Corneal Dystrophies Flashcards
a group of corneal disease that are hereditary & result from faulty metabolism &/or structure
corneal dystrophies
are corneal dystrophies unilateral or bilateral, or both
bilateral
how are corneal dystrophies classified
with respect to the corneal layer that is affected
where are corneal dystrophies primarily located
centrally located
autosomal dominant, rare basement membrane disease with a thickened basement membrane
Meesmann
clinical findings are indicative of which dystrophy
- intraepithelial cysts
- microcysts consisting of degenerated epithelial cell products: appear as tiny, bubble-like, round-oval epithelial opacities
- minimal effect on vision
- normal corneal sensitivity
Meesmann
these signs are indicative of which dystrophy:
- interpalpebral
- slowly progressive
- bilateral & asymmetric
- typically manifests in the first 1-2 years of life
Meesmann
how do you treat Meesmann
lubrication usually sufficient
Meesmann is a dystrophy in which corneal layer
epithelial
which dystrophy is also known as map-dot-fingerprint or Cogan’s dystrophy
EBMD
which dystrophy is the most common corneal dystrophy but is now thought of as a degeneration
EBMD
in EBMD, will there be epithelial staining? when would it stain?
negative staining (stain pools around area) stains when there is RCE involvement
which dystrophy is this
- abnormal formation & maintenance of epithelial basement membrane
- abnormal thickening of BM w/ deposition of fibrillar protein between BM & Bowman’s layer
- deficient basal epithelial cell hemidesmosomes → compensatory aberrant regeneration & duplication of BM
EBMD
what are the 4 pattern types in EBMD, which is most common & which is least common
- maps (most common)
- microcysts
- dots
- fingerprints (least common)
what is the main complication of EBMD
recurrent epithelial erosion
when treating EBMD with RCE what can you not do
do not patch eye → will slow down epithelial healing
EBMD occurs in which layer of the cornea
subepithelial
what dystrophy is an anterior variant of granular stromal dystrophy
Reis-Buckler’s Corneal Dystrophy
when Bowman’s layer is replaced by connective tissue bands what dystrophy occurs
Reis-Buckler’s Corneal Dystrophy
when does Reis-Buckler’s Corneal Dystrophy present & when do symptoms start to present
present at birth → symptomatic within first decade of life
these clinical findings are indicative of which dystrophy
- irregular gray-white geographic-like opacities in Bowman’s layer & anterior stroma
- severe recurrent corneal erosions in childhood, episodes decrease in frequency in 3rd decade
- decreased VA
Reis-Buckler’s Corneal Dystrophy
Reis-Buckler’s Corneal Dystrophy occurs in which corneal layer
stroma
treating RCE with Muro 128 QID does what
may improve epithelial adhesion
why do you treat RCE’s with oral doxycycline
inhibits MMPs
treating RCE’s with topical steroids do what
inhibits MMPs & promotes intracellular adhesion
these clinical findings are indicative of which dystrophy
- amyloid deposits in anterior stroma appear as glass-like branching lines that start in central cornea & spread to the periphery (but spare the limbus)
lattice corneal dystrophy
these symptoms are indicative of which dystrophy
- RCE occur at end of 1st decade
- reduced corneal sensation
- as pts progress in age → increased stromal haze
lattice corneal dystrophy
when does RCE occur in lattice corneal dystrophy?
at end of 1st of decade