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Abstract #45502 Published in IGR 13-2
Iridocorneal Endothelial Syndrome Masquerading as Iris Melanoma in 71 Cases
Shields CL; Shields MV; Viloria V; Pearlstein H; Say EA; Shields JAArchives of Ophthalmology 2011; 129: 1023-1029
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.
Wills Eye Institute, Thomas Jefferson University, PA, USA.
Classification:
9.4.2.1 Iridocorneal endothelial syndrome (ICE, incl. irisatrophy) (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.2 Glaucomas associated with disorders of the cornea, conjunctiva, sclera)
9.4.8 Glaucomas associated with intraocular tumors (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
10 Differential diagnosis e.g. anterior and posterior ischemic optic neuropathy