Corneal Dystrophies

 

Anterior Cornea

Map-Dot-Fingerprint Dystrophy Most common corneal dystrophy Affects epithelium and anterior basement membrane Asymptomatic OR recurrent erosions Autosomal dominant OR sporadic Abnormal BM adhesion complex Maps = gray geographic opacities Dots = cysts Fingerprints = fine linear subepithelial ridges Treat with ATs and NaCl, micropuncture/laser
Reis-Buckler's Dystrophy Reticular opacities in Bowman's layer Autosomal Dominant Recurrent erosions and decreased vision/scarring 1st to 2nd decade of life Honeycomb-like pattern Bowman's replaced by fibrocellular material Treat with ATs and NaCl, superficial keratectomy (to remove scars)
Meesman Dystrophy Extremely rare, slowly progressive Tiny clear cysts throughout epithelium Autosomal Dominant Erosions, blurred vision, mildly decreased acuity (irregular surface) Slit-lamp findings 1st decade, symptoms 4th to 5th decade "Peculiar Substance" - PAS postive Treat with ATs and NaCL

 

 

 

Stromal Dystrophies

Granular Dystrophy Discrete white anterior stromal deposits with intervening clear spaces Autosomal Dominant No Erosions Slit-lamp findings 1st decade, symptoms mid-life Deposition of hyaline material (Mason's Trichrome stain) Slowly progressive with surprisingly good vision Treat with superficial keratectomy
Lattice Dystrophy Amyloid deposits in stroma create linear opacities Stains with Congo Red Autosomal Dominant Appears in early adolescence with decreased vision by 20-40yo Recurrent erosions Most prominent centrally & progress to periphery Meretoja's Syndrome (Type II lattice dystrophy) Systemic amyloidosis in association with corneal findings Cranial & peripheral neuropathies Skin changes Masklike facies
Macular Dystrophy Mucopolysaccharide deposition in stroma Least common of the three major stromal dystrophies Autosomal Recessive Onset in 1st decade Mild recurrent erosions
Central Crystalline Dystrophy of Schnyder Avascular ring of lipid crystals in central cornea Sytemic hyperlipidemia and prominent arcus senilis Knock-kneed (Genu valgum)
Fleck Dystrophy Diffuse comma-shaped opacities in the stroma Rare Autosomal Dominant Keratocytes have intracytoplasmic vacuoles of glycosaminoglycans and lipids

 

 

 

Posterior Cornea

Fuch's Corneal Dystrophy Common Guttae along Descemet's Loss of endothelial cells leads to corneal edema Treat with NaCl/ointments and decreased IOP
Posterior Polymorphous Membranous Dystrophy Endothelium becomes multilayered and develops epithelial features Bilateral Autosomal Dominant Polymorphous opacities at Descemet's appear as vesicles or broad bands with irregular scalloped edges Iris abnormalities Stationary or slowly progressive Periodic observation and glaucoma management

 

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All pages are Copyright ©2006 by Dennis H. Goldsberry, M.D., P.E.
Reproduction or archival of any protion of these pages is strictly prohibited except by express written permission.