Clinical Features of Iridocorneal Endothelial Syndrome in Korean Patients

Abstract

Purpose: To document the distribution and clinical features of iridocorneal endothelial (ICE) syndrome in its various subtypes observed in patients who visited a single institution in Korea.Methods: A retrospective analysis was conducted on the medical records of patients diagnosed with ICE syndrome at the Department of Ophthalmology, Yeungnam University Hospital, from 1993 to 2023. The cases were classified into Chandler syndrome, progressive iris atrophy, and iris nevus syndrome based on the severity of corneal and iris abnormalities. We also examined the presence of glaucoma, corneal edema, and iris abnormalities at diagnosis.Results: In all, 22 eyes from 21 patients with ICE syndrome were included. The mean age at diagnosis was 56.6 ± 16.1 years with a female predominance (20 patients, 95.5%). The syndrome was primarily unilateral (20 patients, 95.5%). Subtypes included 9 eyes with Chandler syndrome (40.9%), 7 eyes with progressive iris atrophy (31.8%), and 6 eyes with iris nevus syndrome (27.3%). Glaucoma was present in 15 eyes (68.2%), with 8 eyes (36.4%) undergoing glaucoma surgery. Of the 7 eyes that had trabeculectomy as initial glaucoma surgery, six experienced poor intraocular pressure control leading to Ahmed valve implantation in 4 cases. Corneal edema was noted in 10 eyes (45.5%) at diagnosis progressing to corneal decompensation in 6 eyes (27.3%).Conclusions: ICE syndrome in Korea predominantly affects middle-aged women and occurs unilaterally with Chandler syndrome being the most common subtype. Given the high risk for glaucoma development, suboptimal outcomes of trabeculectomy, and significant risk for corneal decompensation, ongoing management efforts are essential for glaucoma and corneal function preservation.