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Corneal Dystrophies Flashcards

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1
Q

a group of corneal disease that are hereditary & result from faulty metabolism &/or structure

A

corneal dystrophies

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2
Q

are corneal dystrophies unilateral or bilateral, or both

A

bilateral

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3
Q

how are corneal dystrophies classified

A

with respect to the corneal layer that is affected

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4
Q

where are corneal dystrophies primarily located

A

centrally located

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5
Q

autosomal dominant, rare basement membrane disease with a thickened basement membrane

A

Meesmann

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6
Q

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
A

Meesmann

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7
Q

these signs are indicative of which dystrophy:

  • interpalpebral
  • slowly progressive
  • bilateral & asymmetric
  • typically manifests in the first 1-2 years of life
A

Meesmann

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8
Q

how do you treat Meesmann

A

lubrication usually sufficient

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9
Q

Meesmann is a dystrophy in which corneal layer

A

epithelial

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10
Q

which dystrophy is also known as map-dot-fingerprint or Cogan’s dystrophy

A

EBMD

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11
Q

which dystrophy is the most common corneal dystrophy but is now thought of as a degeneration

A

EBMD

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12
Q

in EBMD, will there be epithelial staining? when would it stain?

A 
negative staining (stain pools around area) 
stains when there is RCE involvement
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13
Q

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
A

EBMD

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14
Q

what are the 4 pattern types in EBMD, which is most common & which is least common

A
  • maps (most common)
  • microcysts
  • dots
  • fingerprints (least common)
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15
Q

what is the main complication of EBMD

A

recurrent epithelial erosion

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16
Q

when treating EBMD with RCE what can you not do

A

do not patch eye → will slow down epithelial healing

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17
Q

EBMD occurs in which layer of the cornea

A

subepithelial

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18
Q

what dystrophy is an anterior variant of granular stromal dystrophy

A

Reis-Buckler’s Corneal Dystrophy

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19
Q

when Bowman’s layer is replaced by connective tissue bands what dystrophy occurs

A

Reis-Buckler’s Corneal Dystrophy

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20
Q

when does Reis-Buckler’s Corneal Dystrophy present & when do symptoms start to present

A

present at birth → symptomatic within first decade of life

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21
Q

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
A

Reis-Buckler’s Corneal Dystrophy

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22
Q

Reis-Buckler’s Corneal Dystrophy occurs in which corneal layer

A

stroma

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23
Q

treating RCE with Muro 128 QID does what

A

may improve epithelial adhesion

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24
Q

why do you treat RCE’s with oral doxycycline

A

inhibits MMPs

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25
treating RCE's with topical steroids do what
inhibits MMPs & promotes intracellular adhesion
26
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
27
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
28
when does RCE occur in lattice corneal dystrophy?
at end of 1st of decade
29
what is the most optimal treatment for lattice corneal dystrophy? can recurrence occur?
keratoplasty (can recur in graft tissue)
30
hyaline deposits in corneal stroma is indicative of which corneal dystrophy
granular dystrophy
31
which corneal dystrophy has these presentations: - 1st decade of life - gray-white *crumb-like opacities* in anterior-mid stroma - present in central cornea - asymptomatic in early stages → VA decreases w/ coalescing of opacities - impaired corneal sensation
granular dystrophy
32
how would you treat granular dystrophy
- phototherapeutic keratectomy | - keratoplasty if vision becomes significantly impaired
33
which corneal dystrophy contains hyaline & amyloid stromal deposits and typically occurs at the end of the 1st decade of life?
Avellino
34
refractive surgery is a contradiction of which corneal dystrophy
Avellino
35
Avellino is a combination of which two dystrophies
granular & lattice dystrophy
36
how can you differentiate Avellino from granular dystrophy
Avellino deposits are more stellate-like
37
which dystrophy is autosomal recessive
macular dystrophy
38
which corneal dystrophy is caused by an aggregation of glycosaminoglycans within the stroma
macular dystrophy
39
what symptom of macular dystrophy occurs within the first decade of life
decrease in VA
40
what symptom is very common in macular dystrophy
RCEs
41
how can you treat macular dystrophy? will there be a recurrence?
keratoplasty recurrence is common
42
these signs are indicative of which corneal dystrophy: - dense grayish-white spots located centrally in anterior stroma - no clean delineation between opacities - progression of opacities cause anterior stromal haze - eventual involvement of entire stroma extending to limbus - reduced corneal sensation
macular dystrophy
43
which dystrophy is a disorder of lipid metabolism & is associated with systemic dyslipidemia
Schnyder dystrophy
44
which corneal dystrophy has phospholipid & cholesterol deposits within the corneal stroma
Schnyder dystrophy
45
when does Schnyder dystrophy manifest
seen as early as 18 months
46
Does Schnyder dystrophy have RCEs
no
47
a patient with Schnyder dystrophy will have reduced corneal sensation around what time
after 50 years
48
these signs are indicative of which corneal dystrophy: - central haze that occurs early - subepithelial crystalline deposits present in 50% of pts - prominent corneal arcus that progresses centrally → eventually causing diffuse stromal haze
Schnyder dystrophy
49
in Schnyder dystrophy, if VA is severely affected, how would you treat it
keratoplasty
50
what is another treatment option for Schnyder dystrophy
phototherapeutic keratectomy
51
what are the 6 corneal dystrophies involving the stroma
Reis-Bucklers, lattice, granular, avellino, macular, Schnyder
52
what are the 2 corneal dystrophies involving the endothelium
posterior polymorphous, Fuch's
53
which corneal dystrophy is caused by abnormal endothelial cells that transform into stratified squamous epithelium
posterior polymorphous
54
in posterior polymorphous, when the abnormal endothelium grows across the trabecular meshwork onto the iris, what will this cause?
increase in IOP
55
these signs are indicative of which corneal dystrophy: - bilateral & symmetric - subtle, vesicular, band-like, or diffuse endothelial lesions
posterior polymorphous
56
which corneal dystrophy tends to be sporadic instead of inherited
Fuch's endothelial dystrophy
57
which corneal dystrophy is characterized by bilateral accelerated endothelial cell loss
Fuch's endothelial dystrophy
58
is Fuch's endothelial dystrophy more common in men or women & occur at what age
women > men (3:1) | age-related: 40+
59
the abnormal thickening of Descemet's membrane & defects in endothelial cell number & shape is indicative of which corneal dystrophy
Fuch's endothelial dystrophy
60
what symptom do pts with Fuch's endothelial dystrophy have
gradual increase in vision blur, especially upon waking up
61
what is the hallmark symptom in Fuch's endothelial dystrophy
vision gets better as the day goes on
62
these signs are indicative of which corneal dystrophy: - corneal guttate: presence of abnormal outgrowth of Descemet's - specular reflection: dark spots caused by disruption of regular endothelial mosaic (beaten metal appearance) - endothelial decompensation → central stromal edema - in advanced stage of disease → epithelial edema can develop & form a bullae
Fuch's endothelial dystrophy
63
what workups can be done to diagnose Fuch's endothelial dystrophy
- pachymetry - specular microscopy - anterior segment OCT
64
what does pachymetry do
determines corneal thickness
65
what does specular microscopy do
endothelial cell count & morphology
66
stromal edema in Fuch's endothelial dystrophy can lead to what
bullous keratopathy (epithelial edema)
67
if Fuch's endothelial dystrophy is in its early stage, what can you treat it with
Muro 128 - topical drops QID - ointment qhs
68
in advanced stage of Fuch's endothelial dystrophy, how do you treat a ruptured bullae
treat epithelial break w/ prophylactic AbX first → bandage soft CLs, cycloplegia
69
with Fuch's endothelial dystrophy, what should you be cautious of & why
cataract surgery → can cause further endothelial decompensation
70
advanced stages of Fuch's endothelial dystrophy usually require what as treatment
keratoplasty

Anterior Segment (37 decks)

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