The IC3D classification of the corneal dystrophies.


Journal Article (Review)

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

Full Text

Cited Authors

  • Weiss, JS; Møller, HU; Lisch, W; Kinoshita, S; Aldave, AJ; Belin, MW; Kivelä, T; Busin, M; Munier, FL; Seitz, B; Sutphin, J; Bredrup, C; Mannis, MJ; Rapuano, CJ; Van Rij, G; Kim, EK; Klintworth, GK

Published Date

  • December 2008

Published In

Volume / Issue

  • 27 Suppl 2 /

Start / End Page

  • S1 - 83

PubMed ID

  • 19337156

Pubmed Central ID

  • 19337156

Electronic International Standard Serial Number (EISSN)

  • 1536-4798

Digital Object Identifier (DOI)

  • 10.1097/ICO.0b013e31817780fb


  • eng spa

Conference Location

  • United States