The iridocorneal endothelial syndrome

Surv Ophthalmol. 2018 Sep-Oct;63(5):665-676. doi: 10.1016/j.survophthal.2018.01.001. Epub 2018 Jan 11.


The iridocorneal endothelial syndrome represents a unique group of ocular pathologies (Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome) characterized by the proliferation of corneal endothelial cells that migrate toward the iridocorneal angle and iris surface causing, to a degree varying according to the subtype, corneal edema and decompensation and secondary glaucoma, whether by obstructing the angle or producing peripheral anterior synechiae by contraction of the basement membrane of the migrating cells over the surface of the iris. A triggering factor, possibly viral, induces the corneal endothelial cells to proliferate and behave like epithelial cells. Diagnosis is made based on typical ocular findings on the cornea and iris. Iridocorneal endothelial syndrome is more frequent in young women, with unilateral involvement in most cases. In vivo confocal microscopy is an excellent diagnostic tool, especially in borderline presentations like early cases of Chandler syndrome, which affects the cornea predominantly. Typical clinical management consists of treating the corneal edema and decompensation, where endothelial keratoplasty techniques have replaced in many cases the need for a penetrating keratoplasty and treating the secondary glaucoma, which usually requires surgical intervention.

Keywords: Chandler's syndrome; Cogan-Reese syndrome; corneal edema; glaucoma; keratoplasty; progressive iris atrophy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Corneal Transplantation / methods
  • Diagnosis, Differential
  • Diagnostic Techniques, Ophthalmological
  • Endothelium, Corneal / surgery
  • Glaucoma / etiology
  • Glaucoma / surgery
  • Humans
  • Iridocorneal Endothelial Syndrome* / complications
  • Iridocorneal Endothelial Syndrome* / diagnosis
  • Iridocorneal Endothelial Syndrome* / etiology
  • Iridocorneal Endothelial Syndrome* / therapy
  • Microscopy, Confocal