Cornea Dystrophy (Cale) Flashcards
(39 cards)
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
