title: Iridocorneal endothelial syndrome masquerading as iris melanoma in 71 cases.
abstract: 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.
authors.affiliation: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA 19107, USA. carol.shields@shieldsoncology.com
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Margaret V
authors.initials: MV
authors.lastname: Shields
authors.forename: Vanessa
authors.initials: V
authors.lastname: Viloria
authors.forename: Haley
authors.initials: H
authors.lastname: Pearlstein
authors.forename: Emil Anthony T
authors.initials: EA
authors.lastname: Say
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D049109:Cell Proliferation; D003937:Diagnosis, Differential; D004728:Endothelium, Corneal / Q000473:pathology; D005127:Eye Color; D005260:Female; D006801:Humans; D057129:Iridocorneal Endothelial Syndrome / Q000175:diagnosis*; D015811:Iris Neoplasms / Q000175:diagnosis*; D008297:Male; D008545:Melanoma / Q000175:diagnosis*; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology; D014129:Trabecular Meshwork / Q000473:pathology; D055815:Young Adult