Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects

Purpose To report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with Iridocorneal endothelial (ICE) syndrome. Methods Retrospective review of 203 consecutive subjects with ICE syndrome at a tertiary eye care centre between January 1988 and June 2013. Results ICE syndrome was present in 223 eyes of 203 subjects, 124 (61%) were female and 79 (39%) were male. The median age at presentation was 43 years (1st (Q1) and 3rd (Q3) quartile; 34, 51 years). ICE syndrome was unilateral in 183 (90%) subjects, and bilateral in 20 (10%) subjects. The most common clinical variant was progressive iris atrophy (PIA, 115; 52% eyes), followed by Chandler syndrome (CS, 87; 39% eyes) and Cogan-Reese syndrome (CRS, 21; 9% eyes). Glaucoma was found in 156 eyes (70%) at presentation and the median (Q1, Q3) intraocular pressure in eyes with glaucoma was 24 (16, 38) mm Hg. Seven eyes developed glaucoma during the follow-up period, increasing the percentage of eyes with glaucoma to 73%. Intraocular pressure was managed medically in 81 eyes (50%) and the other 82 eyes (50%) required surgical intervention. Corneal edema was present in 124 eyes (56%) of which, 32 eyes (14%) required keratoplasty. Conclusions In our study on ICE syndrome in Indian population, the presentation was predominantly uniocular and more common in middle aged women. Progressive iris atrophy was the most common clinical variant. ICE syndrome was associated with glaucoma in over 70% of the eyes and half of the eyes had corneal edema.