Iridocorneal endothelial syndrome: A contemporary review of pathogenesis, multimodal diagnosis, and management.

Authors

Fabián Quirós-Mata, Mauricio Muleiro-Alvarez, Guillermo Raul Vera-Duarte, Arturo Castillo-Rincón, Anna Rebeca Mattsson Reyes, Arturo Ramirez-Miranda, Alejandro Navas, Enrique O Graue-Hernandez, Mbbs Abha Gour, Julie Pegu, Virender Singh Sangwan, Nicolás Kahuam-López

Published in

Survey of ophthalmology. Sep 03, 2026. Epub Sep 03, 2026.

Abstract

Iridocorneal endothelial (ICE) syndrome is a rare acquired anterior segment disorder characterized by abnormal proliferation and migration of corneal endothelial cells, leading to progressive angle closure, iris abnormalities, secondary glaucoma, and corneal decompensation. Historically divided into Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome, ICE is now recognized as a disease spectrum with a shared pathophysiologic basis. Diagnosis and management remain challenging because of variable clinical manifestations and progressive vision-threatening complications. We provide a comprehensive review of ICE syndrome, presenting an updated overview relevant to ophthalmologists on epidemiology, pathogenesis, multimodal diagnosis, differential diagnosis, and current management strategies while highlighting emerging molecular insights and future therapeutic directions.

PMID:
42692090
Bibliographic data and abstract were imported from PubMed on 04 Sep 2026.

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