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Cornea. 2008 Dec;27 Suppl 2:S1-83. doi: 10.1097/ICO.0b013e31817780fb.

The IC3D classification of the corneal dystrophies.

[Article in English, Spanish]

Author information

  • 1Department of Ophthalmology, Kresge Eye Institute, Wayne State University School of Medicine, Detroit, MI 48201, USA. jweiss@med.wayne.edu

Abstract

BACKGROUND:

The recent availability of genetic analyses has demonstrated the shortcomings of the current phenotypic method of corneal dystrophy classification. Abnormalities in different genes can cause a single phenotype, whereas different defects in a single gene can cause different phenotypes. Some disorders termed corneal dystrophies do not appear to have a genetic basis.

PURPOSE:

The purpose of this study was to develop a new classification system for corneal dystrophies, integrating up-to-date information on phenotypic description, pathologic examination, and genetic analysis.

METHODS:

The International Committee for Classification of Corneal Dystrophies (IC3D) was created to devise a current and accurate nomenclature.

RESULTS:

This anatomic classification continues to organize dystrophies according to the level chiefly affected. Each dystrophy has a template summarizing genetic, clinical, and pathologic information. A category number from 1 through 4 is assigned, reflecting the level of evidence supporting the existence of a given dystrophy. The most defined dystrophies belong to category 1 (a well-defined corneal dystrophy in which a gene has been mapped and identified and specific mutations are known) and the least defined belong to category 4 (a suspected dystrophy where the clinical and genetic evidence is not yet convincing). The nomenclature may be updated over time as new information regarding the dystrophies becomes available.

CONCLUSIONS:

The IC3D Classification of Corneal Dystrophies is a new classification system that incorporates many aspects of the traditional definitions of corneal dystrophies with new genetic, clinical, and pathologic information. Standardized templates provide key information that includes a level of evidence for there being a corneal dystrophy. The system is user-friendly and upgradeable and can be retrieved on the website www.corneasociety.org/ic3d.

Comment in

PMID:
19337156
[PubMed - indexed for MEDLINE]
PMCID:
PMC2866169
Free PMC Article

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