Iridocorneal Endothelial Syndrome

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Classification according to ICD-10
H21.1 Degeneration of the iris and ciliary body
ICD-10 online (WHO version 2019)

The iridocorneal endothelial syndrome (ICE syndrome) is a group of very rare diseases of the cornea with progressive proliferation of the endothelium .

The group includes:

The clinical pictures differ in the extent of the atrophy of the iris and changes in the cornea .

The name was coined in 1979 by the American ophthalmologist Myron Yanoff.

distribution

The cause and frequency are not known, the disease usually occurs in women between the ages of 20 and 50.

Virus infections such as herpes simplex viruses or Epstein-Barr virus are discussed as the cause .

Clinical manifestations

Clinical criteria are:

  • Mostly one-sided
  • Holes, atrophy of the iris
  • Corneal edema
  • Deformation of the pupil
  • Synechiae

Frequent complications are secondary glaucoma up to 50%, possibly with neuropathy of the optic nerve and nodular formation of the iris.

therapy

Treatment is mainly directed against glaucoma and corneal dystrophy .

literature

  • P. Chandran, HL Rao, AK Mandal, NS Choudhari, CS Garudadri, S. Senthil: Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. In: PloS one. Vol. 12, No. 3, 2017, p. E0171884, doi: 10.1371 / journal.pone.0171884 , PMID 28282413 , PMC 5345787 (free full text).
  • S. Imamoglu, MS Sevim, HE Yıldız, ET Vural, H. Bardak, Y. Bardak: Surgical outcomes of patients with iridocorneal endothelial syndrome: a case series. In: International ophthalmology. Vol. 37, No. 3, June 2017, pp. 607-613, doi: 10.1007 / s10792-016-0317-1 , PMID 27495952 .
  • T. Gračner, S. Trpin, T. Šarenac, D. Pahor: Diagnostic methods in the clinical examination of iridocorneal endothelial syndrome. In: Der Ophthalmologe: Journal of the German Ophthalmological Society. Vol. 113, No. 12, December 2016, pp. 1074-1077, doi: 10.1007 / s00347-016-0244-6 , PMID 27023272 .
  • EP Aponte, DC Ball, WL Alward: Iridocorneal Endothelial Syndrome in a 14-Year-Old Male. In: Journal of glaucoma. Vol. 25, No. 2, February 2016, pp. E115 – e116, doi: 10.1097 / IJG.0000000000000288 , PMID 26035422 .

Individual evidence

  1. ↑ a b c Iridocorneal Endothelial Syndrome. In: Orphanet (Rare Disease Database).
  2. ↑ a b American Academy of Ophthalmology
  3. ↑ Iris atrophy, essential. In: Orphanet (Rare Disease Database).
  4. ↑ M. Yanoff: Iridocorneal endothelial syndrome: unification of a disease spectrum. In: Survey of ophthalmology. Vol. 24, No. 1, 1979 Jul-Aug, pp. 1-2, PMID 483158 .
  5. ↑ a b c J. Herd: Iridocorneo-endothelial syndrome (ICE-S): classification, clinic and diagnosis. In: Clinical monthly sheets for ophthalmology. Vol. 222, No. 10, October 2005, pp. 797-801, doi: 10.1055 / s-2005-858119 , PMID 16240272 (review).

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