mingfang Xia1,2,3,4, Qing Gu1

  • 1Glaucoma of Ophthalmology, Ningxia Aier Eye Hospital, Yinchuan, Ningxia, China.

Frontiers in Ophthalmology
|October 23, 2025
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Summary

Iridocorneal endothelial syndrome involves corneal cell migration affecting one eye, presenting as iris or corneal changes. Management strategies vary based on specific clinical variations and complications like glaucoma.

Keywords:
Chandler syndromeCogan-Reese syndromeglaucomairidocorneal endothelial syndromeprogressive iris atrophy

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Area of Science:

  • Ophthalmology
  • Corneal Diseases
  • Uveitis

Background:

  • Iridocorneal endothelial syndrome (ICES) is a group of ocular disorders affecting one eye.
  • It is characterized by the abnormal migration of corneal endothelial cells to the iridocorneal angle and iris.
  • ICES typically affects young to middle-aged women and is usually not familial.

Purpose of the Study:

  • To describe the clinical spectrum and management of iridocorneal endothelial syndrome.
  • To outline the distinct variations: progressive essential iris atrophy, Chandler syndrome, and Cogan-Reese syndrome.
  • To review treatment options for corneal and iris manifestations, as well as secondary glaucoma.

Main Methods:

  • Clinical case review and literature synthesis.
  • Description of diagnostic features for each syndrome variation.
  • Overview of surgical and medical treatment modalities.

Main Results:

  • Three main clinical variations of ICES are identified: progressive essential iris atrophy, Chandler syndrome, and Cogan-Reese syndrome.
  • Corneal manifestations like edema may require endothelial keratoplasty (Descemet's stripping automated endothelial keratoplasty, Descemet membrane endothelial keratoplasty).
  • Iris abnormalities can be managed with femtosecond-assisted keratopigmentation, and secondary glaucoma with trabeculectomy or glaucoma drainage devices.

Conclusions:

  • Iridocorneal endothelial syndrome presents with diverse ocular manifestations requiring tailored management.
  • Surgical interventions like keratoplasty, keratopigmentation, and glaucoma surgery are crucial for visual preservation.
  • Early diagnosis and appropriate treatment are essential for managing this rare condition.