This revision of the IC3D classification includes an updated anatomic classification of corneal dystrophies more accurately classifying TGFBI dystrophies that affect multiple layers rather than are confined to one corneal layer. Typical histopathologic and confocal images have been added to the corneal dystrophy templates.

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To describe the results of Descemetstripping automated endothelial keratoplasty (DSAEK) for congenital hereditary endothelial dystrophy (CHED). Methods: The medical records of all patients with CHED who underwent DSAEK at our institution were reviewed. A standard DSAEK was performed in all cases with the exception of the Descemet membrane not being removed in patients younger than 12 months. A thorough ophthalmic examination was performed preoperatively and at each postoperative visit in all patients. Results: Fifteen eyes of 8 patients with phakic eyes (4 male and 4 female) were included. The mean age was 9 years (range, 6 months to 30 years). The average follow-up was 15.9 months (range, 3 to 48 months). There were 4 cases of graft detachment, all of which were managed by rebubbling. All corneas were clear within 1 week after surgery.

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.