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Newer Classification of Corneal Dystrophies

The International Committee for Classification of Corneal Dystrophies (IC3D) was developed to incorporate the traditional classification of corneal dystrophies with new genetic, clinical, and pathologic information. The anatomic classification continues to group dystrophies according to the predominant corneal layer involved. Each dystrophy carries a template summarizing genetic, clinical, and pathologic information. A category number from 1 through 4 is assigned depicting the level of evidence supporting the existence of the particular dystrophy. Category 1 Gene mapped; Specific mutations known. Category 2 Genetic locus mapped onto a specific chromosome; gene not identified Category 3 Well defined dystrophy; genetic locus not known Category 4 Newly described, suspected dystrophies; not established as distinct entities

The category assigned to a specific corneal dystrophy can be expected to change over time as knowledge advances.Eventually, all valid corneal dystrophies should attain the classification of category 1. Genetics of corneal dystrophy Inheritance ANTERIOR CORNEAL DYSTROPHIES Meesmann dystrophy Meesmann dystrophy Stocker-Holt dystrophy Granular corneal dystrophy type III (Reis-Bcklers dystrophy) Thiel-Behnke dystrophy Thiel-Behnke dystrophy Gelatinous droplike corneal dystrophy Subepithelial mucinous corneal dystrophy Lisch epithelial dystrophy Epithelial recurrent erosion dystrophy CORNEAL STROMAL DYSTROPHIES Macular corneal dystrophy Granular corneal dystrophy type I Granular corneal dystrophy type II Avellino dystrophy Lattice corneal dystrophy type I and variants Lattice corneal dystrophy type II Fleck dystrophy Schnyder corneal dystrophy Posterior amorphous corneal dystrophy Congenital stromal dystrophy POSTERIOR DYSTROPHIES Fuchs dystrophy (early onset) Fuchs dystrophy (late onset) Fuchs dystrophy (late onset) Fuchs dystrophy (late onset) Fuchs dystrophy (late onset) Posterior polymorphous dystrophy type 1 Posterior polymorphous dystrophy type 2 Posterior polymorphous dystrophy type 3 Congenital endothelial dystrophy type 1 Congenital endothelial dystrophy type 2 X-linked endothelial corneal dystrophy AD AD AD AD AD AD AR AD XR AD AR AD AD AD AD AD AD AD AD AD AD AD ? ? AD AD AD AD AR XR Gene locus 12q13 17q12 17q12 5q31 5q31 10q23;q24 1p32 Unknown Xp22.3 Unknown 16q22 5q31 5q31 5q31 9q34 2q35 1p34.1p36 Unknown 12q13.2 1p34.3 13pTel-13q12.13 18q21.2q21.32 20p13-p12 10p11.2 20p11.2 1p34.3-p32.3 10p11.2 20p11.2-q11.2 20p13-p12 Unknown Gene KRT3 KRT12 KRT12 TGFBI TGFBI Unknown TACSTD2 (M1S1) Unknown Unknown Unknown CHST6 TGFBI TGFBI TGFBI GSN PIP5K3 UBIAD1 Unknown DCN COL8A Unknown Unknown SLC4A11 TCF8 Unknown COL8A2 TCF8 Unknown SLC4A11 Unknown IC3D Category 1 1 1 1 1 2 1 4 2 3 1 1 1 1 1 1 1 3 1 1 2 2 1 1 2 1 1 2 1 2

Reference: Weiss JS, Mller HU, Lisch W et al The IC3D classification of the corneal dystrophies. Cornea. 2008 Dec;27 Suppl 2:S1-83.

Ritika Sachdev MS, Noopur Gupta MS, DNB


R.P. Centre for Ophthalmic Sciences, All India Institute of Medical Sciences (AIIMS), New Delhi

www.dosonline.org

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