Central cloud-shaped corneal dystrophy François

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

The central cloud-shaped corneal dystrophy François (CCDF) is a very rare congenital form of a corneal dystrophy affecting the stroma with angular or rounded opacities in the cornea without impairment of vision .

Synonym : corneal dystrophy, central cloud-shaped, François type

The name refers to the author of the first description from 1958 by the Belgian ophthalmologist Émile Jules Marie Joseph François .

distribution

The frequency is given as less than 1 in 1,000,000, inheritance is autosomal dominant .

root cause

The cause is not known.

Clinical manifestations

Clinical criteria are:

  • Disease manifestation in the first 10 years of life
  • no symptoms and no progression
  • Incidental finding with central, cloud-shaped, opacities in the stroma of the cornea

Differential diagnosis

Different forms of corneal dystrophy and the clinically indistinguishable degenerative corneal disease of the Vogt type (crocodile shagreen of Bowman's membrane) are to be distinguished .

literature

  • CA Utine, D. Oral, M. Altunsoy, D. Basar, LM Alimgil: In Vivo Confocal Microscopy in Presumed Central Cloudy Dystrophy of François. In: Ophthalmic surgery, lasers & imaging: the official journal of the International Society for Imaging in the Eye. [Electronic publication before printing] March 2010, doi: 10.3928 / 15428877-20100216-02 , PMID 20337272 .
  • CL Karp, IU Scott, WR Green, TS Chang, WW Culbertson: Central cloudy corneal dystrophy of Francois. A clinicopathologic study. In: Archives of ophthalmology. Vol. 115, No. 8, August 1997, pp. 1058-1062, PMID 9258229 .

Individual evidence

  1. ↑ a b c d Central cloud-shaped dystrophy François. In: Orphanet (Rare Disease Database).
  2. ↑ IC3D classification
  3. ↑ J. François: L'hérédité en ophthalmology. In: Bulletin de la Societe belge d'ophtalmologie. Vol. 118, Pt 1 1958, pp. 1-300, PMID 13618716 .
  4. ↑ CCDF.  In: Online Mendelian Inheritance in Man . (English)