Cornea Dystrophy (Cale) Flashcards Preview

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Flashcards in Cornea Dystrophy (Cale) Deck (39):
1

epithelium anatomy and phsiology

genesis, proliferation/migration, healing, cell junctions, relationship to tear film

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stroma anatomy and physiology

generation, transparency, hydrophilic

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endothelium anatomy and physiology

energy, ion pumps

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dystrophy

developmental and hereditary corneal abnormalities, typically symmetric, resulting from faulty metabolism and/ or structure, usually unrelated to other systemic or local diseases

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standard clinical characteristics of corneal dystrophies

1. autosomal- dominant 2. usual onset of corneal findings by age 20 3. bilateral 4. slowly progressive changes 5. no systemic disease association 6. no primary ocular disease history 7. centrally located 8.. primary involvement of single corneal layer

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corneal dystrophies are autosomal dominant

50% or more of family will show similar findings. equal gender distribution

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onset of corneal findings by age 20 but exception to this rule is

fuch's endothelial dystrophy

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most important clinical characteristic of corneal dystrophies

primary involvement of single corneal layer

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Cogan Microcysitic "map-dot-fingerprint" EBMD

most common corneal dystorphy. considered non-hereditary. age 40-70, male=female, asymptomatic. chronic irritation during day, VA fluctuation, photophobia, glare. *Recurrent corneal erosion or RCE

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EBMD

abnormal BM, lacking hemidesmosomes, defects are sporadic along BM. Abnormal attachment of BM to bowmans

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EBMD treatment

acute (RCE)vs chronic. abrasion protocol: bandage CL, pressure patch, antibiotic, NSAID, cycloplegic, doxycycline. Lubricants (ung at bedtime), hypertonics

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meesmann

rare, intraepithelial cysts identified at 6 months, microcysts can rupture later in life. Tearing, photophobia. Vision minimally affected

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intraepithelial cysts

abnormal basal cells and maturation to squamous, thick BM

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Reis-Bucklers Dystrophy

rare, painful RCE age 5-20, decreasing episodes by age 30, Bowmans layer replaced by fibrocellular tissue (may affect anterior stroma), irregular corneal surface, scarring, decrease VA

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stromal dystrophies

lattice, granular, avellino, macular, schnyders, fleck

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lattice dystrophy (type 1)

age 2-10 onset, VA reduction, RCE common, anterior stromal "inter-lacing" filamentous lesions, white spots, central haze. amyloid deposits, rarely get lattice-like dystrophy secondary to amyloidosis

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cracked glass appearance under SL

lattice type I

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lattice type II

similar phenotype but not genotype. central cornea sparing, associated with VII n palsy, peripheral neuropathy, amyloidosis

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Granular

VA reduction, centrally discrete focal white spots all stromal depths, cornflakes, area between lesions is clear (unknown hyaline like material), RCE is rare

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seen earliest of dystrophies

granular (in first decade)

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avellino

granular type II. unique to this area of italy, features similar to lattice and granular, same gene locus as lattice and reis-bucklers

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Macular Dystrophy

exceptions to dystrophy rule: AR, extends to periphery. VA reduction starts in teens, photophobia, RCE less than Lattice, diffuse ground glass haze lesions, corneal haze between lesions, gray white or milky white opacities throughout stroma and limbus to limbus

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most severe and least common dystrophy

macular dystrophy

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excess glycosaminoglycan and abnormal keratocyte storage of mucopulysaccharide is found in

macular dystrophy

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Schnyder (crystalline)

exception to "dystrophy" rule: associated with systemic hypercholesterolemia (cardiovascular risks), mild VA reduction, No RCE, central haze from annulus, dens arcus ring during 3/4th decades

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Fleck Dystrophy

gray/white opacities odd shaped incidental finding all levels of stroma

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endothelial dystrophies

posterior polymorphous (endothelial/desemet's abnormal)
fuchs

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posterior polymorphous dystrophy (PPD)

asymptomatic, rare reduction in VA (20/30), polymorphous opacities at level of Descemets, maybe corneal edema, glaucoma risk (15% may develope increased IOP)

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posterior polymorphous abnormal endothelium grows across

trabeculum into iris. Anterior synechia. Increase IOP. Glaucoma risk

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Fuch's dystrophy

exceptions to dystrophy rule: higher prevalance in females, age related, may extend to periphery, appears as multilayer involvement, not uncommon

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corneal edema results in fuch;s dystrophy due to

primary metabolic incompetence of endothelial cells--> endothelial barrier and pump fail

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fuch's dystrophy symptoms

VA reduction in advancing stages and RCE

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Clinical features for fuch's dystrophy

Guttata, stromal edema, epithelial edema

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Fuch's dystrophy assessment

it is just guttata or is it now fuch's dystrophy?, stromal and epithelial edema occur with breakdown of endothelial barrier and pump. Pachymetry may help assess degree of corneal thickness

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guttata vs FUchs

fuch's has increase number of guttata accompanied by corneal edema

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hassal henle bodies

guttata in periphery

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stromal edema

hazy in appearance, folds in descemet's, bullous keratopathy: severe stromal edemal and corneal thickness >30% --> epithelial edema

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fuchs treatment and management

pt education, lubricants, hypertonics, RCE treatment protocol, Keratoplasty

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congenital hereditary endothelial dystrophy

not typicla dystrophy (AR), present at birth or 1st decade, no guttata, diffuse stromal edema, Tx: poor results with keratoplasty