The Iridocorneal Endothelial Syndrome

Author(s): Silva L (1), Najafi A (2), Suwan Y (3), Teekhasaenee C (4), Ritch R (2)

1 Einhorn Clinical Research Center, The New York Eye and Ear Infirmary of Mount Sinai. 310 East 14th Street. South building. Suite 304. New York, NY 10003. Electronic address: lsilva@nyee.edu.
2 Einhorn Clinical Research Center, The New York Eye and Ear Infirmary of Mount Sinai. 310 East 14th Street. South building. Suite 304. New York, NY 10003.
3 Einhorn Clinical Research Center, The New York Eye and Ear Infirmary of Mount Sinai. 310 East 14th Street. South building. Suite 304. New York, NY 10003; Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
4 Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

The iridocorneal endothelial syndrome (ICE) 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. ICE 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.

Surv Ophthalmol. 2018 Jan 10. pii: S0039-6257(17)30112-1. doi: 10.1016/j.survophthal.2018.01.001.

http://www.ncbi.nlm.nih.gov/pubmed/29331589

Keywords: Chandler syndrome; Cogan-Reese syndrome; Glaucoma; corneal edema; keratoplasty; progressive iris atrophy

Clinical Paper of the Month manager: Andreas Boehm