Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort

PURPOSE: To evaluate the patient demographics and morphological characteristics of corneal endothelium by in vivo confocal microscopy (IVCM), in patients with Iridocorneal Endothelial (ICE) Syndrome. METHODS: In this retrospective observational series, IVCM acquired endothelial images of patients with ICE syndrome were evaluated. ‘ICE cells’ morphology was classified as “−” or “+” if they were larger or smaller than contralateral normal endothelium. It was correlated with patient demographics and clinical manifestations. RESULTS: IVCM was performed on 41 eyes of 21 patients, with 13 males (62%) and 8 females (38%). The disease was unilateral in 19 (90.5%) and bilateral but asymmetric in two (9.5%) patients. Total ICE was seen in 91% eyes. Eighty percent patients (12 out of 15) with ICE—cells were males while 83.3% (5 out of 6) patients with ICE + cells were females. Mean age of patients with ICE- cell type and ICE + cell type was 45.8 ± 17.8 years and 40.3 ± 9.2 years respectively (P = 0.02). Both ICE – and ICE + eyes had similar incidence (33.3%) of corneal edema. ICE + eyes had more severe (grades 2/3) glaucoma (n = 5/6 eyes, 83.3%) compared to ICE – eyes (n = 8/15 eyes, 53.3%). CONCLUSION: A male preponderance, predilection of ICE – and + cell variants for male and female gender respectively, lack of association of the endothelial cell morphology with corneal edema, and apparent association of ICE + phenotype with more severe glaucoma occurring at a relatively younger age, are some novel findings of the present study. In the clinical setting correlation of patient demographics with these IVCM findings may help in better long-term prognostication of eyes with ICE syndrome.