Corneal dystrophies
description
Transcript of Corneal dystrophies
![Page 1: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/1.jpg)
![Page 2: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/2.jpg)
Corneal dystrophies
![Page 3: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/3.jpg)
Corneal dystrophiesGroup of progressive , usually bilateral ,
mostly genetically determined , non inflammatory opacifying disorders
Classification EpithelialBowman layerStromalEndothelial
![Page 4: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/4.jpg)
Epithelial dystrophies
Cogan epithelial basement membrane dystrophy
Meesman epithelial dystrophy
Lisch epithelial dystrophy
![Page 5: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/5.jpg)
Epithelial dystrophies1. Cogan epithelial basement
membrane dystrophyInheritance
SporadicHistology
Thick basement membraneDeposits of fibrillary proteinAbsence of hemidesmosomes
![Page 6: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/6.jpg)
Epithelial dystrophies1. Cogan epithelial basement
membrane dystrophyOnset Signs
Dot like opacitiesEpithelial microcystSubepithelial map like patternWhorled fingerprint – like lines
![Page 7: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/7.jpg)
Epithelial dystrophies2. Meesmann epithelial dystrophy
Inheritance Histology
Basement membrane thickning Intraepithelial cyst
Symptoms Asymptomatic Ocular irritation
![Page 8: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/8.jpg)
Epithelial dystrophies2. Meesmann epithelial dystrophy
Signs Tiny intraepithelial cysts Reduced sensations Slightly thinned
Treatment
![Page 9: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/9.jpg)
Epithelial dystrophies3. Lisch epithelial dystrophy
Inheritance AD or XLD Gene locus on Xp 22.3 in XLD
Signs Grey band with whorls Densely packed microcysts
![Page 10: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/10.jpg)
Bowman layer dystrophies1. Reis – Bucklers dystrophy ( CDB1, GCD type 3)
Inheritance AD Gene locus 5q31
Histology Replacement with fibrous tissue
Onset 1st or 2nd decade
![Page 11: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/11.jpg)
Bowman layer dystrophies1. Reis – Bucklers dystrophy ( CDB1, GCD type 3)
Signs Grey white , fine ,round and polygonal subepithelial opacitiesIncrease with age Reduced corneal sensations
Treatment Directed at recurrent errosionsExcimer laser keratectomy
![Page 12: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/12.jpg)
Bowman layer dystrophies2. Thiel-Behnke dystrophy(CDB2,honeycomb-shaped corneal dystrophy)
Inheritance HistologyOnset
End of 1st decade Recurrent erosions
Signs Honeycomb subepithelial opacity
Treatment
![Page 13: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/13.jpg)
Bowman layer dystrophies3. Schnyder central crystalline dystrophy
Disorder of corneal lipid metabolismInheritanceHistology Onset
2nd decade , visual imparementSigns
Central ,oval, subepithelial crystalline opacity Diffuse corneal haze Prominent arcus in 3rd decade
Treatment
![Page 14: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/14.jpg)
Stromal dystrophies1. Lattice corneal dystrophy type 1(LCD1, Biber-Haab-Dimmer)
Inheritance – AD, 5q31Histology –
Amyloid stain Green birefringence
Onset End of 1st decade
![Page 15: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/15.jpg)
Stromal dystrophies1. Lattice corneal dystrophy type 1
Signs Anterior stromal dotsFine lattice linesSpread Stromal hazeReduced sensations
Treatment PKP or DALK
![Page 16: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/16.jpg)
Stromal dystrophies2. Lattice corneal dystrophy type 2 (LCD2,Finnish type amyloidosis, Meretoja syndrome)
Inheritance – AD , gene locus 9q34Histology – Amyloid deposits in stromaOnset – 2nd decadeSigns – fine lattice lines impaired corneal sensationsTreatment –keratoplasty
![Page 17: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/17.jpg)
Stromal dystrophies3. Lattice corneal dystrophy type 3
Inheritance – ADOnset – 70 – 90 yearsSigns – Thick rope like bands of amyloid
![Page 18: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/18.jpg)
Stromal dystrophies4. Granular corneal dystrophy type 1 (GCD1 , Groenouw type 1)
Inheritance Onset – 1st decadeSigns
Anterior stromal depositsDeeper and outward spreadImpaired corneal sensations
Treatment
![Page 19: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/19.jpg)
Stromal dystrophies5. Granular corneal dystrophy type 2(GCD2, combined granular-lattice dystrophy)
Inheritance Onset – 2nd decadeSigns
Superficial opacitiesDeeper linear opacities
![Page 20: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/20.jpg)
Endothelial dystrophies1. Fuchs endothelial dystrophy
Bilateral accelerated endothelial cell lossMore common in women
Inheritance – ADOnset – Old age, slow progressiveHistology – Guttata
![Page 21: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/21.jpg)
Endothelial dystrophies1. Fuchs endothelial dystrophy
Signs Corneal guttataBeaten metal appearanceStromal edemaEpithelial edemaMicrocyst and bullae
![Page 22: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/22.jpg)
Endothelial dystrophies1. Fuchs endothelial dystrophy
Treatment Conservative options
0.5% topical sodium chlorideCorneal dehydration
Bandage contact lensesPKP or DLEKOther options
Conjunctival flapAmniotic membrane transplant
![Page 23: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/23.jpg)
Endothelial dystrophies2. Posterior polymorphous dystrophy (PPCD)
Innocuos asymptomatic Inheritance – ADOnset - at birthSigns – vesicular endothelial lesion band like or diffuseTreatment
![Page 24: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/24.jpg)
Endothelial dystrophies3. Congenital hereditary endothelial dystrophy
Focal or generalized absence of corneal endothelium
Two main forms CHED1 and CHED2Inheritance Onset – perinatalSigns
Corneal edema Opacification
![Page 25: Corneal dystrophies](https://reader035.fdocuments.us/reader035/viewer/2022062305/568165e9550346895dd90b43/html5/thumbnails/25.jpg)
Thank you