Cornea Dystrophy (Cale) Flashcards
epithelium anatomy and phsiology
genesis, proliferation/migration, healing, cell junctions, relationship to tear film
stroma anatomy and physiology
generation, transparency, hydrophilic
endothelium anatomy and physiology
energy, ion pumps
dystrophy
developmental and hereditary corneal abnormalities, typically symmetric, resulting from faulty metabolism and/ or structure, usually unrelated to other systemic or local diseases
standard clinical characteristics of corneal dystrophies
- 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
corneal dystrophies are autosomal dominant
50% or more of family will show similar findings. equal gender distribution
onset of corneal findings by age 20 but exception to this rule is
fuch’s endothelial dystrophy
most important clinical characteristic of corneal dystrophies
primary involvement of single corneal layer
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
EBMD
abnormal BM, lacking hemidesmosomes, defects are sporadic along BM. Abnormal attachment of BM to bowmans
EBMD treatment
acute (RCE)vs chronic. abrasion protocol: bandage CL, pressure patch, antibiotic, NSAID, cycloplegic, doxycycline. Lubricants (ung at bedtime), hypertonics
meesmann
rare, intraepithelial cysts identified at 6 months, microcysts can rupture later in life. Tearing, photophobia. Vision minimally affected
intraepithelial cysts
abnormal basal cells and maturation to squamous, thick BM
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
stromal dystrophies
lattice, granular, avellino, macular, schnyders, fleck
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
cracked glass appearance under SL
lattice type I
lattice type II
similar phenotype but not genotype. central cornea sparing, associated with VII n palsy, peripheral neuropathy, amyloidosis
Granular
VA reduction, centrally discrete focal white spots all stromal depths, cornflakes, area between lesions is clear (unknown hyaline like material), RCE is rare
seen earliest of dystrophies
granular (in first decade)
avellino
granular type II. unique to this area of italy, features similar to lattice and granular, same gene locus as lattice and reis-bucklers
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
most severe and least common dystrophy
macular dystrophy
excess glycosaminoglycan and abnormal keratocyte storage of mucopulysaccharide is found in
macular dystrophy
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
Fleck Dystrophy
gray/white opacities odd shaped incidental finding all levels of stroma
endothelial dystrophies
posterior polymorphous (endothelial/desemet's abnormal) fuchs
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)
posterior polymorphous abnormal endothelium grows across
trabeculum into iris. Anterior synechia. Increase IOP. Glaucoma risk
Fuch’s dystrophy
exceptions to dystrophy rule: higher prevalance in females, age related, may extend to periphery, appears as multilayer involvement, not uncommon
corneal edema results in fuch;s dystrophy due to
primary metabolic incompetence of endothelial cells–> endothelial barrier and pump fail
fuch’s dystrophy symptoms
VA reduction in advancing stages and RCE
Clinical features for fuch’s dystrophy
Guttata, stromal edema, epithelial edema
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
guttata vs FUchs
fuch’s has increase number of guttata accompanied by corneal edema
hassal henle bodies
guttata in periphery
stromal edema
hazy in appearance, folds in descemet’s, bullous keratopathy: severe stromal edemal and corneal thickness >30% –> epithelial edema
fuchs treatment and management
pt education, lubricants, hypertonics, RCE treatment protocol, Keratoplasty
congenital hereditary endothelial dystrophy
not typicla dystrophy (AR), present at birth or 1st decade, no guttata, diffuse stromal edema, Tx: poor results with keratoplasty
