Sclerocornea
| Classification according to ICD-10 | |
|---|---|
| Q13.3 | Congenital corneal opacity |
| ICD-10 online (WHO version 2019) | |
The sclerocornea is a very rare congenital clouding of the cornea . There is a non- inflammatory vascularization with scarring of the cornea, mostly on the edge. The entire cornea is rarely scarred.
The first description is from 1985 by James H. Elliot and co-workers.
"Sclerocornea" means that the cornea is cloudy and what the sclera looks like, it is one of the most common causes of congenital blindness .
The frequency and cause are unknown, so far fewer than 100 people have been reported to be affected. It is an autosomal dominant supposed inheritance.
The disease usually occurs on both sides without any further abnormalities or associated malformations , more often sporadic than familial inheritance; there are also supposed to be autosomal recessive forms. The extent of the clouding and thus the visual impairment can be very variable.
literature
- A. Mahdavi Fard, L. Pourafkari, ND Nader: Sclerocornea. In: QJM: An International Journal of Medicine . Volume 108, number 11, November 2015, p. 917, doi: 10.1093 / qjmed / hcv110 , PMID 26025687 .
- RD Young, AJ Quantock, C. Sotozono, N. Koizumi, S. Kinoshita: Sulphation patterns of keratan sulphate proteoglycan in sclerocornea resemble cornea rather than sclera. In: The British journal of ophthalmology. Volume 90, number 3, March 2006, pp. 391-393, doi: 10.1136 / bjo.2005.085803 , PMID 16488970 , PMC 1856931 (free full text).
- JH Elliott, SS Feman, DM O'Day, M. Garber: Hereditary sclerocornea. In: Archives of ophthalmology. Volume 103, Number 5, May 1985, pp. 676-679, PMID 3994576 .
Individual evidence
- ↑ Willibald Pschyrembel : Clinical Dictionary , 266th, updated edition, de Gruyter, Berlin 2014, ISBN 978-3-11-033997-0, keyword sclerocornea
- ↑ sclerocornea Isolated congenital. In: Orphanet (Rare Disease Database).
- ↑ JH Elliott, SS Feman, DM O'Day, M. Garber: Hereditary sclerocornea. In: Archives of ophthalmology. Volume 103, Number 5, May 1985, pp. 676-679, PMID 3994576 .
- ^ RA Rezende, UB Uchoa, R. Uchoa, CJ Rapuano, PR Laibson, EJ Cohen: Congenital corneal opacities in a cornea referral practice. In: Cornea. Volume 23, Number 6, August 2004, pp. 565-570, PMID 15256994 .
- ↑ Autosomal sclerocornea, Dominant. In: Online Mendelian Inheritance in Man . (English)
- ^ University of Arizona
