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Iridocorneal endothelial syndrome

Disease definition

Iridocorneal endothelial (ICE) syndrome describes a group of progressive corneal proliferative endotheliopathies comprised of Chandler’s syndrome, Cogan-Reese syndrome and essential iris atrophy (see these terms), affecting mainly young adult females and characterized by iris holes and atrophy, papillary distortion, anterior synechiae, corneal edema and often with secondary glaucoma and corneal decompensation as complications

ORPHA:64734

  • Synonym(s):
    • ICE syndrome
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: H21.1
  • OMIM: -
  • UMLS: C0339285  C1096100
  • MeSH: D057129
  • GARD: 60
  • MedDRA: 10053678

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