Cornea Dystrophy (Cale) Flashcards

1
Q

epithelium anatomy and phsiology

A

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

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2
Q

stroma anatomy and physiology

A

generation, transparency, hydrophilic

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3
Q

endothelium anatomy and physiology

A

energy, ion pumps

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4
Q

dystrophy

A

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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5
Q

standard clinical characteristics of corneal dystrophies

A
  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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6
Q

corneal dystrophies are autosomal dominant

A

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

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7
Q

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

A

fuch’s endothelial dystrophy

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8
Q

most important clinical characteristic of corneal dystrophies

A

primary involvement of single corneal layer

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9
Q

Cogan Microcysitic “map-dot-fingerprint” EBMD

A

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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10
Q

EBMD

A

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

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11
Q

EBMD treatment

A

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

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12
Q

meesmann

A

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

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13
Q

intraepithelial cysts

A

abnormal basal cells and maturation to squamous, thick BM

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14
Q

Reis-Bucklers Dystrophy

A

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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15
Q

stromal dystrophies

A

lattice, granular, avellino, macular, schnyders, fleck

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16
Q

lattice dystrophy (type 1)

A

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

17
Q

cracked glass appearance under SL

A

lattice type I

18
Q

lattice type II

A

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

19
Q

Granular

A

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

20
Q

seen earliest of dystrophies

A

granular (in first decade)

21
Q

avellino

A

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

22
Q

Macular Dystrophy

A

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

23
Q

most severe and least common dystrophy

A

macular dystrophy

24
Q

excess glycosaminoglycan and abnormal keratocyte storage of mucopulysaccharide is found in

A

macular dystrophy

25
Q

Schnyder (crystalline)

A

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

26
Q

Fleck Dystrophy

A

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

27
Q

endothelial dystrophies

A
posterior polymorphous (endothelial/desemet's abnormal)
fuchs
28
Q

posterior polymorphous dystrophy (PPD)

A

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

29
Q

posterior polymorphous abnormal endothelium grows across

A

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

30
Q

Fuch’s dystrophy

A

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

31
Q

corneal edema results in fuch;s dystrophy due to

A

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

32
Q

fuch’s dystrophy symptoms

A

VA reduction in advancing stages and RCE

33
Q

Clinical features for fuch’s dystrophy

A

Guttata, stromal edema, epithelial edema

34
Q

Fuch’s dystrophy assessment

A

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

35
Q

guttata vs FUchs

A

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

36
Q

hassal henle bodies

A

guttata in periphery

37
Q

stromal edema

A

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

38
Q

fuchs treatment and management

A

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

39
Q

congenital hereditary endothelial dystrophy

A

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