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Anterior Segment > Corneal Dystrophies > Flashcards

Corneal Dystrophies Flashcards

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
Q

treating RCE’s with topical steroids do what

A

inhibits MMPs & promotes intracellular adhesion

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26
Q

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)
A

lattice corneal dystrophy

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27
Q

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
A

lattice corneal dystrophy

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28
Q

when does RCE occur in lattice corneal dystrophy?

A

at end of 1st of decade

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29
Q

what is the most optimal treatment for lattice corneal dystrophy?

can recurrence occur?

A

keratoplasty (can recur in graft tissue)

30
Q

hyaline deposits in corneal stroma is indicative of which corneal dystrophy

A

granular dystrophy

31
Q

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
A

granular dystrophy

32
Q

how would you treat granular dystrophy

A
  • phototherapeutic keratectomy

- keratoplasty if vision becomes significantly impaired

33
Q

which corneal dystrophy contains hyaline & amyloid stromal deposits and typically occurs at the end of the 1st decade of life?

34
Q

refractive surgery is a contradiction of which corneal dystrophy

35
Q

Avellino is a combination of which two dystrophies

A

granular & lattice dystrophy

36
Q

how can you differentiate Avellino from granular dystrophy

A

Avellino deposits are more stellate-like

37
Q

which dystrophy is autosomal recessive

A

macular dystrophy

38
Q

which corneal dystrophy is caused by an aggregation of glycosaminoglycans within the stroma

A

macular dystrophy

39
Q

what symptom of macular dystrophy occurs within the first decade of life

A

decrease in VA

40
Q

what symptom is very common in macular dystrophy

41
Q

how can you treat macular dystrophy?

will there be a recurrence?

A

keratoplasty

recurrence is common

42
Q

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
A

macular dystrophy

43
Q

which dystrophy is a disorder of lipid metabolism & is associated with systemic dyslipidemia

A

Schnyder dystrophy

44
Q

which corneal dystrophy has phospholipid & cholesterol deposits within the corneal stroma

A

Schnyder dystrophy

45
Q

when does Schnyder dystrophy manifest

A

seen as early as 18 months

46
Q

Does Schnyder dystrophy have RCEs

47
Q

a patient with Schnyder dystrophy will have reduced corneal sensation around what time

A

after 50 years

48
Q

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
A

Schnyder dystrophy

49
Q

in Schnyder dystrophy, if VA is severely affected, how would you treat it

A

keratoplasty

50
Q

what is another treatment option for Schnyder dystrophy

A

phototherapeutic keratectomy

51
Q

what are the 6 corneal dystrophies involving the stroma

A

Reis-Bucklers, lattice, granular, avellino, macular, Schnyder

52
Q

what are the 2 corneal dystrophies involving the endothelium

A

posterior polymorphous, Fuch’s

53
Q

which corneal dystrophy is caused by abnormal endothelial cells that transform into stratified squamous epithelium

A

posterior polymorphous

54
Q

in posterior polymorphous, when the abnormal endothelium grows across the trabecular meshwork onto the iris, what will this cause?

A

increase in IOP

55
Q

these signs are indicative of which corneal dystrophy:

  • bilateral & symmetric
  • subtle, vesicular, band-like, or diffuse endothelial lesions
A

posterior polymorphous

56
Q

which corneal dystrophy tends to be sporadic instead of inherited

A

Fuch’s endothelial dystrophy

57
Q

which corneal dystrophy is characterized by bilateral accelerated endothelial cell loss

A

Fuch’s endothelial dystrophy

58
Q

is Fuch’s endothelial dystrophy more common in men or women & occur at what age

A

women > men (3:1)

age-related: 40+

59
Q

the abnormal thickening of Descemet’s membrane & defects in endothelial cell number & shape is indicative of which corneal dystrophy

A

Fuch’s endothelial dystrophy

60
Q

what symptom do pts with Fuch’s endothelial dystrophy have

A

gradual increase in vision blur, especially upon waking up

61
Q

what is the hallmark symptom in Fuch’s endothelial dystrophy

A

vision gets better as the day goes on

62
Q

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
A

Fuch’s endothelial dystrophy

63
Q

what workups can be done to diagnose Fuch’s endothelial dystrophy

A
  • pachymetry
  • specular microscopy
  • anterior segment OCT
64
Q

what does pachymetry do

A

determines corneal thickness

65
Q

what does specular microscopy do

A

endothelial cell count & morphology

66
Q

stromal edema in Fuch’s endothelial dystrophy can lead to what

A

bullous keratopathy (epithelial edema)

67
Q

if Fuch’s endothelial dystrophy is in its early stage, what can you treat it with

A

Muro 128

  • topical drops QID
  • ointment qhs
68
Q

in advanced stage of Fuch’s endothelial dystrophy, how do you treat a ruptured bullae

A

treat epithelial break w/ prophylactic AbX first → bandage soft CLs, cycloplegia

69
Q

with Fuch’s endothelial dystrophy, what should you be cautious of & why

A

cataract surgery → can cause further endothelial decompensation

70
Q

advanced stages of Fuch’s endothelial dystrophy usually require what as treatment

A

keratoplasty

Anterior Segment (37 decks)

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