Pre-Descemet corneal dystrophy

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

The pre-Descemet dystrophy (PDCD) is a very rare congenital form of the stroma concerned dystrophy with focal fine gray clouding directly in front of the Descemet's membrane without loss of eyesight .

The name was coined in the first description from 1967 by the American ophthalmologists Merill Grayson and Hans Wilbrandt.

distribution

The frequency is given as less than 1 in 1,000,000, the cause and possible heredity are not known.

Clinical manifestations

Clinical criteria are:

  • Onset of illness usually after the age of 30, occasionally also in small children
  • differently shaped opacities within the cornea

Differential diagnosis

Other forms of corneal dystrophy are to be distinguished.

literature

  • H. Shi, XF Qi, TT Liu, Q. Hao, XH Li, LL Liang, YM Wang, ZH Cui: In vivo confocal microscopy of pre-Descemet corneal dystrophy associated with X-linked ichthyosis: a case report. In: BMC ophthalmology. Vol. 17, No. 1, March 2017, p. 29, doi: 10.1186 / s12886-017-0423-5 , PMID 28302098 , PMC 5356324 (free full text).
  • L. Lagrou, J. Midgley, KG Romanchuk: Punctiform and Polychromatophilic Dominant Pre-Descemet Corneal Dystrophy. In: Cornea. Vol. 35, No. 4, April 2016, pp. 572-575, doi: 10.1097 / ICO.0000000000000772 , PMID 26845315 .
  • C. Hung, RI Ayabe, C. Wang, RF Frausto, AJ Aldave: Pre-Descemet corneal dystrophy and X-linked ichthyosis associated with deletion of Xp22.31 containing the STS gene. In: Cornea. Vol. 32, No. 9, September 2013, pp. 1283-1287, doi: 10.1097 / ICO.0b013e318298e176 , PMID 23807007 , PMC 3740086 (free full text).

Individual evidence

  1. ↑ a b c Pre-Descemet corneal dystrophy. In: Orphanet (Rare Disease Database).
  2. ↑ IC3D classification
  3. ↑ M. Grayson, H. Wilbrandt: Pre-descemet dystrophy. In: American journal of ophthalmology. Vol. 64, No. 2, August 1967, pp. 276-282, PMID 5298532 .