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IC3D Classification of Corneal Dystrophies-Edition 3
Jayne S Weiss1, Christopher J Rapuano2, Berthold Seitz3
1Departments of Ophthalmology, Pathology and Pharmacology, Louisiana State University Eye Center of Excellence, Louisiana State University Health Sciences Center, New Orleans, LA.
The third edition of the International Committee for the Classification of Corneal Dystrophies (IC3D) updates corneal dystrophy classification using recent genetic and phenotypic data. This revision refines the categorization of epithelial recurrent erosion dystrophies and lattice corneal dystrophies.
Area of Science:
- Ophthalmology
- Genetics
- Corneal Diseases
Background:
- The International Committee for the Classification of Corneal Dystrophies (IC3D) was established in 2005.
- Its goal is to create a unified classification system for corneal dystrophies.
- This system integrates phenotypic, histopathological, and genetic data.
Purpose of the Study:
- To present the third edition of the IC3D nomenclature for corneal dystrophies.
- To update the classification based on peer-reviewed literature from 2014-2023.
- To refine the anatomic classification and evidence levels for 22 standardized templates.
Main Methods:
- Systematic review of peer-reviewed publications (2014-2023).
- Updating the anatomic classification of corneal dystrophies.
- Revising 22 standardized templates, including evidence categorization (1-4).
Main Results:
- Epithelial recurrent erosion dystrophies updated, including category 1 (COL17A1 mutations).
- Lisch epithelial corneal dystrophy reclassified as autosomal dominant (MCOLN1 mutations).
- New nomenclature proposed for lattice corneal dystrophy (LCD) variants based on TGFBI mutations; punctiform and polychromatic pre-Descemet corneal dystrophy (PPPCD) identified (PRDX3 mutations).
Conclusions:
- The IC3D third edition offers a comprehensive overview of current corneal dystrophy knowledge.
- This update facilitates standardized diagnosis and research.
- The full article is accessible online.
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