Iridocorneal endothelial syndrome masquerading as iris melanoma in 71 cases.

Objective: To describe the clinical features of iridocorneal endothelial (ICE) syndrome in a group of patients referred because of suspected iris melanoma.

Methods: In a noncomparative case series, we performed medical record review for clinical features of ICE syndrome in 71 patients.

Results: At presentation, the median patient age was 54 years. All patients were referred for evaluation of a pigmented iris mass, suspected to be a melanoma. The iris color was blue or green in 51 (72%) and brown in 20 (28%). The mass proved to be a combination of iris stromal atrophy in 41 cases (58%) with exposure or loss of the underlying iris pigment epithelium; ectropion iridis in 24 (34%), imparting a disfigured iris with dark-brown color; iris nodules in 5 (7%); traction elevation with iris distortion from peripheral anterior synechia in 57 (80%); and corectopia in 53 (75%), a feature commonly found with iris melanoma. The mean extent of iris atrophy was 2 clock hours. Ectropion iridis was unidirectional in 10 and multidirectional in 14. Additional features of ICE included corneal endothelial guttata-like changes in 33 (46%), corneal edema in 7 (10%), iris pigment epithelial transillumination defects in 12 (17%), polycoria in 1 (1%), and secondary glaucoma with intraocular pressure higher than 22 mm Hg in 7 (10%).

Conclusions: Iridocorneal endothelial syndrome can simulate iris melanoma. Features more suggestive of ICE syndrome include corneal endothelial guttata-like changes and edema, peripheral anterior synechia, multidirectional ectropion iridis, and iris atrophy.