1 Corneal Dystrophies What are the four categories

1 Corneal Dystrophies ? ? What are the four categories of corneal dystrophies? ? ?

2 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies What are the four categories of corneal dystrophies? Stromal Dystrophies Endothelial Dystrophies

3 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) ? 2) ? 3) ? 4) ? 5) ? 6) ? Stromal Dystrophies Endothelial Dystrophies What are the six epithelial -stromal TGFBI corneal dystrophies?

4 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies Endothelial Dystrophies What are the six epithelial -stromal TGFBI corneal dystrophies?

5 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) ? 2) ? 3) ? 4) ? 5) ? 6) ? Endothelial Dystrophies What are the six non-TGFBI stromal dystrophies?

6 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies What are the six non-TGFBI stromal dystrophies?

7 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Which stromal dystrophies does the BCSC Cornea book consider the ‘classic 3’? Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies

8 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Which stromal dystrophies does the BCSC Cornea book consider the ‘classic 3’? Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies

9 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What is the well-known mnemonic regarding the classic 3? Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies

10 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What is the well-known mnemonic regarding the classic 3? Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies

11 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What is the well-known mnemonic regarding the classic 3? Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember?

12 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What is the well-known mnemonic regarding the classic 3? Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

13 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Use the mnemonic to identify the names/materials/stains: Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What Marilyn (Start here, and we’ll work Monroe Always our way down the list) Gets granular dystrophy Her hyaline Man Masson trichrome in L well-known lattice dystrophy is the mnemonic A amyloid regarding the classic 3? Red County Congo Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

14 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Use the mnemonic to identify the names/materials/stains: Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What Marilyn macular dystrophy Monroe mucopolysaccharide Always Alcian blue Gets granular dystrophy Her hyaline Man Masson trichrome in L well-known lattice dystrophy is the mnemonic A amyloid regarding the classic 3? Red County Congo Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

15 Macular corneal dystrophy: Mucopolysaccharide: Alcian blue

16 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Use the mnemonic to identify the names/materials/stains: Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What Marilyn macular dystrophy Monroe mucopolysaccharide Always Alcian blue Gets granular dystrophy Her hyaline Man Masson trichrome in L well-known lattice dystrophy is the mnemonic A amyloid regarding the classic 3? Red County Congo Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

17 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Use the mnemonic to identify the names/materials/stains: Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What Marilyn macular dystrophy Monroe mucopolysaccharide Always Alcian blue Gets granular dystrophy Her hyaline Man Masson trichrome in L well-known lattice dystrophy is the mnemonic A amyloid regarding the classic 3? Red County Congo Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

18 Granular corneal dystrophy: Hyaline: Masson trichrome

19 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Use the mnemonic to identify the names/materials/stains: Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What Marilyn macular dystrophy Monroe mucopolysaccharide Always Alcian blue Gets granular dystrophy Her hyaline Man Masson trichrome in L well-known lattice dystrophy is the mnemonic A amyloid regarding the classic 3? Red County Congo Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

20 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Use the mnemonic to identify the names/materials/stains: Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 What Marilyn macular dystrophy Monroe mucopolysaccharide Always Alcian blue Gets granular dystrophy Her hyaline Man Masson trichrome in L well-known lattice dystrophy is the mnemonic A amyloid regarding the classic 3? Red County Congo Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Marilyn Monroe Always Gets Her Man in LA County Endothelial Dystrophies What does the mnemonic help us to remember? The name, abnormal material, and stain for each of the classic 3

21 Lattice corneal dystrophy, type 1 (classic lattice) E. Congo red. F, This same section viewed with polarized light

22 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest ? Lattice Macular ? Granular Lattice ? Macular Granular Least Latest Oldest

23 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest Lattice Macular Granular Least Latest Oldest

24 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest Lattice ? Macular Granular ? Lattice Macular ? Granular Least Latest Oldest

25 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest Lattice Macular Granular Least Latest Oldest

26 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest Lattice ? Granular ? Macular ? Least Latest Oldest

27 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest Lattice Macular Granular Least Latest Oldest

28 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy 3) Congenital stromal corneal dystrophy 4) Fleck corneal dystrophy 5) Posterior amorphous corneal dystrophy 6) Pre-Descemet corneal dystrophy Endothelial Dystrophies Same order as other two, just shifted down by one Note: Same order Big 3 stromal dystrophies: Fill in the table Common? Recur in PK Age of Onset Most Earliest Youngest Lattice Macular Granular Least Latest Oldest

29 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy Give 6 reasons that macular the ‘black sheep’ of the big 3 stromal dystrophies: 3) Congenital stromaliscorneal dystrophy 1) It is AR (others are AD) 4) Fleck corneal dystrophy 2) 5) Posterior amorphous corneal dystrophy 3) The cornea is hazy between lesions (others have clear spaces between lesions) 6) Pre-Descemet corneal dystrophy 4) It’s gene locus is not TGFBI (others are) 5) The cornea tends to thin in macular (others do not) Endothelial Dystrophies 6) It involves the deep stroma, and even the endothelium (others are anterior) 1) Fuchs endothelial corneal dystrophy

30 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy Give 6 reasons that macular the ‘black sheep’ of the big 3 stromal dystrophies: 3) Congenital stromaliscorneal dystrophy 1) It is AR (others are AD) 4) Fleck corneal dystrophy 2) It is limbus-to-limbus (others cleardystrophy peripherally) 5) Posterior amorphous are corneal 3) The cornea is hazy between lesions (others have clear spaces between lesions) 6) Pre-Descemet corneal dystrophy 4) It’s gene locus is not TGFBI (others are) 5) The cornea tends to thin in macular (others do not) Endothelial Dystrophies 6) It involves the deep stroma, and even the endothelium (others are anterior) 1)

31 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy Give 6 reasons that macular the ‘black sheep’ of the big 3 stromal dystrophies: 3) Congenital stromaliscorneal dystrophy 1) It is AR (others are AD) 4) Fleck corneal dystrophy 2) It is limbus-to-limbus (others are clear peripherally) 5) Posterior amorphous corneal dystrophy 3) The cornea is hazy between lesions (others have clear spaces between lesions) 6) Pre-Descemet corneal dystrophy 4) It’s gene locus is not TGFBI (others are) 5) The cornea tends to thin in macular (others do not) Endothelial Dystrophies 6) It involves the deep stroma, and even the endothelium (others are anterior) 1) (See next slide)

Lattice Granular Macular

33 Corneal Dystrophies Epithelial and Subepithelial Dystrophies Epithelial-Stromal TGFBI Dystrophies 1) Reis-Bücklers corneal dystrophy 2) Thiel-Behnke corneal dystrophy 3) Lattice, type 1 4) Lattice, variant types (III, IIIA, I/IIIA, IV) 5) Granular type 1 6) Granular type 2 Stromal Dystrophies 1) Macular corneal dystrophy 2) Schnyder corneal dystrophy Give 6 reasons that macular the ‘black sheep’ of the big 3 stromal dystrophies: 3) Congenital stromaliscorneal dystrophy 1) It is AR (others are AD) 4) Fleck corneal dystrophy 2) It is limbus-to-limbus (others cleardystrophy peripherally) 5) Posterior amorphous are corneal 3) The cornea is hazy between lesions (others have clear spaces between lesions) 6) Pre-Descemet corneal dystrophy 4) It’s gene locus is not TGFBI (others are) 5) The cornea tends to thin in macular (others do not) Endothelial Dystrophies 6) It involves the deep stroma, and even the endothelium (others are anterior) 1) (ditto)

Lattice Granular Macular