Essential iris atrophy

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Classification according to ICD-10
H21.2 Degeneration of the iris and ciliary body
ICD-10 online (WHO version 2019)

The Essential iris atrophy is a very rare form of iridocorneal endothelial syndrome with the main features of progressive atrophy of the iris , corneal edema, ectopia of the cornea and the uvea .

Synonyms are: English ICE syndrome, essential iris atrophy type; iridocorneal endothelial (ICE) syndrome, essential iris atrophy; progressive essential iris atrophy

The first description comes from the year 1903 by the doctor C. Harms.

distribution

The frequency and cause are unknown. So far, over 80 people have been reported.

Clinical manifestations

Clinical criteria are:

  • progressive iris atrophy with holes on the surface
  • Corneal edema
  • Ectopy of the uvea
  • anterior synechia
  • often secondary glaucoma

Differential diagnosis

The other forms of the iridocorneal endothelial syndrome must be distinguished.

literature

  • S. Bandyopadhyay, G. Biswas, J. Biswas, D. Das: A case of essential iris atrophy in a male patient. In: Journal of the Indian Medical Association. Vol. 108, No. 9, September 2010, pp. 609-610, PMID 21510538 .
  • PP Pezzi, M. Marenco, P. Cosimi, G. Mannino, L. Iannetti: Progression of essential iris atrophy studied with confocal microscopy and ultrasound biomicroscopy: a 5-year case report. In: Cornea. Vol. 28, No. 1, January 2009, pp. 99-102, doi : 10.1097 / ICO.0b013e3181822579 , PMID 19092417 .
  • E. Fourmaux, L. Velasque: Atrophie essentielle de l'iris associée à un œdème maculaire cystoïde chronique. In: Journal francais d'ophtalmologie. Vol. 28, No. 4, April 2005, pp. 407-410, PMID 15973203 .

Individual evidence

  1. ↑ a b Iris atrophy, essential. In: Orphanet (Rare Disease Database).
  2. ↑ C. Harms: Unilateral spontaneous gap formation in the iris due to atrophy without mechanical strain. in: Clinical monthly sheets for ophthalmology. 1903; Vol. 41, pp .: 22-8.
  3. ↑ M. Amsler , E. Landolt: To essential iris atrophy. In: Ophthalmologica. Vol. 135, 1958, p. 584, doi : 10.1159 / 000303356 .

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