Fuchs endothelial dystrophy

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Classification according to ICD-10
H18.5 Hereditary corneal dystrophies
ICD-10 online (WHO version 2019)

The Fuchs' dystrophy is an autosomal dominant inherited disease of the cornea of the eye .

Synonyms are:

  • Fuchs corneal epithelial dystrophy
  • Fuchs syndrome II
  • Fuchs-Kraupa syndrome
  • Kraupa syndrome

The name refers to a publication from 1910 by the Austrian ophthalmologist Ernst Fuchs and 1920 by the German ophthalmologist Ernst Kraupa.

Pathogenesis, epidemiology

With age, the endothelial cells on the inside of the cornea perish. This means that they are no longer able to pump water out of the cornea into the anterior chamber. The cornea swells and the corneal epithelium lifts up ( keratitis bullosa ). Even earlier, a thickening of the Descemet membrane due to abnormal endothelial cell products is visible ( cornea guttata ). This is usually only visible in the third to fourth decade of life.

distribution

The frequency is unknown. Women are more often affected. The sex ratio is 3-4: 1. The inheritance is partly autosomal dominant , but sporadic cases have also been described, so that a polygenetic or multifactorial cause can be assumed.

Symptoms

The patient notices a decrease in visual acuity and increased sensitivity to glare . It is characterized by an improvement in the course of the day (because some water evaporates when the lids are open). Later on it can also lead to painful tears in the cornea.

classification

Depending on the genetic basis and age at first onset, 7 forms are currently distinguished. (See web links)

therapy

Saline (hyperosmolar) eye drops, which draw water from the cornea, are used for therapy. If the symptoms progress, a perforating keratoplasty or Descemet Membrane Endothelial Keratoplasty must be performed.

literature

  • A. Knapp: Dystrophia Epithelialis Corneae (Fuchs), with the Report of a Case. In: Transactions of the American Ophthalmological Society. Vol. 12, Pt 3 1911, pp. 745-747, ISSN  0065-9533 . PMID 16692221 . PMC 1322776 (free full text).

Individual evidence

  1. ^ B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9 .
  2. ^ E. Fuchs: Dystrophia epithelialis corneae. In: Albrecht von Graefes Archive for Ophthalmology. 1910, Vol. 76, pp. 478-508.
  3. ^ E. Kraupa: pigmentation of the posterior corneal surface in "Dystrophia epithelialis (Fuchs)". In: Journal of Ophthalmology. Basel, 1920, volume 44, p. 247.
  4. ↑ Fuchs endothelial dystrophy. In: Orphanet (Rare Disease Database).

Web links