The IC3D Classification of the Corneal Dystrophies

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

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288 Citations (Scopus)


Background: The recent availability of genetic analyses has demonstrated the shortcomings of the current phenotypic method of corneal dystrophy classification. Abnormalities in different genes call Cause a single phenotype, whereas different defects in a single gene can Cause different phenotypes. Sonic 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 I 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 imitations 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 call be retrieved on the website
Original languageUndefined/Unknown
Pages (from-to)S1-S42
Issue number10
Publication statusPublished - 2008

Research programs

  • EMC OR-01-60-01

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